HOKPP gene
occurs primarily in Asian males
incidence 2%
Symptoms:
- Episodic severe weakness at the shoulders and hips:
- occurs after sleep or rest
- Occurs on awakening
- rare during exercise
but triggered by rest after exercise.
- lasts 3 - 24 hours
- Cardiac arrhythmia
- Dyspnoea
- Dysphagia
- Eyelid myotonia
Precipitating factors:
- Exercise
- upper respiratory infections
- alcohol
- carbohydrate-rich foods
Investigation: Hypokalemia ( shift of K+ into cells due to increased Na/K-ATPase activity)
Management
Lifestyle Changes :
- low carbohydrate diet
- low sodium diet
- Avoid strenuous exercise
- Avoid alcohol.
- Avoid heavy exercise.
Oral Potassium given during an attack may stop the attack.
Taking potassium supplements will not prevent attacks.
Acetazolamide prevents attacks in many cases"