Van Hove J L, Shanske S, Ciacci F, Ballinger S, Shoffner J S, Wallace D C, Hanioka T, Folkers K, Bossen E H, Kussin P S
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710.
Am J Med Genet. 1994 Jun 1;51(2):114-20. doi: 10.1002/ajmg.1320510207.
A 40-year-old woman presented with profound muscle weakness resulting in failure to wean from a ventilator and persistent lactic acidosis after having recovered from a pneumonia complicated by adult respiratory distress syndrome, myocardial infarction, renal failure and shock. She had a 28 year history of chronic anemia and exercise intolerance. Anemia and thrombocytopenia persisted after admission. Nonobstructive hypertrophic cardiomyopathy was present. A stroke-like episode occurred. A mitochondrial myopathy with deficiencies in complexes IV and II was demonstrated, but no DNA defect has yet been found. This patient represents a distinct clinical presentation of a mitochondrial disorder characterized by late onset mitochondrial myopathy, chronic anemia, cardiomyopathy, and lactic acidosis.