Intended for healthcare professionals

Rapid response to:

Clinical Review

Fortnightly review: Epilepsy in childhood

BMJ 1997; 315 doi: https://doi.org/10.1136/bmj.315.7113.924 (Published 11 October 1997) Cite this as: BMJ 1997;315:924

Rapid Response:

Pyridoxine Dependent Seizures

As an internist and parent of two children with pyridoxine dependent
seizures, I was very pleased to see the inclusion of a trial of pyridoxine
for unexplained seizures in children under two years old. Our first child
was initially diagnosed as having idiopathic infantile spasms at six
months of age. It was not until eight years later when his sister
developed infantile spasms at the age of six months that the correct
diagnosis was made in both children. (I had to suggest the trial of
pyridoxine.) Our son had been seen at several major medical centers across
the United States but pyridoxine dependency was not considered because the
seizures were controlled by very high doses of Klonipin. (In retrospect,
his chewable vitamins and large bowls of cereal were probably helping as
much as anything.) Even though pyridoxine,100mg/day, completely stopped
the seizures in our daughter within three days, increasing the dose to 150
mg/day (10mg/kg/day) in two divided doses had an even more remarkable
effect, especially in improving her verbalization and alertness. She is
now almost three years old and doing very well. Our son has improved by
several grade levels in the last two years on a similar dosing schedule.

Both children started having seizures within a few weeks of my
stopping breast feeding. Since I had continued my prenatal vitamins and
large amounts of pyridoxine are secreted in breast milk, this probably had
a protective effect. Therefore, a history of severe seizures beginning
soon after breast feeding stops may be worth noting.

I have found about twenty families over the last several months who
have children with this disorder. Almost always, there have been
significant delays in getting to the correct diagnosis. Several families
had already lost a child before having the correct diagnosis made in a
sibling later.

Pyridoxine dependency is probably more common than previously thought
and significant improvement may be seen with appropriate treatment even if
the diagnosis is delayed.

Competing interests: No competing interests

15 December 1998
Christine W Parker
Medical Director
Memorial Health Services