Wolf J, Niedermaier N, Bergner R, Lowitzsch K
Neurologische Klinik mit Klinischer Neurophysiologie, Städt, Klinikum Ludwigshafen, Germany.
Dtsch Med Wochenschr. 2001 Aug 24;126(34-35):947-50. doi: 10.1055/s-2001-16581.
A 41-year-old man was admitted to the hospital because of a series of seizures. History revealed increasing weakness and generalized myalgia following a severe respiratory tract infection three months previously. The clinical examination was dominated by reduced alertness and cognitive impairment.
Positive ANA titres pointed to an autoimmune disease; further laboratory tests revealed an antiphospholipid syndrome. The clinical diagnosis of systemic lupus erythematosus was supported by renal biopsy. Transcranial Doppler sono-graphy and magnetic resonance imaging with angiography revealed cerebral vasculitis with multiple intracerebral grey and white matter lesions.
In addition to an immunosuppressive therapy with corticosteroids and intravenous pulses of high-dose cyclophosphamide, oral anticoagulation was given in order to prevent thromboembolic complications. Seizures were treated with valproate. A follow-up MRI revealed multiple glial cell reactions and reduced vasculitic changes.
Initial neuropsychiatric symptoms and seizures prompted the diagnosis of systemic lupus erythematosus with cerebral vasculitis in a 41-year-old patient. Approximately 20 % of all lupus patients initially present with neuropsychiatric disorders. Central nervous system involvement and renal complications determine the course and prognosis of the disease as well as therapeutic decisions. Particularly in young patients with neuropsychiatric symptoms systemic lupus erythematosus should be excluded routinely.
一名41岁男性因一系列癫痫发作入院。病史显示，三个月前严重呼吸道感染后出现日益加重的虚弱和全身肌痛。临床检查以警觉性降低和认知障碍为主。
抗核抗体（ANA）滴度呈阳性提示自身免疫性疾病；进一步实验室检查显示抗磷脂综合征。肾活检支持系统性红斑狼疮的临床诊断。经颅多普勒超声检查及磁共振血管造影显示脑血管炎伴多发脑灰白质病变。
除用皮质类固醇进行免疫抑制治疗及静脉注射大剂量环磷酰胺外，给予口服抗凝治疗以预防血栓栓塞并发症。癫痫发作采用丙戊酸盐治疗。随访磁共振成像显示多发胶质细胞反应及血管炎改变减轻。
一名41岁患者因最初出现的神经精神症状和癫痫发作而诊断为系统性红斑狼疮伴脑血管炎。约20%的狼疮患者最初表现为神经精神障碍。中枢神经系统受累和肾脏并发症决定了疾病的病程和预后以及治疗决策。特别是对于有神经精神症状的年轻患者，应常规排除系统性红斑狼疮。
