Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology
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Transthyretin familial amyloid polyneuropathy (TTR-FAP) is an axonal sensory-motor and autonomic neuropathy. Reliable quantification of sudomotor function could prove essential in the diagnosis and early treatment management. We aim to assess the diagnostic value of a new sudomotor test (Sudoscan) in TTR-FAP. ⋯ Abnormal feet responses on Sudoscan support early diagnosis in TTR-FAP.
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In amyotrophic lateral sclerosis (ALS), fasciculations are believed to become less frequent during disease progression, associated with the loss of motor units. To address this issue, we studied the variation of fasciculation potential (FPs) frequency as evaluated by surface electromyography of the first dorsal interosseous muscle (1st DI) in patients with ALS and other related disorders, and to relate this change with the neurophysiological index (NI), a surrogate measure of functional motor units. ⋯ In ALS, the FP firing frequency does not decline significantly over several months, in spite of a marked reduction in the number of functional motor units.