JNNP: Sensitivity of brain MRI and nervous system examination to upper motor neuron degeneration in patients with amyotrophic lateral sclerosis

The heterogeneity of spinal cord or brainstem lower motor neuron (LMN) and upper motor neuron (UMN) and frontotemporal brain pathology determines the clinical variability of patients with amyotrophic lateral sclerosis (ALS) and contributes to disease progression The early diagnosis and measurement are complicated
.

Clinically, the clinical features of LMN are supported by the results of electromyography, which may be easier to detect and objectively quantify than UMN signs in follow-up studies, because UMN clinical features may be hidden in severely affected limbs of LMN

The heterogeneity of spinal cord or brainstem lower motor neuron (LMN) and upper motor neuron (UMN) and frontotemporal brain pathology determines the clinical variability of patients with amyotrophic lateral sclerosis (ALS) and contributes to disease progression The early diagnosis and measurement are complicated

In clinical studies, such as trials to determine the effective treatment of ALS, the revised ALS Function Rating Scale (ALSFRS-R) is used to measure functional decline
.

However, the A LSFRS-R project is particularly sensitive to the signs of LMN, which means that clinical trials may not be able to measure the effect of UMN treatment

The LSFRS-R project is particularly sensitive to the signs of LMN, which means that clinical trials may not be able to measure the effect of UMN treatment

678 subjects were recruited
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Patients who were followed up at least twice were selected, and 136 patients who were followed up only once were excluded

678 subjects were recruited

The research design of this experiment

The research design of this experiment

The subjects in the healthy control group also underwent neurological examinations to determine that none of them had UMN symptoms that could confuse the results
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Each examiner uses a standardized protocol derived from the neurological examination of the Motor Neuron Disease (MND) Specialist Center

The subjects in the healthy control group also underwent neurological examinations to determine that none of them had UMN symptoms that could confuse the results

The pattern of motor cortex thinning
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The motor cortex is divided into 30 parts in each hemisphere, and the difference between the control group (n=314) and ALS patients (n=192) is measured

The pattern of motor cortex thinning

Use exercise CT and pyramidal tract fiber density as indicators of UMN degeneration (ie, the lower the cortical thickness and fiber density, the more severe the UMN degeneration)

The average UMN degeneration of exercise CT and fiber density measurements worsened in subsequent classifications

Brain MRI can monitor UMN degeneration before symptoms become obvious
.
The results of the study prompted MRI to become a potential biomarker of UMN progress in ALS clinical trials
.
Brain MRI can monitor UMN degeneration before symptoms become obvious
.
The results of the study prompted MRI to become a potential biomarker of UMN progress in ALS clinical trials
.
NitertAD ,TanHH ,WalhoutR NitertAD Nitert TanHH Tan Walhout  R Walhout , et al Sensitivity of brain MRI and neurological examination for detection of upper motor neurone degeneration in amyotrophic lateral sclerosis Journal of Neurology, Neurosurgery & Psychiatry  Published Online First:  18 October 2021.
  Published Online First: doi: 10.
1136/jnnp-2021-327269 doi:

 

 

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