Familial Periodic Paralysis

  • Louis Ptáček
  • Robert C. Griggs


The periodic paralyses have traditionally been divided into hypokalemic, hyperkalemic, normokalemic, and paramyo-tonic forms.1 Over the past decade, a combination of electrophysiologic and molecular biologic studies have clarified the classification of the disease (Table 31.1). It has become apparent that there are two broad categories of disease: hypokalemic periodic paralysis and hyperkalemic periodic paralysis. All forms of periodic paralysis are either autosomal dominantly inherited or occur as sporadic cases that are probably the result of new mutations. Hyperkalemic periodic paralysis usually results from a disorder of the skeletal muscle, voltage-gated sodium channel. The molecular alterations have been defined for most cases.2,3 It is becoming clear that a number of disorders once considered separate entities are in fact allelic to hyperkalemic periodic paralysis including: paramyotonia congenita4,5 and most recently, a form of myotonia without periodic paralysis that is potassium-sensitive.6,7 There are, however, a small proportion of patients with hyperkalemic periodic analysis that is not allelic.8


Sodium Channel Chloride Channel Periodic Paralysis Episodic Ataxia Sodium Channel Gene 
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Copyright information

© Plenum Press, New York 1996

Authors and Affiliations

  • Louis Ptáček
    • 1
  • Robert C. Griggs
    • 2
  1. 1.Department of Neurology, Human Molecular Biology and GeneticsThe University of UtahSalt Lake CityUSA
  2. 2.Department of NeurologyUniversity of RochesterRochesterUSA

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