Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
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J Clin Neurophysiol · Dec 2002
Comparative Study Clinical Trial Controlled Clinical TrialSmall fiber dysfunction predominates in Fabry neuropathy.
Fabry disease is an X-linked recessive disease with a reduction of lysosomal alpha galactosidase A and consecutive storage of glycolipids e.g., in the brain, kidney, skin, and nerve fibers. Cardinal neurologic findings are hypohidrosis, painful episodes, and peripheral neuropathy. So far, the neurophysiological findings regarding the extent of large and small fiber dysfunction are contradictory. ⋯ However, only six patients had pathologic VDT, 19 had increased CDT, and 25 had elevated HPDT at a high level of stimulation. In Fabry patients, small fiber dysfunction is more prominent than large fiber dysfunction, confirming previous findings of sural nerve biopsies. The results suggest a higher vulnerability of small-diameter nerve fibers than of the thickly myelinated fibers.
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This review describes the wide spectrum of episodic phenomena that can occur during sleep. These phenomena include arousal disorders of nonrapid eye movement (NREM) sleep, rapid eye movement (REM) sleep behavior disorder, movement disorders, psychiatric disorders, and epileptic seizures. Each of these entities is discussed in detail, focusing on their clinical manifestations, diagnosis, and treatment. Essential historic elements that distinguish these events and the role of video-EEG-polysomnography in their differential diagnosis are emphasized.