PEHO OR PEHO-LIKE SYNDROME

PEHO syndrome is a rare progressive infantile encephalopathy, with var iable age of onset of hypotonia, convulsions, mental retardation, oede ma, and optic atrophy. Neuroimaging shows cerebellar and brainstem atr ophy in most instances. A PEHO-like syndrome has been described in whi ch those affected do not have the typical changes on neuroimaging. We report four new cases, two isolated cases and two sisters, who might b e part of the PEHO-like syndrome.