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Hemimegalencephaly: what happens when children get older?
Nicky Wu1, Felippe Borlot, Anfal Ali
1Division of Neurology, Epilepsy Genetics Program, Toronto Western Hospital, Krembil Neuroscience Centre, University of Toronto, Toronto, ON, Canada.
Developmental Medicine and Child Neurology
|February 6, 2014
Summary
Adults with hemimegalencephaly (HME) show varied neurological outcomes. Early seizure onset in HME is linked to poorer cognitive and motor development in adulthood.
Area of Science:
- Neurology
- Developmental Neuroscience
- Congenital Malformations
Background:
- Hemimegalencephaly (HME) is a rare congenital brain malformation.
- It is typically associated with developmental delays and severe epilepsy.
- HME in adults is infrequently reported, making adult neurological outcomes poorly understood.
Observation:
- This study retrospectively analyzed neurological findings in five adult HME patients.
- Evaluations included neurocutaneous disorders, cognitive function, seizure control, and treatment outcomes.
- The cohort included patients aged 18-34 years, with most having HME associated with neurocutaneous syndromes.
Findings:
- Intellectual disability varied from none to severe.
- Motor deficits were common, ranging from hemiparesis to non-ambulation.
- Crucially, later seizure onset (after age 7) correlated with better seizure control and psychomotor development in adulthood compared to early-onset seizures.
Implications:
- Age at seizure onset is a significant factor influencing long-term neurological outcomes in adult HME patients.
- Cognitive disability and seizure control are associated with HME in adulthood.
- Further research is needed to understand and manage HME in adult populations.
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