We report a 50-year-old woman who developed localized proximal muscle weakness, in addition to transient elevation of antibodies to GM-1 ganglioside, without multifocal conduction block. She was treated with intraveno...We report a 50-year-old woman who developed localized proximal muscle weakness, in addition to transient elevation of antibodies to GM-1 ganglioside, without multifocal conduction block. She was treated with intravenous immunoglobulin (IVIg) and steroid pulse therapy, which were effective for over 10 years. Her clinical course and laboratory tests were consistent with lower motor neuron syndrome (LMNS) with localized proximal muscle weakness. We suggest that some patients diagnosed as LMNS may remain responsive to IVIg or steroid pulse therapy for a long time.展开更多
Background： Motor dysfunction is common in stroke patients. Clinical electrophysiological studies suggest that transsynaptic degeneration occurred in the lower motor neurons, while pathological evidence is lacked. Th...Background： Motor dysfunction is common in stroke patients. Clinical electrophysiological studies suggest that transsynaptic degeneration occurred in the lower motor neurons, while pathological evidence is lacked. This study aimed to combine the electrophysiological and pathological results to prove the existence of transsynaptic degeneration in the motor system after stroke. Methods： Modified neurologic severity score, electrophysiological, and pathological assessments were evaluated in rats before middle cerebral artery occlusion （MCAO）, and at 24 hours, 7 days, and 14 days after MCAO. Paired and independent-sample t-tests were applied to assess the changes of electrophysiological and pathological data. Results： Compound motor action potential amplitude in the paretic side was significantly lower than the nonparetic side at both 24 hours （61.9 ± 10.4 vs. 66.6 ± 8.9, P 〈 0.05） and 7 days （60.9 ± 8.4 vs. 67.3 ±9.6, P 〈 0.05） after MCAO. Motor unit number estimation of the paretic side was significantly less than the nonparetic side （379.0 ± 84.6 vs. 445.0 ±9.5, P 〈 0.05） at 7 days after MCAO. Until 14 days after stroke, the pathological loss of motor neurons was detected. Motor neurons in 14-day MCAO group were significantly decreased, compared with control group （5.3 ± 0.7 vs. 7.3 ± 1.8, P 〈 0.05）. Conclusions： Both electrophysiological and pathological studies showed transsynaptic degeneration after stroke. This study identified the asynchronization in changes of electrophysiology and pathology. The abnormal physiological changes and function impairment can be detected in the early stage and recovered quickly, while the pathological loss of motor neuron can be detected only in a later stage.展开更多
下运动神经元(lower motor neuron,LMN)综合征是一类定位于脊髓前角细胞及运动神经纤维,表现为肌肉萎缩、无力且无感觉受累的临床综合征。根据发病机制的不同,LMN综合征可分为变性疾病、遗传相关性疾病及免疫介导性疾病。常见的LMN综合...下运动神经元(lower motor neuron,LMN)综合征是一类定位于脊髓前角细胞及运动神经纤维,表现为肌肉萎缩、无力且无感觉受累的临床综合征。根据发病机制的不同,LMN综合征可分为变性疾病、遗传相关性疾病及免疫介导性疾病。常见的LMN综合征包括运动神经元病、脊髓肌萎缩症、多灶性运动神经病等。由于这些疾病的症状多变且诊断标志物相对匮乏,因此临床中的误诊误治非常常见。其中,对免疫介导性LMN综合征的早期诊断十分重要,因为这一类疾病同其他的LMN综合征在治疗及预后方面截然不同。虽然,目前对于这些疾病的认识已逐渐深入,但LMN综合征的诊断及治疗仍是目前临床工作的挑战之一,仍需要更多的研究为临床提供参考。展开更多
文摘We report a 50-year-old woman who developed localized proximal muscle weakness, in addition to transient elevation of antibodies to GM-1 ganglioside, without multifocal conduction block. She was treated with intravenous immunoglobulin (IVIg) and steroid pulse therapy, which were effective for over 10 years. Her clinical course and laboratory tests were consistent with lower motor neuron syndrome (LMNS) with localized proximal muscle weakness. We suggest that some patients diagnosed as LMNS may remain responsive to IVIg or steroid pulse therapy for a long time.
基金This study wasfunded by a grant of National Natural Science Foundation of China （No. 81241045）.
文摘Background： Motor dysfunction is common in stroke patients. Clinical electrophysiological studies suggest that transsynaptic degeneration occurred in the lower motor neurons, while pathological evidence is lacked. This study aimed to combine the electrophysiological and pathological results to prove the existence of transsynaptic degeneration in the motor system after stroke. Methods： Modified neurologic severity score, electrophysiological, and pathological assessments were evaluated in rats before middle cerebral artery occlusion （MCAO）, and at 24 hours, 7 days, and 14 days after MCAO. Paired and independent-sample t-tests were applied to assess the changes of electrophysiological and pathological data. Results： Compound motor action potential amplitude in the paretic side was significantly lower than the nonparetic side at both 24 hours （61.9 ± 10.4 vs. 66.6 ± 8.9, P 〈 0.05） and 7 days （60.9 ± 8.4 vs. 67.3 ±9.6, P 〈 0.05） after MCAO. Motor unit number estimation of the paretic side was significantly less than the nonparetic side （379.0 ± 84.6 vs. 445.0 ±9.5, P 〈 0.05） at 7 days after MCAO. Until 14 days after stroke, the pathological loss of motor neurons was detected. Motor neurons in 14-day MCAO group were significantly decreased, compared with control group （5.3 ± 0.7 vs. 7.3 ± 1.8, P 〈 0.05）. Conclusions： Both electrophysiological and pathological studies showed transsynaptic degeneration after stroke. This study identified the asynchronization in changes of electrophysiology and pathology. The abnormal physiological changes and function impairment can be detected in the early stage and recovered quickly, while the pathological loss of motor neuron can be detected only in a later stage.
文摘下运动神经元(lower motor neuron,LMN)综合征是一类定位于脊髓前角细胞及运动神经纤维,表现为肌肉萎缩、无力且无感觉受累的临床综合征。根据发病机制的不同,LMN综合征可分为变性疾病、遗传相关性疾病及免疫介导性疾病。常见的LMN综合征包括运动神经元病、脊髓肌萎缩症、多灶性运动神经病等。由于这些疾病的症状多变且诊断标志物相对匮乏,因此临床中的误诊误治非常常见。其中,对免疫介导性LMN综合征的早期诊断十分重要,因为这一类疾病同其他的LMN综合征在治疗及预后方面截然不同。虽然,目前对于这些疾病的认识已逐渐深入,但LMN综合征的诊断及治疗仍是目前临床工作的挑战之一,仍需要更多的研究为临床提供参考。
