EEG AND EVOKED-POTENTIALS IN INFANTILE NEURONAL CEROID-LIPOFUSCINOSIS

Sixteen children with infantile neuronal ceroid-lipofuscinosis (INCL), age range 0.5 to 5.4 years, were studied using EEG, electroretinogram s (ERG), visual evoked potentials (VEP) and somatosensory evoked poten tials (SEP). Electroencephalography was the first of these examination s to reveal abnormalities, however the EEG may be normal at the precli nical stage, The first abnormality to appear was an attenuated reactio n to passive eye opening and closing which was followed by disturbance s in background activity and diminution in amplitude, and by disappear ance of sleep spindles, The gradual disappearance of posterior rhythm reactivity and of sleep spindles suggests that thalamic dysfunction pr ogresses with time, EEG inactivity appeared by the age of 3 years, Evo ked potentials were normal in the early stages of the disease, SEP sho wed abnormalities at Stage 2 (1.7 years), while ERG and VEP abnormalit ies appeared at Stage 3 (by the age of 2.5 years), All neurophysiologi cal reactions examined were abolished by the age of 4 years, Follow-up EEG gives important hints as to the early diagnosis of INCL. Progress ion of the disease can be followed by evoked potentials which may also be helpful in the differential diagnostics.