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

Sharpley Hsieh; Jashelle Caga; Felicity V C Leslie; Marlene Shibata; Naomi Daveson; David Foxe; Eleanor Ramsey; Patricia Lillo; Rebekah M Ahmed; Emma Devenney; James R Burrell; John R Hodges; Matthew C Kiernan; Eneida Mioshi

doi:10.1177/0891988715598232

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

J Geriatr Psychiatry Neurol. 2016 Jan;29(1):3-10. doi: 10.1177/0891988715598232. Epub 2015 Aug 6.

Authors

Affiliations

¹ Brain and Mind Research Institute, Sydney, New South Wales, Australia Neuroscience Research Australia, Sydney, New South Wales, Australia ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, New South Wales, Australia sharpley.hsieh@sydney.edu.au.
² Brain and Mind Research Institute, Sydney, New South Wales, Australia.
³ Neuroscience Research Australia, Sydney, New South Wales, Australia.
⁴ Departamento de Neurología Sur, Facultad de Medicina, Universidad de Chile, Santiago, Chile.
⁵ Brain and Mind Research Institute, Sydney, New South Wales, Australia Neuroscience Research Australia, Sydney, New South Wales, Australia Prince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
⁶ Neuroscience Research Australia, Sydney, New South Wales, Australia Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.
⁷ Neuroscience Research Australia, Sydney, New South Wales, Australia ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, New South Wales, Australia.
⁸ Neuroscience Research Australia, Sydney, New South Wales, Australia Department of Psychiatry, Cambridge University, Cambridge, United Kingdom.

PMID: 26251110
DOI: 10.1177/0891988715598232

Abstract

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.

Keywords: amyotrophic lateral sclerosis; behavioral disturbance; cognitive screening; frontotemporal dementia.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

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*

Supplementary concepts

Frontotemporal Dementia With Motor Neuron Disease