title: Intractable seizures from infancy can be associated with dentato-olivary dysplasia.
abstract: Five children with severe developmental delay had intractable fits of various types but tonic, often extensor, seizures were prominent from an early stage. Onset was in the neonatal period in 4 cases. EEGs were severely abnormal and showed a "burst-suppression" pattern in the first months of life. There were no metabolic or consistent neuroradiological abnormalities. A distinctive form of dentato-olivary dysplasia was found in all cases. Inferior olives were hook-shaped, coarse and lacking undulations, while dentate nuclei showed a compact arrangement of interconnected islands. The clinico-pathological findings form a novel nosological entity.
authors.affiliation: Department of Neuropathology, Hospital for Sick Children, London, U.K.
authors.forename: B N
authors.initials: BN
authors.lastname: Harding
authors.forename: S G
authors.initials: SG
authors.lastname: Boyd
mesh_terms: D001034:Apgar Score; D001724:Birth Weight; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000473:pathology / Q000503:physiopathology*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008526:Medulla Oblongata / Q000473:pathology; D009847:Olivary Nucleus / Q000473:pathology; D012249:Rhombencephalon / Q000473:pathology / Q000503:physiopathology*; D012640:Seizures / Q000473:pathology / Q000503:physiopathology*