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神经结节病:一例报告

Neurosarcoidosis: A Case Report.

作者信息

Nascimento Margarida L, Casanova Rui, Ramalho Rocha Filipa, Malheiro Filipa, Araújo Pedro

机构信息

Internal Medicine Department, Hospital Da Luz Lisboa, Lisbon, PRT.

Intensive Care Unit, Hospital Da Luz Lisboa, Lisbon, PRT.

出版信息

Cureus. 2022 Apr 26;14(4):e24501. doi: 10.7759/cureus.24501. eCollection 2022 Apr.

Abstract

Sarcoidosis is a multi-organ granulomatous disease of unknown etiology. Neurological involvement in sarcoidosis is uncommon but cranial mononeuropathies, especially involving the VII and VIII cranial nerves, are highly suggestive of neurosarcoidosis. We report the case of a 54-year-old woman who presented with fever, night sweats, weight loss, polyarthralgia, and bilateral hearing loss. Mediastinal and hilar lymphadenopathies with hypercaptation on positive emission tomography (PET) scans were present. Low-dose steroids were ineffective. She then developed bilateral anterior uveitis and right-sided peripheral facial palsy. Head magnetic resonance imaging (MRI) showed inflammatory involvement of the right cochlea, geniculate ganglion, and bilateral vestibulocochlear bundle. Cerebrospinal fluid analysis was compatible with aseptic meningitis. Excisional biopsy of mediastinal lymph nodes confirmed the presence of noncaseating granulomas. The diagnosis of systemic sarcoidosis with serious neurological involvement was made and treatment with high-dose steroids led to significant clinical improvement. Sarcoidosis remains a diagnosis of exclusion based on supportive clinical, radiological, and histological findings. This case highlights the challenge it was to diagnose this disorder. Neurologic involvement in sarcoidosis is relatively uncommon and has an unpredictable clinical course and prognosis.

摘要

结节病是一种病因不明的多器官肉芽肿性疾病。结节病累及神经系统并不常见,但颅单神经病,尤其是累及第VII和第VIII颅神经,高度提示神经结节病。我们报告一例54岁女性,表现为发热、盗汗、体重减轻、多关节痛和双侧听力丧失。正电子发射断层扫描(PET)显示纵隔和肺门淋巴结肿大伴摄取增加。低剂量类固醇治疗无效。随后她出现双侧前葡萄膜炎和右侧周围性面瘫。头部磁共振成像(MRI)显示右侧耳蜗、膝状神经节和双侧前庭蜗束有炎症累及。脑脊液分析符合无菌性脑膜炎。纵隔淋巴结切除活检证实存在非干酪样肉芽肿。诊断为伴有严重神经系统累及的系统性结节病,高剂量类固醇治疗使临床症状显著改善。结节病仍然是基于支持性临床、放射学和组织学检查结果而排除其他疾病后的诊断。该病例突出了诊断这种疾病的挑战性。结节病累及神经系统相对少见,临床病程和预后不可预测。

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