Comparative Features of the Periodic Paralyses
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| Form | Effect of K+ | Age at Onset | What triggers | What Relieves | Length of Attacks | Myotonia or Paramyotonia | Hypertrophy | Inter-attack Weakness | Possible Cardiac Problems During Attacks | Therapy |
|---|---|---|---|---|---|---|---|---|---|---|
| HypoKPP | Relieves weakness | Most by 25 years | “Fast” Carbs, rest after exertion, sleep, insulin | High K+ food, mild exercise | 1–72 hours | Rarely, but in eyelids often | Yes | Yes, after age 35 | ST depression, T flattens, U rises | K+ sparing diuretics, Diamox, Daranide, K+ |
| HyperKPP | Provokes weakness | First decade | Hunger, K+ in foods, rest after exercise, immobility | Sugar, mild exercise | 5 min – 12 hours | Myotonia in grip and exercised muscles | Yes | Yes | T tall, “tented” R falls, QRS/PR increases, P gone | K+ wasting diuretics, Diamox, Daranide, Albuterol |
| PMC (Hyper) | Provokes weakness | First two years | Exercise, K+ | Sugar, rest | Minutes – days | Face, eyes, jaw, hands. Worsens with cold and activity | Yes | Yes | May show signs of Hyperkalemia | K+ wasting diuretics, Mexiletine |
| PMC von Eulenburg | Relieves weakness | First two years | Exercise, LOW K+ | K+ rest | Minutes – weeks | May be absent at room temp. | Yes | Yes | May show signs of Hypokalemia | K+ sparing diuretics, Mexiletine, K+ |
| Andersen’s syndrome | Provokes weakness while relieving arrhythmia and/or weakness in some patients. | At any age | Varies from patient to patient. Exercise, sleep K+ in some, low K+ in others | Varies from patient to patient. | Varies | No | No | Yes | LQTc, ectopy, bigeminy, tachycardia, (in or out of attack) | Varies, usually beta blockers + treatment for weakness |
| Thyrotoxic HypoKPP | Relieves weakness | At any age, 20–40 year–old asian, native American, 85% males | Hyperthyroidism may be sub–clinical, high carbohydrate meal and exertion. | K+, Treat thyroid condition | Hours to days | No | No | Yes | During attacks ST depression, T wave flattens, U wave appears, sinus tachycardia | Methimazole, Propranolol, K+, until anti–thyroid treatment “radio–iodine 131 or surgery” |
| Acetazolamide– responsive Paramyotonia Congenita |
Provokes pain and stiffness, No weakness | ? | K+, activity | Diamox | ? | Worse with activity | Yes | ? | ? | Diamox |
| Myotonia Fluctuans | Provokes stiffness, NO weakness | K+ | ? | Mild, varies | ? | Diamox, K+ wasting diuretics | ||||
| Myotonia Permanens | Provokes severe myotonia | ? | K+ | ? | Constant | Severe, continuous | Marked | ? | ? | Diamox, K+ wasting diuretics |