Crossover randomized controlled trial of Bumetanide to rescue an attack of exercise induced hand weakness in Hypokalaemic Periodic Paralysis

Hypokalaemic periodic paralysis (HypoPP) is a neuromuscular disorder caused by mutations in the voltage-gated skeletal muscle channel genes CACNA1S and SCN4A. The genetic changes result in an aberrant inward leak current across the sarcolemma, which increases the susceptibility of the muscle membrane to sustained depolarisation at low serum potassium levels. Clinically this manifests as recurrent paralysis.[1] The only pharmacological option to abort acute symptoms in HypoPP is potassium supplementation.

Source: Neuromuscular Disorders - January 2, 2024 Category: Neurology Authors: Renata Siciliani Scalco, Jasper M Morrow, Andreea Manole, Iwona Skorupinska, Federico Ricciardi, Emma Matthews, Michael G Hanna, Doreen Fialho Source Type: research