From the Departments of Radiology (A.K.B., J.J.D., R.R.), Pathology (J.R.H.), and Neurology (H.M.F.) University of Alabama at Birmingham, JT-N432, 619 19th St S, Birmingham, AL-35249-6830.
Radiology. 2014 Dec;273(3):940-7. doi: 10.1148/radiol.14131355.
History A previously healthy 23-year-old white man presented to the emergency department of our hospital with a 2-month history of dysarthria, progressively worsening vertigo, and difficulty walking. A diagnosis of retinitis pigementosa was made in this patient's childhood. He did not have any history of congenital syphilis. He did not have a history of nausea or vomiting, fever, weight loss, headache, photophobia, seizure, extremity weakness, or sensory disturbance. Physical examination revealed dysarthria, dysmetria, and ataxia. Kernig and Brudzinski signs were absent, and pathergy test results were negative. Laboratory evaluation revealed normal complete and differential blood counts and normal serum chemistry, including a normal serum angiotensin-converting enzyme level. Analysis of his serum was negative for antinuclear antibody (or ANA), cytoplasmic antineutrophil cvtoplasmic antibody (or cANCA), Sjögren syndrome antigens A and B (SS-A and SS-B, respectively), antitissue transglutaminase and antiendomysial antibodies, and paraneoplastic profile. Serum analysis was also negative for human immunodeficiency virus type 1 and type 2 RNA, Venereal Disease Research Laboratory (VDRL) test, rapid plasma regain (RPR), and fluorescent treponemal antibody absorption. Cerebrospinal fluid (CSF) analysis revealed clear fluid, a normal glucose level (64 mg/dL [3.6 mmol/L]; normal range, 40-70 mg/dL [2.2-3.9 mmol/L]), an elevated protein level (97 mg/dL; normal range, 12-60 mg/dL), and an elevated white blood cell count (7/mm(3) [0.007 ×10(9)/L] in tube 1 and 17/mm(3) [0.017 × 10(9)/L] in tube 2) with 84% lymphocytes. CSF immunoglobulin G level was elevated (30.1 mg/dL; normal, <5.9 mg/dL); however, there were no oligoclonal bands. Gram staining, acid-fast staining, and lactic acid, cryptococcal antigen, histoplasma antigen, herpes simplex virus polymerase chain reaction, VDRL, and RPR test results for CSF were negative. CSF did not grow any bacteria, fungus, or acid-fast bacillus at culture. CSF flow cytometry did not reveal a monoclonal lymphoid population. Initial imaging included brain magnetic resonance (MR) imaging. Computed tomography (CT) images of the chest, abdomen, and pelvis were normal (not shown). The patient's clinical symptoms and imaging findings responded to treatment with a high dose of oral steroids. However, the patient's symptoms exhibited clinical and radiologic progression after several attempts to taper the steroid dose.
病史 一名既往健康的 23 岁白人男性，因构音障碍、进行性加重的眩晕和行走困难就诊于我院急诊科。该患者在儿童时期被诊断为视网膜色素变性。他没有先天性梅毒的病史。他没有恶心、呕吐、发热、体重减轻、头痛、畏光、癫痫发作、四肢无力或感觉障碍。体格检查显示构音障碍、运动失准和共济失调。克尼格征和布鲁津斯基征均为阴性，帕特试验结果阴性。实验室检查显示全血细胞计数和血清化学均正常，包括正常的血清血管紧张素转换酶水平。血清分析抗核抗体（ANA）、细胞质抗中性粒细胞胞浆抗体（cANCA）、干燥综合征抗原 A 和 B（SS-A 和 SS-B）、抗组织转谷氨酰胺酶和抗内因子抗体以及副肿瘤谱均为阴性。血清分析也为阴性，包括人类免疫缺陷病毒 1 型和 2 型 RNA、性病研究实验室（VDRL）试验、快速血浆反应素（RPR）和荧光密螺旋体抗体吸收试验。脑脊液（CSF）分析显示清亮液体，葡萄糖水平正常（64mg/dL[3.6mmol/L]；正常范围 40-70mg/dL[2.2-3.9mmol/L]），蛋白水平升高（97mg/dL；正常范围 12-60mg/dL），白细胞计数升高（第 1 管 7/mm3[0.007×109/L]，第 2 管 17/mm3[0.017×109/L]），淋巴细胞占 84%。CSF 免疫球蛋白 G 水平升高（30.1mg/dL；正常<5.9mg/dL），但无寡克隆带。革兰氏染色、抗酸染色和乳酸、隐球菌抗原、组织胞浆菌抗原、单纯疱疹病毒聚合酶链反应、VDRL 和 RPR 试验结果均为阴性。CSF 培养未生长任何细菌、真菌或抗酸杆菌。CSF 流式细胞术未显示单克隆淋巴细胞群。初始影像学检查包括脑磁共振成像（MRI）。胸部、腹部和骨盆 CT 图像正常（未显示）。患者的临床症状和影像学表现对大剂量口服类固醇治疗有反应。然而，在几次尝试减少类固醇剂量后，患者的症状出现了临床和影像学进展。
