Table 1

Comparison of the two sets of criteria for the diagnosis of cognitive and behavioural impairment in ALS

ALSFTD-13ALSFTD-24
ALSbi
A diagnosis of ALSbi requires meeting at least two non-overlapping supportive diagnostic features from either the Neary criteria or Hodges criteria for FTD		ALSbi
A diagnosis of ALSbi requires:
1. The identification of apathy with or without other behaviour change
OR
2. Meeting at least two non-overlapping supportive diagnostic features from the Rascovsky criteria
ALSci
A diagnosis of ALSci depends on evidence of cognitive impairment at or below the fifth percentile on at the least two distinct tests of cognition that are sensitive executive functioning		ALSci
A diagnosis of ALSci depends on evidence of either executive dysfunction (including social cognition) or language dysfunction or a combination of the two. Executive impairment is defined as:
1. Impaired verbal fluency (letter)
OR
2. Impairment on two other non-overlapping measures of executive functions (which may include social cognition)
Language impairment is defined as:
(i) Impairment on two non-overlapping tests and in which language impairment is not solely explained by verbal fluency deficits.
ALScbi
Patients who meet the criteria for both ALSci and ALSbi
ALS-FTD ALS-bvFTD ALS-dementia (ALS-D)*, FTD-MND
All patient meeting either the Neary criteria or Hodges criteria for FTD		ALS-FTD, ALS-dementia (ALS-D)*, FTD-MND
A diagnosis of ALS-FTD requires:
1. Evidence of progressive deterioration of behaviour and/or cognition by observation or history
AND
2. The presence of at least 3 of the behavioural/cognitive symptoms outlined by Rascovsky et al (2011)
OR
3. The presence of at least 2 of those behavioural/cognitive symptoms, together with loss of insight and/or psychotic symptoms
OR
4. The presence of language impairment meeting criteria for semantic dementia/ semantic variant PPA or non-fluent variant PPA. This may coexist with behavioural/ cognitive symptoms as outlined above.

  • ALS, amyotrophic lateral sclerosis; ALS-D, ALS with dementia; ALS-FTD, ALS with frontotemporal dementia; ALSbi, ALS with behavioural impairment; ALScbi, ALS with cognitive and behavioural impairment; ALSci, ALS with cognitive impairment; FTD, frontotemporal dementia; FTD-MND, frontotemporal dementia and motor neuron disease; PPA, primary progressive aphasia.