Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study

Table 1

Summary of clinical, language/cognitive, MRI, and neuropathological data. Regarding language deficits, “yes” means marked impairment (moderate or severe), and “mild” stands for very discrete impairments. This information served to classify our cases into one of three PPA variants outlined in the current diagnostic criteria [1].

(a)

Case	PPA subtype	Demographic characteristics			Motor symptoms
		Gender	Age at onset (years)	Disease duration (years)	Parkinsonism	Supranuclear; gaze palsy
1	nfvPPA	M	78	8	Yes	Yes
2	nfvPPA	F	68	8	Yes	Yes
3	nfvPPA	M	76	7	Yes	Yes
4	nfvPPA	F	74	9	Yes	No
5	nfvPPA	M	61	7	Yes	No
6	Progressive dysarthria, later nfvPPA	M	74	7	Yes	No
7	Mixed (nfvPPA+svPPA)	M	69	6	Yes	Yes
8	svPPA	F	68	20	Yes	Yes
9	lvPPA	F	48	6	No	No

(b)

Case	PPA subtype	Language features + speech errors
		Syntax deficits, agrammatism	Low syntactic comprehension	Anomia	Phonemic errors, paraphasia	Repetition (words, sentences)	Apraxia; of speech
1	nfvPPA	Yes	Yes	Yes	Yes	Yes	No
2	nfvPPA	Mild	N/A	Mild	N/A	N/A	N/A
3	nfvPPA	Yes	Yes	Yes	Yes	Yes	Yes
4	nfvPPA	Yes	Yes	Yes	Yes	Yes	No
5	nfvPPA	Yes	Yes	Yes	Yes	No	Yes
6	Progressive dysarthria, later nfvPPA	Yes	Mild impairment	Yes	Yes	Yes	Yes
7	Mixed (nfvPPA+svPPA)	Yes, late mutism	Yes	Yes	Yes	Yes	Yes
8	svPPA	Yes	Yes	Yes	Yes	Yes	Yes
9	lvPPA	Fluent speech	Yes	Yes	Yes	Yes	No

(c)

Case	PPA subtype	Nonlanguage domains				BPSD
		Memory	Attention	Executive; function	Visuospatial
1	nfvPPA	Severe	Severe	Severe	Severe	Apathy, irritability
2	nfvPPA	Severe	Mild	Mild	Moderate	Anxiety, restlessness
3	nfvPPA	Mild	Normal	Mild	Normal	Fluctuating emotional control
4	nfvPPA	Severe	Severe	Severe	Severe	Disinhibition, agitation
5	nfvPPA	Severe	Severe	Severe	Severe	Depression, agitation, anxiety, apathy, disinhibition, irritability
6	Progressive dysarthria, later nfvPPA	Severe	Severe	Severe	Moderate	Depression and negativism
7	Mixed (nfvPPA+svPPA)	Mild	Normal	Moderate	Normal	Apathy, irritability, compulsivity
8	svPPA	N/A	Severe	Severe	Mild	Social disinhibition, negativism, agitation, compulsive behavior
9	lvPPA	Severe	Moderate	Severe	Moderate	Irritability, disinhibition, agitation, compulsivity, negativism

(d)

Case	PPA subtype	MRI				Neuropathology
		Focal atrophy	MTA	PA	Fazekas
1	nfvPPA	Left temporal, bilateral parietal	2	2	1	AD+DLB
2	nfvPPA	Left temporal, bilateral parietal	3	1	2	AD+DLB
3	nfvPPA	Left temporal, bilateral parietal, midbrain	3	2	3	AD+PSP+GGT+ARTAG
4	nfvPPA	Symmetric parietal,right temporal	1	2	0	AD+LATE
5	nfvPPA	Almost symmetric parietal and temporal	2	3	1	AD+FTLD-TDP type A
6	Progressive dysarthria, later nfvPPA	Left temporal lobe and hippocampus, symmetric parietal, and frontal	2	1	3	AD
7	Mixed (nfvPPA+svPPA)	Left temporal lobe, midbrain	3	1	3	GGT+LATE
8	svPPA	Symmetric temporal, left parietal, midbrain	3	3	0	AD+PSP+GGT+ARTAG+LATE
9	lvPPA	Left temporal lobe	1	1	1	AD+DLB