European journal of neurology : the official journal of the European Federation of Neurological Societies
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Case Reports
Leukodystrophies underlying cryptic spastic paraparesis: frequency and phenotype in 76 patients.
In chronic progressive spasticity of the legs many rare causes have to be considered, including leukodystrophies due to neurometabolic disorders. To determine the frequency of leukodystrophies and the phenotypic spectrum patients with cryptic spasticity of the legs were screened for underlying neurometabolic abnormalities. ⋯ Adult patients presenting with cryptic spasticity of the legs should be screened for underlying X-linked adrenoleukodystrophy/adrenomyeloneuropathy and lysosomal disorders, irrespective of the presence of additional complicating symptoms. Leukodystrophies may manifest as late as the sixth decade and hyperintensity of cerebral white matter on magnetic resonance FLAIR images is not obligatory.
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Migraine pathophysiology involves a complex interplay of processes wherein the hormonal, neurotransmitter and inflammatory pathways interact to influence the migraine phenotype. However, all studies pertaining to the role of genetic variants in migraine have been restricted to a specific pathway and none of the studies has looked into inter-pathway genetic analysis. Our aim was to combine all the genetic variants from our previously reported studies to conduct higher order gene-gene interaction analysis using different multi-analytical approaches. ⋯ The present study suggests interactions amongst hormonal, inflammatory and genome-wide associated variants but not with neurotransmitter pathway variants in migraine susceptibility.
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Hemodialysis (HD) may increase the risk of acute subdural hematoma (SDH) with high fatality, but the extent of this disease in non-western populations is unclear. The incidence of and fatality from SDH in patients with end-stage renal disease (ESRD) on HD were examined for an Asian population. ⋯ ESRD patients with HD were demonstrated to be at high risk of subsequent SDH and to have a high mortality risk from SDH. Proper care for HD patients is necessary to prevent the devastating disorder.
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Impairment of cognitive functions occurs in essential tremor (ET) although the mechanism is largely unknown. A possible association between cognitive performance and brain atrophy in ET patients was examined using neuropsychological tests and voxel-based morphometry (VBM). ⋯ A correlation between neurocognitive deficits in ETCI and GMV was observed suggesting that grey matter atrophy appears to be a correlate of cognitive impairment in ET.
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Randomized Controlled Trial Multicenter Study
Pooled analysis of the safety and tolerability of onabotulinumtoxinA in the treatment of chronic migraine.
OnabotulinumtoxinA was effective and well tolerated for prophylaxis of headache in patients with chronic migraine (CM) in two randomized, double-blind, placebo-controlled, phase 3 trials. To further assess the safety and tolerability of onabotulinumtoxinA in CM prophylaxis in adults, the pooled safety data from four double-blind, placebo-controlled trials were analyzed. ⋯ Multiple treatments with onabotulinumtoxinA at doses of 75-260 U administered every 12 weeks, and up to five treatment cycles, were well tolerated for the prophylaxis of headache in adults with CM.