Table 2.
Initial clinical features of the rtvFTD subjects
| Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | |
|---|---|---|---|---|---|
| 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 |
rtvFTD: right temporal variant frontotemporal dementia; bvFTD: behavioural variant frontotemporal dementia; svPPA: semantic variant primary progressive aphasia; MND: motor neuron disease; ADHD: attention deficit hyperactivity disorder; FTD; frontotemporal dementia, CDR; Clinical dementia rating, MMSE; mini-mental state examination, VAT; visual association test, RAVLT; Dutch version of the Rey Auditory Verbal Learning Test, FAB; frontal assessment battery, TMT; trial making test, VOSP; Visual objective and space perception, A; Avarage, LA; low average, N.A.; not available