Hyperkalaemic periodic paralysis: clinical features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Hyperkalaemic periodic paralysis: clinical features. It is a sodium channelopathy. Fully referenced neurology checklist.

Genetics

  • It is a sodium channelopathy
  • It is caused by mutations in the SCN4A gene
  • The transmission is autosomal dominant
  • It results in recurrent attacks of weakness
  • Sporadic cases have been reported
  • It may overlap with paramyotonia congenita (HyperPP/PMC)

Onset features

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Attack pattern

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Attack triggers

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Attack features

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Inter-ictal features

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Differential diagnosis

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References

  1. Bertorini TE. Neuromuscular Disorders: Treatment and Management. Elsevier 2011 Philadelphia p297.
  2. Venance SL, Cannon SC, Fialho D, et al. The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain 2006; 129:8-17.
  3. Meola G, Hanna MG, Fontaine B. Diagnosis and new treatment in muscle channelopathies. JNNP 2009; 80:360-365.
  4. Segawa K, Nishiyama M, Mori I, Kubota T, Takahashi MP. Hyperkalemic periodic paralysis associated with a novel missense variant located in the inner pore of Nav1.4. Brain Dev 2023; 45:205-211. 
  5. Quiroga-Carrillo M, Correa-Arrieta C, Ortiz-Corredor F, Suarez-Obando F. Hyperkalemic periodic paralysis: case report with a SCNA4 gene mutation and literature review. Case Rep Genet 2020; 2020:8843410.
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