Submitted by deb on Sat, 06/25/2011 – 23:22 Pain Often Overlooked Pain is an often overlooked component of the periodic paralyses. Patients who report muscle pain in association with their episodes are too often told that the periodic paralyses are not painful despite many authoritative reports to the contrary. In fact the pain which accompanies the periodic…
Submitted by deb on Sun, 06/26/2011 – 22:13 Myoclonus is the sudden, involuntary jerking of a muscle or group of muscles. Myoclonic jerks may occur alone or in sequence, in a pattern or without pattern. They may occur infrequently or many times each minute. Myoclonus sometimes occurs in response to an external event or when a person…
Submitted by deb on Mon, 06/27/2011 – 20:54 Acetazolamide (aka Diamox) is frequently prescribed as therapy for the periodic paralyses. While most patients take this drug without incident it can interact with other drugs. Physicians should be aware of potential problems which might arise. Acetazolamide can decrease excretion of dextroamphetamine, anticholinergics, mecamylamine, ephedrine, mexiletine, or quinidine because…
Submitted by deb on Mon, 06/27/2011 – 02:57 Giorgio Buzzi, MD, Neurologist Buzzi G, Mostacci B, Sancisi E, Cirignotta F. Sleep complaints in Periodic Paralyses: a web survey. Functional Neurology 2001, 17 (3). From the Sleep Medicine Unit – Dept. of Neurology S.Orsola-Malpighi Hospital – University of Bologna, Italy. SUMMARY Section 1. Background: K+ and REM sleep homeostasis….
Submitted by deb on Wed, 09/07/2011 – 03:04 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. Textbook and Reviews Meola G, Hanna M G and Fontaine B. Diagnosis and Treatment of Muscle Channelopathies. J Neurol Neurosurg Psychiatry 2009;80:360–365. Dr Michael…
Submitted by deb on Thu, 07/26/2012 – 17:00 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,* The molecular composition and drug responses…
Submitted by deb on Wed, 03/13/2013 – 18:08 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…
Submitted by deb on Mon, 06/27/2011 – 02:22 Hao Cheng, MD The use of local anaesthetics on patients with periodic paralysis presents some unusual challenges. Many patients report that these agents trigger weakness or paralysis, a phenomenon which is substantiated in medical literature. Some patients also report that the agents do not produce the desired anaesthesia. The…
Submitted by deb on Fri, 08/12/2011 – 19:51 First Actions: Monitor closely, weakness can rapidly progress to paralysis, torsades de pointes, cardiac arrest and respiratory failure. Paralyzed patients may appear to be unconscious, but are awake and aware. Do not assume they cannot feel pain or hear conversations. Andersen-Tawil syndrome (ATS) is characterized by the triad of…
The Compound Muscle Action Potential (CMAP) test (aka the Exercise EMG) is used to diagnose the periodic paralyses. When done correctly this test is reported to positively identify 70%-80% of patients. The CMAP test is based on the observation that patients with Periodic Paralysis have a greater than normal increase in the compound muscle action…