Recurrent respiratory insufficiency and depressed ventilatory drive complicating mitochondrial myopathies

Barohn, R.J.; Clanton, T.; Sahenk, Z.; Mendell, J.R.

Neurology 40(1): 103-106


ISSN/ISBN: 0028-3878
PMID: 2296355
Document Number: 366483
Three patients with mitochondrial myopathies and progressive external ophthalmoplegia had repeated episodes of respiratory failure requiring assisted ventilation. Studies in these patients and asymptomatic family members, as well as a sporadic case of Kearns-Sayre syndrome, demonstrated markedly depressed ventilatory drive responses to hypoxia. In 2 patients, there was also decreased drive to hypercapnia. The reduced ventilatory drive appears to be due to an altered neural control system that may cause episodic life-threatening hypoventilation occurring especially in relation to surgery, sedation, or intercurrent infection.

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