Ejima M, Ota K, Yamamoto K, Sugishita Y, Maruyama S
Department of Neurology, Tokyo Women's Medical College.
Rinsho Shinkeigaku. 1994 Nov;34(11):1136-41.
A 37-year-old woman with complaints of headache and nausea presented with temporary disturbance of consciousness, bulbar palsy and ataxic speech following flu-like symptoms. After the recovery of her consciousness, she developed orthostatic syncope and numbness all over the body. When she was admitted to our hospital two months later, she showed emaciation, diminished sweat production and butterfly-patch-like pigmentation. Neurologic examinations were remarkable for anisocoric pupils that sluggishly reacted to light, impaired left facial movements, bulbar palsy, numbness of the whole body, total loss of all tendon reflexes, incordination, ataxic gait and severe postural hypotension. Laboratory data included albuminocytogenic dissociation in cerebrospinal fluid, convergence nystagmus and dysmetria in electronystagmography, and right trigeminal paralysis in blink reflex. A sural nerve biopsy showed active axonal degeneration and severe loss of both myelinated and unmyelinated fibers. Examinations of autonomic nervous system disclosed diffuse impairment of sympathetic and parasympathetic postganglionic nerve. Based on these findings she was diagnosed as having acute pandysautonomia. High titer of serum EB virus antibody suggested that acute pandysautonomia and diffuse brainstem impairment may be related to EB virus infection.
一名37岁女性，有头痛和恶心症状，在出现类似流感症状后出现意识暂时障碍、延髓麻痹和共济失调性言语。意识恢复后，她出现体位性晕厥和全身麻木。两个月后入院时，她消瘦、出汗减少且有蝶形斑样色素沉着。神经系统检查显示显著异常，瞳孔不等大，对光反应迟钝，左侧面部运动障碍，延髓麻痹，全身麻木，所有腱反射完全消失，共济失调，共济失调步态以及严重的体位性低血压。实验室检查结果包括脑脊液蛋白细胞分离、眼震电图检查显示的集合性眼球震颤和辨距不良，以及瞬目反射检查显示的右侧三叉神经麻痹。腓肠神经活检显示轴突活动性变性以及有髓和无髓纤维严重丧失。自主神经系统检查揭示交感和副交感神经节后神经弥漫性损害。基于这些发现，她被诊断为急性全自主神经功能不全。高滴度血清EB病毒抗体提示急性全自主神经功能不全和弥漫性脑干损害可能与EB病毒感染有关。
