title: Brain perfusion SPECT and EEG findings in Rett syndrome.
abstract: Thirteen patients (mean age 8.4 + 5.3 years) with Rett syndrome (RS) were studied with EEG and 99mTc-HMPAO SPECT. Eleven patients had background abnormalities and 10 patients paroxysmal activity in EEG. Hypoperfusion of varying severity was detected in 11 patients, 7 patients having multiple lesions. Bifrontal hypoperfusion, observed in 6 patients, was the most distinctive finding. Hypoperfusion was observed also in other cortical regions, except for the occipital lobes. There was no correlation between severity of the background abnormality or presence of paroxysmal activity in EEG and grade of hypoperfusion. There was, however, an association between the severity of hypoperfusion and early manifestation of symptoms in patients with RS. Whether this early-onset group of patients represents a different disease entity or only reflects disease variability the basic pathology being the same, is a possibility that deserves further clarification.
authors.affiliation: Department of Child Neurology, Children's Castle Hospital, Helsinki, Finland.
authors.forename: R
authors.initials: R
authors.lastname: Lappalainen
authors.forename: K
authors.initials: K
authors.lastname: Liewendahl
authors.forename: K
authors.initials: K
authors.lastname: Sainio
authors.forename: P
authors.initials: P
authors.lastname: Nikkinen
authors.forename: R S
authors.initials: RS
authors.lastname: Riikonen
mesh_terms: D000293:Adolescent; D001921:Brain / Q000098:blood supply* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D002560:Cerebrovascular Circulation; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006801:Humans; D015609:Organotechnetium Compounds; D010091:Oximes; D015518:Rett Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D012720:Severity of Illness Index; D019690:Technetium Tc 99m Exametazime; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D015609:Organotechnetium Compounds; D010091:Oximes; D019690:Technetium Tc 99m Exametazime