A patient with a history of pulmonary paracoccidioidomycosis presented with relapsed disease in the central nervous system four years after initial treatment, according to a case report published in Open Forum Infectious Diseases.
Researchers present the case of a man, aged 49 years, originally from Brazil and initially diagnosed with paracoccidioidomycosis in 2011. In the fall of 2017, he presented to an outside hospital with 3-week history of headache, dizziness, and ataxia. His treatment prior to presentation consisted of itraconazole for 2 years starting in 2011, and a right lower lobe wedge resection in 2013. Physical examination on presentation demonstrated bradycardia, dysmetria and right-sided gait instability. His history was also notable for moving to the United States at age 29 years, but reported no international travel after immigrating. However, the patient reported former employment at sugarcane and coffee plantations, a history of heavy alcohol consumption and a 40 pack per year smoking history.
Brain magnetic resonance imaging revealed a 5.0× 3.5× 3.0-cm multilocular, diffusion-restricting cystic mass in the right cerebellum with vasogenic edema and hydrocephaly. The patient underwent a right-sided posterior fossa craniotomy with subtotal resection
of the cerebellar mass. Intraoperative frozen section pathology identified yeast forms suspicious for Paracoccidioides brasiliensis; this was confirmed via methenamine silver staining of permanent sections and fungal cultures, which grew P brasiliensis after 21 days. The postoperative treatment course was an intended 4 week treatment with liposomal amphotericin B (5mg/kg per day) with normal saline prehydration, however acute renal injury required a change to trimethoprim/sulfamethoxazole after 2 weeks. The patient was discharged 22 days after the surgery with a prescription for oral trimethoprim/sulfamethoxazole; 1 month post-discharge, he reported resolution of his presenting symptoms.
Paracoccidioidomycosis is a dimorphic fungal infection endemic to Latin America. This fungal disease is contracted by inhaling conidia in soil and risk factors include agricultural work, male sex, smoking, and alcohol use disorder. Diagnosis is performed via serology, microscopy, and/or culture. Most patients develop asymptomatic pulmonary infections that may reactivate months or years later resulting in chronic disease in oral mucosa, skin and adrenals. In 9% to 25% of cases, paracoccidioidomycosis disseminates to the central nervous system (CNS) and this neuroparacoccidioidomycosis most frequently localizes to the cerebral hemispheres (67%), cerebellum (25%), brain stem (25%), and spinal cord (4%).
Neuroparacoccidioidomycosis has a mortality rate of 44% and 50% of survivors develop long-term neurologic sequelae. Coinfection with HIV and other factors such as solid organ transplantation, active malignancies, or on biologic therapy all confer greater risk for disseminated disease, relapse and mortality.
According to the investigators, “the present case is remarkable for the development of relapsed CNS disease 4 years after antibiotic treatment and resection of affected lung in an immunocompetent patient with no repeated exposures.” There are no current treatment guidelines available from US professional or government bodies and while paracoccidioidomycosis is rare in the United States, practitioners should consider it in immigrants from endemic areas. Furthermore, past treatment does not preclude relapse and a significant fraction of cases can involve the CNS.
Rahman R, Davies L, Mohareb AM, et al. Delayed relapse of Paracoccidioidomycosis in the central nervous system: A case report. Open Forum Infect Dis. 2020;7:ofaa077.