Prognosis of patients with behavioral variant frontotemporal dementia who have focal versus diffuse frontal atrophy

Jin San Lee, 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

Research output: Contribution to journalArticle

2 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)234-242
Number of pages9
JournalJournal of Clinical Neurology (Korea)
Volume13
Issue number3
DOIs
StatePublished - 2017

Keywords

  • Frontotemporal dementia
  • Frontotemporal lobar degeneration
  • Magnetic resonance imaging
  • Prognosis

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Lee, J. S., Jung, N. Y., Jang, Y. K., Kim, H. J., Seo, S. W., Lee, J., Kim, Y. J., Lee, J. H., Kim, B. C., Park, K. W., Yoon, S. J., Jeong, J. H., Kim, S. Y., Kim, S. H., Kim, E. J., Park, K. C., Knopman, D. S., & Na, D. L. (2017). Prognosis of patients with behavioral variant frontotemporal dementia who have focal versus diffuse frontal atrophy. Journal of Clinical Neurology (Korea), 13(3), 234-242. https://doi.org/10.3988/jcn.2017.13.3.234