Electroencephalography in congenital malformations of the central nervous system

Electroencefalografia en las malformaciones congénitas del sistema nervioso central

Electroencephalography in congenital malformations of the central nervous system

Electroencefalografia en las malformaciones congénitas del sistema nervioso central

Patrícia Campos^I; Guillermo Cruz^II; Rodolfo Lizarraga^III; Ernesto Bancalari^IV; Daniel Guillen^IV; Carlos Castañeda^V

^INeuropediatrist, Hospital Cayetano Heredia, Universidad Peruana Cayetano Heredia

^IINeurologist and Electroencephalographist, Instituto Peruano de Seguridad Social

^IIINeurologist and Electro-encephalographist, Hospital de la Sanidad de las Fuerzas Policiales

^IVNeurologist, Hospital Cayetano Heredia

^VNeurologist, Instituto Nacional de Enfermedades Neurológicas

SUMMARY

We studied clinical and EEG features of 36 cases with congenital malformations of the CNS. Patients were followed at the outpatient clinic of Hospital Cayetano Heredia and of Hogar Clinica San Juan de Dios in Lima-Peru, from January 1984 to June 1992. Eighty percent of the patients had convulsive syndromes and mental retardation. The most frequent malformation was agenesis of corpus callosum, and it was not possible to find a "typical" EEG pattern. The second were porencephalic cysts, with a good clinical-EEG correlation. There were two typical cases of schizencephaly, one of hemimegalencephaly with good prognosis, and one of holoprosencephaly. The results are compared to those obtained for a series we previously reported. Data discussed take into account reports on the subject registered in the literature. It is concluded that EEG is an useful method to evaluate possible CNS malformations in developing countries.

Key words: central nervous system, congenital malformations, corpus callosum agnesis, porencephaly, hemimegalencephaly, hydrocephaly, EEG.

RESUMEN

Estudiamos aspectos clínicos e del EEG de 36 casos de malformaciones congénitas del sistema nervioso central. Los pacientes fueron seguidos en los consultorios externos del Hospital Cayetano Heredia y del Hogar Clínica San Juan de Dios en Lima-Peru, desde enero 1984 hasta junio 1992. Ochenta por ciento de los pacientes presentaron sindrome convulsivo y retardo mental. La anormalidad mas frecuente correspondió a agenesia de cuerpo calloso y no fue posible identificar un patron EEG "típico". El segundo lugar correspondió a quistes porencefálicos, con buena correlación clínico-EEG. Ademas, hubieron dos casos clínicamente típicos de esquizencefalia, una hemimegalencefalia con buen prognóstico y un caso de holoprosencefalia. Se comparan los resultados con aquellos de casos previamente revisados. Se discuten los dados frente a la literatura acerca de los patrones EEG mas frecuentemente relatados. Se concluye en la utilidad del EEG en países en desarrollo para hacer posible un alto grado de sospecha de una malformación del SNC aun en ausencia de CAT-scan.

Palabras-clave: sistema nervioso central, malformaciones, agenesia de cuerpo calloso, porencefalia, hemimegalencefalia, holoprosencefalia, EEG.

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Aceite: 10-junho-1994.

Dra. Patricia Campos - Dos de Mayo 649 - Lima 27 - Perú.

• 1. Aicardi J, Chevrie JJ, Rousselie F. Le syndrome spasmes en flexion agenesie calleuse, anomalies chorioretiniennes. Arch Franc Pediatr 1960, 26: 1103-1120.
• 2. Baraitser M. The genetics of neurological disorders. Ed 2. London: Oxford Med Publ, 1990.
• 3. Bogacz J, Rebollo MA. Electroencephalographic abnormalities in non tumor hydrocephalus. EEG Clin Neurophysiol 1962, 14: 123-125.
• 4. Brun A, Probst F. The influence of associated cerebral lesions on the morphology of the acallosal brain: a pathological and encephalographic study. Neuroradiology 1973, 6: 121-131.
• 5. Campos P, Hannuch S, Grossmann R, Longo PH, Ferreira de Andrade MJ. EEG en las malformaciones congenitas del sistema nervioso central. Relato oficial. VIII Congreso Peruano de EEG y Neurofisiologia Clínica, Lima-Peru, Octubre 1983.
• 6. Carmon A, Lavy S, Schwartz A. The dynamics of electroenphalographic abnormalities in porencephalia. Neurology 1964, 14: 757-763.
• 7. Damsbka M, Wisniewski K, Sher J. An autopsy case of hemimegalencephaly Brain Dev 1984, 6: 60-64.
• 8. De Myer W, White PT. EEG in holoprosencephaly (archinencephaly). Arch Neurol 1964, 11: 507-520.
• 9. Fariello R, Chun R, Doro JM, Buncie J, Prichard JS. EEG recognition of Aicardi's syndrome. Arch Neurol 1977, 34: 563-566.
• 10. Fois A, Gibbs EL, Gibbs FA. Bilaterally independent sleep patterns in hydrocephalus. Arch Neurol Psychiatry 1958, 79: 264-268.
• 11. Freeman JM, Gold AP. Porencephaly simulating subdural hematoma in childhood: a clinical syndrome secondary to arterial occlusion. Am J Dis Child 1964, 107: 327-336.
• 12. Fusco L, Ferracuti S, Fariello G, Manfredi M, Vigevano F. Hemimegalencephaly and normal intellectual development. J Neurol Neurosurg Psychiatry 1992, 55: 720-722.
• 13. Goldenshoh LN, Clardy ER, Levine K. Agenesis of the corpus callosum. J Nerv Ment Dis 1941, 93: 5567-5580.
• 14. Green JB, Russell AJ. Electroencephalographic asymmetric with midline cyst and deficient corpus callosum: case report. Neurology 1966, 16: 541-545.
• 15. Gross H, Simányi M. Porencephaly. In Vinken PJ, Bruyn GW (eds). Handbook of clinical neurology, Vol 30. Amsterdam: North Holland Publ Co, 1977.
• 16. Jeret JS, Serur D, Wisniewski KE, Lubin R. Clinicopathological findings associated with agenesis of the corpus callosum. Brain Dev 1987, 9: 255-264.
• 17. Kotlarek F, Rodewing R, Brull D. Computed tomographic findings in congenital hemiparesis in childhood and their relation to etiology and prognosis. Neuropediatrics 1981, 2: 101-109.
• 18. Landy H, Ramsay E, Ajmone-Marsan C, Levin B, Brown J, Pasarin G, Quencer R. Temporal lobectomy for seizures associated with unilateral schizencephaly. Surg Neurol 1992, 37: 477-481.
• 19. Loeser J, Alvord EC. Agenesis of the corpus callosum. Brain 1968, 91: 553-570.
• 20. Martinez-Bermejo A, Lopez V, Arcas J, Perez-Higueras A, Morales C, Pacual-Castroviejo I. Early infantile epileptic encephalopathy: a case associated with hemimegalencephaly. Brain Dev 1992, 12: 425-428.
• 21. Miller GM, Stears JC, Guggenheim MA, Wilkening GN. Schizencephaly: a clinical and CT study. Neurology 1984, 34: 997-1001.
• 22. Murphy JP, Garvin JS. The EEG in porencephaly. Arch Neurol Psychiatry 1947, 58: 436-446.
• 23. Page LK, Broen SB, Gargano SP, Shortz RW. Schizencephaly: a clinical study and review. Child's Brain 1975, 1: 348-358.
• 24. Paladin F, Chiron C, Dulac D, Plouin P, Ponsot G. Electroencephalographic aspects of hemimegalencephaly. Dev Med Child Neurol 1989, 31: 377-383.
• 25. Pampliglione G, Lawrence KM. Electroencephalographic and clinico-pathological observations in hydrocephalic children Arch Dis Child 1962: 37: 491-499.
• 26. Probst PP. The prosencephalies. Berlin: Springer-Verlag, 1979.
• 27. Robain O, Floquet CH, Heldt N, Rozenberg F. Hemimegalencephaly: a clinicopathological study of four cases. Neuropathol Appl Neurobiol 1988, 14: 125-135.
• 28. Shoda T, Murase M, Saheki S, Takeuchi N. Holoprosencephaly: a case of holoprosencephaly with a flat EEC Jpn J Clin Pathol 1990, 38: 1205.
• 29. Toglia JU, Lapayowker MS. Agenesis of the corpus callosum. Confinia Neurol 1966, 27: 342-366.
• 30. Warkany J, Lemire R, Cohen MM. Mental retardation and congenital malformations of the central nervous system. London: Year Book Med Publ 1981.
• 31. Yakovlev PL, Wadsworth RC. Schizencephalics: a study of the congenital clefts in the cerebral mantle. I. Clefts with fused lips. J Neuropathol Exp Neurol 1946, 5: 116-130.
• 32. Yakovlev PL, Wadsworth RC. Schizencephalics: a study of the congenital clefts in the cerebral mantle. II. Clefts with hydrocephalus and lips separated. J Neuropathol Exp Neurol 1946, 5: 169-205.

Publication Dates

• Publication in this collection
  19 Jan 2011
• Date of issue
  Dec 1994