Journal of the Chinese Medical Association : JCMA · 2026
Clinical and electrophysiological features for differentiating MMN from hand-onset ALS.
Сравнительное исследованиеСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
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Fang Shih-Yu, Jih Kang-Yang, Chao Yu-Chi, Sytwu Hou-Ping, Shih Yen-Cheng, Liao Yi-Chu, Lee Yi-Chung
Аннотация
BACKGROUND: Multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) can be difficult to differentiate, particularly at early disease stages for patients with hand-onset weakness and without upper motor neuron (UMN) signs. This study aimed to identify clinical and electrophysiological features that may facilitate early differentiation between MMN and ALS.
METHODS: We retrospectively analyzed the clinical, laboratory, and electrophysiological characteristics of patients diagnosed with MMN and ALS who underwent an identical nerve conduction study protocol comprising extended motor stimulation.
RESULTS: A total of 125 patients (74 men and 51 women) were included, consisting of eight patients with MMN and 117 patients with ALS, including 42 with hand-onset ALS. The patients with MMN had a significantly younger mean age at symptom onset than those with ALS (43.1 vs 58.7 years, p = 0.004). The patients with ALS had greater muscle weakness, more frequent muscle atrophy and fasciculation, UMN signs, and body weight loss. Compared with both the overall ALS and hand-onset ALS groups, the MMN group had significantly lower serum creatine kinase (CK) levels and higher serum IgM levels. Elevated CK levels were observed in approximately one-third of patients with hand-onset ALS, whereas none of the MMN patients had elevated CK levels. Conduction blocks (CB) on nerve conduction studies were more common in the MMN group (87.5%) than in the overall ALS (19.7%, p < 0.001) and hand-onset ALS groups (31.0%, p = 0.005). MMN patients more frequently exhibited definite CBs involving multiple nerves (85.7%) compared with the overall ALS (17.4%, p = 0.002) and hand-onset ALS groups (7.7%, p = 0.001).
CONCLUSION: Our findings suggest that a combination of clinical features, serum CK and IgM levels, and electrophysiological evidence of CB provides valuable clues for distinguishing MMN from ALS.
METHODS: We retrospectively analyzed the clinical, laboratory, and electrophysiological characteristics of patients diagnosed with MMN and ALS who underwent an identical nerve conduction study protocol comprising extended motor stimulation.
RESULTS: A total of 125 patients (74 men and 51 women) were included, consisting of eight patients with MMN and 117 patients with ALS, including 42 with hand-onset ALS. The patients with MMN had a significantly younger mean age at symptom onset than those with ALS (43.1 vs 58.7 years, p = 0.004). The patients with ALS had greater muscle weakness, more frequent muscle atrophy and fasciculation, UMN signs, and body weight loss. Compared with both the overall ALS and hand-onset ALS groups, the MMN group had significantly lower serum creatine kinase (CK) levels and higher serum IgM levels. Elevated CK levels were observed in approximately one-third of patients with hand-onset ALS, whereas none of the MMN patients had elevated CK levels. Conduction blocks (CB) on nerve conduction studies were more common in the MMN group (87.5%) than in the overall ALS (19.7%, p < 0.001) and hand-onset ALS groups (31.0%, p = 0.005). MMN patients more frequently exhibited definite CBs involving multiple nerves (85.7%) compared with the overall ALS (17.4%, p = 0.002) and hand-onset ALS groups (7.7%, p = 0.001).
CONCLUSION: Our findings suggest that a combination of clinical features, serum CK and IgM levels, and electrophysiological evidence of CB provides valuable clues for distinguishing MMN from ALS.