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Journal of Child Neurology
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Hemiconvulsion-Hemiplegia-Epilepsy Syndrome: Characteristic Early Magnetic Resonance Imaging Findings

Jeremy L. Freeman, MBBS

Department of Neurology, Royal Children's Hospital, freemanj{at}cryptic.rch.unimelb.edu.au

Lee T. Coleman, FRACR, MBChb, BSc

Department of Radiology, Royal Children's Hospital

Lindsay J. Smith, MBBS, FRACP

Department of Neurology, Royal Children's Hospital, Department of Neurology, Monash Medical Centre, Melbourne, Australia

Lloyd K. Shield, MBBS, FRACP

Department of Neurology, Royal Children's Hospital

We report three patients with hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and were shown to have striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere, including extensive diffusion-weighted imaging abnormalities in two cases. Two patients subsequently developed progressive and extensive atrophy of the involved hemisphere. These findings are consistent with earlier descriptions of the classic neuroradiologic features of this syndrome and are helpful in the differential diagnosis of acute infantile hemiplegia. Further, the findings support the previously proposed pathogenetic mechanism of neuronal injury caused by status epilepticus. (J Child Neurol 2002;17:10-16).

Journal of Child Neurology, Vol. 17, No. 1, 10-16 (2002)
DOI: 10.1177/088307380201700103


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