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Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy

Title
Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy
Authors
Lee, Jin SanJung, Na-YeonJang, Young KyoungKim, Hee JinSeo, Sang WonLee, JuyounKim, Yeo JinLee, Jae-HongKim, Byeong C.Park, Kyung-WonYoon, Soo JinJeong, Jee H.Kim, Sang YunKim, Seung HyunKim, Eun-JooPark, Key-ChungKnopman, David S.Na, Duk L.
Ewha Authors
정지향
SCOPUS Author ID
정지향scopus
Issue Date
2017
Journal Title
JOURNAL OF CLINICAL NEUROLOGY
ISSN
1738-6586JCR Link2005-5013JCR Link
Citation
vol. 13, no. 3, pp. 234 - 242
Keywords
frontotemporal dementiafrontotemporal lobar degenerationmagnetic resonance imagingprognosis
Publisher
KOREAN NEUROLOGICAL ASSOC
Indexed
SCIE; SCOPUS; KCI WOS
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 maybe associated with the earlier appearance of motor symptoms.
DOI
10.3988/jcn.2017.13.3.234
Appears in Collections:
의학전문대학원 > 의학과 > Journal papers
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