MedWorm: Neurology Top 20

Natalizumab-associated progressive multifocal leucoencephalopathy: a practical approach to risk profiling and monitoring.

Practical Neurology — Thu, 26 Jan 2012 03:31:25 +0100

Authors: Hunt D, Giovannoni G Abstract Natalizumab reduces relapse frequency, delays onset of disease progression and improves disease outcomes in relapsing-remitting multiple sclerosis (MS) and is a cost-effective treatment for rapidly evolving severe relapsing-remitting MS. However, it is associated with the development of progressive multifocal leucoencephalopathy (PML), a serious opportunistic brain infection caused by a neurotropic strain of the JC virus (JCV). Until May 2011, 83 300 patients had received natalizumab for MS. One hundred and twenty-four patients had developed PML, of whom 23 (19%) died. In order to maximise the benefit-risk ratio of natalizumab for MS patients it is important to develop a strategy for risk profiling and monitoring for PML. Central to this is an...

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A practical approach to late-onset cerebellar ataxia: putting the disorder with lack of order into order.

Practical Neurology — Thu, 26 Jan 2012 03:31:35 +0100

Authors: van Gaalen J, van de Warrenburg BP Abstract The clinical management of cerebellar ataxia is challenging, mainly because ataxia is a symptom of many neurological diseases. Many types of ataxia disorders are genetic and some are extremely rare. Here, the authors suggest a diagnostic approach to ataxia developed around a case of sporadic, late-onset, slowly progressive ataxia. Clinical information such as age of onset, rate of progression, family history and certain non-cerebellar features can narrow the differential diagnosis. Brain MRI is almost obligatory and may reveal valuable diagnostic clues. Having ruled out structural lesions, the two other most common diagnoses are inflammatory and degenerative (including genetic) disorders. Although only a minority of underlying di...

[Review] Clinically isolated syndromes

Lancet Neurology — Sat, 21 Jan 2012 01:16:39 +0100

SummaryClinically isolated syndrome (CIS) is a term that describes a first clinical episode with features suggestive of multiple sclerosis (MS). It usually occurs in young adults and affects optic nerves, the brainstem, or the spinal cord. Although patients usually recover from their presenting episode, CIS is often the first manifestation of MS. The most notable risk factors for MS are clinically silent MRI lesions and CSF oligoclonal bands; weak or uncertain risk factors include vitamin D deficiency, Epstein-Barr virus infection, smoking, HLA genes, and miscellaneous immunological abnormalities. (Source: Lancet Neurology)

The usefulness of neuronavigation in functional hemispherectomy

Journal of Epilepsy and Clinical Neurophysiology — Mon, 30 Jan 2012 02:48:25 +0100

We report two cases of 5-year-old girls. The first one had a diagnosis of Rasmussens syndrome. The second one had a large porencephalic cyst secondary to perinatal cerebral ischemia. Despite trials of anticonvulsants, both patients deteriorated, and a functional hemispherectomy guided by neuronavigation was indicated and performed, with low morbidity and excellent seizure control. The neuronavigation proved to be a valuable guidance tool in performing the functional hemispherectomy, making the disconnections more accurate, and thus decreasing the surgical time and blood loss.Aplicabilidade da neuronavegação em hemisferectomia funcional As encefalopatias epilépticas catastróficas da infância compreendem condições graves que associam disfunção cerebral e crises epilépticas refrat...

The Need to Develop More Sensitive Tools to Accurately Detect Clinical Response to Treatment in ADHD

CNS Drugs — Mon, 06 Feb 2012 01:22:15 +0100

(Source: CNS Drugs)

Effects of lexicality and word frequency on brain activation in dyslexic readers.

Brain and Language — Sat, 07 Jan 2012 05:00:00 +0100

Authors: Heim S, Wehnelt A, Grande M, Huber W, Amunts K Abstract We investigated the neural basis of lexical access to written stimuli in adult dyslexics and normal readers via the Lexicality effect (pseudowords>words) and the Frequency effect (low>high frequent words). The participants read aloud German words (with low or high lexical frequency) or pseudowords while being scanned. In both groups, both Lexicality effect and Frequency effect involved Broca's region (areas 44 and 45). Whereas the effects were stronger for dyslexic than normal readers in area 44, area 45 showed the reverse pattern. These findings mimic recent results from an fMRI study on dyslexic primary school children, indicating that lexical access to written stimuli poses increased and enduring difficulties...

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Editorial Board

Parkinsonism and Related Disorders — Fri, 27 Jan 2012 01:46:47 +0100

(Source: Parkinsonism and Related Disorders)

Steady Diet of Mental Stimulation Might Reduce Alzheimer’s Risk

The Doctors Lounge - Neurology — Tue, 24 Jan 2012 01:06:25 +0100

Lifetime of reading, games lowered levels of brain plaques, researchers report (Source: The Doctors Lounge - Neurology)

Re: The beautiful and ethereal neurological exam

Annals of Neurology — Thu, 19 Jan 2012 05:00:00 +0100

(Source: Annals of Neurology)

Echoes from childhood—imitation in Gilles de la Tourette Syndrome

Movement Disorders — Wed, 25 Jan 2012 05:00:00 +0100

Unilateral thalamic stimulation safely improved fragile X–associated tremor ataxia: A case report

Movement Disorders — Fri, 27 Jan 2012 05:00:00 +0100

(Source: Movement Disorders)

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[In Context] News in brief

Lancet Neurology — Sat, 21 Jan 2012 01:16:38 +0100

Although research efforts in multiple sclerosis (MS) have focused largely on white matter pathology, grey matter lesions have emerged as a hallmark of the disease. A new study has taken a step further to show that cortical demyelinating lesions are common at early stages of MS and are inflammatory in nature and strongly associated with meningeal inflammation. The researchers looked at demyelinating lesions in cortical biopsy specimens obtained during diagnostic sampling of white matter lesions (N Engl J Med 2011; 365: 2188–97). (Source: Lancet Neurology)

Hypertension and longitudinal changes in cerebral blood flow. The smart‐mr study

Annals of Neurology — Fri, 03 Feb 2012 05:00:00 +0100

We examined the longitudinal association of hypertension, BP, and antihypertensive drugs with change in parenchymal cerebral blood flow (pCBF) in 575 patients with manifest atherosclerotic disease (mean age 57±10 years) from the SMART‐MR study. Total CBF was measured at baseline and at follow‐up with MR angiography and was expressed per 100mL brain volume as an indicator of cerebral perfusion. Automated brain segmentation was used to quantify brain tissue volumes and cerebrospinal fluid on MRI.Results:Mean (SD) baseline parenchymal CBF was 52.3 (9.8) mL/min/100mL and after 3.9 years (range=3.0‐5.8) of follow‐up declined to 50.7 (10.3) mL/min/100mL. Regression analyses, adjusted for age, sex, follow‐up time, and vascular risk showed that untreated and poorly controlled hypertensi...

Increased medial orbitofrontal [18F]fluorodopa uptake in Parkinsonian impulse control disorders

Movement Disorders — Sun, 05 Feb 2012 05:00:00 +0100

Conclusions:Increased monoaminergic activity in the medial orbitofrontal cortex might be associated with increased sensitivity for ICDs under dopamine‐replacement therapy in PD. © 2012 Movement Disorder Society (Source: Movement Disorders)

Microglia are required for astroglial toll‐like receptor 4 response and for optimal TLR2 and TLR3 response

Glia — Tue, 24 Jan 2012 01:12:05 +0100

In this study, we have rigorously purified astrocytes to determine their capacity for autonomous TLR response, in absence of microglia. We used flow cytometry and differential adhesion as well as a myeloid lineage‐specific suicide gene to purify astrocytes from mixed glial cultures and measured their response to TLR agonists. Our results show that the response of astrocytes to TLR2 and TLR3 agonists is greatly enhanced by, and response to TLR4 agonists is completely dependent on, the presence of functional microglia. In the case of the TLR4 response to lipopolysaccharide, microglia exert their effect on astrocytes at least partially through release of soluble mediators that directly activate or facilitate astrocyte responses. Our findings underline the contribution of glial crosstalk in ...

[Comment] Firategrast—natalizumab in a pill?

Lancet Neurology — Sat, 21 Jan 2012 01:16:38 +0100

The influx of lymphocytes and myeloid cells into the CNS is thought to be important in initiating and perpetuating multiple sclerosis (MS). Migration of leucocytes across biological barriers is a complex, multistep event that is mediated by the in-situ activation of integrins. Traditionally, MS has been believed to be mediated primarily by CD4 T cells. However, accumulating evidence also implies a role for B cells, monocytes, and dendritic cells in the pathogenesis of MS. Since very late activating antigen 4 (VLA-4; α4β1 integrin) is expressed on most activated leucocytes, α4β1 integrins are regarded as a highly relevant molecular target in MS. (Source: Lancet Neurology)

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Study Shows How Stress Triggers Immune System

The Doctors Lounge - Neurology — Mon, 23 Jan 2012 22:00:00 +0100

Conflict fires up molecules related to inflammation, researchers report (Source: The Doctors Lounge - Neurology)

Electroencephalografic abnormalities as prognostic factor in acute coma in non-epileptic children

Journal of Epilepsy and Clinical Neurophysiology — Mon, 30 Jan 2012 02:48:25 +0100

Conclusion: In spite of the fact that our sample is relatively small, we infer that epileptogenic paroxysms in the EEGs of non-epileptic children in coma is relatively common, even not observing a consistent association of this finding with high risk of death. (Source: Journal of Epilepsy and Clinical Neurophysiology)

Validity of a Protocol for Adult Self‐Report of Dyslexia and Related Difficulties

Dyslexia — Fri, 27 Jan 2012 01:40:49 +0100

ConclusionsSelf‐report scales of reading and of attention difficulties are useful for identifying adults with reading and attention difficulties which may confer risks on their children of related problems. It is important for research following children at family risk of dyslexia to be aware of these effects. Copyright © 2012 John Wiley & Sons, Ltd. (Source: Dyslexia)

Enhanced expression of tubulin‐specific chaperone protein a, mitochondrial ribosomal protein S27, and the DNA excision repair protein XPACCH in the song system of juvenile male zebra finches

Journal of Neurobiology — Wed, 18 Jan 2012 02:14:16 +0100

AbstractRecent evidence suggests that sexual dimorphisms in the zebra finch song system and behavior arise due to factors intrinsic to the brain, rather than being solely organized by circulating steroid hormones. The present study examined expression of 10 sex chromosome genes in the song system of 25‐day‐old zebra finches in an attempt to further elucidate these factors. Increased expression in males was confirmed for nine of the genes by real‐time qPCR using cDNA from individual whole telecephalons. In situ hybridization at the same age revealed specific, male‐enhanced mRNA for three of the nine genes in one or more song control nuclei. These genes encode tubulin‐specific chaperone A, mitochondrial ribosomal protein S27, and a DNA repair protein XPACCH. Based on what is curren...