结节病是一种病因不明的多器官肉芽肿性疾病。结节病的神经系统受累并不常见,但颅内单神经病,特别是涉及VII和VIII脑神经,高度怀疑是神经结节病.我们报道了一名54岁的女性发烧的病例,盗汗,减肥,多关节痛,和双侧听力损失。在发射断层扫描(PET)阳性扫描中,存在纵隔和肺门淋巴结肿大。低剂量类固醇无效。然后,她出现了双侧前葡萄膜炎和右侧周围性面神经麻痹。头部磁共振成像(MRI)显示右侧耳蜗有炎症,膝状神经节,和双侧前庭耳蜗束。脑脊液分析与无菌性脑膜炎兼容。纵隔淋巴结切除活检证实存在非干酪性肉芽肿。诊断为患有严重神经系统受累的系统性结节病,并使用大剂量类固醇治疗可明显改善临床效果。结节病仍然是基于支持性临床的排除诊断,放射学,和组织学发现。这个案例凸显了诊断这种疾病的挑战。结节病的神经系统受累相对罕见,并且具有不可预测的临床病程和预后。
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.