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Scleroza laterala primitiva este o afectiune foarte rar intalnita, este cauzata de o pierdere a celulelor piramidale mari si afecteaza in general adutii cu varste cuprinse intre 30 si 50 de ani.
Aceasta boala evolueaza cu paralizia spastica a membrelor, putand inainta lent sau agresiv ca si scleroza laterala amiotrofica.