Mucor fungal encephalitis: case presentation

Abstract

Mucormycosis is a rare and often fatal infection caused by fungi of the Mucorales order, commonly affecting immunocompromised patients and adults with diabetic ketoacidosis. Infants are infected through the gastrointestinal tract or the nasopharynx, from where infection can spread to the brain. Mucor fungus is angiotropic, causing thrombosis, infarction and tissue necrosis. A case of neonatal death by mucor encephalitis is reported. The mother had gestational diabetes on insulin. The female infant was diagnosed with Fallot tetralogy and was admitted to NICU. There was a progressive clinical deteriorationwith signs of infection, but with repeatedly negative full septic screens including fungi, and negative viral control. The brain showed lytic lesions on imaging. Despite treatment with antibiotics and later with anti-fungal drugs, the baby's condition rapidly worsened and she died on day 34. The post-mortem examination revealed mucor hyphae in the brain causing vasculitis and necrotising encephalitis. In addition to tFallot, there was thymus hypoplasia, suggestive of DiGeorge phenotype. CATCH22 cytogenetic examination was negative. Diagnosis of infection with mucor and related fungi is difficult, as cultures are frequently negative. Direct microscopic visualisation of the fungal hyphae in affected tissues is important for the diagnosis, which is often made with delay or only post-mortem.