Hydrocephalus in Neurosarcoidosis: Clinical Course, Radiographic Accompaniments, and Experience with Shunting

Background: Hydrocephalus is an uncommon manifestation of neurosarcoidosis (7-14% of reported cohorts) that poses unique challenges to patient management. Despite being a recognized complication of neurosarcoidosis, very little is known about how hydrocephalus influences its clinical course, management, and prognosis.

Objectives: To characterize hydrocephalus as a clinical manifestation of neurosarcoidosis, highlight which patients required cerebrospinal fluid (CSF) diversion, understand the mediating role of immunomodulatory treatments, and report outcomes in this cohort.

Methods: Patients with a diagnosis of neurosarcoidosis seen at Emory Healthcare [01/2011-8/2021] were included if hydrocephalus was one manifestation of their disease. Means and proportions were compared between shunted and non-shunted groups using the Wilcoxon rank-sum test for continuous variables and the Fisher's exact test for categorical variables.

Results: Twenty-two patients with neurosarcoidosis and hydrocephalus as one disease manifestation were included (22/214, 10.3%). Hydrocephalus was communicating in 13 (13/20, 65.0%) and obstructive in 6 patients (6/20, 30.0%), with features of both seen in 1 patient (1/20, 5.0%). Chronic presentations were typical (12/22, 54.5%) with altered sensorium, gait dysfunction, headache, and weakness being present in the majority of patients. There was a rostral-to-caudal gradient in ventriculomegaly, with the lateral ventricles most affected (20/20, 100%) and the fourth ventricle the least (12/20, 60%). Meningoventricular inflammation was the most common neuroinflammatory accompaniment (18/20, 90.0%), especially infratentorial leptomeningitis (16/20, 80.0%) and fourth ventriculitis (9/20, 45.0%). Thirteen patients (13/22, 59.1%) required ventriculoperitoneal shunts (VPS). Factors associated with shunt placement were younger age at neurosarcoidosis onset (p = 0.019) and hydrocephalus onset (p = 0.015), obstructive hydrocephalus (p = 0.043), and lateral ventriculitis (p = 0.043). In the 6 patients (6/13, 46.2%) with preceding extraventricular drain (EVD) placement, all failed to wean, including 5/6 patients who received high-dose steroids while the EVD was in place. Almost all (19/20, 95.0%) were treated with steroid-sparing agents, including nine (9/20, 45.0%) with tumor necrosis factor (TNF) inhibitors. Modified Rankin Scale score at last outcome was 3.04 (range 0-6).

Conclusion: Patients with neurosarcoidosis and hydrocephalus experience unique challenges in the management of their disease, including the potential need for CSF diversion, in addition to traditional anti-inflammatory treatments. Younger patients, those with obstructive hydrocephalus, and those with lateral ventriculitis warrant particular consideration for VPS placement, but the decision to shunt likely remains a highly individualized one. The requirement for multiple lines of immunotherapy beyond steroids and moderate disability at last follow-up suggest hydrocephalus may reflect a more severe form of neurosarcoidosis.