Periodic paralysis: clinical evaluation in 20 patients

Tengan, Célia Harumi; Oliveira, Acary S. Bulle de; Gabbai, Alberto Alain

doi:10.1590/S0004-282X1994000400008

Abstracts

Twenty patients with periodic paralysis were evaluated and the aspects studied included epidemiological data, clinical manifestations, ancillary tests, treatment and evolution. Sixteen patients had the hypokalemic form (5 familiar, 5 sporadic, 5 thyrotoxic and 1 secondary). No patient with the normokalemic form was detected. Predominance of men was found (14 patients), especially in the cases with hyperthyroidism (5 patients). No thyrotoxic patient was of oriental origin. Only 4 patients had the hyperkalemic form (3 familiar, 1 sporadic). Attacks of paralysis began during the first decade in the hyperkalemic form and up to the third decade in the hypokalemic. In both forms the attacks occurred preferentially in the morning with rest after exercise being the most important precipitating factor. Seventy five percent of the hyperkalemic patients referred brief attacks (<12 hours). Longer attacks were referred by 43% of the hypokalemic patients. The majority of the attacks manifested with a generalized weakness mainly in legs, and its frequency was variable. Creatinokinase was evaluated in 10 patients and 8 of them had levels that varied from 1,1 to 5 times normal. Electromyography was done in 6 patients and myotonic phenomenon was the only abnormality detected in 2 patients. Carbonic anhydrase inhibitors, especially acetazolamide, were used for prophylactic treatment in 9 pacients with good results in all. Although periodic paralysis may be considered a benign disease we found respiratory distress in 5 patients, permanent myopathy in 1, electrocardiographic abnormalities during crises in 4; death during paralysis occurred in 2. Therefore correct diagnosis and immediate treatment are crucial. This study shows that hyperthyroidism is an important cause of periodic paralysis in our country, even in non oriental patients. Hence endocrine investigation is mandatory since this kind of periodic paralysis will only be abated after return to the euthyroid state.

periodic paralysis; hypokalemia; hiperkalemia; hyperthyroidism

Este estudo consiste da avaliação de 20 pacientes com diagnóstico de paralisia periódica (PP) sendo descritos aspectos epidemiológicos, manifestações clínicas, exames subsidiários, tratamento e evolução. Dezesseis pacientes tinham a forma hipocalêmica (5 familiares, 5 esporádicos, 5 tireotóxicas e 1 secundária). Não houve casos de PP normocalêmica. Houve predomínio do sexo masculino (14 pacientes). Todos os 5 pacientes com forma tireotóxica eram do sexo masculino e nenhum deles tinha origem oriental. Somente 4 pacientes tinham a forma hipercalêmica (3 familiares, 1 esporádico). Nas duas formas os ataques ocorreram preferencialmente no período da manhã sendo o repouso após exercício o fator desencadeante mais importante. No entanto observamos que 75% dos pacientes com a forma hipercalêmica referiram crises de curta duração (<12 horas). Crises mais prolongadas foram referidas por 43% dos pacientes com a forma hipocalêmica. A maioria das crises caracterizava-se por fraqueza generalizada, predominando nos membros inferiores e sendo sua frequência variável. A creatinoquinase foi avaliada em 10 pacientes e 8 tinham níveis elevados que variaram de 1,1 a 5 vezes o valor normal. A eletroneuromiografia foi realizada em 6, mas o fenômeno miotônico foi a única alteração encontrada em 2 pacientes. Inibidores da anidrase carbônica, principalmente a acetazolamida, usados no tratamento profilático em 9 pacientes mostraram uma boa resposta em todos. Embora a PP seja algumas vezes considerada doença benigna, encontramos sintomas respiratórios em 5 pacientes, miopatia permanente em 1, alterações eletrocardiográficas durante crise em 4; óbito durante paralisia ocorreu em 2 pacientes. Assim, o correto diagnóstico e o tratamento imediato são cruciais. Este estudo mostra que o hipertireoidismo é importante causa de PP em nosso meio, mesmo em pacientes não orientais. Assim, a investigação endócrina é mandatória já que as crises de paralisia só desaparecem após a normalização dos níveis hormonais.

paralisia periódica; hipopotassemia; hiperpotassemia; hipertireoidismo

Paralisia periódica: análise clínica de 20 pacientes

Periodic paralysis: clinical evaluation in 20 patients

Célia Harumi Tengan; Acary S. Bulle de Oliveira; Alberto Alain Gabbai

Disciplina de Neurologia do Departamento de Neurologia, Neurocirurgia e Neurologia Experimental da Escola Paulista de Medicina

RESUMO

Este estudo consiste da avaliação de 20 pacientes com diagnóstico de paralisia periódica (PP) sendo descritos aspectos epidemiológicos, manifestações clínicas, exames subsidiários, tratamento e evolução. Dezesseis pacientes tinham a forma hipocalêmica (5 familiares, 5 esporádicos, 5 tireotóxicas e 1 secundária). Não houve casos de PP normocalêmica. Houve predomínio do sexo masculino (14 pacientes). Todos os 5 pacientes com forma tireotóxica eram do sexo masculino e nenhum deles tinha origem oriental. Somente 4 pacientes tinham a forma hipercalêmica (3 familiares, 1 esporádico). Nas duas formas os ataques ocorreram preferencialmente no período da manhã sendo o repouso após exercício o fator desencadeante mais importante. No entanto observamos que 75% dos pacientes com a forma hipercalêmica referiram crises de curta duração (<12 horas). Crises mais prolongadas foram referidas por 43% dos pacientes com a forma hipocalêmica. A maioria das crises caracterizava-se por fraqueza generalizada, predominando nos membros inferiores e sendo sua frequência variável. A creatinoquinase foi avaliada em 10 pacientes e 8 tinham níveis elevados que variaram de 1,1 a 5 vezes o valor normal. A eletroneuromiografia foi realizada em 6, mas o fenômeno miotônico foi a única alteração encontrada em 2 pacientes. Inibidores da anidrase carbônica, principalmente a acetazolamida, usados no tratamento profilático em 9 pacientes mostraram uma boa resposta em todos. Embora a PP seja algumas vezes considerada doença benigna, encontramos sintomas respiratórios em 5 pacientes, miopatia permanente em 1, alterações eletrocardiográficas durante crise em 4; óbito durante paralisia ocorreu em 2 pacientes. Assim, o correto diagnóstico e o tratamento imediato são cruciais. Este estudo mostra que o hipertireoidismo é importante causa de PP em nosso meio, mesmo em pacientes não orientais. Assim, a investigação endócrina é mandatória já que as crises de paralisia só desaparecem após a normalização dos níveis hormonais.

Palavras-chave: paralisia periódica, hipopotassemia, hiperpotassemia, hipertireoidismo.

SUMMARY

Twenty patients with periodic paralysis were evaluated and the aspects studied included epidemiological data, clinical manifestations, ancillary tests, treatment and evolution. Sixteen patients had the hypokalemic form (5 familiar, 5 sporadic, 5 thyrotoxic and 1 secondary). No patient with the normokalemic form was detected. Predominance of men was found (14 patients), especially in the cases with hyperthyroidism (5 patients). No thyrotoxic patient was of oriental origin. Only 4 patients had the hyperkalemic form (3 familiar, 1 sporadic). Attacks of paralysis began during the first decade in the hyperkalemic form and up to the third decade in the hypokalemic. In both forms the attacks occurred preferentially in the morning with rest after exercise being the most important precipitating factor. Seventy five percent of the hyperkalemic patients referred brief attacks (<12 hours). Longer attacks were referred by 43% of the hypokalemic patients. The majority of the attacks manifested with a generalized weakness mainly in legs, and its frequency was variable. Creatinokinase was evaluated in 10 patients and 8 of them had levels that varied from 1,1 to 5 times normal. Electromyography was done in 6 patients and myotonic phenomenon was the only abnormality detected in 2 patients. Carbonic anhydrase inhibitors, especially acetazolamide, were used for prophylactic treatment in 9 pacients with good results in all. Although periodic paralysis may be considered a benign disease we found respiratory distress in 5 patients, permanent myopathy in 1, electrocardiographic abnormalities during crises in 4; death during paralysis occurred in 2. Therefore correct diagnosis and immediate treatment are crucial. This study shows that hyperthyroidism is an important cause of periodic paralysis in our country, even in non oriental patients. Hence endocrine investigation is mandatory since this kind of periodic paralysis will only be abated after return to the euthyroid state.

Key words: periodic paralysis, hypokalemia, hiperkalemia, hyperthyroidism.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 13-abril-1994.

Dra. Célia Harumi Tengan - Disciplina de Neurologia, Escola Paulista de Medicina - Rua Botucatu 740 -04023-900 São Paulo SP - Brasil.

1. Au KS, Yeung RTT. Thyrotoxic periodic paralysis: periodic variation in the muscle calcium pump activity. Arch Neurol 1972, 26: 543-546.
2. Bradley WG. Adynamia episódica hereditaria: clinical, pathological and electrophysiological studies in an affected family. Brain 1969, 92: 345-378.
3. Cheah JS, Tock EPC, Kan SP. The light and electron microscopic changes in the skeletal muscles during paralysis in thyrotoxic periodic paralysis. Am J Med Sci 1975, 269: 365-374.
4. Dalakas MC, Engel WK. Treatment of "permanent" muscle weakness in familial hypokalemic periodic paralysis. Muscle Nerve 1983, 6: 182-186.
5. Engel AG. Periodic paralysis. In Engel AG, Banker BQ. Myology. New York: McGraw Hill, 1986, p 1843-1870.
6. Engel AG. Thyroid function and periodic paralysis. Am J Med 1961, 30: 327-333.
7. Fontaine B, Khurana TS, Hoffman EP, Bruns G, Hainess JL, Trofatter JA, Hanson MP, Rich J, McFarlane H, Yasek DM, Romano D, Gusella JF, Brown RH Jr. Hyperkalemic periodic paralysis and the adult muscle sodium channel gene. Science 1990, 250: 1000-1002.
8. Johnsen T. A new standardized and effective method of inducing paralysis without administration of exogenous hormone in patientes with familial periodic paralysis. Acta Neurol Scand 1976, 54: 167-172.
9. Johnsen T. Familial periodic paralysis with hypokalemia. Dan Med Bull 1981, 281: 1-22.
10. Kelley DE, Gharib H, Kennedy FP, Duda RJ, McManis PG. Thyrotoxic periodic paralysis report of 10 cases and review of electromyographic findings. Arch Intern Med 1989, 149: 2597-2600.
11. Koch MC, Ricker K, Otto M, Grimm T, Hoffman EP, Rüdel R, Bender K, Zoll B, Harper PS, Lehmann-Horn F. Confirmation of linkage of hyperkalaemic periodic paralysis to choromosome 17. J Med Genet 1991, 28: 583-586.
12. Layzer RB, Goldfield E. Periodic paralysis caused by abuse of thyroid hormone. Neurology 1974, 24: 949-952.
13. Leung AKC. Familial "hashitoxic" periodic paralysis. J R Soc Med 1985, 78: 638-640.
14. Leung AKC. Familial "hashitoxic" periodic paralysis (letter). J R Soc Med 1989, 82: 245.
15. Links TP, Zwarts MJ, Wilmink JJT, Molenaar WM, Oosterhuis HJGH. Permanent muscle weakness in familial hypokalemic periodic paralysis: clinical, radiological and pathological aspects. Brain 1990, 113: 1873-1889.
16. Marx A, Ruppersberg JP, Pietrzyrk C, Rudel R. Thyrotoxic periodic paralysis and the sodium/potassium pump. Muscle Nerve 1989, 12: 810-815.
17. Meyers KR, Gilden OH, Rinaldi CF, Hansen JL. Periodic muscle weakness, normokalemia and tubular aggregates. Neurology 1972, 22: 269-279.
18. Murakami K. Studies on the intracellular water, sodium and potassium in thyrotoxic myopathy and in thyrotoxic periodic paralysis. Endocrinol Japan 1964, 11: 291-307.
19. Okinaka S, Shizume K, Iinos S, Watanabe A, Irie M, Noguchi A, Kuma S, Kuma K, Ito T. The association of periodic paralysis and hyperthyoidism in Japan. J. Clin Endocrinol Metab 1957, 17: 1454-1459.
20. Pearson CM. The periodic paralysis: diferential features and pathological observations in permanent myopathic weakness. Brain 1964, 87: 341-354.
21. Pereira VG, Wajchenberg BL, Quintão ER. Periodic paralysis associated with hyperthyroidism: study of the precipitating factors. Arq Bras Endocrinol Metabol 1968, 17: 55-67.
22. Ptacek LJ, Gouw L, Kwiecinski H, McManis P, Eendell JR, Barohn R, George AL, Barchi RL, Robertson M, Leppert F. Sodium channel mutations in paramyotonia congenita and hyperkalemic periodic paralysis. Ann Neurol 1993, 33: 300-307.
23. Ptacek LJ, Tyler F, Trimmer JS, Agnew WS, Leppert M. Analysis in a large hyperkalemic periodic paralysis pedigree supports tight linkage to a sodium channel locus. Am J Hum Genet 1991, 49: 378-382.
24. Resnick JS, Dorman JD, Engel WK. Thyrotoxic periodic paralysis. Am J Med 1969, 47: 831-836.
25. Ricker K, Camacho LM, Grafe P, Lehmann-Horn F, Rüdel R. Adynamia episodica hereditaria: what causes the weakness? Muscle Nerve 1989, 12: 883-891.
26. Rojas CV, Wang J, Schwartz LS, Hoffman EP, Powell BR, Brown RH Jr. A met-to-val mutation in the skeletal muscle Na channel -subunit in hyperkalaemic periodic paralysis. Nature 1991, 354: 387-389.
27. Rudel R, Lehmann-Horn F, Ricker K, Küther G. Hypokalemic periodic paralysis: in vitro investigation of muscle fiber membrane parameters. Muscle Nerve 1984, 7: 110-120.
28. Saeian K, Heckerling P. Thyrotoxic periodic paralysis in a hispanic man. Arch Intern Med 1988, 148:708.
29. Shizume K, Shishiba Y, Sakuma M, Yamauchi H, Nakao K, Okinaka S. Studies on electrolyte metabolism in idiopathic and thyrotoxic periodic paralysis: I. arteriovenous differences of electrolyties during induced paralysis. Metabolism 1966, 15: 138-144.
30. Shizume K, Shishiba Y, Sakuma M, Yamauchi H, Nakao K, Okinaka S. Studies on electrolytes metabolism in idiopathic and thyrotoxic periodic paralysis: II. total exchangeable sodium and potassium. Metabolism 1966, 15: 145-152.
31. Sterian L, Maciel RMB. Paralisia periódica tirotóxica: relato de um caso e comentarios etiopatogênicos. Arq Bras Endocrinol Metabol 1983, 27: 161-162.
32. Subramony SH, Wee AS. Exercise and rest in hyperkalemic periodic paralysis. Neurology 1986, 36: 173-177.
33. Takagi A, Schotland DL, Di Mauro S, Rowland LP. Thyrotoxic periodic paralysis: function of sarcoplasmic reticulum and muscle glicogen. Neurology 1973, 23: 1008-1016.
34. Talbott JH. Periodic paralysis: a clinical syndrome. Medicine 1941, 20: 85-143.
35. Tamai H, Tanaka K, Komaki G, Matsubayashi S, Hirata Y, Mori K, Kuma K, Kumagai LF, Nagataki S. HLA and thyrotoxic periodic paralysis in Japanese patients. J Clin Endocrinol Metabol 1987, 64: 1075-1078.
36. Tengan CH, Oliveira ASB, Morita MPA, Kiyomoto BH, Schmidt B, Gabbai AA. Paralisia periódica: estudo anatomo-patológico do músculo esquelético de 14 pacientes. Arq Neuropsiquiatr 1994, 52: 32-40.

Publication Dates

Publication in this collection
19 Jan 2011
Date of issue
Dec 1994

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Au KS, Yeung RTT. Thyrotoxic periodic paralysis: periodic variation in the muscle calcium pump activity. Arch Neurol 1972, 26: 543-546.

[2] 2. Bradley WG. Adynamia episódica hereditaria: clinical, pathological and electrophysiological studies in an affected family. Brain 1969, 92: 345-378.

[3] 3. Cheah JS, Tock EPC, Kan SP. The light and electron microscopic changes in the skeletal muscles during paralysis in thyrotoxic periodic paralysis. Am J Med Sci 1975, 269: 365-374.

[4] 4. Dalakas MC, Engel WK. Treatment of "permanent" muscle weakness in familial hypokalemic periodic paralysis. Muscle Nerve 1983, 6: 182-186.

[5] 5. Engel AG. Periodic paralysis. In Engel AG, Banker BQ. Myology. New York: McGraw Hill, 1986, p 1843-1870.

[6] 6. Engel AG. Thyroid function and periodic paralysis. Am J Med 1961, 30: 327-333.

[7] 7. Fontaine B, Khurana TS, Hoffman EP, Bruns G, Hainess JL, Trofatter JA, Hanson MP, Rich J, McFarlane H, Yasek DM, Romano D, Gusella JF, Brown RH Jr. Hyperkalemic periodic paralysis and the adult muscle sodium channel gene. Science 1990, 250: 1000-1002.

[8] 8. Johnsen T. A new standardized and effective method of inducing paralysis without administration of exogenous hormone in patientes with familial periodic paralysis. Acta Neurol Scand 1976, 54: 167-172.

[9] 9. Johnsen T. Familial periodic paralysis with hypokalemia. Dan Med Bull 1981, 281: 1-22.

[10] 10. Kelley DE, Gharib H, Kennedy FP, Duda RJ, McManis PG. Thyrotoxic periodic paralysis report of 10 cases and review of electromyographic findings. Arch Intern Med 1989, 149: 2597-2600.

[11] 11. Koch MC, Ricker K, Otto M, Grimm T, Hoffman EP, Rüdel R, Bender K, Zoll B, Harper PS, Lehmann-Horn F. Confirmation of linkage of hyperkalaemic periodic paralysis to choromosome 17. J Med Genet 1991, 28: 583-586.

[12] 12. Layzer RB, Goldfield E. Periodic paralysis caused by abuse of thyroid hormone. Neurology 1974, 24: 949-952.

[13] 13. Leung AKC. Familial "hashitoxic" periodic paralysis. J R Soc Med 1985, 78: 638-640.

[14] 14. Leung AKC. Familial "hashitoxic" periodic paralysis (letter). J R Soc Med 1989, 82: 245.

[15] 15. Links TP, Zwarts MJ, Wilmink JJT, Molenaar WM, Oosterhuis HJGH. Permanent muscle weakness in familial hypokalemic periodic paralysis: clinical, radiological and pathological aspects. Brain 1990, 113: 1873-1889.

[16] 16. Marx A, Ruppersberg JP, Pietrzyrk C, Rudel R. Thyrotoxic periodic paralysis and the sodium/potassium pump. Muscle Nerve 1989, 12: 810-815.

[17] 17. Meyers KR, Gilden OH, Rinaldi CF, Hansen JL. Periodic muscle weakness, normokalemia and tubular aggregates. Neurology 1972, 22: 269-279.

[18] 18. Murakami K. Studies on the intracellular water, sodium and potassium in thyrotoxic myopathy and in thyrotoxic periodic paralysis. Endocrinol Japan 1964, 11: 291-307.

[19] 19. Okinaka S, Shizume K, Iinos S, Watanabe A, Irie M, Noguchi A, Kuma S, Kuma K, Ito T. The association of periodic paralysis and hyperthyoidism in Japan. J. Clin Endocrinol Metab 1957, 17: 1454-1459.

[20] 20. Pearson CM. The periodic paralysis: diferential features and pathological observations in permanent myopathic weakness. Brain 1964, 87: 341-354.

[21] 21. Pereira VG, Wajchenberg BL, Quintão ER. Periodic paralysis associated with hyperthyroidism: study of the precipitating factors. Arq Bras Endocrinol Metabol 1968, 17: 55-67.

[22] 22. Ptacek LJ, Gouw L, Kwiecinski H, McManis P, Eendell JR, Barohn R, George AL, Barchi RL, Robertson M, Leppert F. Sodium channel mutations in paramyotonia congenita and hyperkalemic periodic paralysis. Ann Neurol 1993, 33: 300-307.

[23] 23. Ptacek LJ, Tyler F, Trimmer JS, Agnew WS, Leppert M. Analysis in a large hyperkalemic periodic paralysis pedigree supports tight linkage to a sodium channel locus. Am J Hum Genet 1991, 49: 378-382.

[24] 24. Resnick JS, Dorman JD, Engel WK. Thyrotoxic periodic paralysis. Am J Med 1969, 47: 831-836.

[25] 25. Ricker K, Camacho LM, Grafe P, Lehmann-Horn F, Rüdel R. Adynamia episodica hereditaria: what causes the weakness? Muscle Nerve 1989, 12: 883-891.

[26] 26. Rojas CV, Wang J, Schwartz LS, Hoffman EP, Powell BR, Brown RH Jr. A met-to-val mutation in the skeletal muscle Na channel -subunit in hyperkalaemic periodic paralysis. Nature 1991, 354: 387-389.

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

[28] 28. Saeian K, Heckerling P. Thyrotoxic periodic paralysis in a hispanic man. Arch Intern Med 1988, 148:708.

[29] 29. Shizume K, Shishiba Y, Sakuma M, Yamauchi H, Nakao K, Okinaka S. Studies on electrolyte metabolism in idiopathic and thyrotoxic periodic paralysis: I. arteriovenous differences of electrolyties during induced paralysis. Metabolism 1966, 15: 138-144.

[30] 30. Shizume K, Shishiba Y, Sakuma M, Yamauchi H, Nakao K, Okinaka S. Studies on electrolytes metabolism in idiopathic and thyrotoxic periodic paralysis: II. total exchangeable sodium and potassium. Metabolism 1966, 15: 145-152.

[31] 31. Sterian L, Maciel RMB. Paralisia periódica tirotóxica: relato de um caso e comentarios etiopatogênicos. Arq Bras Endocrinol Metabol 1983, 27: 161-162.

[32] 32. Subramony SH, Wee AS. Exercise and rest in hyperkalemic periodic paralysis. Neurology 1986, 36: 173-177.

[33] 33. Takagi A, Schotland DL, Di Mauro S, Rowland LP. Thyrotoxic periodic paralysis: function of sarcoplasmic reticulum and muscle glicogen. Neurology 1973, 23: 1008-1016.

[34] 34. Talbott JH. Periodic paralysis: a clinical syndrome. Medicine 1941, 20: 85-143.

[35] 35. Tamai H, Tanaka K, Komaki G, Matsubayashi S, Hirata Y, Mori K, Kuma K, Kumagai LF, Nagataki S. HLA and thyrotoxic periodic paralysis in Japanese patients. J Clin Endocrinol Metabol 1987, 64: 1075-1078.

[36] 36. Tengan CH, Oliveira ASB, Morita MPA, Kiyomoto BH, Schmidt B, Gabbai AA. Paralisia periódica: estudo anatomo-patológico do músculo esquelético de 14 pacientes. Arq Neuropsiquiatr 1994, 52: 32-40.