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A Fine Motor Task to Study Joint Kinematics in a Preclinical Model of Neurodegenerative Disease
Published on: June 13, 2025
Cognition and behaviour in motor neurone disease
Patricia Lillo1, John R Hodges
1Neuroscience Research Australia, University of New South Wales, Sydney, New South of Wales, Australia. p.lillo@neura.edu.au
Current Opinion in Neurology
|October 22, 2010
Summary
Motor neurone disease (MND) is increasingly recognized as a multi-system disorder with potential cognitive and behavioral changes. These changes, linked to specific protein inclusions, may affect disease progression and survival in a subset of patients.
Area of Science:
- Neuroscience
- Neurology
- Genetics
Background:
- Motor neurone disease (MND) was traditionally viewed as a pure motor syndrome.
- Emerging evidence suggests significant cognitive and behavioral changes in up to 50% of MND patients.
- These changes can be severe, meeting criteria for frontotemporal dementia.
Purpose of the Study:
- To review cognitive and behavioral changes in MND.
- To emphasize recent advancements in understanding these changes.
- To explore the overlap between MND and frontotemporal dementia.
Main Methods:
- Review of recent literature on MND pathology and clinical presentation.
- Analysis of findings related to TDP-43 and FUS inclusions.
- Examination of longitudinal studies on cognitive progression in MND.
Main Results:
- TDP-43 and FUS inclusions are key pathological components in MND and related disorders.
- Mutations in TARDBP and FUS genes are identified.
- Cognitive impairment in MND progresses mildly and affects a proportion of patients, correlating with faster disease and shorter survival.
Conclusions:
- MND is a multi-system disorder with significant overlap with frontotemporal dementia.
- Cognitive and behavioral changes occur in a subset of MND patients.
- The reasons for variability in cognitive and behavioral presentation remain undetermined.
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