Objectives Neurosarcoidosis is a rare condition with a high mortality. Early recognition of symptoms is important to enable prompt interventions. We report a case series of NS with ventriculomegaly that presented with symptoms resembling normal pressure hydrocephalus (NPH).

Design Case series.

Subjects Patients with ventriculomegaly on MR imaging and diagnosis of confirmed or probable NS.

Methods Analysis of medical records for presenting clinical features, neuroradiology, intervention and clinical outcomes in patients with ventriculomegaly and confirmed or probable NS.

Results Four patients (2M:2F) aged 49.0±3.01 years (mean ±SD) were identified. Three had definite NS and one probable. Three presented with gait disturbance and one with memory impairment. MR Imaging (with gadolinium) demonstrated ventriculomegaly with leptomeningeal enhancement. One patient underwent 24 hour ICPM, with a median ICP of 3.47 mmHg and pulse amplitude of 4.35 mmHg. CSF showed mild increase in chronic inflammatory cells in the absence of infection. Patients underwent medical management plus ventriculoperionteal shunt insertion (with adjustable valves set to 5 cm H₂O and anti-siphon devices). There was a propensity for transient development of slit-like ventricles but without other features of over-drainage. All patients showed symptom improvement following CSF diversion at mean follow-up of 27 months.

Conclusions NS can present similarly to NPH. Early combined treatment of CSF diversion and medical management is effective for symptom management.