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Cognitive and behavioural dysfunction in ALS and its assessment 

Cognitive and behavioural dysfunction in ALS and its assessment
Chapter:
Cognitive and behavioural dysfunction in ALS and its assessment
Author(s):

Sharon Abrahams

and Christopher Crockford

DOI:
10.1093/med/9780198757726.003.0008
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date: 26 February 2020

Cognitive and behavioural dysfunction in amyotrophic lateral sclerosis (ALS) occurs in up to half of patients with a spectrum from ALS with no cognitive or behavioural impairment to ALS with frontotemporal dementia (FTD). ~ 15% have a full blown ALS-FTD syndrome, while ~ 35% show milder and specific deficits on verbal fluency, executive and language functions and social cognition. Patients may show a behavioural syndrome that ranges from mild specific difficulties to changes that fulfil diagnostic criteria for behavioural variant-FTD. Apathy is the most prevalent symptom, but disinhibition, perseveration, loss of sympathy/empathy, and change in eating behaviour are also described. The importance of assessment is increasingly recognized. A distinction is made between brief assessment tools useful within ALS clinics and more extensive neuropsychological assessment by a qualified clinical neuropsychologist. Newly developed assessments specifically designed for ALS are available and will make valuable contribution to clinical care.

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