Coccidioidomycosis: neurologic manifestations

Sandeep Khot MD (

Dr. Khot of the University of Washington has no relevant financial relationships to disclose.

)
Amita Singh MD (

Dr. Singh of the University of Washington has no relevant financial relationships to disclose.

)
Christina M Marra MD, editor. (

Dr. Marra of the University of Washington School of Medicine owned stock in Johnson & Johnson and McKesson within the past 12 months.

)
Originally released February 28, 2005; last updated December 30, 2019; expires December 30, 2022

Overview

Coccidioidomycosis is a common fungal infection in the southwestern United States of America and other Central and South American nations. Only a few patients who acquire primary coccidioidomycosis develop neurologic complications. Importantly, these patients are identified by their travel through endemic areas and often present elsewhere. In this article, the authors review the clinical manifestations, diagnostic approach, and management of neurologic manifestations of coccidioidomycosis.

Historical note and terminology

The first prehistoric human infection with Coccidioides immitis was reported in an ancient Indian skeleton from Arizona (Harrison et al 1991). The skeleton resembled a 40- to 50-year-old man who belonged to the Sinagua culture of the American Southwest, dating to 1000 to 1400 AD.

A medical intern and his teacher reported in 1892 the first modern case of coccidioidomycosis in an Argentine soldier. They described progressive, verrucous skin lesions of the face that resembled mycosis fungoides and contained spherical nonmotile organisms with a highly refractile double wall (Posadas 1892). Four years later, the same organism was isolated from similar papulonecrotic skin lesions in 2 immigrants to California (Rixford and Gilchrist 1896). The organism was initially identified as a protozoan and was called Coccidioides because it resembled a coccidian. The current term Coccidioides immitis was coined when immitis (not mild) was added to the name to indicate its virulence (Rixford and Gilchrist 1896). In 1900, Ophuls recognized the true nature of the organism as a dimorphic fungus and not a protozoan (Ophuls and Moffitt 1900). Five years later, he described the first case of coccidioidal meningitis in an autopsy of a patient with widely disseminated disease (Ophuls 1905). The first case of meningitis as the sole site of extrapulmonary involvement was reported in 1924 (Morris 1924). An autopsy series of 14 patients was published in 1936 that included CSF findings in coccidioidal meningitis (Abbott and Cutler 1936). In the following year, the cause of “Valley fever” in California's San Joaquin Valley was confirmed as coccidioidomycosis (Dickson 1937). The epidemiological scale of this disease in the San Joaquin Valley was revealed in 1946 by using a newly developed skin test and serological antigen (coccidioidin) (Smith et al 1946). In 2002, Coccidioides posadassi, formerly known as non-Californian Coccidioides immitis, was isolated as a distinct species with numerous varying DNA polymorphisms, but clinically both species present with the same spectrum of disease (Fisher et al 2002).

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