1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-4-16-a 10.15844/pedneurbriefs-4-2-10 Degenerative Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Oral-Motor Function in Rett Syndrome http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 02 1990 01 08 2016 4 2 16 16 Copyright: © 1990 The Author(s) 1990 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Communication and oral-motor function in Rett syndrome

The communication skills, oral-motor function and respiration patterns of 20 girls with Rett syndrome were studied at the Crippled Childrens Division, Oregon Health Sciences University, Portland, OR.

 Oral-Motor Function Rett Syndrome Midtemporal Regions

The communication skills, oral-motor function and respiration patterns of 20 girls with Rett syndrome were studied at the Crippled Childrens Division, Oregon Health Sciences University, Portland, OR. All patients showed a regression in speech and language function by the onset of Stage II Rett syndrome. Oral-motor tone of the cheeks, lips and tongue changed from hypotonicity in Stages I and III to hypertonicity in Stage IV in a direct relationship with postural tone. Three girls in Stage IV showed fasciculations of the tongue. Most girls in Stages III and IV showed tongue deviation to the left at rest. [1]

COMMENT. This study documents the differences in oral-motor function in the various stages of Rett syndrome. The same author has described abnormal chewing associated with tongue thrusting and involuntary undulating tongue movements in 11 of 13 girls with Rett syndrome [2]. Feeding problems included difficulty in chewing and swallowing, choking and regurgitation.

An EEG study in 52 girls with Rett syndrome [3] showed that seizure discharges occurred in 43 patients and were not related to the onset of clinical seizures. They consisted of sharp waves or spikes, focal or multifocal, mainly limited to the central and midtemporal regions of one or both hemispheres. Light sleep enhanced the presence of discharges. Periodical hyperventilation seen frequently during EEG recordings in this study was not associated with any consistent EEG change. Sleep spindles were conspicuously absent in all records. The authors find the EEG features helpful in confirmation of the diagnosis of Rett syndrome in the appropriate clinical setting.

 Budden S Meek M Henighan C Communication and oral-motor function in Rett syndrome Dev Med Child Neurlo 1990 Jan 32 1 51 55 10.1111/j.1469-8749.1990.tb08466.x 2298336 Budden SS Rett syndrome: studies of 13 affected girls Am J Med Genet 1986 24 99 10.1002/ajmg.1320250511 3087208 Robb SA Harden A Boyd SG Rett syndrome: An EEG study in 52 girls Neuropediatrics 1989 Nov 20 4 192 195 2608163