Objective:

To demonstrate a complex case of post-infectious central nervous system (CNS) vasculitis.

Background:

Cryptococcus meningitis is known to affect immunocompromised individuals. However, it can be seen in immunocompetent individuals as well, and chronic infection can result in complications such as ventriculitis and central nervous system (CNS) infectious vasculitis. Symptoms of these conditions are frequently non-specific like prolonged encephalopathy and headaches.

Design/Methods:

A 50-year-old woman presented to the emergency department with confusion and new-onset fevers after being diagnosed with cryptococcus meningitis months prior. She was previously treated with amphotericin B and fluconazole. She was an immunocompetent host with known environmental exposure history to chickens, soil, and bird droppings. The patient underwent ventriculoperitoneal (VP) shunt placement due to concerns for ventriculitis. Exam was significant for new-onset left cranial nerve III palsy, right-sided weakness, and intermittent tremulous movements in the bilateral upper extremities.

Results:

MRI brain showed new restricted diffusion in the midbrain indicating an acute infarct and concerns for enlarged ventricles, and CTA was concerning for cerebral vasculitis. Cerebral angiogram revealed multifocal segmental wall irregularities involving the anterior cerebral artery and narrowing of the bilateral posterior cerebral arteries. The patient completed a prolonged course of steroids and continued oral fluconazole. After this, the patient had great improvement in her overall functioning and memory and could hold fluent conversation, move all extremities, and perform some activities of daily living that she previously was unable to do.

Conclusions:

This case highlights an interesting combination of likely ventriculomegaly and post-infectious CNS vasculitis. Due to persistent encephalopathy, there was a concern for progressing hydrocephalus. However, the patient’s condition had significantly improved after completion of a prolonged steroid taper. The exact dosing for a steroid taper in post-infectious CNS vasculitis often is unclear and requires close monitoring for clinical changes and adequate collateral history from family to help with dose adjustments.