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2012, Number 1

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Rev Cubana Pediatr 2012; 84 (1)

Etiological characterization of the symptomatic Lennox-Gastaut syndrome

Valdivia ÁI, Marrero MP

Language: Spanish
References: 18
Page: 22-32
PDF size: 82.50 Kb.


ABSTRACT

Introduction: the Lennox-Gastaut syndrome is an age-dependent epileptic encephalopathy very severe due to its drug-resistance and the associated inabilities.
Objectives: to characterize the symptomatic Lennox-Gastaut syndrome, according to sex, onset age and diagnosis, type of predominant crises, findings in neuroimage and etiology.
Methods: a retrospective and descriptive study was conducted in 36 patients discharged from the neuropediatric service of the "Juan Manuel Márquez" Children Hospital diagnosed with symptomatic Lennox-Gastaut syndrome and underwent neuroimage studies (computerized axial tomography and nuclear magnetic resonance). Qualitative variables were statistically described by means of absolute frequencies and percentage figures. For interesting percentages, its 95 % confidence interval (CI) was estimated (95 % CI).
Results and conclusions: the symptomatic Lennox-Gastaut syndrome was more frequent in males starting with epilepsy before one year old. The 33 % had West's syndrome. More than 60 % was diagnosed before the four years old. The more frequent crises were the tonic and atonic ones of neck. The CAT allows locating zones of atrophy and few structural lesions signaled b y nuclear magnetic resonance (NMR) as alterations of neuronal migration. After the central nervous system (CNS) malformations, the more frequent cause was the perinatal hypoxia.


REFERENCES

  1. Archila R, Papazian O. Síndrome de Lennox-Gastaut. Rev Neurol. 1999;29:346-9.

  2. Commission of Classification and Terminology of the International League against Epilepsy. Proposal of a revised classification of epilepsies and epileptic syndromes. Epilepsia. 1989;30:389-99.

  3. Van Rijckevorsel K. Treatment of Lennox-Gastaut syndrome: overview and recent findings. Neuropsychiatric Disease and Treatment. 2008;4(6):1001-19.

  4. Tareq AS, Stephen L. Lennox-Gastaut syndrome, review of the literature and a case report. Head & Face Medicine. 2008;4:9.

  5. Correia JA, Schweder PM, Mews PJ, Patel R, Law AJ. Lennox-Gastaut syndrome and idiopathic intracranial hypertension. J Clin Neurosci. 2010;17(9):1208-9.

  6. Rufo Campos M. Interrelaciones de las encefalopatías epilépticas durante la Infancia. Rev Neurol. 2000;30(1):74-80.

  7. Crumrine P. Lennox-Gastaut Syndrome. J Child Neurol. 2002;17:70.

  8. Shields WD. Diagnosis of Infantile Spasms, Lennox-Gastaut Syndrome, and Progressive Myoclonic Epilepsy. Epilepsia. 2004;45(5):2-4.

  9. Parrini E, Ferrari AR, Dorn T, Walsh CA, Guerrini R. Bilateral frontoparietal polymicrogyria, Lennox-Gastaut syndrome, and GPR56 gene mutations. Epilepsia. 2009;50(6):1344-53.

  10. Alva Moncayo E, Horta Martínez AJ. Asociación entre malformaciones congénitas del sistema nervioso central y epilepsia en pacientes pediátricos de México. Rev Neurol. 2004;39:222-6.

  11. Hancock EC, Cross HH. Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev. 2009;(3):CD003277.

  12. Glauser TA. Lennox-Gastaut Syndrome [homepage en internet]. USA; Medscape Reference; 2011 [citado 25 de Octubre de 2011]. Disponible en: http://emedicine.medscape.com/article/1176735-overview

  13. Herranz JL, Casas-Fernandez C, Campistol J, Campos-Castelló J, Rufo-Campos M, Torres-Falcón A, et al. Lennox-Gastaut syndrome in Spain: a descriptive retrospective epidemiological study. Rev Neurol. 2010;50(12):711-7.

  14. Arzimanoglou A, French J, Blume WT, Cross JH, Ernst JP, Feucht M, et al. Lennox Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol. 2009;8(1):82-93.

  15. Matheos Beato F. Nuevas perspectivas en el tratamiento farmacológico de las epilepsias. Inf Terap Sist Nac Salud. 1999;23:104-11.

  16. RamachandranNair R, Otsubo H, Shroff MM, Ochi A, Weiss SK, Rutka JT, et al. MEG predicts outcome following surgery for intractable epilepsy in children with normal or nonfocal MRI findings. Epilepsia. 2007;48(1):149-57.

  17. Terra-Bustamante VC, Inuzuka LM, Fernandes RM, Escorsi-Rosset S, Wichert-Ana L, Alexandre V, et al. Outcome of hemispheric surgeries for refractory epilepsy in pediatric patients. Childs Nerv Syst. 2007;23(3):321-6.

  18. Piñol-Ripoll G, Mauri-Llerda JA, Rumià J, Carreño M, de la Puerta González-Miró I, Mostacero-Miguel E. Alteraciones de la migración neuronal: una causa de epilepsia farmacorresistente curable. Rev Neurol. 2006;43:20-4.




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