An Atypical Presentation of ‘Possible’ Neurosarcoidosis: A Stepwise Approach to a Case with Review of Literature

Bhanu Gogia, Varun B. Shah, Prashant K. Rai, Karthikram Raghuram and Chilvana Patel

Objective: To report an atypical case that highlights the challenges involved in diagnosing neurosarcoidosis.
Case description: A 38-year-old Caucasian man presented with a three month history of headache and a ten day history of confusion and abnormal movements in the right upper extremity. The patient did not have any other systemic symptoms such as visual disturbances, arthritis, oral or genital ulcers, dry eyes or dry skin, but reported a history of weight loss, night sweats as well. MR brain showed extensive white matter disease with deep grey matter involvement, linear perivascular enhancement and deep medullary venous engorgement, suggesting deep vein thrombosis. Cerebrospinal fluid (CSF) labs revealed a leukocytosis of 98/ul, glucose of 39 mg/dl, and protein of 413 mg/dl. CSF angiotensin converting enzyme (ACE) positive at 4 U/L. The infectious and rheumatological workup were negative including fungal cultures, MTB PCR, Quant Gold test, ANA, Anti SSA, SSB, ANCA, HIV, HBV. Computed tomography (CT) chest showed pulmonary granulomatous disease and calcified hilar lymphadenopathy. Meningeal biopsy was negative for granulomas. After considering and ruling out conditions such as Behcet’s disease, Sjogren’s, vasculitis, and CNS lymphoma. Our patient fitted into ‘possible’ neurosarcoidosis due to a lack of pathological confirmation of granulomatous disease.
Conclusion: Diagnosis of neurosarcoidosis remains challenging with the multitude of symptoms and non-specific laboratory tests. Broad differentials including neoplasm especially lymphoma, infectious etiologies such as fungal, mycobacterium and alternate inflammatory conditions including Behcet’s should be considered and excluded appropriately. Neurosarcoidosis is one of the common inflammatory conditions associated with cerebral venous sinus thrombosis.

Published on: May 27, 2020
doi: 10.17756/jnpn.2020-034
Citation:  Gogia B, Shah VB, Rai PK, Raghuram K, Patel C. 2020. An Atypical Presentation of ‘Possible’ Neurosarcoidosis: A Stepwise Approach to a Case with Review of Literature. J Neuroimaging Psychiatry Neurol 5(1): 16-19.