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Pyridoxine-Dependent Seizures: Report of a Case With Atypical Clinical Features and Abnormal MRI Scans

Department of Pediatrics, Wakayama Red Cross Hospital, Wakayama City, Wakayama Japan

Mitsuyoshi Okumura, MD

Department of Pediatrics, Wakayama Red Cross Hospital, Wakayama City, Wakayama Japan

Jun Arima, MD

Department of Pediatrics, Wakayama Red Cross Hospital, Wakayama City, Wakayama Japan

Shinji Yamakura, MD

Department of Pediatrics, Wakayama Red Cross Hospital, Wakayama City, Wakayama Japan

Toru Momoi, MD

Department of Pediatrics, Wakayama Red Cross Hospital, Wakayama City, Wakayama Japan

A Japanese girl with atypical pyridoxine-dependent seizures is reported. Until 9 months of age the seizures had been controlled by conventional anticonvulsants. The initial administration of pyridoxine was followed by a collapse; the suppression-burst pattern changed to an almost flat pattern in the EEG. T₁- and T₂-weighted magnetic resonance imaging (MRI) scans showed poor differentiation between white and gray matter, and T₂-weighted MRI scans showed periventricular hyperintensity areas adjacent to the posterior horns of lateral ventricles. The findings in this patient indicate that pyridoxine should be given to infants with intractable epilepsy, regardless of the response to anticonvulsants, and that resuscitation facilities should be available during such a trial. (J Child Neurol 1992;7:24-28).

Journal of Child Neurology, Vol. 7, No. 1, 24-28 (1992)
DOI: 10.1177/088307389200700104


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