Iwata M, Kondo M, Ando M, Tano M, Inagaki Y, Shimizu Y, Suzuki T, Ito K, Matsumoto S, Shiroyama N
Department of Internal Medicine, Kariya General Hospital, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1993 Aug;31(8):1050-5.
Sarcoidosis is a multisystem disorder of unknown etiology presenting most frequently with bilateral hilar lymphadenopathy (BHL), pulmonary infiltration, and ocular and skin lesions. Neurological manifestations are found in about 5% of patients with systemic sarcoidosis. We report the case of a 58-year-old woman with neurosarcoidosis manifesting as isolated, peripheral polyneuropathy. This is a rare neurological manifestation. Two years before admission, she was found to have an abnormal chest radiograph showing BHL, and pulmonary sarcoidosis was diagnosed by lung biopsy. Six months later, she noted facial erythematous lesions, and lupus pernio was confirmed by skin biopsy. Fifteen months before admission, she developed visual disturbance of the right eye caused by sarcoid-uveitis. Two months before admission, she complained of paresthesia and weakness of limbs. She was admitted for nerve-biopsy. Sural nerve biopsy revealed sarcoid granulomas in the epineurial space, periangitis and axonal degeneration. Neurologically, the diagnosis of mononeuritis multiplex without cranial nerve palsy was made. Treatment with 30 mg prednisolone daily was initiated, and neurological and extraneurological improvement was noticeable within two weeks of treatment. The dose was carefully lowered over several months.
结节病是一种病因不明的多系统疾病，最常见的表现为双侧肺门淋巴结肿大（BHL）、肺部浸润以及眼部和皮肤病变。约5%的系统性结节病患者会出现神经学表现。我们报告一例58岁女性神经结节病患者，表现为孤立性周围性多发性神经病，这是一种罕见的神经学表现。入院前两年，她的胸部X线片显示异常，提示BHL，经肺活检确诊为肺部结节病。六个月后，她出现面部红斑性病变，经皮肤活检确诊为冻疮样狼疮。入院前十五个月，她因结节性葡萄膜炎出现右眼视力障碍。入院前两个月，她主诉肢体感觉异常和无力。她因神经活检入院。腓肠神经活检显示神经外膜间隙有结节性肉芽肿、血管周围炎和轴突变性。神经学检查诊断为无颅神经麻痹的多发性单神经炎。开始每日服用30毫克泼尼松进行治疗，治疗两周内神经学和神经外表现均有明显改善。数月内小心地逐渐减少剂量。
