Department of Medicine, Aseer Central Hospital, Abha, Saudi Arabia.
Int J Rheum Dis. 2009 Jul;12(2):170-3. doi: 10.1111/j.1756-185X.2009.01401.x.
The classical clinical picture of antiphospholipid antibody syndrome (APS) is characterized by venous and arterial thrombosis, fetal losses and thrombocytopenia in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system involvement is one of the most prominent clinical manifestations of APS, and includes thrombotic events, psychiatric features and a variety of other non-thrombotic neurological syndromes. We present a 9-year-old Saudi girl who developed psychotic illness without thrombotic manifestations. Autoantibodies against cardiolipin were persistent and strongly positive while antinuclear antibodies and antibodies against double-stranded DNA was absent. Her brain computed tomography, magnetic resonance imaging, magnetic resonance arteriography and magnetic resonance venography all were normal. There was no evidence of infection, drug intake or connective tissue disorders, So a diagnosis of primary APS was likely. Starting on antipsychotics only was unsatisfactory and marked improvement occurred after combined treatment with antidepressants (imipramine 10 mg and risperdal 0.2 mg, both once daily), small-dose aspirin (100 mg) and hydroycloroquine (100 mg) both once daily. Unfortunately aspirin was stopped by the family and 5 months later she developed right axillary vein thrombosis. This case presented psychotic illness. Investigations revealed the presence of anticardiolipin antibodies without a thromboembolic picture, mimicking Hughes syndrome but not fulfilling the criteria needed for the diagnosis. Thus, psychosis should be appreciated as a presenting symptom for primary APS and combined treatment with antipsychotics, aspirin and antimalarials is recommended.
抗磷脂抗体综合征(APS)的典型临床表现为在存在抗心磷脂抗体和/或狼疮抗凝物的情况下出现静脉和动脉血栓形成、胎儿丢失和血小板减少。APS 可作为原发性疾病或结缔组织疾病(最常见的是系统性红斑狼疮)的继发性疾病发生。中枢神经系统受累是 APS 最突出的临床表现之一,包括血栓形成事件、精神症状和各种其他非血栓性神经综合征。我们介绍了一位 9 岁的沙特女孩,她出现了没有血栓表现的精神病。抗心磷脂抗体持续存在且强阳性,而抗核抗体和抗双链 DNA 抗体阴性。她的脑计算机断层扫描、磁共振成像、磁共振血管造影和磁共振静脉造影均正常。没有感染、药物摄入或结缔组织疾病的证据,因此可能诊断为原发性 APS。仅使用抗精神病药物治疗效果不佳,在联合使用抗抑郁药(丙咪嗪 10mg 和利培酮 0.2mg,均每日一次)、小剂量阿司匹林(100mg)和羟氯喹(100mg),每日一次)后症状明显改善。不幸的是,阿司匹林被家人停用,5 个月后她出现右侧腋窝静脉血栓形成。该病例表现为精神病。检查显示存在抗心磷脂抗体,但没有血栓栓塞表现,类似于 Hughes 综合征,但不符合诊断所需的标准。因此,应将精神病视为原发性 APS 的首发症状,建议联合使用抗精神病药物、阿司匹林和抗疟药进行治疗。