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Muscular fatigue and decremental response to repetitive nerve stimulation in X‐linked spinobulbar muscular atrophy
Author(s) -
Inoue K.,
Hemmi S.,
Miyaishi M.,
Kutoku Y.,
Murakami T.,
Kurokawa K.,
Sunada Y.
Publication year - 2009
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2008.02349.x
Subject(s) - medicine , compound muscle action potential , repetitive nerve stimulation , atrophy , spinal and bulbar muscular atrophy , stimulation , hand muscles , wrist , muscle contracture , anatomy , electromyography , electrophysiology , physical medicine and rehabilitation , androgen receptor , prostate cancer , cancer
Background and Purpose: We report decremental responses to repetitive nerve stimulation (RNS) in 11 patients diagnosed with X‐linked spinobulbar muscular atrophy (X‐SBMA). Methods: The compound muscle action potential (CMAP) of the right abductor digiti minimi (ADM) and trapezius (TZ) in response to a 3‐Hz stimulation of the ulnar nerve at the wrist and accessory nerve at the neck were recorded by surface electrodes. Results: A decremental response to RNS was observed in 90.9% of the TZ muscle and 27.2% in the ADM muscle of patients with X‐SBMA. Conclusion: These electrophysiological features of X‐SBMA are considered to be useful for diagnosis of X‐SBMA. Furthermore, the waning phenomena that mostly appeared in the TZ muscle and increment of CMAP in RNS after the exercise also suggest a unique manifestation in X‐SBMA.