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Longitudinal evaluation of apparent diffusion coefficient in children with neurofibromatosis type 1
Shams F Sheikh1, Wayne S Kubal, Adam W Anderson
1Diagnostic Imaging, Yale University School of Medicine, New Haven, Connecticut 06520-8042, USA. shams.sheikh@yale.edu
Journal of Computer Assisted Tomography
|September 23, 2003
Summary
Neurofibromatosis type 1 (NF1) brain lesions show increasing diffusion with age in pediatric patients, unlike normal brain development. This suggests changes in water content and cellularity within these NF1 lesions over time.
Area of Science:
- Neuroimaging
- Pediatric Neurology
- Genetics
Background:
- Neurofibromatosis type 1 (NF1) is a genetic disorder.
- NF1 can cause brain lesions, often T2-hyperintense foci.
- These lesions, termed hamartoma-like lesions (HLL), have unclear characteristics.
Purpose of the Study:
- To characterize NF1-related brain lesions (HLL) in pediatric patients.
- To investigate the longitudinal changes in HLL using diffusion-weighted imaging (DWI).
- To compare lesion characteristics with normal brain development.
Main Methods:
- Retrospective study of 12 pediatric NF1 patients (ages 2-20).
- Analysis of 40 lesions across 166 longitudinal MRI scans.
- Measurement of apparent diffusion coefficients (ADCs) in lesions and comparison with 14 healthy children.
Main Results:
- NF1 lesions exhibited higher ADCs than normal brain tissue.
- Lesion ADCs increased with age in pediatric NF1 patients.
- Supratentorial lesions showed a significantly faster rate of ADC increase compared to infratentorial lesions.
Conclusions:
- Increasing ADC in NF1 lesions suggests extracellular water accumulation or reduced cellularity with age.
- Changes may relate to myelin vacuole alterations within NF1 lesions.
- Differential rates of ADC change between supra- and infratentorial lesions indicate potential intrinsic differences or environmental influences.