Document Detail

Brain perfusion SPECT abnormalities in neuronal ceroid lipofuscinoses.
MedLine Citation:
PMID:  9151329     Owner:  NLM     Status:  MEDLINE    
Brain perfusion was studied with the Tc-99m-HMPAO SPECT method in 19 INCL patients, 21 JNCL patients and 5 patients with Jansky-Bielschowsky variant disease (JBVD). The typical SPECT findings at an early stage of INCL were bilateral anterior frontal, posterior temporoparietal and occipital hypoperfusion, whereas reduction in cerebellar perfusion appeared later. However, perfusion of basal ganglia and thalami, although atrophic on MRI, was usually well preserved up to the terminal stage. All JNCL patients except one had at least one hypoperfused area. Mild hypoperfusion was usually located in the parietal and occipital lobes and cerebellum, whereas more severe hypoperfusion was observed in the temporal lobes. In JNCL, SPECT revealed lesions not detected on CT. All JBVD patients had supra- and infratentorial hypoperfusion, which was usually bilateral. This study shows that although in NCLs brain hypoperfusion can appear prior to structural abnormalities seen on MRI or CT, such abnormalities are not always associated with significant hypoperfusion.
K Liewendahl; S L Vanhanen; H Heiskala; R Raininko; P Nikkinen; J Launes; P Santavuori
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Neuropediatrics     Volume:  28     ISSN:  0174-304X     ISO Abbreviation:  Neuropediatrics     Publication Date:  1997 Feb 
Date Detail:
Created Date:  1997-08-20     Completed Date:  1997-08-20     Revised Date:  2008-01-16    
Medline Journal Info:
Nlm Unique ID:  8101187     Medline TA:  Neuropediatrics     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  71-3     Citation Subset:  IM    
Laboratory Department, University of Helsinki, Finland.
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MeSH Terms
Brain / blood supply*,  radionuclide imaging
Child, Preschool
Image Processing, Computer-Assisted
Neuronal Ceroid-Lipofuscinoses / physiopathology,  radionuclide imaging*
Organotechnetium Compounds / diagnostic use
Oximes / diagnostic use
Regional Blood Flow / physiology
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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