A clinical neurophysiology study of Hirayama disease / 中华医学杂志(英文版)
Chinese Medical Journal
;
(24): 1115-1120, 2012.
Artigo
em Inglês
| WPRIM
| ID: wpr-269290
ABSTRACT
<p><b>BACKGROUND</b>Hirayama disease is a rare disease characterized by juvenile-onset of asymmetric amyotrophy, of which etiology has not been clarified. The aim of our study was to investigate the clinical and neurophysiologic characteristics of Hirayama disease.</p><p><b>METHODS</b>Neurophysiological tests, including nerve conduction studies (NCS), F-wave and routine electromyography (EMG), were performed in seventy-three patients with Hirayama disease. EMG was selectively performed on upper and lower extremities, sternocleidomast and thoracic paravertebral muscles according to the clinical features of the patients.</p><p><b>RESULTS</b>Abnormal NCS parameters, including decreased compound muscle action potentials or delayed distal motor latency, were found in 34.2% (25/73) and 12.3% (9/73) of the patients, respectively. A total of 24.6% (18/73) of the patients showed decreased F-wave frequency. EMG demonstrated the presence of neurogenic lesions in all patients with spontaneous potentials, prolonged duration or augmentation of amplitude in motor unit potentials (MUPs), or a single pattern of MUP recruitment. About 17.8% (13/73) of the patients showed neurogenic lesions, mostly in the C7-8 level of the cervical cord, only in the upper extremity of affected side, whereas 35.6% (26/73) of the patients possessed lesions in the upper extremities bilaterally. A total of 46.6% (34/73) of patients exhibited abnormalities in the lower extremities, sterno- cleidomast or thoracic paravertebral muscle. Changes in motor NCS were significantly correlated with muscle strength.</p><p><b>CONCLUSIONS</b>EMG detects diffused subclinical neurogenic lesion in a high proportion of patients with Hirayama disease. Results of our study challenge the hypothesis that Hirayama disease is a type of cervical myelopathy.</p>
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Patologia
/
Imageamento por Ressonância Magnética
/
Atrofias Musculares Espinais da Infância
/
Eletromiografia
/
Condução Nervosa
Limite:
Adolescente
/
Adulto
/
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Chinese Medical Journal
Ano de publicação:
2012
Tipo de documento:
Artigo
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