[Clinical features and treatment of sarcoidosis involving the central nervous system]

J Akimoto, S Yamanaka, Y Takeda, H Ito, M Masuda, H Utsumi
Nō to Shinkei, Brain and Nerve 1998, 50 (12): 1078-86

PURPOSE: 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).

CASES: 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.

RESULTS: 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.

DISCUSSION: 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.

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