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KMID : 1130620170130030234
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Journal of Clinical Neurology
2017 Volume.13 No. 3 p.234 ~ p.242
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Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy
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Lee Jin-San
Jung Na-Yeon
Jang Young-Kyoung
Kim Hee-Jin
Seo Sang-Won
Lee Ju-Youn
Kim Yeo-Jin
Lee Jae-Hong
Kim Byeong-C.
Park Kyung-Won
Yoon Soo-Jin
Jeong Jee-H.
Kim Sang-Yun
Kim Seung-Hyun
Kim Eun-Joo
Park Key-Chung
Knopman David S.
Na Duk-L.
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Abstract
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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.
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KEYWORD
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frontotemporal dementia, frontotemporal lobar degeneration, magnetic resonance imaging, prognosis
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