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[1例癫痫发作7年后诊断为系统性红斑狼疮且在泼尼松龙治疗期间出现舞蹈症的病例]

[A case of systemic lupus erythematosus diagnosed 7 years after epileptic seizure and developed chorea during prednisolone treatment].

作者信息

Wakasugi M, Sato T, Maruyama Y, Ueno M, Arakawa M

机构信息

Department of Internal Medicine, Niigata Prefectural Central Hospital, Jyoetsu-city.

出版信息

Ryumachi. 1996 Jun;36(3):545-50.

PMID:8779791
Abstract

An 18-year-old female with 7 years' history of epilepsy was admitted for developing malar rash. She had been treated with hydantoin for 7 years. Laboratory examinations revealed leukopenia and high titer of anti-dsDNA antibodies. Renal biopsy also showed diffuse segmental mesangial proliferative glomerulonephritis. A diagnosis of systemic lupus erythematosus (SLE) was made, and she received 40 mg of prednisolone daily. At follow up 4 months later since her first visit, she developed choreiform movements involving the right upper and lower limbs, despite no signs of increase in her disease activity. Neither biological false positive testing for syphilis nor the lupus anticoagulant (LAC) was detected. MRI demonstrated no signal abnormalities in the brain. Administration of haloperidol was started and the choreiform movements were decreased. Anticonvulsants are associated with drug-induced lupus. On the other hand, seizure is known to be one of the first manifestations of SLE. In drug-induced lupus, positive testing for anti-dsDNA, anti-Sm antibodies, hypocomplementemia and renal involvement are not a frequent as in SLE. In this case, laboratory findings showed high titer of anti-dsDNA antibodies, positive testing for antihistone, anti-SSA, anti-Ki antibodies, and hypocomplementemia. And mesangial proliferative glomerulonephritis was detected. So we diagnosed her as SLE and suggested that epileptic seizure developed 7 years ago had been the first manifestations of SLE. Neurologic complications of SLE are common, but chorea has been rarely reported. Since it is known that LAC is associated with thrombosis, it has been suggested that small infarctions in the basal ganglia may play a part in the pathogenesis of chorea in SLE. In this case, the LAC was negative and MRI showed no detectable abnormalities. As a result another mechanism may be attributed to chorea in this case.

摘要

一名有7年癫痫病史的18岁女性因出现颧部皮疹入院。她接受苯妥英治疗已7年。实验室检查显示白细胞减少及抗双链DNA抗体高滴度。肾活检也显示弥漫性节段性系膜增生性肾小球肾炎。诊断为系统性红斑狼疮(SLE),她开始每日服用40毫克泼尼松龙。自首次就诊4个月后的随访中,尽管疾病活动度无增加迹象,但她出现了累及右上肢和下肢的舞蹈样动作。未检测到梅毒生物学假阳性试验及狼疮抗凝物(LAC)。MRI显示脑部无信号异常。开始给予氟哌啶醇治疗,舞蹈样动作减少。抗惊厥药与药物性狼疮有关。另一方面,癫痫发作是已知的SLE首发表现之一。在药物性狼疮中,抗双链DNA、抗Sm抗体阳性、补体降低及肾脏受累不如SLE常见。在本病例中,实验室检查结果显示抗双链DNA抗体高滴度、抗组蛋白、抗SSA、抗Ki抗体阳性及补体降低。并检测到系膜增生性肾小球肾炎。所以我们将她诊断为SLE,并提示7年前出现的癫痫发作可能是SLE的首发表现。SLE的神经系统并发症很常见,但舞蹈症很少有报道。由于已知LAC与血栓形成有关,有人提出基底节小梗死可能在SLE舞蹈症的发病机制中起作用。在本病例中,LAC阴性且MRI未显示可检测到的异常。因此,本病例中舞蹈症可能归因于另一种机制。

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