Síndrome de lennox-gastaut com início na vida adulta? a respeito de um caso

Trevisol-Bittencourt, P. C.; Sander, J. W. A. S.

doi:10.1590/S0004-282X1990000400020

Resumos

Apesar de ter sido relatada anteriormente, a sindrome de Lennox-Gastaut (SLG) só foi definitivamente aceita como entidade distinta em 1966. Desde então, o epônimo tem sido utilizado para designar encefalopatia severa da infância, caracterizada fundamentalmente por crises epilépticas refratárias de diversos tipos associadas com alterações eletrográficas típicas. Entretanto, vários autores consideram que o rótulo de SLG está abrigando quadros nosológicos distintos, que deveriam ser separados e individualizados. Ilustrando esta situação, o caso de um paciente que iniciou quadro de «SLG-like» aos 20 anos de idade é relatado. Adicionando mais confusão, alguns casos semelhantes têm sido descritos nos últimos anos sob variada nomenclatura. Desde que um dos principais critérios para o diagnóstico da SLG é a idade dependência, como deverão então ser chamados tais quadros? Revisão dos critérios empregados para o diagnóstico da SLG se faz necessária.

The Lennox-Gastaut syndrome (LGS), although described earlier, was first accepted in 1966 as a form of severe childhood epileptic encephalopathy, characterized by refractory epileptic seizures of diverse types, typical EEG abnormalities and slow mental development. This condition, however, is surrounded by controversy as the clinical criteria used by various authors to define it are quite different. It is very likely that this eponym has been used to harbour distinct epileptic conditions, all of which have in common a slow spike and wave, complex in their EEG recording. Despite the fact that one of the hallmarks of LGS is an onset in early childhood, to add to the confusion, some cases of a LGS-like condition with onset in adulthood have been described. We report here one such case: a 28 year old man whose LGS-like condition started after severe head trauma at the age of 20. In addition we briefly íeview the difficulties in making this diagnosis, using this case to illustrate these aspects.

Síndrome de lennox-gastaut com início na vida adulta? a respeito de um caso

Late onset Lennox-Gastaut like syndrome: a case report

P. C. Trevisol-Bittencourt^I; J. W. A. S. Sander^II

^IDepartamento de Neurologia, Hospital Universitário, Universidade Federal de Santa Catarina

^IIINSEG/Epilepsy Research Group, Institute of Neurology, Queen Square, London, TJK

RESUMO

Apesar de ter sido relatada anteriormente, a sindrome de Lennox-Gastaut (SLG) só foi definitivamente aceita como entidade distinta em 1966. Desde então, o epônimo tem sido utilizado para designar encefalopatia severa da infância, caracterizada fundamentalmente por crises epilépticas refratárias de diversos tipos associadas com alterações eletrográficas típicas. Entretanto, vários autores consideram que o rótulo de SLG está abrigando quadros nosológicos distintos, que deveriam ser separados e individualizados. Ilustrando esta situação, o caso de um paciente que iniciou quadro de «SLG-like» aos 20 anos de idade é relatado. Adicionando mais confusão, alguns casos semelhantes têm sido descritos nos últimos anos sob variada nomenclatura. Desde que um dos principais critérios para o diagnóstico da SLG é a idade dependência, como deverão então ser chamados tais quadros? Revisão dos critérios empregados para o diagnóstico da SLG se faz necessária.

SUMMARY

The Lennox-Gastaut syndrome (LGS), although described earlier, was first accepted in 1966 as a form of severe childhood epileptic encephalopathy, characterized by refractory epileptic seizures of diverse types, typical EEG abnormalities and slow mental development. This condition, however, is surrounded by controversy as the clinical criteria used by various authors to define it are quite different. It is very likely that this eponym has been used to harbour distinct epileptic conditions, all of which have in common a slow spike and wave, complex in their EEG recording. Despite the fact that one of the hallmarks of LGS is an onset in early childhood, to add to the confusion, some cases of a LGS-like condition with onset in adulthood have been described. We report here one such case: a 28 year old man whose LGS-like condition started after severe head trauma at the age of 20. In addition we briefly íeview the difficulties in making this diagnosis, using this case to illustrate these aspects.

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Dr. P. C. Trevisol-Bittencourt - National Hospital, Chalfont C\entre for Epilepsy - Chalfont St. Peter, Gerrards Cross,. Bucks SL9 ORJ - UK.

1. Aicardi J - The problem of the Lennox syndrome. Develop Med Child Neurol 15:77, 1973.
2. Bauer G, Aichner F, Saltuari L - Epilepsies with diffuse slow spikes and waves of late onset. Eur Neurol 22:344, 1983.
3. Bauer G, Benke T, Bohr K - The Lennox-Gastaut syndrome in adulthood. In Nieder-meyer E, Degen R (eds): The Lenox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 317.
4. Beaumanoir A - Les limites nosologiques du syndrome de Lennox-Gastaut. Rev EEG Neurophysiol 11:468, 1981.
5. Beaumanoir A, Foletti G, Magistris M, Volanschi D - Status epilepticus in the Lennox--Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 283.
6. Deonna TH, Ziegler AL, Despland PA - Combined myoclonic-astatic and «benign» focal epilepsy of childhood («atypical benign partial epilepsy*): a separate syndrome? Neuro-pediatrics 17:144, 1986.
7. Gastaut H, Roger J, Soulayrol R, Tassinari C, Regis II, Dravet C, Bernard R, Pinsard N, Saint-Jean M - Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise know as «petit mal variant») or Lennox syndrome. Epilepsia 7:139, 1966.
8. Gastaut H, Gastaut JL, Gonçalves e Silva GE, Fernandez Sanches GR - Relative frequency of different types of epilepsy; a study employing the classification of the international League Against Epilepsy. Epilepsia 16:457, 1975.
9. Ikeno T, Shigematsu H, Miyakoshi M, Ohba A, Yagi K, Seino M - An analytic study of epileptic falls. Epilepsia 26:612, 1985.
10. Karbowski K - Developments in epileptology in the 18th and 19th century prior to the delineation of the Lennox-Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 1.
11. Lennox WG - The petit mal epilepsies. J Am Med Assoc 129:1069, 1945.
12. Lennox WG - Epilepsy and Related Disorders, Vol 1. Little Brown, Boston, I960.
13. Lennox WG, Davis JP - Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics 5:626, 1950.
14. Lipinski CG - Epilepsies with astatic seizures of late onset. Epilepsia 18:13, 1977.
15. Niedermeyer E - The Generalized Epilepsies. Charles Thomas, Springfield, 1972.
16. Niedermeyer E - The Lennox-Gastaut syndrome and its frontiers. Clin Electroenceph 17:117, 1986.
17. Niedermeyer E - The electroencephalogram in the differential diagnosis of the Lennox-Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome, Alan R Liss, Netw York, 1988, pg 177.
18. Noite R, Wolff M, Krageloh-Mann I - The atonic (astatic) drop attacks and their differential diagnosis. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 95:
19. Pazzaglia P, D'Alessandro R, Ambrosetto G, Lugaresi E; - Drop attacks: an ominous change in the evolution of partial epilepsy. Neurology 35:1725, 1985.
20. Roger J, Remy C, Bureau M, Oller-Daurella L, Beaumanoir A, Favel P, Dravet C - Lennox-Gastaut syndrome in the adult. Rev Neurol 143:401, 1987.
21. Roger J, Gambarelli-Dubois D - Neuropathological studies of the Lennox-Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut syndrome, Alan Liss, New York, 1988, pg 79.
22. Sander JWAS, Trevisol-Bittencourt PC, Hardt Y, Shorvon SD - Vigabatrin as an add-on drug in the management of severe epilepsy. Abstracts 18th International Epilepsy Congress, New Delhi, 1989.

Datas de Publicação

Publicação nesta coleção
19 Maio 2011
Data do Fascículo
Dez 1990

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

[1] 1. Aicardi J - The problem of the Lennox syndrome. Develop Med Child Neurol 15:77, 1973.

[2] 2. Bauer G, Aichner F, Saltuari L - Epilepsies with diffuse slow spikes and waves of late onset. Eur Neurol 22:344, 1983.

[3] 3. Bauer G, Benke T, Bohr K - The Lennox-Gastaut syndrome in adulthood. In Nieder-meyer E, Degen R (eds): The Lenox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 317.

[4] 4. Beaumanoir A - Les limites nosologiques du syndrome de Lennox-Gastaut. Rev EEG Neurophysiol 11:468, 1981.

[5] 5. Beaumanoir A, Foletti G, Magistris M, Volanschi D - Status epilepticus in the Lennox--Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 283.

[6] 6. Deonna TH, Ziegler AL, Despland PA - Combined myoclonic-astatic and «benign» focal epilepsy of childhood («atypical benign partial epilepsy*): a separate syndrome? Neuro-pediatrics 17:144, 1986.

[7] 7. Gastaut H, Roger J, Soulayrol R, Tassinari C, Regis II, Dravet C, Bernard R, Pinsard N, Saint-Jean M - Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise know as «petit mal variant») or Lennox syndrome. Epilepsia 7:139, 1966.

[8] 8. Gastaut H, Gastaut JL, Gonçalves e Silva GE, Fernandez Sanches GR - Relative frequency of different types of epilepsy; a study employing the classification of the international League Against Epilepsy. Epilepsia 16:457, 1975.

[9] 9. Ikeno T, Shigematsu H, Miyakoshi M, Ohba A, Yagi K, Seino M - An analytic study of epileptic falls. Epilepsia 26:612, 1985.

[10] 10. Karbowski K - Developments in epileptology in the 18th and 19th century prior to the delineation of the Lennox-Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 1.

[11] 11. Lennox WG - The petit mal epilepsies. J Am Med Assoc 129:1069, 1945.

[12] 12. Lennox WG - Epilepsy and Related Disorders, Vol 1. Little Brown, Boston, I960.

[13] 13. Lennox WG, Davis JP - Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics 5:626, 1950.

[14] 14. Lipinski CG - Epilepsies with astatic seizures of late onset. Epilepsia 18:13, 1977.

[15] 15. Niedermeyer E - The Generalized Epilepsies. Charles Thomas, Springfield, 1972.

[16] 16. Niedermeyer E - The Lennox-Gastaut syndrome and its frontiers. Clin Electroenceph 17:117, 1986.

[17] 17. Niedermeyer E - The electroencephalogram in the differential diagnosis of the Lennox-Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome, Alan R Liss, Netw York, 1988, pg 177.

[18] 18. Noite R, Wolff M, Krageloh-Mann I - The atonic (astatic) drop attacks and their differential diagnosis. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut Syndrome. Alan Liss, New York, 1988, pg 95:

[19] 19. Pazzaglia P, D'Alessandro R, Ambrosetto G, Lugaresi E; - Drop attacks: an ominous change in the evolution of partial epilepsy. Neurology 35:1725, 1985.

[20] 20. Roger J, Remy C, Bureau M, Oller-Daurella L, Beaumanoir A, Favel P, Dravet C - Lennox-Gastaut syndrome in the adult. Rev Neurol 143:401, 1987.

[21] 21. Roger J, Gambarelli-Dubois D - Neuropathological studies of the Lennox-Gastaut syndrome. In Niedermeyer E, Degen R (eds): The Lennox-Gastaut syndrome, Alan Liss, New York, 1988, pg 79.

[22] 22. Sander JWAS, Trevisol-Bittencourt PC, Hardt Y, Shorvon SD - Vigabatrin as an add-on drug in the management of severe epilepsy. Abstracts 18th International Epilepsy Congress, New Delhi, 1989.

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Síndrome de lennox-gastaut com início na vida adulta? a respeito de um caso

Late onset Lennox-Gastaut like syndrome: a case report

Resumos

Datas de Publicação