PDF(Pubmed)
Abstract：
UNASSIGNED: Relapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and summarize the clinical characteristics of RP-associated meningoencephalitis.
UNASSIGNED: A 48-year-old man presented with first-ever seizures that were well controlled by valproate. Physical examination results were unremarkable, except for binaural deformation. The initial brain magnetic resonance imaging (MRI) without contrast and electroencephalogram (EEG) findings were normal. However, the patient subsequently developed recurrent fever, scleritis, headache, lethargy, and left arm paresis. Repeated brain MRI with contrast demonstrated increased enhancement of the pia mater and abnormal diffusion-weighted imaging (DWI) signals in the bilateral auricles. The cerebrospinal fluid (CSF) analysis showed 2 leukocytes/μL, 736.5 mg/L of protein, and no evidence of infectious disease or autoimmune encephalitis. Meningoencephalitis secondary to RP was considered. The patient\'s condition improved significantly and quickly with the administration of dexamethasone (10 mg per day). Oral methylprednisolone was continued, and the patient remained well without relapse during the 9-month follow-up period.
UNASSIGNED: RP-associated meningoencephalitis is rare but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Non-specific parenchymal changes and/or meningeal enhancement can be observed on brain MRI scans. CSF lymphocytic pleocytosis with mild protein elevation was observed in most patients.
UNASSIGNED: A 48-year-old man presented with first-ever seizures that were well controlled by valproate. Physical examination results were unremarkable, except for binaural deformation. The initial brain magnetic resonance imaging (MRI) without contrast and electroencephalogram (EEG) findings were normal. However, the patient subsequently developed recurrent fever, scleritis, headache, lethargy, and left arm paresis. Repeated brain MRI with contrast demonstrated increased enhancement of the pia mater and abnormal diffusion-weighted imaging (DWI) signals in the bilateral auricles. The cerebrospinal fluid (CSF) analysis showed 2 leukocytes/μL, 736.5 mg/L of protein, and no evidence of infectious disease or autoimmune encephalitis. Meningoencephalitis secondary to RP was considered. The patient\'s condition improved significantly and quickly with the administration of dexamethasone (10 mg per day). Oral methylprednisolone was continued, and the patient remained well without relapse during the 9-month follow-up period.
UNASSIGNED: RP-associated meningoencephalitis is rare but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Non-specific parenchymal changes and/or meningeal enhancement can be observed on brain MRI scans. CSF lymphocytic pleocytosis with mild protein elevation was observed in most patients.
摘要：
■复发性多软骨炎（RP）是一种罕见的风湿性疾病，可能会影响神经系统，表现多种多样。在这项研究中,我们介绍一例RP相关性脑膜脑炎的病例并总结其临床特点。
■一名48岁男子首次出现癫痫发作，丙戊酸盐控制良好。体检结果无明显,除了双耳变形。初始脑磁共振成像（MRI）无对比和脑电图（EEG）检查结果正常。然而,病人随后出现反复发热,巩膜炎,头痛,嗜睡,和左臂麻痹.重复的增强脑MRI显示双侧耳廓的软脑膜增强和异常的扩散加权成像（DWI）信号。脑脊液（CSF）分析显示2个白细胞/μL，736.5mg/L的蛋白质，没有传染病或自身免疫性脑炎的证据.考虑继发于RP的脑膜脑炎。给予地塞米松（每天10mg）后，患者的病情得到了显着和迅速的改善。继续口服甲基强的松龙，在9个月的随访期间，患者病情良好，没有复发。
■RP相关的脑膜脑炎是罕见但致命的。虽然症状各不相同，红色或变形的耳朵仍然是最常见和暗示性的特征。在脑MRI扫描中可以观察到非特异性实质改变和/或脑膜增强。在大多数患者中观察到脑脊液淋巴细胞增多伴轻度蛋白升高。
■一名48岁男子首次出现癫痫发作，丙戊酸盐控制良好。体检结果无明显,除了双耳变形。初始脑磁共振成像（MRI）无对比和脑电图（EEG）检查结果正常。然而,病人随后出现反复发热,巩膜炎,头痛,嗜睡,和左臂麻痹.重复的增强脑MRI显示双侧耳廓的软脑膜增强和异常的扩散加权成像（DWI）信号。脑脊液（CSF）分析显示2个白细胞/μL，736.5mg/L的蛋白质，没有传染病或自身免疫性脑炎的证据.考虑继发于RP的脑膜脑炎。给予地塞米松（每天10mg）后，患者的病情得到了显着和迅速的改善。继续口服甲基强的松龙，在9个月的随访期间，患者病情良好，没有复发。
■RP相关的脑膜脑炎是罕见但致命的。虽然症状各不相同，红色或变形的耳朵仍然是最常见和暗示性的特征。在脑MRI扫描中可以观察到非特异性实质改变和/或脑膜增强。在大多数患者中观察到脑脊液淋巴细胞增多伴轻度蛋白升高。
