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Hemimegalencephaly with tuberous sclerosis: a longitudinal imaging study.
Hemant Parmar1, Deepak Patkar, Jeshil Shah
1Department of Radiology, King Edward VII Memorial Hospital, Bombay, India. parurad@hotmail.com
Australasian Radiology
|December 4, 2003
Summary
Hemimegalencephaly, a rare brain malformation, can present with tuberous sclerosis complex. This case highlights evolving imaging findings, including atrophy and tubers, over time.
Area of Science:
- Neurology
- Developmental Neuroscience
- Pediatric Neurology
Background:
- Hemimegalencephaly is a congenital cerebral malformation defined by unilateral brain enlargement and cortical dysplasia.
- Tuberous sclerosis complex (TSC) is a genetic disorder causing tumors to grow in various organs, including the brain.
Observation:
- This report details a child diagnosed with hemimegalencephaly affecting the right cerebral hemisphere, concurrently exhibiting TSC manifestations.
- Initial imaging revealed abnormal myelination and gyral patterns in the enlarged hemisphere.
- Subsequent imaging demonstrated frontoparietal atrophy in the affected hemisphere and compensatory overgrowth in the contralateral hemisphere.
Findings:
- White-matter lesions were more conspicuous in the neonatal period on MRI.
- Cortical tubers associated with TSC became more apparent with age.
- The dynamic changes in brain structure, including atrophy and compensatory growth, were observed over time.
Implications:
- This case underscores the importance of serial neuroimaging in evaluating hemimegalencephaly and associated conditions like TSC.
- Understanding the evolving imaging characteristics aids in accurate diagnosis and management planning.
- The findings contribute to the knowledge of neurodevelopmental trajectories in complex cerebral malformations.