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Case Reports in Medicine
Volume 2010 (2010), Article ID 986302, 3 pages
http://dx.doi.org/10.1155/2010/986302
Case Report

Leigh's Disease: The Acute Clinical Course of a Two-Year-Old Child with Subacute Necrotizing Encephalomyelopathy

Institute of Legal Medicine, University of Munich, Nussbaumstraße 26, 80336 Munich, Germany

Received 4 January 2010; Accepted 7 April 2010

Academic Editor: Mamede de Carvalho

Copyright © 2010 Bettina Zinka et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

We report the untypical clinical course of a previously healthy two-year-old girl, who died suddenly and unexpectedly after an episode of vomiting. At forensic autopsy no other pathological findings could be diagnosed than multiple reddish, sunken areas in brain stem, mesencephalon, and pons. Histologically they presented as areas of spongiosis of the neuropil with prominent endothelial hyperplasia and vascular proliferation whereas nerve cells were well preserved. On the basis of the characteristic neuropathological findings in combination with the age of the child, we had to take into consideration that the child might have died from subacute necrotizing encephalomyelopathy (Leigh's Disease) despite the untypical, fulminant clinical course.