頁籤選單縮合
題 名 | Peripheral Neuropathy in Myotonic Dystrophy |
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作 者 | Tsai, Yu-tai; Huang, Chin-chang; Chu, Chun-che; Kuo, Hung-chou; | 書刊名 | Acta Neurologica Taiwanica |
卷 期 | 9:4 民89.12 |
頁 次 | 頁282-289 |
分類號 | 415.9413 |
關鍵詞 | 強直型肌肉萎縮症; 周邊神經病變; Myotonic dystrophy; Peripheral neuropathy; Electrophysiological study; Sural nerve biopsy; |
語 文 | 英文(English) |
英文摘要 | We retrospectively studied 18 patients (12 men, 6 women) from 15 unrelated families with myotonic dystrophy (MD) in our hospital from 1983 through 1999. The mean age was 40 ± 11 years with a range of 26 to 63 years. The mean duration of MD was 9.2 ± 6.0 years and ranged from 1 to 23 years. Most patients had prominent muscle weakness and decreased or absent tendon reflexes, but no sensory impairments. Using the standard electrophysiological studies, polyneuropathy with sensory and motor involvement were found in eight of 18 patients (44%). As a group in motor nerve conduction study (NCS), the distal latency (DL) was prolonged in the median and ulnar nerves and the nerve conduction velocity (NCV) was slowed in all nerves studied compared with the healthy control subjects. In sensory NCS, the DL was prolonged in the ulnar nerve and the NCV was slowed in the median and ulnar nerves as compared with the healthy control subjects. In one patient, sural nerve biopsy studies showed a decrease of nerve fiber density with predominant loss of large myelinated fibers. The electromicroscopic findings confirmed axonal damage and disruption of the myelin. The data indicate that subclinical polyneuropathy in MD is common and may represent one of the manifestations of the multisystem involvement. |
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