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The Information for Medical Professionals Section

Andersen-Tawil Syndrome

This comprehensive article is from the National Organization for Rare Disorders (NORD)

NORD gratefully acknowledges Rabi Tawil, MD, University of Rochester Medical Center, Professor of Neurology for assistance in the preparation of this report.

Synonyms of Andersen-Tawil Syndrome

Andersen Syndrome
ATS
Long QT Syndrome 7
LQTS7
General Discussion

Summary

Anaesthesia and peri-operative care in the primary Periodic Paralysis Disorders

Adverse reactions to anaesthesia, including life-threatening events, are a serious concern for patients with Periodic Paralysis Disorders. Many of our patients report serious events associated with anesthesia and side effects which may linger for days to months.

There is rarely time for a surgical team to comb through journals and texts for the specialized knowledge needed to care for the patient with periodic paralysis.

FDA Approves Taro’s Keveyis™ (dichlorphenamide) for Primary Periodic Paralysis

FDA Approves Taro’s Keveyis™ (dichlorphenamide) for Primary Hyperkalemic and Hypokalemic Periodic Paralysis

Orphan Drug is First Approved Treatment for Patients Living with Rare Debilitating Disease
 

Keveyis is now supplied free of charge to US patients by Taro Pharmaceuticals. The process is handled by Envoy Health.

The numbers for Envoy are: 1-855-768-9727 and 1-810-553-4090.

You need a prescription from your doctor, but call Envoy first for instructions on how to apply and proceed.

Converting Mg of Potassium to MeQ or MmoL

 

Source: Levitt, Jacob O, Practical aspects in the management of hypokalemic periodic paralysis. Journal of Translational Medicine 2008, 6:18 doi:10.1186/1479-5876-6-18:

The Use of Bactrim in the Periodic Paralyses

The antibiotic Bactrim is well-known to produce muscle weakness, even paralysis in patients with Hypokalemic Periodic Paralysis.

Long-time exercise test in the diagnosis of periodic paralysis

Ding Z, Liu M, Cui L. Reference value of long-time exercise test in the diagnosis of primary periodic paralysis. Chin Med J (Engl). 2014;127(18):3219-23.

Abstract

BACKGROUND:
The long-time exercise test (ET) is used to diagnose the primary periodic paralyses (PPs). However the reference values of ET are many and various. This study aimed to investigate the reference value of long-time ET in the diagnosis of PPs.

Dichlorphenamide: A Review in Primary Periodic Paralyses.

Dichlorphenamide: A Review in Primary Periodic Paralyses. Greig SL; Drugs. 2016 Mar;76(4):501-7. doi: 10.1007/s40265-016-0559-2.

Abstract

Focal and abnormally persistent paralysis associated with congenital paramyotonia

Focal and abnormally persistent paralysis associated with congenital paramyotonia.

Magot A1, David A2, Sternberg D3, Péréon Y1.

British Medical Journal Case Report 2014 Jun 17;2014.

Abstract

Mutations of the skeletal muscle voltage-gated sodium channel (NaV1.4) are an established cause of several clinically distinct forms of periodic paralysis and myotonia. Focal paresis has sometimes already been described. We report a case with atypical clinical manifestation comprising paramyotonia and cold-induced persistent and focal paralysis.

GUIDELINES ved indlæggelse for Hypokalemisk Periodika Paralyse Patient

GUIDELINES ved indlæggelse for Hypokalemisk Periodika Paralyse Patient

Navn & CPRnr
Højde:  cm
Vægt:  kg
Adresse:
Egen læge:
Emergency contact: navn (tlf: )
+ evt personlige assistent og/eller ledsager:

Akut behandling af et hypokalemisk paralytisk anfald

Akut behandling af et hypokalemisk paralytisk anfald

Første skridt:
1. Åndedræts- eller hjerteanfald er mulige
2. Tjek hjerte og vejrtrækning - dilatation i hjertet kan forårsage høj puls og lavt blodtryk.
3. Foretag måling af hjerterytme/EKG.
4. Mål elektrolytter for at bestemme serum K+
5. Giv ilt - blodprøve fra fingeren afspejler måske ikke iltmætningen pga. dilatation
6. Anbring patienten som ved koma for at undgå kvælning.

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