MedWorm.com provides a medical RSS filtering service. Over 6000 RSS medical sources are combined and output via different filters. This feed contains the latest items from the 'Brain' source. Sat, 20 Mar 2010 14:40:36 +0100 http://www.medworm.com/index.php?rid=3384462&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F945%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384462 Sat, 20 Mar 2010 05:20:01 +0100 3384462 http://www.medworm.com/index.php?rid=3384461&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F941%3Frss%3D1 New clinical and employment information, together with over-looked previously published information, on a patient (H.C.) is reviewed. H.C., who died at the age of 76 in 1939, was found, by chance during anatomical dissection, to lack a cerebellum. This synthesis challenges an unusual and interesting account of cerebellar agenesis published in Brain in 1994 by Glickstein (see also Glickstein, 2006), in which the allegedly ‘bogus’ oral history of this individual's motor skills was held to have led to ‘medical myth making’. Part of the burden of the 1994 paper was to show that ‘cerebellar agenesis is always associated with profound motor deficits’. Glickstein therefore focussed on an apparent ‘exception’ to this conclusion, concerning the brain ... Brain journals http://www.medworm.com/rss/comments.php?id=3384461 Sat, 20 Mar 2010 05:20:01 +0100 3384461 http://www.medworm.com/index.php?rid=3384460&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F923%3Frss%3D1 The inferior olivary nuclei clearly play a role in creating oculopalatal tremor, but the exact mechanism is unknown. Oculopalatal tremor develops some time after a lesion in the brain that interrupts inhibition of the inferior olive by the deep cerebellar nuclei. Over time the inferior olive gradually becomes hypertrophic and its neurons enlarge developing abnormal soma-somatic gap junctions. However, results from several experimental studies have confounded the issue because they seem inconsistent with a role for the inferior olive in oculopalatal tremor, or because they ascribe the tremor to other brain areas. Here we look at 3D binocular eye movements in 15 oculopalatal tremor patients and compare their behaviour to the output of our recent mathematical model of oculopalatal tremor. Thi... Brain journals http://www.medworm.com/rss/comments.php?id=3384460 Sat, 20 Mar 2010 05:20:01 +0100 3384460 http://www.medworm.com/index.php?rid=3384459&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F915%3Frss%3D1 In conclusion, limb-shaking transient ischaemic attacks in patients with internal carotid artery occlusion can be recognized by their short duration, are often accompanied by paresis and precipitated by rising or exercise and are indicative of an impaired haemodynamic state of the brain. (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384459 Sat, 20 Mar 2010 05:20:01 +0100 3384459 http://www.medworm.com/index.php?rid=3384458&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F909%3Frss%3D1 Conjugate eye deviation describes the tonic horizontal deviation of the eyes in acute stroke patients. Here we investigate whether measuring patients’ eye-in-head position in clinical magnetic resonance imaging or computed tomography scans obtained at admission shows a specific relationship to spatial neglect. We investigated 124 continuously admitted subjects with unilateral, first-ever left- or right-sided stroke. To control for the possibility that the degree of eye deviation is related to lesion size rather than spatial neglect, overall lesion volume was used as a covariate in the statistical analysis. Horizontal eye-in-head deviation on clinical brain scans appeared to be associated with spatial neglect rather than with brain damage per se. In contrast to the subject groups with... Brain journals http://www.medworm.com/rss/comments.php?id=3384458 Sat, 20 Mar 2010 05:20:01 +0100 3384458 http://www.medworm.com/index.php?rid=3384457&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F895%3Frss%3D1 Unilateral neglect is a disabling syndrome frequently observed following right hemisphere brain damage. Symptoms range from visuo-motor impairments through to deficient visuo-spatial imagery, but impairment can also affect the auditory modality. A short period of adaptation to a rightward prismatic shift of the visual field is known to improve a wide range of hemispatial neglect symptoms, including visuo-manual tasks, mental imagery, postural imbalance, visuo-verbal measures and number bisection. The aim of the present study was to assess whether the beneficial effects of prism adaptation may generalize to auditory manifestations of neglect. Auditory extinction, whose clinical manifestations are independent of the sensory modalities engaged in visuo-manual adaptation, was examined in negle... Brain journals http://www.medworm.com/rss/comments.php?id=3384457 Sat, 20 Mar 2010 05:20:01 +0100 3384457 http://www.medworm.com/index.php?rid=3384456&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F880%3Frss%3D1 Spatial neglect is a perplexing neuropsychological syndrome, in which patients fail to detect (and/or respond to) stimuli located contralaterally to their (most often right) hemispheric lesion. Neglect is characterized by a wide heterogeneity, and a role for multiple components has been suggested, but the exact nature of the critical components remains unclear. Moreover, many different lesion sites have been reported, leading to enduring controversies about the relative contribution of different cortical and/or subcortical brain regions. Here we report a systematic anatomo-functional study of 80 patients with a focal right hemisphere stroke, who were examined by a series of neuropsychological tests assessing different clinical manifestations of neglect. We first performed a statistical fac... Brain journals http://www.medworm.com/rss/comments.php?id=3384456 Sat, 20 Mar 2010 05:20:01 +0100 3384456 http://www.medworm.com/index.php?rid=3384455&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F868%3Frss%3D1 Learning to associate auditory information of speech sounds with visual information of letters is a first and critical step for becoming a skilled reader in alphabetic languages. Nevertheless, it remains largely unknown which brain areas subserve the learning and automation of such associations. Here, we employ functional magnetic resonance imaging to study letter–speech sound integration in children with and without developmental dyslexia. The results demonstrate that dyslexic children show reduced neural integration of letters and speech sounds in the planum temporale/Heschl sulcus and the superior temporal sulcus. While cortical responses to speech sounds in fluent readers were modulated by letter–speech sound congruency with strong suppression effects for incongruent letter... Brain journals http://www.medworm.com/rss/comments.php?id=3384455 Sat, 20 Mar 2010 05:20:01 +0100 3384455 http://www.medworm.com/index.php?rid=3384454&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F858%3Frss%3D1 In this study, we used T2-weighted magnetic resonance imaging at 9.4 T to detect grey matter lesions in fixed post-mortem multiple sclerosis motor cortex. Furthermore, we produced T1, T2 and magnetization transfer ratio maps, and correlated these indices with quantitative histology [neuronal density, intensity of immunostaining for myelin basic protein (reflecting myelin content) and phosphorylated neurofilament (reflecting axonal area)] using t-tests and multivariate regression. In 21 tissue samples, 28 cortical grey matter lesions were visible on both T2-weighted magnetic resonance imaging and sections immunostained for myelin basic protein, 15/28 being mixed white and grey matter and 11/28 subpial cortical grey matter lesions; 2/28 cortical grey matter lesions involved all layers of the... Brain journals http://www.medworm.com/rss/comments.php?id=3384454 Sat, 20 Mar 2010 05:20:01 +0100 3384454 http://www.medworm.com/index.php?rid=3384453&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F847%3Frss%3D1 This study shows the feasibility of using in vivo sodium magnetic resonance imaging at 3 T in patients with multiple sclerosis. Our findings suggest that the abnormal values of the tissue sodium concentration in patients with relapsing–remitting multiple sclerosis might reflect changes in cellular composition of the lesions and/or changes in cellular and metabolic integrity. Sodium magnetic resonance imaging has the potential to provide insight into the pathophysiological mechanisms of tissue injury when correlation with histopathology becomes available. (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384453 Sat, 20 Mar 2010 05:20:00 +0100 3384453 http://www.medworm.com/index.php?rid=3384452&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F835%3Frss%3D1 Astrocytes are prominent participants in the response of the central nervous system to injury, including neuroinflammatory insults. Rodent astrocytes in vitro have been shown to express voltage-gated sodium channels in a dynamic manner, with a switch in expression of tetrodotoxin-sensitive to tetrodotoxin-resistant channels in reactive astrocytes. However, the expression of sodium channels in human astrocytes has not been studied, and it is not known whether there are changes in the expression of sodium channels in reactive astrocytes of the human central nervous system. Here, we demonstrate a focal and robust upregulation of sodium channel Nav1.5 in reactive astrocytes at the borders of, and within, active and chronic multiple sclerosis lesions. Nav1.5 was only detectable at very low leve... Brain journals http://www.medworm.com/rss/comments.php?id=3384452 Sat, 20 Mar 2010 05:20:00 +0100 3384452 http://www.medworm.com/index.php?rid=3384451&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F822%3Frss%3D1 Oligodendrocyte loss and demyelination are major pathological hallmarks of multiple sclerosis. In pattern III lesions, inflammation is minor in the early stages, and oligodendrocyte apoptosis prevails, which appears to be mediated at least in part through mitochondrial injury. Here, we demonstrate poly(ADP-ribose) polymerase activation and apoptosis inducing factor nuclear translocation within apoptotic oligodendrocytes in such multiple sclerosis lesions. The same morphological and molecular pathology was observed in an experimental model of primary demyelination, induced by the mitochondrial toxin cuprizone. Inhibition of poly(ADP-ribose) polymerase in this model attenuated oligodendrocyte depletion and decreased demyelination. Poly(ADP-ribose) polymerase inhibition suppressed c-Jun N-ter... Brain journals http://www.medworm.com/rss/comments.php?id=3384451 Sat, 20 Mar 2010 05:20:00 +0100 3384451 http://www.medworm.com/index.php?rid=3384450&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F808%3Frss%3D1 Microglia, the innate immune cells in the brain, can become chronically activated in response to dopaminergic neuron death, fuelling a self-renewing cycle of microglial activation followed by further neuron damage (reactive microgliosis), which is implicated in the progressive nature of Parkinson’s disease. Here, we use an in vitro approach to separate neuron injury factors from the cellular actors of reactive microgliosis and discover molecular signals responsible for chronic and toxic microglial activation. Upon injury with the dopaminergic neurotoxin 1-methyl-4-phenylpyridinium, N27 cells (dopaminergic neuron cell line) released soluble neuron injury factors that activated microglia and were selectively toxic to dopaminergic neurons in mixed mesencephalic neuron-glia cultures thro... Brain journals http://www.medworm.com/rss/comments.php?id=3384450 Sat, 20 Mar 2010 05:20:00 +0100 3384450 http://www.medworm.com/index.php?rid=3384449&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F797%3Frss%3D1 Mutations of mitochondrial DNA are associated with a wide spectrum of disorders, primarily affecting the central nervous system and muscle function. The specific consequences of mitochondrial DNA mutations for neuronal pathophysiology are not understood. In order to explore the impact of mitochondrial mutations on neuronal biochemistry and physiology, we have used fluorescence imaging techniques to examine changes in mitochondrial function in neurons differentiated from mouse embryonic stem-cell cybrids containing mitochondrial DNA polymorphic variants or mutations. Surprisingly, in neurons carrying a severe mutation in respiratory complex I (<10% residual complex I activity) the mitochondrial membrane potential was significantly increased, but collapsed in response to oligomycin, sugge... Brain journals http://www.medworm.com/rss/comments.php?id=3384449 Sat, 20 Mar 2010 05:20:00 +0100 3384449 http://www.medworm.com/index.php?rid=3384448&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F787%3Frss%3D1 Mutations in the mitochondrial genome are associated with a wide range of neurological symptoms, but many aspects of the basic neuronal pathology are not understood. One candidate mechanism, given the well-established role of mitochondria in calcium buffering, is a deficit in neuronal calcium homoeostasis. We therefore examined calcium responses in the neurons derived from various ‘cybrid’ embryonic stem cell lines carrying different mitochondrial DNA mutations. Brief (~50 ms), focal glutamatergic stimuli induced a transient rise in intracellular calcium concentration, which was visualized by bulk loading the cells with the calcium dye, Oregon Green BAPTA-1. Calcium entered the neurons through N-methyl-d-aspartic acid and voltage-gated calcium channels, as has been described in... Brain journals http://www.medworm.com/rss/comments.php?id=3384448 Sat, 20 Mar 2010 05:20:00 +0100 3384448 http://www.medworm.com/index.php?rid=3384447&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F771%3Frss%3D1 Additional neurological features have recently been described in seven families transmitting pathogenic mutations in OPA1, the most common cause of autosomal dominant optic atrophy. However, the frequency of these syndromal ‘dominant optic atrophy plus’ variants and the extent of neurological involvement have not been established. In this large multi-centre study of 104 patients from 45 independent families, including 60 new cases, we show that extra-ocular neurological complications are common in OPA1 disease, and affect up to 20% of all mutational carriers. Bilateral sensorineural deafness beginning in late childhood and early adulthood was a prominent manifestation, followed by a combination of ataxia, myopathy, peripheral neuropathy and progressive external ophthalmoplegia ... Brain journals http://www.medworm.com/rss/comments.php?id=3384447 Sat, 20 Mar 2010 05:20:00 +0100 3384447 http://www.medworm.com/index.php?rid=3384446&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F762%3Frss%3D1 This study aimed to assess thermal and mechanical perception and pain thresholds in primary idiopathic restless legs syndrome and secondary restless legs syndrome associated with small fibre neuropathy. Twenty-one patients (age: 53.4 ± 8.4, n = 3, male) with primary restless legs syndrome and 13 patients (age: 63.0 ± 8.2, n = 1, male) with secondary restless legs syndrome associated with small fibre neuropathy were compared with 20 healthy subjects (age: 58.0 ± 7.0; n = 2, male). Differential diagnosis of secondary restless legs syndrome associated with small fibre neuropathy was based on clinical symptoms and confirmed with skin biopsies in all patients. A comprehensive quantitative sensory testing protocol encompassing thermal and mechanical detection and pain thresh... Brain journals http://www.medworm.com/rss/comments.php?id=3384446 Sat, 20 Mar 2010 05:20:00 +0100 3384446 http://www.medworm.com/index.php?rid=3384445&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F746%3Frss%3D1 Deep brain stimulation in the subthalamic nucleus is an effective and safe surgical procedure that has been shown to reduce the motor dysfunction of patients with advanced Parkinson’s disease. Bilateral subthalamic nucleus deep brain stimulation, however, has been associated with declines in cognitive and cognitive–motor functioning. It has been hypothesized that spread of current to nonmotor areas of the subthalamic nucleus may be responsible for declines in cognitive and cognitive–motor functioning. The aim of this study was to assess the cognitive–motor performance in advanced Parkinson’s disease patients with subthalamic nucleus deep brain stimulation parameters determined clinically (Clinical) to settings derived from a patient-specific computational mode... Brain journals http://www.medworm.com/rss/comments.php?id=3384445 Sat, 20 Mar 2010 05:20:00 +0100 3384445 http://www.medworm.com/index.php?rid=3384444&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F727%3Frss%3D1 Patients with Parkinson’s disease experience motor and perceptual timing difficulties, which are ameliorated by dopaminergic medication. We investigated the neural correlates of motor timing in Parkinson’s disease, including the effects of dopaminergic medication on patterns of brain activation. Eight patients with Parkinson’s disease and eight healthy controls were scanned with H152 positron emission tomography while engaged in three tasks: synchronization (right index finger tapping in synchrony with a tone presented at 1 Hz), continuation (tapping at 1 Hz in the absence of a tone), and a control simple reaction time task. During the first 6 scans, the patients were assessed after overnight withdrawal of medication. Scans 7–12 were completed with the patients in t... Brain journals http://www.medworm.com/rss/comments.php?id=3384444 Sat, 20 Mar 2010 05:20:00 +0100 3384444 http://www.medworm.com/index.php?rid=3384443&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F713%3Frss%3D1 In this study, the nature of DJ-1 and -synuclein in human cerebrospinal fluid was studied by a combination of western blotting, gel filtration and mass spectrometry. Sensitive and quantitative Luminex assays detecting most, if not all, species of DJ-1 and -synuclein in human cerebrospinal fluid were established. Cerebrospinal fluid concentrations of DJ-1 and -synuclein from 117 patients with Parkinson’s disease, 132 healthy individuals and 50 patients with Alzheimer’s disease were analysed using newly developed, highly sensitive Luminex technology while controlling for several major confounders. A total of 299 individuals and 389 samples were analysed. The results showed that cerebrospinal fluid DJ-1 and -synuclein levels were dependent on age and influenced by the extent of bl... Brain journals http://www.medworm.com/rss/comments.php?id=3384443 Sat, 20 Mar 2010 05:20:00 +0100 3384443 http://www.medworm.com/index.php?rid=3384442&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F701%3Frss%3D1 Neurodegeneration with brain iron accumulation encompasses a heterogeneous group of rare neurodegenerative disorders that are characterized by iron accumulation in the brain. Severe generalized dystonia is frequently a prominent symptom and can be very disabling, causing gait impairment, difficulty with speech and swallowing, pain and respiratory distress. Several case reports and one case series have been published concerning therapeutic outcome of pallidal deep brain stimulation in dystonia caused by neurodegeneration with brain iron degeneration, reporting mostly favourable outcomes. However, with case studies, there may be a reporting bias towards favourable outcome. Thus, we undertook this multi-centre retrospective study to gather worldwide experiences with bilateral pallidal deep br... Brain journals http://www.medworm.com/rss/comments.php?id=3384442 Sat, 20 Mar 2010 05:20:00 +0100 3384442 http://www.medworm.com/index.php?rid=3384441&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F690%3Frss%3D1 Neurophysiological studies have provided evidence of primary motor cortex hyperexcitability in primary dystonia, but several functional imaging studies suggest otherwise. To address this issue, we measured sensorimotor activation at both the regional and network levels in carriers of the DYT1 dystonia mutation and in control subjects. We used 15Oxygen-labelled water and positron emission tomography to scan nine manifesting DYT1 carriers, 10 non-manifesting DYT1 carriers and 12 age-matched controls while they performed a kinematically controlled motor task; they were also scanned in a non-motor audio-visual control condition. Within- and between-group contrasts were analysed with statistical parametric mapping. For network analysis, we first identified a normal motor-related activation patt... Brain journals http://www.medworm.com/rss/comments.php?id=3384441 Sat, 20 Mar 2010 05:20:00 +0100 3384441 http://www.medworm.com/index.php?rid=3384440&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F671%3Frss%3D1 Lesch–Nyhan disease is a neurogenetic disorder caused by deficiency of the enzyme hypoxanthine–guanine phosphoribosyltransferase. The classic form of the disease is described by a characteristic syndrome that includes overproduction of uric acid, severe generalized dystonia, cognitive disability and self-injurious behaviour. In addition to the classic disease, variant forms of the disease occur wherein some clinical features are absent or unusually mild. The current studies provide the results of a prospective and multi-centre international study focusing on neurological manifestations of the largest cohort of Lesch–Nyhan disease variants evaluated to date, with 46 patients from 3 to 65 years of age coming from 34 families. All had evidence for overproduction of uric acid... Brain journals http://www.medworm.com/rss/comments.php?id=3384440 Sat, 20 Mar 2010 05:20:00 +0100 3384440 http://www.medworm.com/index.php?rid=3384439&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F655%3Frss%3D1 In conclusion, a substantial number of the patients with glucose transporter-1 deficiency syndrome do not have epilepsy. Our study demonstrates that a lumbar puncture provides the diagnostic clue to glucose transporter-1 deficiency syndrome and can thereby dramatically reduce diagnostic delay to allow early start of the ketogenic diet. (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384439 Sat, 20 Mar 2010 05:20:00 +0100 3384439 http://www.medworm.com/index.php?rid=3384438&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F652%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384438 Sat, 20 Mar 2010 05:20:00 +0100 3384438 http://www.medworm.com/index.php?rid=3384437&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F651%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384437 Sat, 20 Mar 2010 05:20:00 +0100 3384437 http://www.medworm.com/index.php?rid=3384436&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F649%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384436 Sat, 20 Mar 2010 05:20:00 +0100 3384436 http://www.medworm.com/index.php?rid=3384435&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F645%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384435 Sat, 20 Mar 2010 05:20:00 +0100 3384435 http://www.medworm.com/index.php?rid=3384434&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F3%2F643%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3384434 Sat, 20 Mar 2010 05:20:00 +0100 3384434 http://www.medworm.com/index.php?rid=3274171&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F638%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3274171 Tue, 16 Feb 2010 03:54:46 +0100 3274171 http://www.medworm.com/index.php?rid=3274170&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F625%3Frss%3D1 Patients with schizophrenia consistently show deficient performance on tasks requiring volitional saccades. We previously reported reduced fractional anisotropy in the white matter underlying right dorsal anterior cingulate cortex in schizophrenia, which, along with lower fractional anisotropy in the right frontal eye field and posterior parietal cortex, predicted longer latencies of volitional saccades. This suggests that reduced microstructural integrity of dorsal anterior cingulate cortex white matter disrupts connectivity in the right hemisphere-dominant network for spatial attention and volitional ocular motor control. To test this hypothesis, we examined functional connectivity of the cingulate eye field component of this network, which is located in dorsal anterior cingulate cortex,... Brain journals http://www.medworm.com/rss/comments.php?id=3274170 Tue, 16 Feb 2010 03:54:46 +0100 3274170 http://www.medworm.com/index.php?rid=3274169&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F611%3Frss%3D1 The ‘self’ is a complex multidimensional construct deeply embedded and in many ways defined by our relations with the social world. Individuals with autism are impaired in both self-referential and other-referential social cognitive processing. Atypical neural representation of the self may be a key to understanding the nature of such impairments. Using functional magnetic resonance imaging we scanned adult males with an autism spectrum condition and age and IQ-matched neurotypical males while they made reflective mentalizing or physical judgements about themselves or the British Queen. Neurotypical individuals preferentially recruit the middle cingulate cortex and ventromedial prefrontal cortex in response to self compared with other-referential processing. In autism, ventrome... Brain journals http://www.medworm.com/rss/comments.php?id=3274169 Tue, 16 Feb 2010 03:54:46 +0100 3274169 http://www.medworm.com/index.php?rid=3274168&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F599%3Frss%3D1 Several groups have recently reported that people with autism may suffer from a deficit in visual motion processing and proposed that these deficits may be related to a general dorsal stream dysfunction. In order to test the dorsal stream deficit hypothesis, we investigated coherent and biological motion perception as well as coherent form perception in a group of adolescents with autism and a group of age-matched typically developing controls. If the dorsal stream hypothesis were true, we would expect to document deficits in both coherent and biological motion processing in this group but find no deficit in coherent form perception. Using the method of constant stimuli and standard psychophysical analysis techniques, we measured thresholds for coherent motion, biological motion and cohere... Brain journals http://www.medworm.com/rss/comments.php?id=3274168 Tue, 16 Feb 2010 03:54:46 +0100 3274168 http://www.medworm.com/index.php?rid=3274167&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F591%3Frss%3D1 The mutation of the spatacsin gene is the single most common cause of autosomal recessive hereditary spastic paraplegia with thin corpus callosum. Common clinical, pathological and genetic features between amyotrophic lateral sclerosis and hereditary spastic paraplegia motivated us to investigate 25 families with autosomal recessive juvenile amyotrophic lateral sclerosis and long-term survival for mutations in the spatascin gene. The inclusion criterion was a diagnosis of clinically definite amyotrophic lateral sclerosis according to the revised El Escorial criteria. The exclusion criterion was a diagnosis of hereditary spastic paraplegia with thin corpus callosum in line with an established protocol. Additional pathological and genetic evaluations were also performed. Surprisingly, 12 seq... Brain journals http://www.medworm.com/rss/comments.php?id=3274167 Tue, 16 Feb 2010 03:54:46 +0100 3274167 http://www.medworm.com/index.php?rid=3274166&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F580%3Frss%3D1 Gilles de la Tourette syndrome is a neuropsychiatric disorder in which cortical disinhibition has been proposed as a pathophysiological mechanism involved in the generation of tics. Tics are typically reduced during task performance and concentration. How this task-dependent reduction of motor symptoms is represented in the brain is not yet understood. The aim of the current research was to study motorcortical excitability at rest and during the preparation of a simple motor task. Transcranial magnetic stimulation was used to examine corticospinal excitability, short-interval intracortical inhibition and intracortical facilitation in a group of 11 patients with Gilles de la Tourette syndrome and age-matched healthy controls. Parameters of cortical excitability were evaluated at rest and at... Brain journals http://www.medworm.com/rss/comments.php?id=3274166 Tue, 16 Feb 2010 03:54:46 +0100 3274166 http://www.medworm.com/index.php?rid=3274165&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F568%3Frss%3D1 Rapid eye movement sleep behaviour disorder is characterized by dream-enacting behaviour and impaired motor inhibition during rapid eye movement sleep. Rapid eye movement sleep behaviour disorder is commonly associated with neurodegenerative disorders, but also reported in narcolepsy with cataplexy. Most narcolepsy with cataplexy patients lack the sleep–wake, and rapid eye movement sleep, motor-regulating hypocretin neurons in the lateral hypothalamus. In contrast, rapid eye movement sleep behaviour disorder and hypocretin deficiency are rare in narcolepsy without cataplexy. We hypothesized that rapid eye movement sleep behaviour disorder coexists with cataplexy in narcolepsy due to hypocretin deficiency. In our study, rapid eye movement sleep behaviour disorder was diagnosed by the ... Brain journals http://www.medworm.com/rss/comments.php?id=3274165 Tue, 16 Feb 2010 03:54:46 +0100 3274165 http://www.medworm.com/index.php?rid=3274164&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F557%3Frss%3D1 This study revealed that different psychotic symptoms in dementia with Lewy bodies were associated with distinguishable cerebral networks. Visual hallucinations were related to dysfunction of the parietal and occipital association cortices, misidentifications were related to dysfunction of the limbic-paralimbic structures and delusions were related to dysfunction of the frontal cortices. Our findings provide important insights into the pathophysiological mechanisms underlying psychotic symptoms in dementia with Lewy bodies. (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3274164 Tue, 16 Feb 2010 03:54:46 +0100 3274164 http://www.medworm.com/index.php?rid=3274163&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F540%3Frss%3D1 There are little data on the relationship between Lewy body disease and mild cognitive impairment syndromes. The Mayo Clinic aging and dementia databases in Rochester, Minnesota, and Jacksonville, Florida were queried for cases who were diagnosed with mild cognitive impairment between 1 January 1996 and 30 April 2008, were prospectively followed and were subsequently found to have autopsy-proven Lewy body disease. The presence of rapid eye movement sleep behaviour disorder was specifically assessed. Mild cognitive impairment subtypes were determined by clinical impression and neuropsychological profiles, based on prospective operational criteria. The diagnosis of clinically probable dementia with Lewy bodies was based on the 2005 McKeith criteria. Hippocampal volumes, rate of hippocampal a... Brain journals http://www.medworm.com/rss/comments.php?id=3274163 Tue, 16 Feb 2010 03:54:46 +0100 3274163 http://www.medworm.com/index.php?rid=3274162&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F529%3Frss%3D1 In this study, therefore, we undertook a comprehensive investigation of diffusion behaviour in Alzheimer's disease by analysing each of the component eigenvalues of the diffusion tensor in isolation to test the hypothesis that early Alzheimer's disease is associated with degeneration of a specific neural network. Using tract-based spatial statistics, we performed voxel-wise analyses of fractional anisotropy, axial, radial and mean diffusivities in 25 Alzheimer's disease patients compared with 13 elderly controls. We found that increased absolute (axial, radial and mean) diffusivities in Alzheimer's disease were concordant in a distribution consistent with the network hypothesis, highly statistically significant and far more sensitive than fractional anisotropy reductions. The former three ... Brain journals http://www.medworm.com/rss/comments.php?id=3274162 Tue, 16 Feb 2010 03:54:46 +0100 3274162 http://www.medworm.com/index.php?rid=3274161&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F512%3Frss%3D1 Patients with early age-of-onset Alzheimer’s disease show more rapid progression, more generalized cognitive deficits and greater cortical atrophy and hypometabolism compared to late-onset patients at a similar disease stage. The biological mechanisms that underlie these differences are not well understood. The purpose of this study was to examine in vivo whether metabolic differences between early-onset and late-onset Alzheimer’s disease are associated with differences in the distribution and burden of fibrillar amyloid-β. Patients meeting criteria for probable Alzheimer’s disease (National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's; Disease and Related Disorders Association criteria) were divided based on estimated age at f... Brain journals http://www.medworm.com/rss/comments.php?id=3274161 Tue, 16 Feb 2010 03:54:45 +0100 3274161 http://www.medworm.com/index.php?rid=3274160&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F496%3Frss%3D1 Viral vector-mediated gene transfer utilizing adeno-associated viral vectors has recently entered clinical testing as a novel tool for delivery of therapeutic agents to the brain. Clinical trials in Parkinson’s disease using adeno-associated viral vector-based gene therapy have shown the safety of the approach. Further efforts in this area will show if gene-based approaches can rival the therapeutic efficacy achieved with the best pharmacological therapy or other, already established, surgical interventions. One of the strategies under development for clinical application is continuous 3,4-dihydroxyphenylalanine delivery. This approach has been shown to be efficient in restoring motor function and reducing established dyskinesias in rats with a partial lesion of the nigrostriatal dop... Brain journals http://www.medworm.com/rss/comments.php?id=3274160 Tue, 16 Feb 2010 03:54:45 +0100 3274160 http://www.medworm.com/index.php?rid=3274159&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F482%3Frss%3D1 Grafts of foetal ventral mesencephalon, used in cell replacement therapy for Parkinson’s disease, are known to contain a mix of dopamine neuronal subtypes including the A9 neurons of the substantia nigra and the A10 neurons of the ventral tegmental area. However, the relative importance of these subtypes for functional repair of the brain affected by Parkinson’s disease has not been studied thoroughly. Here, we report results from a series of grafting experiments where the anatomical and functional properties of grafts either selectively lacking in A9 neurons, or with a typical A9/A10 composition were compared. The results show that the A9 component of intrastriatal grafts is of critical importance for recovery in tests on motor performance, in a rodent model of Parkinson&rsquo... Brain journals http://www.medworm.com/rss/comments.php?id=3274159 Tue, 16 Feb 2010 03:54:45 +0100 3274159 http://www.medworm.com/index.php?rid=3274158&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F465%3Frss%3D1 Spinal muscular atrophy, characterized by selective loss of lower motor neurons, is an incurable genetic neurological disease leading to infant mortality. We previously showed that primary neural stem cells derived from spinal cord can ameliorate the spinal muscular atrophy phenotype in mice, but this primary source has limited translational value. Here, we illustrate that pluripotent stem cells from embryonic stem cells show the same potential therapeutic effects as those derived from spinal cord and offer great promise as an unlimited source of neural stem cells for transplantation. We found that embryonic stem cell-derived neural stem cells can differentiate into motor neurons in vitro and in vivo. In addition, following their intrathecal transplantation into spinal muscular atrophy mic... Brain journals http://www.medworm.com/rss/comments.php?id=3274158 Tue, 16 Feb 2010 03:54:45 +0100 3274158 http://www.medworm.com/index.php?rid=3274157&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F448%3Frss%3D1 Retinal pigment epithelial cell malfunction is a causative feature of age-related macular degeneration, and transplantation of new retinal pigment epithelial cells is an attractive strategy to prevent further progression and visual loss. However, transplants have shown limited efficacy, mainly because transplanted cells fail to adhere and migrate onto pathological Bruch’s membrane. Adhesion to Bruch’s membrane is integrin-mediated. Ageing of Bruch’s membrane leads to a decline in integrin ligands and, added to this, wet age-related macular degeneration leads to upregulation of anti-adhesive molecules such as tenascin-C. We have therefore investigated whether manipulation of integrin function in retinal pigment epithelial cells can restore their adhesion and migration on w... Brain journals http://www.medworm.com/rss/comments.php?id=3274157 Tue, 16 Feb 2010 03:54:45 +0100 3274157 http://www.medworm.com/index.php?rid=3274156&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F433%3Frss%3D1 Traumatic injury to the central nervous system results in the disruption of the blood brain/spinal barrier, followed by the invasion of cells and other components of the immune system that can aggravate injury and affect subsequent repair and regeneration. Although studies of chronic neuroinflammation in the injured spinal cord of animals are clinically relevant to most patients living with traumatic injury to the brain or spinal cord, very little is known about chronic neuroinflammation, though several studies have tested the role of neuroinflammation in the acute period after injury. The present study characterizes a novel cell preparation method that assesses, quickly and effectively, the changes in the principal immune cell types by flow cytometry in the injured spinal cord, daily for ... Brain journals http://www.medworm.com/rss/comments.php?id=3274156 Tue, 16 Feb 2010 03:54:45 +0100 3274156 http://www.medworm.com/index.php?rid=3274155&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F421%3Frss%3D1 Schwann cells are attractive candidates for repair of the injured spinal cord. Transplanted Schwann cells are permissive to regeneration, but their ability to promote regeneration into distal spinal cord remains weak despite their production of growth-promoting neurotrophins. Schwann cell activation such as that which accompanies peripheral nerve injury results in massive upregulation of the p75NTR pan-neurotrophin-receptor. Here we test the hypothesis that this p75NTR upregulation following dorsal root injury limits availability of endogenous neurotrophin to axons and restricts regeneration of injured axons into the spinal cord. We injured dorsal roots (fourth cervical to second thoracic) in mice lacking the neurotrophin-binding domain of p75NTR and in wild-type littermates. Axonal regene... Brain journals http://www.medworm.com/rss/comments.php?id=3274155 Tue, 16 Feb 2010 03:54:45 +0100 3274155 http://www.medworm.com/index.php?rid=3274154&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F406%3Frss%3D1 Recent findings suggested that inducing neural cell adhesion molecule polysialylation in rodents is a promising strategy for promoting tissue repair in the injured central nervous system. Since autologous grafting of Schwann cells is one potential strategy to promote central nervous system remyelination, it is essential to show that such a strategy can be translated to adult primate Schwann cells and is of interest for myelin diseases. Adult macaque Schwann cells were transduced with a lentiviral vector encoding sialyltransferase, an enzyme responsible for neural cell adhesion molecule polysialylation. In vitro, we found that ectopic expression of polysialylate promoted adult macaque Schwann cell migration and improved their integration among astrocytes in vitro without modifying their ant... Brain journals http://www.medworm.com/rss/comments.php?id=3274154 Tue, 16 Feb 2010 03:54:45 +0100 3274154 http://www.medworm.com/index.php?rid=3274153&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F389%3Frss%3D1 Stability of the myelin-axon unit is achieved, at least in part, by specialized paranodal junctions comprised of the neuronal heterocomplex of contactin and contactin-associated protein and the myelin protein neurofascin 155. In multiple sclerosis, normal distribution of these proteins is altered, resulting in the loss of the insulating myelin and consequently causing axonal dysfunction. Previously, this laboratory reported that mice lacking the myelin-enriched lipid sulphatide are characterized by a progressive deterioration of the paranodal structure. Here, it is shown that this deterioration is preceded by significant loss of neurofascin 155 clustering at the myelin paranode. Interestingly, prolonged electrophoretic separation revealed the existence of two neurofascin 155 bands, neurofa... Brain journals http://www.medworm.com/rss/comments.php?id=3274153 Tue, 16 Feb 2010 03:54:44 +0100 3274153 http://www.medworm.com/index.php?rid=3274152&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F375%3Frss%3D1 The primary biological function of the endogenous cellular prion protein has remained unclear. We investigated its biological function in the generation of cellular immune responses using cellular prion protein gene-specific small interfering ribonucleic acid in vivo and in vitro. Our results were confirmed by blocking cellular prion protein with monovalent antibodies and by using cellular prion protein-deficient and -transgenic mice. In vivo prion protein gene-small interfering ribonucleic acid treatment effects were of limited duration, restricted to secondary lymphoid organs and resulted in a 70% reduction of cellular prion protein expression in leukocytes. Disruption of cellular prion protein signalling augmented antigen-specific activation and proliferation, and enhanced T cell recept... Brain journals http://www.medworm.com/rss/comments.php?id=3274152 Tue, 16 Feb 2010 03:54:44 +0100 3274152 http://www.medworm.com/index.php?rid=3274151&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F362%3Frss%3D1 The cognitive reserve hypothesis helps to explain the incomplete relationship between brain disease and cognitive status in people with neurologic diseases, including Alzheimer's; disease and multiple sclerosis. Lifetime intellectual enrichment (estimated with education or vocabulary knowledge) lessens the negative impact of brain disease on cognition, such that people with greater enrichment are able to withstand more severe neuropathology before suffering cognitive impairment or dementia. The current research is the first to investigate directly the relationship between intellectual enrichment and an index of cerebral activity (the blood oxygen level dependent signal) in a neurologic sample. Multiple sclerosis patients completed a vocabulary-based estimate of lifetime intellectual enrich... Brain journals http://www.medworm.com/rss/comments.php?id=3274151 Tue, 16 Feb 2010 03:54:44 +0100 3274151 http://www.medworm.com/index.php?rid=3274150&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F349%3Frss%3D1 Neuromyelitis optica is an inflammatory demyelinating disease of the central nervous system associated with autoantibodies against the glial water channel protein aquaporin-4. It has recently been reported that immunoglobulin from neuromyelitis optica patients injected peripherally does not cause lesions in naive rats, but only when pre-existing central nervous system inflammation is present. Here, we investigated whether immunoglobulin G from aquaporin-4-autoantibody-positive neuromyelitis optica patients has the potential to damage the central nervous system either alone or in the presence of human complement. Immunoglobulin G from neuromyelitis optica patients did not activate mouse complement and was not pathogenic when injected into mouse brain. However, co-injection of immunoglobulin... Brain journals http://www.medworm.com/rss/comments.php?id=3274150 Tue, 16 Feb 2010 03:54:44 +0100 3274150 http://www.medworm.com/index.php?rid=3274149&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F333%3Frss%3D1 Distinction between acute disseminated encephalomyelitis and acute multiple sclerosis is often clinically difficult. Perivenous demyelination is the pathological hallmark of acute disseminated encephalomyelitis, whereas confluent demyelination is the hallmark of acute multiple sclerosis. We investigated whether perivenous demyelination versus confluent demyelination distinguishes acute disseminated encephalomyelitis from multiple sclerosis. Patients with perivenous demyelination (n = 13; median age 43 years, range 5–67) on brain biopsy and/or autopsy, ascertained retrospectively, were compared with a cohort with confluent demyelination only (n = 91; 84% multiple sclerosis, 16% isolated syndrome at follow-up; median age 39 years, range 10–69). Clinical presentation, course and t... Brain journals http://www.medworm.com/rss/comments.php?id=3274149 Tue, 16 Feb 2010 03:54:44 +0100 3274149 http://www.medworm.com/index.php?rid=3274148&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F320%3Frss%3D1 Eighty years ago, the Austrian neurologist Josef Gerstmann observed in a few patients a concomitant impairment in discriminating their own fingers, writing by hand, distinguishing left from right and performing calculations. He claimed that this tetrad of symptoms constituted a syndromal entity, assigned it to a lesion of the dominant parietal lobe and suggested that it was due to damage of a common functional denominator. Ever since, these claims have been debated and an astute synopsis and sceptical discussion was presented 40 years ago by MacDonald Critchley in this journal. Nonetheless, Gerstmann's syndrome has continued to intrigue both clinical neurologists and researchers in neuropsychology, and more frequently than not is described in textbooks as an example of parietal lobe damage... Brain journals http://www.medworm.com/rss/comments.php?id=3274148 Tue, 16 Feb 2010 03:54:44 +0100 3274148 http://www.medworm.com/index.php?rid=3274147&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F317%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3274147 Tue, 16 Feb 2010 03:54:44 +0100 3274147 http://www.medworm.com/index.php?rid=3274146&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F314%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3274146 Tue, 16 Feb 2010 03:54:44 +0100 3274146 http://www.medworm.com/index.php?rid=3274145&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F2%2F311%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3274145 Tue, 16 Feb 2010 03:54:44 +0100 3274145 http://www.medworm.com/index.php?rid=3138248&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F307%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3138248 Mon, 04 Jan 2010 12:35:24 +0100 3138248 http://www.medworm.com/index.php?rid=3138247&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F300%3Frss%3D1 We describe these features in a consecutive series of 100 patients seen over a 17-year period; all cases were assessed and followed up in a specialist clinic. The mean age at diagnosis was 64.2 (±7.1) range 40–79 years, but 46 presented after age 65 and 7 after 75; a higher proportion than the existing literature might predict. Fifteen had a first-degree relative with dementia, but in seven this was almost certainly unrelated. Only two had relatives with young-onset dementia. There were no families with more than two affected members. The familial rate was estimated at between 2% and 7% (95% confidence interval 0–12%). Kaplan–Meier analyses indicated a 50% survival of 12.8 years (95% confidence interval 11.9–13.7); a more benign course than suggested by neuro... Brain journals http://www.medworm.com/rss/comments.php?id=3138247 Mon, 04 Jan 2010 12:35:24 +0100 3138247 http://www.medworm.com/index.php?rid=3138246&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F286%3Frss%3D1 This study shows that semantic dementia is associated with anatomical damage to the major superior and inferior temporal white matter connections of the left hemisphere likely involved in semantic and lexical processes, with relative sparing of the fronto-parietal superior longitudinal fasciculus. Fronto-parietal regions connected by this tract were activated normally in the same patients during sublexical reading. These findings contribute to our understanding of the anatomical changes that occur in semantic dementia, and may further help to explain the dissociation between marked single-word and object knowledge deficits, but sparing of phonology and fluency in semantic dementia. (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3138246 Mon, 04 Jan 2010 12:35:24 +0100 3138246 http://www.medworm.com/index.php?rid=3138245&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F272%3Frss%3D1 Little is known about the processing of non-verbal sounds in the primary progressive aphasias. Here, we investigated the processing of complex non-verbal sounds in detail, in a consecutive series of 20 patients with primary progressive aphasia [12 with progressive non-fluent aphasia; eight with semantic dementia]. We designed a novel experimental neuropsychological battery to probe complex sound processing at early perceptual, apperceptive and semantic levels, using within-modality response procedures that minimized other cognitive demands and matching tests in the visual modality. Patients with primary progressive aphasia had deficits of non-verbal sound analysis compared with healthy age-matched individuals. Deficits of auditory early perceptual analysis were more common in progressive n... Brain journals http://www.medworm.com/rss/comments.php?id=3138245 Mon, 04 Jan 2010 12:35:24 +0100 3138245 http://www.medworm.com/index.php?rid=3138244&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F262%3Frss%3D1 The experience of being the initiator of one's own actions seems to be infallible at first glance. Misattributions of agency of one's actions in certain neurological or psychiatric patients reveal, however, that the central mechanisms underlying this experience can go astray. In particular, delusions of influence in schizophrenia might result from deficits in an inferential mechanism that allows distinguishing whether or not a sensory event has been self-produced. This distinction is made by comparing the actual sensory information with the consequences of one's action as predicted on the basis of internal action-related signals such as efference copies. If this internal prediction matches the actual sensory event, an action is registered as self-caused; in case of a mismatch, the differen... Brain journals http://www.medworm.com/rss/comments.php?id=3138244 Mon, 04 Jan 2010 12:35:24 +0100 3138244 http://www.medworm.com/index.php?rid=3138243&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F248%3Frss%3D1 Affective and social processes play a major role in everyday life, but appropriate methods to assess disturbances in these processes after brain lesions are still lacking. Past studies have shown that amygdala damage can impair recognition of facial expressions, particularly fear, as well as processing of gaze direction; but the mechanisms responsible for these deficits remain debated. Recent accounts of human amygdala function suggest that it is a critical structure involved in self-relevance appraisal. According to such accounts, responses to a given facial expression may vary depending on concomitant gaze direction and perceived social meaning. Here we investigated facial emotion recognition and its interaction with gaze in patients with unilateral amygdala damage (n = 19), compared to ... Brain journals http://www.medworm.com/rss/comments.php?id=3138243 Mon, 04 Jan 2010 12:35:24 +0100 3138243 http://www.medworm.com/index.php?rid=3138242&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F234%3Frss%3D1 Many tests of specific ‘executive functions’ show deficits after frontal lobe lesions. These deficits appear on a background of reduced fluid intelligence, best measured with tests of novel problem solving. For a range of specific executive tests, we ask how far frontal deficits can be explained by a general fluid intelligence loss. For some widely used tests, e.g. Wisconsin Card Sorting, we find that fluid intelligence entirely explains frontal deficits. When patients and controls are matched on fluid intelligence, no further frontal deficit remains. For these tasks too, deficits are unrelated to lesion location within the frontal lobe. A second group of tasks, including tests of both cognitive (e.g. Hotel, Proverbs) and social (Faux Pas) function, shows a different pattern. D... Brain journals http://www.medworm.com/rss/comments.php?id=3138242 Mon, 04 Jan 2010 12:35:24 +0100 3138242 http://www.medworm.com/index.php?rid=3138241&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F225%3Frss%3D1 We investigated the role of dopamine in working memory by examining effects of withdrawing dopaminergic medication in patients with Parkinson’s disease. Resistance to distraction during a delayed response task was abnormally enhanced in Parkinson’s disease patients OFF medication relative to controls. Conversely, performance on a backward digit span test was impaired in these same Parkinson’s disease patients OFF medication. Dopaminergic medication reinstated susceptibility to distraction and backward digit span performance, so that performance of Parkinson’s disease patients ON medication did not differ from that of controls. We hypothesize that the enhanced distractor resistance and impaired backward digit span in Parkinson’s disease reflects low dopamine le... Brain journals http://www.medworm.com/rss/comments.php?id=3138241 Mon, 04 Jan 2010 12:35:24 +0100 3138241 http://www.medworm.com/index.php?rid=3138240&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F215%3Frss%3D1 Postural instability and falls are a major source of disability in patients with advanced Parkinson's disease. These problems are currently not well addressed by either pharmacotherapy nor by subthalamic nucleus deep-brain stimulation surgery. The neuroanatomical substrates of posture and gait are poorly understood but a number of important observations suggest a major role for the pedunculopontine nucleus and adjacent areas in the brainstem. We conducted a double-blinded evaluation of unilateral pedunculopontine nucleus deep-brain stimulation in a pilot study in six advanced Parkinson's disease patients with significant gait and postural abnormalities. There was no significant difference in the double-blinded on versus off stimulation Unified Parkinson's Disease Rating Scale motor scores ... Brain journals http://www.medworm.com/rss/comments.php?id=3138240 Mon, 04 Jan 2010 12:35:24 +0100 3138240 http://www.medworm.com/index.php?rid=3138239&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F205%3Frss%3D1 Gait disturbances are frequent and disabling in advanced Parkinson's disease. These symptoms respond poorly to usual medical and surgical treatments but were reported to be improved by stimulation of the pedunculopontine nucleus. We studied the effects of stimulating the pedunculopontine nucleus area in six patients with severe freezing of gait, unresponsive to levodopa and subthalamic nucleus stimulation. Electrodes were implanted bilaterally in the pedunculopontine nucleus area. Electrode placement was checked by postoperative magnetic resonance imaging. The primary outcome measures were a composite gait score, freezing of gait questionnaire score and duration of freezing episodes occurring during a walking protocol at baseline and one-year follow-up. A double-blind cross-over study was ... Brain journals http://www.medworm.com/rss/comments.php?id=3138239 Mon, 04 Jan 2010 12:35:23 +0100 3138239 http://www.medworm.com/index.php?rid=3138238&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F189%3Frss%3D1 Parkinson's disease is the second most common neurodegenerative disease, after Alzheimer's disease, and the most common movement disorder. Drug treatment and deep brain stimulation can ameliorate symptoms, but the progressive degeneration of dopaminergic neurons in the substantia nigra eventually leads to severe motor dysfunction. The transplantation of stem cells has emerged as a promising approach to replace lost neurons in order to restore dopamine levels in the striatum and reactivate functional circuits. We have generated substrate-adherent embryonic stem cell-derived neural aggregates overexpressing the neural cell adhesion molecule L1, because it has shown beneficial functions after central nervous system injury. L1 enhances neurite outgrowth and neuronal migration, differentiation ... Brain journals http://www.medworm.com/rss/comments.php?id=3138238 Mon, 04 Jan 2010 12:35:23 +0100 3138238 http://www.medworm.com/index.php?rid=3138237&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F172%3Frss%3D1 -Synuclein is a major component of Lewy bodies and glial cytoplasmic inclusions, pathological hallmarks of idiopathic Parkinson's disease and multiple system atrophy, and it is assumed to be aetiologically involved in these conditions. However, the quantitative status of brain -synuclein in different Parkinsonian disorders is still unresolved and it is uncertain whether -synuclein accumulation is restricted to regions of pathology. We compared membrane-associated, sodium dodecyl sulfate-soluble -synuclein, both the full-length 17 kDa and high molecular weight species, by western blotting in autopsied brain of patients with Parkinson's disease (brainstem-predominant Lewy body disease: n = 9), multiple system atrophy (n = 11), progressive supranuclear palsy (n = 16), and of normal controls (... Brain journals http://www.medworm.com/rss/comments.php?id=3138237 Mon, 04 Jan 2010 12:35:23 +0100 3138237 http://www.medworm.com/index.php?rid=3138236&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F161%3Frss%3D1 The ‘default network’ is defined as a set of areas, encompassing posterior-cingulate/precuneus, anterior cingulate/mesiofrontal cortex and temporo-parietal junctions, that show more activity at rest than during attention-demanding tasks. Recent studies have shown that it is possible to reliably identify this network in the absence of any task, by resting state functional magnetic resonance imaging connectivity analyses in healthy volunteers. However, the functional significance of these spontaneous brain activity fluctuations remains unclear. The aim of this study was to test if the integrity of this resting-state connectivity pattern in the default network would differ in different pathological alterations of consciousness. Fourteen non-communicative brain-damaged patients and... Brain journals http://www.medworm.com/rss/comments.php?id=3138236 Mon, 04 Jan 2010 12:35:23 +0100 3138236 http://www.medworm.com/index.php?rid=3138235&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F139%3Frss%3D1 Magnetic resonance imaging provides evidence for loss of both white and grey matter, in terms of tissue volume, from the cerebral hemispheres after traumatic brain injury. However, quantitative histopathological data are lacking. From the archive of the Department of Neuropathology at Glasgow, the cerebral cortex of 48 patients was investigated using stereology. Patients had survived 3 months after traumatic brain injury and were classified using the Glasgow Outcome Scale as follows: moderately disabled (n = 13), severely disabled (n = 12) and vegetative state (n = 12); and controls. Some patients from the archive were diagnosed with diffuse axonal injury post-mortem. Comparisons of changes in cortical neuron population across Glasgow Outcome Scale groups between diffuse axonal injury and ... Brain journals http://www.medworm.com/rss/comments.php?id=3138235 Mon, 04 Jan 2010 12:35:23 +0100 3138235 http://www.medworm.com/index.php?rid=3138234&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F126%3Frss%3D1 Secretory leukocyte protease inhibitor is a serine protease inhibitor produced by various cell types, including neutrophils and activated macrophages, and has anti-inflammatory properties. It has been shown to promote wound healing in the skin and other non-neural tissues, however, its role in central nervous system injury was not known. We now report a beneficial role for secretory leukocyte protease inhibitor after spinal cord injury. After spinal cord contusion injury in mice, secretory leukocyte protease inhibitor is expressed primarily by astrocytes and neutrophils but not macrophages. We show, using transgenic mice over-expressing secretory leukocyte protease inhibitor, that this molecule has an early protective effect after spinal cord contusion injury. Furthermore, wild-type mice t... Brain journals http://www.medworm.com/rss/comments.php?id=3138234 Mon, 04 Jan 2010 12:35:23 +0100 3138234 http://www.medworm.com/index.php?rid=3138233&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F117%3Frss%3D1 Baclofen, a gamma-aminobutyric acid receptorB agonist, is used to reduce symptoms of spasticity (hyperreflexia, increases in muscle tone, involuntary muscle activity), but the long-term effects of sustained baclofen use on skeletal muscle properties are unclear. The aim of our study was to evaluate whether baclofen use and paralysis due to cervical spinal cord injury change the contractile properties of human thenar motor units more than paralysis alone. Evoked electromyographic activity and force were recorded in response to intraneural stimulation of single motor axons to thenar motor units. Data from three groups of motor units were compared: 23 paralysed units from spinal cord injured subjects who take baclofen and have done so for a median of 7 years, 25 paralysed units from spinal co... Brain journals http://www.medworm.com/rss/comments.php?id=3138233 Mon, 04 Jan 2010 12:35:23 +0100 3138233 http://www.medworm.com/index.php?rid=3138232&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F105%3Frss%3D1 Amyotrophic lateral sclerosis is a rapidly progressing fatal neurodegenerative disease characterized by the presence of protein inclusions within affected motor neurons. Endoplasmic reticulum stress leading to apoptosis was recently recognized to be an important process in the pathogenesis of sporadic human amyotrophic lateral sclerosis as well as in transgenic models of mutant superoxide dismutase 1-linked familial amyotrophic lateral sclerosis. Endoplasmic reticulum stress occurs early in disease, indicating a critical role in pathogenesis, and involves upregulation of an important endoplasmic reticulum chaperone, protein disulphide isomerase. We aimed to investigate the involvement of protein disulphide isomerase in endoplasmic reticulum stress induction, protein aggregation, inclusion ... Brain journals http://www.medworm.com/rss/comments.php?id=3138232 Mon, 04 Jan 2010 12:35:23 +0100 3138232 http://www.medworm.com/index.php?rid=3138231&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F93%3Frss%3D1 In this study we show that administration of a rapamycin ester (cell cycle inhibitor-779, temsirolimus) improves motor performance in a transgenic mouse model of spinocerebellar ataxia type 3. Temsirolimus inhibits mammalian target of rapamycin and hence upregulates protein degradation by autophagy. Temsirolimus reduces the number of aggregates seen in the brains of transgenic mice and decreases levels of cytosolic soluble mutant ataxin-3, while endogenous wild-type protein levels remain unaffected. Temsirolimus is designed for long-term use in patients and therefore represents a possible therapeutic strategy for the treatment of spinocerebellar ataxia type 3. Using this disease model and treatment paradigm, we employed a microarray approach to investigate transcriptional changes that migh... Brain journals http://www.medworm.com/rss/comments.php?id=3138231 Mon, 04 Jan 2010 12:35:23 +0100 3138231 http://www.medworm.com/index.php?rid=3138230&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F76%3Frss%3D1 In glutaric aciduria type 1, glutaryl-coenzyme A and its derivatives are produced from intracerebral lysine and entrapped at high concentrations within the brain, where they interfere with energy metabolism. Biochemical toxicity is thought to trigger stroke-like striatal degeneration in susceptible children under 2 years of age. Here, we explore vascular derangements that might also contribute to brain damage. We studied injured and non-injured Amish glutaric aciduria type 1 patients using magnetic resonance imaging (n = 26), transcranial Doppler ultrasound (n = 35) and perfusion computed tomography (n = 6). All glutaric aciduria type 1 patients had wide middle cerebral, internal carotid and basilar arteries. In non-injured patients, middle cerebral artery velocities were 18–26% belo... Brain journals http://www.medworm.com/rss/comments.php?id=3138230 Mon, 04 Jan 2010 12:35:23 +0100 3138230 http://www.medworm.com/index.php?rid=3138229&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F60%3Frss%3D1 In this study, we examined the role of netrin-1 and its receptors in mossy fibre guidance and report that enhanced neuronal activity changes netrin-1-mediated cell targeting by the axons under hyperexcitable conditions. Netrin-1 antibody or Dcc ribonucleic acid interference attenuated mossy fibre growth towards CA3 in slice overlay assays. The axons were repelled from CA3 and ultimately innervated the molecular layer when hyperactivity was pharmacologically introduced. We first hypothesized that a reduction in netrin-1 expression in CA3 underlies the phenomenon, but found that its expression was increased. We then examined two possible activity-dependent changes in netrin-1 receptor expression: a reduction in the deleted in colorectal cancer receptor and induction of uncoordinated-5 recept... Brain journals http://www.medworm.com/rss/comments.php?id=3138229 Mon, 04 Jan 2010 12:35:23 +0100 3138229 http://www.medworm.com/index.php?rid=3138228&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F46%3Frss%3D1 The relationship between changes in functional magnetic resonance imaging and neuronal activity remains controversial. Data collected during awake neurosurgical procedures for the treatment of epilepsy provided a rare opportunity to examine this relationship in human temporal association cortex. We obtained functional magnetic resonance imaging blood oxygen dependent signals, single neuronal activity and local field potentials from 8 to 300 Hz at 13 temporal cortical sites, from nine subjects, during paired associate learning and control measures. The relation between the functional magnetic resonance imaging signal and the electrophysiologic parameters was assessed in two ways: colocalization between significant changes in these signals on the same paired associate-control comparisons and... Brain journals http://www.medworm.com/rss/comments.php?id=3138228 Mon, 04 Jan 2010 12:35:23 +0100 3138228 http://www.medworm.com/index.php?rid=3138227&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F33%3Frss%3D1 Interictal high-frequency oscillations over 200 Hz have been recorded with microelectrodes in the seizure onset zone of epileptic patients suffering from mesial temporal lobe epilepsy. Recent work suggests that similar high-frequency oscillations can be detected in the seizure onset zone using standard diagnostic macroelectrodes. However, only a few channels were examined in these studies, so little information is available on the spatial extent of high-frequency oscillations. Here, we present data on high-frequency oscillations recorded from a larger number of intracerebral contacts spatial (mean 38) in 16 patients. Data were obtained from 1 h of interictal recording sampled at 1024 Hz and was analysed using a new semi-automatic detection procedure based on a wavelet decomposition. A deta... Brain journals http://www.medworm.com/rss/comments.php?id=3138227 Mon, 04 Jan 2010 12:35:23 +0100 3138227 http://www.medworm.com/index.php?rid=3138226&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F23%3Frss%3D1 This study aimed to investigate the impact of five microdeletions at the genomic hotspot regions 1q21.1, 15q11.2, 16p11.2, 16p13.11 and 22q11.2 on the genetic risk to common idiopathic generalized epilepsy syndromes. The candidate microdeletions were assessed by high-density single nucleotide polymorphism arrays in 1234 patients with idiopathic generalized epilepsy from North-western Europe and 3022 controls from the German population. Microdeletions were validated by quantitative polymerase chain reaction and their breakpoints refined by array comparative genomic hybridization. In total, 22 patients with idiopathic generalized epilepsy (1.8%) carried one of the five novel microdeletions compared with nine controls (0.3%) (odds ratio = 6.1; 95% confidence interval 2.8–13.2; 2 = 26.7;... Brain journals http://www.medworm.com/rss/comments.php?id=3138226 Mon, 04 Jan 2010 12:35:23 +0100 3138226 http://www.medworm.com/index.php?rid=3138225&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F9%3Frss%3D1 The non-dystrophic myotonias are an important group of skeletal muscle channelopathies electrophysiologically characterized by altered membrane excitability. Many distinct clinical phenotypes are now recognized and range in severity from severe neonatal myotonia with respiratory compromise through to milder late-onset myotonic muscle stiffness. Specific genetic mutations in the major skeletal muscle voltage gated chloride channel gene and in the voltage gated sodium channel gene are causative in most patients. Recent work has allowed more precise correlations between the genotype and the electrophysiological and clinical phenotype. The majority of patients with myotonia have either a primary or secondary loss of membrane chloride conductance predicted to result in reduction of the resting ... Brain journals http://www.medworm.com/rss/comments.php?id=3138225 Mon, 04 Jan 2010 12:35:23 +0100 3138225 http://www.medworm.com/index.php?rid=3138224&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F7%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3138224 Mon, 04 Jan 2010 12:35:23 +0100 3138224 http://www.medworm.com/index.php?rid=3138223&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F3%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3138223 Mon, 04 Jan 2010 12:35:23 +0100 3138223 http://www.medworm.com/index.php?rid=3138222&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F133%2F1%2F1%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3138222 Mon, 04 Jan 2010 12:35:23 +0100 3138222 http://www.medworm.com/index.php?rid=3081026&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3488%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3081026 Sat, 12 Dec 2009 09:12:29 +0100 3081026 http://www.medworm.com/index.php?rid=3081025&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3481%3Frss%3D1 This article discusses Charles Darwin's interest in topics that may broadly be defined as ‘neurological’ in character. Using published and manuscript materials, it examines the sources of Darwin's knowledge of neurological matters and seeks to explain why questions concerning the relation of mind and brain both in humans and other animals were relevant to his wider concerns. The paper concludes with a discussion of Darwin's impact on late 19th and early 20th century neurological thought. The 150th anniversary of the publication of the Origin of Species and the 200th of the birth of its author afford an opportunity to reflect on Charles Darwin's relationship to neurology. The first section of this article considers the part played by what might broadly be defined as ‘neuro... Brain journals http://www.medworm.com/rss/comments.php?id=3081025 Sat, 12 Dec 2009 09:12:29 +0100 3081025 http://www.medworm.com/index.php?rid=3081024&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3467%3Frss%3D1 In this study, using structural and diffusion-weighted magnetic resonance imaging, we investigated the structural differences in the brains of six bilaterally anophthalmic subjects compared with sighted subjects. Surprisingly, the gross structural differences in the brains were small, even in the occipital lobe where only a small region of the primary visual cortex showed a bilateral reduction in grey matter volume in the anophthalmic subjects compared with controls. Regions of increased cortical thickness were apparent on the banks of the Calcarine sulcus, but not in the fundus. Subcortically, the white matter volume around the optic tract and internal capsule in anophthalmic subjects showed a large decrease, yet the optic radiation volume did not differ significantly. However, the white ... Brain journals http://www.medworm.com/rss/comments.php?id=3081024 Sat, 12 Dec 2009 09:12:29 +0100 3081024 http://www.medworm.com/index.php?rid=3081023&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3456%3Frss%3D1 In this study, we examined the correlation between visual and verbal semantic knowledge for cars to determine if visual recognition accuracy could be predicted from verbal semantic scores. We had 33 healthy subjects and six prosopagnosic patients first rated their own knowledge of cars. They were then given a test of verbal semantic knowledge that presented them with the names of car models, to which they were to match the manufacturer. Lastly, they were given a test of visual recognition, presenting them with images of cars to which they were to provide information at three levels of specificity: model, manufacturer and decade of make. In controls, while self-ratings were only moderately correlated with either visual recognition or verbal semantic knowledge, verbal semantic knowledge was ... Brain journals http://www.medworm.com/rss/comments.php?id=3081023 Sat, 12 Dec 2009 09:12:29 +0100 3081023 http://www.medworm.com/index.php?rid=3081022&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3443%3Frss%3D1 Individuals with developmental prosopagnosia exhibit severe and lasting difficulties in recognizing faces despite the absence of apparent brain abnormalities. We used voxel-based morphometry to investigate whether developmental prosopagnosics show subtle neuroanatomical differences from controls. An analysis based on segmentation of T1-weighted images from 17 developmental prosopagnosics and 18 matched controls revealed that they had reduced grey matter volume in the right anterior inferior temporal lobe and in the superior temporal sulcus/middle temporal gyrus bilaterally. In addition, a voxel-based morphometry analysis based on the segmentation of magnetization transfer parameter maps showed that developmental prosopagnosics also had reduced grey matter volume in the right middle fusifor... Brain journals http://www.medworm.com/rss/comments.php?id=3081022 Sat, 12 Dec 2009 09:12:29 +0100 3081022 http://www.medworm.com/index.php?rid=3081021&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3428%3Frss%3D1 Focal brain lesions are assumed to produce language deficits by two basic mechanisms: local cortical dysfunction at the lesion site, and remote cortical dysfunction due to disruption of the transfer and integration of information between connected brain regions. However, functional imaging studies investigating language outcome after aphasic stroke have tended to focus only on the role of local cortical function. In this positron emission tomography functional imaging study, we explored relationships between language comprehension performance after aphasic stroke and the functional connectivity of a key speech-processing region in left anterolateral superior temporal cortex. We compared the organization of left anterolateral superior temporal cortex functional connections during narrative ... Brain journals http://www.medworm.com/rss/comments.php?id=3081021 Sat, 12 Dec 2009 09:12:29 +0100 3081021 http://www.medworm.com/index.php?rid=3081020&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3411%3Frss%3D1 This study used the new lesion analysis technique known as voxel-based lesion-symptom mapping to investigate the locus of lesions that give rise to semantic naming errors. Semantic errors were obtained from 64 individuals with post-stroke aphasia, who also underwent high-resolution structural brain scans. Whole brain voxel-based lesion-symptom mapping was carried out to determine where lesion status predicted semantic error rate. The strongest associations were found in the left anterior to mid middle temporal gyrus. This area also showed strong and significant effects in further analyses that statistically controlled for deficits in pre-lexical, conceptualization processes that might have contributed to semantic error production. This study is the first to demonstrate a specific and neces... Brain journals http://www.medworm.com/rss/comments.php?id=3081020 Sat, 12 Dec 2009 09:12:29 +0100 3081020 http://www.medworm.com/index.php?rid=3081019&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3401%3Frss%3D1 Competing theories of short-term memory function make specific predictions about the functional anatomy of auditory short-term memory and its role in language comprehension. We analysed high-resolution structural magnetic resonance images from 210 stroke patients and employed a novel voxel based analysis to test the relationship between auditory short-term memory and speech comprehension. Using digit span as an index of auditory short-term memory capacity we found that the structural integrity of a posterior region of the superior temporal gyrus and sulcus predicted auditory short-term memory capacity, even when performance on a range of other measures was factored out. We show that the integrity of this region also predicts the ability to comprehend spoken sentences. Our results therefore... Brain journals http://www.medworm.com/rss/comments.php?id=3081019 Sat, 12 Dec 2009 09:12:29 +0100 3081019 http://www.medworm.com/index.php?rid=3081018&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3392%3Frss%3D1 The purpose of this study was to compare the clinical and quantitative magnetic resonance imaging metrics of paediatric-onset multiple sclerosis to adult-onset multiple sclerosis. It was a prospective comparison of clinical and magnetic resonance imaging characteristics of two paediatric onset multiple sclerosis and two adult onset multiple sclerosis groups that were matched for disease duration. The paediatric-onset-C group consisted of children with paediatric-onset multiple sclerosis with mean disease duration of 2.7 years, whereas the paediatric onset-A group consisted of adults with mean disease duration of 20 years. The adult onset multiple sclerosis-1 and adult onset multiple sclerosis-2 groups were matched to the paediatric onset-C and paediatric onset-A groups. The brain magnetic ... Brain journals http://www.medworm.com/rss/comments.php?id=3081018 Sat, 12 Dec 2009 09:12:29 +0100 3081018 http://www.medworm.com/index.php?rid=3081017&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3380%3Frss%3D1 Affective disorders are frequent and disabling conditions in multiple sclerosis; however, the underlying neurobiological mechanisms are still poorly understood and investigated. Previous structural imaging studies have suggested that damage of frontal and temporal cortices plays an important role in the genesis of emotional disorders in multiple sclerosis, although psychosocial factors have been also implicated. However, this initial research may not have fully characterized the brain's functional dynamics of emotional processes in multiple sclerosis. Functional magnetic resonance imaging (fMRI) appears, therefore, to be a sensible tool to explore neurobiological mechanisms of emotions in multiple sclerosis since it also allows investigation of the functional connectivity or ‘communi... Brain journals http://www.medworm.com/rss/comments.php?id=3081017 Sat, 12 Dec 2009 09:12:29 +0100 3081017 http://www.medworm.com/index.php?rid=3081016&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3366%3Frss%3D1 In this study, we first classified patients (n = 330) into six subgroups according to their total white matter lesion loads, and identified structural brain networks for each multiple sclerosis group by thresholding the corresponding inter-regional cortical thickness correlation matrix, followed by a network efficiency analysis with graph theoretical approaches. The structural cortical networks in multiple sclerosis demonstrated efficient small-world architecture regardless of the lesion load, an organization that maximizes the information processing at a relatively low wiring cost. However, we found that the overall small-world network efficiency in multiple sclerosis was significantly disrupted in a manner proportional to the extent of total white matter lesions. Moreover, regional effic... Brain journals http://www.medworm.com/rss/comments.php?id=3081016 Sat, 12 Dec 2009 09:12:29 +0100 3081016 http://www.medworm.com/index.php?rid=3081015&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3353%3Frss%3D1 The effect of interferon-beta in multiple sclerosis is modest and many patients do not respond to treatment. To date, no single biomarker reliably correlates with responsiveness to interferon-β in multiple sclerosis. In the present study, genome-wide expression profiling was performed in peripheral blood mononuclear cells from 47 multiple sclerosis patients treated with interferon-β for a minimum of 2 years and classified as responders and non-responders based on clinical criteria. A validation cohort of 30 multiple sclerosis patients was included in the study to replicate gene-expression findings. Before treatment, interferon-β responders and non-responders were characterized by differential expression of type I interferon-induced genes with overexpression of the type inter... Brain journals http://www.medworm.com/rss/comments.php?id=3081015 Sat, 12 Dec 2009 09:12:29 +0100 3081015 http://www.medworm.com/index.php?rid=3081014&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3342%3Frss%3D1 We established microRNA profiles from active and inactive multiple sclerosis lesions. Using laser capture microdissection from multiple sclerosis lesions to pool single cells and in vitro cultures, we assigned differentially expressed microRNA to specific cell types. Astrocytes contained all 10 microRNA that were most strongly upregulated in active multiple sclerosis lesions, including microRNA-155, which is known to modulate immune responses in different ways but so far had not been assigned to central nervous system resident cells. MicroRNA-155 was expressed in human astrocytes in situ, and further induced with cytokines in human astrocytes in vitro. This was confirmed with astrocyte cultures from microRNA-155-|-lacZ mice. We matched microRNA upregulated in phagocytically active multiple... Brain journals http://www.medworm.com/rss/comments.php?id=3081014 Sat, 12 Dec 2009 09:12:29 +0100 3081014 http://www.medworm.com/index.php?rid=3081013&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3329%3Frss%3D1 In this study, we analysed the frequency and the phenotype of Th17 cells in the cerebrospinal fluid and peripheral blood of multiple sclerosis patients. We show that the frequency of Th17 cells is significantly higher in the cerebrospinal fluid of patients with relapsing-remitting multiple sclerosis during relapse, in comparison to relapsing-remitting patients in remission or to patients with other non-inflammatory neurological diseases. Similarly, in patients with clinically isolated syndrome during their first neurological episode, Th17 cells are more abundant than in clinically isolated syndrome patients with no acute symptoms. Patients with inflammatory neurological diseases other than multiple sclerosis also showed increased frequency of Th17 cells compared to patients with no inflamm... Brain journals http://www.medworm.com/rss/comments.php?id=3081013 Sat, 12 Dec 2009 09:12:29 +0100 3081013 http://www.medworm.com/index.php?rid=3081012&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3318%3Frss%3D1 We report that EBV could not be detected in any of the multiple sclerosis specimens containing white matter lesions by any of the methods employed, yet EBV was readily detectable in multiple Epstein–Barr virus-positive control tissues including several CNS lymphomas. Furthermore, EBV was not detected in our second cohort of multiple sclerosis specimens by in situ hybridization. However, our real-time PCR methodologies, which were capable of detecting very few EBV infected cells, detected EBV at low levels in only 2 of the 12 multiple sclerosis meningeal specimens examined. Our finding that CNS EBV infection was rare in multiple sclerosis brain indicates that EBV infection is unlikely to contribute directly to multiple sclerosis brain pathology in the vast majority of cases. (Source: ... Brain journals http://www.medworm.com/rss/comments.php?id=3081012 Sat, 12 Dec 2009 09:12:29 +0100 3081012 http://www.medworm.com/index.php?rid=3080999&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2Fe134%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3080999 Sat, 12 Dec 2009 09:12:29 +0100 3080999 http://www.medworm.com/index.php?rid=3080998&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2Fe133%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3080998 Sat, 12 Dec 2009 09:12:29 +0100 3080998 http://www.medworm.com/index.php?rid=3080997&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2Fe132%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3080997 Sat, 12 Dec 2009 09:12:29 +0100 3080997 http://www.medworm.com/index.php?rid=3080996&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2Fe131%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3080996 Sat, 12 Dec 2009 09:12:29 +0100 3080996 http://www.medworm.com/index.php?rid=3080995&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2Fe130%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3080995 Sat, 12 Dec 2009 09:12:29 +0100 3080995 http://www.medworm.com/index.php?rid=3081011&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3308%3Frss%3D1 In this study, we aimed to measure cerebrospinal fluid hypocretin-1 levels in Parkinson's disease patients with and without dementia and to study their relationship to dementia and clinical excessive daytime sleepiness, as well as to describe potentially related sleep architecture changes. Twenty-one Parkinson's disease patients without dementia and 20 Parkinson's disease patients with dementia, along with 22 control subjects without sleep complaints, were included. Both Epworth sleepiness scale, obtained with the help of the caregivers, and mini-mental state examination were recorded. Lumbar cerebrospinal fluid hypocretin-1 levels were measured in all individuals using a radio-immunoassay technique. Additionally, eight Parkinson's disease patients without dementia and seven Parkinson's di... Brain journals http://www.medworm.com/rss/comments.php?id=3081011 Sat, 12 Dec 2009 09:12:28 +0100 3081011 http://www.medworm.com/index.php?rid=3081010&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3298%3Frss%3D1 Idiopathic rapid eye movement sleep behaviour disorder is an important risk factor in the development of Parkinson's disease. Numerous potential predictive markers of Parkinson's disease may present before motor symptoms emerge, but testing of these markers in rapid eye movement sleep behaviour disorder has been performed only in small studies. There has been no comparison of markers between patients with idiopathic rapid eye movement sleep behaviour disorder and Parkinson's disease, and between men and women. We evaluated an array of potential Parkinson's disease predictive markers in 159 patients; including 68 with idiopathic rapid eye movement sleep behaviour disorder, 36 controls, 34 Parkinson's patients with rapid eye movement sleep behaviour disorder and 21 Parkinson's patients witho... Brain journals http://www.medworm.com/rss/comments.php?id=3081010 Sat, 12 Dec 2009 09:12:28 +0100 3081010 http://www.medworm.com/index.php?rid=3081009&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3285%3Frss%3D1 Mitochondrial dysfunction hypothetically contributes to neuronal degeneration in patients with Parkinson's disease. While several in vitro data exist, the measurement of cerebral mitochondrial dysfunction in living patients with Parkinson's disease is challenging. Anatomical magnetic resonance imaging combined with phosphorus and proton magnetic resonance spectroscopic imaging provides information about the functional integrity of mitochondria in specific brain areas. We measured partial volume corrected concentrations of low-energy metabolites and high-energy phosphates with sufficient resolution to focus on pathology related target areas in Parkinson's disease. Combined phosphorus and proton magnetic resonance spectroscopic imaging in the mesostriatal region was performed in 16 early and... Brain journals http://www.medworm.com/rss/comments.php?id=3081009 Sat, 12 Dec 2009 09:12:28 +0100 3081009 http://www.medworm.com/index.php?rid=3081008&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3274%3Frss%3D1 The m.3243A>G mutation is the most common pathogenic mutation in mitochondrial DNA. It leads to defective oxidative phosphorylation, decreased oxygen consumption and increased glucose utilization and lactate production in vitro. However, oxygen and glucose metabolism has not been studied in the brain of patients harbouring the m.3243A>G mutation. Therefore, 14 patients with the m.3243A>G mutation, not experiencing acute stroke-like episodes and 14 age-matched controls underwent positron emission tomography using 2-[18F]fluoro-2-deoxyglucose, [15O]H2O and [15O]O2 as the tracers during normoglycaemia. The metabolic rate of oxygen and glucose were determined using a quantitative region of interest analysis. Metabolites in unaffected periventricular tissue were measured using magnetic... Brain journals http://www.medworm.com/rss/comments.php?id=3081008 Sat, 12 Dec 2009 09:12:28 +0100 3081008 http://www.medworm.com/index.php?rid=3081007&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3263%3Frss%3D1 Charcot–Marie-Tooth disease type 1A is the most common inherited neuropathy and is caused by duplication of chromosome 17p11.2 containing the peripheral myelin protein-22 gene. This disease is characterized by uniform slowing of conduction velocities and secondary axonal loss, which are in contrast with non-uniform slowing of conduction velocities in acquired demyelinating disorders, such as chronic inflammatory demyelinating polyradiculoneuropathy. Mechanisms responsible for the slowed conduction velocities and axonal loss in Charcot–Marie-Tooth disease type 1A are poorly understood, in part because of the difficulty in obtaining nerve samples from patients, due to the invasive nature of nerve biopsies. We have utilized glabrous skin biopsies, a minimally invasive procedure, t... Brain journals http://www.medworm.com/rss/comments.php?id=3081007 Sat, 12 Dec 2009 09:12:28 +0100 3081007 http://www.medworm.com/index.php?rid=3081006&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3252%3Frss%3D1 Charcot–Marie-Tooth type 1A is the most prevalent hereditary demyelinating polyneuropathy. The aim of this study was to investigate the natural history of the disease in adults during a 5-year follow-up and to compare the changes over time with those found in normal ageing. In a cohort of 46 adult Charcot–Marie-Tooth type 1A patients, impairments and physical disability were scored at baseline and at 1, 3 and 5 years. Standardized nerve conduction studies and electromyography were performed at baseline and at 5 years. Twenty-six healthy age- and sex-matched controls were evaluated at baseline and at 5 years. Forty-four of 46 Charcot–Marie-Tooth type 1A patients (range 17–69 years) and 26 controls (range 25–65 years) completed the 5-year follow-up. The decrease... Brain journals http://www.medworm.com/rss/comments.php?id=3081006 Sat, 12 Dec 2009 09:12:28 +0100 3081006 http://www.medworm.com/index.php?rid=3081005&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3242%3Frss%3D1 Spinal and bulbar muscular atrophy is an X-linked motor neuron disease caused by a CAG repeat expansion in the androgen receptor gene. To characterize the natural history and define outcome measures for clinical trials, we assessed the clinical history, laboratory findings and muscle strength and function in 57 patients with genetically confirmed disease. We also administered self-assessment questionnaires for activities of daily living, quality of life and erectile function. We found an average delay of over 5 years from onset of weakness to diagnosis. Muscle strength and function correlated directly with serum testosterone levels and inversely with CAG repeat length, age and duration of weakness. Motor unit number estimation was decreased by about half compared to healthy controls. Senso... Brain journals http://www.medworm.com/rss/comments.php?id=3081005 Sat, 12 Dec 2009 09:12:28 +0100 3081005 http://www.medworm.com/index.php?rid=3081004&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3231%3Frss%3D1 A role for the Notch signalling pathway in the formation of arteriovenous malformations during development has been suggested. However, whether Notch signalling is involved in brain arteriovenous malformations in humans remains unclear. Here, we performed immunohistochemistry on surgically resected brain arteriovenous malformations and found that, compared with control brain vascular tissue, Notch-1 signalling was activated in smooth muscle and endothelial cells of the lesional tissue. Western blotting showed an activated form of Notch-1 in brain arteriovenous malformations, irrespective of clinical presentation and with or without preoperative embolization, but not in normal cerebral vessels from controls. In addition, the Notch-1 ligands Jagged-1 and Delta-like-4 and the downstream Notch... Brain journals http://www.medworm.com/rss/comments.php?id=3081004 Sat, 12 Dec 2009 09:12:28 +0100 3081004 http://www.medworm.com/index.php?rid=3081003&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3199%3Frss%3D1 Advances in neuroimaging, developmental biology and molecular genetics have increased the understanding of developmental disorders affecting the midbrain and hindbrain, both as isolated anomalies and as part of larger malformation syndromes. However, the understanding of these malformations and their relationships with other malformations, within the central nervous system and in the rest of the body, remains limited. A new classification system is proposed, based wherever possible, upon embryology and genetics. Proposed categories include: (i) malformations secondary to early anteroposterior and dorsoventral patterning defects, or to misspecification of mid-hindbrain germinal zones; (ii) malformations associated with later generalized developmental disorders that significantly affect the ... Brain journals http://www.medworm.com/rss/comments.php?id=3081003 Sat, 12 Dec 2009 09:12:28 +0100 3081003 http://www.medworm.com/index.php?rid=3081002&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3196%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3081002 Sat, 12 Dec 2009 09:12:28 +0100 3081002 http://www.medworm.com/index.php?rid=3081001&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3193%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3081001 Sat, 12 Dec 2009 09:12:28 +0100 3081001 http://www.medworm.com/index.php?rid=3081000&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F12%2F3191%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=3081000 Sat, 12 Dec 2009 09:12:28 +0100 3081000 http://www.medworm.com/index.php?rid=2932812&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3187%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2932812 Tue, 27 Oct 2009 17:42:28 +0100 2932812 http://www.medworm.com/index.php?rid=2932811&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3175%3Frss%3D1 We have performed a detailed population study of patients with genetic muscle disease in the northern region of England. Our current clinic population comprises over 1100 patients in whom we have molecularly characterized 31 separate muscle disease entities. Diagnostic clarity achieved through careful delineation of clinical features supported by histological, immunological and genetic analysis has allowed us to reach a definitive diagnosis in 75.7% of our patients. We have compared our case profile with that from Walton and Nattrass’ seminal study from 1954, also of the northern region, together with data from other more recent studies from around the world. Point prevalence figures for each of the five major disease categories are comparable with those from other recent studies. My... Brain journals http://www.medworm.com/rss/comments.php?id=2932811 Tue, 27 Oct 2009 17:42:28 +0100 2932811 http://www.medworm.com/index.php?rid=2932810&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3165%3Frss%3D1 This study provides the rationale for a simple genetic test to identify infants with mitochondrial myopathy and good prognosis. (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2932810 Tue, 27 Oct 2009 17:42:28 +0100 2932810 http://www.medworm.com/index.php?rid=2932809&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3152%3Frss%3D1 Cannabinoid-derived drugs are promising agents for the development of novel neuroprotective strategies. Activation of neuronal CB1 cannabinoid receptors attenuates excitotoxic glutamatergic neurotransmission, triggers prosurvival signalling pathways and palliates motor symptoms in animal models of neurodegenerative disorders. However, in Huntington's disease there is a very early downregulation of CB1 receptors in striatal neurons that, together with the undesirable psychoactive effects triggered by CB1 receptor activation, foster the search for alternative pharmacological treatments. Here, we show that CB2 cannabinoid receptor expression increases in striatal microglia of Huntington's disease transgenic mouse models and patients. Genetic ablation of CB2 receptors in R6/2 mice, that expres... Brain journals http://www.medworm.com/rss/comments.php?id=2932809 Tue, 27 Oct 2009 17:42:28 +0100 2932809 http://www.medworm.com/index.php?rid=2932808&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3142%3Frss%3D1 Complex regional pain syndrome (CRPS) occurs after stroke, but most cases develop after peripheral trauma and without evidence of brain trauma. However, CRPS is associated with symptoms that appear similar to those observed in patients suffering from hemispatial neglect. Ten participants (four males) with CRPS of one arm performed temporal order judgements of pairs of vibrotactile stimuli, one delivered to each hand, at one of 10 possible stimulus onset asynchronies, under two conditions: arms held each side of the midline and arms crossed over the midline. Participants released a foot switch to indicate which hand had been stimulated first. The order of conditions was randomized and the foot under which the switch was positioned was counterbalanced. There were two blocks of 150 trials in ... Brain journals http://www.medworm.com/rss/comments.php?id=2932808 Tue, 27 Oct 2009 17:42:27 +0100 2932808 http://www.medworm.com/index.php?rid=2932807&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3134%3Frss%3D1 Several lines of evidence suggest a major role of calcitonin gene-related peptide (CGRP) in the pathogenesis of migraine and other primary headaches. Inhibition of CGRP receptors by olcegepant and telcagepant has been successfully used to treat acute migraine and to reduce the activity of spinal trigeminal neurons involved in meningeal nociception in rodents. The site of CGRP receptor inhibition is unclear, however. In adult Wistar rats anaesthetized with isofluorane systemic intravenous infusion (0.9 mg/kg) or unilateral facial injection (1 mM in 100 µl) of capsaicin was used to induce activity in the trigeminal nociceptive system. Animals were pre-treated either by saline or olcegepant. In comparison with vehicle infusion or the non-injected side of the face, capsaicin significantl... Brain journals http://www.medworm.com/rss/comments.php?id=2932807 Tue, 27 Oct 2009 17:42:27 +0100 2932807 http://www.medworm.com/index.php?rid=2932806&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3122%3Frss%3D1 Following upper limb peripheral nerve transection and surgical repair, some patients regain good sensorimotor function while others do not. Understanding peripheral and central mechanisms that contribute to recovery may facilitate the development of new therapeutic interventions. Plasticity following peripheral nerve transection has been demonstrated throughout the neuroaxis in animal models of nerve injury. However, the brain changes that occur following peripheral nerve transection and surgical repair in humans have not been examined. Furthermore, the extent to which peripheral nerve regeneration influences functional and structural brain changes has not been characterized. Therefore, we asked whether functional changes are accompanied by grey and/or white matter structural changes and w... Brain journals http://www.medworm.com/rss/comments.php?id=2932806 Tue, 27 Oct 2009 17:42:27 +0100 2932806 http://www.medworm.com/index.php?rid=2932805&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3102%3Frss%3D1 Inhibition of central nervous system axon growth is reportedly mediated in part by calcium-dependent phosphorylation of axonal epidermal growth factor receptor, with local administration of the epidermal growth factor receptor kinase inhibitors AG1478 and PD168393 to an optic nerve lesion site promoting adult retinal ganglion cell axon regeneration. Here, we show that epidermal growth factor receptor was neither constitutively expressed, nor activated in optic nerve axons in our non-regenerating and regenerating optic nerve injury models, a finding that is inconsistent with phosphorylated epidermal growth factor receptor-dependent intra-axonal signalling of central nervous system myelin-related axon growth inhibitory ligands. However, epidermal growth factor receptor was localized and acti... Brain journals http://www.medworm.com/rss/comments.php?id=2932805 Tue, 27 Oct 2009 17:42:27 +0100 2932805 http://www.medworm.com/index.php?rid=2932804&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3096%3Frss%3D1 In this study, we investigated whether the human olfactory bulb volume increases with increasing olfactory function due to treatment of chronic rhinosinusitis. Nineteen patients with chronic rhinosinusitis were investigated before and after treatment. For comparison, additional measurements were performed in 18 healthy volunteers. Volumetric measurements of the olfactory bulb were based on planimetric manual contouring of magnetic resonance scans. Olfactory function was evaluated separately for each nostril using tests for odour threshold, odour discrimination and odour identification. Measurements were performed on two occasions, 3 months apart. In healthy controls, the olfactory bulb volume did not change significantly between the two measurements. In contrast, the olfactory bulb volume ... Brain journals http://www.medworm.com/rss/comments.php?id=2932804 Tue, 27 Oct 2009 17:42:27 +0100 2932804 http://www.medworm.com/index.php?rid=2932803&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3087%3Frss%3D1 Posterior fossa syndrome is characterized by cerebellar dysfunction, oromotor/oculomotor apraxia, emotional lability and mutism in patients after infratentorial injury. The underlying neuroanatomical substrates of posterior fossa syndrome are unknown, but dentatothalamocortical tracts have been implicated. We used pre- and postoperative neuroimaging to investigate proximal dentatothalamocortical tract involvement in childhood embryonal brain tumour patients who developed posterior fossa syndrome following tumour resection. Diagnostic imaging from a cohort of 26 paediatric patients previously operated on for an embryonal brain tumour (13 patients prospectively diagnosed with posterior fossa syndrome, and 13 non-affected patients) were evaluated. Preoperative magnetic resonance imaging was u... Brain journals http://www.medworm.com/rss/comments.php?id=2932803 Tue, 27 Oct 2009 17:42:26 +0100 2932803 http://www.medworm.com/index.php?rid=2932802&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3072%3Frss%3D1 In this study, we have quantified the epileptogenic characteristic of brain structures explored by depth electrodes in 36 patients investigated by stereoelectroencephalography and suffering from focal drug-resistant epilepsy associated with focal cortical dysplasias or dysembryoplastic neuroepithelial tumours. This quantification was performed using the ‘Epileptogenicity Index’ method that accounts for both the propensity of a brain area to generate rapid discharges and the time for this area to get involved in the seizure. Epileptogenicity Index values range from 0 (no epileptogenicity) to 1 (maximal epileptogenicity). We determined Epileptogenicity Index from signals recorded in distinct brain structures including the lesional site. We studied the type of epileptogenic zone o... Brain journals http://www.medworm.com/rss/comments.php?id=2932802 Tue, 27 Oct 2009 17:42:26 +0100 2932802 http://www.medworm.com/index.php?rid=2932801&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3060%3Frss%3D1 Magnetoencephalography (MEG) is considered a useful tool for planning electrode placement for chronic intracranial subdural electrocorticography (ECoG) in candidates for epilepsy surgery or even as a substitute for ECoG. MEG recordings are usually interictal and therefore, at best, reflect the interictal ECoG. To estimate the clinical value of MEG, it is important to know how well interictal MEG reflects interictal activity in the ECoG. From 1998 to 2008, 38 candidates for ECoG underwent a 151-channel MEG recording and 3D magnetic resonance imaging as a part of their presurgical evaluation. Interictal MEG spikes were identified, clustered, averaged and modelled using the multiple signal classification algorithm and co-registered to magnetic resonance imaging. ECoG was continuously recorded... Brain journals http://www.medworm.com/rss/comments.php?id=2932801 Tue, 27 Oct 2009 17:42:26 +0100 2932801 http://www.medworm.com/index.php?rid=2932800&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3047%3Frss%3D1 Interictal high frequency oscillations (HFOs), in particular those with frequency components in excess of 200 Hz, have been proposed as important biomarkers of epileptic cortex as well as the genesis of seizures. We investigated the spatial extent, classification and distribution of HFOs using a dense 4 x 4 mm2 two dimensional microelectrode array implanted in the neocortex of four patients undergoing epilepsy surgery. The majority (97%) of oscillations detected included fast ripples and were concentrated in relatively few recording sites. While most HFOs were limited to single channels, ~10% occurred on a larger spatial scale with simultaneous but morphologically distinct detections in multiple channels. Eighty per cent of these large-scale events were associated with interictal epileptif... Brain journals http://www.medworm.com/rss/comments.php?id=2932800 Tue, 27 Oct 2009 17:42:25 +0100 2932800 http://www.medworm.com/index.php?rid=2932799&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3032%3Frss%3D1 In this study, we asked whether a similar population activity is generated in the cornu ammonis 2 region and examined the electrophysiological and neuroanatomical characteristics of human epileptic cornu ammonis 2 neurons that may be involved. Hippocampal slices were prepared from postoperative temporal lobe tissue derived from epileptic patients. Field potentials and multi-unit activity were recorded in vitro using multiple extracellular microelectrodes. Pyramidal cells were characterized in intra-cellular records and were filled with biocytin for subsequent anatomy. Fluorescent immunostaining was made on fixed tissue against the chloride–cation cotransporters sodium-potasium-chloride cotransporter-1 and potassium-chloride cotransporter-2. Light and electron microscopy were used to ... Brain journals http://www.medworm.com/rss/comments.php?id=2932799 Tue, 27 Oct 2009 17:42:25 +0100 2932799 http://www.medworm.com/index.php?rid=2932798&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3021%3Frss%3D1 Affective neuroscience has been strongly influenced by the view that a ‘feeling’ is the perception of somatic changes and has consequently often neglected the neural mechanisms that underlie the integration of somatic and other information in affective experience. Here, we investigate affective processing by means of functional magnetic resonance imaging in nine cortically blind patients. In these patients, unilateral postgeniculate lesions prevent primary cortical visual processing in part of the visual field which, as a result, becomes subjectively blind. Residual subcortical processing of visual information, however, is assumed to occur in the entire visual field. As we have reported earlier, these patients show significant startle reflex potentiation when a threat-related v... Brain journals http://www.medworm.com/rss/comments.php?id=2932798 Tue, 27 Oct 2009 17:42:25 +0100 2932798 http://www.medworm.com/index.php?rid=2932797&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3011%3Frss%3D1 Transcranial direct current stimulation is a painless, non-invasive brain stimulation technique that allows one to induce polarity-specific excitability changes in the human brain. Here, we investigated, for the first time in a ‘proof of principle’ study, the behavioural effect of transcranial direct current stimulation on visuospatial attention in both healthy controls and stroke patients suffering from left visuospatial neglect. We applied anodal, cathoP:dal or sham transcranial direct current stimulation (57 µA/cm2, 10 min) to the left or right posterior parietal cortex. Using a visual detection task in a group of right-handed healthy individuals (n = 20), we observed that transcranial direct current stimulation enhanced or impaired performance depending on stimulation... Brain journals http://www.medworm.com/rss/comments.php?id=2932797 Tue, 27 Oct 2009 17:42:25 +0100 2932797 http://www.medworm.com/index.php?rid=2932796&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F3002%3Frss%3D1 We investigated the role of prefrontal cortex (PFC) in local contextual processing using a combined event-related potentials and lesion approach. Local context was defined as the occurrence of a short predictive series of visual stimuli occurring before delivery of a target event. Targets were preceded by either randomized sequences of standards or by sequences including a three-stimulus predictive sequence signalling the occurrence of a subsequent target event. PFC lesioned patients were impaired in their ability to use local contextual information. The response time for controls revealed a larger benefit for predictable targets than for random targets relative to PFC patients. PFC patients had reduced amplitude of a context-dependent positivity and failed to generate the expected P3b lat... Brain journals http://www.medworm.com/rss/comments.php?id=2932796 Tue, 27 Oct 2009 17:42:25 +0100 2932796 http://www.medworm.com/index.php?rid=2932795&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2994%3Frss%3D1 Distortion of the sense of reality, actualized in delusions and hallucinations, is the key feature of psychosis but the underlying neuronal correlates remain largely unknown. We studied 11 highly functioning subjects with schizophrenia or schizoaffective disorder while they rated the reality of auditory verbal hallucinations (AVH) during functional magnetic resonance imaging (fMRI). The subjective reality of AVH correlated strongly and specifically with the hallucination-related activation strength of the inferior frontal gyri (IFG), including the Broca's language region. Furthermore, how real the hallucination that subjects experienced was depended on the hallucination-related coupling between the IFG, the ventral striatum, the auditory cortex, the right posterior temporal lobe, and the c... Brain journals http://www.medworm.com/rss/comments.php?id=2932795 Tue, 27 Oct 2009 17:42:25 +0100 2932795 http://www.medworm.com/index.php?rid=2932794&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2980%3Frss%3D1 Impaired visual processing may play a role in the pathophysiology of visual hallucinations in Parkinson's disease. In order to study involved neuronal circuitry, we assessed cerebral activation patterns both before and during recognition of gradually revealed images in Parkinson's disease patients with visual hallucinations (PDwithVHs), Parkinson's disease patients without visual hallucinations (PDnonVHs) and healthy controls. We hypothesized that, before image recognition, PDwithVHs would show reduced bottom-up visual activation in occipital-temporal areas and increased (pre)frontal activation, reflecting increased top-down demand. Overshoot of the latter has been proposed to play a role in generating visual hallucinations. Nine non-demented PDwithVHs, 14 PDnonVHs and 13 healthy controls ... Brain journals http://www.medworm.com/rss/comments.php?id=2932794 Tue, 27 Oct 2009 17:42:25 +0100 2932794 http://www.medworm.com/index.php?rid=2932793&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2970%3Frss%3D1 Parkinson's disease is a heterogeneous disorder with multiple factors contributing to disease initiation and progression. Using serial, multi-tracer positron emission tomography imaging, we studied a cohort of 78 subjects with sporadic Parkinson's disease to understand the disease course better. Subjects were scanned with radiotracers of presynaptic dopaminergic integrity at baseline and again after 4 and 8 years of follow-up. Non-linear multivariate regression analyses, using random effects, of the form BPND(t) or Kocc(t) = a*e(–bt–dA) + c, where BPND = tracer binding potential (nondispaceable), KOCC = tracer uptake constant a, b, c and d are regression parameters, t is the symptom duration and A is the age at onset, were utilized to model the longitudinal progression of radio... Brain journals http://www.medworm.com/rss/comments.php?id=2932793 Tue, 27 Oct 2009 17:42:24 +0100 2932793 http://www.medworm.com/index.php?rid=2932792&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2958%3Frss%3D1 Cognitive abnormalities are common in Parkinson's disease, with important social and economic implications. Factors influencing their evolution remain unclear but are crucial to the development of targeted therapeutic strategies. We have investigated the development of cognitive impairment and dementia in Parkinson's disease using a longitudinal approach in a population-representative incident cohort (CamPaIGN study, n = 126) and here present the 5-year follow-up data from this study. Our previous work has implicated two genetic factors in the development of cognitive dysfunction in Parkinson's disease, namely the genes for catechol-O-methyltransferase (COMT Val158Met) and microtubule-associated protein tau (MAPT) H1/H2. Here, we have explored the influence of these genes in our incident c... Brain journals http://www.medworm.com/rss/comments.php?id=2932792 Tue, 27 Oct 2009 17:42:24 +0100 2932792 http://www.medworm.com/index.php?rid=2932791&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2947%3Frss%3D1 We have carried out a systematic review of the case files of 242 donors with pathologically verified Parkinson's disease at the Queen Square Brain Bank for Neurological Disorders in an attempt to corroborate the data-driven subtype classification proposed by Lewis and colleagues (Heterogeneity of Parkinson's disease in the early clinical stages using a data driven approach. J Neurol Neurosurg Psychiatry 2005; 76: 343–8). Cases were segregated into earlier disease onset (25%), tremor dominant (31%), non-tremor dominant (36%) and rapid disease progression without dementia (8%) subgroups. We found a strong association between a non-tremor dominant disease pattern and cognitive disability. The earlier disease onset group had the longest duration to death, and greatest delay to the onset ... Brain journals http://www.medworm.com/rss/comments.php?id=2932791 Tue, 27 Oct 2009 17:42:24 +0100 2932791 http://www.medworm.com/index.php?rid=2932790&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2932%3Frss%3D1 The objective of this study was to examine case-by-case variability in patterns of grey matter atrophy in subjects with the behavioural variant of frontotemporal dementia and to investigate whether behavioural variant of frontotemporal dementia can be divided into distinct anatomical subtypes. Sixty-six subjects that fulfilled clinical criteria for a diagnosis of the behavioural variant of frontotemporal dementia with a volumetric magnetic resonance imaging scan were identified. Grey matter volumes were obtained for 26 regions of interest, covering frontal, temporal and parietal lobes, striatum, insula and supplemental motor area, using the automated anatomical labelling atlas. Regional volumes were divided by total grey matter volume. A hierarchical agglomerative cluster analysis using Wa... Brain journals http://www.medworm.com/rss/comments.php?id=2932790 Tue, 27 Oct 2009 17:42:24 +0100 2932790 http://www.medworm.com/index.php?rid=2932789&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2922%3Frss%3D1 Frontotemporal dementia (FTD) is a clinical syndrome with a heterogeneous molecular basis. The neuropathology associated with most FTD is characterized by abnormal cellular aggregates of either transactive response DNA-binding protein with Mr 43 kDa (TDP-43) or tau protein. However, we recently described a subgroup of FTD patients, representing around 10%, with an unusual clinical phenotype and pathology characterized by frontotemporal lobar degeneration with neuronal inclusions composed of an unidentified ubiquitinated protein (atypical FTLD-U; aFTLD-U). All cases were sporadic and had early-onset FTD with severe progressive behavioural and personality changes in the absence of aphasia or significant motor features. Mutations in the fused in sarcoma (FUS) gene have recently been identifie... Brain journals http://www.medworm.com/rss/comments.php?id=2932789 Tue, 27 Oct 2009 17:42:24 +0100 2932789 http://www.medworm.com/index.php?rid=2932788&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2909%3Frss%3D1 The mammalian brain is a remarkably complex organ comprising millions of neurons, glia and various other cell types. Its impressive cytoarchitecture led to the long standing belief that it is a structurally static organ and thus very sensitive to injury. However, an area of striking structural flexibility has been recently described at the centre of the brain. It is the subependymal zone of the lateral wall of the lateral ventricles. The subependymal zone—like a beating heart—continuously sends new cells to different areas of the brain: neurons to the olfactory bulbs and glial cells to the cortex and the corpus callosum. Interestingly, the generation and flow of cells changes in response to signals from anatomically remote areas of the brain or even from the external environmen... Brain journals http://www.medworm.com/rss/comments.php?id=2932788 Tue, 27 Oct 2009 17:42:24 +0100 2932788 http://www.medworm.com/index.php?rid=2932787&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2906%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2932787 Tue, 27 Oct 2009 17:42:23 +0100 2932787 http://www.medworm.com/index.php?rid=2932786&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2903%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2932786 Tue, 27 Oct 2009 17:42:23 +0100 2932786 http://www.medworm.com/index.php?rid=2932785&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F11%2F2901%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2932785 Tue, 27 Oct 2009 17:42:23 +0100 2932785 http://www.medworm.com/index.php?rid=2873876&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F10%2Fe129%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2873876 Thu, 08 Oct 2009 15:07:40 +0100 2873876 http://www.medworm.com/index.php?rid=2873875&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F10%2Fe128%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2873875 Thu, 08 Oct 2009 15:07:40 +0100 2873875 http://www.medworm.com/index.php?rid=2873874&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F10%2Fe127%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2873874 Thu, 08 Oct 2009 15:07:40 +0100 2873874 http://www.medworm.com/index.php?rid=2873873&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F10%2Fe126%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2873873 Thu, 08 Oct 2009 15:07:40 +0100 2873873 http://www.medworm.com/index.php?rid=2873872&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F10%2Fe125%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2873872 Thu, 08 Oct 2009 15:07:40 +0100 2873872 http://www.medworm.com/index.php?rid=2873871&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F10%2Fe124%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2873871 Thu, 08 Oct 2009 15:07:40 +0100 2873871 http://www.medworm.com/index.php?rid=2873870&cid=s_32201_25_f&fid=32201&url=http%3A%2F%2Fbrain.oxfordjournals.org%2Fcgi%2Fcontent%2Fshort%2F132%2F10%2Fe123%3Frss%3D1 (Source: Brain) Brain journals http://www.medworm.com/rss/comments.php?id=2873870 Thu, 08 Oct 2009 15:07:40 +0100 2873870