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Original Investigation |

Progression in Behavioral Variant Frontotemporal Dementia A Longitudinal Study

Emma Devenney, MRCP1,2,3; Lauren Bartley, BSc1; Chris Hoon, BSc1; Claire O’Callaghan, MD1,3; Fiona Kumfor, PhD1,4,5; Michael Hornberger, PhD1,6; John B. Kwok, PhD1,4; Glenda M. Halliday, PhD1,4; Matthew C. Kiernan, FRACP5; Olivier Piguet, PhD1,4,5; John R. Hodges, MD, FRCP1,4
[+] Author Affiliations
1Neuroscience Research Australia, Sydney, Australia
2Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
3Brain and Mind Research Institute, Sydney, Australia
4School of Medical Sciences, University of New South Wales, Sydney, Australia
5ARC Centre of Excellence in Cognition and Its Disorders, Sydney, Australia
6Department of Clinical Neurosciences, University of Cambridge, Cambridge, England
JAMA Neurol. 2015;72(12):1501-1509. doi:10.1001/jamaneurol.2015.2061.
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Importance  A gap in the literature exists regarding progression in behavioral variant frontotemporal dementia (BVFTD). Guidance is needed concerning markers that will enable clinicians to discriminate FTD more effectively from phenocopies and to identify factors that determine progression and thereby prognosis.

Objectives  To observe longitudinal outcomes and progression in probable and possible BVFTD in accordance with international diagnostic criteria and to identify features that may aid clinicians to prognosticate better in cases of possible BVFTD.

Design, Setting, and Participants  Longitudinal cohort study performed in a specialist tertiary FTD research clinic. Fifty-eight consecutive patients were followed up longitudinally from January 1, 2008, through December 31, 2013, and classified as having possible, probable, or definite BVFTD at presentation and latest review. Final follow-up was completed on December 31, 2013, and data were analyzed from January 1 to August 1, 2014.

Main Outcomes and Measures  Clinical, pathological, genetic, neuropsychological, and neuroimaging data were analyzed to categorize patients, to compare differences between groups with changed and unchanged diagnoses, to determine rates of progression in BVFTD, and to identify prognostic features in possible BVFTD.

Results  At presentation, 38 of the 58 patients fulfilled criteria for probable BVFTD; of these, 36 continued to satisfy probable criteria or underwent conversion to definite criteria over time. The remaining 20 patients satisfied possible criteria only, and 11 of these patients changed categories over time to probable or definite BVFTD and showed progression on cognitive and functional measures (termed changed status). Of these 11 patients, 8 (73%) carried the C9orf72 expansion. A positive family history, memory impairment, and clinical abnormalities at presentation were key features of progression (P < .05). A continuum of neuropsychological scores, progression rates, and atrophy severity emerged across patients in probable, possible, changed status, and nonchanged status groups; patients with probable BVFTD exhibited the most severe abnormalities.

Conclusions and Relevance  Behavioral variant FTD shows variable progression over time. Clinicians can use a detailed neurologic and cognitive assessment to identify key predictive features of progression when faced with possible BVFTD, whereas a diagnosis of probable BVFTD is accurate in a clinical setting.

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Figure 1.
Behavioral Features Across the Spectrum of Behavioral Variant Frontotemporal Dementia (BVFTD)

Behavioral features at presentation in patients with probable and possible BVFTD and patients with changed and unchanged disease status expressed as percentages of the total group.

aP < .05, χ2 test, compared with probable BVFTD.

bP < .05, χ2 test, compared with changed disease status.

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Figure 2.
Patterns of Gray Matter Atrophy Across the Spectrum of Behavioral Variant Frontotemporal Dementia (BVFTD)

Results from voxel-based morphometry analyses demonstrating areas of decreased gray matter density in patients with probable and possible BVFTD and patients with changed and unchanged disease status. Clusters are overlaid on the Montreal Neurological Institute standard brain (FSLDIR/data/standard/MNI152_T1_2mm_Brain; http://fsl.fmrib.ox.ac.uk/fsl/fslwiki/FSLVBM). Colored voxels show regions significant in the analyses (P < .05. corrected for familywise error).

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Figure 3.
Mixed-Model Analyses Showing Progression in Global Cognition and Function Across the Behavioral Variant Frontotemporal Dementia (BVFTD) Spectrum

Results of changes in scores in patients with probable and possible BVFTD and patients with changed and unchanged disease status. Error bars indicate 95% CIs. A and C, Estimated marginal means based on the percentage of change in the Addenbrooke’s Cognitive Examination–Revised (ACE-R) score (range, 0-100; scores >88 indicate normal function) over time. Difference for time and time × diagnosis in probable vs possible BVFTD and changed vs unchanged status, P < .05. B and D, Estimated marginal means based on the change in Functional Rating Scale (FRS) Rasch score (range, −6.66 to 5.39; higher scores indicate higher function) across time. Difference in time and time × diagnosis in probable vs possible BVFTD, P < .001 and P = .26, respectively; changed vs unchanged status, P < .001. P values were calculated using linear mixed effect models.

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