title: Pontoneocerebellar hypoplasia: definition of MR features.
abstract: We report five patients with pontoneocerebellar hypoplasia. Because the issue of cerebellar malformations is a difficult subject, we tried to define criteria for diagnosis on MRI: a thin flat pons with disappearance of the anterior curve, a small cerebellum with predominant flattening of the hemispheres and shortened cerebellar fissures, in contrast to atrophy. The posterior fossa is not enlarged. We emphasize the probable late onset of the disease in fetal life because of the demonstration of the abnormalities at US during the last trimester of the pregnancy in one patient. Prenatal diagnosis is important because of possible autosomal recessive transmission.
authors.affiliation: Service de Radiologie, Hôpital St Vincent de Paul, 82 av Denfert Rochereau, 75674 Paris Cedex 14, France. c.adamsbaum@svp.ap-hop-paris.fr
authors.forename: P
authors.initials: P
authors.lastname: Goasdoué
authors.forename: D
authors.initials: D
authors.lastname: Rodriguez
authors.forename: M L
authors.initials: ML
authors.lastname: Moutard
authors.forename: O
authors.initials: O
authors.lastname: Robain
authors.forename: G
authors.initials: G
authors.lastname: Lalande
authors.forename: C
authors.initials: C
authors.lastname: Adamsbaum
mesh_terms: D002531:Cerebellum / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011247:Pregnancy; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal