Neurology
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To assess corticospinal tract involvement in patients with amyotrophic lateral sclerosis (ALS) by correlating diffusion tensor imaging (DTI) measures with intra- and extracranial central motor conduction time (CMCT) and clinical features of the patients. ⋯ Fractional anisotropy reflects functional abnormality of intracranial corticospinal tracts and can be used for objective evaluation of upper motor neuron impairment in amyotrophic lateral sclerosis.
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Diagnosis of the superior canal dehiscence syndrome (SCDS) relies on symptoms such as sound- or pressure-induced vertigo or oscillopsia, demonstration of sound or pressure-evoked vertical/torsional eye movements, and the presence of a defect in the bony roof overlying the superior semicircular canal. Lowered thresholds for eliciting vestibular-evoked myogenic potentials (VEMPs) provide additional conformation. ⋯ Ocular and cervical vestibular-evoked myogenic potentials evoked by air-conducted sound are equally useful in the diagnosis and follow-up of superior canal dehiscence syndrome. Stimulus thresholds are consistently lowered upon presentation and normalize after corrective surgery. Thresholds for bone vibration, in contrast, have a lower diagnostic yield.
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Clinical Trial
Sumatriptan normalizes the migraine attack-related increase in brain serotonin synthesis.
Altered serotonin (5-HT) neurotransmission has been implicated in the pathophysiology of migraine headache. ⋯ These findings point to a low cortical serotonergic tone in migraine patients interictally. Further, they demonstrate widespread increases in brain serotonin (5-HT) synthetic rate in migraine patients during attacks, and that triptans exert a negative feedback regulation of brain 5-HT synthesis concurrently with modulation of pain pathways.
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To determine the rate of disease progression in Charcot-Marie-Tooth disease type 1A (CMT1A). ⋯ Progression of CMT1A can be detected by both the CMT Neuropathy Score (CMTNS) and the Neuropathy Impairment Score (NIS). This supports the feasibility of clinical trials to detect a slowing of disease progression using either or both of these scales as outcome measures. Since the CMTNS combines symptoms, signs, and electrophysiology and the NIS is based solely on the neurologic examination, the two scales may be complementary.