Katsumi Kurokawa    Department   Kawasaki Medical School  Kawasaki Medical School, Department of General Internal Medicine 1,    Position   Professor
Article types	原著
Language	English
Peer review	Peer reviewed
Title	Muscular fatigue and decremental response to repetitive nerve stimulation in X-linked spinobulbar muscular atrophy
Journal	Formal name：European journal of neurology : the official journal of the European Federation of Neurological Societies Abbreviation：Eur J Neurol ISSN code：13515101/14681331
Volume, Issue, Page	16,pp.76-80
Author and coauthor	Inoue Ken, Hemmi Shoji, Miyashita Masahiro, Kutoku Yumiko, Murakami Tatsufumi, Kurokawa Katsumi, Sunada Yoshihide
Publication date	2009
Summary	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 ADM muscle of patients with X-SBMA. Conclusion: These electrophysiological features of X-SBMA are considered to be usuful 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 menifestation in X-SBMA.