Síndrome de West evoluindo para síndrome de Lennox-Gastaut em paciente com cisticercose cerebral

Frochtengarten, Mario Luiz; Scarante, Ozir

doi:10.1590/S0004-282X1973000400011

Resumos

Os autores relatam a evolução clínico-laboratorial do caso de uma criança que, aos 18 meses de idade, apresentou uma síndrome de West de instalação gradativa, evoluindo ulteriormente para uma síndrome de Lennox-Gastaut, causadas por cisticercose cerebral, etiologia ainda não registrada em nenhum dos quadros.

The case of a 6 year old girl with Lennox-Gastaut's syndrome caused by cysticercosis of the central nervous system (CNS) is reported. The illness began at 18 months of age with right sided convulsions during an upper respiratory tract infection. Electroencephalogram (EEG) showed depression of rapid rythm on the left side. There was progressive mental deterioration, right spastic hemiparesis and aphasia. Then the EEG showed progressively worsening of generalized irritative activity, with spikes and sharp waves. At the age of 3 years there appeared flexion myoclonic spasms and EEG showed a hypsarythmic pattern. Both clinical and EEG picture allowed the diagnosis of West's syndrome. In the following months the patient presented akinetic seizures, myoclonic jerks and generalized convulsive seizures with turning of the head to the right. EEG revealed slow spike-waves thus leading, at this time, to the diagnosis of Lennox-Gastaut's síndrome. Brain cysticercosis was diagnosed on the basis of 8 repeated positive cerebrospinal fluid examinations.

Síndrome de West evoluindo para síndrome de Lennox-Gastaut em paciente com cisticercose cerebral

West's syndrome changing to Lennox-Gastaut's syndrome in a patient with cerebral cysticercosis

Mario Luiz Frochtengarten^I; Ozir Scarante^II

^IEletrencefalografista do Hospital dos Servidores Públicos do Estado de São Paulo (Prof. Roberto Melaragno Filho)

^IIChefe da Seção de Diagnóstico e Terapêutica da Clínica Neurológica do Hospital dos Servidores Públicos do Estado de São Paulo (Prof. Roberto Melaragno Filho)

RESUMO

Os autores relatam a evolução clínico-laboratorial do caso de uma criança que, aos 18 meses de idade, apresentou uma síndrome de West de instalação gradativa, evoluindo ulteriormente para uma síndrome de Lennox-Gastaut, causadas por cisticercose cerebral, etiologia ainda não registrada em nenhum dos quadros.

SUMMARY

The case of a 6 year old girl with Lennox-Gastaut's syndrome caused by cysticercosis of the central nervous system (CNS) is reported. The illness began at 18 months of age with right sided convulsions during an upper respiratory tract infection. Electroencephalogram (EEG) showed depression of rapid rythm on the left side. There was progressive mental deterioration, right spastic hemiparesis and aphasia. Then the EEG showed progressively worsening of generalized irritative activity, with spikes and sharp waves. At the age of 3 years there appeared flexion myoclonic spasms and EEG showed a hypsarythmic pattern. Both clinical and EEG picture allowed the diagnosis of West's syndrome. In the following months the patient presented akinetic seizures, myoclonic jerks and generalized convulsive seizures with turning of the head to the right. EEG revealed slow spike-waves thus leading, at this time, to the diagnosis of Lennox-Gastaut's síndrome. Brain cysticercosis was diagnosed on the basis of 8 repeated positive cerebrospinal fluid examinations.

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Clínica Neurológica Hospital do Servidor Público Estadual Rua Pedro de Toledo 1800 04039 São Paulo, SP Brasil.

1. BAIRD, H. W. & BOROWSKI, L. G. Infantile myoclonic seizures. J. Pediat. 50:332, 1957.
2. CHRISTIAN, W. Atlas de Electroencefalograma Clínica. Versão espanhola. Editorial Paz Montalvo, Madrid, 1971. pág. 109.
3. COLEMAN, M. Infantile spasms associated with 5-hydroxytryptophan administration in patients with Down's syndrome. Neurology (Mineapolis) 21:911, 1971.
4. GASTAUT, H.; SALTIEL, J.; RAYBAUD, C.; PITOT, M. & MAYNADIER, A. A propos du traitement par ACTH des encephalities myocloniques de la première enfance avec dysritmie majeure. Pediatric 14:35, 1959.
5. GASTAUT, H.; ROGER, J. & SOULAYROL, R. Encéphalopathie myoclonique infantile avec hypsarythmie (syndrome de West). In Encyclopedie Médico-Chirurgicale, Neurologie, vol. 1, tome 2, Paris, 1965.
6. GASTAUT, H.; ROGER, J.; SOULAYROL, R.; TASSINARI, C. A.; REGIS, H.; DRAVET, C.; BERNARD, R.; PINSARD, N. & SAINT-JEAN, M. Childhood epileptic encephalopathies with diffuse slow spike-waves (otherwise known as "petit mal variant") or Lennox syndrome. Epilepsia (Amsterdam) 7:139, 1966.
7. LENNOX, W. G. & LENNOX, M. A. Epilepsy and Related Disorders. Little Brown and Co., Boston, 1960, pág. 165.
8. LOW, N. L. Infantile spasms. Pediat. Clin. North America 7:373, 1960.
9. MILLICHAP, J. G.; BICKFORD, R. G.; KLASS, D. W. & BOCKUS, R. E. Infantile spasms, hypsarhythmia and mental retardation. A study of etiologic factors in 61 patients. Epilepsia (Amsterdam) 3:188, 1962.
10. NIEDERMEYER, E. The Generalized Epilepsies. Charles C. Thomas Publisher, Springfield (Illinois), 1972. pág. 58.
11. OLLER-DAURELLA, L. El Síndrome de Lennox y sus Diferentes Formas Clínico-eletroencefalograficas. Editorial Espaxs, Barcelona, 1967. pág. 33.
12. SOREL, L. L'épilepsie myokinetique grave de la première enfance avec pointe-onde lente et son traitement (petit mal variant). Rev. neurol. (Paris), 110:215, 1964.

Datas de Publicação

Publicação nesta coleção
15 Abr 2013
Data do Fascículo
Dez 1973

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

[1] 1. BAIRD, H. W. & BOROWSKI, L. G. Infantile myoclonic seizures. J. Pediat. 50:332, 1957.

[2] 2. CHRISTIAN, W. Atlas de Electroencefalograma Clínica. Versão espanhola. Editorial Paz Montalvo, Madrid, 1971. pág. 109.

[3] 3. COLEMAN, M. Infantile spasms associated with 5-hydroxytryptophan administration in patients with Down's syndrome. Neurology (Mineapolis) 21:911, 1971.

[4] 4. GASTAUT, H.; SALTIEL, J.; RAYBAUD, C.; PITOT, M. & MAYNADIER, A. A propos du traitement par ACTH des encephalities myocloniques de la première enfance avec dysritmie majeure. Pediatric 14:35, 1959.

[5] 5. GASTAUT, H.; ROGER, J. & SOULAYROL, R. Encéphalopathie myoclonique infantile avec hypsarythmie (syndrome de West). In Encyclopedie Médico-Chirurgicale, Neurologie, vol. 1, tome 2, Paris, 1965.

[6] 6. GASTAUT, H.; ROGER, J.; SOULAYROL, R.; TASSINARI, C. A.; REGIS, H.; DRAVET, C.; BERNARD, R.; PINSARD, N. & SAINT-JEAN, M. Childhood epileptic encephalopathies with diffuse slow spike-waves (otherwise known as "petit mal variant") or Lennox syndrome. Epilepsia (Amsterdam) 7:139, 1966.

[7] 7. LENNOX, W. G. & LENNOX, M. A. Epilepsy and Related Disorders. Little Brown and Co., Boston, 1960, pág. 165.

[8] 8. LOW, N. L. Infantile spasms. Pediat. Clin. North America 7:373, 1960.

[9] 9. MILLICHAP, J. G.; BICKFORD, R. G.; KLASS, D. W. & BOCKUS, R. E. Infantile spasms, hypsarhythmia and mental retardation. A study of etiologic factors in 61 patients. Epilepsia (Amsterdam) 3:188, 1962.

[10] 10. NIEDERMEYER, E. The Generalized Epilepsies. Charles C. Thomas Publisher, Springfield (Illinois), 1972. pág. 58.

[11] 11. OLLER-DAURELLA, L. El Síndrome de Lennox y sus Diferentes Formas Clínico-eletroencefalograficas. Editorial Espaxs, Barcelona, 1967. pág. 33.

[12] 12. SOREL, L. L'épilepsie myokinetique grave de la première enfance avec pointe-onde lente et son traitement (petit mal variant). Rev. neurol. (Paris), 110:215, 1964.