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Cognitive and behavioural dysfunction in ALS and its assessment 

Cognitive and behavioural dysfunction in ALS and its assessment
Cognitive and behavioural dysfunction in ALS and its assessment

Sharon Abrahams

and Christopher Crockford

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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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