Dias Rita, Ferreira Inês Henriques, Faria Raquel
Serviço de Medicina interna do Centro Hospitalar e Universitário do Porto, Porto, Portugal.
Unidade de Imunologia Clínica, Centro Hospitalar do Porto,Porto, Portugal.
Eur J Case Rep Intern Med. 2020 Feb 6;7(3):001453. doi: 10.12890/2020_001453. eCollection 2020.
We present a case of a 56-year-old man with a history of episcleritis (left) and cluster headache (left) who had a penetrating trauma of the left eye leading to amaurosis 1 month previously. Since then, he developed multiple cranial neuropathy of the right side (V, VII, VIII, IX, X, XI and XII cranial pairs). Magnetic resonance imaging (MRI) revealed an infiltrative lesion of the base of the skull which extended to the retropharyngeal and jugular space, which progressed to multiple leptomeningeal masses extending to the clivus, despite aggressive immunosuppression. Rebiopsy of 1 meningeal mass supported the diagnosis of neurosarcoidosis. The patient finally responded to high-dose prolonged infliximab therapy, with complete remission.
Neurosarcoidosis can present as multiple cranial neuropathy, with extensive nerve involvement depending on the brain and meningeal lesions.Large leptomeningeal pseudotumoural granulomatous masses should be promptly biopsied and lead to aggressive immunosuppressive treatment.Immunosuppressant weaning should be carried out cautiously to avoid rebound worsening.
我们报告一例56岁男性，有左眼巩膜炎病史和丛集性头痛病史，1个月前左眼穿透性外伤导致失明。此后，他出现了右侧多发颅神经病变（第V、VII、VIII、IX、X、XI和XII对颅神经）。磁共振成像（MRI）显示颅底有浸润性病变，延伸至咽后间隙和颈静脉间隙，尽管进行了积极的免疫抑制治疗，但仍进展为多个软脑膜肿块，延伸至斜坡。对1个脑膜肿块再次活检支持神经结节病的诊断。患者最终对大剂量延长疗程的英夫利昔单抗治疗有反应，完全缓解。
神经结节病可表现为多发颅神经病变，根据脑和脑膜病变情况神经受累范围广泛。对于大的软脑膜假瘤样肉芽肿性肿块应及时活检，并进行积极的免疫抑制治疗。免疫抑制剂的撤药应谨慎进行，以避免病情反弹恶化。
