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Progression in frontotemporal dementia: identifying a benign behavioral variant by magnetic resonance imaging
Rhys R Davies1, Christopher M Kipps, Joanna Mitchell
1Department of Clinical Neurosciences, University of Cambridge, Addenbrooke's Hospital, Cambridge, England.
Objective:
To assess the clinical course and prognosis in patients with behavioral-variant frontotemporal dementia (FTD) lacking evidence of brain atrophy on magnetic resonance imaging (MRI).
Design:
Patients were enrolled into this prospective cohort study over a period of 15 years; cognitive status, duration of symptoms, and behavioral indexes were recorded. Brain MRIs were rated using a standardized scale.
Setting:
Regional early-onset dementia clinic.
Participants:
Thirty-one participants diagnosed clinically with behavioral-variant FTD. Intervention Rating of MRIs.
Main Outcome Measures:
Death or institutionalization after a minimum of 3 years' follow-up indicated poor prognosis, while the ability to live independently was regarded as a good prognosis for the purpose of survival (Kaplan-Meier) and discriminant function analysis.
Results:
Patients with normal or borderline MRI findings (n = 15) showed significantly longer survival to institutionalization or death than those (n = 16) with definite frontotemporal atrophy (mean +/- SE, 9.3 +/- 1.7 years vs 3.0 +/- 0.7 years; P<.01). Using groups defined by 3-year outcome (good or bad prognosis), cerebral atrophy predicted poor outcome while age, symptom duration, cognitive performance, behavioral impairment, and overall disability at baseline did not.
Conclusions:
Patients with FTD with normal MRI results follow a more benign course than cases with atrophy at presentation. The substrate of the behavioral symptoms in such cases may differ from the neurodegenerative pathological features typically associated with FTD.
Insights
Patients with behavioral-variant frontotemporal dementia (FTD) and normal MRI scans have a better prognosis. Lack of brain atrophy suggests a different underlying cause for FTD symptoms.
Area of Science:
- Neurology
- Neuroimaging
- Dementia Research
Background:
- Behavioral-variant frontotemporal dementia (FTD) is a neurodegenerative disorder.
- Clinical presentation of FTD can be heterogeneous.
- The role of brain atrophy in FTD prognosis is not fully understood.
Purpose of the Study:
- To evaluate the clinical course and prognosis of behavioral-variant FTD patients without evident brain atrophy on MRI.
- To compare outcomes between FTD patients with and without MRI-detected atrophy.
Main Methods:
- Prospective cohort study over 15 years.
- Clinical status, symptom duration, and behavioral assessments were recorded.
- Brain MRIs were systematically rated for atrophy.
Main Results:
- Patients with normal/borderline MRI findings (n=15) had significantly longer survival to institutionalization/death (9.3 years) compared to those with atrophy (n=16, 3.0 years).
- Cerebral atrophy at baseline predicted poor 3-year outcome.
- Age, symptom duration, cognitive, behavioral, and disability measures did not predict outcome.
Conclusions:
- Behavioral-variant FTD patients with normal MRI findings exhibit a more favorable clinical course.
- The underlying pathology in FTD cases with normal MRI may differ from typical neurodegenerative features.
