Document Detail

Brain perfusion SPECT and EEG findings in Rett syndrome.
MedLine Citation:
PMID:  9048985     Owner:  NLM     Status:  MEDLINE    
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.
R Lappalainen; K Liewendahl; K Sainio; P Nikkinen; R S Riikonen
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta neurologica Scandinavica     Volume:  95     ISSN:  0001-6314     ISO Abbreviation:  Acta Neurol. Scand.     Publication Date:  1997 Jan 
Date Detail:
Created Date:  1997-05-13     Completed Date:  1997-05-13     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0370336     Medline TA:  Acta Neurol Scand     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  44-50     Citation Subset:  IM    
Department of Child Neurology, Children's Castle Hospital, Helsinki, Finland.
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MeSH Terms
Brain / blood supply*,  physiopathology,  radionuclide imaging*
Cerebrovascular Circulation
Child, Preschool
Epilepsy / diagnosis,  physiopathology
Organotechnetium Compounds / diagnostic use*
Oximes / diagnostic use*
Rett Syndrome / diagnosis*,  physiopathology,  radionuclide imaging*
Severity of Illness Index
Technetium Tc 99m Exametazime
Tomography, Emission-Computed, Single-Photon*
Reg. No./Substance:
0/Organotechnetium Compounds; 0/Oximes; 100504-35-6/Technetium Tc 99m Exametazime

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