Format

Send to

Choose Destination
Arch Dis Child. 1999 Nov;81(5):431-3.

Epidemiology of pyridoxine dependent and pyridoxine responsive seizures in the UK.

Author information

1
Ryegate Centre, Sheffield Children's Hospital, Tapton Crescent, Sheffield S10 5DD, UK. p.s.baxter@sheffield.ac.uk

Abstract

OBJECTIVE:

To study the epidemiology of pyridoxine dependent seizures and other forms of pyridoxine responsive seizures.

DESIGN:

Monthly notifications to the British Paediatric Surveillance Unit over two years. Questionnaire follow up.

SETTING:

UK and the Republic of Ireland.

PATIENTS:

Children aged 15 years or younger whose seizures respond to pyridoxine.

INTERVENTIONS:

None.

MAIN OUTCOME MEASURES:

Numbers of children with definite, probable, and possible pyridoxine dependent seizures or other seizures responsive to pyridoxine.

RESULTS:

Point prevalence and birth incidence: 1/687 000 and 1/783 000, respectively (definite and probable cases); 1/317 000 and 1/157 000, respectively (all types of pyridoxine responsiveness). NOTIFICATIONS: Pyridoxine dependency: 14 definite, 9 probable, and 10 possible cases; neonatal seizures not meeting case definitions: 7; infantile spasms: 5. Eight of 18 families of definite/probable cases had 2 affected siblings. Just over a third had atypical presentations and just under a third had features and/or initial diagnoses of birth asphyxia and neonatal hypoxic ischaemic encephalopathy.

CONCLUSIONS:

Pyridoxine dependency is rare. Atypical presentations are relatively frequent. A trial of pyridoxine is justified in all cases of early onset intractable seizures or status epilepticus, whatever the suspected cause.

PMID:
10519720
PMCID:
PMC1718118
DOI:
10.1136/adc.81.5.431
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for HighWire Icon for PubMed Central
Loading ...
Support Center