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Neuroradiologic Findings in Sengers Syndrome

Authors
Journal
Pediatric Neurology
0887-8994
Publisher
Elsevier
Publication Date
Volume
39
Issue
2
Identifiers
DOI: 10.1016/j.pediatrneurol.2008.05.003
Disciplines
  • Astronomy
  • Medicine

Abstract

Sengers syndrome is characterized by a constellation of congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, and lactic acidosis. Two forms of the disease have been described: a fatal neonatal form, and a more benign form in which patients live into their second or third decades. With the exception of time to death, no findings have distinguished these two forms. We present 3 cases of neonatal Sengers syndrome with significant central nervous system involvement, a finding not previously described. We suggest that the fatal neonatal form of Sengers syndrome would be more accurately described as a mitochondrial encephalomyopathy. Cranial imaging may help distinguish the two types of this syndrome.

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