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Pyridoxine-dependent epilepsy: EEG investigations and long-term follow-up.

Publication ,  Journal Article
Mikati, MA; Trevathan, E; Krishnamoorthy, KS; Lombroso, CT
Published in: Electroencephalogr Clin Neurophysiol
March 1991

The EEG features and clinical correlates were investigated before, directly after, and on long-term follow-up after initiation of pyridoxine therapy in 6 patients with B6-dependent epilepsy. At each phase, the EEG provided important diagnostic and prognostic information. Pre-B6 3 neonates manifested a unique EEG pattern of generalized bursts of 1-4 Hz sharp and slow activity. This pattern has not been previously described in neonates with B6 dependency and in this age group appears to be highly suggestive of the diagnosis. Five patients experienced an apparent initial response to traditional antiepileptics. The parenteral pyridoxine test, performed in all 5, and repeated in 3, proved to be a highly reliable and reproducible diagnostic test. After 50-100 mg of B6 there was cessation of clinical seizures within minutes and of paroxysmal discharges within hours. On long-term follow-up (3-28 years) all 6 patients were seizure free on B6 (10-100 mg/day) monotherapy. Recurrences of seizures and of specific sequential EEG changes (background slowing, photoparoxysmal response, spontaneous discharges, stimulus-induced myoclonus, generalized seizures) occurred upon B6 withdrawal. Long-term prognosis correlated with the EEG. Two patients had persistently abnormal EEG backgrounds and were moderately to severely retarded, while 4 had normal EEGs with normal or near normal development.

Duke Scholars

Published In

Electroencephalogr Clin Neurophysiol

DOI

ISSN

0013-4694

Publication Date

March 1991

Volume

78

Issue

3

Start / End Page

215 / 221

Location

Ireland

Related Subject Headings

  • Pyridoxine
  • Infant, Newborn
  • Infant
  • Humans
  • Follow-Up Studies
  • Epilepsy
  • Electroencephalography
  • Brain
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Mikati, M. A., Trevathan, E., Krishnamoorthy, K. S., & Lombroso, C. T. (1991). Pyridoxine-dependent epilepsy: EEG investigations and long-term follow-up. Electroencephalogr Clin Neurophysiol, 78(3), 215–221. https://doi.org/10.1016/0013-4694(91)90035-3
Mikati, M. A., E. Trevathan, K. S. Krishnamoorthy, and C. T. Lombroso. “Pyridoxine-dependent epilepsy: EEG investigations and long-term follow-up.Electroencephalogr Clin Neurophysiol 78, no. 3 (March 1991): 215–21. https://doi.org/10.1016/0013-4694(91)90035-3.
Mikati MA, Trevathan E, Krishnamoorthy KS, Lombroso CT. Pyridoxine-dependent epilepsy: EEG investigations and long-term follow-up. Electroencephalogr Clin Neurophysiol. 1991 Mar;78(3):215–21.
Mikati, M. A., et al. “Pyridoxine-dependent epilepsy: EEG investigations and long-term follow-up.Electroencephalogr Clin Neurophysiol, vol. 78, no. 3, Mar. 1991, pp. 215–21. Pubmed, doi:10.1016/0013-4694(91)90035-3.
Mikati MA, Trevathan E, Krishnamoorthy KS, Lombroso CT. Pyridoxine-dependent epilepsy: EEG investigations and long-term follow-up. Electroencephalogr Clin Neurophysiol. 1991 Mar;78(3):215–221.

Published In

Electroencephalogr Clin Neurophysiol

DOI

ISSN

0013-4694

Publication Date

March 1991

Volume

78

Issue

3

Start / End Page

215 / 221

Location

Ireland

Related Subject Headings

  • Pyridoxine
  • Infant, Newborn
  • Infant
  • Humans
  • Follow-Up Studies
  • Epilepsy
  • Electroencephalography
  • Brain