Neuropediatrics 1992; 23(3): 165-168
DOI: 10.1055/s-2008-1071335
Case report

© Georg Thieme Verlag KG Stuttgart · New York

Mitochondrial Angiopathy in a Family with MELAS

Ch.  Förster1 , G.  Hübner2 , J.  Müller-Höcker2 , D.  Pongratz3 , P.  Baierl4 , R.  Senger5 , W.  Ruitenbeek5
  • 1Department of Pediatrics, University of Munich, Germany
  • 2Institute of Pathology, University of Munich, Germany
  • 3Department of Medicine, University of Munich, Germany
  • 4Department of Radiology, University of Munich, Germany
  • 5Department of Pediatrics, University of Nijmwegen, The Netherlands
Further Information

Publication History

Publication Date:
19 March 2008 (online)


A family with mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike epidoses (MELAS) affecting mother, son and daughter is described. Biochemical studies on muscle biopsy specimen in one patient revealed NADH dehydrogenase (complex I) deficiency. A mitochondrial angiopathy could be demonstrated by brain and muscle biopsy. It is suggested that the mitochondrial angiopathy is the basic pathogenic mechanism of impaired cerebral circulation in MELAS.