Andersen-Tawil

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.

Section 2. The Periodic Paralysis International/HKPP Listserv Survey: results and discussion.

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.

In July of 1998 we conducted a survey of 64 self-reported clinically diagnosed periodic paralysis patients, all members of the HKPP Listserv. The questionnaire underwent no formal validation process, nor was the data assessed by personnel trained in this field. The patients surveyed were drawn from several countries and across several racial/ethnic lines and backgrounds.

The Compound Muscle Action Potential (CMAP)  test (aka the Exercise EMG) is now being used to diagnose the periodic paralyses. When done correctly this test is reported to positively identify 70%-80% of patients.

Part Four Case Studies - ATS

Patient #18 Two Pregnancies: ATS2;

Hypokalemic episodes Symptoms began in early childhood;

Are your symptoms worse with your menses? Yes

Pregnancy No 1: Age 32 Diagnosed at time of this pregnancy: No

Part One

There is little available medical literature to guide the physician in the care of the pregnant patient who has periodic paralysis. The first part of this packet consists of abstracts and quotations drawn from current literature. But this information is limited. Because Periodic Paralysis International has a number of clients who have given birth we felt it might be helpful to survey them about their experiences. The second, third and forth parts of this packet consists of case studies of women with periodic paralysis who have given birth.

Description of Myoclonus

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 attempts to move. The twitching cannot be controlled by the person experiencing it.

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 paralyses is described in some of the literature as prominent or constant.

What happens during an attack of Periodic Paralysis?

People with Periodic Paralysis (PP) have episodes in which their muscles become weak or paralyzed in response to variations in the amount of potassium in their blood. These variations occur during sleep, they may result from food the person has eaten, from a sudden change in a person's activity level, from rest after activity, emotional stress, from becoming chilled, or a number of other factors. Potassium levels vary all the time.