Paroxysmal alpha activity in Rett syndrome: a case report.

作者信息

Whitney Robyn, Moharir Mahendranath, Allen Anita, Cortez Miguel A

机构信息

Division of Pediatric Neurology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.

Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

出版信息

Pediatr Neurol. 2014 Sep;51(3):421-5. doi: 10.1016/j.pediatrneurol.2014.04.018. Epub 2014 Apr 18.

DOI:10.1016/j.pediatrneurol.2014.04.018

PMID:25160549

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.

摘要