Hydrocephalus with panventricular enlargement as the primary manifestation of neurosarcoidosis: a case report

Hydrocephalus is very uncommon in neurosarcoidosis. To date, there have been only five reported cases of hydrocephalus occurring as the first manifestation of neurosarcoidosis. Such a presentation in a previously healthy patient is challenging to diagnose.

A 31-year-old Japanese man who had no relevant past history other than sinusitis was admitted to our institution complaining of low-grade fever and mild headache. He was alert and neurologically intact. No respiratory symptoms were observed. Laboratory examination revealed mild elevation of erythrocyte sedimentation rate and serum CD4/CD8 ratio. Serum angiotensin-converting enzyme level was in the normal range. His cerebrospinal fluid showed mild pleocytosis and increased protein level. A chest X-ray revealed bihilar lymphadenopathy with normal lung parenchyma. Computed tomography of his head showed remarkable hydrocephalus with dilatation of all ventricles, particularly the fourth. Gadolinium-enhanced magnetic resonance imaging demonstrated leptomeningeal millet seed-like enhancement and multiple small enhancing lesions along the Virchow–Robin spaces. These findings strongly suggested a chronic inflammatory disease such as neurosarcoidosis. To treat the hydrocephalus, a ventriculoperitoneal shunt was inserted. The postoperative course was satisfactory. After surgery, nasal and skin biopsies were performed and pathological analysis revealed non-caseating granulomas consistent with sarcoidosis. The findings of gallium scintigraphy also supported the diagnosis of sarcoidosis. We obtained the definitive diagnosis of sarcoidosis 3 weeks after admission from the pathological findings by the nasal and skin biopsies, and corticosteroid therapy was started after that.

We present a rare case of neurosarcoidosis manifesting as acute hydrocephalus with dilatation of all ventricles, particularly the fourth. As hydrocephalus due to neurosarcoidosis has high morbidity and mortality, early diagnosis and proper treatment are particularly important.