Coccidioides Central Nervous System Infections

Coccidioidomycosis or Valley Fever is caused by the molds (fungi) Coccidioides immitis or posadasii.  Valley fever can be spread in the air, by skin/organ contact, or by sharp object penetration of the skin.  Valley fever varies in severity depending upon size of the infection and health of the person.  Valley fever infection can be asymptomatic, can cause flu-like symptoms such as fever, cough, fatigue, and muscle aches, and can be fatal in severe cases.  Coccidioides infections occur mostly in regions where the mold is present in the soil including Southern California, Arizona, New Mexico, western Texas, Northern Mexico, and arid regions of South America (1). About 150,000 cases of Valley Fever occur annually in Arizona alone and about 100,000 more cases occur annually in California and other states (1).

A particularly badly form of Coccioidies involves infection of the central nervous system (CNS) (2). It often affects young men, with one study of 31 patients with Coccioidies CNS involvement reporting 27 were men with an average age of 41 years (3). Coccioidies can cause spherule bodies in the brain tissue, meniges of the brain or spinal cord (2).

The course of coccidioidal meningitis is usually insidious and progressive.  The patient usually presents with persistent headaches  (2, 3). Later cognitive dysfunctions such as confusion and emotional lability develop  (2, 3). Gait disturbances, neck stiffness, double vision, disorientation, lethargy, stupor develop.  Advanced cases of coccidioidal meningitis are often fatal (2). In half to two thirds of coccidioidal meningitis cases the infection spreads to the lungs and other sites (2).

The serum coccidioidal antibody test is very useful for diagnosing Coccidioides infections (2). Fungal blood culture is positive in less than one third of cases.  Brain and spinal cord neuroimaging such as MRI are often very useful as well (2). A variety of antifungal drugs can be helpful including oral fluconazole or intrathecal amphotericin B (2).

References

1. Johnson L GE, Sanchez J, Bui P, Nobile C, Hoyer K, Peterson M, Ojcius D. Valley Fever: Danger Lurking in a Dust Cloud. Microbes and Infection. 2014;16(8):591-600.
2. Jackson NR, Blair JE, Ampel NM. Central Nervous System Infections Due to Coccidioidomycosis. Journal of fungi (Basel, Switzerland). 2019;5(3).
3. Bouza E, Dreyer JS, Hewitt WL, Meyer RD. Coccidioidal meningitis. An analysis of thirty-one cases and review of the literature. Medicine (Baltimore). 1981;60(3):139-72.