Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis

Uros Klickovic; Luca Zampedri; Nick Zafeiropoulos; Oliver J Ziff; Christopher Dj Sinclair; Stephen Wastling; Magdalena Dudziec; Jodie Allen; Karin Trimmel; Robin S Howard; Andrea Malaspina; Nikhil Sharma; Katie Cl Sidle; Sachit Shah; Christian Nasel; Tarek A Yousry; Linda Greensmith; Jasper M Morrow; John S Thornton; Pietro Fratta

doi:10.1136/jnnp-2024-335571

Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis

Uros Klickovic, Luca Zampedri, Nick Zafeiropoulos, Oliver J Ziff, Christopher Dj Sinclair, Stephen Wastling, Magdalena Dudziec, Jodie Allen, Karin Trimmel, Robin S Howard, Andrea Malaspina, Nikhil Sharma, Katie Cl Sidle, Sachit Shah, Christian Nasel, Tarek A Yousry, Linda Greensmith, Jasper M Morrow, John S Thornton, Pietro Fratta

Klinisches Institut für diagnostische und interventionelle Radiologie (Universitätsklinikum Tulln)

Publikation: Beitrag in Fachzeitschrift (peer-reviewed) › Artikel in Fachzeitschrift

Abstract

BACKGROUND AND OBJECTIVES: Quantitative and operator-independent biomarkers of disease progression are urgently needed in amyotrophic lateral sclerosis (ALS) research. We assess the potential of skeletal muscle MRI as a sensitive and reliable outcome measure for future ALS clinical trials.

METHODS: In this longitudinal cohort study, muscle MRI of head-neck, upper and lower limb regions, alongside clinical and functional assessments, were acquired at three time points over the individual maximum observation period (iMOP) of 1 year in 20 patients with ALS and 16 healthy controls. Quantitative MRI parameters cross-sectional area (CSA), volume (VOL), fat fraction, functional rest muscle area and water T2 (T2m) were correlated with changes in clinical disease severity (functional rating scales and myometry).

RESULTS: Among 20 patients with ALS, 17 completed follow-up. Progressive muscle atrophy (CSA, VOL) was observed at hand (rs=0.66), head-neck (partial η²=0.47) and lower-limb level (thighs: η²=0.56, calves: η²=0.54) over iMOP. MRI changes correlated with leg muscle strength (knee extension: r=0.77; plantar flexion: r=0.78), hand grip strength (r=0.71) and functional rating scales (r=0.68).

INTERPRETATION: Our findings demonstrate the effectiveness of muscle MRI as a sensitive neuroimaging biomarker of disease progression in ALS, highlighting its potential application in clinical trials.

Originalsprache	Englisch
Seiten (von - bis)	908-911
Seitenumfang	4
Fachzeitschrift	Journal of Neurology, Neurosurgery and Psychiatry
Jahrgang	96
Ausgabenummer	9
Frühes Online-Datum	25 März 2025
DOIs	https://doi.org/10.1136/jnnp-2024-335571
Publikationsstatus	Veröffentlicht - 01 Sept. 2025

ASJC Scopus Sachgebiete

Chirurgie
Klinische Neurologie
Psychiatrie und psychische Gesundheit

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10.1136/jnnp-2024-335571

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Klickovic, U., Zampedri, L., Zafeiropoulos, N., Ziff, O. J., Sinclair, C. D., Wastling, S., Dudziec, M., Allen, J., Trimmel, K., Howard, R. S., Malaspina, A., Sharma, N., Sidle, K. C., Shah, S., Nasel, C., Yousry, T. A., Greensmith, L., Morrow, J. M., Thornton, J. S., & Fratta, P. (2025). Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis. Journal of Neurology, Neurosurgery and Psychiatry, 96(9), 908-911. https://doi.org/10.1136/jnnp-2024-335571

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title = "Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis",

abstract = "BACKGROUND AND OBJECTIVES: Quantitative and operator-independent biomarkers of disease progression are urgently needed in amyotrophic lateral sclerosis (ALS) research. We assess the potential of skeletal muscle MRI as a sensitive and reliable outcome measure for future ALS clinical trials.METHODS: In this longitudinal cohort study, muscle MRI of head-neck, upper and lower limb regions, alongside clinical and functional assessments, were acquired at three time points over the individual maximum observation period (iMOP) of 1 year in 20 patients with ALS and 16 healthy controls. Quantitative MRI parameters cross-sectional area (CSA), volume (VOL), fat fraction, functional rest muscle area and water T2 (T2m) were correlated with changes in clinical disease severity (functional rating scales and myometry).RESULTS: Among 20 patients with ALS, 17 completed follow-up. Progressive muscle atrophy (CSA, VOL) was observed at hand (rs=0.66), head-neck (partial η²=0.47) and lower-limb level (thighs: η²=0.56, calves: η²=0.54) over iMOP. MRI changes correlated with leg muscle strength (knee extension: r=0.77; plantar flexion: r=0.78), hand grip strength (r=0.71) and functional rating scales (r=0.68).INTERPRETATION: Our findings demonstrate the effectiveness of muscle MRI as a sensitive neuroimaging biomarker of disease progression in ALS, highlighting its potential application in clinical trials.",

keywords = "ALS, MRI, MUSCLE DISEASE, NEUROMUSCULAR",

author = "Uros Klickovic and Luca Zampedri and Nick Zafeiropoulos and Ziff, \{Oliver J\} and Sinclair, \{Christopher Dj\} and Stephen Wastling and Magdalena Dudziec and Jodie Allen and Karin Trimmel and Howard, \{Robin S\} and Andrea Malaspina and Nikhil Sharma and Sidle, \{Katie Cl\} and Sachit Shah and Christian Nasel and Yousry, \{Tarek A\} and Linda Greensmith and Morrow, \{Jasper M\} and Thornton, \{John S\} and Pietro Fratta",

note = "Publisher Copyright: {\textcopyright} Author(s) (or their employer(s)) 2025.",

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month = sep,

day = "1",

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language = "English",

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Klickovic, U, Zampedri, L, Zafeiropoulos, N, Ziff, OJ, Sinclair, CD, Wastling, S, Dudziec, M, Allen, J, Trimmel, K, Howard, RS, Malaspina, A, Sharma, N, Sidle, KC, Shah, S, Nasel, C, Yousry, TA, Greensmith, L, Morrow, JM, Thornton, JS & Fratta, P 2025, 'Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis', Journal of Neurology, Neurosurgery and Psychiatry, Jg. 96, Nr. 9, S. 908-911. https://doi.org/10.1136/jnnp-2024-335571

TY - JOUR

T1 - Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis

AU - Klickovic, Uros

AU - Zampedri, Luca

AU - Zafeiropoulos, Nick

AU - Ziff, Oliver J

AU - Sinclair, Christopher Dj

AU - Wastling, Stephen

AU - Dudziec, Magdalena

AU - Allen, Jodie

AU - Trimmel, Karin

AU - Howard, Robin S

AU - Malaspina, Andrea

AU - Sharma, Nikhil

AU - Sidle, Katie Cl

AU - Shah, Sachit

AU - Nasel, Christian

AU - Yousry, Tarek A

AU - Greensmith, Linda

AU - Morrow, Jasper M

AU - Thornton, John S

AU - Fratta, Pietro

PY - 2025/9/1

Y1 - 2025/9/1

N2 - BACKGROUND AND OBJECTIVES: Quantitative and operator-independent biomarkers of disease progression are urgently needed in amyotrophic lateral sclerosis (ALS) research. We assess the potential of skeletal muscle MRI as a sensitive and reliable outcome measure for future ALS clinical trials.METHODS: In this longitudinal cohort study, muscle MRI of head-neck, upper and lower limb regions, alongside clinical and functional assessments, were acquired at three time points over the individual maximum observation period (iMOP) of 1 year in 20 patients with ALS and 16 healthy controls. Quantitative MRI parameters cross-sectional area (CSA), volume (VOL), fat fraction, functional rest muscle area and water T2 (T2m) were correlated with changes in clinical disease severity (functional rating scales and myometry).RESULTS: Among 20 patients with ALS, 17 completed follow-up. Progressive muscle atrophy (CSA, VOL) was observed at hand (rs=0.66), head-neck (partial η²=0.47) and lower-limb level (thighs: η²=0.56, calves: η²=0.54) over iMOP. MRI changes correlated with leg muscle strength (knee extension: r=0.77; plantar flexion: r=0.78), hand grip strength (r=0.71) and functional rating scales (r=0.68).INTERPRETATION: Our findings demonstrate the effectiveness of muscle MRI as a sensitive neuroimaging biomarker of disease progression in ALS, highlighting its potential application in clinical trials.

AB - BACKGROUND AND OBJECTIVES: Quantitative and operator-independent biomarkers of disease progression are urgently needed in amyotrophic lateral sclerosis (ALS) research. We assess the potential of skeletal muscle MRI as a sensitive and reliable outcome measure for future ALS clinical trials.METHODS: In this longitudinal cohort study, muscle MRI of head-neck, upper and lower limb regions, alongside clinical and functional assessments, were acquired at three time points over the individual maximum observation period (iMOP) of 1 year in 20 patients with ALS and 16 healthy controls. Quantitative MRI parameters cross-sectional area (CSA), volume (VOL), fat fraction, functional rest muscle area and water T2 (T2m) were correlated with changes in clinical disease severity (functional rating scales and myometry).RESULTS: Among 20 patients with ALS, 17 completed follow-up. Progressive muscle atrophy (CSA, VOL) was observed at hand (rs=0.66), head-neck (partial η²=0.47) and lower-limb level (thighs: η²=0.56, calves: η²=0.54) over iMOP. MRI changes correlated with leg muscle strength (knee extension: r=0.77; plantar flexion: r=0.78), hand grip strength (r=0.71) and functional rating scales (r=0.68).INTERPRETATION: Our findings demonstrate the effectiveness of muscle MRI as a sensitive neuroimaging biomarker of disease progression in ALS, highlighting its potential application in clinical trials.

KW - ALS

KW - MRI

KW - MUSCLE DISEASE

KW - NEUROMUSCULAR

UR - https://www.scopus.com/pages/publications/105001260756

U2 - 10.1136/jnnp-2024-335571

DO - 10.1136/jnnp-2024-335571

M3 - Journal article

C2 - 40132878

SN - 0022-3050

VL - 96

SP - 908

EP - 911

JO - Journal of Neurology, Neurosurgery and Psychiatry

JF - Journal of Neurology, Neurosurgery and Psychiatry

IS - 9

ER -

Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis

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