Abstract
60 cases of chronic spinal muscular atrophy (CSMA) were followed-up for a period varying from 5 to 40 years. The neuromuscular impairment was evaluated by Norris’ ALS score, both at the time of last examination and retrospectively at the time of diagnosis. Age at onset of symptoms was the most important factor in the progression of the neuromuscular damage. Monomelic or asymmetric location of symptoms at the time of diagnosis and duration of the disease were not significantly correlated to the worsening of ALS score.
Sommario
Sessanta casi di atrofia muscolare spinale cronica (CSMA) sono stati seguiti per un periodo compreso tra 5 e 40 anni. Per mezzo dell’ALS score di Norris è stato quantificato il danno neuromuscolare, sia al momento dell’ultima visita, sia, retrospettivamente, al momento della diagnosi. Il più importante fattore nella definizione della progressione del deficit è risultato essere l’età all’esordio dei sintomi. Non è stata evidenziata una relazione significativa tra una localizzazione monomelica o asimmetrica dei sintomi ed il peggioramento dell’ALS score.
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Schiffer, D., Brignolio, F., Chiò, A. et al. A follow-up study of 60 cases of chronic spinal muscular atrophy. Ital J Neuro Sci 9, 47–51 (1988). https://doi.org/10.1007/BF02334407
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DOI: https://doi.org/10.1007/BF02334407