Parental view of epilepsy in Angelman syndrome: a questionnaire study
Department of
Paediatrics, John Radcliffe Hospital, Oxford OX3 9DU, UK
Correspondence to: Dr McShane.
Accepted 17 June 1998
PURPOSE
To explore parents' opinions and concerns
about seizures, anticonvulsants, and the effect of treatment in
children with Angelman syndrome.
DESIGNA postal questionnaire was sent to
members of one of the UK lay groups for Angelman syndrome (ASSERT)
who had a child affected by Angelman syndrome. The questionnaire
requested general medical information and information about the
epilepsy, its treatment, and treatment responses.
RESULTSOne hundred and fifty questionnaires
were sent out with an ASSERT routine mailing and 78 completed
questionnaires were returned. Forty three patients were boys and 35 were girls; ages ranged from 1.7 to 25 years (mean 7.5 years). The
overall general clinical and cytogenetic data were mostly consistent
with previous reports. Epilepsy was reported in 68 children, most of
whom had a detectable cytogenetic deletion. The most common seizure
types reported by the families were absence seizures, tonic clonic
seizures, drop attacks, and myoclonic seizures; in four patients only
febrile seizures occurred. The age at onset of the seizures was < 2
years in more than half of the patients. Anti-epileptic drug treatment with valproate (VPA), clonazepam (CZP), and lamotrigine (LTG) as
monotherapy or a combination of VPA and CZP or VPA and LTG was more
often viewed favourably and considered effective with fewer side
effects on the child's behaviour and alertness, versus more frequent
adverse effects and increased frequency and severity of seizures with
carbamazepine (CBZ) and vigabatrin (VGB) in monotherapy or in
combination with other anti-epileptic drugs. Seizures did tend to
improve with age but were still present and disabling at older ages.
CONCLUSIONSThis is the first study to record
parents' opinions about seizures, anti-epileptic drugs, and treatment
responses in children with Angelman syndrome, and it is one of the
largest series on epilepsy and Angelman syndrome to be reported to date.
© 1998 by Archives of Disease in Childhood
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