Reliable and efficient scale to assess upper motor neuron disease burden in amyotrophic lateral sclerosis

Quinn, Colin; Edmundson, Christyn; Dahodwala, Nabila; Elman, Lauren

doi:10.1002/mus.26764

Cited by 62 publications

(49 citation statements)

References 9 publications

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“…This method proved to be effective. (19) A validation study has shown excellent inter-rater and intra-rater reliability (14).…”

Section: The Penn Upper Motor Neuron Score #mentioning

confidence: 99%

“…Scales using clinical examination findings to grade the upper motor neuron burden (UMNB) have been used in several imaging studies for ALS (8)(9)(10)(11). These scales typically are heavily weighted toward measures of hyperreflexia, but may include measures of muscle tone or movement speed (9,(12)(13)(14). Recently one such scale that includes several types of UMN measures, the Penn Upper Motor Neuron Score # (PUMNS), underwent testing to validate its reliability (14).…”

Section: Scales To Quantify Upper Motor Neuron Functionmentioning

confidence: 99%

“…These scales typically are heavily weighted toward measures of hyperreflexia, but may include measures of muscle tone or movement speed (9,(12)(13)(14). Recently one such scale that includes several types of UMN measures, the Penn Upper Motor Neuron Score # (PUMNS), underwent testing to validate its reliability (14).…”

Section: Scales To Quantify Upper Motor Neuron Functionmentioning

confidence: 99%

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Measuring disease progression in primary lateral sclerosis

Gilmore

Elman

Babu

et al. 2020

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Quantitative measures of disease severity are essential outcome measures for clinical trials. The slow progression of disease in primary lateral sclerosis (PLS) requires clinical measures that are sensitive to changes occurring within the time frame of a clinical trial. Proposed clinical outcome measures include the PLS functional rating scale (PLSFRS), burden scores derived from clinical examination findings, and quantitative measures of motor performance. The PLSFRS has good inter-rater reliability and showed greater longitudinal change over 6-and 12-months compared to the revised ALS functional rating scale. Examination-based upper motor neuron burden (UMNB) scales also have good reliability, and longitudinal studies are in process. Quantitative measures of strength, dexterity, gait, and speech have the potential to provide objective and precise measures of clinical change, but have been the least studied in persons with PLS.

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“…This method proved to be effective. (19) A validation study has shown excellent inter-rater and intra-rater reliability (14).…”

Section: The Penn Upper Motor Neuron Score #mentioning

confidence: 99%

Section: Scales To Quantify Upper Motor Neuron Functionmentioning

confidence: 99%

Section: Scales To Quantify Upper Motor Neuron Functionmentioning

confidence: 99%

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Measuring disease progression in primary lateral sclerosis

Gilmore

Elman

Babu

et al. 2020

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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“…Supplementary clinical information is presented Fig. 1 ., where the ALS functional rating scale (ALSFRS-r) and Penn UMN sub-scale profile of each PLS group are additionally presented [ 13 , 14 ]. Raw grey and white matter data are also presented in box plots for each cohort in Fig.…”

Section: Data Descriptionmentioning

confidence: 99%

Imaging and clinical data indicate considerable disease burden in ‘probable’ PLS: Patients with UMN symptoms for 2–4 years

et al. 2020

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Primary lateral sclerosis (PLS) is an adult-onset upper motor neuron disease manifesting in progressive spasticity and gradually resulting in considerably motor disability. In the absence of early disease-specific diagnostic indicators, the majority of patients with PLS face a circuitous diagnostic journey. Until the recent publication of consensus diagnostic criteria, 4-year symptom duration was required to establish the diagnosis. The new diagnostic criteria introduced the category of ‘probable PLS’ for patients with a symptom duration of 2–4 years. “Evolving diagnostic criteria in primary lateral sclerosis: The clinical and radiological basis of "probable PLS" [1] . This dataset provides radiological metrics in a cohort of ‘probable PLS’ patients, ‘definite PLS’ patients and age-matched healthy controls. Region-of-interest radiological data include diffusivity metrics in the corticospinal tracts and corpus callosum as well as mean cortical thickness values in the pre- and para-central gyri in each hemisphere. Our data indicate considerable grey matter and relatively limited white matter involvement in ‘probable PLS’ which supports the rationale for this diagnostic category as a clinically useful entity. The introduction of this diagnostic category will likely facilitate the timely recruitment of PLS patients into research studies and pharmacological trials before widespread neurodegenerative change ensues.

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“…13,14 The Penn UMN (PUMN) scale is a comprehensive clinical rating scale that summates three separate subscales (CNS-Lability Scale [CNS-LS], modified Ashworth, and reflexes) to measure the UMN dysfunction manifestations of spasticity, hyperreflexia, and pseudobulbar affect in ALS. 15 Quinn et al 16 completed a study to understand the test-retest reliability of the reflexes and spasticity subdomains of the PUMN scale in 15 participants with ALS. This builds on prior knowledge that the CNS-LS subscale has good reliability by itself.…”

mentioning

confidence: 99%