Table 4 Systematic review of descriptions of MAPT R406W patients

[10]

10

7 M, 3 F

54

25

N/R

N/R

7

1

1

1

Aggression 1, Poor judgment and drifting 1

Alcoholism 2

BK, rigidity^c, action T and mild rest T (arms) 1

No vertical gaze, hypometric saccades, PR 1

[19, 33, 45]

5

1 M, 4 F

57

13

N/R

ε3/ε4 1

3

3

“Mostly intact” 3

Para, pers, flu, mutism 2

N/R

N/R

N/R

N/R

[11]

6^d

N/R

58

11

EOAD 4, MCI-EOAD 1

ε3/ε3 3, ε3/ε4 2

4

1

5

5

Withdrawal and adapting difficulties 1, Dis 1

Depr and anxiety 1, emotional shallowness 2

Rigidity, cogwheeling 4, T 3, BK 1, gait disturbance 1, total Parkinsonism 4

Dysphagia 1, PR 1

[42, 47]

5

3 M, 2 F

56

23

N/R

N/R

5

4

Normal

Word comprehension 3, stereotypies 3, flu 3, mutism 1

Dietary change 4, dis 2, stereotypies 2, childish 2

Restlessness 3, depr 1, anxiety 1, suspiciousness 2, emotional shallowness 4

No

N/R

[4]

3

0 M, 3 F

65

N/R

AD 2, MCI 1

ε3/ε2 1, ε3/ε3 1, ε3/ε4 1

3

N/R

N/R

N/R

2

N/R

N/R

N/R

[14, 48]

8^ff

5 M, 2 F

^e

^e

AD 1

N/R

N/R

1^f

N/R

Anomia 2^f, para 1, comp, flu phonemic and semantic errors impaired word finding, poor spelling and aphasia 1^e

Apathy 1^f

N/R

1^f

N/R

[3]

1

0 M, 1 F

56

N/R

N/R

N/R

1

1

1

N/R

Dis and apathy

N/R

Rest T (R), postural T (bilat), mild BK (L < R), mild rigidity (L < R), and mild dystonia

N/R

[49]

1

1 M, 0 F

50

N/R

N/R

ε3/ε3

1

N/R

N/R

N/R

N/R

N/R

N/R

N/R

[44]

1

1 M, 0 F

66

N/R

N/R

N/R

1

N/R

N/R

N/R

N/R

N/R

N/R

No

[35]

1

0 M, 1 F

54

13

Dementia NOS

ε3/ε3

N/R

N/R

N/R

N/R

N/R

N/R

N/R

N/R

[43, 50, 51]

6

2 M, 4 F

52.5

N/R

EOAD 2, familial AD 1

ε3/ε2 2, ε3/ε3 2, ε3/ε4 2

6

1

N/R

N/R

Loss of interest 1, dis 2, mild 1

Disorientation 1

No 6

N/R

[46]

1

1 M, 0 F

47

6

Schizophrenia, primary degenerative dementia

ε3/ε3

N/R

No

N/R

N/R

Self-neglect

Pdel diminished appetite and severe confusion

N/R

N/R

[6]

1

1 M, 0 F

59

14

AD

N/R

1

No

N/R

Sensory aphasia

Violent, angry, aimless walking, stereotypic

Apraxia, delusional mistakes

No

N/R

[12]

2

1 M, 1 F

56.5

N/R

MCI 1, AD 1

N/R

2

N/R

N/R

N/R

N/R

N/R

N/R

N/R

[52]

6

N/R

57.5

11,5

AD 2, dementia NOS 1

N/R

6

N/R

N/R

5

5

N/R

N/R

N/R

[13]

5^f

N/R

60–70^e

N/R

AD 2

ε3/ε3 1^f

N/R

1^f

Prosopagnosia 1^f

Word finding, comprehension and speech output 1^f

Dietary change, argumentative, and dis 1^f

Depr^g, Pdel, hyperreligiosity, compulsory routines, hoarding & Irritability 1^f

Masked faces and mild BK 1^f, total Parkinsonism: 4^f

Slow saccades and square wave jerks^f

[17]

3

1 M, 2 F

54

13

N/R

N/R

N/R

2

Prosopagnosia 2

Dysnomia 2, repetitive 1

Dietary change 2, stereotypies 1

N/R

No

N/R

[53]

1

1 M, 0 F

38

26

BvFTD

N/R

N/R

N/R

N/R

N/R

N/R

Obsessive, hoarding

N/R

N/R

Summary

66^d,fff

25 M, 23 F

55 (IQR 51.25–59.75)

13 (IQR 11–21)

AD 17, any 23

Any ε4 5

39

13^h

6

20^h

26^h

13

9^h

3