Clinicopathological report of cerebroocular myopathy yndrome

PURPOE: To report a cae of cerebroocular myopathy yndrome with a focal abence of the Decemet membrane. DEIGN: Clinicopathological cae report. METHOD: A clinical and hitopathologic examination of eye, brain, and vicera of a white male newborn of normal term, who died at 11 day of age with the diagnoi of cerebroocular myopathy yndrome. REULT: The autopy revealed hazy left cornea, hypoplaia of the cerebellum, and corpu colluum with mild microcephaly. Microcopy of the left eye howed the additional finding of a central focal defect of the Decemet membrane with an abence of the poterior third of central corneal troma and delicate fibrou trand connected to the iri. CONCLUION: The abence of the Decemet membrane and the miing poterior corneal keratocyte a well a the fibrou trand connecting the iri are finding to be added to the previouly reported ign of cerebro-ocular myopathy.