Presentation
Seizures since birth. Delayed milestones
Patient Data
Myelination appears consistent with age on T1W images. There is evidence of thickening of the gyri in both the cerebral hemispheres. There is thinning of the subcortical white matter with smooth brain surface of bilateral posterior temporal lobes, parietal lobes and occipital lobes with loss of interdigitation of the gray and white matter. A circumferential band of hyperintensity is noted on inversion recovery sequence. The sylvian fissures appear shallow and vertically oriented posterosuperiorly. There is no evidence of heterotopia. Corpus callosum and ventricles appear normal.
Case Discussion
Lissencephaly-pachygyria spectrum is a disorder of neuronal migration. This case demonstrates incomplete lissencephaly type I with parietal, occipital and posterior temporal agyria and frontotemporal pachygyria.
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