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[一例与脱髓鞘性周围神经病相关的急性播散性脑脊髓炎(ADEM)]

[A case of acute disseminated encephalomyelitis (ADEM) associated with demyelinating peripheral neuropathy].

作者信息

Aimoto Y, Moriwaka F, Matsumoto A, Tashiro K, Abe K

机构信息

Department of Neurology, Hokkaido University School of Medicine, Sapporo, Japan.

出版信息

No To Shinkei. 1996 Sep;48(9):857-60.

PMID:8888036
Abstract

A 41-year-old man had common cold on April 10, 1991. Five days after this illness he developed fever, dysarthria, visual field impairment, gait disturbance and consciousness disturbance followed. On admission in another hospital, cerebrospinal fluid showed 341/mm3 cells (303 mononuclear cells, 33 polynuclear cells, 5 red cells), protein of 238 mg/dl, and sugar of 59 mg/dl. One month later, the neurological examinations revealed flaccid paraparesis, decreased deep tendon reflexes in the lower extremities, bilateral positive Babinski and Chaddock reflexes, positive Beevor sign, stocking type superficial sensory disturbance, diminished vibration sense in the lower extremities and neurogenic bladder. Eight months later, he improved to be able to walk by himself, but decreased Achilles tendon reflexes, bilateral positive Babinski and Chaddock reflexes and paresthesia over both feet were noted neurologically. Nerve conduction study revealed reduced conduction velocities at tibialis anterior nerves, sural nerves and no response on both deep peroneal nerves. A 1 micron thick epon section of a biopsied sural nerve with toluidine blue stain showed a decreased number of myelinated fibers (6394/mm2) with many thinly myelinated fibers and Renaut bodies. There was no edema nor cell infiltration. Electronmicroscopical findings of their ultrathin sections showed many collagen pockets, denervated Schwann cell clustering and a few onion bulb formations. Teased fiber preparations suggested segmental demyelination and remyelination in many fibers. This case could be regarded as a case of ADEM associated with demyelinating peripheral neuropathy, and the possibility of the simultaneous demyelinating process in the central and peripheral nervous system was discussed.

摘要

一名41岁男性于1991年4月10日患普通感冒。患病5天后,他出现发热、构音障碍、视野缺损、步态障碍,随后出现意识障碍。在另一家医院住院时,脑脊液显示细胞数为341/mm³(303个单核细胞、33个多核细胞、5个红细胞),蛋白为238mg/dl,糖为59mg/dl。1个月后,神经系统检查发现弛缓性双下肢轻瘫、下肢深腱反射减弱、双侧巴宾斯基征和查多克征阳性、比弗征阳性、袜套型浅感觉障碍、下肢振动觉减退以及神经源性膀胱。8个月后,他有所好转,能够自行行走,但神经系统检查发现跟腱反射减弱、双侧巴宾斯基征和查多克征阳性以及双足感觉异常。神经传导研究显示胫前神经、腓肠神经传导速度降低,双侧腓深神经无反应。对活检的腓肠神经进行1微米厚的环氧树脂切片,甲苯胺蓝染色显示有髓纤维数量减少(6394/mm²),有许多薄髓纤维和雷诺体。没有水肿和细胞浸润。超薄切片的电子显微镜检查结果显示有许多胶原小窝、失神经施万细胞聚集和一些洋葱球形成。 teased纤维制备显示许多纤维存在节段性脱髓鞘和再髓鞘化。该病例可被视为与脱髓鞘性周围神经病相关的急性播散性脑脊髓炎病例,并讨论了中枢和周围神经系统同时发生脱髓鞘过程的可能性。

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