ACUTE FATAL PARAINFECTIOUS CEREBELLAR SWELLING IN 2 CHILDREN - A RAREOR AN OVERLOOKED SITUATION

We report 2 previously healthy children who developed sudden unexpecte d respiratory arrest and brain death, during a presumed Epstein-Barr m eningitis in one case and a multisystemic infection of unknown etiolog y in the other. Diffuse swelling of the cerebellum with upward transte ntorial and downward tonsillar herniation, shown by brain CT-scan and MRI obtained after the acute event, was the most probable cause of dea th. Review of CT images performed before or at the onset of deteriorat ion already showed discrete signs of early upward herniation of the ce rebellar vermis that were initially overlooked. At autopsy in the firs t case, an acute lymphomonocytic meningoencephalitis with predominant involvement of the cerebellum was observed. Few similar cases were fou nd in the literature, indicating that acute cerebellar swelling is eit her a very rare or an unrecognized, possibly preventable cause of deat h in acute inflammatory or non-inflammatory encephalopathies in childr en.