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Cognitive assessment

A fuller characterisation of the extent of cognitive and behavioural dysfunction in MND is not simply of academic interest but has important implications given that the burden and stress for carers of patients with FTD is very great. It also has relevance to effective communication, legal issues and end-of-life decision making by patients with MND (Lillo and Hodges 2009).

Ideally, consultation by a neuropsychologist is beneficial if significant cognitive involvement is suspected.

Strong and others 2009

Individuals assessing ALS patients for cognitive or behavioural impairment should be familiar with the physical disease state and expected progression. Accommodation may be required for speech deficits resulting from dysarthria, as well as compromised motor speed.

The participants recognized that due to the complexity of a formal neuropsychological evaluation for a frontotemporal syndrome in an individual with a motor neuron disease, a hierarchical approach might be required. Thus, the participants recommended that the testing paradigms could include a screening assessment (based on empirically-supported screens when available, 2–5 min to maximize efficient use by ALS clinicians or staff), a brief assessment (5–20 min using established cognitive screens, a combination of several cognitive screens, and/or behavioural questionnaires) and, wherever possible, a formal neuropsychological assessment.

A screening assessment should never be used to make the diagnosis of ALS-FTD, ALSbi or ALSci. Although ALS-FTD and ALSbi may be diagnosed without formal neuropsychological testing, this should only occur after a comprehensive neurobehavioural interview is completed, preferably with interviews of both the patient and caregiver, with supporting evidence from a standardized, structured questionnaire.

Similarly, the diagnosis of ALSci requires formal neuropsychological assessment. However, various screening tests, sensitive to executive dysfunction, have been employed in order to determine whether a formal neuropsychological evaluation is warranted.

A formal neuropsychological assessment is mandated in the scenario where either the screening or brief assessment suggests pathology. No single testing paradigm will address the full spectrum of deficits observed in the frontotemporal syndromes of ALS.

Miller and others 2009

How is cognitive or behavioral impairment in ALS diagnosed?

  • Cognitive impairment in ALS is best identified through neuropsychological assessment using standardized measures and normative data. The Mini-Mental State Examination is less sensitive to the cognitive impairment seen in ALS and does not examine for behavioral dysfunction.
  • There is no consensus regarding the best screening tests for cognitive impairment in ALS. Two 1-minute word generation tests had 65% sensitivity, 90% specificity, and 88% positive predictive value in detecting possible, probable, or definite FTD by Neary criteria (Class II). A 1-minute letter fluency measure (F words) had 73% sensitivity, 88% specificity, and 79% accuracy to detect ALSci (Class III). An abbreviated neuropsychological battery demonstrated 88% accuracy (Class III).
  • Conclusion
  • Neuropsychological assessment is possibly effective for identifying cognitive impairment in ALS (1 Class II, 1 Class III).
  • Recommendation
    • Screening tests of executive function may be considered to detect cognitive impairment in patients with ALS prior to confirmation with formal neuropsychological evaluation (Level C).

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