面部起病的感觉运动神经元病及与肌萎缩侧索硬化相关性

发布时间：2018-08-07 06:53

【摘要】：面部起病的感觉运动神经元病(facial onset sensory and motor neuronopathy,FOSMN)是近来新发现的一类罕见的、缓慢进展的神经疾病。其主要特征为以三叉神经分布区的感觉障碍为首发症状,可逐渐发展至头皮、颈部、躯干上部及上肢,运动症状出现于感觉症状之后,如延髓麻痹、肌无力、肌萎缩、肌束颤动等下运动神经元损害症状,少数出现上运动神经元损害体征。所有FOSMN综合征患者角膜反射或瞬目反射减弱或消失。神经传导功能检查示上肢感觉神经动作电位波幅降低。所有患者均出现瞬目反射的异常,表现为R2潜伏期延长或未引出。本综述从流行病学、临床表现、病理、辅助检查、诊断、鉴别诊断、治疗等方面详细对此病进行阐述,并对此病的发病机制及其与肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)的关联进行讨论,从而加深对此种罕见病的认识。
[Abstract]:Sensorimotor neuron disease (facial onset sensory and motor neuronopathy) is a rare and slowly progressive neuropathy. Its main characteristic is that the first symptom is the sensory disorder in the distribution area of trigeminal nerve, which can gradually develop to scalp, neck, upper trunk and upper limb. The motor symptoms appear after the sensory symptoms, such as bulbar palsy, myasthenia, muscle atrophy. Muscle bundle fibrillation and other motor neuron damage symptoms, a few signs of upper motor neuron damage. Corneal reflex or blink reflex was attenuated or disappeared in all patients with FOSMN syndrome. Nerve conduction function test showed that the amplitude of sensory nerve action potential decreased in upper limb. Blink reflexes were found in all patients with prolonged R2 latency or no elicitation. In this review, the epidemiology, clinical manifestations, pathology, adjuvant examination, diagnosis, differential diagnosis and treatment were reviewed in detail. The pathogenesis of the disease and its association with amyotrophic lateral sclerosis (amyotrophic lateral) were also discussed. So as to deepen the understanding of this rare disease.
【学位授予单位】：河北医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R744.8

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