Open AccessHydrocephalus as the first presenting symptom of neurosarcoidosis in two patients: a diagnosis more forthcoming in the context of systemic disease
Author(s) -
Anna McKeever,
Amanda Cox,
Matthew Garnett,
Nicholas G Cunniffe
Publication year - 2019
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2019-229903
Subject(s) - sarcoidosis , neurosarcoidosis , medicine , context (archaeology) , hydrocephalus , disease , systemic disease , multisystem disease , presentation (obstetrics) , pathology , dermatology , surgery , paleontology , biology
Sarcoidosis is a multisystem disorder, characterised histologically by the presence of non-caseating epithelioid granulomas with exclusion of other granulomatous diseases. While the lungs and lymph nodes are affected in 90%, approximately 5% of patients have neurological involvement. The clinical manifestations of neurosarcoidosis (NS) are diverse, making diagnosis especially difficult in patients without known systemic disease. Hydrocephalus occurs in only 9% of patients with NS and although uncommon, is an important manifestation because it is associated with high morbidity and mortality. We report two cases of NS presenting with hydrocephalus, one as the first presentation of sarcoidosis and one in a patient with known multisystem sarcoidosis. The patient without systemic sarcoidosis posed the greater diagnostic challenge and followed a protracted course with multiple surgical interventions, progression of central nervous system inflammation and significant physical disability.