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Figure 37-1. Focal cortical dysplasia. A T2-FLAIR MRI in the coronal plane in a patient with seizures. Deep in the white matter, adjacent to the lateral ventricles, are multifocal heterotopic aggregates of gray matter, more apparent in the left hemisphere.
Figure 37-2. Dandy-Walker syndrome. MRI showing agenesis of the midline cerebellum and large midline cyst, representing the greatly dilated fourth ventricle, which occupies almost the entire posterior fossa. A. Axial view. B. Sagittal view.
Figure 37-3. Sagittal T2-weighted MRI of an adult with tethered cord and a typical lipoma at its caudal extent. No dysraphism is present. The main clinical features were a flaccid bladder, asymmetrical weakness and atrophy of the forelegs, and spasticity in the legs.
Figure 37-4. Chiari-type type I malformation and developmental syringomyelia. T2-weighted MRI of the low-lying cerebellar tonsils below the foramen magnum and behind the upper cervical cord and the syrinx cavity in the upper cord.
Figure 37-5. Adenoma sebaceum of tuberous sclerosis.
Figure 37-6. Shagreen patch on the skin of the lower back in a young patient with tuberous sclerosis.
Figure 37-7. Tuberous sclerosis. A. MRI showing multiple hamartomas. B. Subependymal nodules are demonstrated on CT, where their calcific nature has led them to be termed “brain stones.”
Figure 37-8. Typical large café-au-lait spot. The presence of 6 or more hyperpigmented lesions, each larger than 1.5 cm after puberty (>5 mm prepubertal), is diagnostic of neurofibromatosis type 1.
Figure 37-9. Molluscum fibrosum nonneural skin tumors of von Recklinghausen disease.
Figure 37-10. Hamartomas of the iris (Lisch nodules), typical of neurofibromatosis type 1. (Reproduced by permission from Damato BE, Spalton DJ: The uveal tract. In: Spalton DJ, Hitchings RA, Hunter PA (eds): Atlas of Clinical Ophthalmology, 3rd ed. Oxford, Mosby Elsevier Ltd, 2005.)
Figure 37-11. Neurofibromatosis. Upper left: T2-FLAIR MRI in the axial plane demonstrating multiple foci of hyperintensity, presumably hamartomas, in a patient with neurofibromatosis type 1. Upper right: Axial T1 MRI with gadolinium demonstrating bilateral (right larger than left) schwannomas in a patient with neurofibromatosis type 2. Lower panel: Axial T1 MRI with gadolinium of the thoracic spine showing a large left paraspinal schwannoma emanating from the neural foramen in a patient with schwannomatosis. Note the expansion and remodeling of the vertebral bones surrounding the lesion. (Images courtesy of Dr. Scott R. Plotkin.)
Figure 37-12. Sturge-Weber. Left: Axial non-enhanced CT showing calcification of the right parietal vascular malformation and underlying cortical atrophy. Right: Axial T2-FLAIR MRI showing the pronounced atrophy in the same patient.