氟代脱氧葡萄糖正电子发射断层扫描（FDG-PET）正常的抗Ma2相关脑炎：一例假性惠普尔病
Anti-Ma2-associated encephalitis with normal FDG-PET: a case of pseudo-Whipple's disease.
作者信息
Castle James, Sakonju Ai, Dalmau Josep, Newman-Toker David E
机构信息
Stanford University, CA, USA.
出版信息
Nat Clin Pract Neurol. 2006 Oct;2(10):566-72; quiz 573. doi: 10.1038/ncpneuro0287.
BACKGROUND
A 39-year-old man presented with a history of several months of progressive personality changes, social withdrawal, bradykinesia, mutism, dysphagia, worsening gait, and difficulty with daily living activities. Examination revealed an atypical parkinsonian appearance with incomplete supranuclear ophthalmoplegia and an unusual oculomotor disorder characterized by both low-amplitude, intermittent opsoclonus, and slow, nystagmoid intrusions.
INVESTIGATIONS
Routine laboratory testing, autoimmune and infectious serologies, brain MRI, lumbar puncture, electroencephalogram, whole-body CT scan, paraneoplastic serologies, small bowel biopsy, 18F-fluorodeoxyglucose positron emission tomography CT scan, brain biopsy, and testicular ultrasound.
DIAGNOSIS
Anti-Ma2 paraneoplastic encephalitis in association with metastatic testicular cancer; initially misdiagnosed as CNS Whipple's disease.
MANAGEMENT
Corticosteroids, intravenous immunoglobulins, orchiectomy, muscle relaxants, mycophenolate mofetil, plasmapheresis, and bleomycin, etoposide and platinum chemotherapy.
背景
一名39岁男性，有几个月渐进性人格改变、社交退缩、运动迟缓、缄默症、吞咽困难、步态恶化及日常生活活动困难的病史。检查发现有非典型帕金森样表现，伴有不完全性核上性眼肌麻痹，以及一种不寻常的眼球运动障碍，其特征为低幅度、间歇性眼阵挛和缓慢的眼球震颤样侵入。
检查
常规实验室检查、自身免疫和感染血清学检查、脑部磁共振成像、腰椎穿刺、脑电图、全身计算机断层扫描、副肿瘤血清学检查、小肠活检、18F-氟脱氧葡萄糖正电子发射断层扫描计算机断层扫描、脑活检及睾丸超声检查。
诊断
抗Ma2副肿瘤性脑炎合并转移性睾丸癌；最初误诊为中枢神经系统惠普尔病。
治疗
使用皮质类固醇、静脉注射免疫球蛋白、睾丸切除术、肌肉松弛剂、霉酚酸酯、血浆置换，以及博来霉素、依托泊苷和铂类化疗。
Zotero 插件