Diagnosis and Treatment in Neurosarcoidosis

Abstract

Sarcoidosis is an inflammatory multisystem disorder, affecting many systems such as lung, lymph nodes, skin and eye involvement. Nervous system involvement is often seen in 5-15% of patients with systemic sarcoidosis in the first two years. Preceding to systemic involvement the initial symptom as neurological complaints has been rarely reported. Lacking of any specific, clinical and / or radiological findings for neurosarcoidosis in these cases, it could be difficult to make an accurate diagnosis and histopathological evaluation may be required. Due to rarity and complexity diagnosis of the neurosarcoidosis, in this study, clinical, radiological and / or histopathological features, treatment modalities of the 7 neurosarcoidosis patients to be presented with detailed investigations of different neurological symptoms were evaluated.

Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis

“…The individual study periods varied between 3 and 31 years (median 13 years, IQR 9–15). Only one of 29 studies was performed prospectively (3%); [6] 24 of 29 (83%) were single-centre studies [6, 9, 11–19, 21–27, 29–31, 34–36]. Inclusion criteria were described in 27 of 29 studies (93%) and were based on the Zajicek criteria in 11 studies [8, 9, 25, 27–31, 33, 35, 36].…”

“…Four studies included patients with definite or probable neurosarcoidosis according to these criteria, and seven included definite, probable, or possible neurosarcoidosis [8, 9, 25, 27–31, 33, 35, 36]. Eleven studies included biopsy-proven sarcoidosis [6, 11, 14, 16, 18, 20–23, 32, 34]. Five studies included patients with the diagnosis based on either histological evidence of non-caseating granulomas or clinical and radiologic findings consistent with the diagnosis sarcoidosis [13, 15, 17, 19, 24].…”

“…Five studies included patients with the diagnosis based on either histological evidence of non-caseating granulomas or clinical and radiologic findings consistent with the diagnosis sarcoidosis [13, 15, 17, 19, 24]. Twelve studies considered only central nervous system involvement of neurosarcoidosis [8, 9, 12, 17, 18, 24, 26–28, 30, 33, 34]. Calculation of the heterogeneity of data reported in the pooled study results showed significant heterogeneity ( I 2  > 50%) occurred for 48 of 110 studied parameters (Additional file 1: Table S2 A-C).

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Hydrocephalus and myelopathy: A rare and challenging case of sarcoidosis and review of literature

“…9 Neurologic manifestations due to systemic sarcoidosis are far more rare and lethal finding, occurring in 5%-15% of systemic sarcoidosis cases. 10,11 In addition to systemic symptoms, a systematic review by Mubarik et al reported neurosarcoidosis (NS) commonly presenting as a cranial nerve palsy, usually the facial nerve in up to 50% of patients, headache, and sensory or motor impairment in about 30% of cases. 12 NS presenting with neurological symptoms alone and with hydrocephalus and spinal cord involvement, as seen in our patient presented, is far more uncommon occurring in only about 1% of cases.…”

“…8,14 Corticosteroids are suggested as the first-line treatment for NS, usually at a dose of 1 mg/kg/dose of prednisolone with a taper for 6-8 months. 10 However, long-term use of steroids has been associated with severe and debilitating side effects, and therefore, second-line treatment with methotrexate, cyclophosphamide, and other immunosuppressive agents has been used. 15 TNF-alpha antagonists are beginning to be used in randomized clinical control trials in combination with first-and second-line therapies; however, there is still no well-structured guidelines in mainstreaming their use.…”