Eneida Mioshi, PhDNeuroscience Research Australia

Background in frontotemporal dementia

Impact of FTD in everyday abilities

Impact on carers

Disease progression

General discussion

Frontotemporal dementia

FTD

Behavioural variant Language variants(progressive aphasia)

Semantic

Dementia

Progressive

Nonfluent

Aphasia

Slowly progressive insidious onset

No insight

Alterations in personality and behaviour

Apathy

Disinhibition

Loss of empathy and warmth

Alterations in eating habits and preferences

Strange obsessions and rituals

Poor planning and organisation

Mental rigidity

Decline in self-care

Semantic dementia

(SD)

Naming problems

Word comprehension is

impaired

Loss of semantic

knowledge

Progressive nonfluent

aphasia (PNFA)

Markedly reduced

conversational speech

Difficulties in language

output

Preserved word

knowledge

Memory

Executive function

Visuospatial

Language

General cognition (ACE-R; MMSE)

SD = PNFA < bvFTD

Neurology/

Psychology

Pathology

Genetics

Neuroimaging

Everyday

life

Dis

trib

utio

n o

f patie

nts

0%

20%

40%

60%

80%

100%

PNFA Semantic Dementia bv-FTD AD

Moderate to severe

Severe to very severe impairment

No change

Marginal to mild impairment

Mioshi et al, Neurology, 2007

0%

20%

40%

60%

80%

100%

PNFA Semantic

Dementia

bv-FTD AD

Moderate to severe

Severe to very severe impairment

No change

Marginal to mild impairment

Dis

trib

utio

n o

f patie

nts

Mioshi et al, Neurology, 2007Mioshi et al, Neurology, 2007

BvFTD shows marked ADL disability, more so than AD

Language variants show ADL impairment that cannot be explained only by language deficits

ADL impairment does not correlate with standard cognitive assessments

bv-FTD

phen

bv-FTD

pat

h

SemDem

PNFA

0

20

40

60

80

100baseline

12 m___*

Patient groups

Sco

res

bv-FTD

phen

bv-FTD

pat

h

Sem

Dem

PNFA

0

20

40

60

80

100

___*

___* ___* baseline

12 m

Patient groups

% a

bil

ity

ACE-R DAD

Mioshi et al, Dementia and Geriatric Cognitive Disorders, 2009

n=36

Very mild

Mild

Moderate

Severe

Very severe

Profound

bvFTD: n=28 ; PNFA: n=21 ; SemDem: n=26 Mioshi et al, Neurology, 2010

CDR vs FRS

Disability varies according to dementia

subtype

Cognitive assessment alone does not

reflect patient disability

Dementia staging and progression is

specific to dementia subtypes

All patients and their familieswww.ftdrg.org

Carer burden in FTD is higher than in AD (Boutoleau-Bretonniere et al 2008; Riedijk et al 2006; Mourik et al 2004)

Behavioural changes are correlated with

carer burden in FTD (Boutoleau-Bretonniere et al 2008)

Behavioural change:

(Mioshi et al 2009)

Mioshi et al, Dementia and Geriatric Cognitive Disorders, 2009

Stress and Depression

Mioshi et al, Dementia and Geriatric Cognitive Disorders, 2009

FTD Carer• Patient-based variables and carer-based variables

• DEPRESSION: 58% variance

AD Carer• Patient-based variables

• Carer-based variables

• DEPRESSION and number of high contact roles: 54.4%

Mioshi et al, Dementia and Geriatric Cognitive Disorders, 2009

Motor

Cognitive Behavioural

Functional

disabilityCarer Stress

Mioshi et al, Neurology, 2010 (in press)

Severe

Mioshi et al, Dementia and Geriatric Cognitive Disorders, 2009n=36