Neurological Sciences
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Factors affecting the retrieval of famous names
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Abstract Tests of famous faces are used to study language and memory. Yet, the effect of stimulus properties on performance has not
been fully investigated. To identify factors influencing proper name retrieval and to probe stimulus-specific parameters within
proper name lexicon, we analysed the results obtained by 300 healthy participants on a test of famous faces that includes
74 personalities. A factor analysis yielded five main factors that were characterized by language (national or foreign names),
epoch of peak popularity (current, recent or past) and occupation (politicians, entertainment and sports) ...
Source: Neurological Sciences - November 11, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Heterogeneous seizure manifestations in Hypomelanosis of Ito: report of four new cases and review of the literature
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We report
on four new HI patients presenting with heterogeneous seizure manifestations and we review the literature concerning epileptic
seizures in HI. At one extreme, there are patients with generalized seizures well controlled by drug treatment, whereas at
the opposite there are patients with severe, often pharmacoresistant, focal seizures. The genetic substrate for HI syndrome
is not homogenous and only partially understood. Further researches are required to shed light on the pathogenesis of HI phenotypes.
Content Type Journal ArticleCategory Review ArticleDOI 10.1007/s10072-009-0160-5Authors
Komi Assogba, Ce...
Source: Neurological Sciences - November 10, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Borrelia burgdorferi, a great chameleon: know it to recognize it!
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We describe a clinical case of neuroborreliosis that occurred in the absence of classical
erythema migrans or arthralgia. Magnetic resonance imaging findings compatible with simil-vasculitis and demyelinating lesions
associated with the presence of anti-B. burgdorferi antibodies in the plasma or cerebrospinal liquid is an indication for antimicrobial treatment against B. burgdorferi. An early diagnosis and a prompt establishment of an adequate antibiotic treatment is needed for a successful recovery.
Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10072-009-0175-yAuthors
Iolanda Santino, Sapienza Univer...
Source: Neurological Sciences - November 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Treatment of multiple sclerosis: role of natalizumab
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Abstract The results on relapse rate and disease progression of available drugs for multiple sclerosis are shown, as well as their
most relevant side effects. Results from pivotal and long-term follw-up studies support the efficacy and safety of intererons
and glatiramer acetate. The treatment with mitoxantrone is limited by the occurrence of infertility, cardiotoxicy and leukaemia.
Efficacy and tolerability of natalizumab are undisputable, compared to other drugs. Risks related to its treatment are PML,
opportunistic infections, hepatotoxicity, melanoma, and their occurrence needs to be more exactly assesse...
Source: Neurological Sciences - November 3, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Autologous haematopoietic stem-cell transplantation in multiple sclerosis: benefits and risks
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Abstract Autologous haematopoietic stem-cell transplantation has been evaluated over the last years as a possible new therapeutic strategy
in severe forms of multiple sclerosis unresponsive to the approved therapies. Up to now, more than 400 patients have been
treated and numerous are the phase I and phase II studies which addressed the feasibility of this treatment, the efficacy,
side effects and transplant-related mortality. The clinical response is strongly related to the intensity of the conditioning
regimen utilized as well as to the phase of the disease course in which the therapy is carried out. Rapid...
Source: Neurological Sciences - November 3, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Mitoxantrone: benefits and risks in multiple sclerosis patients
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Abstract Mitoxantrone (MTX) is a synthetic antineoplastic cytotoxic drug, active both on proliferative and non-proliferative cells.
The efficacy of MTX has been suggested by many open-label or observational studies and demonstrated in four randomized controlled
clinical trials (RCTs). It is indicated for reducing neurological disability and the frequency of clinical relapses in patients
with progressive relapsing and worsening relapsing–remitting MS patients. The short-term most frequent adverse events observed
in RCTs have been nausea/vomiting, alopecia, an increased risk of urinary and respiratory tract ...
Source: Neurological Sciences - November 3, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Safety profile of Tysabri: international risk management plan
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Abstract Therapeutic monoclonal antibodies are potent new tools for a molecular targeted approach to modify the course of multiple
sclerosis (MS). Natalizumab is a monoclonal antibody targeted against alpha-4 integrin that has proved to be very effective
in the treatment of MS. It is well tolerated, although severe side effects have been reported that have conditioned its use
as a second-line drug for the treatment of MS. The clinical benefit of natalizumab should be weighed carefully against the
potential risk of serious adverse events. Therefore, risk management plans have already been developed in order t...
Source: Neurological Sciences - November 3, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Cyclophosphamide as second-line therapy in multiple sclerosis: benefits and risks
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Abstract Cyclophosphamide (Cy) is an alkylating agent used over the past 40 years to halt rapidly progressive forms of multiple sclerosis
(MS). High doses of Cy produce marked immunosuppression and an anti-inflammatory immune deviation. Cy is most effective in
young patients, with very active MS (frequent relapses, rapid accumulation of disability, and gad+ lesions on brain MRI).
Monthly intravenous pulses of Cy for 1 year, followed by bimonthly pulses for the second year are a well-tolerated protocol
in MS. Most side effects (mild alopecia, nausea and vomiting, and cystitis) are transient, dose de...
Source: Neurological Sciences - November 3, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
New oral drugs for multiple sclerosis
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Abstract Disease-modifying treatments are now available in relapsing–remitting and secondary progressive multiple sclerosis (MS), and
their beneficial effects have been shown in several clinical studies. However, as these treatments are only partially effective
in halting the MS disease process and are frequently associated with side effects and suboptimal patient adherence, new oral
therapeutic approaches are warranted. This review focuses on advances in current and novel oral treatment approaches for MS.
Several pivotal reports have provided promising results for new oral therapies evaluating the safety ...
Source: Neurological Sciences - November 3, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
The pharmacovigilance program on natalizumab in Italy: 2 years of experience
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Abstract At the end of 2006 a country-based surveillance program on natalizumab therapy in multiple sclerosis was settled in Italy
by a collaborative effort of the Italian Drug Agency (AIFA) and a group of experts and neurologists appointed by the National
Society of Neurology (SIN). After 2 years, 1,818 patients are registered in the database. The majority of cases (88.6%) failed
the therapy with beta interferon or glatiramer acetate and had relapses or accumulated disability during immunomodulating
treatment, while 11.4% of patients enrolled in the surveillance study were not previously treated with i...
Source: Neurological Sciences - November 3, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Interleukin-1B and interleukin-1 receptor antagonist gene polymorphisms in Greek multiple sclerosis (MS) patients with bout-onset MS
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Abstract We investigated the association of specific polymorphisms of the interleukin IL-1b (AvaI −511 and TaqI +3,953) and IL-1 receptor antagonist (IL-1RN) (a variable number of tandem repeats; VNTR) genes with both the susceptibility
to and the clinical characteristics in Greek multiple sclerosis (MS) patients cohort with bout-onset. Genotypes were determined
from 351 patients with clinically definite MS and 375 age- and sex-matched healthy controls. Our results showed no significant
differences in the distribution of these polymorphisms between MS patients and controls. Furthermore, stratification for c...
Source: Neurological Sciences - October 30, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
The existence of phonatory instability in multiple sclerosis: an acoustic and electroglottographic study
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The objective of the study was to investigate the phonatory function
in MS patients using objective measures. The speech of 64 MS patients and 64 pair-matched controls was recorded and analyzed
using electroglottography, a physiological method that measures the electrical conductance during the opening and closing
phases of the vibrating vocal folds. Electroglottographic parameters were correlated with clinical and MRI features. Most
of the variables that have been used for the analysis of voice (vocal jitter P = 0.041, standard deviation of the average fundamental frequency P = 0.018, and the mean ...
Source: Neurological Sciences - October 30, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Central pontine and extrapontine myelinolysis despite careful correction of hyponatremia: clinical and neuropathological findings of a case
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We report clinical, radiological and pathological findings in a patient with central pontine and extrapontine myelinolysis.
The patient was a 61-year-old woman who had a radical mastectomy for breast cancer. Based on clinical evidence, acute hyponatremia
had set in only a few days before onset of symptoms. The patient’s disease progressed in two stages and became more severe
during slow hyponatremia correction after 9 days from onset. Diffusion MRI provided early evidence of neurological lesions.
In spite of a therapeutic attempt, the patient died unexpectedly 18 days after onset of her neurological disease...
Source: Neurological Sciences - October 30, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Unexplained dyspnea in an old patient with recurrent stroke: platypnea–orthodeoxia syndrome and evidence of patent foramen ovale
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Abstract Platypnea–orthodeoxia is a syndrome characterized by dyspnea and hypoxemia in orthostatism relieved by supine position. This
phenomenon is frequently associated with interatrial communication, mainly patent foramen ovale. The association of platypnea–orthodeoxia
syndrome with recurrent stroke and patent foramen ovale is extremely uncommon. A 73-year-old woman experienced recurrent attacks
of dyspnea after an ischemic stroke. Arterial blood gas analysis changes in upright and supine position confirmed the diagnosis
of platypnea–orthodeoxia syndrome. Contrast-enhanced transthoracic echocardiogra...
Source: Neurological Sciences - October 30, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Cystic neurohypophysial germinoma associated with moyamoya disease
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This report describes a case of
a cystic neurohypophysial germinoma associated with moyamoya disease. A 15-year-old girl with central diabetes insipidus was
admitted to the hospital. Radiological examination revealed a suprasellar tumor and moyamoya disease. Surgery and pathological
study confirmed pure germinoma. After the patient was treated with radiation and chemotherapy, the tumor was controlled without
any effect on the moyamoya vessels. This report describes a rare incidental combination of moyamoya disease and a suprasellar
germinoma. In the management of such cases, physicians should be aware of the symptoms,...
Source: Neurological Sciences - October 30, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Late-onset choreoathetotic syndrome following heart surgery
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Abstract Choreoathetotic syndromes are frequently observed in children after congenital cardiopathy surgery. To report the case of
an adult patient who developed a choreoathetotic syndrome after cardiac operation, probably related to a transitory hypometabolism
of basal ganglia. A 52-year-old patient underwent heart surgery under circulatory arrest and deep hypothermia, for type III
dissecting thoracic aorta aneurysm. Two weeks later she developed an acute choreic syndrome. The positron emission tomography
using fluorodeoxyglucose (FDGC-PET) showed a bilateral hypometabolism of basal ganglia. After haloperid...
Source: Neurological Sciences - October 27, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Dementia and capsular genu ischemia in patients with severe bacterial meningitis
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Abstract Infarction in the genu of the internal capsule causes dementia that is characterized by abulia, lethargy and memory loss without
obvious motor palsy (capsular genu syndrome). We found infarction or decreased cerebral blood flow in the genu of the internal
capsule in 6 of 13 patients with severe bacterial meningitis. Four of these six patients developed post-meningitis dementia,
characterized by abulia, lethargy, and memory loss. Of 24 patients with viral meningitis, none developed capsular genu ischemia
or post-meningitis dementia. In patients with severe bacterial meningitis, capsular genu ischemia...
Source: Neurological Sciences - October 16, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
The first case history of multiple sclerosis: Augustus d’Esté (1794–1848)
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Abstract The personal diary of Sir Augustus d’Esté, born 1794 grandson of King George III of England, reveals a medical history strongly
suggesting that Augustus suffered from multiple sclerosis (MS). It could well be the first record of a person having this
disease. Charcot coined the term sclérose en plaques 20 years after the death of this patient in 1848. The onset of this man’s
MS seems to have been in 1822 with bilateral optic neuritis, the disease gradually developing in the classic manner with bouts
derived from different loci in the central nervous system and eventually a secondary progre...
Source: Neurological Sciences - October 16, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Alzheimer’s disease and endothelial dysfunction
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Abstract Recent studies suggest strong interactions between cerebrovascular and Alzheimer’s disease (AD) pathology. These conditions
share common risk factors and individuals having both frequently show greater cognitive impairment than those affected by
only one disease. Many studies point to early vascular dysregulations in AD. The exchange between vascular and neural cells
occurs through mechanisms not completely understood, involving interactions among endothelial, glial, neuronal and smooth
muscle cells within the neurovascular unit. Studies suggest that the dysregulation of the unit is likely associa...
Source: Neurological Sciences - October 16, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Treatment and outcome of children with cerebral cavernomas: a survey on 32 patients
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Abstract We prospectively followed-up 32 pediatric patients with cerebral cavernomas (CCs) to better define surgical indications in
this population. Three groups of patients were identified: (a) children with macrohemorrhage (21 patients, 65.6%), (b) children
with localized or diffuse headache (6 patients, 18.8%) and (c) children with epilepsy (5 patients, 15.6%). Surgery was performed
in 28 out of the 32 (87.5%) subjects. New transient post-operative neurological deficits were observed in two children. One
child developed a post-operative hematoma. At a median follow-up of 4 years (range 1–11 ye...
Source: Neurological Sciences - October 16, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Utilization and outcome of thrombolytic therapy for acute stroke in Pakistan
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In conclusion, the utilization of thrombolytic
therapy is low and complication rate is high. Interventions for training physicians are needed.
Content Type Journal ArticleCategory Brief CommunicationDOI 10.1007/s10072-009-0159-yAuthors
Mohammad Wasay, Aga Khan University Department of Neurology and Medicine Stadium Road Karachi 74800 PakistanHazim Barohi, Aga Khan University Department of Neurology and Medicine Stadium Road Karachi 74800 PakistanAbdul Malik, Liaquat National Hospital Department of Neurology Karachi PakistanAdnan Yousuf, Aga Khan University Department of Neurology and Medicine Stadium Road Karachi 748...
Source: Neurological Sciences - October 9, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Concordance between severity of disease, disability and health-related quality of life in Myasthenia gravis
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Abstract Aim of this study is to verify whether there is concordance between disease’s severity, health-related quality of life (HRQoL)
and disability in patients with Myasthenia gravis (MG). 102 MG patients were clustered on the basis of HRQoL and disability
scores into three groups: low disability and low HRQoL decrement (51 patients), intermediate disability and HRQoL decrement
(28 patients), severe disability and high HRQoL decrement (23 patients). Cross tabulation with symmetric measures (Cramer’s V and Contingency Coefficient) was used to verify the relationships between disease severity groups...
Source: Neurological Sciences - October 9, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Familial hemifacial spasm and determinants of late onset
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Abstract The role of hypertension in the late onset of hemifacial spasm (HFS) is evaluated in a family, spanning four generations.
Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed a variable anatomical relationship between
nervous and vascular structures in the symptomatic cerebello-pontine angle. In one case, showing neurovascular conflict (NVC),
microvascular surgical decompression was followed by clinical resolution of HFS. Neuroimaging suggesting NVC was found in
all symptomatic patients of the last two generations and in three younger subjects not affected by HFS. As a ...
Source: Neurological Sciences - October 8, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Leprosy: report of a case with severe peripheral neuropathy
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Abstract Leprosy (Hansen’s disease) is a chronic granulomatous infectious disease, caused by Mycobacterium leprae, with cutaneous and neurological manifestations. Leprosy is very rare in Europe but some cases are reported, especially among
people coming from endemic areas. Here, we report a case of Hansen’s disease and emphasize the importance of a prompt diagnosis
and treatment also in non-endemic areas.
Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10072-009-0152-5Authors
Paolo Aridon, University of Palermo Dipartimento Universitario di Neuroscienze Cliniche (DiNeC) Via Gaetano La Log...
Source: Neurological Sciences - October 8, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Reversible posterior leukoencephalopathy syndrome in childhood: report of nine cases and review of the literature
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We presented nine patients with RPLS who had primary diagnoses such as acute post-streptococcal glomerulonephritis,
idiopathic hypertension, the performing of intravenous immunoglobulin for infection with crescentic glomerulonephritis, erythrocyte
transfusion for severe iron deficiency, l-asparaginase treatment for acute lymphoblastic leukemia and performing of granulocyte-colony stimulating factor for ulcerative
colitis due to neutropenia. Early recognition of RPLS as complication during different diseases and therapy in childhood may
facilitate precise diagnosis and appropriate treatment.
Content Type Journal Arti...
Source: Neurological Sciences - October 7, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Cerebral venous thrombosis with dural arteriovenous fistulas and antiphospholipid syndrome: a case report
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Content Type Journal ArticleCategory Letter to the EditorDOI 10.1007/s10072-009-0166-zAuthors
Yasuo Miki, Aomori Prefectural Central Hospital Department of Neurology Higashi Tsukurimichi 2-1-1 Aomori JapanMasahiko Tomiyama, Aomori Prefectural Central Hospital Department of Neurology Higashi Tsukurimichi 2-1-1 Aomori JapanAkira Arai, Aomori Prefectural Central Hospital Department of Neurology Higashi Tsukurimichi 2-1-1 Aomori JapanTamaki Kimura, Aomori Prefectural Central Hospital Department of Neurology Higashi Tsukurimichi 2-1-1 Aomori JapanChieko Suzuki, Aomori Prefectural Central Hospital Department of Neurology Hig...
Source: Neurological Sciences - October 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Psychiatric symptoms in Parkinson’s disease assessed with the SCL-90R self-reported questionnaire
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Abstract The frequency of psychopathological symptoms in patients with Parkinson’s disease (PD) is often underestimated because of
the lack of comprehensive evaluation tools. A total of 486 consecutive non-demented PD patients completed the Symptom Checklist
90 Revised (SCL-90R) self-reported questionnaire, a validated tool for the assessment of psychopathological symptoms on nine
dimensions. Somatization, depression, anxiety and obsessive–compulsive behaviors were reported by nearly half of the PD patients.
They were more likely to occur in females. Disease-related factors such as duration, severity and...
Source: Neurological Sciences - October 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Thoracic extradural haematoma after epidural anaesthesia
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Abstract The administration of analgesics to the thoracic spine is established practice in the operating room, minimizing the need
for systemic anaesthetic administration during thoracic surgery. Complications arising from thoracic epidural anaesthesia
are uncommon but potentially disastrous. Here, we report the case of a 43-year-old woman who developed a thoracic epidural
haematoma with paraplegia a few hours after the removal of an epidural catheter. The patient underwent emergency thoracic
laminectomy and clot evacuation. After a 4 months period, there was almost complete neurological recovery. Epidu...
Source: Neurological Sciences - October 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Isolated ataxia after pure left insular cortex infarction
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In conclusions, left insular cortex
lesions should be considered in the differential diagnosis of isolated truncal ataxia.
Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10072-009-0164-1Authors
Li-Min Liou, Kaohsiung Medical University Department of Neurology, Kaohsiung Municipal Hsiao-kang Hospital Kaohsiung TaiwanYuh-Cherng Guo, Kaohsiung Medical University Department of Neurology, Kaohsiung Municipal Hsiao-kang Hospital Kaohsiung TaiwanChiou-Lian Lai, Kaohsiung Medical University Department of and Master’s Program in Neurology, Faculty of Medicine, College of Medicine Kaohsiung TaiwanChin-Ling Tsai...
Source: Neurological Sciences - October 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Cerebellar degeneration and ocular myasthenia gravis in a patient with recurring ovarian carcinoma
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We report a patient with history of ovarian carcinoma that presented with ptosis, diplopia and gait ataxia. Neurophysiological
examination and laboratory tests revealed the presence of MG and PCD. An integrated FDG-PET/contrast-enhanced CT scan showed
tumor recurrence. This is to the best of our knowledge the first association of MG and PCD with recurring ovarian carcinoma.
Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10072-009-0154-3Authors
Pietro Caliandro, Università Cattolica del Sacro Cuore Neurology Department, Istituto di Neurologia Pol. “A: Gemelli” Largo Gemelli 8 00168 Rome ItalyMarco ...
Source: Neurological Sciences - October 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Advanced age as a contraindication to microvascular decompression for drug-resistant trigeminal neuralgia: evidence of prejudice?
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Abstract Due to the alleged higher risk of complications of microvascular decompression for trigeminal neuralgia in elderly we evaluated
its age-related results. A retrospective analysis of clinical outcome and complications was performed in 476 patients affected
by drug-resistant trigeminal neuralgia who underwent microvascular decompression. As much as 117 patients older than 65 years
(Group 1) and 359 under the age of 65 (Group 2) were followed-up for a period of 7–138 months. Pain relief was complete without
medication in 84.5% in Group 1. Morbidity included slight trigeminal hypoesthesia in ...
Source: Neurological Sciences - October 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
A case report of cranial polyneuropathy in Behcet’s disease
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We report a 19-year-old man with BD who presented
with cranial polyneuropathy secondary to aseptic meningitis. He made a gradual though incomplete recovery with steroid therapy.
Cranial polyneuropathy secondary to meningeal involvement can be a presentation of n-BD, and Behcet’s disease should be a
consideration in patients with idiopathic cranial polyneuropathy.
Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10072-009-0162-3Authors
Ali Alrefai, Jordan University of Science and Technology (JUST) Department of Neuroscience PO Box 3030 Irbid 22110 JordanKhalid El-Salem, Jordan University of Science an...
Source: Neurological Sciences - October 6, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Harmful effect of kainic acid on brain ischemic damage is not related to duration of status epilepticus
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This study
indicates that there is dissociation between stopping status epilepticus and modifying its long-term consequences.
Content Type Journal ArticleCategory Brief CommunicationDOI 10.1007/s10072-009-0115-xAuthors
Henry Hasson, Albert Einstein College of Medicine of Yeshiva University Saul R. Korey Department of Neurology Bronx NY USASamit Malhotra, SUNY Downstate Medical Center Department of Neurology Brooklyn NY USAFilippo S. Giorgi, University of Pisa Section of Neurology, Department of Neuroscience Pisa ItalyDaniel M. Rosenbaum, SUNY Downstate Medical Center Department of Neurology Brooklyn NY USASolomon L. ...
Source: Neurological Sciences - September 25, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Perceptive aspects of visual aura
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Abstract Visual aura is the most common feature associated with migraine, though it can occur separately. In both cases it often represents
a dramatic event, especially for patients who experience it for the first time. Besides, its subjective characteristics may
illuminate on the functional architecture of the visual cortex. Repetitive events of migraine and visual aura have been suggested
to affect the visual system in the long run, both on the cortical and precortical level. In effect, objective investigation
of visual functions in patients support the idea that a selective damage does occur, so that more...
Source: Neurological Sciences - September 25, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Speech-induced blepharospasm
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We report
the clinical vignette of two patients manifesting an unusual presentation of primary blepharospasm, specifically triggered
by voiced speech and associated with an otherwise ‘typical’ presentation of primary adult-onset dystonia in the lower face,
larynx or upper limb. Speech-induced primary blepharospasm seems a rare occurrence, representing 1.3% of our clinic-based
series of 149 patients with primary adult-onset primary blepharospasm. In these atypical patients, the feature of speech inducibility
suggests that the abnormal surrounding inhibition between cortical subregions representing laryngeal and orb...
Source: Neurological Sciences - September 24, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Progressive necrotic encephalopathy following tacrolimus therapy for liver transplantation
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We describe a 57-year-old man who underwent liver transplantation and was
started on immunosuppressive therapy with tacrolimus (FK506). Six months later, he started complaining of a progressive motor
and sensory impairment of the left side, together with cognitive impairment. Brain MRI showed an enlarging lesion of the white
matter with peripheral contrast enhancement. PET study indicated severe hypometabolism in the right hemisphere and spectroscopic
MRI showed a peak of choline and relative reduction of other metabolites. Findings of CSF examinations and cultures, serology,
and molecular techniques were normal....
Source: Neurological Sciences - September 24, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Dopamine transporter (DAT1) VNTR polymorphism in 12 Indian populations
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Abstract The dopamine transporter (DAT1) is a membrane spanning protein that binds the neurotransmitter dopamine and performs re-uptake
of dopamine from the synapse into a neuron. The gene encoding DAT1 consists of 15 exons spanning 60 kb on chromosome 5p15.32.
Several studies have investigated the possible associations between variants in DAT1 gene and psychiatric disorders. The present
study aimed to determine the distribution of the variable number of tandem repeat (VNTR) polymorphism in the 3′ untranslated
region of DAT1 in 12 Indian populations. A total of 471 healthy unrelated individuals in 12 ...
Source: Neurological Sciences - September 24, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Paragangliomas of head and neck: a treatment option with CyberKnife radiosurgery
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In conclusion, CyberKnife radiosurgery appears to be both safe and effective
in the treatment of skull base paragangliomas. Determining whether long-term complications will arise will require further
investigation.
Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s10072-009-0138-3Authors
Livia C. Bianchi, CyberKnife Center CDI Milan ItalyMarcello Marchetti, Fondazione IRCCS Istituto Neurologico Carlo Besta Department of Neurosurgery, Division of Radiotherapy Milan ItalyLorenzo Brait, CyberKnife Center CDI Milan ItalyAchille Bergantin, CyberKnife Center CDI Milan ItalyIda Milanesi, Fondazione IRCCS Is...
Source: Neurological Sciences - September 23, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Spinocerebellar ataxia type 11 in the Chinese Han population
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Abstract The spinocerebellar ataxias (SCAs) are a clinically and genetically heterogeneous group of neurodegenerative diseases. Researchers
have recently found that SCA type 11 (SCA11) is associated with mutations in the TTBK2 gene. In our previous work, we performed mutation detection in SCA1, 2, 3, 6, 7, 8, 10, 12, 17 and dentatorubral-pallidoluysian
atrophy gene in Chinese SCA patients, but the genes responsible for approximately 40% of our patients have not yet been identified.
To investigate the frequency of SCA11 in Chinese SCA patients, we examined the TTBK2 gene in 68 unrelated probands diagnosed with...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Muscle carnitine deficiency: adult onset lipid storage myopathy with sensory neuropathy
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We report herein a 29-year-old man with muscle carnitine deficiency who developed not only a lipid storage myopathy, but also
a severe sensory neuropathy. Oral therapy with levo-carnitine (3 g per day) for 3 months produced a remarkable improvement
of the myopathy and sensory neuropathy. Six months later, he remained in good condition under strict dietary control. This
report emphasizes that severe neuropathy may occur in some patients with muscle carnitine deficiency, and highlights the need
for the neurologist’s familiarity with those afflicted to achieve optimal clinical management.
Content Type Journ...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Novel MAPT Val75Ala mutation and PSEN2 Arg62Hys in two siblings with frontotemporal dementia
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Abstract A clinical and molecular overlap between Alzheimer’s disease (AD) and frontotemporal dementia (FTD) has been reported. Presenilins
have been associated with FTD or with FTD-like phenotype, while mutations in the MAPT gene have been linked to a clinical phenotype of AD. We performed a clinical and genetic examination in two FTD siblings
and their family tree has been reconstructed. We identified a novel Val75Ala MAPT mutation in one patient and in the other the Arg62His Presenilin2 mutation. The DNA variations identified, defined mutations
by frequency, per se are not causative of the disease. These...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Myeloneuropathy due to copper deficiency: clinical and MRI findings after copper supplementation
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In conclusion, in acquired copper-deficiency-associated myelopathy, correction of blood
and MRI alterations precedes that of neurological manifestations, which may remain suboptimal.
Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10072-009-0126-7Authors
Laura Bolamperti, Ospedale Maggiore della Carità Clinica Neurologica C.so Mazzini, 18 28100 Novara ItalyMaurizio A. Leone, Ospedale Maggiore della Carità Clinica Neurologica C.so Mazzini, 18 28100 Novara ItalyAlessandro Stecco, Amedeo Avogadro University Department of Radiology Novara ItalyMonica Reggiani, Amedeo Avogadro University Clinica Neurologica...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Irreversible striatal neuroimaging abnormalities secondary to prolonged, uncontrolled diabetes mellitus in the setting of progressive focal neurological symptoms
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We report a 56-year-old man with a prolonged, uncontrolled hyperglycemia (HbA1C:
13.8%) and striatal hyperintensity on T1-weighted MR imaging presenting as reversible focal neurological deficit and irreversible
neuroimaging abnormalities on the fourth month when blood sugar was under control (HbA1C 6.0 mg/dl). We hypothesize that neuroimaging
abnormalities in our case may be a sequence of an “ischemic insult” caused by prolonged, uncontrolled hyperglycemia. Whether
the signal abnormalities on neuroimaging studies or the clinical syndrome are reversible (patients with HCHB) or irreversible
(such as in our case...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Neuromyotonia in idiopathic hypoparathyroidism
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Abstract Α 62-year-old man was presented with bilateral cataract, proximal muscle weakness and difficulty in relaxing the hands after
voluntary contraction. Idiopathic hypoparathyroidism was diagnosed. Electrophysiological study showed spontaneous motor unit
action potentials, occurring and disappearing abruptly, as duplets, triplets and multiplets, with an intra-burst frequency
of up to 100 Hz and some of them waning in amplitude, findings consistent with neuromyotonia.
Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s10072-009-0140-9Authors
Thomas Zambelis, University of Athens D...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Neurosyphilis in the age of AIDS: clinical and laboratory features
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Abstract Neurosyphilis remains to be a challenging diagnostic possibility worldwide. The aim of our study was to identify and report
the clinical and laboratory profile of neurosyphilis, comparing features of HIV-infected and HIV-negative patients. A retrospective
investigation of all cases of neurosyphilis, defined as positive VDRL test on cerebrospinal fluid, diagnosed at Hospital das
Clínicas, Ribeirão Preto School of Medicine between January 1988 and December 2005, was carried out. We identified 35 patients
with a mean age of 42.1 years, 28.6% of them HIV infected and 74.3% of them were male. HIV-...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Size frequency distribution of the β-amyloid (aβ) deposits in dementia with Lewy bodies with associated Alzheimer’s disease pathology
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The objective is to study β-amyloid (Aβ) deposition in dementia with Lewy bodies (DLB) with Alzheimer’s disease (AD) pathology
(DLB/AD). The size frequency distributions of the Aβ deposits were studied and fitted by log-normal and power-law models.
Patients were ten clinically and pathologically diagnosed DLB/AD cases. Size distributions had a single peak and were positively
skewed and similar to those described in AD and Down’s syndrome. Size distributions had smaller means in DLB/AD than in AD.
Log-normal and power-law models were fitted to the size distributions of the classic and diffuse deposits, respective...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Self-help group and medication overuse headache: preliminary data
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The objective of the study is to investigate the benefits of joining a self-help group for patients with medication overuse
headache (MOH). A self-help group is a voluntary gathering of a small number of persons who share a common problem. Little
is known about support groups for people with chronic non-malignant pain such as MOH. Eight patients with refractory MOH attended
a self-help group twice a month. During the meetings, patients were asked to focus on their headache experiences. Our data
showed an increase in resourcefulness in coping with pain and a reduction in cephalalgiophobia. All patients reported general
...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Effects of treadmill training on walking economy in Parkinson’s disease: a pilot study
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This study evaluated whether the effects of treadmill training are associated with an improvement of walking economy. Ten
patients with idiopathic PD underwent treadmill training (30 min, three times a week for 4 weeks). Walking performance (Τimed
Up and Go, 6-min and 10-m walking tests) and metabolic function (oxygen uptake, heart and respiratory rate) were evaluated
before training, at the end of treatment and after 30 days with two different graded exercises (treadmill and cycloergometer).
Training significantly improved walking performance. Oxygen uptake, and heart and respiratory rates were signifi...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Syringomyelia associated with Chiari I malformation
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Abstract An 18-year-old man with progressive paraparesis, thermal hypoesthesia, sweating abnormalities, bladder dysfunction, severe
orthostatic hypotension, bilateral Babinski sign, underwent a brain MRI scan that showed downward displacement of cerebellar
tonsils through the foramen magnum, consistent with Chiari I malformation, compression of the brainstem–spinal cord junction,
and C1–D11 syringomyelia (6.5 mm diameter at C2 level) consistent with Chiari I syndrome. Suboccipital craniectomy and duraplasty
were performed. A C2 partial laminectomy and ablation of posterior arch of the atlas was perf...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
Lack of effect of cannabis-based treatment on clinical and laboratory measures in multiple sclerosis
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Abstract The endocannabinoid system (ECS) is involved in the pathophysiology of multiple sclerosis (MS), and relief from pain and spasticity
has been reported in MS patients self-medicating with marijuana. A cannabis-based medication containing Δ9-tetrahydrocannabinol and cannabidiol (Sativex®) has been approved in some countries for the treatment of MS-associated pain. The effects of this pharmaceutical preparation
on other clinically relevant aspects of MS pathophysiology, however, are still unclear. In 20 MS patients, we measured the
effects of Sativex® on clinically measured spasticity and on neurophys...
Source: Neurological Sciences - September 19, 2009 Category: Neurology Tags: Neurological Sciences Source Type: journals
