Neurology: gradual freezing syndrome, abnormal eye movements or predictable cognitive changes

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects upper motor neurons (UMN) and lower motor neurons (LMN)
.

Although ALS has long been considered a pure motor system disease, it is now accepted that cognitive and behavioral changes suggesting frontal lobe dysfunction are often part of the clinical syndrome

Some studies have also shown that different types of eye movement abnormalities (OMAs) may also occur in ALS
.

However, in ALS patients, the frequency of clinically significant OMAs found through standard neurological examinations rather than quantitative video oculographic measurements , and their relationship to underlying motor and cognitive phenotypes are still unknown

Videos , and their relationship to underlying motor and cognitive phenotypes are still unknown

Recently, people have studied the relationship between OMAs assessed by video-oculographic registration and clinical and neuropsychological performance
.

Interestingly, through the revised ALS Functional Rating Scale (ALSFRS-R) score, it is found that there is a clear correlation with dysfunction, and it is also significantly related to the cognitive score of the Edinburgh Cognitive and Behavioral ALS Screening (ECAS) Relevance

Screening

In this way, Istituto Auxologico Italiano and others in Italy explored the frequency of clinically obvious OMAs in a large number of ALS patients, determined their patterns, and correlated these findings with motor and cognitive behavioral characteristics
.

They retrospectively evaluated the inpatient cohort of three consecutive Italian ALS patients and the control group to assess the frequency of OMA and changes in cognitive behavior
.

The ALS population is divided into a discovery queue (discovery) and a replication queue (replication)

They included 864 ALS (635 discoverers, 229 replicators), 798 cognitively accessible subjects, and 171 AD subjects
.

In the ALS cohort, 10.
5% of people had detected OMAs, while only 1.
6% of the cognitively accessible control group (p=1.
2x10-14) and 11.
4% of AD patients (p=ns)
.

The most common defects are abnormalities of smooth muscle and cystic muscle
.

The frequency of OMAs is higher in patients with bulbar onset, prominent upper motor neuron signs, and advanced disease

In the two ALS cohorts, cognitive dysfunction was significantly more frequent in patients with OMAs (p=1.
1x10-25)
.

In addition, OMAs are significantly related to the severity of cognitive impairment and the pathological scores of specific areas of ECAS ALS
.

OMAs are significantly related to the severity of cognitive impairment and the pathological scores of specific areas of ECAS ALS
.

Finally, OMAs can be observed in 35.
0% of cognitively impaired ALS and 11.
4% of AD patients (p=6.
4x10-7), which indicates that the frontal eye movement area may be involved in ALS
.

Patients with ALS show a series of clinically obvious OMAs, and these changes are clearly correlated with changes in cognition (not behavior)
.

OMAs may be a sign of neurodegeneration, and bedside assessment is a rapid and highly specific tool for detecting cognitive impairment in ALS

These changes are clearly related to changes in cognition (not behavior)

 

Original source:
Poletti B, Solca F, Carelli L, et al.

Association of Clinically Evident Eye Movement Abnormalities With Motor and Cognitive Features in Patients With Motor Neuron Disorders .

Association of Clinically Evident Eye Movement Abnormalities With Motor and Cognitive Features in Patients With Motor Neuron Disorders in this message