Revised Upper Limb Module

COA At-a-Glance

Evidence of cognitive interviewing of draft instrument in target patient population

Evidence of internal consistency

Evidence of test-retest or inter-rater reliability

Evidence of concurrent validity

Evidence of known-groups validity

Evidence of ability to detect change over time

Evidence of responder thresholds

Inclusion of the COA in product labelling

Daily function
Fine motor function


Instrument Name: Revised Upper Limb Module

Abbreviation: RULM

Points for Consideration:

Can be difficult to score in contracted patients. Psychometrics have been evaluated.

Description of Tool:

The Revised Upper Limb Module is a ClinRO which includes 20 tasks. Tasks are rated as can/canNot complete or on a 3-point verbal response scale from "(0) unable," to "(2) Able, No difficulty". Higher scores represent better physical functioning.

Minimum Qualification Required by COA Administrator: PhD or MA

Year: 2017

Objective of Development:

To assess motor performance in the upper limbs for individuals with Spinal Muscular Atrophy (SMA)

Population of Development: Age range (therapeutic indication):

3-71 years (Spinal Muscular Atrophy)

Pediatric Population(s) in which COA has been used:

Nervous System Diseases

COA type: ClinRO

Number of Items 20

Mode of Administration: Interviewer-administered

Data Collection Mode: Paper and pen

Time for Completion: 5 to 20 minutes

Response Scales: Varies by item: Dichotomous: Can or Can Not 3-point verbal rating scale ranging from 0:

Summary of Scoring:

Available Scores:
Global score ranging from 0 to 37


Score Interpretation:
Higher score = Better physical functioning

Content Validity

Evidence of Literature Review: Yes

Evidence of Instrument Review: Yes

Evidence of Clinical or Expert Input: Yes

Evidence of concept elicitation in target patient population: None identified

Evidence of a Saturation Grid: None identified

Evidence for Selection of Data Collection Method: None identified

Recall/Observation Period: Present time

Evidence for Selection of Reponse Options: None identified

Evidence of cognitive interviewing of draft instrument in target patient population: None identified

Evidence of Preliminary Scoring of Items and Domains: Yes

Evidence related to respondent and administrator burden: None identified

Evidence of a Conceptual Framework: Yes

Evidence of an item-tracking matrix: None identified

Evidence related to item selection: Yes

Evidence of re-testing the final version: Yes


Internal consistency (Cronbach's alpha): None identified

Evidence of internal consistency:

Inter-rater/ inter-interviewer reliability (kappa):

Mazzone (2016) Intraclass Correlation Coefficient (ICC) The all 17 physical therapists: 0.928 UK: 0.933 US: 0.920 Italy: 0.947 - Population/Disease: Physical therapists; n= 3 (UK), 11 (US), 3 (Italy), age Not stated Glanzmann A (2017) - Intraclass Correlation Coefficient (ICC): Initial inter-rater reliability for all 3 assessments of total score and individual items was excellent (r = 0.867–0.994). Annual retraining reliability results were similar for the HFMSE (r=0.887–0.996) and RULM (r = 0.932–0.982) and slightly lower for CHOP INTEND (r = 0.817–0.889). - Population/Disease: Patients from multicenter clinical trials in spinal muscular atrophy (SMA), n=125

Evidence of test-retest or inter-rater reliability: Yes


Known-group validity:

Pera (2019)
KNown-groups validity:
- Measure/Groups of patients: Ambulant and Non-ambulant type 3 SMA patients (n= 32 and 22 respectively) and type 2 patients (n= 60) with different ages (<5 years, 5-9 years, 10-14 years and ≥ 15 years)
- A priori hypotheses: Not stated
- Were hypotheses confirmed: Not applicable
- Results: ANOVA, p<0.0001 for all results
The mean values were significantly different between type 2 patients (14.8±6.6), Non-ambulant type 3 patients (27.4±6.9) and ambulant type 3 patients (34.2±3.7), p<0.0001 for all results
A significant interaction between the 3 SMA groups and age was found (p= 0.011)
- Population/Disease: Patients with Ambulant and Non-ambulant type 2 and 3; n= 114, aged from 2.7 to 49.7, mean 13.3±10.1

AlfaNo LN (2020)
- Measure/Groups of patients: Correlation with the Ability Captured Through Interactive Video Evaluation
- A priori hypotheses: Not stated
- Were hypotheses confirmed: Not applicable
- Results: Spearman correlation coefficient
Significant correlation was found between the RULM and the ACTIVE (rho= 0.92; p<0.001)
- Population/Disease: Patients with SMA 2 (n= 27, mean age 11 years and 5 months) or with SMA 3 (n=10, mean age 12 years)

Evidence of Translatability Assessment: None identified

Evidence related to missing data: None identified

Evidence for Selection of Recall Period: None identified

Evidence of Administration Instructions and Training Provided: Yes

Evidence of concurrent validity: None identified

Evidence of known-groups validity: Yes

Ability to Detect Change

Ability to detect change (Responsiveness):

None identified

Responder Thresholds

Responder Thresholds:

Stolte B (2020)
- Population/Disease: Patients with SMA type 2 (n= 15, mean range age 31.4 (18-52) or with SMA type 3 (n= 36, mean age range age 37.6 (18-74 years) and classified according to their ambulatory status (44% of the SMA type 3 patients were able to walk; None of the SMA type 2 patients were ambulatory
-Methods used: Distributions based
Three different MCIR valued SEm, 1/2 SD and 1/3 SD were calculated with the overall cohort and by subgroups
Overall cohort: SEm= 2.9 ; 1/2 SD = 6.4 and 1/3 SD= 4.3
Non-ambulatory group: SEm= 2.0 ; 1/2 SD = 4.4 and 1/3 SD= 2.9
Ambulatory group: SEm= 0.4 ; 1/2 SD = 0.8 and 1/3 SD= 0.6
SMA type 2 group: SEm= 1.2 ; 1/2 SD = 2.7 and 1/3 SD= 1.8
SMA type 3 group: SEm= 2.7; 1/2 SD = 5.9 and 1/3 SD = 3.9

Evidence of responder thresholds: Yes

Reference(s) of development / validation

Mazzone ES, Mayhew A, Montes J, Ramsey D, Fanelli L, Young SD, Salazar R, De Sanctis R, Pasternak A, Glanzman A, Coratti G, Civitello M, Forcina N, Gee R, Duong T, Pane M, Scoto M, Pera MC, Messina S, Tennekoon G, Day JW, Darras BT, De Vivo DC, Finkel R, Muntoni F, Mercuri E. Revised upper limb module for spinal muscular atrophy: Development of a new module. Muscle Nerve. 2017 Jun;55(6):869-874
PubMed Abstract:

Pera MC, Coratti G, Mazzone ES, Montes J, Scoto M, De Sanctis R, Main M, Mayhew A, Muni Lofra R, Dunaway Young S, Glanzman AM, Duong T, Pasternak A, Ramsey D, Darras B, Day JW, Finkel RS, De Vivo DC, Sormani MP, Bovis F, Straub V, Muntoni F, Pane M, Mercuri E; iSMAC Consortium Group. Revised upper limb module for spinal muscular atrophy: 12 month changes. Muscle Nerve. 2019 Apr;59(4):426-430
PubMed Abstract:

Other references

Stolte B, Bois JM, Bolz S. Minimal clinically important differences in functional motor scores in adults with spinal muscular atrophy. Eur J Neurol. 2020 Dec;27(12):2586-2594.

AlfaNo LN, Miller NF, IammariNo MA. ACTIVE (Ability Captured Through Interactive Video Evaluation) workspace volume video game to quantify meaningful change in spinal muscular atrophy. Dev Med Child Neurol. 2020 Mar;62(3):303-309.

[Conference Abstract] Glanzman A, Mazzone E, Young SD. Reliability of functional outcome measures in spinal muscular atrophy: Results from multi-centered, global, phase 3 clinical trials (S13.004). Neurology Apr 2017, 88 (16 Supplement) S13.00

Inclusion of the COA in product labelling


Existence of Scoring / Interpretation / User Manual


Original language and translations

Original languages:
English for the UK
English for the USA
Italian for Italy

References of translations

None identified

Authors and contact information

Eugenio Mercuri, MD
Paediatric Neurology
Catholic University


Not reported

Review copy

Available in RULM manual