Rett syndrome and epilepsy: An update for child neurologists

Alison Dolce, Bruria Ben-Zeev, Sakkubai Naidu, Eric H. Kossoff

Research output: Contribution to journalArticle

45 Citations (Scopus)

Abstract

Rett syndrome, a neurogenetic disorder predominantly affecting females, has many characteristic features including psychomotor retardation, impaired language development, hand stereotypies, gait dysfunction, and acquired microcephaly. Although each of these features undoubtedly contributes to the morbidity of this neurologic disorder, epilepsy is perhaps one of the most well-described and problematic, affecting as many as 50%-90% of patients. Seizures can often be refractory, requiring polytherapy and consideration of nonpharmacologic management (e.g., ketogenic diets and vagus nerve stimulation). In addition, many nonepileptic symptoms of Rett syndrome can occasionally be difficult to differentiate from seizures making clinical management and family counseling challenging. Our goal in this review is to better define the clinical and electrophysiological aspects of the epilepsy associated with Rett syndrome and provide practical guidance regarding management.

Original languageEnglish (US)
Pages (from-to)337-345
Number of pages9
JournalPediatric Neurology
Volume48
Issue number5
DOIs
StatePublished - May 2013

Fingerprint

Rett Syndrome
Epilepsy
Seizures
Ketogenic Diet
Vagus Nerve Stimulation
Microcephaly
Language Development
Nervous System Diseases
Gait
Counseling
Hand
Morbidity
Neurologists

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

Rett syndrome and epilepsy : An update for child neurologists. / Dolce, Alison; Ben-Zeev, Bruria; Naidu, Sakkubai; Kossoff, Eric H.

In: Pediatric Neurology, Vol. 48, No. 5, 05.2013, p. 337-345.

Research output: Contribution to journalArticle

Dolce, Alison ; Ben-Zeev, Bruria ; Naidu, Sakkubai ; Kossoff, Eric H. / Rett syndrome and epilepsy : An update for child neurologists. In: Pediatric Neurology. 2013 ; Vol. 48, No. 5. pp. 337-345.
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