Affordable Access

Publisher Website

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.

There are no comments yet on this publication. Be the first to share your thoughts.

Statistics

Seen <100 times
0 Comments

More articles like this

[Neuroradiologic findings in Möbius syndrome].

on RöFo : Fortschritte auf dem G... May 2007

CHARGE syndrome: Neuroradiological findings

on European Journal of Radiology... Jan 01, 2010

Neuroradiologic findings in Sotos syndrome.

on Journal of Child Neurology July 2006

Neuroradiological findings in the northern epileps...

on Acta neurologica Scandinavica December 1994
More articles like this..