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Rev."],"published-print":{"date-parts":[[2002,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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\u2010central 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\u2010EEG 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 \u201chigher\u201d cortical pathways. Somatosensory\u2010evoked potentials may be characterized by \u201cgiant\u201d responses, suggesting cortical hyperexcitability. An increased incidence of long QT intervals during electrocardiographic recordings and diminished heart\u2010rate 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\u2010genotype correlations and characterization of animal models. MRDD Research Reviews 2002;8:66\u201371. \u00a9 2002 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/mrdd.10024","type":"journal-article","created":{"date-parts":[[2002,10,6]],"date-time":"2002-10-06T12:27:22Z","timestamp":1033907242000},"page":"66-71","source":"Crossref","is-referenced-by-count":44,"title":["Neurophysiology of Rett syndrome"],"prefix":"10.1002","volume":"8","author":[{"given":"Daniel G.","family":"Glaze","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2002,5,30]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"40","article-title":"Epilepsy in Rett syndrome\u2014a brief overview","volume":"6","author":"Aicardi J","year":"1997","journal-title":"Eur Child Adolesc Psychiatry"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0013-4694(90)90081-T"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199503000-00006"},{"key":"e_1_2_1_5_1","first-page":"72","volume-title":"Rett syndrome\u2014clinical and biological aspects","author":"Bader G","year":"1993"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0387-7604(87)80076-1"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0387-7604(89)80077-4"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0387-7604(89)80078-6"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1090-3798(98)80028-7"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1994.tb02933.x"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.1991.tb04660.x"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.80.5.470"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.53.10.874"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199907000-00006"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(95)70490-6"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/0887-8994(88)90081-1"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410210410"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1987.00520220051016"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0013-4694(97)00084-9"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410430410"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-973480"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410140412"},{"key":"e_1_2_1_23_1","first-page":"47","volume-title":"Rett syndrome: a suggested staging system for describing impairment profile with increasing age towards adolescence","author":"Hagberg B","year":"1986"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/0013-4694(89)90025-4"},{"key":"e_1_2_1_25_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-973794"},{"key":"e_1_2_1_26_1","unstructured":"HoHH WongPKH RobertsonR.1988.Rett syndrome: clinical profile and EEG abnormalities. 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