Objective:

To present a rare case of cryptococcal meningitis (CM) in a young male with hyper-IgE syndrome (HIES), manifesting primarily with stroke-like symptoms.

Background:

Cryptococcal meningitis (CM), caused by Cryptococcus neoformans, is the most prevalent fungal meningitis, particularly in individuals with impaired immunity. The diverse clinical manifestations of CM and clinical course from exposure to symptom onset present significant diagnostic challenges. Diagnosis is typically confirmed through cerebrospinal fluid analysis and Cryptococcal antigen detection, while brain imaging (CT or MRI) aids in excluding alternative infections or identifying cerebrovascular issues.

Design/Methods:

NA

Results:

A 32-year-old male with a history of HIES and recurrent fungal infections presented after a new-onset seizure. Symptoms included headache, photophobia, fever, nausea, and neck stiffness. Initial labs revealed leukocytosis and mild electrolyte abnormalities. CT head and EEG were unremarkable. Brain MRI showed right lacunar infarct and evolving left lacunar infarct. The patient had no traditional stroke risk factors; a hypercoagulable workup was initiated. He was started on aspirin and later therapeutic anticoagulation after repeat MRI revealed bilateral infarcts. During hospitalization, he had another seizure and was intubated for airway protection. A lumbar puncture, initially declined, later revealed normal glucose/protein but positive fungal cultures for Cryptococcus. Antifungal therapy with amphotericin B and flucytosine was started. The patient was transferred to a tertiary center for further management.

Conclusions:

This case highlights the diagnostic challenges of CM with non-specific symptoms and normal early imaging. In patients with primary immunodeficiencies like HIES, CM may present with cerebrovascular manifestations. Stroke should prompt consideration of infectious etiologies, particularly in the absence of common vascular risk factors. Early recognition and antifungal treatment are crucial to prevent neurologic sequelae. Greater awareness and reporting of such cases may help guide future diagnostic and treatment strategies.