Síndrome de West: a propósito de nove casos

Kamiyama, Marina Aya; Yoshinaga, Lúcia; Tonholo-Silva, Edward R.

doi:10.1590/S0004-282X1993000300011

Resumos

A síndrome de West é forma de epilepsia generalizada que se inicia no primeiro ano de vida, com pico de incidência entre 5 e 8 meses, caracterizada por espasmos ou mioclonias maciças, regressão do desenvolvimento neuropsicomotor e alteração eletrencefalográfica denominada hipsarritmia. Relatamos nove casos de síndrome de West, discutindo aspectos clínicos, etiológicos, evolutivos e terapêuticos.

epilepsia; síndrome de West; espasmos infantis

West syndrome is a peculiar form of epilepsy of infancy and childhood characterized by spasms or massive myoclonus, regression neuropsychomotor development, and EEC abnormalities referred as hipsarrhythmia. We report nine cases of West syndrome discussing clinical, etiological, evolutive and therapeutic features.

epilepsy; West syndrome; infantile spasms

Síndrome de West: a propósito de nove casos

West syndrome: report of nine eases

Marina Aya Kamiyama^I; Lúcia Yoshinaga^I; Edward R. Tonholo-Silva^II

^IAcadêmica de Medicina, FMM. Estudo realizado no Ambulatório de Epilepsia do Hospital de Clínicas da Faculdade de Medicina de Marília (FMM)

^IIProfessor Assistente de Neurologia, FMM. Estudo realizado no Ambulatório de Epilepsia do Hospital de Clínicas da Faculdade de Medicina de Marília (FMM)

RESUMO

A síndrome de West é forma de epilepsia generalizada que se inicia no primeiro ano de vida, com pico de incidência entre 5 e 8 meses, caracterizada por espasmos ou mioclonias maciças, regressão do desenvolvimento neuropsicomotor e alteração eletrencefalográfica denominada hipsarritmia. Relatamos nove casos de síndrome de West, discutindo aspectos clínicos, etiológicos, evolutivos e terapêuticos.

Palavras-chave: epilepsia, síndrome de West, espasmos infantis.

SUMMARY

West syndrome is a peculiar form of epilepsy of infancy and childhood characterized by spasms or massive myoclonus, regression neuropsychomotor development, and EEC abnormalities referred as hipsarrhythmia. We report nine cases of West syndrome discussing clinical, etiological, evolutive and therapeutic features.

Key words: epilepsy, West syndrome, infantile spasms.

Full text available only in PDF format.

Texto completo disponível apenas em PDF.

Aceite: 22-fevereiro-1993.

Dr. Edward Roberto Tonholo-Silva Rua Júlio de Mesquita 295 - 17515-230 Marília SP - Brasil.

Referências bibliográficas

1. Albuquerque M, Muszkat M, Vergani MIC, Campos CJR. Síndrome de West: apresentação de 25 casos. Rev Paul Pediatr 1990, 8:76-78.
2. Antoniuk SA. Síndrome de West: urgência em pediatria. J Pediatr 1901, 67:205-206.
3. Chevrie JJ. Syndrome de West. Encycl Méd Chir Neurologie. Paris, 1979, 17045 A80.
4. Chevrie JJ, Aicardi J. Facteurs étiologiques des spasmes en flexion du nourisson: étude de 81 cas. Arch Franç Pédiat 1967, 24:511-520.
5. Duffner PK, Cohen ME. Infantile spasms associated with histidinemia. Neurology 1975, 25:195-197.
6. Fejerman N, Medina CS. Síndrome de West. In Fejerman N, Medina CS (eds): Convulsiones en la infancia, diagnóstico y tratamiento. Ed 2. Buenos Aires: Ateneo, 1986, p 128-147.
7. Gastaut H, Soulayrol R, Roger J, Pinsard N (eds). L'encéphalopatie myoclonique infantile avec hipsarrythmie (Syndrome de West). Paris: Masson, 1964.
8. Gibbs FA, Gibbs EL. Atlas of electroencephalography, epilepsy. Cambridge: Addison-Wesley, 1952, Vol 2.
9. Jeavons PM. West syndrome: infantile spasms. In Roger J, Dravet C, Bureau M, Dreifuss FE, Wolf P (eds): Epileptic syndromes in infancy, childhood and adolescence. London: John Libbey, 1985, p 42-50.
10. Kellaway P, Hrachovy RA, Frost JD. Precise characterization and quantification of infantile spasms. Ann Neurol 1979, 6:214-218.
11. Lacy JR, Penry JK. Infantile spasms. New York: Raven Press, 1976.
12. Lombroso CT. A prospective study of infantile spasms: clinic and therapeutic correlations. Epilepsia 1983, 24:135-158.
13. Marques-Dias MJ. Síndrome de West. In Diament A, Cypel S (eds): Neurologia Infantil-Lefèvre. Ed 2. Rio de Janeiro: Ateneu, 1990, p 1025-1030.
14. Siemes H, Spohr HL, Michael T, Nau H. Therapy of infantile spasms with valproate: results of a prospective study. Epilepsia 1988, 29:553-560.
15. Singer WD, Rabe EF, Haller JS. The effect of ACTH therapy upon infantile spasms. J Pediatr 1980, 96:485-489.
16. Sorel L. A propos de 187 observations de la maladie des spasmes en flexion de la première enfance à Symptomatologie typique et atypique et son traitement: évolution thérapeutique à long terme. In Lugaresi E, Fazzaglia P, Tassinari CA (eds): Evolution and prognosis of epilepsies. Bologna: Auto Gaggi, 1973, p 85-89.
17. Sorel L, Dusaucy-Bauloye A. A propos de 21 cas d'hipsarythmie de Gibbs: son traitement spectaculaire par l'ACTH. Acta Neurol Belg 1958, 58:130-141.
18. Vasquez HJ, Turner M. Epilepsia em flexão generalizada. Arch Argent Pediatr 1951, 35:111-141.
19. West WJ. On a peculiar form of infantile convulsions. Lancet 1841, 1:724-725.

Datas de Publicação

Publicação nesta coleção
19 Jan 2011
Data do Fascículo
Set 1993

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

[1] 1. Albuquerque M, Muszkat M, Vergani MIC, Campos CJR. Síndrome de West: apresentação de 25 casos. Rev Paul Pediatr 1990, 8:76-78.

[2] 2. Antoniuk SA. Síndrome de West: urgência em pediatria. J Pediatr 1901, 67:205-206.

[3] 3. Chevrie JJ. Syndrome de West. Encycl Méd Chir Neurologie. Paris, 1979, 17045 A80.

[4] 4. Chevrie JJ, Aicardi J. Facteurs étiologiques des spasmes en flexion du nourisson: étude de 81 cas. Arch Franç Pédiat 1967, 24:511-520.

[5] 5. Duffner PK, Cohen ME. Infantile spasms associated with histidinemia. Neurology 1975, 25:195-197.

[6] 6. Fejerman N, Medina CS. Síndrome de West. In Fejerman N, Medina CS (eds): Convulsiones en la infancia, diagnóstico y tratamiento. Ed 2. Buenos Aires: Ateneo, 1986, p 128-147.

[7] 7. Gastaut H, Soulayrol R, Roger J, Pinsard N (eds). L'encéphalopatie myoclonique infantile avec hipsarrythmie (Syndrome de West). Paris: Masson, 1964.

[8] 8. Gibbs FA, Gibbs EL. Atlas of electroencephalography, epilepsy. Cambridge: Addison-Wesley, 1952, Vol 2.

[9] 9. Jeavons PM. West syndrome: infantile spasms. In Roger J, Dravet C, Bureau M, Dreifuss FE, Wolf P (eds): Epileptic syndromes in infancy, childhood and adolescence. London: John Libbey, 1985, p 42-50.

[10] 10. Kellaway P, Hrachovy RA, Frost JD. Precise characterization and quantification of infantile spasms. Ann Neurol 1979, 6:214-218.

[11] 11. Lacy JR, Penry JK. Infantile spasms. New York: Raven Press, 1976.

[12] 12. Lombroso CT. A prospective study of infantile spasms: clinic and therapeutic correlations. Epilepsia 1983, 24:135-158.

[13] 13. Marques-Dias MJ. Síndrome de West. In Diament A, Cypel S (eds): Neurologia Infantil-Lefèvre. Ed 2. Rio de Janeiro: Ateneu, 1990, p 1025-1030.

[14] 14. Siemes H, Spohr HL, Michael T, Nau H. Therapy of infantile spasms with valproate: results of a prospective study. Epilepsia 1988, 29:553-560.

[15] 15. Singer WD, Rabe EF, Haller JS. The effect of ACTH therapy upon infantile spasms. J Pediatr 1980, 96:485-489.

[16] 16. Sorel L. A propos de 187 observations de la maladie des spasmes en flexion de la première enfance à Symptomatologie typique et atypique et son traitement: évolution thérapeutique à long terme. In Lugaresi E, Fazzaglia P, Tassinari CA (eds): Evolution and prognosis of epilepsies. Bologna: Auto Gaggi, 1973, p 85-89.

[17] 17. Sorel L, Dusaucy-Bauloye A. A propos de 21 cas d'hipsarythmie de Gibbs: son traitement spectaculaire par l'ACTH. Acta Neurol Belg 1958, 58:130-141.

[18] 18. Vasquez HJ, Turner M. Epilepsia em flexão generalizada. Arch Argent Pediatr 1951, 35:111-141.

[19] 19. West WJ. On a peculiar form of infantile convulsions. Lancet 1841, 1:724-725.