Doctor

The Information for Medical Professionals Section

A Quick Overview of Hypokalemic Periodic Paralysis

 

1) Patient has "attacks" of weakness and/or floppy paralysis.

2) First attacks occur by the age of 16 in 65% of patients.

Periodic Paralysis and the New Mother

Anticipating the Baby

 

Expectant mothers and their doctors can take the secure thought that hundreds of women with periodic paralysis have been members of our Listserv since 1995, and an overwhelming majority of them have been mothers. To date not a single one has been unable to care for her child due to her periodic paralysis.

Meet Our Patients

Irm's Story

The PPI's Client Services Representative in The Netherlands is Irm. Irm and her son were clinically diagnosed with HypoKPP-plus in Germany in September 2011, but she has had symptoms since she was a young child. At the age of 7, she told her parents, “My head is awake but my body still sleeps”. 

Physician's Reading Room

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.

ECG Features in ATS Patients With KCNJ2 Mutations

Electrocardiographic Features in Andersen-Tawil Syndrome
Patients With KCNJ2 Mutations

Characteristic T-U–Wave Patterns Predict the KCNJ2 Genotype

Li Zhang, MD; D. Woodrow Benson, MD, PhD; Martin Tristani-Firouzi, MD; Louis J. Ptacek, MD;
Rabi Tawil, MD; Peter J. Schwartz, MD; Alfred L. George, MD; Minoru Horie, MD, PhD;
Gregor Andelfinger, MD; Gregory L. Snow, PhD; Ying-Hui Fu, PhD;
Michael J. Ackerman, MD, PhD; G. Michael Vincent, MD

Nondystrophic Myotonias and Periodic Paralyses

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.

Our thanks to Professor Dr. Lehmann-Horn, Dr. Jurkat-Rott and Dr. Rudel for your kind permission to include this valuable information on our website!

How is periodic paralysis diagnosed?

Do you suspect that you might have periodic paralysis? The periodic paralyses are a rare group of disorders and there are many conditions which cause an imbalance in serum potassium. So how does the doctor tell the difference between paralysis or weakness caused by an ion channelopathy and any of the other numerous disorders, conditions and reactions which might produce the same symptoms?

Management Strategies For Hypokalemic Periodic Paralysis

In 2009 Professor Dr. Frank Lehmann-Horn of Ulm University, Ulm Germany, gave a presentation on managing hypokalemic periodic paralysis at the Periodic Paralysis Association's conference held in Orlando Florida. This presentation contained so much valuable information in such a compact and understandable format that we asked Dr. Lehmann-Horn for his permission to include it on our website, for the benefit of physicians and patients.

Emergency Care for an Attack of Andersen-Tawil Syndrome

First Actions:

  • Respiratory and cardiac arrest are possible.
  • Call for Cardiac Monitoring/EKG
  • Draw electrolytes to determine serum K+
  • Provide 02 as thimble may not reflect 02 saturation due to cardiac dilitation
  • Lie patient in coma position to avoid aspiration.

Anesthesia in Hypokalemic Periodic Paralysis

Provided by Frank Lehmann-Horn MD, PhD

From
Lehmann-Horn F, Rüdel R, Jurkat-Rott K. Chapter 46: Nondystrophic myotonias and periodic paralyses. In: Myology, edited by AG Engel, C Franzini-Armstrong. McGraw-Hill, New York, 3rd edition, 2004, pp. 1257-1300.
and
Klingler W, Lehmann-Horn, Jurkat-Rott K. Complications of anesthesia in neuromuscular disorders. Neuromuscular Disord, 15:195-206, 2005.

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