Hyperkalemic Periodic Paralysis

Disease Description and Diagnostic Criteria

http://neuromuscular.wustl.edu/mother/activity.html#hrpp

Hyperkalemic Periodic Paralysis: Neuromuscular Disease Center: Washington University School of Medicine, St. Louis, MO:  http://neuromuscular.wustl.edu/

http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=hyper-pp


Differential Diagnosis

Differential Diagnosis can be read here


Diagnostic Work Up

Diagnostic Work Up

Involve an Endocrinologist to rule out adrenal causes of hyperkalemia.


Emergency Management

Hyperkalemic Periodic Paralysis Management: Acute Attack

Control of hyperkalemia:

  • Consider just giving candy bar and waiting. Depends on degree of paralysis.
  • Insulin (with glucose to prevent hypoglycemia)
  • Albuterol nebulizer
  • Sodium Bicarbonate – 44-50 mEq over 5 min.
  • Hypertonic saline
  • Hydrochlorothiazide
  • Calcium administration intravenously (1 amp of 10% calcium glucoate) – under cardiac monitoring and only if EKG shows signs of severe hyperkalemia
  • Position patient comfortably
  • EKG for rhythm, especially QT interval.
  • Monitor serum potassium q30min to120min, depending on condition of patient and medications being administered.

Do not leave patient unattended!

Condition can change for better or worse rapidly. Risk of aspiration.

If associated with myoclonus (jerks), consider small dose of benzodiazepine as jerk represents exercise and trigger of paralysis is rest after exercise.

For myotonia, keep muscles warm (temperature).


Therapy

Some experts feel that there is no role for kayexalate, a potassium binding resin, in the treatment of hyperkalemic periodic paralysis.  This is because kayexalate is relatively slow acting and corrective shifts in potassium needed for the treatment of attacks of hyperkalemic periodic paralysis must be rapid, as induced by glucose and either endogenous or exogenous insulin or albuterol.  Chronic potassium lowering is achieved with potassuim-wasting diuretics rather than a potassium binding resin.

Behavioral Managment:

Some patients find that sleeping late on weekends triggers attacks (perhaps from prolonged rest or from too long a time without eating).  HyperPP patients find that keeping their weekday waking schedule during the weekends helps to avoid weekend morning attacks.  Some find that even getting up just to eat and then returning to bed can be helpful.


Useful Articles

The following survey was published in Journal of Neurology


Additional Resources

The following survey was published in Journal of Neurology:

http://link.springer.com/content/pdf/10.1007%2Fs00415-013-7025-9.pdf