BRAIN PERFUSION SPECT IN JUVENILE NEURONAL CEROID-LIPOFUSCINOSIS

The juvenile neuronal ceroid-lipofuscinosis (JNCL) is a recessively in herited progressive encephalopathy. We studied 21 JNCL patients with a duration of illness of 1 to 17 years by Tc-99m-HM-PAO single photon e mission computed tomography (SPECT) and correlated the findings with c linical parameters. All patients had at least one hypoperfused brain a rea, the median number of such areas was 5 per patient. Parietally, oc cipitally, and in the cerebellar lobes hypoperfusion was usually mild whereas it was temporally more severe. Right parietal hypoperfusion co rrelated to neurological dysfunction.