Unusual EEG theta rhythms over central region in Rett Syndrome: Considerations of the underlying dysfunction

Ernst Niedermeyer, Sakkubai Naidu, Cynthia Plate

Research output: Contribution to journalArticle

Abstract

In 10 female patients (age 2-26 years) with clinical evidence of Rett Syndrome (RS), unusual and prominent rhythmical theta activity (45/sec or 5- 6/sec) proved to be the outstanding EEG feature. This pattern was present in waking state and/or sleep. When it was noted in the waking state, the localization (vertex, central region and vicinity) and blocking responses to active or passive movements suggested a slow equivalent of Rolandic mu rhythm (in two patients associated with a posterior 10-12/sec alpha rhythm). In sleep, rhythmical theta activity was either Rolandic or more diffuse, sometimes independently occurring with central spikes. The prominent rhythmical 4-5/sec or 5-6/sec activity and its relationship to Rolandic mu rhythm suggest a dysfunction of the motor cortex in RS. This would be congruent with the frequent observation of central spikes. EEG evidence of motor cortex dysfunction might be helpful in the understanding of this enigmatic disorder and conducive to the following hypothesis: RS is characterized by motor cortical dyscontrol due to primary frontal lobe dysfunction. The usefulness of EEG, however, is limited to insights into the (secondary) motor-cortex dysfunction.

Original languageEnglish (US)
Pages (from-to)36-43
Number of pages8
JournalClinical EEG Electroencephalography
Volume28
Issue number1
StatePublished - 1997

Fingerprint

Theta Rhythm
Rett Syndrome
Motor Cortex
Electroencephalography
Sleep
Alpha Rhythm
Frontal Lobe
Observation

Keywords

  • Electroencephalography
  • Motor Dyscontrol
  • Mu Rhythm
  • Rett Syndrome
  • Rolandic Spikes
  • Sensorimotor Region
  • Theta Rhythm, Atypical

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Unusual EEG theta rhythms over central region in Rett Syndrome : Considerations of the underlying dysfunction. / Niedermeyer, Ernst; Naidu, Sakkubai; Plate, Cynthia.

In: Clinical EEG Electroencephalography, Vol. 28, No. 1, 1997, p. 36-43.

Research output: Contribution to journalArticle

@article{de3a2539910e4e148dda38c019ec7cdf,
title = "Unusual EEG theta rhythms over central region in Rett Syndrome: Considerations of the underlying dysfunction",
abstract = "In 10 female patients (age 2-26 years) with clinical evidence of Rett Syndrome (RS), unusual and prominent rhythmical theta activity (45/sec or 5- 6/sec) proved to be the outstanding EEG feature. This pattern was present in waking state and/or sleep. When it was noted in the waking state, the localization (vertex, central region and vicinity) and blocking responses to active or passive movements suggested a slow equivalent of Rolandic mu rhythm (in two patients associated with a posterior 10-12/sec alpha rhythm). In sleep, rhythmical theta activity was either Rolandic or more diffuse, sometimes independently occurring with central spikes. The prominent rhythmical 4-5/sec or 5-6/sec activity and its relationship to Rolandic mu rhythm suggest a dysfunction of the motor cortex in RS. This would be congruent with the frequent observation of central spikes. EEG evidence of motor cortex dysfunction might be helpful in the understanding of this enigmatic disorder and conducive to the following hypothesis: RS is characterized by motor cortical dyscontrol due to primary frontal lobe dysfunction. The usefulness of EEG, however, is limited to insights into the (secondary) motor-cortex dysfunction.",
keywords = "Electroencephalography, Motor Dyscontrol, Mu Rhythm, Rett Syndrome, Rolandic Spikes, Sensorimotor Region, Theta Rhythm, Atypical",
author = "Ernst Niedermeyer and Sakkubai Naidu and Cynthia Plate",
year = "1997",
language = "English (US)",
volume = "28",
pages = "36--43",
journal = "Clinical EEG and Neuroscience",
issn = "0009-9155",
publisher = "EEG and Clinical Neuroscience Society (ECNS)",
number = "1",

}

TY - JOUR

T1 - Unusual EEG theta rhythms over central region in Rett Syndrome

T2 - Considerations of the underlying dysfunction

AU - Niedermeyer, Ernst

AU - Naidu, Sakkubai

AU - Plate, Cynthia

PY - 1997

Y1 - 1997

N2 - In 10 female patients (age 2-26 years) with clinical evidence of Rett Syndrome (RS), unusual and prominent rhythmical theta activity (45/sec or 5- 6/sec) proved to be the outstanding EEG feature. This pattern was present in waking state and/or sleep. When it was noted in the waking state, the localization (vertex, central region and vicinity) and blocking responses to active or passive movements suggested a slow equivalent of Rolandic mu rhythm (in two patients associated with a posterior 10-12/sec alpha rhythm). In sleep, rhythmical theta activity was either Rolandic or more diffuse, sometimes independently occurring with central spikes. The prominent rhythmical 4-5/sec or 5-6/sec activity and its relationship to Rolandic mu rhythm suggest a dysfunction of the motor cortex in RS. This would be congruent with the frequent observation of central spikes. EEG evidence of motor cortex dysfunction might be helpful in the understanding of this enigmatic disorder and conducive to the following hypothesis: RS is characterized by motor cortical dyscontrol due to primary frontal lobe dysfunction. The usefulness of EEG, however, is limited to insights into the (secondary) motor-cortex dysfunction.

AB - In 10 female patients (age 2-26 years) with clinical evidence of Rett Syndrome (RS), unusual and prominent rhythmical theta activity (45/sec or 5- 6/sec) proved to be the outstanding EEG feature. This pattern was present in waking state and/or sleep. When it was noted in the waking state, the localization (vertex, central region and vicinity) and blocking responses to active or passive movements suggested a slow equivalent of Rolandic mu rhythm (in two patients associated with a posterior 10-12/sec alpha rhythm). In sleep, rhythmical theta activity was either Rolandic or more diffuse, sometimes independently occurring with central spikes. The prominent rhythmical 4-5/sec or 5-6/sec activity and its relationship to Rolandic mu rhythm suggest a dysfunction of the motor cortex in RS. This would be congruent with the frequent observation of central spikes. EEG evidence of motor cortex dysfunction might be helpful in the understanding of this enigmatic disorder and conducive to the following hypothesis: RS is characterized by motor cortical dyscontrol due to primary frontal lobe dysfunction. The usefulness of EEG, however, is limited to insights into the (secondary) motor-cortex dysfunction.

KW - Electroencephalography

KW - Motor Dyscontrol

KW - Mu Rhythm

KW - Rett Syndrome

KW - Rolandic Spikes

KW - Sensorimotor Region

KW - Theta Rhythm, Atypical

UR - http://www.scopus.com/inward/record.url?scp=0031021545&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0031021545&partnerID=8YFLogxK

M3 - Article

C2 - 9013049

AN - SCOPUS:0031021545

VL - 28

SP - 36

EP - 43

JO - Clinical EEG and Neuroscience

JF - Clinical EEG and Neuroscience

SN - 0009-9155

IS - 1

ER -