Katsumi Kurokawa
Department Kawasaki Medical School Kawasaki Medical School, Department of General Internal Medicine 1, Position Professor |
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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. |