Coccidioidomycosis of the Central Nervous System: Neuropathological and Vasculopathic Manifestations and Clinical Correlates

Abstract

Infection due to the fungus Coccidioides immitis, which is endemic to the southwestern United States, is currently occurring in epidemic proportions. Although it usually presents as a subclinical infection or a mild, self-limited pulmonary infection, disseminated infection can occur, especially in immunosuppressed patients. In patients with disseminated coccidioidomycosis, CNS spread is commonly responsible for significant morbidity and mortality. The neurological presentations of C. immitis infection of the CNS are variable. The spectrum of neuropathological change ranges from meningitis to meningoencephalitis and meningomyelitis with extensive parenchymal destruction, sometimes as a result of an associated endarteritis obliterans. We describe a patient with AIDS who developed CNS coccidioidomycosis, and we report the findings of a clinicopathological analysis of eight patients with fatal CNS coccidioidomycosis who were autopsied at UCLA Medical Center. CNS coccidioidomycosis should be considered in the differential diagnosis of patients with neurological symptoms, especially when they are immunosuppressed and live in regions where the disease is endemic.

Efficacy of Antigen 2/Proline-Rich Antigen cDNA-Transfected Dendritic Cells in Immunization of Mice against Coccidioides posadasii

“…Once inside the host, the arthroconidia develop into endosporulating spherules that gradually spread systemically. The clinical manifestations of the disease range from a primary benign pulmonary infection to a progressive pulmonary or extrapulmonary disease involving skin, bones, CNS, and other organ systems (3). On the basis of a wide recognition of its potential use in bioterrorism, Coccidioides has been included in the list of Select Agents (4).…”

Coccidioidal meningitis: Update on epidemiology, clinical features, diagnosis, and management

“…CM is typically located in the basilar meninges, but may be a diffuse process, or uncommonly may involve the lateral cerebral ventricles. The pathology consists of suppurative and granulomatous infl ammation [7,8]. The chronic infl ammatory process results in the formation of a thick membrane of infl ammation and fi brous tissue, which may obliterate the subarachnoid space in some areas, or cause obstruction to the fl ow of the cerebrospinal fl uid (CSF), resulting in hydrocephalus and ventricular dilatation [7].…”

“…Systemic symptoms such as fever, sweats, anorexia with weight loss, or generalized weakness may or may not be present [6•,13]. However, symptoms commonly seen with CM include any one or more of the following: persistent headache, nausea, photophobia, neck pain and stiffness, back pain, confusion, decline in cognition or memory, emotional lability, and hearing or visual changes [6•, 8,13]. Because headache is common in patients with primary coccidioidomycosis (without meningitis), this symptom is not specifi c for CM [13].…”

“…Coccidioidal skin lesions located on the face may be associated with concurrent CM [14]. Focal neurologic defi cits or seizures in patients with CM may result from vasculitis or mass lesions [8]. Mass lesions of the central nervous system (CNS) are not common and may be caused by local extension from infected leptomeninges; however, in about one third of cases, they occur in the absence of identifi able meningitis [15].…”

Central nervous system coccidioides immitis infections

“…The most common presentation of CNS disease is subacute or chronic granulomatous meningitis primarily in adults [6][7][8] but occasionally children develop similar meningitis [9]. However, the fungal meningitis lacks specific characteristics that distinguish it from other types of subacute meningitis, such as tuberculous meningitis.…”