Disorders of histogenesis: the neurocutaneous syndromes

MRI is uniquely suited to demonstration of the wide array of brain abnormalities in the neurocutaneous syndromes, due to its ability to provide previously unattainable neuroanatomic information on T1-weighted images and its extreme sensitivity to changes in water content of brain structures on T2-weighted images. It therefore is assisting physicians in better understanding the full expression of these heritable disorders, such as the extent of optic pathway involvement in optic nerve glioma, the presence of hamartomas in TS, and abnormal myelination in Sturge-Weber syndrome. It may in the future have a role in genetic counseling in TS. CT provides complimentary information to MRI in the evaluation of intracranial pathology in the neurocutaneous syndromes. Bone abnormalities and abnormal states of calcification may at times still be better evaluated by CT. Additionally, CT is a less expensive means of following existent abnormalities over time.