Rhombencephalitis, a forgotten diagnosis in the post-partum period: a case report

Rhombencephalitis (RE) or brainstem encephalitis is a rare but potentially deadly condition. It often affects hosts with weaker or altered immune response. A previously healthy 30-year-old female, three months post-partum, presented with a ten-day history of headache, vomiting, seizures and consciousness deterioration. MRI showed suspect brainstem glioma and hydrocephalus. Initial treatment aimed to reduce high intracranial pressure with an external ventricular drain and high-dose steroids. Despite negative blood cultures, CSF sampling was overlooked as RE was not suspected. The condition proved fatal four days later. Post-mortem histology revealed the RE as the cause of death, but PCR did not show an infectious ethology. With patients in the post-partum period with progressive neurological deterioration and FLAIR2-changes in brainstem MRI, RE should be considered early on.