Development of a disability scale for XXXX type 1.

@entity1 @entity4094 @entity66 @entity6045 @entity73 @entity2683 @entity8525 @entity3918
OBJECTIVES: @entity3918 type 1 ( @entity6045 ) is a @entity66 . Many tests in the literature have evaluated single aspects of @entity6045 @entity1 , mainly focusing on @entity73 , without an overall quantification of the different disease-specific neurological features. We developed and validated a new functional scale for @entity6045 @entity1 based on @entity4094 ( @entity4094 ) and disability. MATERIALS AND METHODS: Thirty-three @entity1 were tested in basal condition, 18 were re-evaluated after therapeutic intervention with @entity2683 , and 13 at one year follow-up without treatment. The scale includes 21 ordinal items in four areas: neuropsychology, motricity, @entity8525 and daily life activities. We evaluated inter- and intra-observer reliability (intraclass correlation coefficient, ICC and Spearman correlations, respectively), internal consistency (Cronbach's alpha), external validity (Spearman correlations between each area and other clinical and objective measurements and scales), and sensitivity to clinical changes after treatment or at follow-up. RESULTS: Our analysis provided good results for inter-observer agreement (ICC = 0.72-0.97), intra-observer reliability, and internal consistency for all areas (Cronbach's a > 0.73). Total score and single area subscores were significantly correlated to objective measurements, disease duration and multisystem involvement. Finally, the scale was sensitive to clinical changes disclosing a significant improvement after treatment in the items assessing @entity8525 , and also to disease progression showing a significant worsening in all areas but @entity8525 in untreated @entity1 . DISCUSSION: Our scale provides a new practical measure to evaluate @entity4094 and disability of @entity6045 @entity1 . Further longitudinal studies are warranted to confirm its reliability in tracking disease progression and severity over a longer period of time.
