Cognitive and Behavioral Symptoms in ALSFTD: Detection, Differentiation, and Progression

Brief screening tools that detect and differentiate patients with amyotrophic lateral sclerosis and frontotemporal dementia (ALSFTD) from those more subtle cognitive or behavioral symptoms (ALS plus) and motor symptoms only (ALS pure) is pertinent in a clinical setting. The utility of 2 validated an...

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Bibliographic Details
Published in:Journal of geriatric psychiatry and neurology 2016-01, Vol.29 (1), p.3-10
Main Authors: Hsieh, Sharpley, Caga, Jashelle, Leslie, Felicity V. C., Shibata, Marlene, Daveson, Naomi, Foxe, David, Ramsey, Eleanor, Lillo, Patricia, Ahmed, Rebekah M., Devenney, Emma, Burrell, James R., Hodges, John R., Kiernan, Matthew C., Mioshi, Eneida
Format: Article
Language:English
Subjects:
Aged
Amyotrophic Lateral Sclerosis - complications
Amyotrophic Lateral Sclerosis - psychology
Behavior - physiology
Behavioral Symptoms - diagnosis
Behavioral Symptoms - psychology
Cognition - physiology
Cognition Disorders - diagnosis
Cognition Disorders - etiology
Disease Progression
Female
Frontotemporal Dementia - complications
Frontotemporal Dementia - psychology
Humans
Male
Middle Aged
Neuropsychological Tests
Psychometrics - instrumentation
Surveys and Questionnaires - standards
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Summary:Brief screening tools that detect and differentiate patients with amyotrophic lateral sclerosis and frontotemporal dementia (ALSFTD) from those more subtle cognitive or behavioral symptoms (ALS plus) and motor symptoms only (ALS pure) is pertinent in a clinical setting. The utility of 2 validated and data-driven tests (Mini-Addenbrooke’s Cognitive Examination [M-ACE] and Motor Neuron Disease Behavioral Scale [MiND-B]) was investigated in 70 ALS patients (24 ALSFTD, 19 ALS plus, and 27 ALS pure). More than 90% of patients with ALSFTD scored at or below the cutoff on the M-ACE, whereas this was seen in only about 20% of ALS patients without dementia. The MiND-B differentiated between ALS pure and ALS plus diagnostic categories. Rasch modeling of M-ACE and MiND-B items revealed early cognitive (fluency, memory recall) and behavioral (apathy) symptoms in ALSFTD. The combined use of the M-ACE and MiND-B detects patients with ALSFTD, differentiates along the ALS continuum, and offers insight into the progression of nonmotor symptomatology in ALSFTD.
ISSN:0891-9887
1552-5708
DOI:10.1177/0891988715598232