RT Journal Article
SR Electronic
T1 Recurrent respiratory insufficiency and depressed ventilatory drive complicating mitochondrial myopathies
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 103
OP 103
DO 10.1212/WNL.40.1.103
VO 40
IS 1
A1 Barohn, Richard J.
A1 Clanton, Thomas
A1 Sahenk, Zarife
A1 Mendell, Jerry R.
YR 1990
UL http://n.neurology.org/content/40/1/103.abstract
AB 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.