*Paramyotonia Congenita

Physician’s Summary Paramyotonia Congenita (PMC) is one of the periodic paralyses caused by mutations in the sodium channel. PMC causes muscle stiffness (myotonia) which is made worse by chilling or activity. Myotonia usually eases when the patients moves about, or “warms up” through physical activity. In PMC myotonia develops during activity, which is paradoxical or…

*Paramyotonia Congenita FAQ

Paramyotonia congenita (PMC) is a muscle disorder which causes muscle stiffness (myotonia) that appears during exercise and becomes much more severe upon exposure to cold. The myotonia which occurs during attacks of PMC may be so severe that the patient cannot inhale and exhale properly, and movement becomes difficult.  The medical term myotonia means that…

Paramyotonia Congenita & Paramyotonia Syndromes

Submitted by deb on Sun, 06/26/2011 – 00:05 Paramyotonia Congenita (PMC) is one of the periodic paralyses caused by mutations in the sodium channel. PMC causes muscle stiffness (myotonia) which is made worse by chilling or activity. Myotonia usually eases when the patients moves about, or “warms up” through physical activity. In PMC myotonia develops during activity, which is paradoxical or…

Focal and abnormally persistent paralysis associated with congenital paramyotonia

Submitted by deb on Sun, 11/09/2014 – 21:32 Focal and abnormally persistent paralysis associated with congenital paramyotonia. Magot A1, David A2, Sternberg D3, Péréon Y1. British Medical Journal Case Report 2014 Jun 17;2014. Abstract Mutations of the skeletal muscle voltage-gated sodium channel (NaV1.4) are an established cause of several clinically distinct forms of periodic paralysis and myotonia….

The Abortive Attack – Weakness Between Attacks

Submitted by deb on Mon, 06/27/2011 – 23:16 Are Weakness and Fatigue Between Paralytic Attacks Symptoms of Periodic Paralysis? Patients frequently report that their physician denies that muscle weakness and fatigue between paralytic attacks are a part of periodic paralysis. While it was reported in the past that patients with periodic paralysis had ‘normal’ muscle strength and…

Malignant Hyperthermia and HyperKPP/PMC

Submitted by deb on Mon, 06/27/2011 – 20:16 Patients with Sodium channel HyperKPP and PMC mutations are at increased risk of Malignant Hyperthermia; It has long been recognized that patients with hyperkalemic periodic paralysis are at increased risk for Malignant Hyperthermia (MH) during surgery, showing as fulminant muscle rigidity as seen in the jaw muscles, rhabdomyolysis, hypercarbia, and…

Emergency Treatment of the HyperKPP and/or PMC Attack

Submitted by deb on Sat, 08/06/2011 – 22:54 Hyperkalemic Periodic Paralysis and Paramyotonia Congenita can occur singly or in combination.  While most attacks of Hyperkalemic Periodic Paralysis are brief and do not require emergency intervention, occasionally the serum potassium level will be high enough to cause cardiac distress, or muscle stiffness may interfere with respiration.  Attacks of…