Hypokalemic

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.

Periodic Paralysis Patients at Increased Risk of MH

Patients with Hyperkalemic periodic paralysis and Paramyotonia Congenita are at increased risk for Malignant Hyperthermia (MH) during surgery. 

Patients who take potassium chloride long-term, i.e., are at risk for developing vitamin B-12 deficiency. Potassium supplements such as K-Dur, Micro-K, Slow-K, K-Lyte, etc. interfere with the absorption of vitamin B-12 and can eventually lead to the depletion of body stores of this crucial vitamin. ^{1, 2, 3}

The regular colonoscopy prep can cause a hypokalemic episode in a patient with hypokalemic periodic paralysis. This colonoscopy prep, adapted to avoid hypokalemia, has been used by numerous patients with HypoKPP on the HKPP Listserv without provoking an episode afterwards. 

Colonoscopy Prep:

Day 1 Normal diet with an herbal tea laxative before bed.

Day 2 No solid foods, milk products or liquids with red or purple coloring; Suggested liquids are low sodium chicken stock, tea without milk, and water throughout the day.

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 Two Case Studies - HypoKPP

Patient #1 Three Pregnancies:

Familial HypoKPP Symptoms began at age of 13 years with three paralytic episodes of 48-72 hour duration.

Are your symptoms worse with your menses? No

Pregnancy No 1: Age 20

Diagnosed at time of this pregnancy: Yes

Episodes consisted of minor weakness and fatigue once or twice a month. No medications during pregnancy. Maintained normal activity. Uncomplicated labour with forceps delivery of 7 lb. healthy male child three weeks short of due date.

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.