Cell-mediated and humoral immunity in west syndrome

Montelli, Terezinha C. B.; Iwasso, Maria Tereza R.; Peraçoli, Maria Terezinha S.; Mota, Norma Gerusa S.

doi:10.1590/S0004-282X1981000100001

Abstracts

The immunological status of five children with West syndrome consequent to previous cerebral lesions was investigated. Three children had West syndrome and two were in transition from West to Lennox-Gastaut syndrome. All of them showed cellular immunological deficiencies in the following tests: sensitization to DNCB, intracutaneous reaction to PHA, inhibition of leucocyte migration, blastic transformation of lymphocytes, T and B lymphocytes in peripheric blood and levels of serum immunoglobulins. These immunological deficiencies, of different degrees of severity, were associated to frequent infections in these children. A possible association between the immunological deficiencies and autoimmunity is discussed.

Realizamos avaliações de imunidade celular e humoral em 5 crianças com sindrome de West secundária a lesões cerebrais. Todas apresentaram deficiência de imunidade celular, detectada pelas seguintes provas: sensibilização ao DNCB, reação intracutânea à PHA, inibição da migração de leucócitos, transformação blástica de linfócitos, determinação de linfócitos T e B no sangue periférico e dosagem de imunoglobulinas séricas. Estes exames foram realizados durante a síndrome de West em três crianças e durante evolução de síndrome de West para síndrome de Lennox-Gastaut em duas. Estas alterações imunitárias, de graus variáveis, associam-se a infecções frequentes nessas crianças. Discute-se uma possível associação desses defeitos imunitários com auto-imunidade.

Cell-mediated and humoral immunity in west syndrome

Imunidade celular e humoral na sindrome de West

Terezinha C. B. Montelli^I; Maria Tereza R. Iwasso^II; Maria Terezinha S. Peraçoli^II; Norma Gerusa S. Mota^II

^IDepartment of Neurology, Faculdade de Medicina de Botucatu

^IIDepartment of Immunology, I.B.B.M.A., Botucatu, Brasil

SUMMARY

The immunological status of five children with West syndrome consequent to previous cerebral lesions was investigated. Three children had West syndrome and two were in transition from West to Lennox-Gastaut syndrome. All of them showed cellular immunological deficiencies in the following tests: sensitization to DNCB, intracutaneous reaction to PHA, inhibition of leucocyte migration, blastic transformation of lymphocytes, T and B lymphocytes in peripheric blood and levels of serum immunoglobulins. These immunological deficiencies, of different degrees of severity, were associated to frequent infections in these children. A possible association between the immunological deficiencies and autoimmunity is discussed.

RESUMO

Realizamos avaliações de imunidade celular e humoral em 5 crianças com sindrome de West secundária a lesões cerebrais. Todas apresentaram deficiência de imunidade celular, detectada pelas seguintes provas: sensibilização ao DNCB, reação intracutânea à PHA, inibição da migração de leucócitos, transformação blástica de linfócitos, determinação de linfócitos T e B no sangue periférico e dosagem de imunoglobulinas séricas. Estes exames foram realizados durante a síndrome de West em três crianças e durante evolução de síndrome de West para síndrome de Lennox-Gastaut em duas. Estas alterações imunitárias, de graus variáveis, associam-se a infecções frequentes nessas crianças. Discute-se uma possível associação desses defeitos imunitários com auto-imunidade.

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Faculdade de Medicina de Botucatu - Departamento de Neurologia e Psiquitria - 18600 Rubião Junior - Botucatu, SP - Brasil.

1. AIRÓ, R.; MIHAILESCU, E.; ASTALDI, G. & MEARDI, G. - Skin reactions to phytohemagglutinin. Lancet 1:899, 1967.
2. ALLISON, A. A.; DENMAN, A. M. & BARNES, R. D. - Cooperating and controllling functions of thymus: derived lymphocytes in relation to autoimmunity. Lancet 2:135, 1971.
3. BEAUSSART, M. - Encephalopathie myocloniqt.e du nourrison avec hypsarrhythmia: étude EEG. avant et après traitement par ACTH Rev. Neurol. (Paris) 103:243, 1960.
4. BONFORTE, R. J.; TOPILSKY, M.; SILTZBACH, L. E. & GLADE, P. R. Phytohemagglutinin skin test: a possible in vivo measure of cell-mediated immunity J. Pediatrics. 81:775, 1972.
5. BURGIO, G. R.; CURTONI, E.; GENOVA, R. & MAGRINI, V. - Skin reactivity in childhood: phytohaemagglutinin (PHA) skin test and streptokinase (STK) skin test. Pediatric Research 5:88. 1971.
6. CLAMAN, H. N. & MOORHEAD, I. W. - Heterogeneity of thymus-dependent lymphoid cell functions in the mouse. In Proc. 3 rd. Lepetit Colloquium, North Holland Publ. Co., Amsterdam, pp. 133, 1972.
7. CONVIT, I.; PINARDI, M. E. & ROSAS₎ P. A. - Some considerations regarding the immunology of leprosy. Internat J. Leprosy 39:566, 1971.
8. CURTIS, J. E. & HERSH, E. - Cellular immunity in man: correlation of leukocyte migration inhibition factor formation and delayed hypersensitivity. Cellular Immunology, 8:55. 1973.
9. DIAZ-JOU ANEN, E.; STRICKLAND, R. G. & WILLIAMS, R. C. - Studies on human lymphocytes in the newborn and the aged. Amer. J. Medicine 58:620, 1975.
10. FLEISHER, J.; LUCKASEN, JR.; SABAD, A.; GERTZ, R. C. & KERSEY, J. H. - T and B lymphocyte subpopulation in children. Pediatrics 55:162, 1975.
11. FONTANA, A.; GROB. P. J.; SAUTER, R. & JOLLER, H. - IgA deficiency epilepsy and hydantoin medication. Lancet 2:228, 1979.
12. FRIEDMAN, E. & PAMPIGLIONE, E.; - Prognostic implications of EEG. findings of hypsarrhytmia in first year of life. British Med. J. 4;323, 1971.
13. GASTAUT, H. J. L.; GASTAUT, H.; BERNARD, H. R. R.; PINSARD, N.; SAINT-JEAN, M.; ROGER, J. & DRAVET, C. - Computerized Tomography in the study of West's syndrome. Develop. Med. Child Neurol 20:21, 1978.
14. GROB, P. J. & HEROLD, G. E. - Immunological abnormalities and hydantoins. Bristish Me J. J. 2:561, 1972.
15. HAGBERG, B. - The libriun analogue Mogadon in the treatment of epilepsy children. In Proceedings of the eightenth Congress of Scandinavian Neurologists. Helsinki. Acta Neurol. Scandnavica supl. 31:167, 1967.
16. IRULEGUI, I.; FERRI, R. G.; BARBIERI, D.; QUARENTEI, G.; GONZALES, C. H.; PENNA, H. A. O.; CORRADINI, H. B.; PENNAPHIM, D. & LAGÔA, N. - Immunoglobulins in normal children by Immunoelectrophoresis and quantitative radial immunodiffusion: values from birth to three years. Rev. do Hospital das Clinicas da Faculdade de Medicina de São Paulo 30:198, 1976.
17. LAWLOR, G. J. JR.; STIEHM, E. R.; KAPLAN, M. S.; SENGAR, D. P. S. & TERASARI, P. I. - Phytohemagglutinin (PHA) skin test in the diagnosis os cellular immunodeficiency. J. of Allergy and Clinical Immunol. 52:31, 1973.
18. LISON, M. P. - Contribuição ao estudo da ação de derivados benzoadiazepínicos na epilepsia. Tese_j Faculdade de Medicina de Ribeirão Preto, USP, 1968.
19. LOW, N. L. - Infantile spasms with mental retardation: treatment with cortisone and adrenocorticotropin. Pediatrics 22:1165, 1958.
20. MANCINI, G.; CARBONARA, A. O. & HEREMANS, J. F. - Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry 2: 235, 1965.
21. MENDES, E. & RAPHAEL, A. - Impaired delayed hypersensitivity in patients with South American blastomycosis. J. of Allergy and Clinical Immunol. 47:17, 1971.
22. MENDES, E.; RAPHAEL, A.; MOTA, N. G. S. & MENDES, N. F. - Cell-mediated immunity in leprosy and transfer of delayed hypersensitivity reactions. J. of Allergy and Clinical Immunol. 53:233, 1974 a.
23. MENDES, N. F.; MIKI, S. S. & PEIXINHO, Z. F. - Combined detection of human T and B lymphocyte by rosette formation with sheep erythrocytes and zymosan-C3 complexes. J. of Immunol. 113:531 1974 b.
24. MILLICHAP, J. G. & BICKFORD, R. G. - Infantile spasms, hypsarrhythmia and mental retardation. J. of American Medical Association. 182:523, 1962.
25. MILLICHAP, J. G. & ORTIZ, W. R. - Nitrazepan in mioclonic epilepsies. Amer. J. Dis. Children 112:242, 1966.
26. MORISON, W. L. - Effect of phytohaemagglutinin in the leucocyte migration inhibition test as a measure of cell-mediated immunity. J. of Clinical Pathol. 27:113, 1974.
27. MUSSATTI, C. C; REZKALLAH, M. T.; MENDES, E. & MENDES, N. P. - In vivo and in vitro evaluation of cell-mediated immunity in patients with paracoccidioidomycosis. Cellular Immunology 24:365, 1976.
28. NASPITZ, C. K. & RITCHER, M. - The action of phytohemagglutinin in vivo and in vitro: a review. Progress in Allergy 12:1, 1968.
29. REINSKOV, T. - Demonstration of precipitating antibody to extract of brain tissue in patients with hypsarrhythmia. Acta Paediatrica, suppl. 140:73, 1963.
30. RIIKONEN, R. - Cytomegalovirus infection and infantile spasms. Develop. Med. Child Neurol. 20:570, 1978.
31. STILLER, C. R.; RUSSEL, A. S. & DOSSETOR, J. B. - Auto immunity: present concepts. Ann. Internal Medicine 82:405, 1975.
32. VALDIMARSSON, H.; HIGGS, I. M.; WILLS, R. S.; YAMAMURA, M.; HOBBS, J. R. & HOLT, P. J. L. - Immune abnormalities associated with chronic mucocutaneous candidiasis. Cellular Immunology 6:248, 1973.
33. 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
20 Aug 2012
Date of issue
Mar 1981

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

[1] 1. AIRÓ, R.; MIHAILESCU, E.; ASTALDI, G. & MEARDI, G. - Skin reactions to phytohemagglutinin. Lancet 1:899, 1967.

[2] 2. ALLISON, A. A.; DENMAN, A. M. & BARNES, R. D. - Cooperating and controllling functions of thymus: derived lymphocytes in relation to autoimmunity. Lancet 2:135, 1971.

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

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

[5] 5. BURGIO, G. R.; CURTONI, E.; GENOVA, R. & MAGRINI, V. - Skin reactivity in childhood: phytohaemagglutinin (PHA) skin test and streptokinase (STK) skin test. Pediatric Research 5:88. 1971.

[6] 6. CLAMAN, H. N. & MOORHEAD, I. W. - Heterogeneity of thymus-dependent lymphoid cell functions in the mouse. In Proc. 3 rd. Lepetit Colloquium, North Holland Publ. Co., Amsterdam, pp. 133, 1972.

[7] 7. CONVIT, I.; PINARDI, M. E. & ROSAS₎ P. A. - Some considerations regarding the immunology of leprosy. Internat J. Leprosy 39:566, 1971.

[8] 8. CURTIS, J. E. & HERSH, E. - Cellular immunity in man: correlation of leukocyte migration inhibition factor formation and delayed hypersensitivity. Cellular Immunology, 8:55. 1973.

[9] 9. DIAZ-JOU ANEN, E.; STRICKLAND, R. G. & WILLIAMS, R. C. - Studies on human lymphocytes in the newborn and the aged. Amer. J. Medicine 58:620, 1975.

[10] 10. FLEISHER, J.; LUCKASEN, JR.; SABAD, A.; GERTZ, R. C. & KERSEY, J. H. - T and B lymphocyte subpopulation in children. Pediatrics 55:162, 1975.

[11] 11. FONTANA, A.; GROB. P. J.; SAUTER, R. & JOLLER, H. - IgA deficiency epilepsy and hydantoin medication. Lancet 2:228, 1979.

[12] 12. FRIEDMAN, E. & PAMPIGLIONE, E.; - Prognostic implications of EEG. findings of hypsarrhytmia in first year of life. British Med. J. 4;323, 1971.

[13] 13. GASTAUT, H. J. L.; GASTAUT, H.; BERNARD, H. R. R.; PINSARD, N.; SAINT-JEAN, M.; ROGER, J. & DRAVET, C. - Computerized Tomography in the study of West's syndrome. Develop. Med. Child Neurol 20:21, 1978.

[14] 14. GROB, P. J. & HEROLD, G. E. - Immunological abnormalities and hydantoins. Bristish Me J. J. 2:561, 1972.

[15] 15. HAGBERG, B. - The libriun analogue Mogadon in the treatment of epilepsy children. In Proceedings of the eightenth Congress of Scandinavian Neurologists. Helsinki. Acta Neurol. Scandnavica supl. 31:167, 1967.

[16] 16. IRULEGUI, I.; FERRI, R. G.; BARBIERI, D.; QUARENTEI, G.; GONZALES, C. H.; PENNA, H. A. O.; CORRADINI, H. B.; PENNAPHIM, D. & LAGÔA, N. - Immunoglobulins in normal children by Immunoelectrophoresis and quantitative radial immunodiffusion: values from birth to three years. Rev. do Hospital das Clinicas da Faculdade de Medicina de São Paulo 30:198, 1976.

[17] 17. LAWLOR, G. J. JR.; STIEHM, E. R.; KAPLAN, M. S.; SENGAR, D. P. S. & TERASARI, P. I. - Phytohemagglutinin (PHA) skin test in the diagnosis os cellular immunodeficiency. J. of Allergy and Clinical Immunol. 52:31, 1973.

[18] 18. LISON, M. P. - Contribuição ao estudo da ação de derivados benzoadiazepínicos na epilepsia. Tese_j Faculdade de Medicina de Ribeirão Preto, USP, 1968.

[19] 19. LOW, N. L. - Infantile spasms with mental retardation: treatment with cortisone and adrenocorticotropin. Pediatrics 22:1165, 1958.

[20] 20. MANCINI, G.; CARBONARA, A. O. & HEREMANS, J. F. - Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry 2: 235, 1965.

[21] 21. MENDES, E. & RAPHAEL, A. - Impaired delayed hypersensitivity in patients with South American blastomycosis. J. of Allergy and Clinical Immunol. 47:17, 1971.

[22] 22. MENDES, E.; RAPHAEL, A.; MOTA, N. G. S. & MENDES, N. F. - Cell-mediated immunity in leprosy and transfer of delayed hypersensitivity reactions. J. of Allergy and Clinical Immunol. 53:233, 1974 a.

[23] 23. MENDES, N. F.; MIKI, S. S. & PEIXINHO, Z. F. - Combined detection of human T and B lymphocyte by rosette formation with sheep erythrocytes and zymosan-C3 complexes. J. of Immunol. 113:531 1974 b.

[24] 24. MILLICHAP, J. G. & BICKFORD, R. G. - Infantile spasms, hypsarrhythmia and mental retardation. J. of American Medical Association. 182:523, 1962.

[25] 25. MILLICHAP, J. G. & ORTIZ, W. R. - Nitrazepan in mioclonic epilepsies. Amer. J. Dis. Children 112:242, 1966.

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

[27] 27. MUSSATTI, C. C; REZKALLAH, M. T.; MENDES, E. & MENDES, N. P. - In vivo and in vitro evaluation of cell-mediated immunity in patients with paracoccidioidomycosis. Cellular Immunology 24:365, 1976.

[28] 28. NASPITZ, C. K. & RITCHER, M. - The action of phytohemagglutinin in vivo and in vitro: a review. Progress in Allergy 12:1, 1968.

[29] 29. REINSKOV, T. - Demonstration of precipitating antibody to extract of brain tissue in patients with hypsarrhythmia. Acta Paediatrica, suppl. 140:73, 1963.

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

[31] 31. STILLER, C. R.; RUSSEL, A. S. & DOSSETOR, J. B. - Auto immunity: present concepts. Ann. Internal Medicine 82:405, 1975.

[32] 32. VALDIMARSSON, H.; HIGGS, I. M.; WILLS, R. S.; YAMAMURA, M.; HOBBS, J. R. & HOLT, P. J. L. - Immune abnormalities associated with chronic mucocutaneous candidiasis. Cellular Immunology 6:248, 1973.

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