Published online February 16, 2007
PEDIATRICS Vol. 119 No. 3 March 2007, pp. e768-e772 (doi:10.1542/peds.2006-0763)

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EXPERIENCE & REASON

Radiologic and Neurophysiologic Aspects of Stroke-like Episodes in Children With Congenital Disorder of Glycosylation Type Ia

Argirios Dinopoulos, MD, PhD^a, Ismail Mohamed, MD^a, Blaise Jones, MD^b, Sanjai Rao, DO^a, David Franz, MD^a, Ton deGrauw, MD, PhD^a

^a Divisions of Neurology
^b Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio

ABSTRACT

In an effort to shed light on the mechanism of hemiparetic stroke-like events experienced by patients with congenital disorder of glycosylation type Ia, we evaluated 3 children with this disorder by brain imaging studies and continuous electroencephalogram monitoring during such events. No evidence of ischemia or infarction was revealed on imaging studies and electrographic seizures or intermittent epileptiform activity was demonstrated on electrographic recordings. All 3 patients showed clinical and electrographic improvement after administration of antiepileptic medication. Epileptic phenomena can complicate the stroke-like events of patients with congenital disorder of glycosylation type Ia, and the cause of the hemiparesis may indeed be an active epileptic inhibitory process. As such, electroencephalogram monitoring is warranted, and treatment with anticonvulsant agents is indicated.

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Key Words: hemiparesis • stroke-like • epilepsy • congenital disorder of glycosylation • CDG

Abbreviations: CDG, congenital disorders of glycosylation • EEG, electroencephalogram • ED, emergency department • DWI, diffusion-weighted imaging

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Accepted Sep 18, 2006.

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