Thyrotoxic

Leaky channels make weak muscles: Mutations in the skeletal muscle voltage-gated calcium channel (CaV 1.1) have been associated with hypokalemic periodic paralysis, but how these mutations cause the symptoms of the disorder has always been unclear. In this article the authors describe how they used mice with the CaV 1.1 mutation  to investigate how the mutation causes the features of HypoKPP.

PLoS One. 2012; 7(7): e40235.
Published online 2012 July 10.

Splicing of the rSlo Gene Affects the Molecular Composition and Drug Response of Ca2+-Activated K+ Channels in Skeletal Muscle

Maria Maddalena Dinardo,#1 Giulia Camerino,#1 Antonietta Mele,1 Ramon Latorre,2 Diana Conte Camerino,1 and Domenico Tricarico1,*

Abstract

Puzzled by the terms membrane potential, sodium channel, potassium channel and sodium-potassium pump?  

This cute cartoon explains what each is and shows how the potassium and sodium channels, and the sodium potassium pump maintain muscle membrane potential.  Although this uses nerves to illustrate how the principle works, the action is the same in muscle membrane. Click here to watch.

For your convenience, arranged by topic, a "shelf" of journal articles on the periodic paralyses.

If you have a favorite link to suggest please pass it along.

This chapter from McGraw Hill's Myology, third edition; editors Andrew Engel and Clara Franzini-Armstrong,  give a comprehensive look at the nondystrophic myotonias and periodic paralyses, written by one of the world's top teams in the field.

Chapter from Myology, third edition: Nondystrophic Myotonias and Periodic Paralyses by Frank Lehmann-Horn, Reinhardt Rudel and Karin Jurkat-Rott.

Thank you Professor Dr. Lehmann-Horn, Dr. Jurkat-Rott and Dr. Rudel for your kind permission to include this valuable information on our website!

Do you suspect that you might have periodic paralysis? The periodic paralyses are a rare group of disorders and there are many conditions which cause an imbalance in serum potassium. So how does the doctor tell the difference between paralysis or weakness caused by an ion channelopathy and any of the other numerous disorders, conditions and reactions which might produce the same symptoms?

Hospital Management Guidelines for Thyrotoxic Periodic Paralysis Patients

Patient's Name:

Birthdate: ________Height: _______Weight: _______

Address:

Primary Physician:

Emergency contact:  Your emergency contact should be someone who can speak for you when you cannot advocate for yourself.
Name:                                                                               Relationship:
home phone#                                         cell #                            FAX:

A True Endocrine Emergency

Patients with Thyrotoxic Hypokalemic Periodic Paralysis typically present at the ER with an acute episode of paralysis involving the muscles of the extremities and limb girdles. The lower limbs are more frequently and severely involved than the upper. Weakness may be asymmetrical. Proximal strength is more severely impaired than distal strength. Respiratory and cardiac arrest are possible. Lie patient in coma position to avoid aspiration. 

Cardiac Signs