Background： Nowadays, it is widely known that decremental responses in low-frequency repetitive nerve stimulation （LF-RNS） are frequently observed in patients with amyotrophic lateral sclerosis （ALS）. The patholo...Background： Nowadays, it is widely known that decremental responses in low-frequency repetitive nerve stimulation （LF-RNS） are frequently observed in patients with amyotrophic lateral sclerosis （ALS）. The pathological mechanism of this phenomenon remains unknown. This study aimed to illuminate the features of RNS in Chinese patients with ALS. Methods： Clinical and electrophysiological data of 146 probable and definite ALS patients who underwent RNS were retrospectively enrolled and analyzed. LF-RNS （3 Hz） was performed in trapezius, deltoid, abductor digiti minimi （ADM）, quadriceps femoris, and tibialis anterior. High-frequency RNS （HF-RNS, 10 Hz） was performed only in ADM. The two-sample t-test and Chi-squared test were used for statistical analysis.Results： Decremental responses to LF-RNS （≥ 10%） in at least one muscle were detected in 83 （56.8%） of the cases and were most commonly seen in trapezius and deltoid. The incidence ofdecremental response was higher in patients with upper limb onset. Incremental responses to HF-RNS （≥60%） in ADM were observed in 6 （5.6%） of the cases. In 106 muscles with decremental response, 62 （57.4%） muscles had a continuous decremental pattern, more than a U-shape pattern （37 cases, 34.3%）. Nineteen cases showed definite decrements in LF-RNS tests in trapezius, while no abnormalities were found in the electromyography and neurological examination of the sternocleidomastoid muscle, supplied by the accessory nerve as well.Conclusions： Decremental responses in the RNS are commonly observed in ALS patients. The findings regarding the trapezius indicated that some ALS onsets could be initiated by a ＂dying back＂ process, with destruction of neuromuscular junctions （NMJs） before motor neurons. Incremental responses in the ADM implied damage of the NMJs involved both the post and presynaptic membranes.展开更多
基金This study was supported by grants from the National Natural Science Foundation of China （No. 81671278 and No. 81601096）, One Hundred Advantage Projects ＂Fund of Chinese PLA General Hospital＂ （No. YS201415）, and Key Research and Development Plan of Hainan Province （No. ZDFY2016120）.
文摘Background： Nowadays, it is widely known that decremental responses in low-frequency repetitive nerve stimulation （LF-RNS） are frequently observed in patients with amyotrophic lateral sclerosis （ALS）. The pathological mechanism of this phenomenon remains unknown. This study aimed to illuminate the features of RNS in Chinese patients with ALS. Methods： Clinical and electrophysiological data of 146 probable and definite ALS patients who underwent RNS were retrospectively enrolled and analyzed. LF-RNS （3 Hz） was performed in trapezius, deltoid, abductor digiti minimi （ADM）, quadriceps femoris, and tibialis anterior. High-frequency RNS （HF-RNS, 10 Hz） was performed only in ADM. The two-sample t-test and Chi-squared test were used for statistical analysis.Results： Decremental responses to LF-RNS （≥ 10%） in at least one muscle were detected in 83 （56.8%） of the cases and were most commonly seen in trapezius and deltoid. The incidence ofdecremental response was higher in patients with upper limb onset. Incremental responses to HF-RNS （≥60%） in ADM were observed in 6 （5.6%） of the cases. In 106 muscles with decremental response, 62 （57.4%） muscles had a continuous decremental pattern, more than a U-shape pattern （37 cases, 34.3%）. Nineteen cases showed definite decrements in LF-RNS tests in trapezius, while no abnormalities were found in the electromyography and neurological examination of the sternocleidomastoid muscle, supplied by the accessory nerve as well.Conclusions： Decremental responses in the RNS are commonly observed in ALS patients. The findings regarding the trapezius indicated that some ALS onsets could be initiated by a ＂dying back＂ process, with destruction of neuromuscular junctions （NMJs） before motor neurons. Incremental responses in the ADM implied damage of the NMJs involved both the post and presynaptic membranes.
