Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jan 2010
Phenotypic spectrum of hereditary neuralgic amyotrophy caused by the SEPT9 R88W mutation.
Hereditary neuralgic amyotrophy (HNA), also known as hereditary brachial plexus neuropathy, has phenotypic and genetic heterogeneity. Mutations in the septin 9 (SEPT9) gene were recently identified in some HNA patients. The phenotypic spectrum of HNA caused by SEPT9 mutations is not well known. ⋯ The SEPT9 R88W mutation in this family causes selective involvement of the brachial plexus and upper-limb nerves. Wider and more universal recognition of clinical hallmarks and genetic counselling are of diagnostic importance for HNA caused by the SEPT9 mutation.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2010
Multicenter StudyA multicentre study on the clinical utility of post-traumatic amnesia duration in predicting global outcome after moderate-severe traumatic brain injury.
Past research shows that post-traumatic amnesia (PTA) duration is a particularly robust traumatic brain injury (TBI) outcome predictor, but low specificity limits its clinical utility. ⋯ Two PTA durations, 4 weeks and 8 weeks, emerged as particularly salient GOS probability thresholds that may aid prognostication after TBI.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2010
Diffusion tensor imaging detects age related white matter change over a 2 year follow-up which is associated with working memory decline.
Diffusion tensor imaging (DTI) is a sensitive method for detecting white matter damage, and in cross sectional studies DTI measures correlate with age related cognitive decline. However, there are few data on whether DTI can detect age related changes over short time periods and whether such change correlates with cognitive function. ⋯ DTI is sensitive to age related change in white matter ultrastructure and appears useful for monitoring age related white matter change even over short time periods.
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J. Neurol. Neurosurg. Psychiatr. · Jan 2010
Neuromyelitis optica-IgG (aquaporin-4) autoantibodies in immune mediated optic neuritis.
The clinical course of immune mediated optic neuritis (ON) will depend on the specific underlying inflammatory disease. These disorders have traditionally been classified according to clinical and MRI findings. ⋯ The proportion seropositive was 56% for NMO (n = 9), 0% for MSON (n = 28) and 5% for the remaining diagnostic categories (CRION (n = 19), RION (n = 17) and SION (n = 41)). Testing for NMO-IgG in patients with recurrent or severe ON who lack convincing evidence of MS may identify patients who would benefit from immunosuppression rather than MS directed immunomodulatory therapies.
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J. Neurol. Neurosurg. Psychiatr. · Dec 2009
A reappraisal of the value of lateral spread response monitoring in the treatment of hemifacial spasm by microvascular decompression.
Lateral spread response (LSR) to the electrical stimulation of a facial nerve branch is a specific electrophysiological feature of primary hemifacial spasm (HFS). The curative treatment of HFS is based on surgical microvascular decompression (MVD). However, the outcome of this procedure is not always satisfactory. ⋯ Monitoring MVD by recording LSRs intraoperatively could be of value not only to indicate the resolution of the vasculonervous conflict at the end of surgery, but also to predict a successful clinical outcome in the long term after the surgical intervention.