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Neurology 1999;53:855
© 1999 American Academy of Neurology
Brief Communications

Age and cause of death in mitochondrial diseases

Thomas Klopstock, MD, Michaela Jaksch, MD and Thomas Gasser, MD

From the Department of Neurology (Drs. Klopstock and Gasser), Klinikum Großhadern, Ludwig-Maximilians-University Munich; and the Institute of Clinical Chemistry (Dr. Jaksch), Molecular Diagnostics and Mitochondrial Genetics, Academic Hospital Schwabing, Munich, Germany.

Address correspondence and reprint requests to Dr. Thomas Klopstock, Department of Neurology, Klinikum Großhadern, Ludwig-Maximilians-University Munich, 81366 Munich, Germany.

We report on the age and the causes of death in 16 patients with mitochondrial diseases. Nine patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) died at a mean age of 34 years and three patients with chronic progressive external ophthalmoplegia at a mean age of 56 years. The causes of death were cardiopulmonary failure (n = 5), status epilepticus (n = 4), aspiration pneumonia (n = 2), pulmonary embolism (n = 2), renal failure (n = 1), metabolic disturbance (n = 1), and unknown causes (n = 1). Thus, many patients in this series died of medical complications, some of which may be prevented.

Key words: Mitochondrial disease—Mitochondrial encephalomyopathy—lactic acidosis—and stroke-like episodes—Causes of death.




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