General Child Neurology
May. 31, 2021
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• Perhexiline is a prophylactic antianginal agent that enhances cardiac oxygen efficiency by inhibiting mitochondrial carnitine palmitoyltransferase-1, which results in an increase in glucose metabolism at the expense of fatty acid metabolism.
• The principal neurotoxic effect of perhexiline is a predominantly demyelinating sensorimotor polyneuropathy.
• The toxic mechanism is likely related to accumulation of lysosomal inclusions in Schwann cells and other tissues. Like amiodarone, perhexiline is a cationic amphiphilic drug that can penetrate lysosomes.
Perhexiline maleate is an orally-administered prophylactic agent that was introduced in the 1970s for the treatment of angina pectoris and variant angina (Prinzmetal) (14; 19). A beneficial effect has been suggested in elderly patients with severe aortic stenosis (27) or congestive heart failure (01). Interest in this drug has increased in recent years. Its actions include coronary vasodilatation by a direct effect on vascular smooth muscle and blocking exercise-induced tachycardia. It acts as a cardiac metabolic agent, enhancing oxygen efficiency by inhibiting mitochondrial carnitine palmitoyltransferase-1, resulting in an increase in glucose metabolism at the expense of fatty acid metabolism (10; 01). The principal neurotoxic effect of perhexiline is a predominantly demyelinating peripheral neuropathy, an uncommon finding in toxic neuropathies. There are additional reports of raised intracranial pressure with papilledema and a proximal myopathy.
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