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Neuropediatrics 1990; 21(3): 133-135
DOI: 10.1055/s-2008-1071479
Original article

© Georg Thieme Verlag KG Stuttgart · New York

Acute Hemiparesis as the Presenting Sign in a Heterozygote for Ornithine Transcarbamylase Deficiency

T. J. de Grauw , L. M. E. Smit , M.  Brockstedt , Y.  Meijer , J.  v. d. Klei-v. Moorsel , C.  Jakobs
  • Dept. of Pediatrics, Free University Hospital, 1007 MB Amsterdam, The Netherlands
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

Strokes in children occur in conjunction with cardiac disease, hematological disorders, trauma, intracranial infections and migraine. Recently several inborn errors of metabolism have been recognized as possible causes of stroke-like symptoms. We describe a female heterozygote of ornithine transcarbamylase deficiency, who presented with convulsions and right sided hemiplegia. MR-imaging of the brain demonstrated an acute ischemic lesion in the left hemisphere.

In addition to other known metabolic causes of stroke like attacks urea cycle defects should be considered in the differential diagnosis of acute hemiplegia in childhood.

Key words

Acute hemiplegia - OTC deficiency - Heterozygote

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