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Posterior agyria-pachygyria with polymicrogyria: evidence for an inherited neuronal migration disorder
C D Ferrie1, G D Jackson, S Giannakodimos
1Department of Clinical Neurophysiology and Epilepsy, St. Thomas' Hospital, London, UK.
Neurology
|January 1, 1995
Abstract:
We describe two brothers with mental retardation and refractory epilepsy. MRI revealed symmetrical agyria-pachygyria of the temporo-occipito-parietal regions, areas of deeply infolded polymicrogyric parietal cortex, and dilated occipital horns (colpocephaly). The stereotyped clinical, EEG, and MRI findings suggest that this may be a distinct inherited condition and imply that agyria-pachygyria with polymicrogyria is not always sporadic.