Brain perfusion SPECT in juvenile neuronal ceroid lipofuscinosis.
The juvenile neuronal ceroid-lipofuscinosis (JNCL) is a recessively inherited progressive encephalopathy. We studied 21 JNCL patients with a duration of illness of 1 to 17 years by 99mTc-HM-PAO single photon emission computed tomography (SPECT) and correlated the findings with clinical parameters. All patients had at least one hypoperfused brain area, the median number of such areas was 5 per patient. Parietally, occipitally, and in the cerebellar lobes hypoperfusion was usually mild whereas it was temporally more severe. Right parietal hypoperfusion correlated to neurological dysfunction.
Launes
J
J
Department of Neurology, University Central Hospital, Helsinki, Finland.
Heiskala
H
H
Nikkinen
P
P
Santavuori
P
P
D000293:Adolescent; D001931:Brain Mapping; D002531:Cerebellum / Q000098:blood supply*; D002540:Cerebral Cortex / Q000098:blood supply*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D007223:Infant; D008297:Male; D009460:Neurologic Examination; D009472:Neuronal Ceroid-Lipofuscinoses / Q000000981:diagnostic imaging* / Q000235:genetics; D015609:Organotechnetium Compounds; D010091:Oximes; D011877:Radionuclide Imaging; D012039:Regional Blood Flow / Q000502:physiology; D019690:Technetium Tc 99m Exametazime
D015609:Organotechnetium Compounds; D010091:Oximes; D019690:Technetium Tc 99m Exametazime