Table 2.

NPI mean (standard deviation, StDev) scores and percentage of patients exhibiting abnormal behaviour in all PPA patients and in the PPA subgroups. Behaviours exhibited by ≥50% of patients in each subgroup are indicated in bold. The NPI score is based on use of discrete scales: for each behaviour, the score (individual behaviours/12, total/144) shown is the mean product of individual scores on scales of severity [1, mild – 3, severe] × frequency [1, occasionally – 4, very frequently]; for severity of caregiver distress [total scores/60 only shown], 0, no distress – 5, extremely distressing.

	ALL		SD		PNFA		LPA		GRN-PPA
	Mean (StDev)	%	Mean (StDev)	%	Mean (StDev)	%	Mean (StDev)	%	Mean (StDev)	%
Delusions	0.4 (1.3)	9	0.4 (1.3)	11	0.4 (1.6)	7	0.4 (1.1)	14	0.0 (0.0)	0
Hallucinations	0.1 (0.4)	6	0.2 (0.7)	11	0.0 (0.0)	0	0.1 (0.4)	14	0.0 (0.0)	0
Agitation/aggression	0.9 (1.4)	50	0.7 (1.0)	44	0.9 (1.2)	50	1.4 (2.1)	57	0.3 (0.6)	33
Depression/dysphoria	1.3 (1.8)	56	1.1 (0.9)	78	1.6 (2.2)	57	0.4 (0.8)	29	2.0 (3.5)	33
Anxiety	1.2 (1.8)	50	0.8 (0.8)	56	0.8 (1.6)	36	2.6 (2.6)	71	0.7 (1.2)	33
Elation/euphoria	0.6 (1.6)	19	0.8 (2.0)	22	0.5 (1.6)	14	0.1 (0.4)	14	1.3 (2.3)	33
Apathy/indifference	1.7 (2.4)	56	0.7 (1.1)	33	2.1 (2.9)	64	2.0 (2.8)	57	1.7 (1.5)	67
Disinhibition	1.3 (2.5)	38	2.0 (2.9)	67	0.7 (2.4)	14	1.7 (2.4)	43	0.7 (1.2)	33
Irritability/lability	1.4 (1.9)	56	1.2 (1.4)	78	0.9 (2.1)	29	2.4 (2.3)	71	1.3 (1.2)	67
Aberrant motor behaviour	0.7 (1.6)	22	0.3 (0.7)	22	0.5 (1.2)	21	1.7 (2.9)	29	0.0 (0.0)	0
Abnormal sleep	0.8 (1.6)	25	1.1 (1.5)	44	0.8 (1.6)	21	0.9 (2.3)	14	0.0 (0.0)	0
Abnormal appetite/eating disorders	2.0 (3.1)	50	1.7 (1.6)	67	2.6 (4.3)	43	1.4 (2.3)	43	1.3 (2.3)	33
Total	12.2 (12.4)	88	11.0 (7.4)	100	11.9 (14.8)	79	15.3 (15.3)	86	9.3 (8.1)	67
Caregiver distress total	9.2 (6.0)	88	9.4 (6.1)	100	7.4 (6.0)	79	9.6 (6.8)	86	5.3 (4.60	67