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

  title={Reliable and efficient scale to assess upper motor neuron disease burden in amyotrophic lateral sclerosis},
  author={Colin Quinn and Christyn Edmundson and Nabila Dahodwala and Lauren Elman},
  journal={Muscle \& Nerve},
  pages={508 - 511}
Validation of biomarkers of upper motor neuron (UMN) impairment in amyotrophic lateral sclerosis (ALS) requires a reliable clinical assessment of UMN findings. The Penn Upper Motor Neuron Score© (PUMNS) is a standardized measure of UMN signs in ALS. Our aims were to evaluate its intra‐ and inter‐rater reliability, and to examine inter‐item reliability as a proxy for item relatedness and scale efficiency. 

Upper motor neuron dysfunction is associated with the presence of behavioural impairment in patients with amyotrophic lateral sclerosis

The aim of this study was to assess whether the burden of upper and lower motor neuron involvement is associated with the presence of cognitive and behavioural impairment with amyotrophic lateral sclerosis.

Upper motor neuron burden measurement in motor neuron diseases: Does one scale fit all?

  • Suma Babu
  • Psychology, Medicine
    Muscle & nerve
  • 2020
The study provided evidence that the summated score of the reflexes and spasticity subdomains of the PUMN had good interrater reliability between two blinded raters, providing evidence of its clinical relevance in assessing ALS in patients.

Longitudinal evaluation of upper motor neuron burden scales in primary lateral sclerosis

  • M. FloeterTianxia Wu
  • Medicine, Psychology
    Amyotrophic lateral sclerosis & frontotemporal degeneration
  • 2020
In a cohort of PLS patients enrolled in a natural history study, the three UMNB scales exhibited modest linear increases over the first 8 years of symptoms, followed by a plateau, which should consider stratification of patients by disease duration.

Measuring disease progression in primary lateral sclerosis

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.

Extra-motor cerebral changes and manifestations in primary lateral sclerosis.

The data suggest that PLS should no longer be considered a neurodegenerative disorder selectively affecting the pyramidal system and is instead considered a multidisciplinary management of this low-incidence condition.

Early diagnosis of amyotrophic lateral sclerosis by threshold tracking and conventional transcranial magnetic stimulation

This study compared A‐S ICI and T‐SICI for sensitivity and clinical usefulness as biomarkers for ALS.

CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement

CSF pNfH is significantly higher inclassic and UMNp-ALS compared to UMN diseases with a better prognosis such as PLS and hSP, and its prognostic role is confirmed in classic and bulbar ALS, but not among U MNp, where clinical signs remained the only independent prognostic factors.

Elevated plasma phosphorylated tau in amyotrophic lateral sclerosis relates to lower motor neuron dysfunction

Novel evidence is found that plasma p-tau181 is elevated in ALS and may be a novel marker specific to LMN dysfunction, and a detailed clinical evaluation is performed to unravel the potential source of this unexpected observation.

Evolving diagnostic criteria in primary lateral sclerosis: The clinical and radiological basis of “probable PLS”



Widespread grey matter pathology dominates the longitudinal cerebral MRI and clinical landscape of amyotrophic lateral sclerosis

A characteristic white matter tract pathological signature is seen cross-sectionally, while cortical involvement dominates longitudinally in amyotrophic lateral sclerosis, which has implications for the development of biomarkers for diagnosis versus therapeutic monitoring.

Linear Associations between Clinically Assessed Upper Motor Neuron Disease and Diffusion Tensor Imaging Metrics in Amyotrophic Lateral Sclerosis

Measured by the template-space ROI method, both MD and FA were linearly associated with the Penn UMN Score, supporting the hypothesis that DTI alterations reflect UMN pathology as assessed by the clinical examination.

Low signal intensity in motor cortex on susceptibility-weighted MR imaging is correlated with clinical signs of amyotrophic lateral sclerosis: a pilot study

Investigation of the relationship between clinical symptoms and susceptibility changes in the motor cortex measured using susceptibility-weighted MR imaging taken by readily available 3-T MRI in clinical practice indicated that low signal intensity in motor cortex on susceptibility- weighed MR imaging may correspond to clinical symptoms, particularly upper motor neuron dysfunction.

A proton magnetic resonance spectroscopic study in ALS

In vivo 1H-MRS of the subcortical white matter in the motor region is unlikely to be sensitive enough to detect early disease changes in ALS, however, changes in the NAA/(Cr+PCr) metabolic peak area ratios correlate with clinical measures of disease severity, and this measure may be useful in monitoring disease progression.

Lowered cerebral glucose utilization in amyotrophic lateral sclerosis

The demonstration of severe generalized hypometabolism in structurally normal cortex indicates that some cortical neurons exist in a state of neuronal nonfunction, rather than cell death, and that anatomoclinical correlations may be more complex.

Glial activation colocalizes with structural abnormalities in amyotrophic lateral sclerosis

Increased uptake of the glial marker [11C]-PBR28 colocalizes with changes in FA and cortical thinning, suggesting a link between disease mechanisms (gliosis and inflammation) and structural changes (cortical thinning and white and gray matter changes).

A self report measure of affective lability

The Center for Neurologic Study- Lability Scale (CNS-LS) is a short, easily administered, and psychometrically sound measure of affective lability for use with patients with ALS and has potential applications as both a clinical screening device and a research tool.

Measuring pseudobulbar affect in ALS

  • Richard SmithJ. BergL. PopeR. Thisted
  • Psychology, Biology
    Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
  • 2004
The Center for Neurologic Study-Lability Scale is a self- administered scale that has been validated in a large population of ALS patients and has proven to be a useful diagnostic instrument and a robust clinical trial endpoint in ALS.

Interrater reliability of a modified Ashworth scale of muscle spasticity.

The relationship between the raters' judgments was significant and the reliability was good, and it is believed these results to be positive enough to encourage further trials of the modified Ashworth scale for grading spasticity.

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

  • Presented at: Northeast ALS Consortium Meeting