Cryptococcal Meningitis as the Initial Presentation of Sarcoidosis in an Immunocompetent Patient
Objective:
To discuss a case of sarcoidosis complicated by cryptococcal meningitis presenting with intermittent headaches.
Background:
A 48-year-old man with diabetes and hypertension presented to outpatient neurology clinic with six weeks of intermittent headaches associated with transient visual obscurations and a fifteen-pound weight loss. He had presented to the emergency room twice before, was treated for migraine and discharged without neuroimaging. Initial exam was notable for bilateral disc edema and cotton wool spots. Brain MRI revealed foci of T2 hyperintensity in the right caudate body without enhancement or diffusion restriction. MRA head and neck and MRV showed areas of decreased flow in the cavernous internal carotid arteries and posterior cerebral arteries and decreased flow in the left transverse and bilateral sigmoid sinuses. Cerebrospinal fluid testing showed normal opening pressure (17), pleocytosis of 120 cells/mm3 (61% neutrophils), elevated protein (161 mg/dL), hypoglycorrhachia (31 mg/dL, serum glucose 103 mg/dL), elevated ACE (4.1 U/L), and positive cryptococcal antigen. Serum studies were notable for elevated angiotensin converting enzyme level (ACE, 99 U/L), HIV nonreactive, CD4 count 698. He was admitted to the hospital for initiation of liposomal amphotericin B and flucytosine for two weeks followed by fluconazole for 8 weeks and is currently on fluconazole maintenance therapy, with complete resolution of headaches and neurologic symptoms. CT chest revealed extensive foci of lymphatic nodularity with hilar and mediastinal lymphadenopathy. Bronchoscopy showed non-necrotizing granulomas in his right lower lobe, confirming concurrent sarcoidosis.
Conclusions:
Cryptococcal meningitis is a complication of sarcoidosis independent of immunosuppressive therapy and even in the absence of CD4 lymphocytopenia. Qualitative CD4 dysfunction and impaired phagocytic activity have been previously implicated. Neurosarcoidosis and CNS cryptococcosis share similar CSF profiles which contributes to this diagnostic challenge and underscores the importance of maintaining a high clinical suspicion.
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