Patient 1 (Figure
, IV.1) CT axial images of the brain (A&B, age = 21 days) demonstrating brain underdevelopment with large extracerebral CSF spaces. The frontal and temporal lobes sulci [A] are more developed compared to Patient 6 [G&H], but still immature. The Sylvian fissures [B] are more developed and have visible frontal and temporal opercula (arrows). [C&D]. Patient 2 (Figure 1, IV.3). [C] CT image of the brain (age = 3 days) showing remarkable brain underdevelopment, very few sulci in the frontal and temporal lobes, and large extracerebral CSF spaces. The Sylvian fissures are underdeveloped and wide open. [D] Sagittal T1-weighted MR image of the brain demonstrating small cerebellar vermis (arrow) and enlarged cisterna magna and CSF spaces in the posterior fossa. The corpus callosum is fully developed and the brain stem has normal appearance. [E&F]. Reconstructed sagittal [E] and direct axial [F] CT images of the brain (Patient 3: Figure 1, IV.4) at the age of four days demonstrating remarkable cerebral and cerebellar underdevelopment evident by simplified gyral pattern (arrows) and enlargement of extracerebral CSF spaces. [G&H] Axial T2-weighted MR images of the brain (Patient 6: Figure 1, IV. 11) at the age of one day showing simplified gyral pattern. The sulci of the frontal and temporal lobes are very few and shallow [G], while the Sylvian fissures are wide open (asterisk). Better gyration and deeper sulci are seen in the brain convexity around the Rolandic fissure [H]. The extracerebral CSF spaces are remarkably enlarged.