Abstract
Study Objective
To describe features of menstruation, menstrual-related symptoms, and menstrual management
in females with Rett syndrome (RTT) to help develop a clinical approach to these parameters
in RTT.
Design
Retrospective cross-sectional chart review and prospective survey.
Setting
Cincinnati Children's Hospital Medical Center, Rett Syndrome and Related Spectrum
Disorders Clinic.
Participants
Females with RTT (12-55 years of age) and their caregivers.
Main Outcome Measures
Descriptive data on features of menstruation and menstrual-related symptoms in individuals
with Rett syndrome; prevalence, types, reason for use/discontinuation, and efficacy
of hormonal treatment in females with RTT.
Results
Age at menarche, menstrual cycle length, and menstrual period length in females with
RTT are comparable to those in typically developing females and females with other
neurodevelopmental disabilities. Dysmenorrhea and emotional lability are common menstrual
cycle−related changes among females with RTT; 22.1% of participants also reported
catamenial seizures. Oral progestin, combined oral contraceptive pill, and depot-medroxyprogesterone
acetate (DMPA) were effectively used to suppress or regulate menstruation and to manage
menstrual-related symptoms.
Conclusions
Characteristics of menstruation in females with RTT are comparable to those of typically
developing females, with the exception of increase in catamenial seizure activity.
Hormonal treatments are used for management of menstruation, dysmenorrhea, and seizures.
Choice of hormonal treatment is influenced by bone health and immobility in females
with RTT.
Key Words
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Article info
Publication history
Published online: November 10, 2020
Footnotes
Shannon M. Standridge is a consultant to Acadia and Zogenix and is on the Speakers Bureau for Greenwich Biosciences. With this exception, the authors have no additional disclosures or conflicts of interest to report.
These findings were presented as a poster presentation at the Child Neurology Society Annual Meeting; October 23-26, 2019, Charlotte, NC.
Identification
Copyright
© 2021 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc.
