Cerebellar Cortical Dysplasia: MR Findings in a Complex Entity
Gustavo Soto-Ares
,a,
Christine Delmairea,
Berengere Deriesa,
Louis Valleea and
Jean Pierre Pruvoa
a From the Departments of Neuroradiology (G.S-A., C.D., B.D., J.P.P.) and Pediatric Neurology (L.V.), Hôpital Roger Salengro, Chru Lille France.
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FIG 1. Patient 15, Dandy-Walker complex. Axial inversion-recovery (IR) T1-weighted image (11520/60/400/2 [TR/TE/TI/excitations]) shows bilateral, vertical, orientated folia of the cerebellar cortex associated with an enlarged fourth ventricle.
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FIG 2. Patient 8, possible Joubert malformation.
A, Coronal IR T1-weighted image (11520/60/400/2) shows rostral vermian dysgenesis (black arrowhead), bilateral abnormal arborization of the white matter (double black arrowhead), and linear heterotopia (white arrow).
B, Axial T2-weighted image (5000/120/2 [TR/TE/excitations]) shows vermian dysgenesis (short arrow) associated with mega cisterna magna and prominent appearance of the superior cerebellar peduncles (long arrow).
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FIG 3. Patient 1. Axial T2-weighted images (5000/120/2) show cystlike inclusions (arrows), cortical dysplasia (arrowhead), and pons hypoplasia (black arrowhead) (A) associated with medial vermian fissure (long arrow), occipital polymicrogyria, white matter hyperintensity, and ventricular enlargement (B)
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FIG 4. Patient 4. A, Axial IR T1-weighted image (11520/60/400/2) shows right cerebellar hypertrophy with vertical folia in an 8-year-old boy with ataxia and psychomotor retardation.
B, Coronal T2-weighted image (5000/120/2) shows associated malformations: minor parietal lobe (double arrow) and ventricle enlargement, nodular heterotopia (long arrow) and white matter hyperintensity (short arrows).
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FIG 5. Patient 5. Ten-month-old boy with spastic tetraparesia, developmental delay, and difficulty in swallowing. Coronal T2-weighted image (5000/120/2) shows right cerebellar cortical disorganization without normal fissures associated with hyperplasia of the right cerebellar lobe and cortical thickening. Note white matter hyperintensities (black arrowheads) and cortical dysplasia (white arrows) in supratentorial images
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FIG 6. Patient 16. MR imaging findings in a 4-year-old boy with microcephalia, motor delay, and facial deformities.
A, Coronal IR T1-weighted image (11520/60/400/2) shows bilateral cerebellar defective foliation and irregularity of the gray/white matter junction (black arrowheads). Note subependymal heterotopia along the floor of the left temporal horn (white arrow).
B, Coronal IR T1-weighted images (11520/60/400/2) shows heterotopia (white arrow) and associated cortical dysplasia of parahippocampal gyrus (asterisk).
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FIG 7. Patient 17. MR imaging findings in a 1 year-old boy with facial deformities, hypotonia, and developmental delay.
A, Coronal IR T1-weighted image (11520/60/400/2) shows medial vermian fissure (arrow).
B, Sagittal IR T1-weighted image (same parameters) shows lack of normal fissures of the vermis associated with inferior vermis hypoplasia.
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AJNR - American Journal of Neuroradiology