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May 1, 1987Muscle & NerveOpen Access

Adynamia episodica hereditaria with myotonia: A non‐inactivating sodium current and the effect of extracellular pH

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Population

Intact muscle fibers biopsied from a single patient with adynamia episodica hereditaria with…

Comparison

Changes in extracellular potassium… vs Normal muscle fibers

Design

Preclinical

Authors

FLFrank Lehmann‐HornUniversität UlmGKG. KütherMedizinische Hochschule HannoverKRK. RickerUniversity of Minnesota

Discussion

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Implication

Suggests pH modulation as potential strategy in periodic paralysis; leaves open translation from this animal model to patients.

Structured PICO

P
Population
Intact muscle fibers biopsied from a single patient with adynamia episodica hereditaria with electromyographic signs of myotonia
I
Intervention
Changes in extracellular potassium concentration, tetrodotoxin application, lowering of extracellular pH, and hydrochlorothiazide
C
Comparator
Normal muscle fibers
O
Outcome
Electrophysiological properties including membrane resting potential, intracellular sodium activity, force of contraction, and inward current characteristicssurrogate

The study identifies a non-inactivating sodium current as the mechanism for paralysis in adynamia episodica hereditaria, which can be antagonized by lowering extracellular pH.

Cite This Study

Lehmann‐Horn et al. (1987) studied this question.

synapsesocial.com/papers/6a70a248ac440176ef294cbbhttps://doi.org/10.1002/mus.880100414
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Two cases of adynamia episodica hereditaria: In vitro investigation of muscle cell membrane and contraction parameters1983 · 100 citations
  2. 2Hyperkalemic periodic paralysis. Relationships between changes in plasma water, electrolytes, insulin and catecholamines during attacks1980 · 43 citations
  3. 3Familial Hyperkalemic Paralysis with Myotonia1961 · 50 citations
  4. 4Properties of persistent sodium conductance and calcium conductance of layer V neurons from cat sensorimotor cortex in vitro1985 · 412 citations
  5. 5Regulation of plasma potassium in hyperkalemic periodic paralysis1979 · 22 citations