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[系统性红斑狼疮的中枢神经系统受累]

[Central nervous system involvement in systemic lupus erythematosus].

作者信息

Louarn F, Mas J L, Degos J D

出版信息

Rev Neurol (Paris). 1984;140(2):110-6.

PMID:6710010
Abstract

Three cases are presented, in two of which the CNS lesions revealed the presence of systemic lupus erythematosus (SLE). The diagnosis of SLE was certain according to the criteria of the ARA, and it was further confirmed by results of renal needle puncture biopsy. Case 1: A 16-year-old adolescent developed choreic movements followed, one month later, by psychotic symptoms suggesting a mixed hebephrenic-catatonic schizophrenic affection. Cutaneous lesions and signs of renal insufficiency 3 months later established that these disorders were related to SLE. A favourable outcome was observed rapidly for the systemic signs, recovery from neuropsychic symptoms being obtained after 3 months only but then in a few days. This course suggests the diagnosis of a "functional psychosis" of lupus origin. Case 2: A 24-year-old woman developed left hemiparesis followed by febrile coma. The slowly favourable course of the disease led to the appearance of a progressive dementia, with numerous epileptic seizures. Although tests for antinuclear antibodies were negative and the ESR was normal, several minor biological anomalies were suggestive of a systemic disease and the diagnosis of SLE was finally established. Corticotherapy produced only slight transient improvement. This progression towards dementia with progressive cerebral atrophy is most probably related to cerebral lupus lesions, the initial coma in the absence of any other apparent cause possibly being the first sign. Case 3: A 47-year-old woman developed simultaneously or separately episodes of arthralgia and uveitis of unknown origin over a 12-year period, and attacks of regressive multilocular neurological deficiency over a 15-year period.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

本文报告了3例病例,其中2例中枢神经系统病变显示存在系统性红斑狼疮(SLE)。根据美国风湿病学会(ARA)的标准,SLE的诊断明确,肾穿刺活检结果进一步证实了该诊断。病例1:一名16岁青少年出现舞蹈样动作,1个月后出现精神病性症状,提示为青春型-紧张型精神分裂症混合型。3个月后出现皮肤病变和肾功能不全体征,表明这些疾病与SLE有关。全身症状迅速好转,神经精神症状仅在3个月后开始恢复,但几天后即康复。此病程提示为狼疮源性“功能性精神病”。病例2:一名24岁女性出现左侧偏瘫,随后出现发热性昏迷。疾病缓慢好转,继而出现进行性痴呆,并伴有多次癫痫发作。尽管抗核抗体检测为阴性,血沉正常,但一些轻微的生物学异常提示存在系统性疾病,最终确诊为SLE。皮质激素治疗仅产生轻微的短暂改善。这种向痴呆伴进行性脑萎缩的进展很可能与脑狼疮病变有关,最初无明显原因的昏迷可能是首发症状。病例3:一名47岁女性在12年期间同时或分别出现不明原因的关节痛和葡萄膜炎发作,并在15年期间出现退行性多灶性神经功能缺损发作。(摘要截短于250字)

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