Insulin Receptors, Insulin Secretion, and Glucose Disappearance Rate in Patients With Periodic Hypokalaemic Paralysis

Abstract

In a study of 6 male patients with periodic hypokalaemic paralysis (PHP), we found reduced insulin binding to monocytes as compared with a group of 25 normal subjects (P < 0.1). The decreased insulin binding was caused by the decreased binding affinity. During induction of paralysis by a prolonged oral glucose load, one patient showed 24-h variations in the insulin binding to monocytes not differing from those observed in normals. After iv administration of glucose, these patients showed an elevated initial insulin response compared with the normals (P < 0.1). However, the iv glucose tolerance tests revealed normal glucose disappearance rates. We conclude that changes in insulin receptor binding do not appear to be of pathophysiological significance for eliciting the parese attacks in PHP. However, the increased insulin response, following carbohydrate intake, might be of significance in the generation of paralytic attacks in patients with PHP.Familial periodic hypokalaemic paralysis (PHP) is a rare muscle disease characterised by attacks of flaccid paresis lasting from a few hours to several

A reduced number of insulin receptors in patients with Prader-Willi syndrome

“…Insulin receptor defects have previously been demonstrated in other clinical syndromes such as generalized lipodystrophy (Oseid et al 1977), ataxia telangiectasis (Bar et al 1978), periodic hypokalaemic paralysis (Johnson & Beck-Nielsen 1979), Klinefelter's syndrome (Breyer et al 1981) and acanthosis nigricans (Bar et al 1980). The finding in this paper add PW syndrome to the groups of syndromes characterized by insulin resi¬ stance and an insulin receptor defect.…”