Neuropsychological profile of a 9-year-old child with subcortical band heterotopia or 'double cortex'
R Jacobs1, V Anderson, A S Harvey
1Department of Psychology, Murdoch Children's Research Institute, Melbourne, Australia. jacobsr@cryptic.rch.unimelb.edu.au
Insights
Subcortical band heterotopia (SBH) is a rare brain malformation affecting neuronal migration. Neuropsychological assessment revealed processing speed and information registration deficits in a 9-year-old female with SBH.
Area of Science:
- Neuroscience
- Developmental Biology
- Clinical Neurology
Background:
- Subcortical band heterotopia (SBH), or 'double cortex,' is a rare congenital brain malformation.
- Characterized by a band of heterotopic grey matter between the cortex and ventricles.
- Primarily affects females, with occasional X-linked dominant familial inheritance.
Observation:
- This study details the neuropsychological assessment of a 9-year-old female diagnosed with SBH.
- MRI findings show a characteristic band of grey matter separated by white matter.
- Previous cognitive assessments focused on global intellectual functioning.
Findings:
- Significant reduction in visuomotor and oral processing speed.
- Impaired immediate information registration.
- Deficits impact skill acquisition, classroom learning, and social interaction.
Implications:
- Highlights the need for detailed neuropsychological evaluations in SBH.
- Identifies specific cognitive challenges associated with SBH.
- Informs educational and therapeutic interventions for affected individuals.
Abstract:
Subcortical band heterotopia (SBH) or 'double cortex', is a congenital brain abnormality that results from aberrant migration of neurons during development of the cortex. MRI shows a continuous band of heterotopic grey matter located between the cortex and ventricular walls, separated from them by a thin layer of white matter. The condition is quite rare, found predominantly in females, and is occasionally familial with an X-linked dominant inheritance. Current research has focused on genetic and neurological correlates, with cognitive assessment restricted to a global measure of general intellectual functioning. This paper describes in detail the results of a neuropsychological assessment of a 9-year-old female recently diagnosed with SBH. Predominant features were a significantly reduced speed of processing for visuomotor and oral output and reduced immediate registration of information. This difficulty has functional implications affecting skill acquisition, learning in the classroom, and social interaction.
