Brain perfusion SPECT abnormalities in neuronal ceroid lipofuscinoses

• Published in: Neuropediatrics Vol. 28; no. 1; p. 71
• Main Authors: Liewendahl, K, Vanhanen, S L, Heiskala, H, Raininko, R, Nikkinen, P, Launes, J, Santavuori, P
• Format: Journal Article
• Language: English
• Published: Germany 01.02.1997
• Subjects: Adolescent; Adult; Brain - blood supply; Brain - diagnostic imaging; Child; Child, Preschool; Female; Humans; Image Processing, Computer-Assisted; Infant; Male; Neuronal Ceroid-Lipofuscinoses - diagnostic imaging; Neuronal Ceroid-Lipofuscinoses - physiopathology; Organotechnetium Compounds; Oximes; Regional Blood Flow - physiology; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon
• ISSN: 0174-304X
• DOI: 10.1055/s-2007-973674

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.