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The Myopathy of Hyperkalemic Periodic Paralysis: An Electron Microscopic Study

The Myopathy of Hyperkalemic Periodic Paralysis: An Electron Microscopic Study Abstract HYPERKALEMIC periodic paralysis has been recognized as a distinct disorder only since 1951 when several generations of a family were described suffering from episodes of periodic paralysis without an accompanying fall in the serum potassium.1 Gamstorp further elucidated the main clinical features of the disease and established that the serum potassium was elevated during the paralytic attack.2,3 Later reports emphasized the common occurrence of myotonic features4,5 and that permanent myopathic weakness involving the abdominal and proximal limb girdle muscles may occur.6,7 Early muscle biopsies failed to reveal significant histopathological alterations.8,9 However, more recent reports have demonstrated infrequent central vacuolation of minor proportions with some nonspecific myopathic features7,10; these changes are similar to, but less marked, than those in hypokalemic periodic paralysis. Publications on the fine structure of skeletal muscle in this condition have shown few abnormalities11 or vacuoles possibly arising from the References 1. Tyler, F.H., et al: Studies in Disorders of Muscle: VII. Clinical Manifestations and Inheritance of a Type of Periodic Paralysis Without Hypotassemia , J Clin Invest 30:492, 1951.Crossref 2. Gamstorp, I.: Adynamia Episcodica Hereditaria , Acta Paediat 45( (suppl 108) ):1, 1956.Crossref 3. Gamstorp, I.: A Study of Transient Muscular Weakness , Acta Neurol Scand 38:3, 1962.Crossref 4. Van der Meulen, J.P.; Gilbert, J.F.; and Kane, C.A.: Familial Hyperkalemic Periodic Paralysis With Myotonia , New Eng J Med 264:1, 1961.Crossref 5. Van't Hoff, W.: Familial Myotonic Periodic Paralysis , Quart J Med 31:385, 1962. 6. McArdle, B.: Adynamia Episodica Hereditaria and Its Treatment , Brain 85:121, 1962.Crossref 7. Pearson, C.M.: The Periodic Paralyses: Differential Features and Pathological Observations in Permanent Myopathic Weakness , Brain 87:341, 1964.Crossref 8. Gamstorp, I., et al: Adynamia Episodica Hereditaria , Amer J Med 23:385, 1957.Crossref 9. Klein, R.; Egan, T.; and Usher, P.: Changes in Sodium, Potassium and Water in Hyperkalemic Familial Periodic Paralysis , Metabolism 9:105, 1960. 10. Bekeny, G.: über irreversible Muskelveränderungen bei der paroxysmalen Luhmung auf Grund bioptischer Muskeluntersuchungen , Deutsch Z Nervenheilk 182:119-154, 1961. 11. Jaffurs, W.J., et al: Hyperkalemic Paralysis (Adynamia Episodica Hereditaria): Ultrastructural Studies of Muscle in Two Cases , Metabolism 12:740, 1963. 12. Van Bogaert, L., et al: Syndrome Myopathique au cours d'une maladie de Gamstorp: Etude Anatomique , Acta Neurol Belg 67:133, 1967. 13. Shy, G.M., et al: Studies in Familial Periodic Paralysis , Exper Neurol 3:53, 1961.Crossref 14. Howes, E.L., Jr.; Price, H.M., and Blumberg, J.M.: Hypokalemic Periodic Paralysis: Electron Microscopic Changes in the Sarcoplasm , Neurology 16:242, 1966.Crossref 15. Engel, A.G.: Electron Microscopic Observations in Primary Hypokalemic and Thyrotoxic Periodic Paralyses , Mayo Clin Proc 41:797, 1966. 16. Engel, W.K.: Review of Current Concepts of Myopathies , Clin Orthop 39:100, 1965. 17. Macdonald, R.D.; Rewcastle, N.B.; and Humphrey, J.G.: The Myopathy of Hypokalemic Periodic Paralysis : An Electron Microscopic Study , to be published. 18. Odor, D.L.; Patel, A.N.; and Pearce, L.A.: Familial Hypokalemic Periodic Paralysis With Permanent Myopathy: A Clinical and Ultrastructural Study , J Neuropath Exp Neurol 26:98, 1967.Crossref 19. Schotland, D.L.: Ultrastructural Abnormalities in Myotonic Dystrophy Including an Unusual T System Alteration , J Neuropath Exp Neurol 27:109, 1968. 20. Pellegrini, C., and Franzini, C.: An Electron Microscopic Study of Denervation Atrophy in Red and White Skeletal Muscle Fibres , J Cell Biol 17:327, 1963.Crossref 21. Price, H.M.; Howes, E.L.; and Blumberg, J.M.: Ultrastructural Alterations in Skeletal Muscle Fibers Injured by Cold: The Acute Degenerative Changes , Lab Invest 13:1264, 1964. 22. Engel, W.K.: The Essentiality of Histo and Cytochemical Studies of Skeletal Muscle in the Investigation of Neuromuscular Disease , Neurology 12:778, 1962.Crossref 23. Iscove, N.; Rewcastle, N.B.; and Humphrey, J.G.: Cytoplasmic Bodies in Denervation Atrophy of Human Skeletal Muscle, to be published. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

The Myopathy of Hyperkalemic Periodic Paralysis: An Electron Microscopic Study

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References (22)

Publisher
American Medical Association
Copyright
Copyright © 1968 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1968.00480030052005
Publisher site
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Abstract

Abstract HYPERKALEMIC periodic paralysis has been recognized as a distinct disorder only since 1951 when several generations of a family were described suffering from episodes of periodic paralysis without an accompanying fall in the serum potassium.1 Gamstorp further elucidated the main clinical features of the disease and established that the serum potassium was elevated during the paralytic attack.2,3 Later reports emphasized the common occurrence of myotonic features4,5 and that permanent myopathic weakness involving the abdominal and proximal limb girdle muscles may occur.6,7 Early muscle biopsies failed to reveal significant histopathological alterations.8,9 However, more recent reports have demonstrated infrequent central vacuolation of minor proportions with some nonspecific myopathic features7,10; these changes are similar to, but less marked, than those in hypokalemic periodic paralysis. Publications on the fine structure of skeletal muscle in this condition have shown few abnormalities11 or vacuoles possibly arising from the References 1. Tyler, F.H., et al: Studies in Disorders of Muscle: VII. Clinical Manifestations and Inheritance of a Type of Periodic Paralysis Without Hypotassemia , J Clin Invest 30:492, 1951.Crossref 2. Gamstorp, I.: Adynamia Episcodica Hereditaria , Acta Paediat 45( (suppl 108) ):1, 1956.Crossref 3. Gamstorp, I.: A Study of Transient Muscular Weakness , Acta Neurol Scand 38:3, 1962.Crossref 4. Van der Meulen, J.P.; Gilbert, J.F.; and Kane, C.A.: Familial Hyperkalemic Periodic Paralysis With Myotonia , New Eng J Med 264:1, 1961.Crossref 5. Van't Hoff, W.: Familial Myotonic Periodic Paralysis , Quart J Med 31:385, 1962. 6. McArdle, B.: Adynamia Episodica Hereditaria and Its Treatment , Brain 85:121, 1962.Crossref 7. Pearson, C.M.: The Periodic Paralyses: Differential Features and Pathological Observations in Permanent Myopathic Weakness , Brain 87:341, 1964.Crossref 8. Gamstorp, I., et al: Adynamia Episodica Hereditaria , Amer J Med 23:385, 1957.Crossref 9. Klein, R.; Egan, T.; and Usher, P.: Changes in Sodium, Potassium and Water in Hyperkalemic Familial Periodic Paralysis , Metabolism 9:105, 1960. 10. Bekeny, G.: über irreversible Muskelveränderungen bei der paroxysmalen Luhmung auf Grund bioptischer Muskeluntersuchungen , Deutsch Z Nervenheilk 182:119-154, 1961. 11. Jaffurs, W.J., et al: Hyperkalemic Paralysis (Adynamia Episodica Hereditaria): Ultrastructural Studies of Muscle in Two Cases , Metabolism 12:740, 1963. 12. Van Bogaert, L., et al: Syndrome Myopathique au cours d'une maladie de Gamstorp: Etude Anatomique , Acta Neurol Belg 67:133, 1967. 13. Shy, G.M., et al: Studies in Familial Periodic Paralysis , Exper Neurol 3:53, 1961.Crossref 14. Howes, E.L., Jr.; Price, H.M., and Blumberg, J.M.: Hypokalemic Periodic Paralysis: Electron Microscopic Changes in the Sarcoplasm , Neurology 16:242, 1966.Crossref 15. Engel, A.G.: Electron Microscopic Observations in Primary Hypokalemic and Thyrotoxic Periodic Paralyses , Mayo Clin Proc 41:797, 1966. 16. Engel, W.K.: Review of Current Concepts of Myopathies , Clin Orthop 39:100, 1965. 17. Macdonald, R.D.; Rewcastle, N.B.; and Humphrey, J.G.: The Myopathy of Hypokalemic Periodic Paralysis : An Electron Microscopic Study , to be published. 18. Odor, D.L.; Patel, A.N.; and Pearce, L.A.: Familial Hypokalemic Periodic Paralysis With Permanent Myopathy: A Clinical and Ultrastructural Study , J Neuropath Exp Neurol 26:98, 1967.Crossref 19. Schotland, D.L.: Ultrastructural Abnormalities in Myotonic Dystrophy Including an Unusual T System Alteration , J Neuropath Exp Neurol 27:109, 1968. 20. Pellegrini, C., and Franzini, C.: An Electron Microscopic Study of Denervation Atrophy in Red and White Skeletal Muscle Fibres , J Cell Biol 17:327, 1963.Crossref 21. Price, H.M.; Howes, E.L.; and Blumberg, J.M.: Ultrastructural Alterations in Skeletal Muscle Fibers Injured by Cold: The Acute Degenerative Changes , Lab Invest 13:1264, 1964. 22. Engel, W.K.: The Essentiality of Histo and Cytochemical Studies of Skeletal Muscle in the Investigation of Neuromuscular Disease , Neurology 12:778, 1962.Crossref 23. Iscove, N.; Rewcastle, N.B.; and Humphrey, J.G.: Cytoplasmic Bodies in Denervation Atrophy of Human Skeletal Muscle, to be published.

Journal

Archives of NeurologyAmerican Medical Association

Published: Sep 1, 1968

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