Last updated Tuesday, July 3rd, 2012
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
BACKGROUND AND PURPOSE:
Last updated Tuesday, July 3rd, 2012
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.
Last updated Thursday, June 21st, 2012
1) Patient has "attacks" of weakness and/or floppy paralysis.
2) First attacks occur by the age of 16 in 65% of patients.
Last updated Wednesday, August 31st, 2011
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!
Last updated Sunday, July 17th, 2011
Clinical Synopsis
Thyrotoxic periodic paralysis (THKPP) is an uncommon disorder characterized by simultaneous thyrotoxicosis, hypokalemia, and paralysis that occurs primarily in males of Asian descent, including patients of Japanese, Chinese, Vietnamese, Korean and Filipino ancestry.
Last updated Saturday, July 2nd, 2011
Introduction:
The major features found in all the periodic paralyses are:
1. Attacks almost always begin before the age of 25, though occasionally later in life. ATS patients can develop first symptoms at any age.
2. Serum potassium fluctuates shortly before an attack, but serum potassium level will normalize before the attacks resolves and will be within the normal range between attacks. This is not a disorder of the blood but one in which there is an inappropriate shift of fluid, K+ and NA+ during attacks.
Last updated Wednesday, June 29th, 2011
Andersen-Tawil Syndrome (ATS) is a genetic disorder which causes a distinctive pattern of features;
1) Episodes of muscle weakness and/or flaccid paralysis (i.e periodic paralysis)
2) Certain kinds of heart rhythm disturbances and
3) Differences in facial and body structure.
Last updated Tuesday, June 28th, 2011
Clinical Signs
Hyperkalemic Periodic Paralysis is a genetically determined disorder which causes attacks of weakness and flaccid paralysis. Attacks last from 10 min to 1 hour, very rarely up to 1-2 days. Some patients experience only a few attacks over their lifetime, others have attacks every day. Diagnosis is suggested by attacks of weakness during which the patient's serum potassium is above 5.0 mmol/l or rises at least .5 of a mmol/l above their normal level when not in an attack.
Last updated Tuesday, June 28th, 2011
Hypokalemic periodic paralysis (HypoKPP) is one of the primary forms of periodic paralysis, caused by one or more mutations in the calcium, sodium or potassium ion channels in muscle membrane.
Features of Hypokalemic Periodic Paralysis
There are two forms of HypoKPP, a paralytic form and a myopathic form.
Last updated Monday, June 27th, 2011
Are Weakness and Fatigue Between Paralytic Attacks Symptoms of Periodic Paralysis?
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