European Journal of Neurology 2001, 8: 643±658

The many faces of crossed aphasia in dextrals: report of nine cases
and review of the literature
P. MarieÈna, S. Engelborghsa, L. A. Vignolob and P. P. De Deyna
a

Department of Neurology, General Hospital Middelheim, Antwerp, Belgium and Laboratory of Neurochemistry and Behaviour, Born-Bunge

Foundation, University of Antwerp, Belgium; and bDepartment of Neurology, Clinica Neurologica della UniversitaÁ, Spedali Civili, Brescia,
Italy

Keywords:

crossed aphasia,
neurolinguistics,
neuropsychology,
review
Received 20 April 2001
Accepted 25 July 2001

A substantial body of the aphasia literature has been devoted to the topic of crossed
aphasia in dextrals (CAD) over the past century but still no theory exists that explains
the anomalous organization of neurocognitive functions in this population. However,
if strict selection criteria are applied only some cases of vascular CAD are reported in
which the correlation between neurocognitive disturbances and the locus of the brain
lesion is studied. This study describes nine new cases of vascular CAD who underwent
in-depth neurolinguistic and neuropsychological investigations. Our analysis shows
the semiological variability of CAD phenomena and the many faces of the lesionbehaviour relationships of this exceptional neurobiological condition.

Introduction
Crossed aphasia in dextrals (CAD) ± i.e. aphasia
following a right hemisphere lesion in right-handers ±
is rare. Notwithstanding its low incidence, which is
variably estimated between 0.38 and 3% (HeÂcaen et al.,
1971; Carr et al., 1981) in patients with vascular lesions,
more than 200 CAD patients have been reported since
the initial description of the concept by Byrom
Bramwell in 1899. Although many issues remain
unsettled, continuous attention for CAD has led to a
substantial increase of insights in the neurobiological
mechanisms underlying the functional neurocognitive
organization of the brain.
One of the most in¯uential and widely held views on
CAD of the last decades was advanced by Brown and
coworkers in the 1970s (Brown and Wilson, 1973;
Brown, 1976; Brown and HeÂcaen, 1976). In accord with
the classical concept of acquired childhood aphasia and
aphasia in sinistrals they de®ned CAD as prototypically
non-¯uent and often transient regardless of the lesion
site (MarieÈn et al., 2001b,c). Many subsequent studies
added evidence to this view supporting the concept of
more di€use language representation as the consequence of a disrupted lateralization process (e.g. Urbain
et al., 1978; Barroche et al., 1979; Goldstein et al.,
1979; Pillon et al., 1979; Taylor and Salomon, 1979;
Donoso et al., 1980; Barroche et al., 1981; Delreux
et al., 1989). Since the advent of advanced neuroimaging techniques the validity of this view has been
challenged. From the 1980s onwards studies have
Correspondence: Peter MarieÈn, A. Z. Middelheim, Department of
Neurology, Lindendreef 1, B-2020 Antwerp, Belgium
(fax: +32-3-281-37-48; e-mail: petermarien@skynet.be).

ã 2001 EFNS

documented in CAD similar aphasia types, lesionbehaviour relationships and recovery patterns as in
uncrossed aphasia (UCA) (e.g. Carr et al., 1981;
Yarnell, 1981; Joanette et al., 1982; Henderson, 1983;
Castro-Caldas and Confraria, 1984; Sweet et al., 1984;
Alexander et al., 1989). However, in comparison with
UCA the number of CAD cases that violate classically
expected lesion-behaviour relationships is disproportionately high. In this respect, review studies show that
<13% of the cases of UCA display anomalous lesionbehaviour correlates (Basso et al., 1985). This proportion increases to 35% in CAD (Alexander et al., 1989;
Coppens and Hungerford, 1998).
This study reports nine CAD patients who developed
aphasia after a vascular lesion of the right hemisphere.
Detailed clinical and neurocognitive data are reported.
The cases presented underscore the variability of
neurocognitive symptoms in CAD and show that
lesion-behaviour relationships even extend beyond the
clear-cut distinction of the mirror-image versus anomalous dichotomy.

Subjects and methods
During the past 10 years 19 right-handed patients with
aphasia following a right hemisphere lesion have been
examined in the neurolinguistic department of A. Z.
Middelheim. Nine of these patients ful®lling the following criteria were selected for the present study: (i) clearcut evidence of aphasia, (ii) aphasia producing lesion in
the right hemisphere ascertained by computerized
tomography (CT), Magnetic Resonance Imaging
(MRI) or post-mortem examination, (iii) vascular etiology, (iv) absence of early brain damage or a history of
seizures in early childhood, (v) absence of familial

643

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P. MarieÈn et al.

sinistrality. Nine of the 19 cases were excluded for the
following reasons: non-vascular aetiology (®ve cases),
familial sinistrality (one case), additional vascular damage to the language areas of the left hemisphere (two
cases), incomplete record lacking information on familial left-handedness, early brain damage and developmental milestones (one case). One case represents CAD
in acquired childhood aphasia and was extensively
reported previously (MarieÈn et al., 2001a; MarieÈn et al.,
2001b,c). All cases withheld for the present study are
adults. Six patients are men, three are women.
In agreement with a three epoch time-frame for
models of aphasia (Mazzocchi and Vignolo, 1979; Basso
et al., 1985; Alexander, 1989) most patients were examined at several periods post-onset. To avoid remote
functional e€ects of the lesion, generally accounting for
the instability of the aphasic pro®le during the ®rst 2 or
3 weeks post-onset (acute phase), formal language
assessments were especially performed in the lesion
phase. This phase (which might last up to 4 months)
o€ers a reliable basis for anatomoclinical correlations
(Mazzocchi and Vignolo, 1979; Alexander, 1989). To
capture various e€ects of recovery and functional brain
reorganization, some patients (n ˆ 3) could also be
examined in the late phase, commencing around
4 months after onset of the neurological symptoms.
The neurolinguistic test battery consisted of a selection of tests including the Dutch version of the
Aachener Aphasie Test (AAT) (Graetz et al., 1992),
the Boston Diagnostic Aphasia Examination (BDAE)
(Goodglass and Kaplan, 1983), the Token Test
(De Renzi and Vignolo, 1962), the Boston Naming
Test (BNT) (Kaplan et al., 1983; MarieÈn et al., 1998), a
phonological and semantic verbal ¯uency task (unpublished tests) and subtests of the Dutch version of the
Psycholinguistic Assessments of Language Processing
in Aphasia (PALPA) (Bastiaanse et al., 1995).
Neuropsychological investigations were based on a
selection of standardized tests. This consisted of: the
Mini Mental State Examination (MMSE) (Folstein
et al., 1975), the Alzheimer's Disease Assessment Scale
(ADAS) (Rosen et al., 1984), the Coloured Progressive
Matrices (Raven, 1976), the Wechsler Adult Intelligence
Scale (WAIS) (Stinissen et al., 1970), the Hierarchic
Dementia Scale (HDS) (Cole and Dastoor, 1987),
the Wechsler Memory Scales (WMS and WMS-R)
(Wechsler, 1945, 1984), Rey's 15 words, the ReyOsterrieth ®gure, the Wisconsin Card Sorting Test
(Grant and Berg, 1993), the Stroop Colour-Word Test
(Lezak, 1983), the Right±Left Orientation test, the
Visual Form Discrimination test and the Judgment of
Line Orientation test (JLO) (Benton et al., 1983).
Handedness was formally assessed with the Edinburgh
Inventory (EI) (Old®eld, 1971).

Case summaries
Table 1 displays a summary of demographic data,
neurolinguistic ®ndings, lesion site and etiology.
Table 2 displays a summary of neuropsychological
®ndings.
Case 1

History
A 55-year-old trans-sexual man developed speech
problems and paresis of the left hemicorpus. On
admission he was well-oriented, alert and co-operative.
Neurological examination revealed expressive aphasia,
moderate left hemiparesis and central facial nerve palsy.
The left plantar response was extensor. Sensory examination disclosed left hypoesthesia. On confrontation
no visual ®eld defects or spatial neglect phenomena were
encountered. Besides a trans-sexual operation, carried
out 3 years before this admission, medical history was
unremarkable. The patient worked as a technician and
had an education level of 13 years. For 10 of 10 unimanual tasks a strong and consistent right hand
preference was indicated and later con®rmed by means
of the EI which yielded a Laterality Quotient (LQ)
of +90. Family history of left-handedness or ambidexterity was negative. Both parents, siblings and the
grandparents were dextrals.
On admission CT scan of the brain did not reveal
abnormalities. Seven days post-onset a repeat CT scan
of the brain showed extensive infarction in the right
parietal region. MRI of the brain (Fig. 1A) performed
5 months after stroke con®rmed the CT ®ndings and
ruled out left hemisphere involvement.
After 2 years the neurological condition had
improved to a discrete residual left hemiparesis. Neuropsychological and neurolinguistic examinations were
performed 6 days, 3, 12 and 24 months after stroke.
Neurolinguistic and neuropsychological ®ndings
Following extensive infarction in the right parietal
lobe, this patient developed persistent non-¯uent
aphasia. In the acute phase, neurolinguistic investigations revealed a dissociation between nearly intact
receptive and markedly disturbed expressive language
functions. Auditory±verbal and written language comprehension were only disturbed for syntactically complex language. In speech as well as in oral reading
phonological disturbances were found. Agrammatism
in speech, writing and oral reading was re¯ected by
telegrammatic style, omission of determinators, deletion of pronomina, deletion of ¯exional morphemes
and derivational errors. The neurolinguistic pro®le,
strongly resembling crossed Broca aphasia with

ã 2001 EFNS European Journal of Neurology 8, 643±658

ã 2001 EFNS European Journal of Neurology 8, 643±658

55/M

63/M

75/F

79/F

38/M

61/M

86/F

62/M

73/M

1

2

3

4

5

6

7

8

9

+++
+++
+++

+++
+++

Day 26
Month 18
Day 1

Day 2
Day 3
Day 9
Day 44
Day 15
Day 31
Day 1
Day 31
Day 18
Month 3
Month 18
Day 6
Day 32
+++
++
++

+++
++

++

+
+
+++
+++
++
+
+
+
+

++
+++

+
+

+++
+++
+

Week 7
Week 16
Admission

+

Compreh.

+++

+++
+
+

Fluency

Day 6
Month 3
Month 12/24
Acute phase

Time post onset

+++
++
+++
+++
++
+
+
+++
++

+++
+++
++

++
++
+++

+++
+++
+

++
++
++
+++

Repetition

+++
+++
++
+
+
+
+++
+++
++
+
+
+++
++

+++

+++
+++

+++

+

Naming

++
+
+++
+++
++
+
+
++
+

+++
+++
+

+

+++

+++
+++
+

+++

+++
+

Reading

+++
++
+++
+++
++
+
+
+++
+++

+++
+++
+

+++
+

+++
+++
+

+++
++
++
+++

Writing

Global aphasia
Global aphasia
Conduction aphasia
Conduction aphasia
Severe Broca aphasia
Broca aphasia
Neologistic jargon aphasia
Neologistic jargon aphasia
Severe Broca aphasia
Broca aphasia, speech apraxia
Broca aphasia, speech apraxia
Conduction aphasia
Conduction aphasia

Dynamic aphasia
Dynamic aphasia
Mutism

Severe Wernicke aphasia
Severe Wernicke aphasia
Minor non-¯uent aphasia

Broca aphasia, agrammatism
Broca aphasia, agrammatism
Severe Broca aphasia
Severe Wernicke aphasia

Aphasia typology

Temporo-parietal

Fronto-temporo-parietal

Temporo-parietal

Fronto-temporo-parietal

Temporal

Caudate nucleus, corona radiata,
external capsule, putamen, globus
pallidus of the lentiform nucleus

Putamen, globus pallidus, parietal
PVWM

Temporo-parietal

Parietal

Lesion site

Infarction

Infarction

Haemorrhage

Infarction

Infarction

Infarction

Infarction

Haemorrhage
(AVM)

Infarction

Cause

Compreh.: comprehension; M: male; F: female; PVWM: periventricular white matter; +++: severely disturbed; ++: moderately disturbed; +: mildly disturbed; not disturbed (blank); AVM:
arteriovenous malformation.

Age/gender

Case

Aphasia symptoms

Table 1 Case summaries: aphasic symptoms, lesion site and cause

The many faces of CAD

645

+
)

)

Month 18
Day 9

Day 44

Day 32

Month 3

Day 31

)

+

)

+

)
+

Week 16
Day 27

Day 32

+
)
)

Dysprosodia

Day 7
Month 5
Week 7

Time post
onset

)

+

+

)

)

+
)

+
+

)
)
+

Visual
neglect

+

+

+

+

+

+
+

)
+

)
)
+

Construct.
apr.

)

)

)

)

)

)
)

)
)

)
)
)

Ideomotor
apr.

)

)

)

)

)

)
)

)
)

)
)
)

Ideational
apr.

)

+

)

)

)

)
)

)
)

)
)
)

Oral
apr.

)

)

+

)

)

)
)

+
+

+
)
+

Anosognosia

+

+

+

)

)

?
)

?
?

+
)
?

Acalculia

Construct.: constructional; apr.: apraxia; M: male; F: female; PVWM: periventricular white matter; +: present; ): absent; ?: no information.

64/F

61/M

6

9

38/M

5

62/M

75/F

3

8

63/M

2

86/F

55/M

1

7

Age/
gender

Case

Table 2 Case summaries: neurocognitive symptoms

Finger agnosia

Memory; VIQ & PIQ

?

None
Discrepancy verbal
>non-verbal;
frontal dysfunctions
Discrepancy verbal
>non-verbal
Memory disturbances

Right/left disorientation
None
Left motor, tactile
& auditory neglect
?
Desinhibition

Additional disturbances

Temporo-temporoparietal infarction
Fronto-parietal
Haemorrhage
Fronto-temporoparietal infarction
Temporo-parietal
infarction

Temporal infarction

Infarction: putamen,
globus pallidus,
& parietal PVWM

Temporo-parietal
haemorrhage

Parietal infarction

Lesion site & etiology

646
P. MarieÈn et al.

ã 2001 EFNS European Journal of Neurology 8, 643±658

The many faces of CAD

receptive and expressive agrammatism, was further
characterized by phonological jargonagraphia and
phonological alexia.
Three months after stroke receptive agrammatism
receded, speaking rate normalized, phonematic paraphasias decreased, letter reading normalized and word
reading improved. The phonological jargon agraphia
had evolved to phonological agraphia. Notwithstanding further improvement, residual agrammatism with
phonematic distortions persisted in oral as well as
written language. After month 9, the agraphic symptoms did not further improve.
As no ®nger agnosia was found, neuropsychological
investigations only disclosed an incomplete Gerstmann's syndrome (acute phase) from which the
patient rapidly recovered. Aside from these focal
dysfunctions no additional neurocognitive de®cits
were found.
Case 2

History
A 63-year-old man was admitted to hospital when
found on the street not being able to stand or making
himself verbally clear. On admission the patient was
alert and co-operative, responded verbally very inconsistently to simple oral commands and produced wellarticulated jargon. He was unable to repeat, read or
write. Neurological examination further revealed a left
hemiparesis. Sensory examination disclosed left hypoesthesia and allesthesia. On confrontation left hemianopia and spatial neglect were found. A CT scan of
the brain showed a large haemorrhage of approximately 4 cm diameter in the right temporo-parietal
region associated with a midline shift to the left
(Fig. 1B).
Within the next 2 days the patient's neurological
condition progressively worsened. A repeat CT scan of
the brain showed marked increase of the intraparenchymal blood collection, increased midline shift and
intraventricular blood. A panarteriography of the
cerebral vessels disclosed an arteriovenous (AV) malformation in the right posterior parietal region. He was
operated and the AV malformation was coagulated.
Information about familial handedness, medical history
and schooling was hetero-anamnestically obtained from
the patient's near relatives (ex-wife and younger
brother). He had 10 years of schooling and was trained
as a bricklayer. He had not had early developmental or
scholastic problems. At age 55 he broke up with his
family and relatives and became homeless. His parents,
both his siblings and his two children were strong
dextrals. Apart from alcohol abuse, medical antecedents were negative.

ã 2001 EFNS European Journal of Neurology 8, 643±658

647

Seven weeks after stroke onset, the patient was
referred to our department. Neurocognitive investigations were performed at week 7 and 16.
Neurolinguistic and neuropsychological ®ndings
Neurolinguistic investigations showed severe and persistent crossed Wernicke's aphasia with auditory±verbal
agnosia, jargonagraphia and jargonalexia. The condition did not improve during 2 months of follow-up.
Auditory±verbal agnosia and severe semantic jargon
persisted in oral±verbal language. Written language
remained disturbed by perseverative jargon. Although
severely limited by the aphasic disturbances, neuropsychological assessments revealed multimodal hemi-neglect, severe constructional apraxia and anosognosia for
neurocognitive and motor dysfunctions.
Case 3

History
A 75-year-old woman was admitted to hospital after
sudden onset of expressive language disturbances. She
was strongly right-handed by self-report and, as later
assessed by the EI, obtained an LQ of +100. There was
no family history of left-handedness or ambidexterity.
On admission, she was alert and co-operative. The
neurological examination revealed non-¯uent aphasia
characterized by reduced verbal output, word-®nding
disturbances and phonemic paraphasias in both oral
and written language. CT scan of the brain on admission
was negative. On day 6 of admission the neurological
condition abruptly deteriorated. Within a few hours the
patient became mute and stuporous. A score of eight
was found on the Glasgow Coma Scale. There was left
hemiplegia with left-sided hyperre¯exia and central
facial nerve palsy. Co-ordination and sensation could
not be tested. Repeat CT and MRI of the brain disclosed
a vascular lesion involving the crus anterior of the
internal capsule, the globus pallidus, the putamen, the
external capsule, the claustrum and the paraventricular
white matter of the right parietal lobe (Fig. 1C). Eight
days later the patient regained consciousness. Pyramidal
symptoms persisted. Severe non-¯uent aphasia and
paralytic dysarthria disrupted communication. Echo
doppler-examination of the carotid arteries disclosed
occlusion of the right internal carotid artery. Eighteen
months after stroke the neurological examination still
showed a severe left sensory-motor syndrome. Neurocognitive investigations were performed at day 27 and
repeated 18 months post-stroke.
Neurolinguistic and neuropsychological ®ndings
At onset, this patient presented mild non-¯uent aphasia. After consistent improvement during the early

648

P. MarieÈn et al.

acute phase, the neurological condition acutely deteriorated and induced permanent non-¯uent aphasia.
Auditory±verbal and reading comprehension were
mildly impaired. Marked adynamia, word-®nding
problems, phonematic errors, reduced sentence length,
dysprosodia and spastic dysarthria characterized spon-

taneous oral±verbal output. In contrast to spontaneous
speech, imposed speech was signi®cantly better preserved. Formal language tests revealed mild anomia,
mild repetition disturbances and normal syntax. Oral
reading was disturbed by phonological and visual errors
and normalized after 18 months. A similar dissociation

ã 2001 EFNS European Journal of Neurology 8, 643±658

The many faces of CAD

Figure 1 (A) T2-weighted brain MRI (case 1) performed
5 months after stroke demonstrates a temporo-parietal infarction
involving homologous Wernicke's area and the angular gyrus of
the right hemisphere. (B) Non-enhanced CT scan of the brain
(case 2) performed in the acute phase shows a large right temporoparietal haematoma surrounded by oedema, a midline shift and an
intraventricular blood level in the left occipital horn of the lateral
ventricle. (C) T1-weighted brain MRI (case 3) revealing a right
subcortical haemorrhagic infarction in the putamen. The lesion
encroached upon the crus anterior of the internal capsule, the
globus pallidus, the external capsule and the claustrum (not shown
on this slice). (D) Non-enhanced CT scan of the brain (case 4)
performed in the acute phase shows an ischaemic right subcortical
lesion involving the caudate nucleus, the internal capsule, the
external capsule and the putamen. (E) Non-enhanced CT scan of
the brain (case 5) performed in the acute phase shows a hypodense
cortico-subcortical lesion in the right temporal lobe. (F) Brain
MRI axial FLAIR (case 6) performed 45 days after onset of the
neurological symptoms shows an extensive fronto-temporo-parietal infarction in the right hemisphere. (G) Brain MRI axial
FLAIR slice (case 7) demonstrates an extensive right temporoparietal infarction, extensive bilateral white matter damage and
di€use cortical atrophy. (H) Brain MRI axial FLAIR (case 8) on
day 25 of admission shows an extensive ischaemic lesion in the
vascular territory of right middle cerebral artery. (I) Brain MRI
axial FLAIR (case 9) performed 30 days after the stroke demonstrates an ischaemic lesion in the right temporo-parietal region
involving the entire lateral part of the temporal lobe, the external
capsule and the subcortical white matter of the parietal lobe.

between self-generated and imposed language characterized written language. After 18 months, the patient
was still not able to write anything spontaneously
although agraphic symptoms had clearly improved.
Neuropsychological assessments disclosed left-sided
neglect, a€erent dysgraphia and constructional apraxia.
These symptoms improved but did not resolve after
18 months.

649

lesion involving the parietal white matter of the right
hemisphere. The lesion encroached upon the basal
ganglia.
99m
Tc-HMPAO SPECT of the brain performed on
the day of admission showed marked decrease of rCBF
in the right hemisphere including the basal ganglia and
the fronto-temporo-parietal region with slight occipital
extension. Crossed cerebellar diaschisis was re¯ected by
an area of diminished perfusion in the left hemisphere
of the cerebellum. No perfusional de®cits were encountered in the left cerebral hemisphere.
A neurolinguistic bed-side screening on the second
day of admission evidenced a deterioration of language
functions. Auditory±verbal comprehension was, in
contrast to the day before, limited to elementary daily
language. She could only respond to simple questions
and was not able to express herself neither on command
nor spontaneously. There was no automatic, overlearned language use. Repetition was impossible. All
she was able to produce were some undi€erentiated,
poorly articulated, dysarthric grunts. Reading comprehension was totally disturbed and writing resulted in
perseverative scribbles. On day 3 of admission she
became globally aphasic. Consciousness remained normal. The next day her physical condition worsened
progressively as a result of pneumonia. Within the next
few days she became comatose. She deceased on day 13
of admission.
Post-mortem examination was performed. Gross
inspection of the brain after formalin ®xation showed
haemorrhagic transformation of the ischaemic lesions.
These involved the head, body and tail of the caudate
nucleus, the corona radiata, the external capsule and
the entire putamen. The genu and posterior limb of the
internal capsule were extensively damaged (Fig. 2A±C).
Macroscopical examination of the left hemisphere did
not reveal vascular damage.

Case 4

History
A 79-year-old right-handed woman with 9 years of
schooling acutely developed mutism and left hemiplegia
after a subcortical right hemisphere stroke. On admission the neurological examination additionally disclosed left homonymous hemianopia. The patient was
alert and responded adequately to verbal instructions
but could not speak. Any attempt to speak resulted in
mere grunting. She could read and write. Non-verbal
responses to the EI revealed an LQ of +100. Near
relatives con®rmed this strong and consistent right
hand preference. Familial antecedents of left-handedness, evidence of early brain damage or a history of
seizures were excluded. CT scan of the brain on
admission (Fig. 1D) revealed a hypodense subcortical

ã 2001 EFNS European Journal of Neurology 8, 643±658

Case 5

History
A 38-year-old man was admitted to hospital after acute
onset of left-sided weakness and speech diculties. On
admission the patient was alert, well-oriented and
co-operative. His speech was ¯uent but contained many
aphasic errors. Auditory±verbal comprehension was
normal. Mild left sensory-motor de®cits were found.
On confrontation no visual ®eld defects or spatial
neglect phenomena were encountered. Apart from some
minor stroke-like episodes during the week prior to
admission, medical history was unremarkable. He was
fully right-handed on the EI (LQ + 100) and had no
familial antecedents of left-handedness. His educational
level was 12 years. He was employed as a clerk in a ®rm.

650

P. MarieÈn et al.

Neurolinguistic and neuropsychological ®ndings
Nine days after onset of the neurological symptoms,
formal language assessments in this patient revealed
conduction aphasia with severe anomia. Auditory±
verbal and written language comprehension were normal. Notwithstanding some phonological errors, oral
reading and spelling were within the normal range.
Oral±verbal language, however, was disproportionately impaired. Phonemic paraphasias, phonemic
approximations, numerous self-corrections and severe
anomia disturbed otherwise ¯uent speech. Imposed
speech was primarily a€ected by marked repetition
disturbances with relatively intact number repetition.
Severe constructional and drawing apraxia, modalityspeci®c impairments of memory, attention and frontal
lobe functions characterized the neuropsychological
pro®le. In the lesion phase formal language assessments
did not disclose severe residual de®cits. Mild phonological disturbances persisted, however, in self-generated
speech. Frontal lobe dysfunctions (problem solving)
resolved in the lesion phase but constructional and
drawing apraxia as well as visual memory and attention
problems remained.
Case 6

Figure 2 Serial 1.0-cm-thick coronal sections (case 4) from rostral
to caudal (A,B,C) show haemorrhagic transformation of the right
hemispheric ischaemic lesions involving the head, body and tail of
the caudate nucleus, the genu and posterior limb of the internal
capsule, the corona radiata, the external capsule and the entire
putamen.

CT scan of the brain 2 days after admission
revealed a hypodense right cortico-subcortical temporal lesion (Fig. 1E). MRI of the brain ruled out
structural damage of the left hemisphere. Neurocognitive assessments were performed 9 days and 44 days
after stroke.

History
A 61-year-old right-handed house-painter with 8 years
of schooling was admitted to hospital for intens
retrosternal pain. A transmural anterior myocardial
infarction was diagnosed. Medical antecedents were
negative. During the ®rst night of admission he
developed severe aphasia and left hemiplegia as the
result of a haemorrhagic right hemisphere stroke. The
neurological examination additionally showed gross
auditory±verbal comprehension disturbances, incomprehensible speech, left hemianopia and spatial neglect.
No reliable information was obtained regarding sensory functions. A CT scan of the brain disclosed
an extensive right fronto-temporo-parietal hypodense
lesion with slight mass e€ect. A focal hyperdense area
indicated a haemorrhagic component. MRI of the
brain performed 45 days after the onset of ictus
con®rmed the structural integrity of the left hemisphere
(Fig. 1F).
The EI indicated strong right hand preference
(LQ + 80). There were no familial antecedents of
left-handedness. After 1 month the hemianopia and
visual neglect phenomena had resolved. However, a
predominantly non-¯uent aphasia, hemiparesis and
hypoesthesia persisted. Language was assessed 15 and
31 days after stroke. Neuropsychological examinations
were performed on day 32.

ã 2001 EFNS European Journal of Neurology 8, 643±658

The many faces of CAD

651

Neurolinguistic and neuropsychological ®ndings
Following a haemorrhagic stroke in the posterior right
hemisphere this 61-year-old dextral patient developed
severe Broca aphasia, expressive agrammatism, paralytic dysarthria, dysprosodia and mild a€erent dysgraphia. Disrupted syntactic knowledge only induced on
high linguistic complexity levels auditory±verbal comprehension disturbances. Reading comprehension,
however, was normal. Phonological disturbances distorted visual confrontation naming. Verbal ¯uency was
reduced. Spontaneous speech was limited to fragmented
attempts and characterized by numerous phonemic
paraphasias, agrammatism, word-®nding disturbances,
articulatory distortions and long pauses. During
follow-up the aphasia ameliorated. However, 1 month
after onset of stroke self-generated speech was still
dys¯uent, agrammatic and characterized by phonemic
paraphasias. Repetition had not normalized. Dysarthric
symptoms had receded but prosody remained abnormal. After remission of phonological errors oral reading also normalized. During follow-up the a€erent
dysgraphia ameliorated.
Neuropsychological investigations revealed visuoconstructive apraxia and disturbed visual memory.

paresis. The left plantar response was extensor. Left
sensory disturbances persisted. On confrontation left
hemianopia was found. 99mTc-HMPAO SPECT of the
brain disclosed hypoperfusion of the entire vascular
territory of the right middle cerebral artery. Relative
hypoperfusion of the left cerebellar hemisphere was
attributed to crossed cerebellar diaschisis. No perfusional de®cits were observed in the left hemisphere.
Neurocognitive assessments were performed 31 days
after stroke.

Case 7

Case 8

History
An 86-year-old woman was admitted to hospital the
day she developed severe aphasia and left hemiplegia.
On admission she was confused and disoriented.
In addition to ¯uent aphasia and left hemiplegia the
neurological examination indicated left sensory loss
and left hemianopia with visual neglect. Medical
history included arterial hypertension. A grand-daughter, who lived together with the patient, reported
onset of mild memory diculties 2 months prior to
this admission. These had no major repercussions on
the patient's activities of daily life. The patient was
said to be a strong dextral and had no familial history
of left-handedness. An LQ of + 100 was heteroanamnestically obtained. CT scan of the brain on the
third day of admission revealed an extensive temporoparietal hypodense lesion encroaching upon the basal
ganglia of the right hemisphere. MRI of the brain
additionally disclosed di€use cortical atrophy and
extensive white matter damage in both cerebral
hemispheres (Fig. 1G).
One month after onset of symptomatology she was
referred to our department. The patient was welloriented and co-operative. Aside from neologistic
jargon aphasia, the neurological examination revealed
a ¯accid left hemiparesis (4/5) with hyperre¯exia,
Ho€mann±TroÈmner sign and central facial nerve

History
A 61-year-old patient with 11 years of schooling
developed left hemiplegia and aphasia the night before
admission. On admission he uttered some undi€erentiated sounds and displayed severe comprehension disturbances. Reading and writing was not possible. The
neurological examination additionally revealed a left
central facial nerve palsy, left hemiparesis and left-sided
hyperre¯exia. The left plantar response was extensor.
The patient's medical history included arterial hypertension, acute myocardial infarction, coronary artery
bypass and a surgically treated aneurysm of the
abdominal aorta. He was strongly right-handed
(LQ + 100) and had no familial tract of left-handedness. CT scan of the brain on the day of admission
showed some old periventricular lacunar infarctions.
Four days later repeat CT disclosed an extensive
ischaemic lesion in the right temporo-parieto-occipital
region. Clinically relevant damage to the left hemisphere was excluded by means of brain MRI (Fig. 1H).
Doppler duplex echography of the cervical blood
vessels showed thrombosis of the right internal carotid
artery as con®rmed by means of MR angiography.
Formal neurolinguistic tests performed 18 days, 3 and
10 months after stroke revealed persistent non-¯uent
aphasia. Neuropsychological assessments were performed 3 months after stroke.

ã 2001 EFNS European Journal of Neurology 8, 643±658

Neurolinguistic and neuropsychological ®ndings
This patient developed neologistic jargon aphasia.
Auditory±verbal comprehension was grossly disturbed
at basic linguistic levels. Speech mainly consisted of
perseverative neologistic jargon intermingled with
Dutch and French words. Articulation and prosody
were preserved. Repetition, visual confrontation
naming and syntax were severely distorted. Because of
the severity of the aphasia, extensive neuropsychological investigations could not be performed. However,
a concise psychometric screening revealed total acalculia, left visuo-spatial neglect, constructional apraxia
and anosognosia.

652

P. MarieÈn et al.

Neurolinguistic and neuropsychological ®ndings
In the acute phase this patient showed severe non-¯uent
aphasia associated with expressive and receptive
agrammatism, alexia and agraphia. In the lesion phase,
language assessments revealed moderate auditory-verbal comprehension disturbances. Agrammatic errors,
phonematic paraphasias, sound distortions and disrupted speech. Visual confrontation naming and repetition
were most impaired at the sentence level. Left visual
neglect errors and phonemic paralexias characterized
oral reading. Reading comprehension was mildly
disturbed for words and grossly defective for sentences.
Dictational writing was mildly a€ected. Apart from a
slight improvement of grammatical competence, the
aphasic disturbances did not resolve during follow-up.
Ten months post-stroke moderate Broca aphasia
with mild speech apraxia and agrammatism persisted.
Neuropsychological investigations disclosed pathological verbal and performance IQ levels, disturbed visual
and verbal memory, constructional apraxia and left
visuo-spatial neglect.
Case 9

History
A 73-year-old right-handed man was admitted to
hospital with angina pectoris. Four days after the
patient underwent a coronary artery bypass graft, he
developed left hemiparesis and near mutism associated
with good comprehension. Atrial ®brillation was diagnosed. The clinical neurological examination revealed
left central facial nerve palsy and left hemiparesis.
MRI of the brain 30 days after the stroke showed an
ischaemic lesion in the right temporo-parietal region
involving the entire lateral part of the temporal lobe,
the external capsule and the subcortical white matter of
the parietal lobe (Fig. 1I). Left hemiparesis and facial
nerve palsy spontaneously recuperated within a few
days.
Medical history consisted of arterial hypertension.
The patient was fully right-handed (LQ + 100) and
had no familial antecedents of left-handedness. Intensive physical therapy and speech and language training
was started which resulted in almost complete recuperation of motor symptoms and partial amelioration
of aphasia. Neurocognitive examinations were performed six and 32 days after onset of neurological
symptoms.
Neurolinguistic and neuropsychological ®ndings
At onset this patient developed severe conduction-like
aphasia with total agraphia. Auditory±verbal comprehension was mildly disturbed. Oral±verbal output
consisted of a severe lack of nouns, numerous `condu-

ites d'approches' and phonemic paraphasias. Articulation was not disturbed and prosody and syntax were
normal. He could not repeat. Although he could
comprehend the meaning of written words, oral reading
was impossible. There was total agraphia. During the
lesion phase the aphasia evolved to typical conduction
aphasia. Reassessment of spoken language 25 days later
revealed normalization of written comprehension.
Persistent de®cits remained at all linguistic levels
(auditory±verbal comprehension, speech, written
language). On the neuropsychological level a partial
Gerstmann syndrome accompanied mild constructional
apraxia. Verbal memory could not be evaluated. Visual
memory was normal.

Results
Neurolinguistic ®ndings

Neurolinguistic information was obtained in the acute
phase for the entire group of nine cases. In-depth
language assessments were performed in the lesion
phase in eight patients (case 1, 2, 3, 4, 6, 7, 8, 9). One
patient deceased in the acute phase (case 4). Four
patients had a longitudinal follow-up extending beyond
the lesion phase for more than 1 year (case 1, 3, 8).
Five patients presented non-¯uent aphasia (three
men, two women), four showed ¯uent aphasia (two
men, two women). The non-¯uent group comprises
three patients with Broca aphasia (case 1, 6, 8), one
patient with dynamic aphasia (case 3) and one patient
with global aphasia (case 4). Two patients with conduction aphasia (case 5, 9), one patient with Wernicke
aphasia (case 2) and one patient with neologistic jargon
aphasia (case 7) belong to the ¯uent group. Mean age
distribution between the non-¯uent and ¯uent group
was not di€erent (66.8 and 65 years, respectively).
All patients displayed the most pronounced aphasic
de®cits in the acute phase. The aphasic pro®le of
patient 3, who showed mild non-¯uent aphasia at onset
and more severe dynamic aphasia on day 26, is not an
exception to this observation because language functions acutely deteriorated on day 6 as the consequence
of a progressive stroke. Neurolinguistic data of the
eight patients examined in the subsequent lesion phase
showed that aphasia did not resolve rapidly. Two
patients had no change of aphasic symptoms (case 2, 7).
Four patients (case 1, 5, 6, 8) had improved at all
linguistic levels: ¯uency, auditory±verbal comprehension, repetition, naming, reading and writing. Two
patients displayed modality-speci®c improvements (case
3, 9). In contrast to oral language, written language
functions ameliorated in case 3. The reversed pattern
was found for case 9. In this case oral but not written

ã 2001 EFNS European Journal of Neurology 8, 643±658

The many faces of CAD

language improved. All four patients in whom formal
language assessments were performed in the late phase
displayed residual aphasic de®cits. Mild Broca aphasia
persisted in case 1, moderate Broca aphasia in case 8
and severe dynamic aphasia in case 3. Lesion phase
data further disclosed a dissociated pattern of impairment between oral and written language in three
patients (case 1, 3, 9). In these patients spelling was
disproportionately more a€ected than oral language.
Oral and written language were equally disturbed in the
other four patients.
Five of the eight patients examined in the lesion
phase had lesion-behaviour relationships comparable
with those following an analogous lesion in the left
hemisphere and are therefore mirror-image representatives (case 2, 3, 5, 7, 9). Case 2 developed persistent
Wernicke aphasia following a temporo-parietal lesion.
Patient 3 presented dynamic aphasia and spastic
dysarthria after a progressive subcortical stroke (basal
ganglia and paraventricular white matter of the
parietal lobe). Patient 5 developed conduction aphasia
in association with a cortico-subcortical temporal
lesion. Patient 7 showed neologistic jargon aphasia
after an extensive temporo-parietal lesion and patient 9
displayed conduction aphasia following damage to the
entire lateral part of the temporal lobe, the external
capsule and the subcortical white matter of the
parietal lobe. The lesion-aphasia pro®le shared by
the other three patients is anomalous because unexpected symptoms were found given the lesion site (case
1, 6, 8). Patient 1 showed predominantly non-¯uent
aphasia in association with a posteriorly located right
hemisphere lesion involving Wernicke's area proper.
Patients 6 and 8 developed Broca aphasia with relative
sparing of auditory±verbal and reading comprehension
rather than global aphasia despite large perisylvian
lesions causing destruction of the classical language
areas.

653

Left visual neglect characteristics were present in four
patients (case 2, 3, 7, 8). One patient displayed in
addition to visual neglect, severe left motor, tactile and
auditory inattention (case 2).
Gnostic de®cits were encountered in four patients
(case 1, 2, 3, 7). All these patients displayed severe
anosognosia. Finger agnosia was found in two patients
(case 2, 7). Visual agnosia was not found in our study
sample. All patients with longitudinal neuropsychological follow-up, recovered agnostic symptoms (case 1, 3).
Calculation was not tested in two patients (case
2, 3). Four patients presented acalculia (case 1, 7, 8, 9).
Partial Gerstmann syndrome (disturbed right±left discrimination, ®ngeragnosia, acalculia, agraphia) with
three of four cardinal symptoms present was found in
two patients (case 1, 9).
In three patients a Wechsler IQ was assessed in the
lesion phase (case 1, 7, 8). One patient had an entirely
normal IQ pro®le (case 1). One patient had defective IQ
levels with verbal and performance capacities equally
a€ected (case 8). One patient had a normal VIQ and a
pathological PIQ (case 5). Spatial reasoning was
examined in two patients by means of the Coloured
Progressive Matrices (case 3, 9). Patient 3 performed at
a very low level (percentile 10), patient 9 obtained a
normal score (percentile 50).
Memory was formally evaluated in six patients. Five
patients were subjected to the Wechsler Memory Scale
(case 1, 5, 6, 8, 9), one patient to the memory subtests of
the Hierarchic Dementia Scale (case 3). Two patients
had normal memory (case 1, 3). Two patients displayed
a signi®cant discrepancy between normal verbal memory and defective visual memory (case 5, 6). Patient 9
displayed the reversed pro®le characterized by normal
visual memory and inassessible verbal memory functions. Memory was equally distorted at verbal and
visual levels in one patient (case 8).

Discussion
Neuropsychological ®ndings

In all but one patient (case 4) neuropsychological
investigations were performed in the lesion phase. Two
patients were also extensively tested in the acute phase
(case 1, 5) and two patients were re-examined in the late
phase (case 1, 5). Severe ¯uent aphasia in two patients
rendered extensive psychometric investigation impossible (case 2, 7). However, minimal neurocognitive
information on praxis, visuo-spatial functions, and
gnosis was obtained in all cases.
None of the patients under consideration showed
ideomotor or ideational apraxia. Oral apraxia was
present in one patient (case 8). Seven patients had
constructional apraxia (case 2, 3, 5, 6, 7, 8, 9).

ã 2001 EFNS European Journal of Neurology 8, 643±658

All but one of the nine cases (case 8) presented ®t
current criteria for a diagnosis of vascular CAD:
(i) aphasia evidenced, (ii) aphasiogenic lesion of vascular origin in the right hemisphere, (iii) strong right-hand
preference with no familial antecedents of left-handedness, (iv) structural integrity of the left hemisphere
ascertained, and (v) absence of early brain damage.
In patient 8, extensive ischaemic damage in the periand para-ventricular white matter of the left hemisphere
casts doubt on a clear-cut CAD diagnosis. We did not
exclude this case for the present study because no direct
clinical evidence was found for the relevance of these
lesions on the aphasic symptoms: (i) the patient did not
display any type of subcortical aphasia compatible with

654

P. MarieÈn et al.

the subcortical localization of the lesions in the left
hemisphere (Alexander, 1989), (ii) the typical region of
destruction of the patient's aphasia (neologistic jargon
aphasia) was not damaged in the left but in the
right hemisphere (Wernicke's area) (Buckingham and
Kertesz, 1976) and (iii) in accordance with the ®ndings
of high performance MRI studies (Awad et al., 1986;
Hunt et al., 1989; Fazekas et al., 1991; Fukui et al.,
1994; Pantoni and Garcia, 1995) the subcortical left
hemisphere lesions observed in this 86-year-old patient
might represent clinically silent neuroradiological phenomena attributable to high age. Moreover, the fact
that the aphasia in this patient occurred simultaneously
with a left sensory±motor syndrome, has been considered in several prior CAD reports of patients with
additional lesions in the left hemisphere a convincing
point in favour of the aphasiogenic power of the right
hemisphere damage (e.g. Yokoyama et al., 1981; Sweet
et al., 1984; Alexander et al., 1989; Caramelli et al.,
1994). Related to this issue, Castro-Caldas et al. (1986)
called attention to the importance to carefully control
for aetiology when a diagnosis of CAD is suspected.
They reported a right-handed patient with post-traumatic conduction aphasia in whom the CT scan only
disclosed a right frontal lesion. The EEG, however,
additionally revealed a left occipito-temporal dysfunction that accounted for the aphasic manifestations. As a
result, Castro-Caldas et al. (1986) cautioned against a
possible bias of including traumatic cases in series of
CAD and attributed the reported much higher incidence of CAD in traumatic patients (e.g. 18% in the
Mohr et al., 1980 study) to undetected contrecoup
lesions in the left hemisphere.
Regarding the overall group characteristics, our
group of nine vascular CAD patients seems to
constitute a highly representative sample of some
recently changed insights in CAD population characteristics. In comparison with patients with UCA, most
studies of the 1970s and the early 1980s unequivocally
emphasized the relative young age of CAD patients
(Boller, 1973; Brown and HeÂcaen, 1976; Urbain et al.,
1978; Goldstein et al., 1979; Donoso et al., 1980;
Joanette et al., 1982). Brown and HeÂcaen (1976), for
instance, reported in their review of 29 unselected
CAD patients a mean age of 44.7 (SD 14.0) years.
Subsequent studies on larger population samples did
not con®rm CAD patients to be relatively younger
than UCA patients. In their comparative analysis of
39 vascular CAD patients with 390 UCA patients,
Castro-Caldas and Confraria (1984) found an average
age of 57.4 years for both groups and attributed the
contrasting ®ndings of prior studies to the inclusion of
patients with non-vascular aetiology. Donoso (1984)
and Coppens and Hungerford (1998) reported com-

parable mean age ®gures of 57.4 and 57.3 years,
respectively, for adult CAD. The mean age of our
group (65.7 years) is apparently higher than the ®gures
previously reported. Although our limited sample size
does not allow rigid conclusions regarding this di€erence, the mean age ®gure supports the view that
vascular CAD patients are not by de®nition younger
than UCA patients and seems to indicate that age is
not a distinctive feature of vascular adult CAD. In the
late 1970s also, the suggestion that the degree of left
hemisphere dominance for language is lower in women
than in men (Brown and HeÂcaen, 1976; Andrews,
1977; McGlone, 1977) established the view that CAD
should be more frequent in women (Urbain et al.,
1978; Donoso et al., 1980). Several later studies,
however, consistently observed the opposite gender
di€erence (Joanette et al., 1982; Castro-Caldas and
Confraria, 1984; Donoso, 1984; Coppens and
Hungerford, 1998). Castro-Caldas and Confraria
(1984), for instance, showed that the male to female
ratio was higher in their group of CAD patients
(32 men and seven women) than in their group of
UCA patients (229 men and 161 women). The present
sample of patients includes six men and three women
which contradicts the prior opinion that the CAD
population consists of more women than men.
In further contrast to the in¯uencial view advanced in
the 1970s (Brown and Wilson, 1973; Brown, 1976;
Brown and HeÂcaen, 1976; Urbain et al., 1978; Barroche
et al., 1979; Goldstein et al., 1979; Pillon et al., 1979;
Donoso et al., 1980; Barroche et al., 1981; Delreux
et al., 1989) our data support the view that CAD does
not consist of a semiologically uniform syndrome but of
a variety of symptoms comparable with UCA (Carr
et al., 1981; Joanette et al., 1982; Henderson, 1983;
Castro-Caldas and Confraria, 1984; Alexander et al.,
1989; LanoeÈ et al., 1992; Coppens and Hungerford,
1998). In our patient group a broad and rich spectrum
of non-¯uent and ¯uent symptoms characterized the
aphasia pro®les in the acute, lesion and late phase. In
the lesion phase, ®ve of the eight patients examined
displayed non-¯uent aphasia and three ¯uent aphasia.
This non-¯uent to ¯uent ratio of 62.5 to 37.5% is
comparable with the percentages found by Carr et al.
(1981) (72 versus 27%) who in addition referred to
similar distribution ®gures for UCA. Our lesion phase
data add further evidence to Castro-Caldas and Confraria's ®ndings (1984) that CAD and UCA populations
are comparable in terms of aphasia type distribution
with Broca aphasia as the most frequent type. A similar
pattern of age di€erences among vascular UCA and
CAD patients was also found in our population with
Broca aphasics being younger than Wernicke aphasics
(59.5 vs. 74.5 years, respectively). However, mean age

ã 2001 EFNS European Journal of Neurology 8, 643±658

The many faces of CAD

distribution between our non-¯uent and ¯uent group of
adult patients did not di€er (66.8 and 65 years,
respectively).
In contrast to the long-standing opinion that CAD is
essentially a transient phenomenon (Bramwell, 1899;
Holmes and Sado€, 1966) or that it recovers much
better and faster than UCA (Foroglou et al., 1975;
Brown, 1976; Brown and HeÂcaen, 1976; Urbain et al.,
1978; Goldstein et al., 1979; Donoso et al., 1980;
Pita et al., 1997; OÈzeren et al., 1998) Coppens and
Hungerford (1998) recently showed that the proportion
of CAD cases associated with rapid recovery only
represented a minority (< 9%) of the 114 reviewed
cases with a vascular aetiology. These authors additionally demonstrated that the cases with transient
aphasia shared no other common characteristics than
their rapid and favourable outcome. In none of our
patients the aphasic syndrome receded rapidly or
completely. All but two patients (cases 2 and 7)
improved after the acute phase. Four patients (case 1,
5, 6, 8) improved at all linguistic levels and two patients
displayed modality-speci®c improvements (case 3, 9).
All three patients with a follow-up in the late phase
(case 1, 3, 8) still displayed aphasic de®cits of variable
severity. Mild Broca aphasia persisted in patient 1,
moderate Broca aphasia in patient 8 and severe
dynamic aphasia in patient 3.
Although striking semiological resemblances and
highly comparable population characteristics hold
between UCA and CAD, our data clearly demonstrate
that CAD cannot be merely viewed as the mirror-image
pattern of UCA in the opposite hemisphere (Joanette
et al., 1982). Five of the eight patients (62.5%) examined in the lesion phase had lesion-behaviour relationships comparable with those following an analogous
lesion in the left hemisphere and could therefore
be considered mirror-image representatives (case 2, 3,
5, 7, 9). The lesion-aphasia pro®le shared by the three
other patients (37.5%) was anomalous because unexpected aphasia types were found, given the site of the
lesion (case 1, 6, 8). As a consequence, the number of
cases that violate classically expected lesion-aphasia
correlates is much higher than the degree of anatomoclinical variability encountered in UCA populations
(e.g. less than 13% in the Basso et al., 1985 study).
Patient 1 showed Broca aphasia in association with a
posterior lesion involving the region homologous to
Wernicke's area proper in the right hemisphere.
Patients 6 and 8 developed Broca aphasia (and not
global aphasia) in association with a large perisylvian
lesion causing destruction of the homologous classical
language areas in the right hemisphere. This pattern
seems to indicate that language functions in these
patients are represented bilaterally: expressive language

ã 2001 EFNS European Journal of Neurology 8, 643±658

655

functions in the right hemisphere and receptive language functions in the left hemisphere. A similar
hypothesis of dissociated bilateral language representation might be advanced for the other anomalous CAD
case with a focal posterior lesion and non-¯uent
aphasia (case 1). However, the fact that in this patient
the aphasiogenic lesion spared the anterior part of the
right hemisphere leaves open the possibility of an
intra-hemispheric inversion of the language centres in
the right hemisphere (expressive language located
posteriorly and receptive language anteriorly). Although more research on larger CAD populations is
needed to correlate type of aphasia with lesion-aphasia
con®gurations, the observation that all four patients
with an anomalous lesion-aphasia pro®le are of the
non-¯uent type is striking and contrasts with Alexander
et al.'s ®nding (1989) that all 12 anomalous cases
(35.3%) identi®ed in a series of 34 published CAD cases
had ¯uent aphasia. However, it needs to be emphasized
that correlations with Alexander et al.'s group (1989)
are dicult because the operational criteria applied for
CAD were di€erent and the assessment and de®nition
of lesion-aphasia pro®les not based on the same
parameters. The same holds for a comparison with
the 26 anomalous CAD cases (35.1%) identi®ed by
Coppens and Hungerford (1998) in their review of 114
cases with vascular aetiology, but it seems that in this
series non-¯uent aphasias are overrepresented as well.
An interesting line of future research in the understanding of the mechanisms underlying language organization might consist of a further speci®cation and
delineation of the anatomoclinical con®gurations in
anomalous CAD by means of a methodological stringent approach that incorporates in addition to aetiological and time based factors also uniform parameters at
the linguistic description level.
On the neuropsychological level our observations
con®rm that a variety of neurocognitive de®cits may
accompany CAD. Castro-Caldas et al. (1987) observed
that in contrast to a lower incidence of ideomotor
apraxia (17%), the incidence of oral apraxia in acute
CAD (52%) is comparable with its incidence in acute
UCA (60%). These observations led to the view that
oral praxis tends to be lateralized in close functional and
anatomic association with language and ideomotor
skills with hand preference. Our data, however, demonstrate that the mechanisms subserving oral praxis are
dissociable from oral language since oral apraxia was
only present in one patient with non-¯uent aphasia (case
9). Typical non-dominant hemisphere symptoms such
as left visuo-spatial neglect and visuo-constructional
apraxia have been reported to represent frequent phenomena in CAD. Castro-Caldas et al. (1987) found in
82% of the 66 reviewed CAD cases left visuospatial

656

P. MarieÈn et al.

neglect, an incidence which is twice as high the reported
frequency of 33±46% in cases with right hemisphere
lesions and left hemisphere language dominance (Hier
et al., 1983). Lesion phase data obtained in our sample
do not con®rm the view that neglect is a much more
frequent phenomenon in CAD as it was only observed in
four patients (case 2, 3, 7, 8). The relatively rare presence
of left-sided neglect in our study group contradicts
Castro-Caldas et al.'s hypothesis (1987) that in CAD
the right hemisphere subserves both visuo-spatial functions and language and seems to suggest that in
anomalous dextrals the mechanisms underlying attention and language may lateralize independently of one
another to either hemisphere. On the other hand, the
fact that seven of the eight patients in our series
displayed symptoms of constructional apraxia (case 2,
3, 4, 6, 7, 8, 9) agrees with the ®ndings of Castro-Caldas
et al. (1987) that the 76% ®gure for constructional
apraxia in CAD is signi®cantly higher than the number
of 45% reported for UCA. The observation that all
patients with visuo-spatial neglect presented with constructional apraxia and that only three patients had
constructional apraxia in isolation re¯ects the double
nature of constructional apraxia as a visuo-spatial
induced (case 2, 3, 7, 8) and as a genuine apraxic
phenomenon (5, 6, 9). As this distinction is rarely
reported in the CAD literature the incidence of constructional apraxia as a genuine apraxic disturbance
might have been overestimated in prior reports.
In the literature on CAD to date there is little
reference to other neurocognitive areas such as gnosis,
calculation, orientation, memory and intelligence. Our
data, however, seem to suggest that a more systematic
approach of these functions might contribute to the
further understanding of CAD. Most of the patients in
the present study displayed severe modality-speci®c
gnostic de®cits: anosognosia (case 1, 2, 3, 7), ®nger
agnosia (case 2, 7) and auditory±verbal agnosia (case 2).
Recovery of gnostic functions was complete in both
patients with longitudinal neuropsychological follow-up
(case 1, 3). The majority of patients in whom calculation was formally tested (4 of 6) showed arithmetic
disturbances (case 1, 7, 8, 9). Partial Gerstmann's
syndrome (disturbed right±left discrimination, ®ngeragnosia, acalculia, agraphia) with three of four cardinal
symptoms present was found in two patients (case 1, 9)
in the acute phase. No consistent pattern was found in
the Wechsler IQ and Wechsler memory pro®les of the
patients examined with these tests. One patient had a
normal IQ (case 1). One patient (case 8) displayed a
general decline of intelligence whereas another patient
had a signi®cant asymmetry between the verbal and
performal IQ: VIQ > PIQ (case 5). Two patients
subjected to the Wechsler Memory Scale obtained

normal results (case 1, 3). A generalized deterioration of
verbal and visual memory was found in patient 7. Two
patients displayed a modality-speci®c decrease: verbal
memory was superior to visual memory in cases 5 and 6
while the reverse pattern was found in case 9. This study
does not allow to draw ®rm conclusions about the
incidence and signi®cance of accompanying neurocognitive symptoms in CAD but it may be interesting to
note that aphasia did not by de®nition disrupt verbally
mediated neurocognitive functions such as verbal intelligence and verbal memory.
The neurolinguistic and neuropsychological data of
the ®rst patient of this study illustrate the relevance of
our plea for a more systematic approach of neuropsychological functions in CAD and add evidence to
the importance of this approach in the identi®cation of
brain-behaviour relationships in anomalous dextrals.
In this patient with an anomalous inter- and intrahemispheric lesion-aphasia pro®le (crossed Broca aphasia after a parietal infarction), in-depth neurocognitive
assessments disclosed a con®guration of symptoms
suggesting a complete situs inversus of neurocognitive
functions. In the ®rst place, a partial crossed Gerstmann's syndrome (Gerstmann, 1940) was found after
destruction of the typical lesion area (left parietal
region with the angular gyrus crucially involved) in the
right hemisphere. The fact that this syndrome was
caused by a homologous lesion in the opposite hemisphere adds evidence to the dominant qualities of the
right hemisphere and seems to implicate that on the
intrahemispherical level at least some dominant parietal
brain functions were not organized anomalously. In
addition, the absence of typical `minor' hemisphere
symptoms such as left neglect and visuo-spatial de®cits
indicates that attentional and visuo-spatial functions
were not typically subserved by the posterior right
hemisphere. However, it remains unsettled whether
these functions were strongly lateralized in the `minor'
left hemisphere or represented anomalously in the
structurally intact parts of the right hemisphere.
Although it is plausible to believe that a single gene is
responsible for the laterality of many brain functions
(Alexander and Annett, 1996), no theory of CAD to
date can suciently account for the dissociations in the
intra-hemispherical organization of the dominant hemisphere functions in this patient. Future studies are
needed in which the application of well-balanced
functional neuroimaging paradigms might contribute
to the clari®cation of these issues.

Conclusions
Several conclusions can be made from this study. First,
there is no consensus in the literature on CAD

ã 2001 EFNS European Journal of Neurology 8, 643±658

The many faces of CAD

regarding the relevance of additional vascular lesions in
the left hemisphere. Therefore, a common standpoint is
needed to de®ne the relevance of these lesions for
conclusions regarding anatomoclinical correlations
in CAD. Secondly, our study adds evidence to some
recently changed insights in CAD. Regarding the
population characteristics our study suggests that:
(i) vascular CAD patients are not younger than UCA
patients and (ii) the CAD group does not consist of
more women than men. On the semiological level our
study corroborates the view that CAD is not a
semiologically uniform syndrome with good recovery
but built-up by a variety of symptoms with an aphasia
type distribution and an evolution comparable with
UCA. Thirdly, our data show that on the anatomoclinical level CAD can not be merely viewed as the
mirror-image pattern of UCA in the opposite hemisphere and that di€erent, even dissociated inter- and
intra-hemispherical language lateralization patterns are
likely to occur far much frequently than in UCA.
Fourthly, it is demonstrated that a variety of neurocognitive de®cits may accompany CAD. Our data
support the hypothesis that the mechanisms subserving
oral praxis are dissociable from oral language but do
not con®rm the view that the right hemisphere subserves both visuo-spatial functions and language. They
further indicate that the incidence of constructional
apraxia as a genuine apraxic disturbance might have
been overestimated in prior reports and demonstrate
our plea that a more systematic approach of neurocognitive functions might contribute to the further
understanding of the semiological variability of CAD
phenomena and the many faces of the lesion-behaviour relationships in this exceptional neurobiological
condition.

Acknowledgements
The technical assistance (photographic work) of Mrs Inge
Bats and Mr Jan Cruysberghs is gratefully acknowledged. Supported by OCMW Medical Research Foundation and Born-Bunge Foundation, University of
Antwerp. S.E. is a Research Assistant of the Fund for
Scienti®c Research ± Flanders (F.W.O. ± Vlaanderen).

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