Senda Y, Sugimura K, Koike Y, Matsuoka Y, Takahashi A
Rinsho Shinkeigaku. 1989 Mar;29(3):332-5.
A 22-year-old woman admitted to the hospital on Mar. 19, 1985 because of syncopal attacks and numbness on the limbs. Examination revealed peripheral sympathetic as well as parasympathetic dysfunctions and loss of myelinated as well as unmyelinated fibers in a biopsied sural nerve. A diagnosis of acute autonomic and sensory neuropathy (AASN) was made. She was treated with prednisolone but did not respond to the therapy. Only L-DOPS was effective on orthostatic hypotension. Recovery of the neurological conditions was insufficient till May 1987, when she complained of easy fatigability when speaking and chewing. Pharmacological and electrodiagnostic tests showed typical myasthenic phenomenon. At this time, serum anti-ACh receptor antibody was 741nmol/l and a diagnosis of myasthenia gravis was confirmed. Anti-ACh receptor antibody was proven to be elevated in the serum collected on April 1985 when the initial symptom of AASN had appeared. A thymectomy resulted in prompt and complete remission of semiologies of both myasthenia gravis and AASN. These suggest that immunological abnormality might play an important role in the pathogenesis of AASN in this case.
一名22岁女性于1985年3月19日入院,原因是晕厥发作和四肢麻木。检查发现外周交感神经和副交感神经功能障碍,活检的腓肠神经中有髓鞘和无髓鞘纤维均有丢失。诊断为急性自主神经和感觉神经病(AASN)。她接受了泼尼松龙治疗,但对该治疗无反应。只有左旋多巴(L-DOPS)对直立性低血压有效。直到1987年5月,她的神经状况恢复仍不充分,当时她诉说说话和咀嚼时容易疲劳。药理学和电诊断测试显示典型的肌无力现象。此时,血清抗乙酰胆碱受体抗体为741nmol/l,重症肌无力诊断得到证实。1985年4月,即AASN初始症状出现时采集的血清中,抗乙酰胆碱受体抗体被证明升高。胸腺切除术使重症肌无力和AASN的症状迅速完全缓解。这些表明免疫异常可能在该病例AASN的发病机制中起重要作用。