title: Supratentorial functional disturbances in two children with cerebellar cortical dysplasia.
abstract: When evaluating children with mental retardation, subtle cerebral and cerebellar morphologic anomalies are often noted at Magnetic Resonance Imaging (MRI). Some, such as cerebellar cortical dysplasia (CCD), have been considered as subtle markers of cerebral dysgenesis. Their functional significance and their effect on brain function, remain unknown. To study supratentorial functional disturbances related to CCD we performed Positron-Emission-Tomography (PET) studies in two children with isolated CCD, in order to investigate the degree of involvement of supratentorial structures. One had developmental delay, motor disturbances and ataxia, and the other one only had mental retardation. PET studies revealed hypoperfusion and hypometabolism within the vermis, thalamus and the right striatum in one case, and hypometabolism in the basal ganglia and cerebellar deep grey nuclei in the other case. Our results could lead to a hypothesis explaining motor disturbances as well as cognitive impairment, and could suggest a pathological functional significance of CCD. Nevertheless, the relationship between these findings and mental retardation needs further investigation.
authors.affiliation: Clinique de Neuroradiologie, Hôpital Erasme, Brussels, Belgium. jissendi@netscape.net
authors.forename: P
authors.initials: P
authors.lastname: Jissendi Tchofo
authors.forename: X
authors.initials: X
authors.lastname: De Tiege
authors.forename: S
authors.initials: S
authors.lastname: Goldman
authors.forename: P
authors.initials: P
authors.lastname: Van Bogaert
authors.forename: P H
authors.initials: PH
authors.lastname: David
authors.forename: J P
authors.initials: JP
authors.lastname: Pruvo
authors.forename: G
authors.initials: G
authors.lastname: Soto Ares
mesh_terms: D001786:Blood Glucose / Q000378:metabolism*; D001921:Brain / Q000002:abnormalities* / Q000098:blood supply / Q000000981:diagnostic imaging; D002525:Cerebellar Cortex / Q000002:abnormalities* / Q000000981:diagnostic imaging; D002526:Cerebellar Diseases / Q000000981:diagnostic imaging*; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000098:blood supply / Q000000981:diagnostic imaging; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000000981:diagnostic imaging*; D005260:Female; D019788:Fluorodeoxyglucose F18; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000000981:diagnostic imaging*; D008607:Intellectual Disability / Q000000981:diagnostic imaging*; D008279:Magnetic Resonance Imaging; D011596:Psychomotor Disorders / Q000000981:diagnostic imaging*; D011877:Radionuclide Imaging; D012016:Reference Values; D012039:Regional Blood Flow / Q000502:physiology
chemical_list: D001786:Blood Glucose; D019788:Fluorodeoxyglucose F18