NEURONAL CEROIDLIPOFUSCINOSIS: CCT FINDINGS IN FOURTEEN PATIENTS

Abstract

CTT [cranial computer tomography] was performed in 14 children/adolescents with juvenile ceroid lipofuscinosis (JNCL) at different stages of the disease. CCT was normal in 7 patients, mildly abnormal in 3, clearly abnormal in another 3 and severely abnormal in 1 patient. Characteristic findings were symmetrical enlargement of subarachnoid spaces and of the lateral and 3rd ventricles. White matter showed no changes in density. In later stages the 4th ventricle was enlarged and cerebellar atrophy was seen. CCT findings were not closely correlated with onset and/or severity of the neurological abnormalities. CCT findings were positively correlated with age of the patients: CCT was usually normal if patients were < 10-yr-old, and usually abnormal if patients were > 14-yr-old.