. Author manuscript; available in PMC: 2017 Jun 1.

Published in final edited form as: JAMA Neurol. 2016 Jun 1;73(6):733–742. doi: 10.1001/jamaneurol.2016.0412

Table 1.

Demographic and cognitive data in nfvPPA-PSP, nfvPPA-CBD, and controls at initial visit.

Demographic Data	All 4R tau	PSP (n=5)	CBD (n=9)	control (n=10)

Gender (M/F)	4/10	1/4	3/6	3/7
Handedness (R/L)	13/1	4/1	9/0	10/0
Education, y	17 (12–21)	16.4 ± 3.9	18 (12–20)	17 (14–20)
Age at symptom onset, y	62.5 (51–79)	15 (12–21)	61 (51–79)	n/a
Age at initial evaluation, y	66.5 (54–81)	70 (62–72)	65.3 ± 9.1	71.5 (57–78)
Survival, y	7.23 (4.4–11.6)	*9.6 (6.4–11.6)*^c	*6.4 (4.4–10.3)*^c	n/a

General Cognitive Data

MMSE	27 (20–30)^a	28 (24–30)^a	27 (20–29)^a	30 (28–30)
CDR sum of boxes	2 (0–4.5)	1.5 (0–2.5)	2 (1–4.5)	n/a
GDS total	5 (0–28)^a	19 (3–28)^a ^b	4 (0–16)	3.5 (0–13)
NPI total	10.5 (1–50)	16.5 (8–50)	10.5 (1–38)	n/a
Digits Backward	3 (2–6)^a	3 (2–6)	3 (2–4)^a	5 (3–7)
Modified trails (lines per min)	9.3 (0.5–32.3)^a	2 (0.5–32.3)^a	10.1 (4–26.3)^a	30 (14–40)
Calculation	5 (2–5)	5 (2–5)	5 (3–5)	5 (4–5)
Benson figure copy	15 (13–17)	15 (13–16)	15 (13–17)	16 (13–17)
Benson figure recall	11.5 (3–17)	10 (3–13)	12 (9–17)	14 (7–17)
CVLT-MS Total recall	25 (16–34)^a	26 (16–28)^a	23.5 (17–34)^a	32 (26–35)
CVLT-MS 10min free recall	6 (4–8)^a	7 (4–8)^a	6 (5–8)^a	8.5 (5–9)

Language Cognitive test

AOS rating (MSE, 7)	2 (1–4)	1 (1–4)	2 (1–4)	n/a
Dysarthria rating (MSE, 7)	2 (0–7)	4 (2–7)^b	0 (0–4)^b	n/a
Speech fluency (WAB, 10)	9 (4–10)	9 (6–9)	9 (4–10)	n/a
Information content (WAB, 10)	10 (5–10)	10 (5–10)	10 (9–10)	n/a
Sequential commands (WAB, 80)	73.5 (49–80)^a	*80 (69–80)*^c	*68 (49–80)*^a ^c	80 (76–80)
Grammar comprehension (%)	81 (65–100)^a	*98 (80–100)*^c	*81 (65–98)*^a ^c	100 (92–100)
Repetition (WAB, 100)	91.5 (52–100)^a	95 (52–100)^a	88 (64–100)^a	100 (96–100)
Word recognition (WAB, 60)	60 (55–60)	60 (59–60)	60 (55–60)	60 (60–60)
Boston Naming Test (BNT, 15)	13.5 (11–15)^a	12 (11–15)^a	14 (11–15)^a	15 (14–15)
Phonemic fluency (D words)	4.5 (0–13)^a	5 (2–13)^a	4 (0–6)^a	17 (14–24)
Semantic fluency (animals)	9 (4–22)^a	9 (6–22)^a	9 (4–13)^a	26 (14–33)

Spontaneous Speech sample analysis (Picnic scene)

Total narrative words	66.5 (9–452)^a	69 (9–452)	64 (14–131)^a	140 (89–238)
Words per minute	55.5 (11–90.5)^a	65.9 (18.8–90.5)^a	54.8 (10.7–70.4)^a	154.7 (112–198)
Proportion of syntactic errors	4 (0–35)^a	3.3 (0–11.11)^a	4.8 (0–35)^a	0 (0–1.02)
Proportion of words in sentences	0.91 (0–1)^a	0.9 (0.6–1)^a	0.83 (0–1)^a	1 (0.88–1)
Proportion of distortions (per 100wrds)	6.3 (0–33.3)^a	10.7 (1.5–33.3)^a	4 (0–31.25)^a	0 (0–1.33)

p< 0.05 vs controls;

p< 0.05 PSP vs CBD;

Italicized= trend p≤0.10 PSP vs CBD. Kruskal-Wallis and post-hoc Mann-Whitney U tests performed. MMSE: minimental state examination. CDR: clinical dementia rating scale. GDS: geriatric depression scale. NPI: neuropsychiatric inventory. CVLT: California verbal learning test. AOS: apraxia of speech.