Abstract
A prospective study of 32 children with epileptic encephalopathies 12 years or younger revealed a high incidence of focal cortical metabolic defects on 18-fluorodeoxyglucose positron emission tomography (PET) not suspected from clinical, EEG, or magnetic resonance imaging findings. PET scans were normal in all five children with typical de novo Lennox-Gastaut syndrome but showed cortical metabolic abnormalities in three out of four with atypical de novo Lennox-Gastaut syndrome, five out of six with Lennox-Gastaut syndrome following infantile spasms, six out of eight with severe myoclonic epilepsy in infancy, one out of two with epilepsy with myoclonic-astatic seizures, and four out of six with an unclassified epileptic encephalopathy. This suggests that some children with epileptic encephalopathies previously thought to have primary generalised seizures or seizures due to multifocal pathology may have unifocal cortical origin for their seizures. Such an origin may be amenable to surgery.
Full text
PDFImages in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Aicardi J. Epileptic encephalopathies of early childhood. Curr Opin Neurol Neurosurg. 1992 Jun;5(3):344–348. [PubMed] [Google Scholar]
- Aicardi J., Levy Gomes A. Clinical and electroencephalographic symptomatology of the 'genuine' Lennox-Gastaut syndrome and its differentiation from other forms of epilepsy of early childhood. Epilepsy Res Suppl. 1992;6:185–193. [PubMed] [Google Scholar]
- Carrazana E. J., Lombroso C. T., Mikati M., Helmers S., Holmes G. L. Facilitation of infantile spasms by partial seizures. Epilepsia. 1993 Jan-Feb;34(1):97–109. doi: 10.1111/j.1528-1157.1993.tb02381.x. [DOI] [PubMed] [Google Scholar]
- Chugani H. T., Mazziotta J. C., Engel J., Jr, Phelps M. E. The Lennox-Gastaut syndrome: metabolic subtypes determined by 2-deoxy-2[18F]fluoro-D-glucose positron emission tomography. Ann Neurol. 1987 Jan;21(1):4–13. doi: 10.1002/ana.410210104. [DOI] [PubMed] [Google Scholar]
- Chugani H. T., Phelps M. E., Mazziotta J. C. Positron emission tomography study of human brain functional development. Ann Neurol. 1987 Oct;22(4):487–497. doi: 10.1002/ana.410220408. [DOI] [PubMed] [Google Scholar]
- Chugani H. T., Shewmon D. A., Shields W. D., Sankar R., Comair Y., Vinters H. V., Peacock W. J. Surgery for intractable infantile spasms: neuroimaging perspectives. Epilepsia. 1993 Jul-Aug;34(4):764–771. doi: 10.1111/j.1528-1157.1993.tb00459.x. [DOI] [PubMed] [Google Scholar]
- Chugani H. T., Shields W. D., Shewmon D. A., Olson D. M., Phelps M. E., Peacock W. J. Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment. Ann Neurol. 1990 Apr;27(4):406–413. doi: 10.1002/ana.410270408. [DOI] [PubMed] [Google Scholar]
- De Vivo D. C., Trifiletti R. R., Jacobson R. I., Ronen G. M., Behmand R. A., Harik S. I. Defective glucose transport across the blood-brain barrier as a cause of persistent hypoglycorrhachia, seizures, and developmental delay. N Engl J Med. 1991 Sep 5;325(10):703–709. doi: 10.1056/NEJM199109053251006. [DOI] [PubMed] [Google Scholar]
- Donat J. F. The age-dependent epileptic encephalopathies. J Child Neurol. 1992 Jan;7(1):7–21. doi: 10.1177/088307389200700102. [DOI] [PubMed] [Google Scholar]
- Dulac O., N'Guyen T. The Lennox-Gastaut syndrome. Epilepsia. 1993;34 (Suppl 7):S7–17. doi: 10.1111/j.1528-1157.1993.tb04593.x. [DOI] [PubMed] [Google Scholar]
- Gur R. C., Sussman N. M., Alavi A., Gur R. E., Rosen A. D., O'Connor M., Goldberg H. I., Greenberg J. H., Reivich M. Positron emission tomography in two cases of childhood epileptic encephalopathy (Lennox-Gastaut syndrome). Neurology. 1982 Oct;32(10):1191–1194. doi: 10.1212/wnl.32.10.1191. [DOI] [PubMed] [Google Scholar]
- Jackson G. D. New techniques in magnetic resonance and epilepsy. Epilepsia. 1994;35 (Suppl 6):S2–13. doi: 10.1111/j.1528-1157.1994.tb05985.x. [DOI] [PubMed] [Google Scholar]
- Maeda N., Watanabe K., Negoro T., Aso K., Ohki T., Ito K., Kato T. Evolutional changes of cortical hypometabolism in West's syndrome. Lancet. 1994 Jun 25;343(8913):1620–1623. doi: 10.1016/s0140-6736(94)93065-1. [DOI] [PubMed] [Google Scholar]
- Miyauchi T., Nomura Y., Ohno S., Kishimoto H., Matsushita M. Positron emission tomography in three cases of Lennox-Gastaut syndrome. Jpn J Psychiatry Neurol. 1988 Dec;42(4):795–804. doi: 10.1111/j.1440-1819.1988.tb01168.x. [DOI] [PubMed] [Google Scholar]
- Olson D. M., Chugani H. T., Shewmon D. A., Phelps M. E., Peacock W. J. Electrocorticographic confirmation of focal positron emission tomographic abnormalities in children with intractable epilepsy. Epilepsia. 1990 Nov-Dec;31(6):731–739. doi: 10.1111/j.1528-1157.1990.tb05514.x. [DOI] [PubMed] [Google Scholar]
- Plouin P., Dulac O., Jalin C., Chiron C. Twenty-four-hour ambulatory EEG monitoring in infantile spasms. Epilepsia. 1993 Jul-Aug;34(4):686–691. doi: 10.1111/j.1528-1157.1993.tb00447.x. [DOI] [PubMed] [Google Scholar]
- Renier W. O., Gabreëls F. J., Jaspar H. H. Morphological and biochemical analysis of a brain biopsy in a case of idiopathic Lennox-Gastaut syndrome. Epilepsia. 1988 Sep-Oct;29(5):644–649. doi: 10.1111/j.1528-1157.1988.tb03776.x. [DOI] [PubMed] [Google Scholar]
- Renier W. O., Renkawek K. Clinical and neuropathologic findings in a case of severe myoclonic epilepsy of infancy. Epilepsia. 1990 May-Jun;31(3):287–291. doi: 10.1111/j.1528-1157.1990.tb05378.x. [DOI] [PubMed] [Google Scholar]
- Snead O. C., 3rd, Nelson M. D., Jr PET does not eliminate need for extraoperative, intracranial monitoring in pediatric epilepsy surgery. Pediatr Neurol. 1993 Sep-Oct;9(5):409–411. doi: 10.1016/0887-8994(93)90115-s. [DOI] [PubMed] [Google Scholar]
- Theodore W. H., Rose D., Patronas N., Sato S., Holmes M., Bairamian D., Porter R. J., Di Chiro G., Larson S., Fishbein D. Cerebral glucose metabolism in the Lennox-Gastaut syndrome. Ann Neurol. 1987 Jan;21(1):14–21. doi: 10.1002/ana.410210105. [DOI] [PubMed] [Google Scholar]
- Vinters H. V., De Rosa M. J., Farrell M. A. Neuropathologic study of resected cerebral tissue from patients with infantile spasms. Epilepsia. 1993 Jul-Aug;34(4):772–779. doi: 10.1111/j.1528-1157.1993.tb00460.x. [DOI] [PubMed] [Google Scholar]
- Vinters H. V., Fisher R. S., Cornford M. E., Mah V., Secor D. L., De Rosa M. J., Comair Y. G., Peacock W. J., Shields W. D. Morphological substrates of infantile spasms: studies based on surgically resected cerebral tissue. Childs Nerv Syst. 1992 Feb;8(1):8–17. doi: 10.1007/BF00316556. [DOI] [PubMed] [Google Scholar]
- Yakoub M., Dulac O., Jambaqué I., Chiron C., Plouin P. Early diagnosis of severe myoclonic epilepsy in infancy. Brain Dev. 1992 Sep;14(5):299–303. doi: 10.1016/s0387-7604(12)80147-1. [DOI] [PubMed] [Google Scholar]
- Yanai K., Iinuma K., Matsuzawa T., Ito M., Miyabayashi S., Narisawa K., Ido T., Yamada K., Tada K. Cerebral glucose utilization in pediatric neurological disorders determined by positron emission tomography. Eur J Nucl Med. 1987;13(6):292–296. doi: 10.1007/BF00256553. [DOI] [PubMed] [Google Scholar]