A vitamin a day keeps the doctor away: The need for high quality pyridoxal-5 '-phosphate

N. N. Stolwijk, M. M. Brands, L. S. Smit, V. van der Wel, C. E. M. Hollak, C. D. van Karnebeek

Research output: Contribution to journalArticleAcademicpeer-review

4 Citations (Scopus)
59 Downloads (Pure)

Abstract

Background
A rare subset of vitamin B6 responsive seizure disorders does not respond to pyridoxine, and requires the active form of vitamin B6, pyridoxal-5′-phosphate (PLP), to maintain seizure control. Patients with PLP-responsive seizures are dependent on chronic PLP treatment, yet no licensed PLP product is available. PLP food supplements, a product category regulated less stringently than medication, may prove of insufficient effectiveness and safety. Here we describe and discuss three patient scenarios which illustrate this conundrum.
Methods
Medical and laboratory records were reviewed with retrospective extraction for three unrelated patients who suffered complications during treatment with PLP food supplements.
Results
– Two cases of PNPO deficiency and one case of PLP-dependent epileptic encephalopathy without a (genetic) diagnosis are reported. These patients are critically dependent on PLP for seizure control and have suffered complications due to insufficient quality of these food supplements during the course of treatment. Complications include the occurrence of seizures following the administration of suspected low quality PLP, inactive PLP due to light exposure, a PLP intoxication, resisting administration and post-administration vomiting as a result of the ingestion of large amounts of capsules per day.
Conclusion
– This case series illustrates that the reliance on food supplements as anti-seizure therapy is not without risk. The treatment of PLP-dependent seizures exemplifies that PLP is administered as medication, thus there is a clear need for licensed vitamin products of pharmaceutical quality.
Original languageEnglish
Pages (from-to)25-29
Number of pages5
JournalEuropean Journal of Paediatric Neurology
Volume39
Early online date7 May 2022
DOIs
Publication statusPublished - Jul 2022

Bibliographical note

Funding:
CvK's research is supported by a Stichting Metakids grant.
NS, VW, and CH are members of the platform Medicine for Society, for which funding is provided by the Postcodeloterij.

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