[Nonpharmacological, ubiquitous, rapid, fused graphic elements in the electroencephalographic pattern of 2 sisters with type A sulfatidosis and ponto-cerebellar systemic atrophy].
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.
D000293:Adolescent; D000328:Adult; D001284:Atrophy; D002531:Cerebellum / Q000473:pathology; D002540:Cerebral Cortex / Q000503:physiopathology*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D011149:Pons / Q000473:pathology
Grafoelementi rapidi fusati ubiquitari non farmacologici nel tracciato elettroencefalografico di due sorelle con sulfatidosi di tipo A ed atrofia sistemica ponto-cerebellare