Encephalocraniocutaneous Lipomatosis: Complete Neuroradiologic Evaluation and Follow-up of Two Cases
Cecilia Parazzini
,a,
Fabio Triulzia,
Gianni Russoa,
Massimo Mastrangeloa and
Giuseppe Scottia
a From the Departments of Neuroradiology (C.P., F.T., G.S.) and Pediatrics (G.R.), Scientific Institute H. S. Raffaele, and the Department of Neurophysiopathology (M.M.), University of Milan, Italy.
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FIG 1. Case 1.
A and B, CT scans obtained at 20 days of life show an enlargement of the right lateral ventricle, mainly at the occipital horn, and of the right sylvian fissure, with incomplete opercularization of the insula (A), and a large right middle cranial fossa arachnoid cyst and a cerebellopontine angle lipoma on the same side (arrowhead, B).
C and D, MR images obtained at 19 months of age. Axial T2-weighted image (3000/120/1 [TR/TE/excitations]) (C) confirms the enlargement of the right lateral ventricle and the abnormal opercularization of the insula; the right hemispheric cortex appears thick, with few and irregular sulci. Coronal T1-weighted image (510/15/2) (D) shows the right pontocerebellar angle lipoma and the temporal arachnoid cyst; a subtle hemispheric subdural collection, without mass effect, is appreciable on the right.
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FIG 2. Case 2.
A, Contrast-enhanced CT scan obtained at 17 days of life shows a widening of the right lateral ventricle and sylvian fissure and areas of slight hyperdensity in the right temporooccipital region. (Courtesy of Dr. Nadia Colombo, Milan, Italy.)
B, Axial T2-weighted MR image (3000/120/1) obtained at 4 months of age shows an increase in size of the right lateral ventricle and a widening of the right hemispheric subarachnoid spaces. A lack of insular opercularization and cortical dysplasia in the right temporoparietooccipital region is well seen; the abnormal cortex is thicker than normal, with multiple and small gyri.
C and D, MR study obtained at 16 months of age. Coronal reconstruction image obtained with the MP-RAGE technique (C) confirms cortical dysplasia; the temporal cortex appears thick, with irregular gyri. On axial T2 weighted image (3000/120/1) (D), myelination seems to be normal.
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