Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · May 2017
What does the ALSFRS-R really measure? A longitudinal and survival analysis of functional dimension subscores in amyotrophic lateral sclerosis.
ALS functional rating scale (revised) (ALSFRS-R) is the most widely used functional rating system in patients with amyotrophic lateral sclerosis (ALS). However, heterogeneity in ALSFRS-R progression renders analysis challenging. We have explored the characteristics of total ALSFRS-R, and ALSFRS-R subscores in longitudinal and survival models, to determine whether subscore analysis enhances the precision of the instrument. ⋯ Our analysis builds on previous knowledge of ALSFRS-R subscores. Decline in ALSFRS-R motor subscores in patients with spinal-onset disease, and decline in ALSFRS-R bulbar subscores in patients with bulbar-onset disease, may predate reported disease onset dates. Respiratory subscores were not prognostically informative after adjustment for bulbar and motor subscores. These results provide robust evidence that the ALSFRS-R should not be reported as a single combined score, but rather as domain specific subscores.
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J. Neurol. Neurosurg. Psychiatr. · May 2017
Review Meta AnalysisPredictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis.
To undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). ⋯ Several clinical variables were strongly associated with shorter survival in PSP and MSA. Results on most prognostic factors were consistent across methodologically diverse studies; however, the lack of commonality of prognostic factors investigated is a significant limitation.
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Chorea, a movement disorder characterised by a continuous flow of unpredictable muscle contractions, has a myriad of genetic and non-genetic causes. Although autoimmune processes are rare aetiology of chorea, they are relevant both for researchers and clinicians. The aim of this article is to provide a review of the epidemiology, clinical and laboratory features, pathogenesis and management of the most common autoimmune causes of chorea. Emphasis is given particularly to Sydenham's chorea, systemic lupus erythematosus, primary antiphospolipid antibody syndrome, paraneoplastic chorea and anti-N-methyl-d-aspartate receptor encephalitis.
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J. Neurol. Neurosurg. Psychiatr. · May 2017
An adverse lipid profile and increased levels of adiposity significantly predict clinical course after a first demyelinating event.
To investigate the prospective associations between adiposity and lipid-related variables and conversion to multiple sclerosis (MS), time to subsequent relapse and progression in disability. ⋯ Higher levels of adiposity, non-HDL and TC/HDL ratio were prospectively associated with a higher rate of disability progression, and higher adiposity and triglycerides were associated with relapse but not with conversion to MS. Improving the lipid profile and losing weight into the healthy range could reduce the accumulation of disability.
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J. Neurol. Neurosurg. Psychiatr. · May 2017
Posterior fossa decompression in Chiari I improves denervation of the paraspinal muscles.
To investigate whether posterior fossa decompression (PFD) could improve denervation of the paraspinal muscles in patients with Chiari I malformation (CMI). ⋯ In patients with CMI, treatment with PFD led to a decrease in the Bax/Bcl-2 ratio at both the mRNA and protein levels, indicating an attenuated susceptibility to apoptotic cell death. These data, coupled with the observed improvements in histopathological features of the myofibres, suggest that PFD in Chiari I ameliorates denervation of the paraspinal muscles.