Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy.
10.3988/jcn.2017.13.3.234
- Author:
Jin San LEE
1
;
Na Yeon JUNG
;
Young Kyoung JANG
;
Hee Jin KIM
;
Sang Won SEO
;
Juyoun LEE
;
Yeo Jin KIM
;
Jae Hong LEE
;
Byeong C KIM
;
Kyung Won PARK
;
Soo Jin YOON
;
Jee H JEONG
;
Sang Yun KIM
;
Seung Hyun KIM
;
Eun Joo KIM
;
Key Chung PARK
;
David S KNOPMAN
;
Duk L NA
Author Information
1. Department of Neurology, Kyung Hee University Hospital, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
frontotemporal dementia;
frontotemporal lobar degeneration;
magnetic resonance imaging;
prognosis
- MeSH:
Atrophy*;
Disease Progression;
Frontal Lobe;
Frontotemporal Dementia*;
Frontotemporal Lobar Degeneration;
Humans;
Magnetic Resonance Imaging;
Mortality;
Neurobehavioral Manifestations;
Neuropsychological Tests;
Parkinson Disease;
Prognosis*
- From:Journal of Clinical Neurology
2017;13(3):234-242
- CountryRepublic of Korea
- Language:English
-
Abstract:
BACKGROUND AND PURPOSE: Only a few studies have investigated the relationship between different subtypes and disease progression or prognosis in patients with behavioral variant frontotemporal dementia (bvFTD). Since a localized injury often produces more focal signs than a diffuse injury, we hypothesized that the clinical characteristics differ between patients with bvFTD who show diffuse frontal lobe atrophy (D-type) on axial magnetic resonance imaging (MRI) scans versus those with focal or circumscribed frontal lobe atrophy (F-type). METHODS: In total, 94 MRI scans (74 scans from bvFTD and 20 scans from age-matched normal controls) were classified into 35 D- and 39 F-type bvFTD cases based on an axial MRI visual rating scale. We compared baseline clinical characteristics, progression in motor and cognitive symptoms, and survival times between D- and F-types. Survival analyses were performed for 62 of the 74 patients. RESULTS: While D-type performed better on neuropsychological tests than F-type at baseline, D-type had higher baseline scores on the Unified Parkinson's Disease Rating Scale (UPDRS) Part III. Evaluations of motor progression showed that the disease duration with motor symptoms was shorter in D-type than F-type. Moreover, the survival time was shorter in D-type (6.9 years) than F-type (9.4 years). Cox regression analyses revealed that a high UPDRS Part III score at baseline contributed to an increased risk of mortality, regardless of the pattern of atrophy. CONCLUSIONS: The prognosis is worse for D-type than for those with F-type. Shorter survival in D-type may be associated with the earlier appearance of motor symptoms.
