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
Aberrant interhemispheric homotopic functional and structural connectivity in amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is an idiopathic and fatal neurodegenerative disease of the human motor system. While microstructural alterations in corpus callosum (CC) have been identified as a consistent feature of ALS, studies directly examining interhemispheric neural connectivity are still lacking. To shed more light on the pathophysiology of ALS, the present study aims to examine alterations of interhemispheric structural and functional connectivity in individuals with ALS. ⋯ The present data provided direct evidence confirming and extending the view of impaired interhemispheric neural communications mediated by CC, providing a new perspective for examinations and understanding the pathophysiology of ALS.
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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
rTMS affects working memory performance, brain activation and functional connectivity in patients with multiple sclerosis.
To investigate the effects of high-frequency repetitive transcranial magnetic stimulation (rTMS) of the right dorsolateral prefrontal cortex (DLPFC) on working memory performance, while measuring task-related brain activation and task-related brain connectivity in patients with multiple sclerosis (MS). ⋯ In patients with MS, N-back accuracy improved while frontal hyperactivation (seen at baseline relative to HCs) disappeared after real-rTMS. Together with the changes in functional connectivity after real-rTMS in patients, these findings may represent an rTMS-induced change in network efficiency in patients with MS, shifting patients' brain function towards the healthy situation. This implicates a potentially relevant role for rTMS in cognitive rehabilitation in MS.
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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.