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Emotion perception, but not affect perception, is impaired with semantic

memory loss

Supplementary Online Materials

Additional Patient Data

Patients were tested in the neurology clinic at a local Boston hospital. We

report the neuropsychological tests that were administered as part of a battery

performed by a trained neuropsychologist.

Case 1

Case history and presentation

EG is a 70-year-old male high school graduate who worked for most of his

adult life as an engineer at a construction company. He presented in 2006 with

gradually progressive symptoms of difficulty with “memory” and word finding in

speech. At the time of presentation, his family reported that he was having difficulty

remembering words and had stopped reading books. He was still doing the family

finances without difficulty. His family did not report changes in mood or personality.

On his first evaluation in 2006, he demonstrated word-finding difficulties and

anomia but normal affect and comportment. Structural MRI demonstrated focal

atrophy of the left temporal pole and he was diagnosed with Primary Progressive

Aphasia-Semantic variant (PPA-S; Gorno-Tempini et al., 2011a) of semantic

dementia. Findings from a full neuropsychological battery performed in 2006 are as

follows.

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Intellectual abilities. This patient had normal intellectual abilities. He had

an average estimated intelligence score on the Weschler Test of Adult Reading and

performed superiorly on the Raven’s Progressive Matrices.

Attention and Executive function. This patient had normal attention and

executive function. Performance was in the average range on an auditory attention

span test and on the Mental Control subtest of the Weschler Memory Scale III. He

was in the superior range for Part A of the Trail Making Test (19 sec). He had one

sequencing error and one set-shifting error on Part B of the Trail Making Test, but

time to completion was 73 sec.

Memory. This patient had normal recognition memory, but consistent with

the semantic dementia diagnosis, had poor performance on the word recall portion

of the Consortium to Establish a Registry for Alzheimer’s Disease memory task.

Visuospatial performance. This patient had normal visuospatial skills. He

performed well with no omissions on the letter cancellation task, had intact

performance on the Visual Organization Test, and performed flawlessly on the

Benton Visual Form Discrimination Task.

Language. Consistent with the diagnosis of semantic dementia, this patient

had impaired performance on language tests. Lexical verbal fluency was impaired

(total FAS = 21), as was semantic verbal fluency (animals = 11). Performance on the

Boston Naming Task was also impaired (32/60 spontaneously plus 4 additional

items with phonemic cues).

Profile at time of emotion testing

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By 2008, when EG completed the present experimental tasks, he had

undergone progressive impairment in the domain of language and semantic

memory. He was exhibiting anomia and semantic memory impairment with fluent

and articulate speech. Performance on confrontational naming (8/15 on

abbreviated Boston Naming Test) and verbal fluency tasks was severely impaired (3

F words and 7 animals in one minute each) although he was still able to repeat

lengthy sentences. He had severe impairments in both immediate and delayed

verbal memory and was no longer able to use cues to increase his memory

performance (no words recalled after a brief delay). His Mini Mental State Exam

score of 19/30 was impaired only as a result of his word retrieval impairment.

Amidst these language deficits, EG demonstrated normal memory for visual

material and normal visual construction (e.g., he was able to draw a clock and place

the hands correctly to 10 minutes after 11). His calculational abilities remained

normal: he was able to perform simple addition and subtraction. His mood was

judged clinically to be normal and he demonstrated appropriate affect during

clinical and research visits. His son reported no significant behavioral problems at

home.

Case 2

Case history and presentation

FZ is a 64-year old male with a Masters of Business Administration and

Certified Public Accountant degree, who worked as an accountant. He presented in

2009 with gradually progressive word finding difficulties. In particular, he forgot

names of people he had known for many years. Once an avid reader, he began

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having difficulties following newspaper articles. He reported mild depression and

anxiety as a result of his symptoms, but was not diagnosable with a formal mood

disorder. He had no behavioral symptoms or personality change. On his first

evaluation, he demonstrated significant word finding difficulties and semantic

paraphasic errors during casual conversation. Structural MRI demonstrated

bilateral temporal pole atrophy with more prominent atrophy in the left temporal

pole. He was diagnosed with PPA-S (semantic dementia). Findings from a full

neuropsychological battery performed in 2009 are as follows.

Intellectual abilities. This patient had normal intellectual abilities. He had

average Perceptual Reasoning and Working Memory on the Weschler Adult

Intelligence Scale-IV. He performed in the high range on Matrix Reasoning (91st

percentile).

Attention and Executive function. This patient had normal attention and

executive function. His digit span was high average (7 forward and 5 in reverse), as

was his visual attention (forward span= 6, backward span = 4-5). He had no

difficulty on a graphomotor sequencing task (alternating M and N). Performance on

the DKEFS Trail Making Test was borderline (Trails A, 43 seconds, 4th percentile;

Trails B, 101 seconds, 2nd percentile).  He demonstrated no difficulty on a go/no-go

task.  His was able to quickly learn a serial hand movement sequence with his right

hand and was able to transfer learning to his left hand.  Concept formation and

cognitive flexibility was preserved (WCST, 6/6 categories).

Memory. This patient had normal recognition memory, but consistent with

the semantic dementia diagnosis, had poor performance on the word retrieval. On

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the CERAD 10-word list, his immediate recall was normal (5, 8, and 8 words; Total

Learning = 21, 47th percentile).  After 8 minutes, he only recalled 3 words (1st

percentile). Delayed recognition was in-line with encoding, (10/10 words, 1 false

positive).  On the Free and Cued Selective Reminding Test, he had mild difficulty

retrieving new material across the three learning trials (Free Recall = 22), but

benefited from the provision of cues paired with the items at the time of learning

(Free and Cued Total = 44).

Visuospatial skills. This patient had normal visuospatial skills. His

performance on the Rey-O figure copy was fully intact although the design was

drawn in a segmented fashion.

Language.  Consistent with the diagnosis of semantic dementia, this patient

had impaired performance on language tests. Visual confrontation naming was

impaired on two measures (Boston Naming Test, 33/60, Multilingual Aphasia Exam:

Visual Naming, 21/30). Phonemic cueing was only minimally helpful, with an

additional 3 items named.  He had mild difficulty on an auditory word picture

matching task, selecting the correct picture (out of 2 choices) for 29/32 concrete

words (5th percentile).  He also had difficulty comprehending abstract words, where

accuracy in choosing which of two words matched a target word's meaning (e.g.,

does naïve mean innocent or stupid?) fell in the impaired range.  In contrast, his

ability to access other aspects spoken language, including phonological,

morphological and syntactic information was intact. He was able to distinguish

words from non-words, to distinguish word meaning based on morphology (e.g.,

employer vs. employee, bus vs. busses), and was able to appreciate the syntactic

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form of sentences as assessed on a sentence-picture matching task, however, and all

scores fell in the average range. Basic comprehension using the token test was

preserved.  

Profile at time of emotion testing

By 2012, when FZ completed the present experimental tasks, he had

undergone progressive impairment in the domain of language and semantic

memory. He was exhibiting anomia and semantic memory impairment with fluent

and articulate speech. Performance verbal fluency tasks was impaired (Verbal

fluency for animals = 8; vegetables = 2). His Mini Mental State Exam was 25/30.

Verbal memory encoding was impaired but he retained what he learned after a 20-

minute delay (Wechsler Logical Memory Immediate = 4; Delayed = 4).

Amidst these language deficits, FZ displayed normal visuospatial function (he

was able to copy a picture of a pentagon on the Mini Mental State Exam and

accurately draw a clock with the instructed time). Although some aspects of

executive function demonstrated progressive impairment from 2009 (Trail Making

Test A 50s; Trail Making Test B 123 s), others remained relatively intact (e.g., digit

span forward 7, backward, 6). His mood was judged clinically to be normal and he

demonstrated appropriate affect during clinical and research visits. His wife

reported no behavioral problems at home.

Case 3

Case history and presentation

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CP is a 53-year-old female with a Master’s Degree in Education who worked

as an administrator at a bank before retiring. She presented in 2008 with gradually

progressive word finding difficulties. At the time of presentation, her family

reported that she had been struggling to find words (mostly nouns) and had

experienced some minor difficulties with memory retrieval for conversations. They

denied any changes in mood or personality. On her first evaluation, she

demonstrated word-finding difficulty with anomia and pauses during spontaneous

speech but normal affect and comportment. Structural MRI demonstrated left

temporal pole atrophy. She was diagnosed with PPA-S (semantic dementia).

Findings from a full neuropsychological battery performed in 2008 are as follows.

Intellectual abilities. This patient had normal intellectual abilities. She had

a high average estimated intelligence score on the Weschler Test of Adult Reading.

Attention and Executive function. This patient had normal attention and

executive function. Performance was in the superior range on an auditory attention

span test and average on the Mental Control subtest of the Weschler Memory Scale

III. Digit span was 7 forward and 6 in reverse. Performance on a number-letter

sequencing task was in the average range. Wisconsin Card Sort was performed at an

average level with no perseverative errors and 6 categories obtained. Performance

on DKEFS Trail Making Test was in the high average to superior range. Color word

interference was in the average to high average range.

Memory. This patient had normal memory. Performance on the Weschler

Memory Scale III Logical Memory was in the high average range for immediate

recall (75th percentile), with 30 minute delayed recall in the high average range (84th

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percentile). One hundred percent of encoded material was retained after the delay.

Visual memory was in the high average range (20-minute delayed recall of Rey-O

figure = 86th percentile).

Visuospatial skills. This patient had normal visuospatial skills. Her

visuospatial attention was high average on the Weschler Adult Intelligence Scale III.

Rey-O figure copy was 95th percentile.

Language. Consistent with the semantic dementia diagnosis, this patient had

impaired performance on language tests. Performance on the Boston Naming Task

was impaired (17/32 spontaneously; 1 additional correct response was obtained

with phonemic cues; semantically correct descriptions were often obtained (e.g.,

“you throw it” for dart). Semantic access from pictures was relatively maintained on

the Pyramids and Palm Trees test (49/52). Lexical verbal fluency was average (total

FAS = 29) but semantic verbal fluency was borderline (DKEFS category total = 25).

Profile at time of emotion testing

By 2011, when CP completed the present experimental tasks, she had

undergone progressive impairment in the domain of language and semantic

memory. She was exhibiting severe anomia and semantic memory impairment with

fluent and articulate speech. Her Mini Mental State Exam was 27/30, with errors

due to language deficits. Amidst these language deficits, her basic calculational skills

(addition and subtraction), visuospatial skills (e.g., she was able to copy a picture of

a pentagon on the Mini Mental State Exam and accurately draw a clock with the

instructed time) and attention remained intact. Her mood was judged clinically to be

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normal and she demonstrated appropriate affect and comportment during clinical

and research visits. Her husband reported no behavioral problems at home.

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