https://scholars.lib.ntu.edu.tw/handle/123456789/434506
Title: | Vitamin B6 related epilepsy during childhood | Authors: | Wang H.-S. MENG-FAI KUO |
Keywords: | Epilepsy; Hyperprolinemia type II; Hypophosphatasia; Pyridoxine phosphate oxidase; Pyridoxine-dependent epilepsy; Vitamin B6 | Issue Date: | 2007 | Journal Volume: | 30 | Journal Issue: | 5 | Start page/Pages: | 396-401 | Source: | Chang Gung Medical Journal | Abstract: | In some patients without vitamin B6 deficiency, epilepsy can not be controlled without an extra supplement of vitamin B6. The therapeutic role of pyridoxal phosphate (PLP), the active form of vitamin B 6, may not be replaced with other forms of vitamin B6 sometimes. Until now, four inborn errors of metabolism are known to affect vitamin B6 concentrations in the brain. Three of them are hyperprolinemia type 2, antiquitin deficiency, and pyridoxine phosphate oxidase deficiency. The fourth disorder occurs in neonates with hypophosphatasia and congenital rickets. All patients with these conditions present with early-onset epilepsy that is resistant to conventional antiepileptic medications. Patients with three of the conditions respond to any form of vitamin B6. Only those with pyridoxine phosphate oxidase deficiency respond to PLP instead of pyridoxine. Interestingly, the authors have successfully treated many patients without the above four disorders using vitamin B6, and have found that the treatment was more effective with PLP than with pyridoxine, though the mechanism is not known. Since PLP is as inexpensive as pyridoxine, we suggest replacing PLP for pyridoxine when treating children with epilepsy. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-36549011402&partnerID=40&md5=81332bd9610d141216583e1c0bcc93af https://scholars.lib.ntu.edu.tw/handle/123456789/434506 |
ISSN: | 0255-8270 | SDG/Keyword: | anticonvulsive agent; pyridoxal 5 phosphate; pyridoxine; proline; pyridoxal 5 phosphate; pyridoxamine phosphate oxidase; pyridoxine; antiquitin deficiency; benign childhood epilepsy; brain level; cost effectiveness analysis; disease course; drug effect; drug efficacy; enzyme deficiency; human; hyperprolinemia; hypophosphatasia; inborn error of metabolism; optimal drug dose; pyridoxine deficiency; pyridoxine phosphate oxidase deficiency; review; rickets; blood; epilepsy; inborn error of metabolism; metabolism; pyridoxine deficiency; Epilepsy; Humans; Metabolism, Inborn Errors; Proline; Pyridoxal Phosphate; Pyridoxaminephosphate Oxidase; Pyridoxine; Vitamin B 6; Vitamin B 6 Deficiency |
Appears in Collections: | 醫學系 |
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