Progressive Neurochemical Abnormalities in Cognitive and Motor Subgroups of Amyotrophic Lateral Sclerosis: A Prospective Multicenter Study.
Adult
Aged
Amyotrophic Lateral Sclerosis
/ complications
Aspartic Acid
/ analogs & derivatives
Cognitive Dysfunction
/ diagnostic imaging
Disease Progression
Female
Humans
Longitudinal Studies
Magnetic Resonance Spectroscopy
/ methods
Male
Middle Aged
Motor Cortex
/ diagnostic imaging
Prefrontal Cortex
/ diagnostic imaging
Severity of Illness Index
Journal
Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060
Informations de publication
Date de publication:
24 08 2021
24 08 2021
Historique:
received:
13
01
2021
accepted:
19
05
2021
entrez:
24
8
2021
pubmed:
25
8
2021
medline:
4
9
2021
Statut:
ppublish
Résumé
To evaluate progressive cerebral degeneration in amyotrophic lateral sclerosis (ALS) by assessing alterations in Seventy-six patients with ALS and 59 healthy controls were enrolled in a prospective, longitudinal, multicenter study in the Canadian ALS Neuroimaging Consortium. Participants underwent serial clinical evaluations and magnetic resonance spectroscopy at baseline and 4 and 8 months using a harmonized protocol across 5 centers. NAA ratios were quantified in the motor cortex and prefrontal cortex. Patients were stratified into subgroups based on disease progression rate, upper motor neuron (UMN) signs, and cognitive status. Linear mixed models were used for baseline and longitudinal comparisons of NAA metabolite ratios. Patients with ALS had reduced NAA ratios in the motor cortex at baseline ( Progressive degeneration of the motor cortex in ALS is associated with more aggressive clinical presentations. These findings provide biological evidence of variable spatial and temporal cerebral degeneration linked to the disease heterogeneity of ALS. The use of standardized imaging protocols may have a role in clinical trials for patient selection or subgrouping. This study provides Class II evidence that MRS NAA metabolite ratios of the motor cortex are associated with more rapid disease progression and greater UMN signs in patients with ALS. ClinicalTrials.gov Identifier: NCT02405182.
Identifiants
pubmed: 34426551
pii: WNL.0000000000012367
doi: 10.1212/WNL.0000000000012367
pmc: PMC8397589
doi:
Substances chimiques
Aspartic Acid
30KYC7MIAI
N-acetylaspartate
997-55-7
Banques de données
ClinicalTrials.gov
['NCT02405182']
Types de publication
Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e803-e813Subventions
Organisme : CIHR
Pays : Canada
Informations de copyright
© 2021 American Academy of Neurology.
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