Two sisters, affected by late infantile type Sulfatidosis A, died at 22 and 15 years old respectively, presented, after autoptic examination, also a ponto-cerebellar athrophy. The EEG finding was characterized by few bioelectric abnormalities in the earlier studies of the disease and, afterwards, a progressive slower electric activities, with isolated asymmetric spikes. The typical aspects in the EEG recordings of our two patients were the presence of many rapid and diffuse elements, shay wawes without any pharmacological treatment. The significance of these peculiar recordings is there discussed.
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