發(fā)布時間：2018-08-07 06:53

【摘要】：面部起病的感覺運動神經(jīng)元病(facial onset sensory and motor neuronopathy,FOSMN)是近來新發(fā)現(xiàn)的一類罕見的、緩慢進展的神經(jīng)疾病。其主要特征為以三叉神經(jīng)分布區(qū)的感覺障礙為首發(fā)癥狀,可逐漸發(fā)展至頭皮、頸部、軀干上部及上肢,運動癥狀出現(xiàn)于感覺癥狀之后,如延髓麻痹、肌無力、肌萎縮、肌束顫動等下運動神經(jīng)元損害癥狀,少數(shù)出現(xiàn)上運動神經(jīng)元損害體征。所有FOSMN綜合征患者角膜反射或瞬目反射減弱或消失。神經(jīng)傳導功能檢查示上肢感覺神經(jīng)動作電位波幅降低。所有患者均出現(xiàn)瞬目反射的異常,表現(xiàn)為R2潛伏期延長或未引出。本綜述從流行病學、臨床表現(xiàn)、病理、輔助檢查、診斷、鑒別診斷、治療等方面詳細對此病進行闡述,并對此病的發(fā)病機制及其與肌萎縮側索硬化(amyotrophic lateral sclerosis,ALS)的關聯(lián)進行討論,從而加深對此種罕見病的認識。
[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.
【學位授予單位】：河北醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2017
【分類號】：R744.8

【參考文獻】

相關期刊論文 前7條

1 楊驪鵬;劉曉魯;孫慶利;樊東升;;伴自主神經(jīng)功能障礙的面部起病的感覺運動神經(jīng)元病1例報告[J];北京醫(yī)學;2016年05期

2 唐一麟;朱雯華;喬凱;程忻;趙重波;陳[?;王亮;盧家紅;;面部起病的感覺運動神經(jīng)元病一例[J];中華神經(jīng)科雜志;2013年10期

3 張華綱;唐璐;張楠;樊東升;;中國家族性肌萎縮側索硬化患者超氧化物歧化酶1基因突變與臨床表型[J];中華神經(jīng)科雜志;2012年07期

4 崔麗英;蒲傳強;樊東升;劉明生;;中國肌萎縮側索硬化診斷和治療指南[J];中華神經(jīng)科雜志;2012年07期

5 徐潔蓮;;慢性炎性脫髓鞘性多發(fā)性神經(jīng)病的診斷和治療[J];中外醫(yī)學研究;2011年28期

6 王心寧;崔麗英;;超氧化物歧化酶-1毒性作用在肌萎縮側索硬化發(fā)病機制中的研究進展[J];中華醫(yī)學雜志;2011年27期

7 劉明生;;糖尿病周圍神經(jīng)病診斷和治療共識[J];中華神經(jīng)科雜志;2013年11期

，

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