Doctor

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 Three Case Studies - HyperKPP and PMC

Patient #11 Two Pregnancies:

Familial HyperKPP and PMC Von Eulenburg Symptoms began at age of 11 years

Are your symptoms worse with your menses? No

Pregnancy No 1: Age 27

Diagnosed at time of this pregnancy: No

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.

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.