Abstract
Background
Rett syndrome is a severe neurodevelopmental disorder that primarily affects females.
Classically the disorder is characterized by early normal development, followed by
a period of regression and later recovery or stagnation. Typical features include
a loss of purposeful hand skills, development of hand stereotypies, loss of spoken
language, gait abnormalities, and acquired microcephaly. Epilepsy affects between
70% and 90% of individuals with Rett syndrome. A number of stereotypical electroencephalography
findings have been reported in Rett syndrome.
Patient description
We report a 9-year-old girl with Rett syndrome and epilepsy with a unique electroencephalography
finding consisting of intermittent paroxysms of alpha activity in both wakefulness
and sleep without clinical signs.
Results
This unique electroencephalography signature has not previously been reported in the
English literature.
Conclusions
Knowledge of this unique electroencephalography pattern of diffuse paroxysmal alpha
activity represents an additional distinct feature of the electroencephalogram in
Rett syndrome and expands the spectrum of electroencephalography abnormalities in
Rett syndrome.
Keywords
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Article info
Publication history
Published online: April 21, 2014
Accepted:
April 15,
2014
Received:
March 19,
2014
Identification
Copyright
© 2014 Published by Elsevier Inc. All rights reserved.
