DECREMENTAL MOTOR-RESPONSES TO REPETITIVE NERVE-STIMULATION IN ALS

DECREMENTAL MOTOR-RESPONSES TO REPETITIVE NERVE-STIMULATION IN ALS
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DOI:
10.1002/mus.880170708
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发表时间:
1994-07-01
期刊:
影响因子:
3.4
通讯作者:
BOLAND, D
BOLAND, D
中科院分区:
医学3区
文献类型:
--
作者:
KILLIAN, JM;WILFONG, AA;BOLAND, D

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对192例肌萎缩侧索硬化症(ALS)患者进行了缓慢重复神经刺激(RNS)。五十六例患者(29%)表现出典型的神经肌肉衰减10-43%(平均16.8%),而44例患者(23%)表现出临界衰减5- 9%。斜方肌在显示缺陷方面比远端小鱼际肌明显更敏感。在连续随访的30例患者中,递减反应保持不变或随时间增加。然而,25%的患者尽管疾病进展,但仍然没有表现出降低。减量与临床严重程度、疾病分期或疾病进展之间无统计学相关性。发现至少50%的ALS患者显示出一定程度的RNS递减的肌肉提示,神经肌肉接头的功能改变伴随着这种疾病。(C)1994年,John Wiley and Sons,Inc.
Repetitive nerve stimulation (RNS) of the trapezius muscle at slow rates was performed on 192 patients with amyotrophic lateral sclerosis (ALS). Fifty-six patients (29%) showed classical neuromuscular decrement of 10-43% (mean 16.8%) while 44 patients (23%) had a borderline decrement of 5-9%. The trapezius was significantly more sensitive in revealing the defect than the distal hypothenar muscles. In 30 patients followed serially, the decremental response remained constant or increased with time. However, 25% of patients continued to show no decrement in spite of progression of disease. No statistical correlation was found between decrement and clinical severity, disease staging, or disease progression. The finding that at least 50% of ALS patients show some degree of decrement on RNS of the trapezius muscle suggests that functional alterations of the neuromuscular junction accompany this disease. (C) 1994 John Wiley and Sons, Inc.