J Clin Neurol.  2017 Jul;13(3):234-242. 10.3988/jcn.2017.13.3.234.

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

• Lee JS
• Jung NY
• Jang YK
• Kim HJ
• Seo SW
• Lee J
• Kim YJ
• Lee JH
• Kim BC
• Park KW
• Yoon SJ
• Jeong JH
• Kim SY
• Kim SH
• Kim EJ
• Park KC
• Knopman DS
• Na DL

• KMID: 2391666
• DOI: http://doi.org/10.3988/jcn.2017.13.3.234

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.

Keyword

frontotemporal dementia; frontotemporal lobar degeneration; magnetic resonance imaging; prognosis

MeSH Terms

Atrophy*
Disease Progression
Frontal Lobe
Frontotemporal Dementia*
Frontotemporal Lobar Degeneration
Humans
Magnetic Resonance Imaging
Mortality
Neurobehavioral Manifestations
Neuropsychological Tests
Parkinson Disease
Prognosis*

Figure

• 

  Fig. 1 Standard images for the magnetic resonance imaging (MRI) visual rating. Standard no. 1 (A), standard no. 2 (B), and standard no. 3 (C). The images in each column represent the following levels: the slice just superior to the lateral ventricle on either side of the brain (left), the slice just superior to the last view of the insular cortex on either side of the brain (middle), and the slice with the most complete view of the orbitofrontal cortex around the interpeduncular cistern (right).

• 

  Fig. 2 Kaplan-Meier plots for groups defined by MRI visual rating subtypes. The end point is predefined as the occurrence of death (| or Δ: cases where death had not occurred by the final follow-up, p=0.048 in log-rank test). D-type: diffuse type, F-type: focal type.

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