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一例罕见的仅累及延髓的神经结节病,酷似恶性脑肿瘤。

A rare case of neurosarcoidosis occurred only in the medulla oblongata mimicking malignant brain tumor.

作者信息

Murayama Kentaro, Inoue Akihiro, Nakamura Yawara, Ochi Masayuki, Shigekawa Seiji, Watanabe Hideaki, Kitazawa Riko, Kunieda Takeharu

机构信息

Department of Neurosurgery, Ehime University School of Medicine, Toon, Ehime, Japan.

Department of Neurology and Geriatric Medicine, Ehime University School of Medicine, Toon, Ehime, Japan.

出版信息

Surg Neurol Int. 2021 May 31;12:243. doi: 10.25259/SNI_195_2021. eCollection 2021.

Abstract

BACKGROUND

Sarcoidosis is a multisystem disorder characterized by noncaseating epithelioid granulomas. However, neurosarcoidosis occurring only in the medulla oblongata is very rare and lacks specific imaging and clinical features. We report a rare case of neurosarcoidosis arising from the medulla oblongata alone, suggesting the significance of pathological findings for accurate diagnosis.

CASE DESCRIPTION

A 78-year-old woman with a history of rheumatoid arthritis was admitted to our hospital with a 3-month history of progressive numbness in bilateral lower extremities and gait disturbance. Neurological examination on admission showed mild bilateral paired paralysis of the lower limbs (manual muscle test: right 2/V; left 4/V) and marked numbness in the right lower limb. Neuroimaging revealed a solid mass with clear boundaries in the dorsal medulla oblongata appearing hypointense on T1-weighted imaging (WI), hyperintense on T2-WI, and hypointense on diffusion WI (DWI), with strong enhancement on gadolinium-enhanced T1-WI. Cerebrospinal fluid analysis showed moderately elevated levels of protein and lymphocytic cells. Biopsy to determine the exact diagnosis revealed histological findings of noncaseating epithelioid granulomas and inflammatory infiltration, consistent with sarcoidosis. Postoperatively, corticosteroid therapy with prednisolone was initiated as soon as possible, resulting in marked reductions in lesion size. Follow-up neuroimaging after 12 months showed no signs of recurrence.

CONCLUSION

Neurosarcoidosis is difficult to diagnose from routine neuroimaging and laboratory findings. Accurate diagnosis requires careful identification of clinical signs, hypointensity on DWI, and morphological findings from surgical biopsy.

摘要

背景

结节病是一种以非干酪样上皮样肉芽肿为特征的多系统疾病。然而,仅发生于延髓的神经结节病非常罕见,且缺乏特异性的影像学和临床特征。我们报告一例罕见的仅起源于延髓的神经结节病病例,提示病理结果对准确诊断的重要性。

病例描述

一名78岁有类风湿关节炎病史的女性因双下肢进行性麻木3个月及步态障碍入院。入院时神经系统检查显示双下肢轻度双侧对称性瘫痪(徒手肌力检查:右侧2/5级;左侧4/5级),右下肢明显麻木。神经影像学检查显示延髓背侧有一个边界清晰的实性肿块,在T1加权成像(WI)上呈低信号,T2加权成像上呈高信号,扩散加权成像(DWI)上呈低信号,钆增强T1加权成像上有明显强化。脑脊液分析显示蛋白和淋巴细胞水平中度升高。为明确诊断进行的活检显示有非干酪样上皮样肉芽肿和炎症浸润的组织学表现,符合结节病。术后尽快开始使用泼尼松龙进行皮质类固醇治疗,病灶大小明显缩小。12个月后的随访神经影像学检查未显示复发迹象。

结论

神经结节病难以从常规神经影像学和实验室检查结果中诊断出来。准确诊断需要仔细识别临床体征、DWI上的低信号以及手术活检的形态学表现。

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