Neuropsychological profile of children with subcortical band heterotopia.
OBJECTIVE
Subcortical band heterotopia (SBH) or 'double cortex' is a malformation of cortical development resulting from impaired neuronal migration. So far, research has focused on the neurological, neuroimaging, and genetic correlates of SBH. More recently, clinical reports and small sample studies have documented neuropsychological dysfunction in patients with this malformation. This study aimed to characterize further the phenotype of patients with SBH by describing the neuropsychological profiles of children.


METHODS
Seven children (six females) aged 4 to 15 years were assessed for cognitive functioning (intellectual ability, processing speed, attention, working memory) and academic achievement (reading, spelling, arithmetic). Parents completed questionnaires examining their child's social skills and problem behaviours. Magnetic resonance images (MRI) conducted for routine clinical follow-up were coded by a paediatric neurologist. Genetic and seizure history were obtained from medical records.


RESULTS
There was variation in the neurological, neuroimaging, and genetic presentation of children in the sample. Impairments were observed in all areas of neuropsychological functioning examined. Intellectual ability was generally within the 'extremely low' range (full-scale IQ 44-74; performance IQ 45-72; verbal IQ 57-80). Generalized impairments in cognitive skills were typical, with severe impairments (scores greater than 2SD below the test mean) reported in processing speed, working memory, and arithmetic. Impairments in academic, social, and behavioural functioning were less generalized. No clear relationship between neuroimaging and neuropsychological impairments was found.


CONCLUSIONS
Children with SBH demonstrate cognitive, academic, social, and behavioural problems, with the greatest difficulties in processing speed and complex cognitive skills.
Spencer-Smith
Megan
M
Murdoch Childrens Research Institute, Melbourne, Australia. megan.spencer-smith@mcri.edu.au
Leventer
Richard
R
Jacobs
Rani
R
Luca
Cinzia D E
CD
Anderson
Vicki
V
D000293:Adolescent; D001288:Attention / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D054221:Classical Lissencephalies and Subcortical Band Heterotopias / Q000473:pathology / Q000503:physiopathology* / Q000523:psychology*; D003071:Cognition / Q000502:physiology*; D015331:Cohort Studies; D004522:Educational Status; D005260:Female; D006801:Humans; D007360:Intelligence / Q000502:physiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009043:Motor Activity / Q000502:physiology*; D009483:Neuropsychological Tests; D012189:Retrospective Studies