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Dense amnesia in a professional

musician following herpes simplex virus
encephalitis
a a b
Barbara A. Wilson , Alan D. Baddeley & Narinder Kapur
a
MRC Applied Psychology Unit , Cambridge
b
Wessex Neurological Centre , Southampton
Published online: 04 Jan 2008.

To cite this article: Barbara A. Wilson , Alan D. Baddeley & Narinder Kapur (1995) Dense amnesia
in a professional musician following herpes simplex virus encephalitis, Journal of Clinical and
Experimental Neuropsychology, 17:5, 668-681, DOI: 10.1080/01688639508405157

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 Journal of Clinical and Experimental Neuropsychology 1380-3395/95/1705-668$6.00
1995, Vol. 17, No. 5 , pp. 668-681 0Swets & Zeitlinger

Dense Amnesia in a Professional Musician Following Herpes

Simplex Virus Encephalitis*
Barbara A. Wilson’, Alan D. Baddeley ’, and Narinder Kapur2
‘MRC Applied Psychology Unit, Cambridge, and 2Wessex Neurological Centre, Southampton

ABSTRACT
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We describe the memory functioning of C, a professional musician who became amnesic following herpes
simplex encephalitis in 1985. Although transient amnesia in a professional musician has previously been
described, this is the first reported case of chronic amnesia i n a highly talented professional musician. C is
unusual in three respects. First, his amnesia is particularly severe. Second, his amnesia includes semantic
as well as episodic memory deficits. Third, he believes he has just woken up and his preoccupation with
this state of ‘just wakening’ has persisted for over 9 years. This appears to be the result of a delusion rather
than the consequence of his amnesia.

The amnesic syndrome can result from a number
of neurological conditions including bilateral
hippocampal surgery (Scoville & Milner, 1957),
encephalitis (Cermak & O’Connor, 1983;
Damasio, Eslinger, Damasio, Van Hoesen, &
Cornell, 1985; Warrington & McCarthy, 1988),
thalamic lesions (Squire & Moore, 1979) and
Korsakoff’s syndrome (Butters, 1979).
The human amnesic syndrome is charac-
terised by (a) severe difficulty in learning and
remembering new information of nearly all
kinds, (b) normal short-term memory (STM) as
measured by digit span and the recency effect in
free recall, and (c) (usually) a period of retro-
grade amnesia (RA).
Semantic memory is usually found to be in-
tact on most semantic memory tests. In 1988 we
reported the case of KJ (Wilson & Baddeley,
1988), a man with a very pure amnesic syn-
drome. We demonstrated that KJ was unim-
paired on cognitive tasks that did not involve
episodic memory and that he showed good per-
formance on tests of vocabulary, verbal fluency,
semantic category judgement, and speed of sen-
tence verification. This suggested an intact se-
mantic memory but we went on to demonstrate
that KJ’s capacity for registering new material
in semantic memory was far from normal. We
therefore suggested that KJ showed a sparing of
old and overlearned memories whether semantic
or episodic rather than the specific preservation
of semantic memory.
It is possible to find patients with impaired
semantic memory and intact episodic memory
(e.g., Hodges, Patterson, Oxbury, & Funnell,
1992a; Hodges, Salmon & Butters, 1992b).
Hodges et al. (1992a) define semantic memory
as the “...component of long-term memory
which contains the permanent representation of
our knowledge of objects, facts and concepts as
well as words and their meaning” (p. 301). It is
possible to find selective deficits of visual se-
mantic memory with intact knowledge of verbal
semantics. This phenomenon is seen in associa-

* We wish to thank C’s wife for providing information on his personal life and his musical ability. We are also
grateful to Dr. John Hodges of Addenbrooke’s Hospital for the loan of his semantic memory battery. Dr. Kapur
acknowledges the support of the Wellcome Trust (grant number 03619 I).
Address for correspondence: Dr. B. A. Wilson,MRC Applied Psychology Unit, 15 Chaucer Road, Cambridge
CB2 2EF, England.
Accepted for publication: January 26, 1995.
 DENSE AMNESIA FOLLOWING HERPES SIMPLEX
tive visual agnosia (Farah, 1990). A reversal of
this is found in subjects who have normal visual
semantic memory but have lost the ability to
access meaning through written or spoken lan-
guage (Warrington & McCarthy, 1983). It is of
course also possible to lose semantic informa-
tion across all modalities (Sartori &Job, 1988).
The patient we describe below has both epi-
sodic and semantic memory deficits for both
visual and verbal information. He is not a “pure
amnesic” since he performs in the impaired
range on tests of naming and on tests of execu-
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tive function suggesting frontal lobe damage.
Nevertheless, his IQ remains in the average
range and his performance on many cognitive
tasks is intact. However, we have never encoun-
tered someone so densely amnesic as C. Nor
have we seen anyone so convinced that he has
just woken up from a period of sleep, illness, or
death-like state. He has recorded this moment of
awakening in his diary many times a day for
over 9 years. The well known amnesic patient
HM (Scoville, 1954) has described his amnesia
as being like sleep but he does not live in a mo-
ment of ‘just awakening’. In a report by Milner,
Corkin, and Teuber ( I 968), HM is said to have
remarked “Every day is alone in itself, whatever
enjoyment I’ve had and whatever sorrow I’ve
had” (p. 2 17). Unlike, HM, C is not aware that
there have been other days.
CASE REPORT
At the time of his illness in March 1985, C was
a gifted musical scholar and chorus master and
one of the world’s leading authorities on Renais-
sance music. At the age of 46, he developed an
influenza-like illness with headache and fever.
He was admitted to hospital after 12 days. Her-
pes simplex virus encephalitis (HSVE) was di-
agnosed a day later. The total period of coma
was 16 days. He had one grand ma1 seizure dur-
ing coma. After emerging from coma, and about
two and a half weeks since the onset of his ill-
ness, C’s hospital notes reported him as having
a memory of about 3-s duration although it is
not clear how this was determined. His conver-
sation was said to be marked by confabulation
669
and perseveration, and his retrograde amnesia
was considered to be about 7 months (although
it was not clear how this was established and
was probably inadequately assessed).
A CT scan indicated areas of low density par-
ticularly in the left temporal lobe extending into
the inferior and posterior frontal lobe and into
the right medial temporal lobe. No further CT
scans have been performed but in July 1991 C
had an MRI scan (see Figures 1 a and 1 b).
Detailed anatomical analyses of C’s MRI
scans by three independent raters showed
marked abnormality to be present in the follow-
ing structures - both hippocampal formations,
both amygdalas, the substantia innominata on
both sides, both mammillary bodies, both tem-
poral poles, the left fornix, the left inferior tem-
poral gyrus, the anterior portion of the left mid-
dle temporal gyrus, the anterior portion of the
left superior temporal gyrus, and the left insula.
In addition, there was mild abnormality in the
following structures - the posterior portion of
the left middle temporal gyrus, the left medial
frontal cortex, the left striatum, the right insula,
the right fornix, and the anterior portion of the
right inferior temporal gyrus. The third ventricle
and both lateral ventricles were considered to be
significantly dilated. It is of note that both the
left and right thalamus were rated as being in-
tact, and that no other frontal lobe abnormality
was found apart from the left medial frontal ab-
normality mentioned above.
A speech therapy assessment was carried out
in April and May 1985. The therapist noted such
“extreme distractibility” that C could only be
“partially assessed” on the Boston Diagnostic
Aphasia Examination. The report noted impair-
ment in auditory comprehension at complex lev-
els where retention of information is necessary.
C’s ability to understand single words was also
affected. For example, when asked to define
“eyelid”, he said, “I don’t know that word, it
must be used by a great specialist”. The speech
therapist also noted that C’s comprehension of
complex written material was affected. He read
aloud with 100% accuracy but complained that
words had changed their meanings.
 670 BARBARA A . WILSON ET AL.
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Fig. I . Figure l a shows T, weighted coronal view of C’s MRI scan, with the large left temporal lobe lesion and
smaller right temporal lobe lesion being evident. Figure I b provides a T, weighted axial image, with a
similar lesion profile being apparent. See text for details of specific anatomical structures that were
involved.
 DENSE AMNESIA FOLLOWING HERPES SIMPLEX
A psychiatric report of C dated July, 1985
described the following:
I . Severe organic memory deficit;
2. Severe depressive reaction in the presence
of some insight;
3. Retention of many cognitive abilities such
as language and music.
He was referred to the author (BAW), i n Oc-
tober 1985. A letter from his wife at the time
said “Since the beginning of this month C... has
developed a compulsion to write things down all
the time ... things look different if he looks
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away”. She also described how C tried to work
out reasons for his illness: For example, he
sometimes assumed someone wanted ‘to shut
him up’ because he had uncovered an interna-
tional conspiracy. Furthermore, he attributed the
reason for this to whatever was on the front page
of the Times newspaper. One day he believed
the conspiracy was due to King Hussein and
Geoffrey Howe, and on another occasion he sus-
pected Mrs Victoria Gillick, mother of 10, who
was in the news because of her views on contra-
ception.
This letter also described how C believed he
had worked at the hospital (in which he was now
a patient) many years ago. He claimed that when
he was a student at University he organised fel-
low students to work at the hospital in the vaca-
tion period when the staff were on holiday. He
then added that he did this for the whole coun-
try. Sometimes he saw a nurse who had been on
his ward for several months and pointed her out
as someone who worked there when he ran the
place. C’s student vacation work was i n fact as a
waiter i n a hotel.
For the first assessment, C was seen approxi-
mately once a month between November, 1985
and May, 1986. He was always accompanied by
his wife but sometimes he was seen alone while
his wife was interviewed separately.
C was not an easy man to test. He frequently
became angry saying that he had just woken up
for the first time i n months, that he had not seen
or heard anything until that moment so how
could he know the answers to the questions.
This theme was repeated every few minutes. The
tests that he appeared to enjoy most were the
performance items of the Wechsler Adult Intelli-
67 1
gence Scale (Wechsler, 1955). Some tests were
abandoned because of his increasing frustration
and annoyance. For example, he became ex-
tremely angry during administration of the Sea-
shore Test of Musical Abilities (Seashore,
Lewis, & Saetveit, 1960) saying “1 am a world
famous musician. How dare you ask me if I can
discriminate between different pitches?”. It is
highly unlikely that this test would have caused
C any difficulty at all. Testing proceeded slowly
and despite C’s difficult behaviour, the results
were considered to be reliable estimates of his
current levels of functioning because he eventu-
ally cooperated with most tests given frequent
breaks between subtasks.
The confabulation noticed in the 1985 letter
from his wife has persisted. When seen in Octo-
ber I985 at a hospital he had not entered before,
as far as we knew, he said he used to run the
hospital during the summer vacation when the
doctors and nurses were on holiday. This con-
fabulation is easy to elicit and C has repeated it
on numerous occasions. A more recent develop-
ment is an hallucination which first appeared in
1990 i n which C ‘hears’ his own music being
played around the hospital. Nobody else can
hear this.
Since the first assessment in 198986, C has
been reassessed in 1989 as part of a long-term
follow-up study of memory-impaired people
(Wilson, 199 1 ), and further assessed in 199 1 and
1992.
ASSESSMENT
General Intellectual Functioning
The Wechsler Adult Intelligerzce Scale (Wechs-
ler, 1955) was administered in 1985 and the
Wechsler Adult Intelligence Scule - ReL’ised
(Wechsler, 1981) was administered in 1992. The
age-scaled scores for both occasions are dis-
played in Table 1 .
In 1985, all age-scaled scores were within the
normal range. Scores ranged from a low of 8 for
Digit Symbol, to a high of 14 for Block Design.
Verbal and Performance IQ scores were 105 and
106, respectively, that is, within the average
 672 BARBARA A. WILSON ET AL.

Table I . Age-Scaled Scores on the WAIS and WAIS-R Obtained by C.

1985 WAIS results 1992 WAIS-R results

Verbal subtests
Information 11 8
Comprehension 11 8
Arithmetic 13 12
Similarities 9 6
Digit Span 10 9
Vocabulary 12 12
Verbal IQ 105 92
Prrfi:forinancrsubtests
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Digit Symbol 8 II
Picture Completion 12 10
Block Design 14 15
Picture Arrangement 11 8
Object Assembly 10 12
Performance IQ 106 105
Full Scale I 0 106 97

range but lower than one would expect from C’s
educational level (university student), occupa-
tion, and status.
In 1992, C was reassessed with the WAIS-R,
which typically estimates both verbal and per-
formance IQs to be 7 to 8 points lower than
those obtained on the WAIS. In this more recent
assessment C’s verbal IQ appears to have de-
clined a little even allowing for the expected 7-
or 8-point discrepancy, whereas his performance
I Q appears to be stable - or when allowing for
the different versions, may have increased
slightly (see Table 1).
Upon examination of individual verbal re-
sponses, there would appear to be an impover-
ishment in the quality of C’s responses which
suggests that he is forgetting knowledge about
the world. For example, on the Similarities
subtest, his 1985 response to the question: “In
what way are North and West alike?” was “Part
of the compass”. In 1992, he responded, “At
right angles to each other”. Similarly, when
asked “In what way are air and water alike?” he
said, “Both have oxygen” in 1985, and “Natu-
rally part of the climate” in 1992.
Nurional Adult Reading Test (Nelson, 1982).
This test requires subjects to read and pronounce
a range of orthographically irregular words
ranging from common to rare. The predicted
premorbid IQ can be estimated from the number
of errors on the test. In 1985, C’s estimated
premorbid IQ on this test was 122 (superior
range but probably an underestimate) when he
made 7 errors. In 1992, he made 20 errors giving
him a predicted premorbid IQ of 111 on the
WAIS and 106 on the WAIS-R. Again, this sug-
gests that he is forgetting some of his general
knowledge.
Frontal Lobe Tests
Nelson’s (1976) modified version of the Wis-
consin Card Sorting Test was administered in
1985 and 1992, together with various tests of
fluency. Results are presented in Table 2.
His rule breaking in the fluency tasks in-
cluded “sausage” for an animal (this came im-
mediately after “dog”), and “hump” which
came immediately after “camel”. Neologisms
included spitch, strick, strungle, and swingle.
When asked to produce the names of as many
musicians as he could in 1 min, C named only
four: Mozart, Beethoven, Bach, and Haydn. He
 DENSE AMNESIA FOLLOWING HERPES SIMPLEX
Table 2. Frontal Lobe Test Scores Obtained by C .
Test 1985
Wisconsin Card Sorting Test Categories = 4
(Nelson’s 1976 modified ver- Total Errors = 2 I
sion) Perseverative Errors = 8
Fluency S (90”)= I2 correct
V (90”)= 3 correct
Perseverations = 14
Rule breaking = 4
Neologisms = 2
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Animals (90”)= 12 correct
Perseverations = 8
Rule breaking = 2
produced no famous painters nor artists, and
only one writer (Dickens) during separate 60-s
intervals.
Perceptual Tests
Scores on the performance subtests of the WAIS
(administered in 1985) suggested that this man’s
visuo-spatial and visuo-perceptual skills were
within the average range. He also copied accu-
rately the Rey-Osterreith Complex Figure
(Osterreith, 1944). However, his wife reported
at that time that he could not tell the difference
between jam, honey, and marmalade although he
“knew” that he preferred honey. She also re-
ported that he had attempted to spread cottage
cheese on his bread thinking that it was butter,
that he could not tell which object on the wash-
basin was toothpaste (although he knew which
was the toothbrush), he had given her talcum
powder when she had asked for soap, he had
called a scarf an “umbrella”, he had confused
mantelpiece and wardrobe, and he had mistaken
his wife’s sweater for his shirt. This suggested
some visual semantic memory deficits that were
not investigated in any detail until 1991/1992.
673
I992
Categories = 6
Total Errors = 0
Perseverative Errors = 0
S (60”)= 8 correct
Perseverations = 0
Neologisms = 4
F (60”)= I0 correct
Perseverations = 2
A (60”)= 9 correct
Perseverations = 3
Neologisms = 1
Aniniuls (60”)= 10 correct
Perseverations = 2
Musicians (60”)= 4 correct
MEMORY
Immediate Memory
In 1985, 1989, and 1992 immediate memory
span for this patient was in the average range
with a forward digit span of 6, a backward digit
span of 4, and a sequential visual tapping span
of 6 on each of the three occasions.
Episodic Memory
The Rivermead Behavioural Memor.v Test (Wil-
son, Cockburn, & Baddeley, 1985) was adminis-
tered in 1985 and 1989. This test of everyday
memory problems comprises 12 items. Non-
memory-impaired subjects under the age of 65
typically pass at least 10 of these. C failed 12
items on both occasions suggesting very severe
impairment. Furthermore his raw score was 4/93
and 3/93 in 1989. KJ, the pure amnesic patient
reported in 1988 (Wilson & Baddeley) scored
23/93.
Prose recall
On the logical memory (prose recall) passages
of the Wechsler Memory Scale (Wechsler,
1945) administered in 1985, C scored only 1 on
immediate recall and 0 on delayed recall despite
being prompted with the first few words. Again
this suggests severe impairment. In addition, he
confabulated on the immediate recall. In the
 674 BARBARA A. WILSON ET AL.

case of the first prose passage, the Anna Thomp-
son story from the Wechsler Memory Scale, C
scored 1 point for Police Station (City Hall in
the American version) but said “There was an
interview with a girl about a case. They asked
her a number of questions”. For the second, the
American liner story, he said “ A crash - people
were thrown off a ship and picked up by another
ship. There was chaos because of the speed the
boats were travelling. It was a question of tim-
ing. Information was by no means clear and
children were screaming”. In 1989 he failed to
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earlier, was administered to C in 1989. His in-
dex scores on ( 1 ) general memory, (2) verbal
memory, (3) visual memory, and (4) delayed
memory were all below SO, that is, the lowest
possible score index. On the remaining index,
attention and concentration, he scored 105,
which is in the average range. He refused to par-
ticipate in the paired associate learning test (he
scored 7/18 on easy pairs and O/I 2 on hard pairs
in 1985). On the visual reproduction subtest he
scored I7 (4th percentile) on immediate recall,
and 0 at delayed recall. In summary, C exhibits

recall anything from either of the two stories in
both the immediate and delayed recall condi-
tions although he no longer confabulated, saying
“It’s all gone”.
Rey Osterreith Coinplex Figure
Despite a good copy (33/36), on both occasions
C failed to recall any of the figure after a delay.
Recognitiori memory
Two of the 12 RBMT subtests assess recogni-
tion memory. On the picture recognition subtest,
in which 10 items are shown one at a time and
after a few minutes delay must be recognised
from a set of 20, C denied seeing any of the
items before, both in 1985 and 1989. Similarly,
on the face recognition subtest involving five
faces plus five distractors, he again denied see-
ing any of them before. This is an extremely
poor score even for amnesic subjects, given that
the RBMT recognition memory tests comprise
far fewer items than most recognition memory
tests.
Wechsler Memory Scule - Revised (Wechsler,
1987). This test, which had not been available
a dense anterograde amnesia. The density of his
amnesia is further illustrated by the fact that of
a group of 10 memory-impaired HSVE patients,
C showed the most severe anterograde memory
impairment (Kapur et al., 1994).
Autobiographical Memory
C was a5sessed on the Autobiographical Mem-
ory Interview (Kopelman, Wilson, & Baddeley,
1989) in 1985 (prior to publication) and 1992.
The AM1 asks subjects about their memory for
(a) childhood, (b) early adult life, and (c) recent
times, that is, the past year. For each time period
the subject is tested on both factual questions
(e.g., “What was the name of your first school?
Can you tell me the names of three friends or
teachers from that time”), and on autobiograph-
ical incidents (e.g., “Tell me about something
that happened to you while you were at your
first school/first job/previous hospital”). At
each time period and on both occasions C scored
i n the impaired range (see Table 3). He also of-
fered the previously described confabulation
about working in the hospital when he was a
university student at both test administrations in
1985 and 1989.

Table 3. Performance ot C on the Autobiographical Memory Interview.

Time period Personal Semantic Autobiographical Incidents

(max. 21 for each time period) (max. 9 for each period)
I985 I992 I985 1992
Childhood 6 I1 2 3
Early Adulthood 4 6 I I
Recent 0.5 I 0 0
Total 10.5 18 3 4
 DENSE AMNESIA FOLLOWING HERPES SIMPLEX
These scores indicate marginal improvement,
particularly in recall of factual (personal seman-
tic) information from childhood. The recall of
autobiographical episodes or incidents is essen-
tially unchanged.
Retrograde Amnesia
On the Prices Test of retrograde amnesia (Wil-
son & Cockburn, 1988), C was asked to estimate
the current price of 12 common objects such as
a first-class stamp and a pint of milk. His error
score in 1985 was 18 (the mean error score for
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controls is 0 and for Korsakoff patients is 18).
Again this suggests that he is many years out of
date.
In 1989 a modified version of Kapur’s retro-
grade amnesia test (Kapur, Young, Bateman, &
Kennedy, 1989) was administered to C. He was
asked to indicate whether famous people were
dead or alive, and when and how they died. He
scored 1/20 (Elvis Presley) and stated that he
had never heard of such people as John Lennon,
Lee Harvey Oswald, or John F. Kennedy. He
stated that he had vaguely heard of Margaret
Thatcher, Mohammed Ali, and Cliff Richard but
could not say anything about them.
As a student C had spent 4 years in Cam-
bridge and had returned frequently since that
time. When shown photographs of Cambridge
he recognised only one of the most distinctive
landmarks, that of King’s College Chapel. He
failed to recognise his own college.
Semantic Processing
This is a test from the Speed and Capacity of
Language Processing (SCOLP) (Baddeley,
Emslie, & Nimmo-Smith, 1992). The subject is
required to verify as many sentences as possible
in 2 min. These are simple sentences that are
obviously true (e.g., ‘Rats have teeth’) or obvi-
ously false (e.g., ‘Nuns are made in factories’).
This test has been shown to be sensitive to the
effects of head injury and of ageing (Baddeley,
Harris, Sunderland, Watts, & Wilson, 1987). In
1985, C’s performance was in the impaired
range with a mean time per sentence of 4.8 s
(age-matched controls take about 2 s per item)
and 6 errors (controls rarely make more than one
or two errors). His errors included “Dragonflies
675
have wings” marked as false and “Forks have a
lot of industry” marked as true. His reading
aloud of these sentences is fast and accurate in-
dicating that his difficulty is more likely to be
due to a semantic memory impairment, than to
reading problems.
In contrast to the WAIS and NART results,
C’s performance on the Semantic Processing
Test improved between 1985 and 1992. He com-
pleted 49 items in 2 min in 1992 as compared to
25 items on the earlier occasion, yielding a mean
time per item of 2.44 s, that is, an improvement
of almost 100%. He made only one error in the
recent assessment as compared to six errors in
an earlier assessment.
Other Tests of Semantic Memory
On the Graded Naming Test (GNT; McKenna &
Warrington, 1983), C correctly named only 2 of
a possible 30 pictures (Kangaroo and Radius).
Some of his errors were obviously due to word
finding problems, for example, ‘a swimming
tortoise’ for ‘turtle’. Others suggested object
recognition difficulties, for example, ‘a wor-
shipping point for certain cultures’ for ‘scare-
crow’. Similarly, when asked on a later occasion
to define the 30 objects in the GNT when given
the name in the absence of the picture, C pro-
duced some strange answers. For example, to
the question, “What is a scarecrow?” he re-
plied, “A bird that flies and makes funny
noises”. Some of his responses to the pictures
and words are displayed in Table 4.
On a semantic memory battery (Hodges,
Salmon, & Butters, 1992) C exhibited a relative
discrepancy between living and nonliving items
with performance on living things being poorer.
His scores on four subtests of this battery are
reported in Tables 5 and 6.
Reaction Time
A simple reaction time task was administered to
this patient in order to assess his speed of motor
response to a cognitively undemanding task. The
task was the same as that used by Wilson,
Baddeley, Shiel, and Patton (1992) in which
subjects are required to press a button as quickly
as possible whenever a red light appears. The
light appears randomly every few seconds. The
 676 BARBARA A. WILSON ET AL.

Table 4. Responses of patient C to 10 of the 30 objects in the Graded Naming Test (McKenna & Warrington,
1983)

Stimulus Response to Picture Response to Spoken Name

Kangaroo Australia, South America, South Africa ... Jumps up and down - Australia - animal
Kangaroo
Scarecrow Worshipping point for certain cultures Bird that flies and makes funny noises
Tweezers Caterpillar - no not a caterpillar a hand Things for picking up very small objects -
thing for picking up a caterpillar - small metal
things
Corkscrew For drilling a hole in wood For drilling holes in corks - to let metal or
wire come through. Also for ... I’ve for-
gotten. For screwing through corks. Very
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common.
Sporran (and kilt) A man with everything back to front. A No idea.
skirt with the inside on the outside. Middle
East.
Sundial Clock of a curious kind held up to catch a A dial to the sun or through the sun depend-
shadow from the sun. ing on how you look at it.
Anteater Looks like a .... all nose and no mouth - Something that eats an ant but it can’t be
an imaginative animal. that - there’s another meaning.
Pagoda Top of a church somewhere or other - Don’t know.
dates from 1200 or 1250 - somewhere like
Italy.
Mitre A crown - duchess’ crown - a crownette Oh gosh -can’t remember- afamiliarthing -
from the French. could be something you wrote down on paper.
Sextant Looks like a telescope or something - get Something to do with six. Six times some-
an angle from its height rather than distance. thing.

Table 5. Scores on Category Fluency for Living and Nonliving Items ( 1 min Each Category) Obtained by C.
______ ~

Living Total Correct Nonliving Total Correct

Responses Responses
Animals 9 Household items II
Birds 6 Vehicles 7
Water creatures 3 Musical instruments 12
Dogs 0 Boats 10

Overall Total 18 40

mean time over a 5-min period is calculated. For Behaviour

C, the mean time was 0.24 s. This compares
favourably to a group of 12 subjects with the
pure amnesic syndrome reported by Wilson e t
al. (1992). T h e mean response time f o r this
group was 0.25 s; responses for control subjects,
head-injured patients with chronic memory im-
pairment, and patients in posttraumatic amnesia
were 0.30, 0.33, and 0.47 s, respectively. This
suggests that C’s SRT is unimpaired and that h e
does not exhibit the kind of confused state that is
seen in patients with PTA.
C remained difficult to test throughout; h e con-
tinued to state that h e had just woken up, had
heard nothing, seen nothing, tasted nothing, and
it was “like being dead”. In essence, there was
little change in his behaviour or, indeed, in his
memory functioning since the original assess-
ment.
 DENSE AMNESIA FOLLOWING HERPES SIMPLEX 677

Table 6 . Scores on Naming Pictures, Naming to Description, and Recognition of Pictures for Living and Nonliv-
ing Items Obtained by C.
Living Nonliving
1 Naming Pictures Total 11/24 20124
a) Land animals 9/12 a) Household items 911 2
b) Water creatures 116 b) Vehicles 516
c) Birds 116 c) Musical instruments 616
2 Naming to Description Total 2/12 8/12
a) Land animals 1/4 a) Household items 314
b) Water creatures 014 b) Vehicles 314
c) Birds 1/4 c) Musical instruments 214
3 Recognition of Pictures Total 17/24 24/24
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a) Domestic animals 616 a) Electrical items 616

b) Foreign animals 4/6 b) Household items 616
c) Water creatures 216 c) Vehicles 616
d) Birds 516 d) Musical instruments 616

DISCUSSION cortex was spared but excision of the mesial sur-

We believe that C is unusual in three respects:
(a) his amnesia is particularly severe, (b) he has
semantic as well as episodic memory deficits,
and (c) he believes that he has just woken up
(his preoccupation with this state of just awak-
ening has persisted for over 9 years).
Severity of Amnesia
How does C compare with other amnesic sub-
jects? The most famous of these is, without
doubt, HM, first reported by Scoville and Milner
( 1957). C seems to differ from HM in a number
of ways. C has a longer retrograde amnesia and
a more severe loss of autobiographical memory.
HM, on the other hand, appeared to be able to
remember events until about 2 years prior to his
operation (Milner et al., 1968). There are no re-
ports that HM suffers from semantic memory
impairments. C is constantly preoccupied with
having just woken up. HM described his amne-
sia as being like sleep, stating, “Every day is
alone in itself, whatever joy I’ve had and what-
ever sorrow I’ve had” (Milner et a]., 1968, p.
2 17). Unlike HM, C is unaware that there have
been other days prior to the one in which he
finds himself.
HM’s lesions were restricted to the mesial
parts of both temporal lobes. The temporal neo-
face was very radical, extending some “ ...8
centimetres from the tips of the temporal lobes
and destroying the anterior two thirds of the hip-
pocampus bilaterally, as well as the uncus and
amygdala” (Milner, 1966, p. 112). C’s lesions
were more extensive, with virtually all of the
left temporal lobe affected and a smaller part of
the right temporal lobe.
Several patients have been reported following
encephalitis, including SS by Cermak and
O’Connor (l983), RFR by Warrington and Mc-
Carthy (1 988), and Boswell by Damasio et al.
(1985) and Tranel and Damasio (1993). SS
shows a temporal gradient with events from the
1930s being recalled better than those from the
1970s. C does not show this temporal gradient in
his recall of public events, possibly because of a
floor effect resulting from his uniformly low
performance. However, his autobiographical
memory does show a gradient with childhood
memories being less impaired than those of
early adulthood, while memory for recent events
was virtually totally absent. SS also appears to
have better recall of personal than of public
events whereas C is impaired in both domains.
Cermak and O’Connor report that SS retains
an ability to entertain his visitors with anecdotes
about his childhood and young adult years. C
does not do this, although he can at times be per-
 678 BARBARA A. WILSON ET AL.
suaded to tell visitors about the history of con-
ducting. C differs further from SS in that he has
more neuropsychological dysfunction, espe-
cially in regard to semantic memory, and he has
a tendency to confabulate.
In comparison with RFR (Warrington & Mc-
Carthy, 1988) who has also been assessed by
one of the authors (BAW), C once again has a
longer period of RA, semantic memory prob-
lems, and is far more agitated and difficult to
test. RFR’s scores on the Wechsler Memory
Scale - Revised are also higher than those ob-
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tained by C. For example, RFR’s verbal memory
index is 62 and his visual memory index 64,
whereas C scored below SO on both measures.
In many ways C is more similar to Boswell
(Damasio et al., 1985) in that both have wide-
spread temporal lobe damage, extensive retro-
grade amnesia, and both confabulate. However,
C’s anterograde amnesia appears to be even
more extensive than Boswell’s (for example,
Boswell recalled four items of the Rey
Osterreith Figure after a delay whereas C scored
zero). C also has a higher FSIQ than Boswell.
Parkin (1984) argues that Korsakoff patients
show a more extensive RA than patients with
purely temporal lesions, yet even these patients
rarely show as devastating a loss as C. He is cer-
tainly not like a typical Korsakoff patient in
terms of personality. Butters (1 979), for exam-
ple, says of the typical Korsakoff patient, “Re-
gardless of the patient’s premorbid personality,
he is extremely passive, malleable and emotion-
ally flat in the chronic Korsakoff state” (p.
453).
According to Parkin (1984), amnesic patients
with damage to the diencephalon show a differ-
ent pattern of deficits from amnesic patients
with damage to the temporal lobes. C, of course,
has damage to both these systems. The original
scan indicated frontal lobe involvement whereas
the report of the MRI scan indicated that there
was widening of the third ventricle which i s part
of the diencephalon, and also abnormality of the
mammillary bodies. C, then has almost complete
destruction of the hippocampi, more lateral
damage to the cortex and white matter of the
temporal lobes, and diencephalic damage which
are probably responsible for the extensive RA.
Semantic Memory Impairment
In retrospect, there was considerable evidence
for the presence of semantic memory disorders
as far back as 1985. C’s wife reported that he
could not tell the difference between jam, honey,
and marmalade, nor which of several bottles and
one tube in his bathroom was toothpaste. He
confused the mantelpiece and the wardrobe,
passed his wife talcum powder when she asked
for soap, called a scarf an umbrella, and mistook
his wife’s sweater for his shirt. In addition, a
speech therapist’s report from 198s concluded
that C could read aloud with 100% accuracy but
that he complained that words had changed their
meaning. C’s semantic memory was investi-
gated in greater detail in 199 1 and 1992. On the
Graded Naming Test (McKenna & Warrington,
1983) he scored only 2 of a possible score of 30.
Although some of his errors were obviously due
to word-finding difficulties (e.g., “a jail thing
for putting round your wrists” for ‘handcuffs’),
others suggested object recognition difficulties
(see Table 4).
On the semantic memory test (Hodges et al.,
1992), C showed a discrepancy between living
and man-made items, matched for frequency,
with his knowledge of man-made items, al-
though not perfect, being better than his knowl-
edge of living items. C’s scores are a little worse
than those of people with mild to moderate de-
mentia of the Alzheimer type reported by
Hodges et al. (1992).
Several studies, including Warrington and
Shallice ( 1 984) and Hodges et at. ( 1 992b) dis-
cuss whether the semantic memory deficits seen
in their patients are due to a deficit of storage of
information or of access to the semantic memory
store.
Consistency of performance across tests and
across modalities, preservation of superordinate
knowledge. and disproportionate loss of infor-
mation about low frequency items are consid-
ered to be indicators of storage deficits, while
modality-specific deficits and inconsistency
across test sessions indicate access problems.
On the whole, C’s performance supports the
storage disorder hypothesis.
It is also possible that C is slowly forgetting
his general knowledge, perhaps due to the fact
 DENSE AMNESIA FOLLOWING HERPES SIMPLEX
that as the years pass he is no longer rehearsing
information through exposure to conversation,
newspapers, television, and other media. The
qualitative and quantitative changes in the ver-
bal questions of the WAIS and the decrease in
his scores on the NART would support this. In
1985, C made 7 errors on the NART, and, in
1992, he made 20 errors. His predicted
premorbid IQ in 1985 was 122. This is almost
certainly an underestimate given his musical
success and scholarship and the fact that C com-
pleted difficult crosswords daily prior to his ill-
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ness. We know that the NART is unreliable for
people who are significantly above or signiti-
cantly below average (Nelson & Willison,
1991). Even so, it is surprising to find a decline
of 1 1 points over 7 years. The reason for the de-
terioration could well be that he is slowly forget-
ting lexical information. Adler (1944, 1950),
described a patient with apperceptive agnosia,
a n d also d i s c u s s e d t h e p o s s i b i l i t y t h a t
unpractised skills deteriorate over time because
of disuse.
Preoccupation with Yust awakening ’
One of the most striking features of C is his per-
sistent claim that he has only just recovered con-
sciousness. It appears to represent an extremely
powerful and strongly-held belief. At first sight,
i t appears to be readily interpretable; orientation
in time and place depends upon the continuous
operation of episodic memory. In C’s case, the
extent of the active episodic record is limited to
a matter of seconds or, at most, a few minutes.
In the absence of any clear record of anything
earlier than this, apart from distant memories
from the past, C concludes that he has just
emerged from a long period of unconsciousness.
At one level this is an entirely plausible con-
clusion, given the evidence available to C. It
would, therefore, seem to be telling us some-
thing important about sense of self and orienta-
tion in time and place. However, before accept-
ing this conclusion at face value, we should con-
sider two problems. The first is that of why am-
nesic patients do not all report such an experi-
ence. It does appear to be the case that C is quite
strikingly densely amnesic, so i t is possible that
this accounts for the difference, though logically
679
one might perhaps expect less densely amnesic
patients to conclude that they had also just re-
covered consciousness, but perhaps a few hours
rather than seconds ago; this does not appear to
be a common experience.
Even more problematic for a straightforward
interpretation of the phenomenon is the fact that
C appears to be quite impervious to evidence to
the contrary. He has now tilled many notebooks
with literally hundreds of claims to have “just
recovered consciousness”, each followed by a
different time. It seems inconceivable that, faced
with this overwhelming evidence, he does not
consider alternative hypotheses. Furthermore,
when the possibility of profound memory loss is
presented to him as an alternative to his pre-
ferred explanation, he responds by denying it
and showing signs of anxiety and epileptogenic
belching. Similarly, when shown a video of him-
self playing the piano and asked to explain it, he
is at a loss, and again becomes agitated. It is as
if the conviction of reawakening has become an
all-powerful delusion, which like many schizo-
phrenic delusions can be interpreted as an at-
tempt to provide a rational and acceptable solu-
tion to strange and dramatic experiences
(Baddeley, Thornton, Chua, & McKenna, in
press; Maher, 1974). One might well imagine
that, particularly initially, C’s belief that he had
just recovered consciousness was both plausible
and reassuring, compared to the terrible alterna-
tive of acknowledging the enormity of the mem-
ory deficit he had sustained. It seems likely that
the belief has b e c o m e strengthened and
proceduralised by repeated exercise, to a point at
which it blocks out the awful alternative.
What about C’s musical abilities? It is prov-
ing difficult to assess this formally although we
plan to report his apparently preserved musical
skills in a separate paper. Suffice it to say here
that C’s wife believes there has been virtually no
change in his ability to sight-read although his
pianistic skills have declined. She feels that he is
less confident when playing and plays some
pieces at a speed beyond his present capacity to
perform accurately, although premorbidly he
could have played them at that speed with ease.
However, he can sight-read, obey repeat marks
within a short page, and understand the signifi-
 680 BARBARA A. WILSON ET AL.
cance of a metronome mark. He can ornament,
p l a y f r o m a figured b a s s , t r a n s p o s e , a n d
extemporise. To the untrained ear he appears as
musically skillful as ever.
In conclusion, C appears to have a more se-
vere anterograde amnesia than any other patient
previously reported. His retrograde amnesia ex-
tends back for most of his life and he has seman-
tic memory impairments for both visual and ver-
bal material. Despite the extent and severity of
his amnesia, his musical skills appear to b e in-
tact and will b e reported in a subsequent paper.
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