Non-compliance in pyridoxine-dependent seizures and a way out

Authors: Kumar, Praveen¹; Gupta, Vijay K; Mishra, Devendra²

Source: Journal of Paediatrics and Child Health, Volume 43, Numbers 7-8, July/August 2007 , pp. 571-572(2)

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Abstract:

Pyridoxine dependency is a rare autosomal-recessive disorder causing intractable seizures in neonates and infants. Life-long therapy with pyridoxine is required for prevention of seizure recurrence and for an optimum intellectual outcome. With the availability of newer biochemical confirmatory tests, the conventional pyridoxine-withdrawal test is being used less frequently for diagnosis. This report describes an infant whose parents were non-compliant with pyridoxine therapy and proposes that pyridoxine-withdrawal test may be useful in demonstrating to parents the need for life-long pyridoxine therapy, thereby reducing non-compliance.

Keywords: counselling; neonatal seizures; pyridoxine-withdrawal; status epilepticus

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1440-1754.2007.01142.x

Affiliations: 1: Department of Pediatrics, Dr Ram Manohar Lohia Hospital, New Delhi and 2: Division of Pediatric Neurology, Department of Pediatrics, Chacha Nehru Bal Chikitsalaya (Maulana Azad Medical College), Geeta Colony, New Delhi, India

Publication date: 2007-07-01