Presentation
Convulsions since the age of 6 months, with delayed milestone and weakness. Some convulsive attacks were prolonged. No history of perinatal hypoxia. Normal vaginal delivery and normal breastfeeding till the age of 6 months then developed convulsions and decreased ability of breastfeeding.
Patient Data
Smooth surface of the cerebrum with thick cortex, and decreased cortical sulci (pachygyria) with posterior predilection, suggestive of lissencephaly-pachygyria spectrum.
This is associated with atrophic brain changes as well as periventricular (notably peri-trigonal) cystic changes surrounded by leukomalacia, suggestive of sequels of hypoxic-ischemic insult with periventricular leukomalacia.
Diffuse atrophic brain changes, notably along the posterior fossa, with thinning out of both inferior cerebellar peduncles. Supra- and infratentorial ventricular dilatation is noted, sparing the frontal horns of both lateral ventricles.
Incidentally noted, bilateral mastoiditis.
Case Discussion
Imaging features are suggestive of lissencephaly-pachygyria spectrum (Lissencephaly type I with posterior predilection) with periventricular changes suggestive of periventricular leukomalacia. Although no history of perinatal hypoxia, white matter ischemia can develop as a result of repeated hypoxia during profound convulsive attacks.
Case courtesy Dr. Mohamed Kayed, MD and Dr. Sara Abdelsalam, MS.
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