BioMed Research International / 2013 / Article / Tab 1

Review Article

Functional Status in Behavioral Variant Frontotemporal Dementia: A Systematic Review

Table 1

Summary of clinical studies examining the functional profile of bvFTD patients

Author/yearObjectives SampleCognitive/functionality measures Results

Piquard et al., 2004 [7] Study relationship between functional impairment and performance in planning activities in FTLD and AD patients (11 FTLD and 29 AD)Dementia rating scale (DRS)
Mini-mental state examination (MMSE)
Cognitive difficulties scale (CDS)
Physical self-maintenance scale (ADL)
Instrumental activities of daily living (IADL)
Social activities scale (SADL)
Tower of London
Dysexecutive questionnaire (DEX), the key search, the zoo map, and the six elements from the behavioural assessment of the dysexecutive syndrome (BADS)
No significant differences between bvFTD and AD on ADLs. No relationship between planning and executive function tests with functionality in either patient group
An association was found between CDR and MMSE and functional performance in the two groups

Marra et al., 2007 [11]Compare performance of demented elderly with different levels of severity in questionnaires on basic activities of daily living (BADL) and instrumental activities of daily living (IADL). Determine correlation between the ADL questionnaires applied patients
( , vascular = 7, mixed = 4,
frontotemporal = 2, and
others* = 3)
Clinical dementia rating
Lawton-Brody index
Katz index
Functional activities questionnaire (PFAQ)
IADLs were more impaired at early stages of dementia and BADLs at more advanced stages

Mioshi et al., 2007 [9]Assess ADLs and cognitive performance in subtypes of frontotemporal dementia, bvFTD, nonfluent progressive aphasia (NFPA), semantic dementia (SD)
Ascertain the relationship between functional deficits and cognitive dysfunction of patients and compare against patients with AD
patients with dementia (bvFTD = 15, NFPA = 10, SD 15, and AD = 19)Disability Assessment for dementia (DAD)
Addenbrooke’s cognitive examination-revised (ACE-R)
Clinical dementia rating scale (CDR)
For functional performance, bvFTD patients were most impaired (56% with functionality preserved), whereas NFPA and SD patients were less impaired (83% and 85% were functionally preserved). AD subjects had intermediate performance (76% showed preserved functionality). On ACE-R, the NFPA and SD groups had worse performance than bvFTD and AD patients. DAD performance did not correlate with cognitive measures, CDR, or time diagnosed with the disease

Razani et al., 2007a [8]Correlate performance on executive function tasks with functional performance patients with dementia (3 FTLD, 21 AD, and 10 VD) and 35 healthy controlsWisconsin card sorting test (WCST)
Verbal fluency (FAS)
Modified version of the Lawton-Brody measure (IADL)
Direct assessment of functional status (DAFS)
Significant correlation between verbal fluency and DAFS. Moderate correlation between WCST and performance on subjective functionality scale

Razani et al., 2007b [17]Assess the relationship between performance on ADLs in patients with mild dementia and the relationship with emotional burden of caregivers patients with dementia (5 FTLD, 23 AD, 5 VD, and 1 with undiagnosed form of dementia)Direct assessment of functional status (DAFS)
Lawton-Brody measure (IADL)
Caregiver burden inventory (CBI)
Brief symptom inventory (BSI)
Significant correlations between DAFS, caregiver burden (CBI), and psychological problems (BSI) On DAFS, impaired orientation, communication, finances, and transport were associated with greater burden and hostility in caregivers Impaired financial ability was strongest predictor of time dependent on caregivers, whereas impairment on transport domain predicted time since clinical diagnosis

Wicklund et al., 2007 [12]Compare functional status in FTLD and AD with AD, 57 with bvFTD, and 61 with PPAInstrumental activities of daily living (IADL)Functional ability was moderately impaired in AD and FTD and mildly impaired in PPA For all groups, more complex ADLs were impaired early on, with relative preservation of self-care activities. The communication score was the least impaired next to self-care for FTD and AD and the most impaired for PPA patients

Bressan et al., 2007 [18]Compare information from caregiver against direct assessment of patient functional performance patients with dementia, comprising 1 FTLD, 10 AD, 5 VD, 3 alcoholic dementia, 3 D. with Lewy Bodies, 1 corticobasal, and 2 dementias due to severe hypoxiaPfeffer functional activities questionnaire (PFAQ)
Observation of patients during simulated activities included in the PFAQ
Significant differences found between caregiver responses and direct observation of patient performance. Caregivers underestimated functional capacity of patients

Carvalho et al., 2008 [13]Compare functional communication abilities in FTLD and AD patients. patients with AD and 8 with FTLDFunctional assessment of communication skills (ASHA-FACS)Functional communication abilities were similar for patients with AD and FTLD. FTLD patients had worse performance on inference comprehension while AD patients had worse performance on basic transactions with money

Bahia et al., 2008 [14]Verify utility of a functional and behavioral inventory in differential diagnosis between FTLD and AD patients with AD and 12 with FTLDFrontal behavioral inventory (FBI)
Disability assessment for dementia (DAD)
FIB proved accurate for differential diagnosis between FTLD and AD
DAD failed to differentiate groups but helped assess severity of the condition

Mioshi and Hodges, 2009a [15]Examine changes in activities of daily living (ADL), including subcomponents of initiation, planning, or execution of DAD patients with bvFTDphen, 6 with bvFTD path, 11 with SemDem, and 9 PNFAAddenbrooke’s cognitive examination-revised (ACE-R)
Disability assessment for dementia (DAD)
Pathological bvFTD, SemDem, and PNFA groups showed significant decline in ADLs after 12 months, while the phenocopy subgroup did not Each variant showed different profiles of decline in subcomponents of ADLs

Mioshi et al., 2009b [19]Describe activities of daily living in behavioral variant frontotemporal dementia, and correlate in caregiver and performance-based assessments patients with bvFDTAddenbrooke’s cognitive examination-revised (ACE-R)
Disability assessment for dementia (DAD)
Frontal systems behavior scale (frontal dysfunction)
Assessment of motor and process skills
A model combining the global cognition and frontal dysfunction explained the variance on ADL performance. A qualitative rating distinguished between pathologic and phenocopy patients better than the performance-based assessment

Kipps et al., 2009 [16] Compare emotion recognition behavior and social functioning in AD and bvFTD patients , 14 patients with bvFTD and 14 with ADEmotion hexagon
Disability assessment for dementia (DAD)
The Cambridge behavioural inventory (CBI)
bvFTD group had worse functional deficit, higher scores on the CBI, and poorer emotion recognition compared with the AD group Recognition of emotions was not correlated with DAD but instead with apathy on the CBI

Mioshi et al., 2010 [10]Develop a novel tool to characterize FTLD severity based on functional disability and behavioral changes. Assess rate of change over time in 3 FTLD variants (bvFTD, SD, and NFPA) patients, 28 bvFTD, 21 NFPA, 26 SD, and 20 controlsAddenbrooke’s cognitive examination-revised (ACE-R)
Clinical dementia rating (CDR)
Frontotemporal dementia rating scale (FRS)
FTLD progression differed among variants bvFTD patients progressed rapidly whereas subjects with SD presented slower evolution FDRS proved a good scale for staging and determining FTLD progression

Josephs et al., 2011 [2]Relate behavioral, neuropsychological, and sociodemographic factors to determine predictive factors for functional decline in bvFTD patients with bvFTDClinical dementia rating (CDR)
Trail making parts A and B
Boston naming test
Category fluency (sum of animals, vegetables, and fruits)
Mini-mental status examination (MMSE)
Neuropsychiatric inventory (NPI)
Wechsler adults intelligence scale (WAIS-R)
Magnetic resonance imaging analysis generating an anatomical subtype
Predominantly frontotemporal and frontal atrophy, worse performance on executive, visuospatial and language functions, loss of inhibition, agitation/aggression and nighttime behaviors, and greater age at disease onset were associated with faster rate of functional decline

Notes. FTLD: frontotemporal lobar degeneration, bvFTD: behavioral variant frontotemporal dementia, NFPA: nonfluent progressive aphasia, SD: semantic dementia, VD: vascular dementia, and AD: dementia of the Alzheimer type.
*Dementias caused by traumatic brain injury or by normal pressure hydrocephalus.

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