Paralisia periodica familiar: estudo de oito casos

Assis, José Lamartine De; Scaff, Milberto; Levy, José Antonio; Callegaro, Dagoberto; Marchiori, Paulo

doi:10.1590/S0004-282X1980000400009

Resumos

Foram estudados oito pacientes com Paralisia Periódica Familiar sob os aspectos clínico, hidro-eletrolítico, eletrofisiológico, histológico e terapêutico. Houve predomínio significante em pacientes do sexo masculino. A idade média do início da moléstia foi de 15 anos. As manifestações clínicas foram concor-dantes com as referidas na literatura. Todos os pacientes tinham a forma hipocalêmica exceto um que evoluiu com hipercalemia. Alguns pacientes (21,4%) tinham natremias no limite superior da normalidade enquanto em um número igual de pacientes as natremias estavam elevadas, o que sugeria liberação de aldosterona. Os achados eletrofisiológicos foram concordantes com os da literatura. O estudo histológico de músculos realizado em cinco pacientes foi normal em 30% deles e, naqueles com crises repetidas e freqüentes, foram encontradas atrofias de fibras musculares. Foram feitos tratamentos preventivos, e nos pacientes com hipocalemia foram administradas doses suplementares de potássio em solução aquosa de 10-25% diariamente.

The authors studied 8 patients with Familial Periodic Paralysis on the clinical, hydroelectrolytic, electrophysiologic, histologic and therapeutic points of view. There was significant predominance in males. The onset of the symptoms in our group began under fifteen years of age. The clinical manifestations were similar to those referred in the literature. The hypokalemic form was found in all patients except in one case that had hyperkalemic form. Natremias on the superior border of the normality, were seen in 21.4% of the cases, and in 21.4% the natremias were higher; this fact suggested aldosterone liberation of the muscle. The histologic examination performed in 5 patients, was normal in 20% of them and, in those cases with repetitive and frequent crises, atrophic muscle fibers were seen. Prophylactic treatments were made. The patients with hypokalemic form received supplementary doses of potassium.

Paralisia periodica familiar: estudo de oito casos

Familial periodic paralysis: a study of 8 cases

José Lamartine De Assis^I; Milberto Scaff^II; José Antonio Levy^II; Dagoberto Callegaro^III; Paulo Marchiori^IV

^IPorf. Adjunto. Divisão de Neurologia de Adultos (Prof. Horácio M. Canelas) do Departamento de Neuropsiquiatria do Hospital das Clínicas da FMUSP

^IIProf. Livre-Docente. Divisão de Neurologia de Adultos (Prof. Horácio M. Canelas) do Departamento de Neuropsiquiatria do Hospital das Clínicas da FMUSP

^IIIProf. Assistente. Divisão de Neurologia de Adultos (Prof. Horácio M. Canelas) do Departamento de Neuropsiquiatria do Hospital das Clínicas da FMUSP

^IVProf. Assistente do Instituto do Coração (Divisão Cardiologia Social) do Hospital das Clínicas da FMUSP

RESUMO

Foram estudados oito pacientes com Paralisia Periódica Familiar sob os aspectos clínico, hidro-eletrolítico, eletrofisiológico, histológico e terapêutico. Houve predomínio significante em pacientes do sexo masculino. A idade média do início da moléstia foi de 15 anos. As manifestações clínicas foram concor-dantes com as referidas na literatura. Todos os pacientes tinham a forma hipocalêmica exceto um que evoluiu com hipercalemia. Alguns pacientes (21,4%) tinham natremias no limite superior da normalidade enquanto em um número igual de pacientes as natremias estavam elevadas, o que sugeria liberação de aldosterona. Os achados eletrofisiológicos foram concordantes com os da literatura. O estudo histológico de músculos realizado em cinco pacientes foi normal em 30% deles e, naqueles com crises repetidas e freqüentes, foram encontradas atrofias de fibras musculares.

Foram feitos tratamentos preventivos, e nos pacientes com hipocalemia foram administradas doses suplementares de potássio em solução aquosa de 10-25% diariamente.

SUMMARY

The authors studied 8 patients with Familial Periodic Paralysis on the clinical, hydroelectrolytic, electrophysiologic, histologic and therapeutic points of view. There was significant predominance in males. The onset of the symptoms in our group began under fifteen years of age. The clinical manifestations were similar to those referred in the literature. The hypokalemic form was found in all patients except in one case that had hyperkalemic form. Natremias on the superior border of the normality, were seen in 21.4% of the cases, and in 21.4% the natremias were higher; this fact suggested aldosterone liberation of the muscle. The histologic examination performed in 5 patients, was normal in 20% of them and, in those cases with repetitive and frequent crises, atrophic muscle fibers were seen. Prophylactic treatments were made. The patients with hypokalemic form received supplementary doses of potassium.

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Trabalho realizado na Divisão de Neurologia de Adultos (Prof. Horácio M. Canelas) do Departamento de Neuropsiquiatria do Hospital das Clínicas da FMUSP.

Departamento de Neuropsiquiatria, Divisão de Neurologia - Faculdade de Medicina da Universidade do São Paulo -. Caixa Postal 3461 - 01000 São Paulo, SP - Brasil.

1. BURUMA, O. J. S. & BOTS, A. M. - Myopathy in familial hypokalemic periodic paralisis independent of paralytic attack, Acta neurol. acand. 57:171, 1978.
2. CAMPA, F. J. & SANDERS, B. D. - Familial hypokalemic periodic paralysis: local recovery after nerve stimulation. Arch. Neurol. (Chicago) 31; 110, 1974.
3. ENGEL, AG. - Hypokalemic and hyperkalemic periodic paralysis. In GOLDENSOHN, E. S. &· APPEL, S. H. - Scientific Approaches to Clinical Neurology. Lea & Febiger, Philadelphia, 1977. V. 2, p. 1742-1765.
4. ENGEL, A. G. - Evolution and content of vacuoles in primary hypokalemic periodic paralysis. Proc. Mayo Clin. 45:774, 1970.
5. JARREL, M. A.; GREER, M. & MAREN, T. H. - The effect of acidosis in hypokalemic periodic paralysis. Arch. Neurol. (Chicago) 33:791, 1976.
6. JOHNSEN, T. - Effect upon serum insulin, glucose and potassium concentrations of acetazolamide during attacks of familial periodic hypokalemic paralysis. Acta neurol. scand. 56:533, 1977.
7. JOHNSEN, T. - Trial of the prophylactic effect of diazoxide in the treatment of familial periodic hypokalemia. Acta neurol. scand. 56:525, 1977.
8. KOBAYASHI, A. L. D. - Familial periodic paralysis: a case report and review. Neb. St. med. J. 60:110, 1975.
9. OTSUKA, M. & OHTSUKI, I. - Mechanism of muscular paralysis by insulin with special reference to periodic paralysis. Amer. J. Physiol. 219:1178, 1970.
10. RAPHAEL, J. C; SIMON, N. & GOULON, M. Paralysie avec hypokaliémie par transfert, traitée par acétazolamide. Sem. Paris 53:1167, 1977.
11. SICA, B. E, P. & AGUILERA, N. - Electrophysiological studies in hypokalemic periodic paralysis. Medicine 32:93, 1972.
12. SHISHIBA, Y.; SHIMIZU, T.; SAITO, T. A SHIZUME, K. - Elevated immuno-reactive insulin concentration during spontaneous attacks in thyrotoxic periodic paralysis. Metabolism 21:285, 1972.
13. VALLAT, G.; JANBON, C; BRUNEL, M.; VIVES, J. F. & ROUY, M. J. - Paralysie periodique familiale hypokaliémique avec modifications életrocardiographiques à l'état de base. Sem HôpParis 53:1163, 1977.
14. VISKOPER, R. J.; LICHT, A. & CHACO, J. - Acetazolamide treatment in hypokalemic periodic paralysis: a metabolic and electromyographic study. Amer. J. med. Sci. 266:119, 1973.
15- VROOM, F. Q.; JARRELL, M, A, & MAREN, T. H. - Acetazolamide treatment of hypokalemic periodic paralysis: probable mechanism of action. Arch. Neurol. (Chicago) 32:385, 1975.

Datas de Publicação

Publicação nesta coleção
23 Ago 2012
Data do Fascículo
Dez 1980

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

[1] 1. BURUMA, O. J. S. & BOTS, A. M. - Myopathy in familial hypokalemic periodic paralisis independent of paralytic attack, Acta neurol. acand. 57:171, 1978.

[2] 2. CAMPA, F. J. & SANDERS, B. D. - Familial hypokalemic periodic paralysis: local recovery after nerve stimulation. Arch. Neurol. (Chicago) 31; 110, 1974.

[3] 3. ENGEL, AG. - Hypokalemic and hyperkalemic periodic paralysis. In GOLDENSOHN, E. S. &· APPEL, S. H. - Scientific Approaches to Clinical Neurology. Lea & Febiger, Philadelphia, 1977. V. 2, p. 1742-1765.

[4] 4. ENGEL, A. G. - Evolution and content of vacuoles in primary hypokalemic periodic paralysis. Proc. Mayo Clin. 45:774, 1970.

[5] 5. JARREL, M. A.; GREER, M. & MAREN, T. H. - The effect of acidosis in hypokalemic periodic paralysis. Arch. Neurol. (Chicago) 33:791, 1976.

[6] 6. JOHNSEN, T. - Effect upon serum insulin, glucose and potassium concentrations of acetazolamide during attacks of familial periodic hypokalemic paralysis. Acta neurol. scand. 56:533, 1977.

[7] 7. JOHNSEN, T. - Trial of the prophylactic effect of diazoxide in the treatment of familial periodic hypokalemia. Acta neurol. scand. 56:525, 1977.

[8] 8. KOBAYASHI, A. L. D. - Familial periodic paralysis: a case report and review. Neb. St. med. J. 60:110, 1975.

[9] 9. OTSUKA, M. & OHTSUKI, I. - Mechanism of muscular paralysis by insulin with special reference to periodic paralysis. Amer. J. Physiol. 219:1178, 1970.

[10] 10. RAPHAEL, J. C; SIMON, N. & GOULON, M. Paralysie avec hypokaliémie par transfert, traitée par acétazolamide. Sem. Paris 53:1167, 1977.

[11] 11. SICA, B. E, P. & AGUILERA, N. - Electrophysiological studies in hypokalemic periodic paralysis. Medicine 32:93, 1972.

[12] 12. SHISHIBA, Y.; SHIMIZU, T.; SAITO, T. A SHIZUME, K. - Elevated immuno-reactive insulin concentration during spontaneous attacks in thyrotoxic periodic paralysis. Metabolism 21:285, 1972.

[13] 13. VALLAT, G.; JANBON, C; BRUNEL, M.; VIVES, J. F. & ROUY, M. J. - Paralysie periodique familiale hypokaliémique avec modifications életrocardiographiques à l'état de base. Sem HôpParis 53:1163, 1977.

[14] 14. VISKOPER, R. J.; LICHT, A. & CHACO, J. - Acetazolamide treatment in hypokalemic periodic paralysis: a metabolic and electromyographic study. Amer. J. med. Sci. 266:119, 1973.

[15] 15- VROOM, F. Q.; JARRELL, M, A, & MAREN, T. H. - Acetazolamide treatment of hypokalemic periodic paralysis: probable mechanism of action. Arch. Neurol. (Chicago) 32:385, 1975.