Abstract

Coccidioidomycosis is a common infection in the western and southwestern United States as well as parts of Mexico and Central and South America and is due to the soil-dwelling fungi Coccidioides. Central nervous system (CNS) infection is an uncommon manifestation that is nearly always fatal if untreated. The presentation is subtle, commonly with headache and decreased mentation. The diagnosis should be considered in patients with these symptoms in association with a positive serum coccidioidal antibody test. The diagnosis can only be established by analysis of cerebrospinal fluid (CSF), which typically demonstrates a lymphocytic pleocytosis, hypoglycorrhachia, elevated protein, and positive CSF coccidioidal antibody. Cultures are infrequently positive but a proprietary coccidioidal antigen test has reasonable sensitivity. Current therapy usually begins with fluconazole at 800 mg daily but other triazole antifungals also have efficacy and are often used if fluconazole fails. Triazole therapy should be lifelong. Intrathecal amphotericin B, the original treatment, is now reserved for those in whom triazoles have failed. There are several distinct complications of CNS coccidioidal infection, the most common of which is hydrocephalus. This is nearly always communicating and requires mechanical shunting in addition to antifungal therapy. Other complications include cerebral vasculitis, brain abscess, and arachnoiditis. Management of these is difficult and not well established.

Highlights

  • Coccidioidomycosis is an infection caused by the soil-dwelling fungi Coccidioides

  • We review the medical literature and offer our approach regarding the diagnosis and management of various forms of central nervous system (CNS) coccidioidomycosis

  • Over 95% of cerebrospinal fluid (CSF) samples are positive for CF antibody in cases of coccidioidal meningitis [17,18]

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Summary

Introduction

Coccidioidomycosis is an infection caused by the soil-dwelling fungi Coccidioides. It is endemic to areas of the southwestern United States, the San Joaquin Valley of California and the south-central region of Arizona, as well as northern Mexico, and parts of Central and South America. Central nervous system (CNS) infections are among the most devasting manifestations of coccidioidomycosis. We review the medical literature and offer our approach regarding the diagnosis and management of various forms of CNS coccidioidomycosis. We spend most of the time on coccidioidal meningitis since it is the most common manifestation of CNS involvement

Epidemiology and Risk Factors
Pathogenesis
Clinical Presentation
Diagnosis
Treatment
Complications
Findings
Summary

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