Kohlhaas K, Brechmann T, Vorgerd M
Neurologische Universitäts-Klinik, Bergmannsheil GmbH, Ruhr-Universität Bochum, Germany.
Dtsch Med Wochenschr. 2007 Aug;132(34-35):1748-52. doi: 10.1055/s-2007-984960.
A 53-year-old male was admitted with an acute brainstem syndrome. He developed a severe fluctuating psychosis. Because of the worsening neurological symptoms he was admitted to our neurological clinic five months after onset of the disease. On admission he showed signs of a productive psychosis in addition to akinetic-rigid parkinsonism and cerebellar symptoms.
Laboratory tests revealed a HBeAg-negative hepatitis B. The initial neuroradiolgical studies showed multiple supratentorial and periventricular ischemic and hemorrhagic lesions. MR-angiography and conventional cerebral angiography demonstrated multiple irregularities of the intracranial vessels and vascular occlusions, findings which were compatible with cerebral vasculitis.
DIAGNOSIS, THERAPY AND COURSE: The laboratory and neuroradiological studies indicated a hepatitis B-associated polyarteriitis nodosa and cerebral vasculitis. He was given oral immunsuppressive therapy (prednisolone 60 mg daily) and virostatic drug (lamivudine 100 mg daily). When the steroid dosis was reduced to 40 mg prednisolon a severe relapse of the encephalopathy occurred which was treated with the atypical antipsychotic drug risperidon, 3 mg daily, and intravenous methylprednisolone plus plasmaphereses. Later he was given prednisolone (60 mg daily) and lamivudine (100 mg daily) again which has so far stabilized the clinical course.
The main treatment of the rare hepatitis B-associated polyarteriitis nodosa with cerebral vasculitis consists of oral steroids in combination with antiviral drugs. Depending on the course of the disease an escalating steroid pulse administration and plasmaphereses should be considered.
一名53岁男性因急性脑干综合征入院。他出现了严重的波动性精神病症状。由于神经症状不断恶化,在疾病发作五个月后他被收入我们的神经科诊所。入院时,除了运动不能-强直型帕金森综合征和小脑症状外,他还表现出有幻觉的精神病症状。
实验室检查显示乙肝e抗原阴性的乙型肝炎。最初的神经放射学研究显示幕上和脑室周围有多处缺血性和出血性病变。磁共振血管造影和传统脑血管造影显示颅内血管多处不规则及血管闭塞,这些表现符合脑血管炎。
诊断、治疗及病程:实验室和神经放射学研究表明为乙型肝炎相关性结节性多动脉炎和脑血管炎。给予他口服免疫抑制治疗(泼尼松龙每日60毫克)和抗病毒药物(拉米夫定每日100毫克)。当类固醇剂量减至泼尼松龙40毫克时,脑病严重复发,使用非典型抗精神病药物利培酮每日3毫克以及静脉注射甲泼尼龙加血浆置换进行治疗。后来再次给予他泼尼松龙(每日60毫克)和拉米夫定(每日100毫克),目前临床病程已稳定。
罕见的乙型肝炎相关性结节性多动脉炎合并脑血管炎的主要治疗方法是口服类固醇联合抗病毒药物。根据疾病进程,应考虑逐步增加类固醇冲击治疗及血浆置换。