What does the ALSFRS-R really measure? A longitudinal and survival analysis of functional dimension subscores in amyotrophic lateral sclerosis

Rooney, James; Burke, Tom; Vajda, Alice; Heverin, Mark; Hardiman, Orla

doi:10.1136/jnnp-2016-314661

Cited by 105 publications

(80 citation statements)

References 21 publications

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“…ALS progression is measured functionally using the ALS Functional Rating Scale – Revised, which uses 12 questions scored between 0 (no function) and 4 (full function) to generate a summary score 27 . The scale is widely used but has some limitations, since the subscores correlate more accurately with progression in different clinical subtypes 28 .…”

Section: What Is Als?mentioning

confidence: 99%

What causes amyotrophic lateral sclerosis?

2017

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Amyotrophic lateral sclerosis is a neurodegenerative disease predominantly affecting upper and lower motor neurons, resulting in progressive paralysis and death from respiratory failure within 2 to 3 years. The peak age of onset is 55 to 70 years, with a male predominance. The causes of amyotrophic lateral sclerosis are only partly known, but they include some environmental risk factors as well as several genes that have been identified as harbouring disease-associated variation. Here we review the nature, epidemiology, genetic associations, and environmental exposures associated with amyotrophic lateral sclerosis.

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Section: What Is Als?mentioning

confidence: 99%

What causes amyotrophic lateral sclerosis?

2017

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“…Assessment of clinical progression was done using a modified Rankin scale and Amyotrophic Lateral Sclerosis Rating Scale Revised (ALSFRS-R) differences (total score and 4 main domains) [9] through time, though this latter scale has not been validated for hSP and PLS. Spasticity at the time of sampling and at the last observation was assessed using the Ashworth scale.…”

Section: Methodsmentioning

confidence: 99%

Cerebrospinal Fluid Neurofilaments May Discriminate Upper Motor Neuron Syndromes: A Pilot Study

et al. 2018

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Background: Patients presenting with upper motor neuron (UMN) signs may widely diverge in prognosis, ranging from amyotrophic lateral sclerosis (ALS) to primary lateral sclerosis (PLS) and hereditary spastic paraplegia (hSP). Neurofilaments are emerging as potential diagnostic and prognostic biomarkers for ALS, but the diagnosis of UMN syndromes still relies mostly on clinical long-term observation and on familiarity or genetic confirmation. Objectives: To test whether phosphorylated neurofilament heavy chain (pNfH) may discriminate different UMN syndromes at diagnosis and to test their prognostic role among these diseases. Methods: We measured the cerebrospinal fluid (CSF) and serum pNfH of 30 patients presenting with UMN signs and diagnosed with ALS, hSP, and PLS, plus 9 healthy controls (HC). Results: ALS patients had higher levels of pNfH in CSF and serum compared to HC (p < 0.001 and p < 0.001 in CSF and serum, respectively) and PLS (p = 0.015 and p = 0.038) and hSP (p = 0.003 and p = 0.001) patients. PLS and hSP patients had similar CSF and serum pNfH concentrations, but a higher CSF pNfH concentration, compared to HC (p = 0.002 and p = 0.003 for PLS and hSP, respectively). Receiver operating characteristic curves for discriminating ALS from PLS and hSP showed an area under the curve of 0.79 for CSF and 0.81 for serum. In multivariable survival analysis including relevant clinical factors CSF pNfH represented the strongest variable predicting survival (HR 40.43; 95% CI 3.49–467.79, p = 0.003) independently of clinical group. Conclusions: Despite some statistical instability of the results due to limitations in sample size, our study supports the role of CSF pNfH as a prognostic biomarker for motor neuron diseases presenting with UMN signs. A potential power to discriminate between ALS and other UMN syndromes at presentation, and between all of the examined MND and HC, has been detected for both CSF and serum pNfH.

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“…In their JNNP manuscript, Rooney et al apply these ideas to their Irish database of 407 ALS patients' ALSFRS scores 5. In general, they confirm the Italian findings,4 noting in particular and perhaps counterintuitively, that respiratory subscores by themselves are not predictive of outcome.…”

Section: The Als Functional Rating Scale Interrogates Bulbar Motor Amentioning

confidence: 88%

New ideas on the ALS Functional Rating Scale

Swash

2016

J Neurol Neurosurg Psychiatry

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What does the ALSFRS-R really measure? A longitudinal and survival analysis of functional dimension subscores in amyotrophic lateral sclerosis

Cited by 105 publications

References 21 publications

What causes amyotrophic lateral sclerosis?

What causes amyotrophic lateral sclerosis?

Cerebrospinal Fluid Neurofilaments May Discriminate Upper Motor Neuron Syndromes: A Pilot Study

New ideas on the ALS Functional Rating Scale

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