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A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis ALS
Published on: February 21, 2011
Development of a Rasch-Built Amyotrophic Lateral Sclerosis Impairment Multidomain Scale to Measure Disease
Adriaan D de Jongh1, Ruben P A van Eijk1, Leonhard A Bakker1
1From the Department of Neurology (A.D.d.J., R.P.A.v.E., L.A.B., T.M.B., M.A.v.E., J.H.V., L.H.v.d.B.), UMC Utrecht Brain Center, University Medical Center Utrecht; Department of Neurology (A.D.d.J.), Tergooi Hospital, Blaricum; Biostatistics & Research Support (R.P.A.v.E.), Julius Center for Health Sciences and Primary Care, Department of Rehabilitation, Physical Therapy Science and Sports (L.A.B., A.B., J.M.A.V.-M., E.T.K.), UMC Utrecht Brain Center, and Center of Excellence for Rehabilitation Medicine (A.B., J.M.A.V.-M., E.T.K.), UMC Utrecht Brain Center, University Medical Center Utrecht, and De Hoogstraat Rehabilitation; and ALS Patients Connected (C.v.d.M.), Bilthoven, the Netherlands.
The new ALS Impairment Multidomain Scale (AIMS) offers a more accurate way to track amyotrophic lateral sclerosis progression. This multidomain scale improves treatment trial efficiency by better characterizing disease severity and prognosis.
Area of Science:
- Neurology
- Clinical Trials
- Biostatistics
Background:
- Current amyotrophic lateral sclerosis (ALS) scales may not accurately reflect individual disease severity or prognosis.
- Composite scores risk masking treatment efficacy if not all disease dimensions are equally affected.
Purpose of the Study:
- To develop the ALS Impairment Multidomain Scale (AIMS) for comprehensive disease characterization.
- To enhance the identification of effective ALS treatments.
Main Methods:
- A preliminary questionnaire was administered to 367 ALS patients over 12 months.
- Rasch analysis and signal-to-noise optimization were used to construct the multidomain scale.
- Reliability, longitudinal decline, and survival associations were evaluated.
Main Results:
- The AIMS comprises unidimensional bulbar, motor, and respiratory subscales, validated by Rasch analysis.
- Subscales demonstrated excellent test-retest reliability (0.91-0.94) and strong survival correlation (p < 0.001).
- AIMS use in clinical trials could reduce required sample sizes by 16.3% (6-month) and 25.9% (12-month) compared to ALSFRS-R.
Conclusions:
- The AIMS provides a more precise characterization of ALS disease severity than single scores.
- AIMS subscales are reliable, optimized for progression measurement, and strongly linked to survival.
- The AIMS facilitates easier administration and may improve the detection of effective ALS therapies.
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