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Table 1 Reclassification of the reported molecular neuropathologies

From: Right temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review

 

Publications

N

Reported molecular neuropathology

Adapted diagnosis

1

[52]

1

FTLD-TDP type C + CTD

FTLD-TDP type C + CTD

2

[69]

1

FTLD-tau-PSP + TDP type A

FTLD-tau-PSP + TDP type A

3

[54]

9

FTLD-tau-PiD (n = 1)

FTLD-TDP type C (n = 8)

FTLD-tau-PiD (n = 1)

FTLD-TDP type C (n = 8)

4

[61]

1

FTLD-TDP type C + tau-PSP

FTLD-TDP type C + tau-PSP

5

[64]

1

FTLD-TDP type A

FTLD-TDP type A

6

[63]

1

FTLD-TDP type A

FTLD-TDP type A

7

[70]

1

FTLD-tau-PiD

FTLD-tau-PiD

8

[67]

1

TDP-43 pathology in all cortical layers. NCI, with crescentic, round, skein-like and granular types. Short threads accompanied the NCI. Due to the admixture of neuronal cytoplasmic inclusion subtypes seen in FTLD-TDP type A and type B, presence of type A threads, but involvement of all cortical layers (type B), the pattern of TDP-43 inclusions is unclassifiable. Skein-like inclusions in lower motor neurons, producing the neuropathological diagnosis of motor neuron disease. Thal amyloid plaque stage 4, Braak 1. 4R-only atypical tauopathy

FTLD-TDP type A-B + AD + 4R tau

9

[55]

1

FTLD-tau-GGT

FTLD-tau-GGT

10

[38]

7

FTLD-TDP type C (n = 1)

FTLD-TDP type C + CTD (n = 6)

FTLD-TDP type C (n = 1)

FTLD-TDP type C + CTD (n = 6)

11

[56]

2

FTLD-TDP Mackenzie type 3 + MND (n = 1)

TDP Mackenzie type 3 + MND + AD (n = 1)

FTLD TDP type B + MND*

FTLD TDP type B + MND* + AD

12

[66]

1

FTLD-FUS

FTLD-FUS

13

[68]

1

FTLD-TDP Mackenzie type 3 + MND

FTLD-TDP type B + MND*

14

[60]

1

FTLD-TDP-43 pathology with NII

FTLD-TDP type A-B

15

[62]

1

FTLD-TDP Cairns type 2 + MND

FTLD-TDP type B + MND*

16

[65]

1

Immunohistochemistry using antibodies to ubiquitin showed NCIs, some of these inclusions were also immunoreactive for phosphorylated TDP-43 antibodies. We identified no DN, but a few NCI, which were positive for both ubiquitin and phosphorylated TDP-43

FTLD-TDP type A-B + MND*

17

[4]

1

Mixed Alzheimer and cortical Lewy body disease (n = 1)

AD + DLB (n = 1)

18

[38]

8

FTLD-tau-PiD (n = 1)

FTLD-tau-MAPT (n = 7)

FTLD-tau-PiD (n = 1)

FTLD-tau-MAPT (n = 7)

19

[59]

1

TDP-43 pathology with NII

FTLD-TDP type A-B

20

[71]

1

Tau-negative, TDP-43-positive neuronal cytoplasmic inclusions and dystrophic neurites were found. Numerous NFTs and senile plaques with amyloid angiopathy indicated advanced Alzheimer disease

FTLD-TDP type A-B + AD

21

[58]

1

TDP43 pathology with NII + MND

FTLD-TDP type A-B + MND*

  1. TDP: TAR DNA-binding protein 43; TAU: tau protein; MND: motor neuron disease; MAPT: microtubule associated protein; FUS: fused in sarcoma protein; PiD: Pick’s disease; PSP: progressive supranuclear palsy; FTLD-U: frontotemporal lobar degeneration with tau-negative, ubiquitin-immunoreactive pathology; AD: Alzheimer’s disease; DLB: dementia with Lewy bodies; NII: neuronal cytoplasmic and intranuclear inclusions
  2. *: Clinically diagnosed with MND