Ondine's Curse Consequent to Recurrent Respiratory Failure in a Man with Leber Hereditary Optic Neuropathy

J Korean Neurol Assoc > Volume 22(4); 2004 > Article

Journal of the Korean Neurological Association 2004;22(4): 414-417.

재발성 호흡부전 후에 Ondine's Curse를 보인 Leber 유전성시신경병증 1예

하상원, 안성기 이상암

울산대학교 의과대학 신경과학교실

Ondine's Curse Consequent to Recurrent Respiratory Failure in a Man with Leber Hereditary Optic Neuropathy

Sangwon Ha

Department of Neurology, University of Ulsan College of Medicine, Seoul, Korea

Abstract

Leber's hereditary optic neuropathy (LHON) is a disease due to mitochondrial DNA mutations. We report a man with LHON, who presented with four episodes of respiratory failure. In the last episode, he had a loss of the automatic component of the respiratory drive but retained the voluntary component. Brain MRI showed lesions in the midbrain, pons, and medulla oblongata, involving the neuroanatomic areas of the medullary respiratory center. Our patient provides a further example of the broad manifestations of mitochondrial disease. Key Words: Leber's hereditary optic neuropathy, Recurrent respiratory failure, Medulla oblongata