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ALSFRS and appel ALS scores: Discordance with disease progression
Author(s) -
Voustianiouk Andrei,
Seidel Gregory,
Panchal Janki,
Sivak Mark,
Czaplinski Adam,
Yen Albert,
Appel Stanley H.,
Lange Dale J.
Publication year - 2008
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20977
Subject(s) - rating scale , amyotrophic lateral sclerosis , disease , medicine , physical therapy , psychology , developmental psychology
Progression of disease and effectiveness of therapy in patients with amyotrophic lateral sclerosis (ALS) are determined by both questionnaire‐ and examination‐based measures. To determine whether both types of measurement tools are equally predictive at all stages of disease, we compared questionnaire‐based ALS Functional Rating Scale (ALSFRS) scores to the examination‐based Appel ALS (AALS) scores at different stages of disease. Same‐day scores were obtained during 174 visits in 62 patients with definite or probable ALS. Using normalized scores, correlation between the scales and predictability were best in mildly affected patients. Predictions of ALSFRS based on AALS scores were less than half as precise in the later stages of disease. Both scales showed significant change with disease progression, but ALSFRS consistently underestimated disease severity defined by AALS ( P < 0.001). Questionnaire‐based measurements should be compared against objective scales at all stages of disease severity before they are accepted as primary endpoint measures. Muscle Nerve, 2008