Acute Fatal Parainfectious Cerebellar Swelling in Two Children. A Rare or an Overlooked Situation?

Abstract

We report 2 previously healthy children who developed sudden unexpected respiratory arrest and brain death, during a presumed Epstein-Barr meningitis in one case and a multisystemic infection of unknown etiology in the other. Diffuse swelling of the cerebellum with upward transtentorial and downward tonsillar herniation, shown by brain CT-scan and MRI obtained after the acute event, was the most probable cause of death. Review of CT images performed before or at the onset of deterioration already showed discrete signs of early upward herniation of the cerebellar vermis that were initially overlooked. At autopsy in the first case, an acute lymphomonocytic meningoencephalitis with predominant involvement of the cerebellum was observed. Few similar cases were found in the literature, indicating that acute cerebellar swelling is either a very rare or an unrecognized, possibly preventable cause of death in acute inflammatory or non-inflammatory encephalopathies in children.

Abbreviations

CT: Computed tomography
MRI: Magnetic Resonance Imaging
EBV: Epstein-Barr virus
VCA: Viral capsid antigen
CSF: Cerebrospinal fluid

Key words

Acute encephalopathy - Cerebellitis, cerebellar edema