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抗磷脂综合征与大脑

APS and the brain.

作者信息

Sastre-Garriga J, Montalban X

机构信息

Unitat de Neuroimmunology Clínica, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

出版信息

Lupus. 2003;12(12):877-82. doi: 10.1191/0961203303lu496oa.

Abstract

Antiphospholipid antibody syndrome (APS) may present with neurological syndromes. Cerebrovascular disease, chorea/ballismus, epileptic seizures, headache, cognitive impairment, transverse myelopathy, Devic's syndrome and multiple sclerosis-like presentations feature among others. Cerebrovascular disease is one of the most common presenting symptoms of APS, second only to deep vein thrombosis, and accounts for half of neurological manifestations in patients with APS; accelerated atherogenesis and cardioembolism are the most likely mechanisms implicated. Though infrequent, chorea is consistently associated with APS; the pathogenetic role of antiphospholipid antibodies (APLab) in this case might be routed through cerebrovascular disease in some cases and through purely immunological pathways in others. Both ischemic and immunological mechanisms have been demonstrated in the pathogenesis of epileptic seizures, which may account for 7% of neurological manifestations in APS. Although frequent in APS, a causative link between APLab and most common types of headache (migraine and tension-type headache) is more than dubious. Cognitive impairment may derive from a well-defined clinical tableau of multi-infarct dementia. Nevertheless, (highly frequent) less severe cognitive impairment has also been associated with the presence of APLab in the absence of magnetic resonance findings. A relationship between APS and transverse myelopathy seems likely but small numbers in the studies published to date preclude definite statements; routinely testing for APLab patients with neurological manifestations suggestive of multiple sclerosis seems to be unrecommended at the present time.

摘要

抗磷脂抗体综合征(APS)可能会出现神经综合征。脑血管疾病、舞蹈症/手足徐动症、癫痫发作、头痛、认知障碍、横贯性脊髓炎、视神经脊髓炎谱系疾病及类似多发性硬化的表现等都有报道。脑血管疾病是APS最常见的症状之一,仅次于深静脉血栓形成,占APS患者神经表现的一半;动脉粥样硬化加速和心源性栓塞是最可能的相关机制。舞蹈症虽不常见,但一直与APS相关;抗磷脂抗体(APLab)在这种情况下的致病作用,在某些病例中可能通过脑血管疾病介导,而在其他病例中则通过纯免疫途径介导。癫痫发作的发病机制已证实存在缺血和免疫两种机制,癫痫发作可能占APS神经表现的7%。虽然在APS中很常见,但APLab与最常见的头痛类型(偏头痛和紧张型头痛)之间的因果关系很值得怀疑。认知障碍可能源于明确的多梗死性痴呆临床症状。然而,在没有磁共振成像结果的情况下,(非常常见的)不太严重的认知障碍也与APLab的存在有关。APS与横贯性脊髓炎之间似乎存在关联,但迄今为止发表的研究数量较少,无法得出明确结论;目前似乎不建议对有提示多发性硬化的神经表现的患者常规检测APLab。

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