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Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

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Published on: July 16, 2021

Motor function and behaviour across the ALS-FTD spectrum.

D De Silva^1,2, S Hsieh³, J Caga³

¹Neuroscience Research Australia, Sydney, NSW, Australia.

Acta Neurologica Scandinavica

|July 31, 2015

Summary

This summary is machine-generated.

Behavioral disturbances are common in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The Frontotemporal Dementia Functional Rating Scale (FTDFRS) shows decline in FTD and ALS-FTD, and a trend in ALS, independent of motor function.

Keywords:

amyotrophic lateral sclerosis dementia neurodegenerative disorders

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Area of Science:

Neurology
Neuroscience
Clinical Research

Background:

Behavioral and functional disturbances are characteristic of frontotemporal dementia (FTD).
These disturbances are also observed in amyotrophic lateral sclerosis (ALS) and in patients with combined ALS and FTD (FTD-ALS).

Purpose of the Study:

To investigate the progression of behavioral disturbances in ALS and FTD.
Utilize the Frontotemporal Dementia Functional Rating Scale (FTDFRS) for assessment.

Main Methods:

Recruited patients with ALS, FTD-ALS, and FTD from specialist clinics.
Conducted baseline and longitudinal assessments using the FTDFRS and the ALS Functional Rating Scale-Revised (ALSFRS-R).

Main Results:

Moderate to severe behavioral disturbance was frequent at baseline in ALS (47.6%), less so than in behavioral variant FTD (bvFTD), with FTD-ALS patients showing intermediate impairment.
The ALSFRS-R did not correlate with the FTDFRS.
Significant behavioral deterioration occurred in bvFTD and FTD-ALS patients during follow-up, with a trend towards decline in ALS patients (P=0.06).

Conclusions:

While motor disturbance is the primary marker in ALS, behavioral and functional impairments are common and can worsen independently of motor function.
The FTDFRS can offer valuable insights for assessing and monitoring ALS patients.