Rett Syndrome

Topics: Epilepsy, Anticonvulsant, Reflex Pages: 3 (539 words) Published: June 29, 2010
stereotypic hand wringing and washing
movements. All her milestones virtually
regressed over one year after an episode of
bronchopneumonia. Environmental factors
like fever has been reported to trigger the onset
of RS in genetically predisposed subjects,
though there are hardly any reports in the
literature. Seizures are one of the supportive
criteria but these are overestimated. Nonseizure
events like twitching/jerking/head
pupillary dilatation/breath holding and
hyperventilation are usually confused with
seizures(2). Role of video EEG is very
important for diagnosis and to avoid the
overprescription and adverse effects of
antiepileptic drugs. Myoclonus although
reported has not been characterized. Children
with RS are prone to sudden death. Prolonged
QT interval/low heart rate/hyperventilation
and breath holding spells are important
components of this syndrome.
Treatment is symptomatic. For seizures
lamotrigine is better. Beside seizure control, it
improves concentration(3). Children with Rett
syndrome have low levels of L-carnitine and
vitamin E. These two agents have promising
role in the management of Rett syndrome(4).
We also prescribed vitamin E and carnitine for
four months but the child did not show any
improvement. Subsequently the child was put
on lamotrigine. After two months her social
interaction, visual contact and language
improved, but there was no change in her
stereotypic hand movements. After six month
of treatment she showed significant
improvement in her social behavior.
K .S. Rana,
M.N.G. Nair,
Department of Pediatrics,
Armed Forces Medical College,
Pune 411 040, India.
Rett’s Syndrome Following
Rett syndrome (RS), a neurodevelopment
disorder is almost exclusively seen in girls.
Diagnosis is essentially clinical and is based on...
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