Table 2 Initial clinical features of the rtvFTD subjects

Age

58

68

59

59

63

Sex

Male

Male

Male

Male

Female

Handedness

Right

Right

Right

Right

Right

Symptoms

 Prosopagnosia

√

√

√

 Memory deficit

√

√

√

√

√

 Disinhibition

√

√

√

√

 Apathy-inertia

√

√

√

√

√

 Alexithymia

√

√

√

 Bizarre preoccupations

√

√

√

√

√

 Lack of logical reasoning

√

√

√

√

√

 Pathological dwelling on one activity

√

√

√

√

√

 Change of personal taste

√

√

 Nicotine/alcohol abuse

√

 Hyperalgesia

√

√

 Over sleeping during the day

√

√

 Word finding difficulties

√

√

√

√

 Naming difficulties

√

√

 Single word comprehension deficit

√

 Depression

√

√

√

√

√

 Slowness

√

√

√

√

 Motor restless

√

 Hyper-orality

Diagnosis prior to autopsy

FTD + MND

FTD

FTD + atypical parkinsonism

FTD

FTD

Family History

Father had psychiatric symptoms, sister had paranoid disorder

Two brothers and mother had dementia at the age of 70 s with behavioural problems

Mother had psychiatric symptoms and attempt a suicide, uncle (maternal) had dementia at the age of 85

Father and brother had depression, son had ADHD

Mother attempt a suicide

MRI anterior temporal R/L

2/0

3/2

2/0

3/1

4/3

MRI mesial temporal R/L

3/0

4/3

2/1

4/0

3/2

MRI frontal R/L

1/0

1/1

1/0

1/1

1/0

SPECT/PET

Right temporal hypo-perfusion

N.A

Right temporal hypo-perfusion

N.A

N.A

Genetic analysis

N.A

N.A

MAPT (+)

Ser352Leu

N.A

MAPT (negative)

PRGN (negative)

C9orf72 (negative)

CDR

0.5

0.5

0.5

0.5

1

MMSE

27/30

25/30

23/30

22/30

25/30

FAB

N.A

N.A

14/18

18/18

14/18

VAT-A

N.A

6/12

4/12

4/12

7/12

RAVLT delayed recall

N.A

0/15

N.A

12/15

N.A

VAT naming

N.A

N.A

10/12

12/12

6/12

Digit span forward

N.A

N.A

12/16

13/16

8/16

Digit span backward

N.A

N.A

8/16

7/16

8/16

TMT A

N.A

N.A

57’’ (A)

69’’ (A)

49’’ (A)

TMT B

N.A

N.A

169’’ (LA)

166’’ (LA)

102’’ (A)

VOSP-Dot Counting

N.A

N.A

10/10

8/10

9/10