Correlation of electroencephalogram, respiration and movement in the Rett syndrome

Kerr, Alison; Southall, D P; Amos, Patricia; Cooper, Rosemary; Samuels, Martin; Mitchell, Jane; Stephenson, John B.

doi:10.1016/s0387-7604(12)80179-3

Cited by 36 publications

(24 citation statements)

References 12 publications

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“…In our experience, injury to other people is most frequently complained of in the mobile individual who may wish for friendly contact or may express desire, distress, frustration or anger in this way, yet little difference is demonstrated here between the study and control groups. It is a universal observation that when the individual with Rett becomes agitated the hand stereotypy worsens (Kerr et al. 1987, 1990).…”

Section: Discussionmentioning

confidence: 99%

People withMECP2mutation‐positive Rett disorder who converse

Kerr

Archer

Evans

et al. 2005

J intellect Disabil Res

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Section: Discussionmentioning

confidence: 99%

People withMECP2mutation‐positive Rett disorder who converse

Kerr

Archer

Evans

et al. 2005

J intellect Disabil Res

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“…During sleep, this breathing pattern is absent [Glaze et al, 1987a;Kerr et al, 1990;Southall et al, 1988]. During periods of regular awake breathing the EEG may show nonepileptic paroxysms of slow activity (1.5-4 Hz), while slowing of the EEG during hyperventilation typically recorded in healthy children voluntarily hyperventilating is not recorded in RS girls .…”

Section: Stage IVmentioning

confidence: 99%

Neurophysiology of Rett syndrome

Glaze

2002

Ment. Retard. Dev. Disabil. Res. Rev.

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Neurophysiological evaluations have been widely applied in the study of Rett syndrome (RS) to provide information concerning the developmental aspects of RS; the character and extent of involvement of the central, peripheral, and autonomic nervous system pathways; and evaluation of the clinical symptomatology of RS. The electroencephalogram (EEG) is invariably abnormal and shows characteristic, though not diagnostic, changes: loss of expected developmental features; the appearance of focal, multifocal, and generalized epileptiform abnormalities; and the occurrence of rhythmic slow (theta) activity primarily in the frontal-central regions. Epileptic seizures are reported to occur frequently in RS, and partial and generalized seizures may be experienced by RS girls. However, many events presumed to be seizures have no EEG correlate during video-EEG monitoring, suggesting the possibility of a nonepileptic mechanism. Such monitoring may be necessary to determine appropriate use of antiepileptic drugs. Evoked potentials typically demonstrate intact peripheral auditory and visual pathways and suggest dysfunction of central or "higher" cortical pathways. Somatosensory-evoked potentials may be characterized by "giant" responses, suggesting cortical hyperexcitability. An increased incidence of long QT intervals during electrocardiographic recordings and diminished heart-rate variability, suggesting impairment of the autonomic nervous system, are described in RS. With the discovery of the genetic basis of RS, neurophysiological studies will provide parameters for phenotype-genotype correlations and characterization of animal models.

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“…The chief practical difficulty arises in distinguishing clinically between epileptic seizures and the more frequent non‐epileptic vacant spells associated with poor autonomic regulation (Glaze et al., 1998; Julu et al., 2001; Kerr et al., 1990). Both may be present in the same person and it may be impossible to make a correct diagnosis without monitoring the electroencephalogram, respiratory rhythm, blood gases and cardiac indices together with video recordings (Julu et al., 2001).…”

Section: Investigating the Symptomatology In Rett Syndromementioning

confidence: 99%