Paramyotonia

Paramyotonia Congentia and Paramyotonia Syndromes

Anaesthesia and peri-operative care in the primary Periodic Paralysis Disorders

Adverse reactions to anaesthesia, including life-threatening events, are a serious concern for patients with Periodic Paralysis Disorders. Many of our patients report serious events associated with anesthesia and side effects which may linger for days to months.

There is rarely time for a surgical team to comb through journals and texts for the specialized knowledge needed to care for the patient with periodic paralysis.

Converting Mg of Potassium to MeQ or MmoL

 

Source: Levitt, Jacob O, Practical aspects in the management of hypokalemic periodic paralysis. Journal of Translational Medicine 2008, 6:18 doi:10.1186/1479-5876-6-18:

The Use of Bactrim in the Periodic Paralyses

The antibiotic Bactrim is well-known to produce muscle weakness, even paralysis in patients with Hypokalemic Periodic Paralysis.

Focal and abnormally persistent paralysis associated with congenital paramyotonia

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. Focal paresis has sometimes already been described. We report a case with atypical clinical manifestation comprising paramyotonia and cold-induced persistent and focal paralysis.

Patient Medical Information Template in Spanish

Our Spanish-speaking patients may access the Spanish language Medical Information Template. It is in Open Document Format, so patients can add their own information to the template. Thanks to Barbara Baker and friends for translating our Patient's Information Sheet for us.

Leaky Channels make weak muscles

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.

Hospital Management Guidelines for Paramyotonia Congenita Patients

 

Hospital Management Guidelines for Paramyotonia Congenita 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:

New Findings Suggest Genetics Behind Drug Response

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

Measuring quality of life impairment in periodic paralyses


Measuring quality of life impairment in skeletal muscle channelopathies.


Eur J Neurol. 2012 May 19. doi: 10.1111/j.1468-1331.2012.03751.x. [Epub ahead of print]

Sansone VA, Ricci C, Montanari M, Apolone G, Rose M, Meola G; INQoL Group.
Source
Department of Neurology, University of Milan, IRCCS Policlinico San Donato, Milan, Italy.


Abstract
   Link to pdf copy of entire article 

BACKGROUND AND PURPOSE:

The Action Potential - A Movie!

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.

Pages

Subscribe to RSS - Paramyotonia