Cerebral phaeohyphomycosis: Case report

Abstract

Cerebral phaeohyphomycosis is a rare and frequently fatal disease. This disease is often caused by hematogenous spread of pathogens that are inoculated in the skin of the extremities after slight or minor trauma, and its mortality rate is rather high despite aggressive treatment. Our patient presented with headache and pyrexia. She was diagnosed with fungal meningitis and treated by systemic administration of voriconazole (VRCZ). However, after initial improvement, meningitis recurred. MRI of the brain showed multiple small masses in the cerebral hemisphere and she was thus referred to our Department of Neurosurgery. On admission, an examination showed that the masses were deeply located in the brain and were too small to be excised; therefore, treatment with systemic VRCZ and intrathecal amphotericin B was initially selected. However, the intracerebral masses continued to grow; therefore, they were surgically excised. Histological examination of the surgical specimens at that time identified the masses as granuloma caused by infection with Aspergillus niger. After the surgery, her general condition improved; therefore treatment with systemic and intrathecal antifungal agents were continued. However, the intracerebral masses recurred, and despite further aggressive surgical treatment and systemic and intrathecal antifungal administration, she died 43 months after the initial diagnosis. Autopsy examination showed that the cerebral lesions were phaeohyphomycotic granulomas. This paper describes the clinical presentation, histopathological results and treatment for this rare disease.

Feohifomicosis, una infección fúngica oportunista emergente

“…En casos cerebrales se dice que los hongos penetran por vía respiratoria y se diseminan vía hematógena, se inhalan las conidias que originan un cuadro pulmonar, casi siempre asintomático, y a partir de este foco por neurotropismo se disemina al cerebro (17), también pueden ser secundarios a inoculación traumática en piel con posterior diseminación (18).…”

“…Generalmente secundaria a diseminación hematógena (2,17,18). Más frecuente en inmunosuprimidos, raro en inmunocompetentes (17).…”

“…Más frecuente en inmunosuprimidos, raro en inmunocompetentes (17). Lo más común es que se manifieste como absceso cerebral único, también se ha descrito meningitis, encefalitis, mielietis o aracnoiditis (17,18,28).…”

“…Los abscesos cerebrales, visualizados radiológicamente como una masa encapsulada (17,18), tienen tejido de granulación, acompañado de fibrosis, linfocitos, neutrófilos y células gigantes; pueden verse, además, elementos fúngicos dispersos o dentro de células multinucleadas gigantes (2,12).…”

“…El voriconazol puede ser superior para infecciones del sistema nervioso central debido a su capacidad para lograr buenas concentraciones en el líquido cefalorraquídeo (17,18,34). El posaconazol es alternativo, bien tolerado, con menos experiencia clínica, pero con excelentes resultados como tratamiento de rescate tras el fracaso con otros antifúngicos (33).…”