Muscle membrane potentials in episodic adynamia

doi:10.1016/0013-4694(63)90071-3

Electroencephalography and Clinical Neurophysiology

Volume 15, Issue 3, June 1963, Pages 508-519

https://doi.org/10.1016/0013-4694(63)90071-3 Get rights and content

Abstract

1.
1. In vivo measurements of membrane potentials of human muscles (M. vastus med,; nerve block anaesthesia) have been performed in a healthy young man, a patient with periodic paralysis and a patient with episodic Adynamia (e.A.) Control measurements were performed in rats.
2.
2. The resting potentials of striated muscles of rats under general anaesthesia were − 79.9 ± 73.3 mV. In the healthy man (without general anaesthesia) the mean value was −87.4 ± 8.9 mV and in the patient with periodic paralysis it was −85.6 ± 6.1 mV in a free interval. During the attempt to provoke a paralytic attack in this patient the average dropped to −77.1 ± 8.2 mV, whereas the serum K⁺ fell from 4.25 to 3.17 mequiv./1. The questionable significance of this slight drop of membrane potential is discussed.
3.
3. In the patient with e.A. the resting potential, even in the free interval, (serum K⁺ 3.75 mequiv./l) was only −68.5 ± 5.9 mV. During an induced attack, with a rise in serum potassium to 6.9 mequiv./l, the average value dropped to −46.3 ± 6.6 mV and during a spontaneous attack, with a serum potassium of 5.24 mequiv./l, the mean value was −51.5 ± 6.3 mV.
4.
4. The significance of the results is discussed. The pathogenetic mechanism of e.A. can be explained by a disturbance of sodium permeability of muscle membranes.

Résumé

1.
1. Des mesures de potentiels des membranes des muscles humains (M. vastus med.; anesthésic par blocage des nerfs) ont été effectuées in vivo sur un jeune homme en bonne santé sur un malade atteint de paralysie familiale périodique, et sur un malade atteint d'adynamie épisodique (e.A.). Des mesures de controle ont été effectuées sur le rat.
2.
2. Les potentiels de repos des muscles striés chez le rat sous anésthésie générale étaient de −79.9 ± 7.3 mV. Chez l'homme en bonne santé (sans anésthésie générale) la valeur moyenne était de −87.4 ± 8.9 mV et chez le malade atteint de paralysie familiale périodique elle était de −85.6 ± 6.1 mV pendant un intervalle libre. Au cours d'une tentative pour provoquer une crise paralytique chez ce malade, la moyenne est tombée à −77.1 ± 8.2 mV, tandis que le sérum K⁺ est tombée de 4.25 à 3.17 méquiv./l. Une discussion porte sur le probléme de savoir si ce léger abaissement du potentiel du membrane est significatif ou non.
3.
3. Chez le malade atteint de e.A., le potentiel de repos, même pendant l'intervalle libre (sérum K⁺ 3.75 méquiv./l) était seulement de 68.5 ± 5.9 mV. Au cours d'une crise provquée, pendant laquelle le sérum K⁺ est monté á 6.9 méquiv./l, la valeur moyenne est tombée à 46.3 ± 6.6 mV, et au cours d'une crise spontanée, ou le sérum K⁺ était de 5.24 méquiv./l. la valeur moyenne était de 51.5 ± 6.3 mV.
4.
4. La signification des résultats est discutée. Le mécanisme pathogénique de e.A. peut s'expliquer par une modification de la perméabilité au sodium des membranes musculaires.

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2002, Neuromuscular Disorders
The periodic paralyses are rare disorders of skeletal muscle characterized by episodic attacks of weakness due to intermittent failure of electrical excitability. Familial forms of periodic paralysis are all caused by mutations in genes coding for voltage-gated ion channels. New discoveries in the past 2 years have broadened our views on the diversity of phenotypes produced by mutations of a single channel gene and have led to the identification of potassium channel mutations, in addition to those previously found in sodium and calcium channels. This review focuses on the clinical features, molecular genetic defects, and pathophysiologic mechanisms that underlie familial periodic paralysis.
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The myotonias and periodic paralyses are a diverse group of skeletal muscle disorders that share a common pathophysiological mechanism: all are caused by derangements in the electrical excitability of the sarcolemma. Mutations within coding regions of ion-channel genes have been identified recently as the underlying molecular defects in these heritable disorders. Chloride-channel mutations cause a reduction in the resting conductance, which enhances excitability and gives rise to myotonia. By contrast, missense mutations in the L-type Ca²⁺ channel reduce the electrical excitability of the fiber and cause a form of periodic paralysis. Mutations of the sodium channel impair inactivation of the channel, which, depending on the type and severity of the functional defect, results in either paralysis or myotonia.
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Functional expression of sodium channel mutations identified in families with periodic paralysis
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Two mutations in the sodium channel α subunit that have been implicated as the cause of periodic paralysis were studied by functional expression in a mammalian cell line. Both mutations disrupted inactivation without affecting the time course of the onset of the sodium current or the single-channel conductance. This is the same functional defect that was observed in myotubes cultured from affected patients and proves that these mutations are not benign polymorphisms. Unlike the currents in the myotubes, however, there was no consistent potassium dependence for the noninactivating component. These mutations also define new regions of the sodium channel α subunit that are involved in the process of inactivation.

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^☆: The work has been supported by the MDAA.

^∗: We wish to acknowledge gratefully the help of Dr. David Sheldon, who patiently carried out the operations, and of Dr. S. M. Chowdbury and Miss Diane Miller for technical assistance.

²: Dr. O. D. Creutzfeldt, Max-Planck-Institut für Psychiatrie, München 23, Germany.

³: Dr. W. M. Fowler, Department of Physical Medicine, UCLA Medical School, Los Angeles 24, Calif. (U.S.A.).

¹: Present address: Dr. B. C. Abbott, Department of Zoology, UCLA, Los Angeles 24, Calif. (U.S.A.).

⁴: Dr. C. M. Pearson, Department of Medicine, UCLA Medical School, Los Angeles 24, Calif. (U.S.A.).

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Article preview

Electroencephalography and Clinical Neurophysiology

Abstract

Résumé

References (23)

Unstable membrane potential in human myotonic muscle

Electroenceph. clin. Neurophysiol.

Studies in familial periodic paralysis

Exp. Neurol.

The effect of internal and external potassium concentration on the membrane potential of frog muscle

J. Physiol. (Lond.)

Potassium chloride movement and the membrane potential of frog muscle

J. Physiol. (Lond.)

The normal membrane resting potential of mammalian skeletal muscle measured in vivo

J. cell. comp. Physiol.

Some aspects of the pathophysiology of Adynamia episodica hereditaria

Dan. med. Bull.

Paresis and hyperexcitability in Adynamia episodica hereditaria

Neurology

Local activity at a depolarized nerve muscle junction

J. Physiol. (Lond.)

Die episodische Adynamie (Adynamia episodica hereditaria Gamstorp), eine familiäre hyperkalämische Lähmung

Fortschr. Neurol. Psychiat.

Membranpotentiale in menschlichen Muskeln bei gesunden Personen und Patienten mit Adynamia episodica hereditaria und periodischer Lähmung

Naturwissenschaften

A study of some electrophysiological properties of human intercostal muscle

J. Physiol. (Lond.)

Cited by (93)

Channelopathies of Skeletal Muscle Excitability

Channelopathies of skeletal muscle excitability

Myotonia and Periodic Paralysis

An expanding view for the molecular basis of familial periodic paralysis

Ion-channel defects and aberrant excitability in myotonia and periodic paralysis

Functional expression of sodium channel mutations identified in families with periodic paralysis

Muscle membrane potentials in episodic adynamiaPotentiels de membranes musculaires dans l'adynamie épisodique☆

Abstract

Résumé

Electroenceph. clin. Neurophysiol.

Exp. Neurol.

The effect of internal and external potassium concentration on the membrane potential of frog muscle

J. Physiol. (Lond.)

Potassium chloride movement and the membrane potential of frog muscle

J. Physiol. (Lond.)

The normal membrane resting potential of mammalian skeletal muscle measured in vivo

J. cell. comp. Physiol.

Some aspects of the pathophysiology of Adynamia episodica hereditaria

Dan. med. Bull.

Paresis and hyperexcitability in Adynamia episodica hereditaria

Neurology

Local activity at a depolarized nerve muscle junction

J. Physiol. (Lond.)

Die episodische Adynamie (Adynamia episodica hereditaria Gamstorp), eine familiäre hyperkalämische Lähmung

Fortschr. Neurol. Psychiat.

Membranpotentiale in menschlichen Muskeln bei gesunden Personen und Patienten mit Adynamia episodica hereditaria und periodischer Lähmung

Naturwissenschaften

A study of some electrophysiological properties of human intercostal muscle

J. Physiol. (Lond.)