Immunological disturbance in West and Lennox-Gastaut syndromes

Montelli, Terezinha C. B.; Mota, Norma G. S.; Peraçoli, M. Terezinha S.; Torres, Elza A.; Rezkallah-Iwasso, M. Thereza

doi:10.1590/S0004-282X1984000200005

Abstracts

Cell-mediated and humoral immunity were investigated in 18 patients with West syndrome, 12 with Lennox-Gastaut syndrome and 19 healthy controls. The study included determination of Ô and Â peripheral blood lymphocytes, serum levels of IgG, IgA and IgM, skin sensibilization with DNCB, intracutaneous PHA, leucocyte migration inhibition test and lymphocyte blastic transformation in the presence of PHA. Cell-mediated deficiency was detected in 28 children whereas low levels of immunoglobulins were observed only in 6 children. Immunological disturbances were more prominent in children with West syndrome.

Investigamos a imunidade humoral e celular em 18 pacientes com sindrome de West, 12 com sindrome de Lennox-Gastaut e 19 controles. Os exames realizados foram os seguintes: percentagem de linfócitos Ô e Â no sangue periférico, niveis séricos de IgG, IgA e IgM, sensibilização cutânea com o DNCB, PHA intra dérmica, teste de inibição de migração de leucócitos e transformação blástica de linfócitos em presença de PHA. Detectamos deficiência de imunidade celular em 28 crianças, (18 com sindrome de West e 10 com sindrome de Lennox-Gastaut) e baixos níveis de imunoglobulinas em apenas 6. A depressão imunitária mostrou-se mais intensa nas crianças que apresentam sindrome de West.

Immunological disturbance in West and Lennox-Gastaut syndromes

Distúrbios imunológicos nas sindromes de West e Lennox-Gastaut

Terezinha C. B. Montelli^I; Norma G. S. Mota^II; M. Terezinha S. Peraçoli^II; Elza A. Torres^II; M. Thereza Rezkallah-Iwasso^II

^IDepartment of Neurology, IBBMA-UNESP

^IIDepartment of Immunology, IBBMA-UNESP

SUMMARY

Cell-mediated and humoral immunity were investigated in 18 patients with West syndrome, 12 with Lennox-Gastaut syndrome and 19 healthy controls. The study included determination of Ô and Â peripheral blood lymphocytes, serum levels of IgG, IgA and IgM, skin sensibilization with DNCB, intracutaneous PHA, leucocyte migration inhibition test and lymphocyte blastic transformation in the presence of PHA. Cell-mediated deficiency was detected in 28 children whereas low levels of immunoglobulins were observed only in 6 children. Immunological disturbances were more prominent in children with West syndrome.

RESUMO

Investigamos a imunidade humoral e celular em 18 pacientes com sindrome de West, 12 com sindrome de Lennox-Gastaut e 19 controles. Os exames realizados foram os seguintes: percentagem de linfócitos Ô e Â no sangue periférico, niveis séricos de IgG, IgA e IgM, sensibilização cutânea com o DNCB, PHA intra dérmica, teste de inibição de migração de leucócitos e transformação blástica de linfócitos em presença de PHA.

Detectamos deficiência de imunidade celular em 28 crianças, (18 com sindrome de West e 10 com sindrome de Lennox-Gastaut) e baixos níveis de imunoglobulinas em apenas 6.

A depressão imunitária mostrou-se mais intensa nas crianças que apresentam sindrome de West.

Full text available only in PDF format.

Texto completo disponível apenas em PDF.

Departamento de Neurologia IBBMA, UNESP - 18610 Rubião Junior, Botucatu, SP - Brasil.

1. BEAUSSART, M. - Encephalopathie myoclonique du nourrison avec hypsarrhythmia: étude EEG, avant et après traitement par ACTH. Rev. neurol. (Paris) 103:243, 1960.
2. BONFORTE, R. J.; TOPILSKY, M.; SILTZBACH, L. E. & GALDE P. R. - Phytohemagglutitinin skin test: a possible in vivo measure of cell-mediated immunity. J. Pediatrics 81:775, 1972.
3. BUGIANI, O.; TOSO, V.; GATTI, R.; MANCARDI, G. L. & LEONARDI, A. - Primary immunodeficiency with early encephalopathy in two siblings. Eur. Neurol. 13:405, 1975.
4. CHEVRIE, J. J. - Syndrome de West. Encycl. Med. Chir. Neurologie 17045 A80, 5. Paris, 1979.
5. FONTANA, Á.; GROB, P. J.; SAUTER R. & JOLLER, H. - IgA deficiency, epilepsy and hydantoin medication. Lancet 2:228, 1976.
6. FRIEDMAN, E. & PAMPIGLIONE, E. - Prognostic implications of EEG findings of hypsarrhythmia in first year of life. Brit. med. J. 4:32, 1971.
7. GROB, P. J. & HEROLD, G. E. - Immunological abnormalities and hydantoins. Brit. med. J. 2:561, 1972.
8. HAGBERG, B. - The librium analogue Mogadon in the treatment of epilepsy children. In Proccedings of the eigtenth Congress of Scandinavian Neurologists. Helsinki. Acta neurol. scand. (suppl.) 31:167, 1967.
9. IRULEGUI, I.; FERRI, R. G.; BARBIERI, D.; QUARENTEI, G.; GONZALES, C. Ç.; PENNA, H. A. O.; CORRADINI, Ç. Â.; PENNAPHIM, D. & LAGOA, N. - Immunoglobulins in normal children by Immunoelectrophoresis and quantitative radial immunodiffusion: values from birth to three years. Rev. Hosp. Clin. Fac. Med. S. Paulo 30:198, 1976.
10. LAWLOR, G. J., Jr.; STIEHM, E. R.; KAPLAN, M. S.; SENGAR, D. P. S. & TERASAKI, P. I. - Phytohemagglutinin (PHA) skin test in the diagnosis of cellular immunodeficiency. J. Allergy clin. Immunol. 52:31, 1973.
11. MANCINI, G.; CARBONARA, H. O.; HEREMANS, J. F. - Immunochemical quantification on antigens by single radial immunodiffusion. Immunochemistry 2:235, 1965.
12. MENDES, Å.; RAPHAEL, Ç.; MOTA, Í. G. S. & MENDES, Í. F. - Cell-mediated immunity in leprosy and transfer of delayed hipersensivity reactions. J. Allergy clin. Immunol. 53:233, 1974.
13. MENDES, Í. F.; MIKI, S. S. & PEIXINHO, Æ. F. - Combined detection and human Ô and Â lymphocyte by rosette formation with sheep erythrocytes and zymosan-G complexes. J. Immunol. 113:531, 1974.
14. MILLICHÁP, J. G. & ORTIZ, W. R. - Nitrazepan in mioclonic epilepsies. Amer. J. Dis. Child. 112:242, 1966.
15. MONTELLI, T. C. B.; REZKALLAH-IWASSO, Ì. T.; PERAÇOLI, Ì. T. S. & MOTA, Í. G. S. - Cell-mediated and humoral immunity in West syndrome. Arq. Neuro. Psiquiat. (São Paulo) 39:1, 1981.
16. MORISON, W. L. - Effect of phytohaemagglutinin in the leucocyte migration inhibition test as a mesure of cell-mediated immunity. J. clin. Pathol. 27:113, 1974.
17. MUSSATTI, C. C; REZKALLAH, Ì. T.; MENDES, Å. & MENDES, Í. F. - In vivo and in vitro evaluation of cell-mediated immunity in patients with paracoccidioidomycosis. Cell. Immunol. 24:365, 1976.
18. NEUWELT, E. A. & KEMP CLARK, W. - Ataxia teleangiectasia (chap 5). In: NEIXWELT, Å. Á.; KEMP CLARK W. (eds.) Clinical aspects of neuroimmunology. Willians & Wilkins, 1978. p. 120.
19. NEUWELT, E. A. & KEMP CLARK, W. - Methods and techniques important in neuroimmunology (chap 2). In: NEUWELT, Å. Á.; KEMP CLARK, E. (eds.) Clinical aspects of neuroimmunology. Willians & Wilkins, 1978. p. 15.
20. NIWA, Y.; NIWA, H.; HOHMURA, J.; OU, D. W. YOKOYAMA, Ì. M. - Studies on the dinitroclorobenzene (DNCB) sensitization test. Ann. Allergy 48:108, 1982.
21. RIIKONEN, R. - Cytomegalovirus infection and infantile spasms. Develop. Med. Child. Neurol. 20:570, 1978.
22. RYNIEWICZ, B. & PAWINSKA, M. - Preliminary immunological studies in spinal muscular atrophy. Eur. J. Pediatr. 128:57, 1978.
23. SANJEEV RAI, B.; KRISHNAMURTHY, P. N. & RÀMA RAO, B. R. - Study of cell-mediated immunity in malnourished children using DNCB skin sensitization test. Ind. Pediatr. 18:29, 1981.
24. TART ARA, Á.; VERRI, A. P.; NESPOLI, L.; MOGLIA, A. & BOTTA, M. G. - Immunological findings in epileptic and febrile convulsion patients before and under treatment. Eur. Neurol. 20:306, 1981.
25. VOLZKE, E.; DOOSE, H. & STEPHAN E. - The treatment of infantile spasms and by hypsarrhythmia with Mogadon. Epilepsia 8:64, 1967.

Publication Dates

Publication in this collection
13 Aug 2012
Date of issue
June 1984

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

[1] 1. BEAUSSART, M. - Encephalopathie myoclonique du nourrison avec hypsarrhythmia: étude EEG, avant et après traitement par ACTH. Rev. neurol. (Paris) 103:243, 1960.

[2] 2. BONFORTE, R. J.; TOPILSKY, M.; SILTZBACH, L. E. & GALDE P. R. - Phytohemagglutitinin skin test: a possible in vivo measure of cell-mediated immunity. J. Pediatrics 81:775, 1972.

[3] 3. BUGIANI, O.; TOSO, V.; GATTI, R.; MANCARDI, G. L. & LEONARDI, A. - Primary immunodeficiency with early encephalopathy in two siblings. Eur. Neurol. 13:405, 1975.

[4] 4. CHEVRIE, J. J. - Syndrome de West. Encycl. Med. Chir. Neurologie 17045 A80, 5. Paris, 1979.

[5] 5. FONTANA, Á.; GROB, P. J.; SAUTER R. & JOLLER, H. - IgA deficiency, epilepsy and hydantoin medication. Lancet 2:228, 1976.

[6] 6. FRIEDMAN, E. & PAMPIGLIONE, E. - Prognostic implications of EEG findings of hypsarrhythmia in first year of life. Brit. med. J. 4:32, 1971.

[7] 7. GROB, P. J. & HEROLD, G. E. - Immunological abnormalities and hydantoins. Brit. med. J. 2:561, 1972.

[8] 8. HAGBERG, B. - The librium analogue Mogadon in the treatment of epilepsy children. In Proccedings of the eigtenth Congress of Scandinavian Neurologists. Helsinki. Acta neurol. scand. (suppl.) 31:167, 1967.

[9] 9. IRULEGUI, I.; FERRI, R. G.; BARBIERI, D.; QUARENTEI, G.; GONZALES, C. Ç.; PENNA, H. A. O.; CORRADINI, Ç. Â.; PENNAPHIM, D. & LAGOA, N. - Immunoglobulins in normal children by Immunoelectrophoresis and quantitative radial immunodiffusion: values from birth to three years. Rev. Hosp. Clin. Fac. Med. S. Paulo 30:198, 1976.

[10] 10. LAWLOR, G. J., Jr.; STIEHM, E. R.; KAPLAN, M. S.; SENGAR, D. P. S. & TERASAKI, P. I. - Phytohemagglutinin (PHA) skin test in the diagnosis of cellular immunodeficiency. J. Allergy clin. Immunol. 52:31, 1973.

[11] 11. MANCINI, G.; CARBONARA, H. O.; HEREMANS, J. F. - Immunochemical quantification on antigens by single radial immunodiffusion. Immunochemistry 2:235, 1965.

[12] 12. MENDES, Å.; RAPHAEL, Ç.; MOTA, Í. G. S. & MENDES, Í. F. - Cell-mediated immunity in leprosy and transfer of delayed hipersensivity reactions. J. Allergy clin. Immunol. 53:233, 1974.

[13] 13. MENDES, Í. F.; MIKI, S. S. & PEIXINHO, Æ. F. - Combined detection and human Ô and Â lymphocyte by rosette formation with sheep erythrocytes and zymosan-G complexes. J. Immunol. 113:531, 1974.

[14] 14. MILLICHÁP, J. G. & ORTIZ, W. R. - Nitrazepan in mioclonic epilepsies. Amer. J. Dis. Child. 112:242, 1966.

[15] 15. MONTELLI, T. C. B.; REZKALLAH-IWASSO, Ì. T.; PERAÇOLI, Ì. T. S. & MOTA, Í. G. S. - Cell-mediated and humoral immunity in West syndrome. Arq. Neuro. Psiquiat. (São Paulo) 39:1, 1981.

[16] 16. MORISON, W. L. - Effect of phytohaemagglutinin in the leucocyte migration inhibition test as a mesure of cell-mediated immunity. J. clin. Pathol. 27:113, 1974.

[17] 17. MUSSATTI, C. C; REZKALLAH, Ì. T.; MENDES, Å. & MENDES, Í. F. - In vivo and in vitro evaluation of cell-mediated immunity in patients with paracoccidioidomycosis. Cell. Immunol. 24:365, 1976.

[18] 18. NEUWELT, E. A. & KEMP CLARK, W. - Ataxia teleangiectasia (chap 5). In: NEIXWELT, Å. Á.; KEMP CLARK W. (eds.) Clinical aspects of neuroimmunology. Willians & Wilkins, 1978. p. 120.

[19] 19. NEUWELT, E. A. & KEMP CLARK, W. - Methods and techniques important in neuroimmunology (chap 2). In: NEUWELT, Å. Á.; KEMP CLARK, E. (eds.) Clinical aspects of neuroimmunology. Willians & Wilkins, 1978. p. 15.

[20] 20. NIWA, Y.; NIWA, H.; HOHMURA, J.; OU, D. W. YOKOYAMA, Ì. M. - Studies on the dinitroclorobenzene (DNCB) sensitization test. Ann. Allergy 48:108, 1982.

[21] 21. RIIKONEN, R. - Cytomegalovirus infection and infantile spasms. Develop. Med. Child. Neurol. 20:570, 1978.

[22] 22. RYNIEWICZ, B. & PAWINSKA, M. - Preliminary immunological studies in spinal muscular atrophy. Eur. J. Pediatr. 128:57, 1978.

[23] 23. SANJEEV RAI, B.; KRISHNAMURTHY, P. N. & RÀMA RAO, B. R. - Study of cell-mediated immunity in malnourished children using DNCB skin sensitization test. Ind. Pediatr. 18:29, 1981.

[24] 24. TART ARA, Á.; VERRI, A. P.; NESPOLI, L.; MOGLIA, A. & BOTTA, M. G. - Immunological findings in epileptic and febrile convulsion patients before and under treatment. Eur. Neurol. 20:306, 1981.

[25] 25. VOLZKE, E.; DOOSE, H. & STEPHAN E. - The treatment of infantile spasms and by hypsarrhythmia with Mogadon. Epilepsia 8:64, 1967.