Neurology: Gradually freezing people: The cortical function gradually degenerates, which is closely related to the worsening of the disease

The clinical heterogeneity of amyotrophic lateral sclerosis (ALS) has led to the challenge of developing targeted therapies and evaluating disease-modifying treatment outcomes
.

Clinical trials will benefit from cohorts with the same phenotype (a method of enrichment in clinical trials) and non-invasive biomarkers that can accurately and objectively monitor disease progression

diagnosis

N-Acetyl Aspartate (NAA) is a sign of neuronal integrity, when taken in absolute value and combined with other metabolites (including creatine (Cr, a sign of energy metabolism), choline (Cho, a sign of membrane turnover) The ratio of Inositol (Ino, a presumptive sign of gliosis) is reduced when expressed
.

This is observed in both exercise and extra-exercise areas, including the frontal lobe

A key requirement is to evaluate spectral performance data in multi-center studies to tap the potential of MRS-derived biomarkers and promote clinical trials

It is also assumed that:
(1) NAA ratio (decreased neuronal integrity) in the motor and prefrontal lobe area gradually decreases;
(2) ALS table with faster clinical progress, greater burden on UMN (upper motor neuron), and stronger cognitive impairment Type NAA drops faster
.

The study was conducted as part of the Canadian ALS Neuroimaging Consortium (CALSNIC), using a unified imaging and clinical program from 5 centers

They included 76 ALS patients and 59 healthy controls who participated in a prospective, longitudinal, multicenter study of the Canadian ALS Neuroimaging Alliance (CALSNIC)
.

Participants underwent continuous clinical evaluation and MR spectroscopy at baseline, 4 months, and 8 months using a unified protocol from 5 centers

They found that: ALS patients had a lower NAA ratio in the motor cortex at baseline (P <0.

001)

The severity of UMN signs has not changed significantly over time
.

Only patients with cognitive impairment had a lower NAA ratio in the prefrontal cortex (P <0.

The significance of this is that the progressive degeneration of the ALS motor cortex was found to be associated with more aggressive clinical manifestations
.

These findings provide biological evidence of variable spatial and temporal brain degeneration associated with ALS disease heterogeneity

It was found that the progressive degeneration of the ALS motor cortex was associated with more aggressive clinical manifestations
.
It was found that the progressive degeneration of the ALS motor cortex was associated with more aggressive clinical manifestations
.

Original source:

Progressive Neurochemical Abnormalities in Cognitive and Motor Subgroups of Amyotrophic Lateral Sclerosis: A Prospective Multicenter Study
Daniel Ta, Abdullah Ishaque, Ojas Srivastava, Chris Hanstock, PeterSeres, Dean T Eurich, Collin Luk, Hannah Briemberg, RichardFrayne, Angela L.
Genge, Simon J Graham, Lawrence Korngut, Lorne Zinman, Sanjay Kalra
Neurology Jun 2021, 10.
1212/WNL.
0000000000012367; DOI:10.
1212/WNL.
0000000000012367

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