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Muscular fatigue and decremental response to repetitive nerve stimulation in X-linked spinobulbar muscular atrophy. 2009

K Inoue, and S Hemmi, and M Miyaishi, and Y Kutoku, and T Murakami, and K Kurokawa, and Y Sunada
Department of Neurology, Kawasaki Medical School, Okayama, Japan. kenin_04@yahoo.co.jp

OBJECTIVE 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. CONCLUSIONS 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.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D004558 Electric Stimulation Use of electric potential or currents to elicit biological responses. Stimulation, Electric,Electrical Stimulation,Electric Stimulations,Electrical Stimulations,Stimulation, Electrical,Stimulations, Electric,Stimulations, Electrical
D004568 Electrodiagnosis Diagnosis of disease states by recording the spontaneous electrical activity of tissues or organs or by the response to stimulation of electrically excitable tissue. Electrodiagnoses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D000369 Aged, 80 and over Persons 80 years of age and older. Oldest Old
D055534 Bulbo-Spinal Atrophy, X-Linked An X-linked recessive form of spinal muscular atrophy. It is due to a mutation of the gene encoding the ANDROGEN RECEPTOR. Atrophy, Muscular, Spinobulbar,Kennedy Syndrome,Muscular Atrophy, Spinobulbar,Spinobulbar Muscular Atrophy,Bulbospinal Muscular Atrophy, X-linked,Kennedy Disease,Kennedy Spinal and Bulbar Muscular Atrophy,Kennedy's Disease,Spinal And Bulbar Muscular Atrophy, X-Linked 1,Spinal and Bulbar Muscular Atrophy,X-Linked Bulbo-Spinal Atrophy,X-Linked Spinal and Bulbar Muscular Atrophy,X-linked Bulbospinal Muscular Atrophy,Atrophies, X-Linked Bulbo-Spinal,Atrophy, Spinobulbar Muscular,Atrophy, X-Linked Bulbo-Spinal,Bulbo Spinal Atrophy, X Linked,Bulbo-Spinal Atrophies, X-Linked,Bulbospinal Muscular Atrophy, X linked,Spinal And Bulbar Muscular Atrophy, X Linked 1,Spinobulbar Muscular Atrophies,X Linked Bulbo Spinal Atrophy,X Linked Spinal and Bulbar Muscular Atrophy,X linked Bulbospinal Muscular Atrophy,X-Linked Bulbo-Spinal Atrophies
D018763 Muscle Fatigue A state arrived at through prolonged and strong contraction of a muscle. Studies in athletes during prolonged submaximal exercise have shown that muscle fatigue increases in almost direct proportion to the rate of muscle glycogen depletion. Muscle fatigue in short-term maximal exercise is associated with oxygen lack and an increased level of blood and muscle lactic acid, and an accompanying increase in hydrogen-ion concentration in the exercised muscle. Fatigue, Muscle,Muscular Fatigue,Fatigue, Muscular

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