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A clinical neurophysiology study of hirayama disease

Chinese Medical Journal 2012年第6期125卷 1115-1120页

作者： WANG Xin-ning CUI Li-ying LIU Ming-sheng GUAN Yu-zhou LI Ben-hong DU Hua Department of Neurology Peking Union Medical College HospitalChinese Academy of Medical Science and Peking Union Medical CollegeBeijing 100730China

Background 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. Methods 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. Results 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. Conclusions 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.

关键词： hirayama disease nerve conduction study electromyography F-wave

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Pattern Differences of Small Hand Muscle Atrophy in Amyotrophic Lateral Sclerosis and Mimic Disorders

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Chinese Medical Journal 2016年第7期129卷 792-798页

作者： Jia Fang Ming-Sheng Liu Yu-Zhou Guan Hua Du Ben-Hong Li Bo Cui Qing-Yun Ding Li-Ying Cui Department of Neurology Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing 100730 China

Background： Amyotrophic lateral sclerosis （ALS） and some mimic disorders, such as distal-type cervical spondylotic amyotrophy （LISA）, hirayama disease （HD）, and spinobulbar muscular atrophy （SBMA） may present with intrinsic hand muscle atrophy. This study aimed to investigate different patterns of small hand muscle involvement in ALS and some mimic disorders. Methods： We compared the abductor digiti minimi/abductor pollicis brevis （ADM/APB） compound muscle action potential （（＇MAP） ratios between 200 ALS patients, 95 patients with distal-type CSA, 88 HD patients, 43 SBMA patients, and 150 normal controls. Results： The ADM/APB （？MAP amplitude ratio was significantly higher in the ALS patients （P 〈 0.001） than that in the normal controls. The ADM/APB CMAP amplitude ratio was significantly reduced in the patients with distal-type CSA （P 〈 0,001 ） and the H I） patients （P 〈 0.001 ） compared with that in the normal controls. The patients with distal-type CSA had significantly lower APB CMAP amplitude than the HD patients （P- 0.004）. The ADM/APB CMAP amplitude ratio was significantly lower in the HD patients （P 〈 0.001 ） than that in the patients with distal-type CSA. The ADM/APB CMAP amplitude ratio of the SBMA patients was similar to that of the normal controls （P = 0.862）. An absent APB CMAP and an abnormally high ADM/APB CMAP amplitude ratio （_〉4.5） were observed exclusively in the ALS patients. Conclusions： The different patterns of small hand muscle atrophy between the ALS patients and the patients with mimic disorders presumably reflect distinct pathophysiological mechanisms underlying different disorders, and may aid in distinguishing between ALS and mimic disorders.

关键词： Amyotrophic Lateral Sclerosis Cervical Spondylotic Amyotrophy hirayama disease Spinobulbar Muscular Atrophy Split Hand

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