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Journal of Child Neurology
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Extreme Microcephaly With Agyria-Pachygyria, Partial Agenesis of the Corpus Callosum, and Pontocerebellar Dysplasia

László Sztriha, MD, PhD, DSc , MD, FRCP

Department of Paediatrics sztriha{at}uaeu.ac.ae.

Lihadh I. Al-Gazali

Department of Paediatrics Faculty of Medicine and Health Sciences United Arab Emirates University Al Ain, United Arab Emirates

A 1-year-old boy with extreme microcephaly and a complex brain malformation is reported. Magnetic resonance imaging revealed an abnormal gyral pattern with features of the agyria-pachygyria spectrum, partial agenesis of the corpus callosum, severely hypoplastic posterior cerebellar vermis, and an abnormal foliation pattern of the cerebellar hemispheres associated with a flat and wide isthmus and pons. Although this phenotype shares some features with malformations classified as microcephaly with a simplified gyral pattern, microlissencephaly, or lissencephaly with cerebellar hypoplasia, none of the several subgroups of these categories are identical to the cerebral dysgenesis found in this patient. (J Child Neurol 2005;20:170—172).

Journal of Child Neurology, Vol. 20, No. 2, 170-172 (2005)
DOI: 10.1177/08830738050200021301
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