Hydrocephalus as the first presenting symptom of neurosarcoidosis in two patients: a diagnosis more forthcoming in the context of systemic disease

Abstract

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.

Hydrocephalus as the presenting symptom of sarcoidosis: A case report and review of literature

“…We present one such case in this report. To the best of our knowledge, only 21 others exist in the literature, of which we were able to include 19 in this report (Table ) . Hydrocephalus typically presents with nonspecific symptoms, and the diagnosis is made based on imaging .…”

“…To the best of our knowledge, only 21 others exist in the literature, of which we were able to include 19 in this report ( Table 1). [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Hydrocephalus typically presents with nonspecific symptoms, and the diagnosis is made based on imaging. 8 The differential diagnosis is extensive (Table 2) making the correct attribution difficult due to a lack of definitive diagnostic testing for neurosarcoidosis.…”

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

“…4 Hydrocephalus and spinal cord involvement are the rarest presentations of NS. 3,4 Hydrocephalus, either communicating or noncommunicating, has been noted in 6%-9% of cases of NS, 4,5 while spinal cord involvement occurs in approximately 6%-8% of NS cases 6 and occurring in <1% of all sarcoidosis cases. 3 The clinical presentations of NS can be very broad and atypical, which makes the diagnosis of such disease exceedingly challenging especially in previously healthy individuals who come with hydrocephalus and/or myelopathy as initial manifestations without other evident symptoms of sarcoidosis.…”

“…3 The clinical presentations of NS can be very broad and atypical, which makes the diagnosis of such disease exceedingly challenging especially in previously healthy individuals who come with hydrocephalus and/or myelopathy as initial manifestations without other evident symptoms of sarcoidosis. 4,5,7 We report a rare case of NS with hydrocephalus and subsequent myelopathy as the initial presentations of the disease to feature the challenge of diagnosing NS in the absence of known systemic sarcoidosis and to emphasize the importance of timely diagnosis and appropriate treatment with steroid in reducing subsequent neurological sequelae.…”

Difficulties in the Diagnostic of Neurosarcoidosis (Clinical Case)

“…Течение заболевания хроническое, темп развития симптоматики медленный с длительными ремиссиями либо быстрый с неуклонным прогрессированием симптомов [7]. Очевидно, что помимо симптоматики, связанной с локализацией гранулем в ткани мозга, возникают симптомы, обусловленные расстройствами кровообращения и ликвороциркуляции [8].…”