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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 23:1101-1104, August 2002
© 2002 American Society of Neuroradiology

Case Report
PEDIATRICS

Neuropathologic and MR Imaging Correlation in a Neonatal Case of Cerebellar Cortical Dysplasia

Gustavo Soto-Ares^a, Louise Devisme^c, Sylvie Jorriot^d, Berengere Deries^a, Jean P. Pruvo^a and Marie M. Ruchoux^b

^a Department of Neuroradiology, Hôpital Roger Salengro, Chru Lille, France
^b Department of Neuropathology, Hôpital Roger Salengro, Chru Lille, France
^c Department of Anatomy-Pathology, Hôpital Calmette, Chru Lille, France
^d Department of Neonatology, Hôpital Jeanne de Flandre, Chru Lille, France

Address reprint requests to Gustavo Soto-Ares, MD, Department of Neuroradiology, Hôpital Roger Salengro, Chru Lille 59037 Lille, France

Little documentation of the correlation between MR imaging findings in isolated cerebellar cortical dysplasia (CCD) and its neuropathologic characteristics exists in the recent literature. We documented a postmortem neuropathologic study of a clinically and radiologically well-documented case of CCD in a neonate with severe hypotonia and status epilepticus. MR imaging revealed a global vermian hypoplasia with marked cortical dysplasia. CCD was associated with a voluminous heterotopic mass. The postmortem neuropathologic study confirmed vermian hypoplasia and CCD, which consisted of right cerebellar cortical polymicrogyria with subcortical heterotopia. CCD is a pathologic entity that could be well diagnosed with MR imaging even in the neonatal period.

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