Akimoto J, Yamanaka S, Takeda Y, Ito H, Masuda M, Utsumi H
Department of Neurosurgery, Tanashi Dai-ichi Hospital, Tokyo, Japan.
No To Shinkei. 1998 Dec;50(12):1078-86.
The purpose of this study was to investigate the clinical features, diagnosis, and treatment modalities of three cases with neurosarcoidosis, which involved the central nervous system (CNS).
Three men with neurosarcoidosis, aged 27, 29 and 60 years, are presented. Two of them had previously been given a diagnosis of sarcoidosis. The clinical symptoms of these cases included diabetes insipidus, pituitary dysfunction, seizure, mental disorder, visual field disturbance and pyramidal tract signs. In these cases, CT scan and MRI showed the presence of a tumor near the pituitary gland, diffuse nodules in the subarachnoid space or meningoencephalitis associated with angitis. The level of angiotensin converting enzyme (ACE) in the sera and in the cerebrospinal fluid, were elevated in the two cases who had no brain biopsy. All three cases were treated with steroids; two of them received pulse steroid therapy.
The two cases who received pulse steroid therapy responded quickly, with improvement in clinical features, serum ACE levels and neuroradiological findings. Under oral administration of steroids, all three cases recovered with complete remission of neurosarcoidosis except for endocrinological symptoms.
The main pathological changes of neurosarcoidosis are granulomatous angitis of the venular walls and occasionally, of the capillaries near the meninx and Virchow-Robin space. The patients also had symptoms of secondary meningoencephalitis. These changes were mainly located in the hypothalamus and pituitary gland. The patients had complex symptoms resulting from endocrine system granuloma, as well as from cerebral ischemia. The severity of the disease and effectiveness of treatment, can be evaluated by measuring ACE levels in the cerebrospinal fluid (over 1. 0 IU/l), and by Gd-enhanced MRI. Early pulse steroid therapy with subsequent oral steroid administration is thought to be important for neurosarcoidosis treatment, in order to prevent irreversible damage in the CNS.
本研究旨在探讨3例累及中枢神经系统（CNS）的神经结节病患者的临床特征、诊断及治疗方式。
报告了3例神经结节病男性患者，年龄分别为27岁、29岁和60岁。其中2例先前已被诊断为结节病。这些病例的临床症状包括尿崩症、垂体功能障碍、癫痫发作、精神障碍、视野障碍和锥体束征。在这些病例中，CT扫描和MRI显示垂体附近有肿瘤、蛛网膜下腔弥漫性结节或与血管炎相关的脑膜脑炎。2例未进行脑活检的患者血清和脑脊液中的血管紧张素转换酶（ACE）水平升高。所有3例患者均接受了类固醇治疗；其中2例接受了脉冲类固醇治疗。
接受脉冲类固醇治疗的2例患者反应迅速，临床特征、血清ACE水平和神经影像学表现均有改善。在口服类固醇治疗下，除内分泌症状外，所有3例患者均康复，神经结节病完全缓解。
神经结节病的主要病理变化是小静脉壁的肉芽肿性血管炎，偶尔也见于脑膜和Virchow-Robin间隙附近的毛细血管。患者还出现继发性脑膜脑炎症状。这些变化主要位于下丘脑和垂体。患者因内分泌系统肉芽肿以及脑缺血而出现复杂症状。可通过测量脑脊液中的ACE水平（超过1.0 IU/l）和钆增强MRI来评估疾病的严重程度和治疗效果。早期进行脉冲类固醇治疗，随后口服类固醇，被认为对神经结节病的治疗很重要，以防止中枢神经系统发生不可逆损伤。
