Case Presentation:
A 50 year old African-American male presented with 2 weeks of headaches, neck pain, and photophobia. Exam revealed positive meningeal signs and a fever of 101oF. CT head and MRI showed small cerebellar infarcts. Lumbar puncture was done and empiric acyclovir, vancomycin, and ceftriaxone begun. After the LP, the headache improved. Cerebrospinal fluid showed a total nucleated cell count of 90cells/uL, 60% segmental neutrophils, glucose of 16mg/dL and total protein of 94.8mg/dL. Gram stain and India ink were positive for cryptococcus and amphotericin B and flucytosine were started. The course was complicated by acute tubular necrosis due to amphotericin B prompting a change to fluconazole. Renal function improved, but he developed anterior uveitis and papilledema. Repeat LP had an opening pressure of 45mmHg, and his need for therapeutic LPs became more frequent. Acetazolamide was started and amphotericin B resumed.
Once cryptococcal meningitis was diagnosed an extensive workup for immunocompromisation was done. HIV ELISA and quantiferon gold were negative, but t-cell subsets showed a CD4 count of 120 and a CD8 of 126. A CT was negative for malignancy, but showed hilar lymphadenopathy. After an exhaustive workup, records from outside facilities were obtained which showed biopsy results of sarcoidosis. The patient never followed up and had never been on immunosupppresives. He was started on high dose prednisone but developed a herpes zoster rash requiring acyclovir. The patient improved and was discharged to rehab.
Discussion:
Cryptococcus neoformans is an encapsulated yeast that causes a variety of infections in an immunocompromised host, classically HIV positive patients with CD4 <100cells/uL(1). Several case reports describe cryptococcus in patients who are HIV negative but have other immunocompromised states, i.e. steroid use, transplants, leukemia, lymphoma and sarcoidosis (2). Sarcoidosis is a granulomatous disease that impairs cell-mediated immunity due to sequestration of CD4 in granulomas. These patients have a low CD4 with decreased CD4/8 ratios and are typically on chronic corticosteroids, both of which are culprits in predisposition to cryptococcus (3, 4). The degree of CD4 lymphopenia may correlate with overall disease severity (5). Cryptococcal meningitis in patients with sarcoidosis on steroids is well described, but few cases have been reported in those naïve to immunosuppressive therapy, as was the case with our patient. We found only four such reports (3).
Conclusions:
This case highlights that sarcoidosis is an independent risk factor for opportunistic infections diagnosed in hospitalized patients and illustrates the importance of measuring an opening pressure when performing an LPs. Knowledge of the elevated pressure would have suggested a fungal cause in our patient whom at time of diagnosis had no obvious source of immunosuppression.