NEURONAL CEROID-LIPOFUSCINOSES IN ITALY - AN EPIDEMIOLOGIC-STUDY

To establish the incidence of neuronal ceroid-lipofuscinoses (NCL) in Italy, we sent a questionnaire to all Neuropediatric and Child Neurops ychiatric Departments (answer rate 15/34 = 44%). Diagnoses were accept ed only when based on firm clinical and/or electron microscopic criter ia, We collected 58 eases born between 1966-1991 (2 infantile NCL, 37 late infantile NCL, and 19 juvenile NCL), The incidence was calculated only on patients born between 1974-1984. In this period, the incidenc e of overall NCL in the Italian population was calculated to be 0.56 p er 100,000 live births (0.36 for late infantile NCL, and 0.20 for juve nile NCL). Our data show that infantile NCL is very rare in Italy, and that late infantile seems to be the most frequent form of NCL. (C) 19 95 Wiley-Liss, Inc.