Skip to main content
SpringerLink
Log in

Disorders of Muscle

The Periodic Paralyses

  • Chapter
Clinical Disorders of Membrane Transport Processes

Abstract

The periodic paralyses are characterized by episodic attacks of paralysis. They are usually classified as primary (familial) or secondary and, according to the associated change in serum potassium, as hypokalemic, hyperkalemic, or normokalemic. The pattern of electrolyte changes is usually similar from attack to attack in a given patient; however, rarely, a single patient will have paralysis associated with an elevated, depressed, or unchanged serum K+ concentration.(1) The different forms of periodic paralysis share several features: (1) the attacks may last from minutes to days and occur sporadically, (2) the paralysis can be focal or generalized, (3) the tendon reflexes are depressed, (4) respiratory and cranial nerve-innervated muscles are relatively spared, (5) rest after exercise can provoke an attack whereas continuous activity can allay paresis, (6) cooling of muscles may produce weakness, (7) whereas early in the course of the disease the patient may have normal strength between attacks, progressive proximal weakness can develop after repeated attacks in the primary forms of periodic paralysis.(2)

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Chesson, A. L., S. S. Schochet, and B. H. Peter. 1979. Biphasic periodic paralysis. Arch. Neurol. 36: 700–704.

    PubMed  Google Scholar 

  2. Pearson, C. M. 1964. The periodic paralysis: Differential features and pathological observations in permanent myopathic weakness. Brain 87: 341–358.

    PubMed  CAS  Google Scholar 

  3. Hartwig, H. 1875. Ober emien fall von intermittiereuder paralysis spinalis. Zentralbi. Med. Wiss. 13: 428.

    Google Scholar 

  4. Endo, M. 1977. Calcium release from the sarcoplasmic reticulum. Physiol. Rev. 57: 71–102.

    PubMed  CAS  Google Scholar 

  5. Johnsen, T. 1981. Familial periodic paralysis with hypokalemia: Experimental and clinical investigations. Dan. Med. Bull. 28: 127.

    Google Scholar 

  6. Talbot, J. H. 1941. Periodic paralysis. Medicine (Baltimore) 20: 85–99.

    Google Scholar 

  7. Sagild, U. 1963. Hypoglycemia induced by potassium administration during attacks of periodic paralysis. Acta Med. Scand. 173: 329–332.

    PubMed  CAS  Google Scholar 

  8. Engel, A. G. 1977. Hypokalemic and hyperkalemic periodic paralysis. In: Scientific Approach to Clinical Neurology. E. S. Goldensohn and S. H. Appel, eds. Lea & Febiger, Philadelphia. pp. 1742–1765.

    Google Scholar 

  9. Biermond, A., and A. P. Daniels. 1934. Familial periodic paralysis and its transition into spinal muscular atrophy. Brain 57: 91–109.

    Google Scholar 

  10. Allott, E. N., and B. McArdle. 1938. Further observations on familial periodic paralysis. Clin. Sci. Mol. Med. 3: 229–245.

    CAS  Google Scholar 

  11. Tones, C. F., R. C. Griggs, R. T. Moxley, and A. N. Bender. 1981. Hypokalemic periodic paralysis exacerbated by acetazolamide. Neurology (New York) 31: 1423–1428.

    Google Scholar 

  12. Van Buchem, F. S. P. 1957. The electrocardiogram and potassium metabolism. Am. J. Med. 23: 376–384.

    Google Scholar 

  13. Mathew, R., and P. S. Gupta. 1979. Periodic paralysis (report of six cases). J. Assoc. Physicians India 27: 663–667.

    PubMed  CAS  Google Scholar 

  14. Wolf, P., J. Griffiths, J. Koett, and J. Howell. 1979. The presence of serum creatine kinase 2 (MB) in hypokalemic familial periodic paralysis. Enzyme 24: 197–199.

    PubMed  CAS  Google Scholar 

  15. Resnick, J. S., and W. K. Engel. 1967. Myotonic lid lag in hypokalemic periodic paralysis. J. Neurol. Neurosurg. Psychiatry 30: 47–51.

    Google Scholar 

  16. McArdle, B. 1956. Familial periodic paralysis. Br. Med. Bull. 12: 226–229.

    PubMed  CAS  Google Scholar 

  17. Shy, M., T. Wanko, P. T. Rowley, and A. G. Engel. 1961. Studies in familial periodic paralysis. Exp. Neurol. 3: 53–121.

    Google Scholar 

  18. Engel, A. G., E. H. Lambert, J. W. Rosevear, and W. N. Tauxe. 1965. Clinical and EMG studies in a patient with primary hypokalemic periodic paralysis. Am. J. Med. 38: 626–640.

    PubMed  CAS  Google Scholar 

  19. Buruma, O. J. S., and G. T. A. M. Bots. 1978. Myopathy in familial hypokalemic periodic paralysis independent of paralytic attacks. Acta Neurol. Scand. 57: 171–179.

    PubMed  CAS  Google Scholar 

  20. Buruma, O. J. S., J. J. Schipperheyn, and G. T. A. M. Bots. 1981. Heart muscle disease in familial hypokalemic periodic paralysis. Acta Neurol. Scand. 64: 12–21.

    PubMed  CAS  Google Scholar 

  21. Chen, R. F. 1959. Familial periodic paralysis. Arch. Neurol. 1: 475–480.

    PubMed  CAS  Google Scholar 

  22. Satoyoshi, E., K. Murakami, H. Kowa, M. Kinoshita, and Y. Mishiyama. 1963. Periodic paralysis in hyperthyroidism. Neurology (Minneapolis) 13: 746–752.

    CAS  Google Scholar 

  23. Engel, A. G. 1966. Electron microscopic observations in primary hypokalemic and thyrotoxic periodic paralysis. Mayo Clin. Proc. 41: 797–808.

    PubMed  CAS  Google Scholar 

  24. Engel, A. G. 1961. Thyroid function and periodic paralysis. Am. J. Med. 30: 327–333.

    PubMed  CAS  Google Scholar 

  25. Okihiro, M. M., and R. A. Nordyke. 1966. Hypokalemic periodic paralysis experimental precipitation with sodium lithyronine. J. Am. Med. Assoc. 198: 277–279.

    Google Scholar 

  26. Layzer, R. B., and E. Goldfield. 1974. Periodic paralysis caused by abuse of thyroid hormone. Neurology (Minneapolis) 24: 949–955.

    CAS  Google Scholar 

  27. Ober, K. P., and J. F. Hennessy. 1981. Jodbasedow and thyrotoxic periodic paralysis. Arch. Intern. Med. 141: 1225–1227.

    PubMed  CAS  Google Scholar 

  28. Ruff, R. L. 1979. Insulin-induced weakness in hypokalemic myopathy. Ann. Neurol. 6: 139–140.

    PubMed  CAS  Google Scholar 

  29. Conn, J. W. 1955. Presidential address. I. Painting background. II. Primary aldosteronism. J. Lab. Clin. Med. 45: 3–28.

    PubMed  CAS  Google Scholar 

  30. Raskin, R. J., J. T. Tesan, and O. J. Lawless. 1981. Hypokalemic periodic paralysis in Sjogren’s syndrome. Arch. Intern. Med. 141: 1671–1673.

    PubMed  CAS  Google Scholar 

  31. Milne, M. D., S. W. Stanbury, and A. E. Thompson. 1952. Observations on Fanconi syndrome and renal hyperchloremic acidosis in adults. Q. J. Med. 21: 61–74.

    PubMed  CAS  Google Scholar 

  32. Bull, G. M., A. M. Joekes, and K. G. Lowe. 1950. Renal function studies in acute tubular necrosis. Clin. Sci. 9: 379–385.

    Google Scholar 

  33. Sataline, L. R., and J. M. Simonelli. 1961. Potassium paresis following ureterosignoidostomy. J. Urol. 85: 559–564.

    PubMed  CAS  Google Scholar 

  34. Cohen, T. 1959. Hypokalemic muscle paralysis associated with administration of chlorothiazide. J. Am. Med. Assoc. 170: 2083–2086.

    PubMed  CAS  Google Scholar 

  35. Salassa, R. M., V. R. Mattox, and J. W. Rosevear. 1962. Inhibition of the `mineralo-corticoid’ activity by licorice by spironolactone. J. Clin. Endocrinol. Metab. 22: 1156–1159.

    PubMed  CAS  Google Scholar 

  36. Goulon, M., M. Rapin, J. Lissac, J. J. Pocidolo, and O. Mantel. 1962. Quadriplegia with hypokalemia and hypokalemic acidosis secondary to the absorption of ammonium chloride over a three-year period. Bull. Soc. Hop. Med. Paris 113: 113–115.

    Google Scholar 

  37. Keye, J. D. 1952. Death in potassium deficiency. Circulation 5: 766–769.

    PubMed  Google Scholar 

  38. Keyloun, V. E., and W. J. Grace. 1967. Villous adenoma of the rectum associated with severe electrolyte imbalance. Am. J. Dig. Dis. 12: 104–108.

    PubMed  CAS  Google Scholar 

  39. Verner, V. J., and A. B. Morrison. 1958. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am. J. Med. 25: 374–382.

    PubMed  CAS  Google Scholar 

  40. Schwartz, W. B., and A. S. Relman. 1953. Metabolic and renal studies in chronic potassium depletion resulting from overuse of laxatives. J. Clin. Invest. 32: 258–265.

    PubMed  CAS  Google Scholar 

  41. Lewi, Z., and Y. Bar-Khayim. 1964. Food poisoning from barium carbonate. Lancet 2: 342–344.

    PubMed  CAS  Google Scholar 

  42. Gamstorp, I., M. Hauge, H. F. Helweg-Larsen, H. Mjönes, and U. Sagild. 1957. Adynamia episodica hereditaria. Am. J. Med. 23: 385–390.

    PubMed  CAS  Google Scholar 

  43. Armstrong, F. B. 1962. Hyperkalemic familial periodic paralysis. Ann. Intern. Med. 57: 455–461.

    PubMed  CAS  Google Scholar 

  44. McArdle, B. 1962. Adynamia episodica hereditaria and its treatment. Brain 85: 121–148.

    Google Scholar 

  45. Layzer, R. B., R. E. Lovelace, and L. P. Rowland. 1967. Hyperkalemic periodic paralysis. Arch. Neurol. 16: 455.

    PubMed  CAS  Google Scholar 

  46. Klein, R., and J. E. Haddow. 1970. Arterial-venous differences in serum potassium concentrations in paramyotonia. Pediatr. Res. 4: 286–294.

    PubMed  CAS  Google Scholar 

  47. Thomas, C. 1978. Recent findings on the geneology, clinical aspects, and histology of a family with periodic hyperkalemic paralysis. Rev. Neurol. 134: 45–58.

    PubMed  CAS  Google Scholar 

  48. Dyken, M. L., and G. D. Timmons. 1963. Hyperkalemic periodic paralysis with hypocalcemic episode. Arch. Neurol. 9: 508–517.

    PubMed  CAS  Google Scholar 

  49. Riggs, J. E., R. T. Moxley, R. C. Griggs, and F. A. Homer. 1981. Hyperkalemic periodic paralysis: An apparent sporadic case. Neurology (New York) 31: 1157–1159.

    CAS  Google Scholar 

  50. Hudson, A. J., K. P. Strickland, and A. J. Wilensky. 1967. Serum enzyme studies in familial hyperkalemic periodic paralysis. Clin. Chim. Acta 17: 331–336.

    PubMed  CAS  Google Scholar 

  51. Streeten, D. H. P., T. G. Dalakos, and H. Fellerman. 1971. Studies on hyperkalemic periodic paralysis: Evidence of changes in plasma Na and CI and induction of paralysis by adrenal glucocorticoids. J. Clin. Invest. 50: 142–155.

    PubMed  CAS  Google Scholar 

  52. McDonald, R. D., N. B. Rewcastle, and J. G. Humphrey. 1968. The myopathy of hyperkalemic periodic paralysis. Arch. Neuol. 19: 274–279.

    Google Scholar 

  53. Carson, M. J., and C. M. Pearson. 1964. Familial hyperkalemic periodic paralysis with myotonic features. J. Pediatr. 64: 853–865.

    PubMed  CAS  Google Scholar 

  54. Buchthal, F., L. Engback, and I. Gamstorp. 1958. Paresis and hyperexcitability in adynamia episodica hereditaria. Neurology (Minneapolis) 8: 347–351.

    CAS  Google Scholar 

  55. Segura, R. P., and J. H. Petajan. 1979. Neural hyperexcitability in hyperkalemic periodic paralysis. Muscle Nerve 2: 245–249.

    PubMed  CAS  Google Scholar 

  56. Bradley, W. G. 1969. Adynamia episodica hereditaria. Brain 92: 345–378.

    PubMed  CAS  Google Scholar 

  57. Garcin, R., P. Rondot, and M. Fardeau. 1964. Sur les accidents neuromusculaires et en particulier sur une `myopathie vacuolaire’ observés au cours d’un traitement prolongé par la chloroquine: Amélioration rapid apres arrêt due médicament. Rev. Neurol. 111: 117–126.

    Google Scholar 

  58. Bull, G. M., A. B. Carter, and K. G. Lowe. 1953. Hyperpotassaemic paralysis. Lancet 2: 60–63.

    Google Scholar 

  59. Marks, L. J., and E. Feit. 1953. Flaccid quadriplegia, hyperkalemia, and Addison’s disease. Arch. Intern. Med. 91: 56–57.

    CAS  Google Scholar 

  60. Richardson, G. O., and J. C. Sibley. 1953. Flaccid quadriplegia associated with hyperpotasseamia. Can. Med. Assoc. J. 69: 504–506.

    PubMed  CAS  Google Scholar 

  61. Mollaret, P., M. Goulon, and M. Tomilhac. 1958. Contribution a l’étude des paralyses avel hyperkaliémie. I. Röle de l’insuffisance cortico-surrénalienne. Rev. Neurol. 98: 341–357.

    PubMed  CAS  Google Scholar 

  62. Pollen, R. H., and R. H. Williams. 1960. Hyperkalemic neuromyopathy in Addison’s disease. N. Engl. J. Med. 263: 273–278.

    PubMed  CAS  Google Scholar 

  63. Faw, M. L., and R. V. Ewer. 1962. Intermittent paralysis and chronic adrenal insufficiency. Ann. Intern. Med. 57: 461–463.

    PubMed  CAS  Google Scholar 

  64. Bell, H., W. L. Hayes, and J. Vosburgh. 1965. Hyperkalemic paralysis due to adrenal insufficiency. Arch. Intern. Med. 115: 418–420.

    PubMed  CAS  Google Scholar 

  65. Daughaday, W. H., and D. Rendleman. 1967. Severe symptomatic hyperkalemia in an adrenalectomized woman due to enhanced mineralocorticoid requirement. Ann. Intern. Med. 66: 1197–1202.

    PubMed  CAS  Google Scholar 

  66. Van Dellen, R. C., and D. C. Purnell. 1969. Hyperkalemic paralysis due to adrenal insufficiency. Mayo Clin. Proc. 44: 904–914.

    PubMed  Google Scholar 

  67. Vilchez, J. J., A. Cabello, J. Bendito, and T. Villarroya. 1980. Hyperkalemic paralysis neuropathy and persistent motor neuron discharges at rest in Addison’s disease. J. Neurol. Neurosurg. Psychiatry 43: 818–822.

    PubMed  CAS  Google Scholar 

  68. Ucezne, E. O., and B. P. Harrold. 1980. Hyperkalemic paralysis due to spironolactone. Postgrad. Med. J. 56: 256.

    Google Scholar 

  69. Poskanzer, D. C., and D. N. S. Kerr. 1961. A third type of periodic paralysis with normokalemia and favourable response to sodium chloride. Am. J. Med. 31: 328–336.

    PubMed  CAS  Google Scholar 

  70. Meyers, K. R., D. H. Gilden, C. F. Rinaldi, and J. L. Hansen. 1972. Periodic muscle weakness, normokalemia, and tubular aggregates. Neurology (Minneapolis) 22: 269–274.

    CAS  Google Scholar 

  71. Tyler, F. H., F. E. Stephens, F. D. Gunn, and G. T. Perkoff. 1951. Studies in disorders of muscle. VII. Clinical manifestations and inheritance of a type of periodic paralysis without hypopotassemia. J. Clin. Invest. 30: 492–502.

    PubMed  CAS  Google Scholar 

  72. Stevens, J. R. 1954. Familial periodic paralysis, myotonia, progressive amyotrophy, and pes cavus in members of a single family. A.M.A. Arch. Neurol. Psychiat. 72: 726–734.

    CAS  Google Scholar 

  73. Klein, R. 1963. Periodic paralysis with cardiac arrhythmia. J. Pediatr. 62: 371–376.

    PubMed  CAS  Google Scholar 

  74. Iannoccone, S. T., K. Bove, B. Nagy, and F. J. Samaha. 1982. Familial dystrophy associated with periodic paralysis and a unique deficit of the major protein of sarcoplasmic reticulum. Ann. Neurol. 12: 108–109.

    Google Scholar 

  75. Goldflam, S. 1895. Weitere Mittheilung uber die paroxysmale, familiare Lahmung. Dtsch. Z. Nervenheilkd. 7: 1–12.

    Google Scholar 

  76. Biczyskowa, W., A. Fidzianska, and H. Jedrzejowska. 1969. Light and electron microscopic study of the muscles in hypokalemic periodic paralysis. Acta Neuropathol. 12: 329–346.

    PubMed  CAS  Google Scholar 

  77. Gordon, A. M., J. R. Green, and D. Lagonoff. 1970. Studies on a patient with hypokalemic familial periodic paralysis. Am. J. Med. 48: 185.

    PubMed  CAS  Google Scholar 

  78. Engel, A. G. 1970. Evolution and contents of vacuoles in primary hypokalemic periodic paralysis. Mayo Clin. Proc. 45: 774–814.

    PubMed  CAS  Google Scholar 

  79. Pellissier, J. F., M. C. Faugere, D. Gambarelli, and M. Toba. 1980. Histological, histochemical and ultrastructural studies in five cases. Semin. Hop. Paris 56: 1393–1404.

    CAS  Google Scholar 

  80. Norris, F. H., B. J. Panner, and J. M. Stormont. 1968. Thyrotoxic periodic paralysis: Metabolic and ultrastructural studies. Arch. Neurol. 19: 85–98.

    Google Scholar 

  81. Takaci, A., D. L. Schotland, S. DiMauro, and L. P. Rowland. 1973. Thyrotoxic periodic paralysis: Function of SR and muscle glycogen. Neurology (Minneapolis) 23: 100.

    Google Scholar 

  82. Bradley, W. G. 1969. Ultrastructural changes in adynamia episodica hereditaria and normokalemic familial periodic paralysis. Brain 42: 379–390.

    Google Scholar 

  83. Walter, G. F., and L. Auböck. 1980. Case report: Ultrastructural changes of muscles during attacks of adynamia episodic hereditaria. Neuropathol. Appl. Neurobiol. 6: 313–317.

    PubMed  CAS  Google Scholar 

  84. Dyken, M., W. Zeman, and T. Rusche. 1969. Hypokalemic periodic paralysis: Children with permanent myopathic weakness. Neurology (Minneapolis) 19: 691–699.

    CAS  Google Scholar 

  85. Wainwright, J., and D. Pirie. 1980. Hypokalemic myopathy. S. Afr. Med. J. 58: 81–84.

    PubMed  CAS  Google Scholar 

  86. Kao, L. I., and A. M. Gordon. 1977. Alteration of skeletal muscle cellular structures by potassium depletion. Neurology (Minneapolis) 27: 855–860.

    CAS  Google Scholar 

  87. DeCosta, W. J. P. 1979. Experimental hypokalaemic ultrastructural changes in rat gastrocnemius muscle. Acta Neurol. 45: 79–82.

    Google Scholar 

  88. Corbett, A. J., and M. Pollock. 1981. Experimental potassium depletion myopathy. J. Neurol. Sci. 49: 193–206.

    PubMed  CAS  Google Scholar 

  89. Vastola, E. F., and C. A. Bertrand. 1956. Intracellular water and potassium in periodic paralysis. Neurology (Minneapolis) 6: 523–530.

    CAS  Google Scholar 

  90. Conn, J. W., and D. H. Streeten. 1960. Intermittent aldosteronism in periodic paralysis. In: Metabolic Basis of Inherited Disease. J. B. Stanbury, J. B. Wyngaarden, and P. S. Fredrickson, eds. McGraw-Hill, New York. pp. 883–898.

    Google Scholar 

  91. Talso, P. J., M. F. Glenn, Y. T. Oester, and J. Fudema. 1963. Body composition in hypokalemic familial periodic paralysis. Ann. N.Y. Acad. Sci. 110: 994–1001.

    Google Scholar 

  92. Kreindler, A., V. Ionasesco, I. Petrovici, A. Petresco, and G. Calcaianu. 1964. Etudes cliniques et de laboratoire dans la paralysie periodique familiale avec hypokalémie. Rev. Neurol. 110: 377–385.

    Google Scholar 

  93. Niall, J. F., and R. K. Poy. 1966. Studies in familial hypokalemic periodic paralysis. Aust. Ann. Med. 15: 352–356.

    PubMed  CAS  Google Scholar 

  94. Shishiba, Y., K. Shizume, M. Sakuma, H. Yamauchi, K. Nakao, and S. Okinaka. 1966. Studies on electrolyte metabolism in idiopathic and thyrotoxic periodic paralysis. III. Intra-and extra-cellular concentrations of potassium and sodium in muscle and their changes during induced attacks of paralysis. Metabolism 15: 153–162.

    PubMed  CAS  Google Scholar 

  95. Hofmann, W. W., and R. A. Smith. 1970. Hypokalaemic periodic paralysis studied in vitro. Brain 93: 445–474.

    CAS  Google Scholar 

  96. Klein, R., T. Egan, and P. Usher. 1960. Changes in sodium, potassium and water in hyperkalemic familial periodic paralysis. Metabolism 9: 1005–1024.

    PubMed  CAS  Google Scholar 

  97. Conn, J. W., S. S. Fajans, L. H. Louis, D. H. P. Streeten, and R. D. Johnson. 1957. Intermittent aldosteronism in periodic paral- ysis: Dependence of attacks on retention of sodium, and failure to induce attacks by restriction of dietary sodium. Lancet 1: 802–805.

    Google Scholar 

  98. Gordon, M. 1978. Periodic familial paralysis with hypokalemia. Hemodynamic and metabolic studies: favorable effect of acetazolamide. Rev. Neurol. 134: 655–672.

    Google Scholar 

  99. Grob, D., R. J. Johns, and Ä. Liljestrand. 1957. Potassium movement in patients with familial periodic paralysis: Relationship to defect in muscle function. Am. J. Med. 23: 356–375.

    PubMed  CAS  Google Scholar 

  100. Sonkodi, S. 1980. Familial periodic paralysis (hypopotassaemic form). Wien. Klin. Wochenschr. 92: 610–613.

    PubMed  CAS  Google Scholar 

  101. Zierler, K. L., and R. Andres. 1957. Movement of potassium into skeletal muscle during spontaneous attack in family periodic paralysis. J. Clin. Invest. 36: 730–737.

    PubMed  CAS  Google Scholar 

  102. Shizume, K., Y. Shishiba, M. Sakuma, H. Yamauchi, K. Nakao, and S. Okinaka. 1966. Studies on electrolyte metabolism in idiopathic and thyrotoxic periodic paralysis. I. Arteriovenous differences of electrolytes during induced paralysis. Metabolism 15: 138–144.

    PubMed  CAS  Google Scholar 

  103. Buruma, O. J., T. M. A. R. Dubbelman, A. W. de Bruyne, and J. van Steveninck. 1978. Erythrocyte membrane studies in familial hypokalemic periodic paralysis. Arch. Neurol. 35: 615–616.

    PubMed  CAS  Google Scholar 

  104. Engel, A. G., C. S. Potter, and J. W. Rosevear. 1967. Studies on carbohydrate metabolism and mitochondrial respiratory activities in primary hypokalemic periodic paralysis. Neurology (Minneapolis) 17: 329–338.

    CAS  Google Scholar 

  105. Streeten, D. H. P., and P. J. Speller. 1974. The role of mineralocorticoids in the pathogenesis of hypokalemic periodic paralysis. J. Clin. Endocrinol. Metab. 39: 326–333.

    PubMed  CAS  Google Scholar 

  106. Stearns, R. H., M. Cox, P. U. Feig, and I. Singer. 1984. Internal potassium balance and the control of the plasma potassium concentration. Medicine (Baltimore) 60: 339–354.

    Google Scholar 

  107. Samaha, F. J. 1969. Sodium-potassium adenosine triphosphate in diseased muscle: Studies on periodic paralysis, myasthenia gravis, and Eaton-Lambert syndrome. Neurology (Minneapolis) 19:551–552.

    CAS  Google Scholar 

  108. Clausen, T., and J. A. Flatman. 1977. The effect of catecholamines on Na-K transport and membrane potential in rat soleus muscle. J. Physiol. (London) 270: 383–414.

    CAS  Google Scholar 

  109. Clausen, T., and P. G. Kohn. 1977. The effect of insulin on the transport of sodium and potassium in rat soleus muscle. J. Physiol. (London) 265: 19–42.

    CAS  Google Scholar 

  110. Flatman, J. A., and T. Clausen. 1979. Combined effects of adrenaline and insulin on active electrogenic Na-K transport in rat soleus muscle. Nature (London) 281: 580–581.

    CAS  Google Scholar 

  111. Moore, R. D., and J. L. Rabovsky. 1979. Mechanism of insulin action on resting membrane potential of frog skeletal muscle. Am. J. Physiol. 236: C249 - C254.

    PubMed  CAS  Google Scholar 

  112. Erlij, D., and H. F. Schoen. 1981. Effects of insulin on alkali-cation movements across muscle and epithelial cell membranes. Ann. N.Y. Acad. Sci. 372: 272–290.

    PubMed  CAS  Google Scholar 

  113. Clausen, T. 1982. Adrenergic control of Na+-K+ homeostasis. Acta Med. Scand. (Suppl.) 672: 111–115.

    Google Scholar 

  114. Viskoper, R. J., J. Fidel, T. Horn, D. Trivoni, and J. Chaco. 1973. On the beneficial action of acetazolamide in hypokalemic periodic paralysis: Study of carbohydrate metabolism. Am. J. Med. Sci. 226: 125–129.

    Google Scholar 

  115. Vallat, G. 1977. Hypokalemic familial periodic paralysis with basal electrocardiographic changes. Semin. Hop. Paris 53: 1163–1165.

    CAS  Google Scholar 

  116. Hofmann, W. W., B. T. Adornato, and H. Reich. 1983. The relationship of insulin receptors to hypokalemic periodic paralysis. Muscle Nerve 6: 48–51.

    PubMed  CAS  Google Scholar 

  117. Shizume, K., Y. Shishiba, M. Sakuma, H. Yamauchi, K. Nakao, and S. Okinaka. 1966. Studies on electrolyte metabolism in idiopathic and thyrotoxic periodic paralysis. II. Total exchangeable sodium and potassium. Metabolism 15: 145–152.

    PubMed  CAS  Google Scholar 

  118. Biron, R., R. Burger, A. Chinet, T. Clausen, and R. DuBoisFerrierer. 1979. Thyroid hormones and the energetics of active sodium-potassium transport in mammalian skeletal muscles. J. Physiol. (London) 297: 47–60.

    CAS  Google Scholar 

  119. Sharma, V. K., and S. P. Banerjee. 1978. ß-Adrenergic receptors in rat skeletal muscle: Effects of thyroidectomy. Biochim. Biophys. Acta 539: 538–542.

    PubMed  CAS  Google Scholar 

  120. Smith, E. K. M., and P. D. Samuel. 1970. Abnormalities in the sodium pump of erythryoctes from patients with hypothyroidism. Clin. Sci. 38: 49–61.

    PubMed  CAS  Google Scholar 

  121. Shishiba, Y., T. Shimizu, and T. Saito. 1972. Elevated immunoreactive insulin concentration during spontaneous attacks in thyrotoxic periodic paralysis. Metabolism 21: 285–290.

    PubMed  CAS  Google Scholar 

  122. Offerijns, F. G. J., D. Westerink, and A. F. Willebrands. 1958. The relation of potassium deficiency to muscular paralysis by insulin. J. Physiol. (London) 141: 377–384.

    CAS  Google Scholar 

  123. Norgaard, A., K. Kjeldsen, and T. Clausen. 1981. Potassium depletion decreases the number of 3H-ouabain binding sites and the active Na-K transport in skeletal muscle. Nature (London) 293: 739–741.

    CAS  Google Scholar 

  124. Erdmann, E., and W. Krawietz. 1977. Increased number of ouabain binding sites in human erythrocyte membranes in chronic hypokalemia. Acta Biol. Med. Ger. 36: 879–883.

    PubMed  CAS  Google Scholar 

  125. Akaike, N. 1981. Sodium pump in skeletal muscle: Central nervous system-induced suppression by a-adrenoreceptors. Science 213: 1252–1254.

    PubMed  CAS  Google Scholar 

  126. Clausen, T., K. Kjeldsen, and A. Norgaard. 1983. Effects of denervation on sodium, potassium and [3H] ouabain binding in muscles of normal and potassium-depleted rats. J. Physiol. (London) 345: 123–134.

    CAS  Google Scholar 

  127. Akaike, N. 1975. Contribution of an electrogenic sodium pump to membrane potential in mammalian skeletal muscle fibers. J. Physiol. (London) 245: 499–520.

    CAS  Google Scholar 

  128. Akaike, N. 1978. Resting and action potentials in white muscle of potassium deficient rats. Comp. Biochem. Physiol. 61A: 629–633.

    Google Scholar 

  129. Lewis, E. D., R. C. Griggs, and R. T. Moxley. 1979. Regulation of plasma potassium in hyperkalemic periodic paralysis. Neurology (New York) 29: 1131–1137.

    CAS  Google Scholar 

  130. Hoskins, B., T. H. Maren, and F. Q. Vroom. 1974. Acetazolamide and potassium flux in RBC’s-Studies in patients with hyperkalemic periodic paralysis. Arch. Neurol. 31: 187–189.

    PubMed  CAS  Google Scholar 

  131. Clausen, T., P. Wang, H. Orskov, and O. Kristensen. 1980. Hyperkalemic periodic paralysis: Relationships between changes in plasma water, electrolytes, insulin and catecholamine during attacks. Scand. J. Clin. Lab. Invest. 40: 211–220.

    PubMed  CAS  Google Scholar 

  132. Streeten, D. H. P., T. G. Dalakos, and H. Fellerman. 1971. Studies on hyperkalemic periodic paralysis: Evidence of changes in plasma number, CL, and induction of paralysis by adrenal glucocorticoids. J. Clin. Invest. 50: 142–155.

    PubMed  CAS  Google Scholar 

  133. Ruff, R. L. 1985. Endocrine myopathies (hyper-and hypofunction of adrenal, thyroid, pituitary, parathyroid glands, and iatrogenic steroid myopathy). In: Myology. B. Q. Banker and A. G. Engel, eds. McGraw-Hill, New York.

    Google Scholar 

  134. Engel, A. G., and E. H. Lambert. 1969. Calcium activation of electrically inexcitable muscle fibers in primary hypokalemic periodic paralysis. Neurology (Minneapolis) 19: 851–858.

    CAS  Google Scholar 

  135. Engel, A. G., C. S. Potter, and J. W. Rosevear. 1964. Nucleotides and adenosine monophosphate deaminase activity in muscle in primary hypokalemic periodic paralysis. Nature (London) 292: 670–672.

    Google Scholar 

  136. Au, K. S., and R. T. T. Yeung. 1972. Thyrotoxic periodic paralysis: Periodic variation in the muscle calcium pump activity. Arch. Neurol. 26: 543–548.

    PubMed  CAS  Google Scholar 

  137. DeGrandis, D., A. Fiaschi, E. Tomelleri, and D. Orrico. 1978. Hypokalemic periodic paralysis: A single fiber electromyographic study. J. Neurol. Sci. 37: 107–112.

    CAS  Google Scholar 

  138. Brooks, J. E. 1969. Hyperkalemic periodic paralysis. Arch. Neurol. 20: 13–18.

    PubMed  CAS  Google Scholar 

  139. Norris, F. B., Jr. 1962. Unstable membrane potential in human myotonic muscle. Electroencephalogr. Clin. Neurophysiol. 14: 197–201.

    PubMed  Google Scholar 

  140. Creutzfeldt, O. D., B. C. Abbott, W. M. Fowler, and C. M. Pearson. 1963. Muscle membrane potentials in episodic adynamia. Electroencephalogr. Clin. Neurophysiol. 15: 508–519.

    PubMed  CAS  Google Scholar 

  141. Riecker, G., and H. D. Bolte. 1966. Membranpotentiale eizelner skeletmuskelzellen bein kypkaliamischer periodischer muskelparalyse. Klin. Wochenschr. 44: 804–807.

    PubMed  CAS  Google Scholar 

  142. McComas, A. J., K. Mrozek, and W. G. Bradley. 1968. The nature of the electrophysiological disorder in adynamia episodica. J. Neurol. Neurosurg. Psychiatry 31: 448–452.

    PubMed  CAS  Google Scholar 

  143. Lehmann-Horn, F., R. Rudel, K. Ricker, H. Lorkovic, R. Dengler, and H. C. Hopf. 1983. Two cases of adynamia episodica hereditaria in vitro investigation of muscle cell membrane and contraction parameters. Muscle Nerve 6: 113–121.

    PubMed  CAS  Google Scholar 

  144. Rudel, R., F. Lehmann-Horn, K. Ricker, and G. Küther. 1984. Hypokalemic periodic paralysis in vitro investigation of muscle fiber membrane parameters. Muscle Nerve 7: 110–120.

    PubMed  CAS  Google Scholar 

  145. Hodgkin, A. L., and A. F. Huxley. 1952. A quantitative description of membrane current and its application to conduction and excitation in nerve. J. Physiol. (London) 117: 500–544.

    CAS  Google Scholar 

  146. Almers, W., P. R. Stanfield, and W. Stühmer. 1983. Slow changes in currents through sodium channels in frog muscle membrane. J. Physiol. (London) 339: 253–271.

    CAS  Google Scholar 

  147. Almers, W., W. M. Roberts, and R. L. Ruff. 1984. Voltage clamp of rat and human skeletal muscle: Measurements with an improved loose-patch technique. J. Physiol. (London) 347: 751–768.

    CAS  Google Scholar 

  148. Roberts, W. M., R. L. Ruff, and W. Almers. 1983. Sodium currents in human muscle under voltage clamp. Soc. Neurosci. Abstr. 9: 1278.

    Google Scholar 

  149. Ruff, R. L., W. Stühmer, and W. Almers. 1982. The effect of glucocorticoid treatment on mammalian muscle excitability. Pfluegers Arch. 395: 132–137.

    CAS  Google Scholar 

  150. Ruff, R. L., D. Martyn, and A. M. Gordon. 1982. Glucocorticoid treatment does not alter muscle membrane excitability. Am. J. Physiol. 243: E512 - E521.

    PubMed  CAS  Google Scholar 

  151. Kao, L. I., and A. M. Gordon. 1965. Mechanism of insulin-induced paralysis of muscles from potassium-depleted rats. Science 188: 740.

    Google Scholar 

  152. Gordon, A. M., and L. I. Kao. 1978. Disorders of muscle membranes: The periodic paralyses. In: Physiology of Membrane Disorders. T. E. Andreoli, J. F. Hoffman, and D. D. Fanestil, eds. Plenum Press, New York. pp. 817–829.

    Google Scholar 

  153. Layzer, R. B. 1982. Periodic paralysis and the sodium-potassium pump. Ann. Neurol. 11: 547–552.

    PubMed  CAS  Google Scholar 

  154. Almers, W. 1979. Potassium concentration changes in the transverse tubules of vertebrate skeletal muscle. Fed. Proc. 39: 1527–1532.

    Google Scholar 

  155. Venosa, R. A., and P. Horwicz. 1981. Density and apparent location of the sodium pump in frog sartorius muscle. J. Membr. Biol. 59: 225–232.

    PubMed  CAS  Google Scholar 

  156. Akaike, N. 1982. Hyperpolarization of mammalian skeletal muscle fibers in K-free media. Am. J. Physiol. 242: C12 - C18.

    PubMed  CAS  Google Scholar 

  157. Stanfield, P. R., N. B. Standen, C. A. Leech, and F. M. Ashcroft. 1980. Inward rectification in skeletal muscle fibers. In: Advances in Physiological Science, Volume V. E. Varga, A. Kover, T. Kovacs, and L. Kovacs, eds. Pergamon Press, Elmsford, N.Y. pp. 247–262.

    Google Scholar 

  158. Sestoft, L. 1967. Direct transition from hypo-to hyperkalemic paralysis during potassium treatment of familial periodic paralysis. Dan. Med. Bull. 14: 157–160.

    PubMed  CAS  Google Scholar 

  159. Lehmann-Horn, F., R. Rüdel, R. Dengler, H. Lorkovic, A. Haass, and K. Ricker. 1981. Membrane defects in paramyotonia congenita with and without myotonia in a warm environment. Muscle Nerve 4: 396–406.

    PubMed  CAS  Google Scholar 

  160. Ricker, K., A. Haass, R. Rüdel, R. Böhlen, and H. G. Mertens. 1980. Successful treatment of paramyotonia congenita (Eulen-berg): Muscle stiffness and weakness prevented by tocainide. J. Neurol. Neurosurg. Psychiatry 43: 812–826.

    Google Scholar 

  161. Ricker, K., R. Bohlen, and R. Rohkamm. 1983. Different effectiveness of tocainide and hydrochlorothiazide in paramyotonia congenita with hyperkalemic episodic paralysis. Neurology (Cleveland) 33: 1615–1618.

    CAS  Google Scholar 

  162. Osaku, M., and I. Ohtsuki. 1970. Mechanism of muscular paralysis by insulin with special reference to periodic paralysis. Am. J. Physiol. 219: 1173–1182.

    Google Scholar 

  163. Dengler, R., W. W. Hofmann, and R. Rüdel. 1979. Effect of potassium depletion and insulin on resting and stimulated skeletal rat muscle. J. Neurol. Neurosurg. Psychiatry 42: 818–826.

    PubMed  CAS  Google Scholar 

  164. Huang, K. W. 1943. Pa-Ping: Transient stimulating family periodic paralysis. Chin. Med. J. 61: 305–312.

    Google Scholar 

  165. Henderson, E. G. 1974. Strophanthidin sensitive electrogenic mechanisms in frog sartorius muscles exposed to barium. Pfluegers Arch. 350: 81–95.

    CAS  Google Scholar 

  166. Campa, J. F., and D. B. Sanders. 1974. Familial hypokalemic periodic paralysis. Local recovery after nerve stimulation. Arch. Neurol. 31: 110–115.

    PubMed  CAS  Google Scholar 

  167. Bezanilla, F., and C. M. Armstrong. 1972. Negative conductance caused by entry of sodium and cesium ions into the potassium channels of squid axon. J. Gen. Physiol. 60: 588–608.

    PubMed  CAS  Google Scholar 

  168. Johnsen, T. 1977. Trial of the prophylactic effect of diazoxide in the treatment of familial periodic hypokalemia. Acta Neurol. Scand. 56: 525–532.

    PubMed  CAS  Google Scholar 

  169. Griggs, R. C., W. K. Engel, and J. S. Resnick. 1970. Acetazolamide treatment of hypokalemic periodic paralysis. Ann. Intern. Med. 73: 39–48.

    PubMed  CAS  Google Scholar 

  170. Viskoper, R. J., A. Licht, J. Fidel, and J. Chaco. 1973. On the beneficial action of acetazolamide treatment in hypokalemic periodic paralysis: A metabolic and electromyographic study. Am. J. Med. Sci. 266: 119–123.

    CAS  Google Scholar 

  171. Johnsen, T. 1977. Effect upon serum insulin, glucose and potassium concentrations of acetazolamide during attacks of familial periodic paralysis. Acta Neurol. Scand. 56: 533–541.

    PubMed  CAS  Google Scholar 

  172. Gerard, J. M. 1979. Familial periodic paralysis with hypokalemia, hyperaldosteronism, and extracellular vacuolization. Rev. Neurol. 134: 761–772.

    Google Scholar 

  173. Riggs, J. E., R. C. Griggs, and R. T. Moxley. 1984. Dissociation of glucose and potassium arterial-venous differences across the forearm by acetazolamide. Arch. Neurol. 41: 35–38.

    PubMed  CAS  Google Scholar 

  174. Campbell, D. T., and B. Hille. 1976. Kinetic and pharmacological properties of the sodium channel of frog skeletal muscle. J. Gen. Physiol. 67: 309–323.

    PubMed  CAS  Google Scholar 

  175. Conway, M. J., J. A. Seibel, and R. P. Eaton. 1974. Thyrotoxicosis and periodic paralysis improvement with beta blockade. Ann. Intern. Med. 81: 332–336.

    PubMed  CAS  Google Scholar 

  176. Yeung, R. T. F., and T. F. Tse. 1974. Thyrotoxic periodic paralysis: Effect of propranolol. Am. J. Med. 57: 584–590.

    PubMed  CAS  Google Scholar 

  177. Resnick, J. 1969. Thyrotoxic periodic paralysis. Am. J. Med. 47: 831–838.

    PubMed  CAS  Google Scholar 

  178. Riggs, J. E., R. C. Griggs, R. T. Moxley, and E. D. Lewis. 1981. Acute effects of acetazolamide in hyperkalemic periodic paralysis. Neurology (New York) 31: 725–729.

    CAS  Google Scholar 

  179. Hoskins, B. 1977. Studies on the mechanism of action of acetazolamide in the prophylaxis of hyperkalemic periodic paralysis. Life Sci. 20: 343–350.

    PubMed  CAS  Google Scholar 

  180. Wang, P., and T. Clausen. 1975. Treatment of attacks in hyperkalaemic familial periodic paralysis by inhalation of salbutamol. Lancet 1: 221–223.

    Google Scholar 

  181. Wang, P., T. Clausen, and H. Oerskov. 1978. Salbutamol inhalations suppress attacks of hyperkalemia in familial periodic paralysis. Monogr. Hum. Genet. 10: 62–65.

    PubMed  CAS  Google Scholar 

  182. Dahl-Jorgensen, K., and H. Michalsen. 1979. Adynamia episodica herediteria: Treatment with salbutamol. Acta Paediatr. Scand. 68: 583–585.

    PubMed  CAS  Google Scholar 

  183. Adrian, R. H., and W. H. Freygang. 1962. Potassium conductance of frog muscle membrane under controlled voltage. J. Physiol. (London) 163: 104–138.

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Cleveland Veterans Administration Hospital and Case Western Reserve University, Cleveland, Ohio, 44106, USA

    Robert L. Ruff

  2. Department of Physiology and Biophysics, University of Washington School of Medicine, Seattle, Washington, 98195, USA

    Albert M. Gordon

Authors
  1. Robert L. Ruff
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Albert M. Gordon
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Internal Medicine, University of Texas Medical School, 77225, Houston, Texas, USA

    Thomas E. Andreoli M.D. (Edward Randall III Professor and Chairman, Professor of Physiology and Cell Biology) (Edward Randall III Professor and Chairman, Professor of Physiology and Cell Biology)

  2. Yale University School of Medicine, New Haven, Connecticut, USA

    Joseph F. Hoffman Ph.D.

  3. University of California, San Diego, La Jolla, California, USA

    Darrell D. Fanestil M.D.

  4. University of Texas Medical School, Houston, Texas, USA

    Stanley G. Schultz M.D.

Rights and permissions

Reprints and permissions

Copyright information

© 1987 Plenum Publishing Corporation

About this chapter

Cite this chapter

Ruff, R.L., Gordon, A.M. (1987). Disorders of Muscle. In: Andreoli, T.E., Hoffman, J.F., Fanestil, D.D., Schultz, S.G. (eds) Clinical Disorders of Membrane Transport Processes. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-1286-4_4

Download citation

  • DOI: https://doi.org/10.1007/978-1-4684-1286-4_4

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-0-306-42699-5

  • Online ISBN: 978-1-4684-1286-4

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation