CC BY-NC-ND 4.0 · J Neurol Surg Rep 2019; 80(03): e31-e35
DOI: 10.1055/s-0039-1687886
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Invasive Cryptococcal Meningitis Presenting as a Skull Base Mass in An Immunocompetent Host: A Case Report

Joshua Prickett
1  Department of Skull Base and Minimally Invasive Neurosurgery, Swedish Neuroscience Institute, Seattle, Washington, United States
,
Juan Altafulla
1  Department of Skull Base and Minimally Invasive Neurosurgery, Swedish Neuroscience Institute, Seattle, Washington, United States
2  Department of Neuroanatomy, Seattle Science Foundation, Seattle, Washington, United States
3  Department of Neurological Surgery, Hospital Santo Tomas, Panama, Panama
,
Anna Knisely
1  Department of Skull Base and Minimally Invasive Neurosurgery, Swedish Neuroscience Institute, Seattle, Washington, United States
,
Zachary Litvack
1  Department of Skull Base and Minimally Invasive Neurosurgery, Swedish Neuroscience Institute, Seattle, Washington, United States
› Author Affiliations
Funding Sources This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Further Information

Publication History

14 November 2018

18 January 2019

Publication Date:
18 July 2019 (online)

  

Abstract

Introduction Encephalitis due to Cryptococcus neoformans has been seen almost exclusively in patients with severe compromise of their immune systems, such as acquired immune deficiency syndrome (AIDS). Fungal sinusitis with frank invasion through the cranial base and subsequent seeding of the central nervous system is rare, but should be considered in the differential of patients presenting with meningitis and sinus/skull base lesions even without obvious immune compromise. Improvements in diagnostic testing has increased the ability to correctly identify and new antimicrobials have allowed a condition that once carried a high morbidity and mortality to be managed with better outcomes. We present our treatment algorithm for successful management of an immunocompetent patient with extensive fungal encephalitis due to erosion through the skull base.

Case Description The patient is a 59-year-old male presenting unresponsive with sphenoid mass erosive of the skull base and symptoms of meningitis and encephalitis due to C. neoformans. Magnetic resonance imaging (MRI) at presentation demonstrated extensive diencephalic invasion, and a sphenoid mass with erosion of the skull base. Lumbar puncture (LP) confirmed elevated opening pressure of 45 cm H2O, and cultures confirmed infection with C. neoformans. He underwent operative sinonasal debridement followed by placement of an external ventricular drain for management of hydrocephalus. He was treated aggressively with a combination of both intravenous (IV) amphotericin B daily and intrathecal amphotericin B via the ventriculostomy thrice weekly. By the 2nd week of treatment, patient regained consciousness. After 4 weeks of therapy, cerebrospinal fluid (CSF) cultures turned negative, and the external ventricular drain (EVD) was converted to a ventriculoperitoneal shunt (VPS) to manage chronic postinfectious hydrocephalus. We also placed a contralateral Ommaya reservoir to permit continued weekly intrathecal amphotericin B without violation of the shunt valve. With each instillation, the shunt was set to its highest setting to minimize CSF egress for 6 hours then reset to its “drainage” setting. After an additional 6 weeks of outpatient therapy, intrathecal therapy was discontinued. We continued CSF surveillance via Ommaya sampling monthly. At 9-month follow-up, he has remained clinically stable without evidence of recurrent infection. He has residual mild cognitive deficits, but is living semiindependently with his brother.

Conclusions Fungal sinusitis is uncommon, especially in those without significantly compromised immune systems. Invasive fungal meningitis resulting in meningitis and encephalitis is even rarer. The condition carries high morbidity and mortality that can only be mitigated with a multidisciplinary effort by neurosurgery, otolaryngology, and infectious disease specialists. While there are no clear treatment guidelines, we present an approach that may permit longer term independent survival.