Article abstract-The behavioral and anatomic correlates of pure alexia were analyzed in 16 patients.

Right homonymous hemianopia failed to appear in three patients, who had right achromatopsia instead. Color anomia and unilateral
optic ataxia were seen in e:ixpatients. Memory defects were found in two patients. Visual agnosia was noted in two. No
patient had visual disorientation.The crucial anatomic correlate of alexia was a lesion of the paraventricular white matter
of the left occipital lobe, capable of compromising both interhemisphericand intrahemisphericvisual pathways.The lesion
associated with color anonnia was in the mesial occipitotemporaljunction of the left hemisphere.
NEUROLOGY (Cleveland) 1983;33:1573-83

The anatomic basis

of pure alexia
Antonio R. Damasio, MD, and Hanna Damasio, MD
The neuropathologic basis of the alexias was introduced by Dejerine in 1891.lHis patient with ahxia
and agraphia had an infarct of the angular gyrus in
the left hemisphere. The next year, Dejerine2
described a man with alexia, but with retained ability
to write. The lesions associated with the alexia were
located in the left occipital lobe and in the splenium
of the corpus callosum.
The anatomic differences in the pathologic correlates of the two cases led Dejerine to the following
postulates: (1) The angular gyms contained a center
that was crucial for the interpretation of visual verbal material and for the operatioh of writing, a center
where the optical image of letters would be located;
(2) Damage to the visual pathways from both the left
and right hemispheres, on their way to the angular
gyrus, would affect reading but not writing, since the
angular gyrus itself coulld still arouse the optic image
of letters necessary to gvide the movements of writing.
Later similar cases were described and interpreted
as proposed by Dejerine. But while alexia-withagraphia continued to be encountered and related to
lesions of the angular gyrus, pure alexia was often
overlooked in clinical and theoretical discussions.
Interest in the entity was only revived when Geschwindd made it a cornerstone of his disconnection
theory.
Contemporary reports on pure alexia have supported Dejerine and Geschwinds fundamental concepts. Nonetheless, some behavorial and anatomic
aspects of pure alexia remain unexplored, and we
address them now. There has been no decisive
account of the variety of left occipital lesions neces-

sary and sufficientto cause the syndrome, or of the

meaning of a separate callosal lesion. The role of
right hemianopia and the problem of color anomia
remain unclear. (The traditional term color anomia
will be utilized to designate the two-way defect of
naming colors on confrontation and pointing to colors given their names. The designation is not ideal
but it avoids the ambiguity of alternative choices.)
Finally, there is a need to elucidate the relation
between alexia and achromatopsia, visual agnosia,
the amnesic syndromes, Holmes visual disorientation, and optic ataxia.

Method. We have studied 23 patients with pure

alexia. Twenty had CTs, and 2 came to postmortem.
We describe behavioral and anatomic findings in 16
patients (table 1)who had a focal vascular lesion and
were right handed (several patients with tumor had
comparable lesions, but they were excluded to preserve the homogeneity of the sample). All cases were
evaluated with neuropsychological and neuroophthalmologic methods in standard use in our
institution: (1) WAIS, (2) Multilingual Aphasia
E ~ a m i n a t i o n(3)
, ~ Visual Retention Test,s (4) Tests
of Line Orientation and Facial Recognition,j7 (5)
Ishiharas test,8 (6) Farnsworth-Munsell 100 Hue
Test,g and (7) Goldman perimetry. The criteria for
the diagnosis of alexia was that of Benson and Geschwind? severe disturbance of reading comprehension, linguistically correct writing (spontaneously or
on dictation), normal oral spelling, and absence of
aphasia and dementia. All our patients had (1)intact
writing (spontaneous and to dictation); (2) a complaint of reading impairment; (3) defective reading as

From the Department of Neurology (Divsion of Behavioral Neurology), University of Iowa College of Medicine, Iowa City, IA.
Supported by a University of Iowa Faculty Scholar Award to ARD.
Accepted for publication March 25, 1983.
Address correspondence and repirint requests to Dr. Damasio, Department of Neurology, University of Iowa Hospitals and Clinics, Iowa City, IA 52242.

December 1983 NEUROLOGY 33 1673

Table 1. Behavioral findings in 10 patients with pure alexia

Patient

Sex

Age

Form
vision

Color
vision

1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16

M
M
M
M
F
M
M
F
M
M
F
M
M
F
M
M

14
66
71
75
64
73
64
71
68
66
58
61
76
69
59
73

RSQ
RSQ
RSQ
RSQ
RHH
RHH
RHH
RHH
RHH
RHH
RHH
RHH
RHH
RHH
RHH
RHH

Achroma
Achroma
Achroma
Achroma

Color
anomia

Memory
defect

Optic
ataxia

+
+
+
+
+

+
+

Visual
agnosia

+
+
+
+
+
+

* Patient had left hemiachromatopia. Color naming could not be tested.

RHH Right homonymous hemianopia.
RSQ Right superior quadrantanopia.

tested by the (a) Reading Comprehension of Words

and Phrases Test (from Bentons Multilingual
Aphasia Examination4), (b) Reading Sentences and
Paragraphs Test (from the Boston Diagnostic
Aphasia Examination), and (c) Wide Range
Achievement Test.I2The aim of the behavioral study
was to establish the presence or absence of (1) defects
of form vision (hemianopia or quadrantanopia), (2)
defects of color vision (achromatopsia), (3) color
anomia, (4) visual agnosia, (5) visual disorientation,
(6) optic ataxia, and (7) amnesia.
The anatomic study was carried out in CTs by
plotting images on anatomic templates of corresponding cerebral sections. Neuropathologic examination was performed in two cases. The anatomic
analysis evaluated the following structures: (1) primary and association visual cortices, (2) white matter of the occipital and temporal lobes, (3) splenium
of the corpus callosum and forceps major, and (4)
hippocampal region (hippocampal and parahippocampal cortices). Cortical damage was analyzed in
three regions: (1) mesial occipital (including the
occipital pole and posterior half of all cortex behind
the splenium), (2) mesial occipitotemporal (including the anterior half of all cortex behind the splenium
and the mesial temporal cortex), and (3) lateral
occipital. Analysis of white matter damage included
(1) corpus callosum (the splenium proper), (2)
forceps major (continuation of the callosum into the
occipital substance), and (3) paraventricular region
(the area behind, beneath, and to each side of the
occipital horn).
Finally, we analyzed the comparable behavioral
and anatomic data of the cases of Dejerine,2 Geschwind and Fusillo,13Greenblatt,14 and Mohr et al.15

1574 NEUROLOGY 33 December 1983

Table 2. Major behavioral findings in the patients of

Dejerine, Greenblatt, Geschwind, and Mohr
Pure Color Right
Right Memorq
hemidefect
alexia anomia hemianopia achromatopsia
Dejerine2
(1892)
GreenblattI4
(1973)
Geschwindl:?
(1966)
MohrI5
(1971)

+
+
+

+
+

1-

The first three had pure alexia (with right hemiachromatopsia in Dejerines case, right hemianopia
in Geschwinds, and neither in Greenblatts). The
case of Mohr et a1 had color anomia without alexia.
These cases were chosen because of the availability
of autopsy studies and of the paradigmatic nature of
their presentations.

Results. Behavioral findings (table 1). All patients

had right visual field defects. In 13, there was complete right hemianopia. In 3, there was right upper
quadrantanopia. The latter had achromatopsia in
the right inferior quadrant. Seven of the 16 patients
had color anomia. Three of the 16 patients had
achromatopsia in the right visual field. None of those
had color anomia in the color-intact field. Two of the
16 patients had verbal memory defects. Two others
had prosopagnosia. None had visual disorientation,

Table 3. Anatomic survey of abnormal CT images in the left hemisphere of 16 patients with pure alexia

Patient

Lateral
occipital

Cortical involvement
Mesial
Mesial
occipital
Occip temp

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

+
+

2
3
4
5
6
7
8
9
10
11
12
13
14
15

+
+
+

White matter involvement

Corpus
Forceps
Paracallosum
major
ventricular

+
+
+
+
+
+

+
+
+
+

+
+

+
+
+
+
+

16

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

Table 4. Anatomic survey of the Dejerine, Greenblatt, Geschwind, and Mohr cases
Cortical involvement
Fusiform
gvus
Dejerine
(1892)
GreenblattI
(1973)
Geschwind
(1966)
Mohr?
(1971)

Lingual
gnus

Parahippocampal
gyrus

+
+
+

+
+

+
+

but six had optic ataxia in movements of the right

hand within the preserved left visual field. A summary of the behavioral findings in the historical cases
is given in table 2.
Anatomic findings (table 3). Cortical involvement:
Thirteen patients had damage in the mesial occipital
region; 7 had occipitotemporal lesions. Involvement
of lateral occipital region was seen in five patients.
White matter i n v o l v e m e Only 2 of the 16 patients
had lesions in the splenium proper, but callosal pathways were involved in 11of the patients a t the forceps
major, and in 13 at the paraventricular region.
A summary of the anatomic findings in the historical cases is presented in table 4. In Dejerines
patient, the most caudal aspect of the lingual gyrus
was affected, but not its rostra1 end. The base of the
cuneus was involved. In horizontal sections, the
white matter of the occipital lobe was destroyed by a
wedge-shaped lesion; the apex pointed to the tip of
the occipital horn, and the base occupied the occipital pole. The splenium of the corpus callosum was

White matter involvement

Corpus callosum
Forceps
Ventral
Dorsal
major

+
+
+

+
+

+
+

Paraventricular

+
+
+

compromised by a small ventral infarct in its ventral

portion, located about the midline. Geschwinds
patient had lesions in the left side of the splenium
and forceps major, left mesial occipital and temporal
cortex (encompassing all of lingual and fusiform gyri
and hippocampal region) and left paraventricular
region. (There were also lesions in the left thalamus
that were not pertinent to the issue of alexia.) Mohrs
patient had a lesion of the left mesial occipitotemporal region, but the splenium and the paraventricular area were intact.
In conclusion, the lesions associated with true
alexia include three major types. Type I is found in
patients with right hemianopia and color anomia
(figures 1, 2, 3, and 4). Verbal amnesia may or may
not be present. In vascular lesions, the syndrome is
caused by an extensive infarction of the posterior
cerebral artery territory. Calcarine, splenial, and
mesial temporal branches are involved. Accordingly,
damage encompasses the white matter of the
the occipitotemporal junction (including the para-

December 1983 NEUROLOGY 33 1676

Figure 1. CT of patient 6,
who had pure alexia,
RHH, color anomia, and
verbal amnesia. The
lesion involves both the
lateral and mesial
aspects of the occipital
lobe (arrow 2), extends
into the mesial
occipitotemporal
junction (arrow 3), and
reaches even further
anteriorly to involve
most of the mesial
temporal lobe (as can be
seen in the first three
cuts-arrow 6). I t also
involves the
paraventricular white
matter (arrow 1) and the
forceps major (arrow 4)
as well as the corpus
callosum proper (arrow
5).

ventricular area), mesial occipital cortex (inferiorly

and superiorly), the left half of the splenium and
forceps major, and, in some cases, the mesial temporal cortex (the hippocampal region immediately
anterior to lingual gyrus). Supply to the thalamus
and cerebral peduncle may be compromised. (Peduncular lesions cause the hemiplegic syndrome of the
posterior cerebral artery.16) The prototype of this
case is Geschwind and F~sillos.~
In type 11, the
patients have right hemianopia, but not color
anomia or verbal amnesia. There is damage to optic
radiations or to the calcarine region, and compromise of the interhemispheric pathways within the
left occipital lobe, due to a paraventricular lesion
(figures 5 and 6). In type 111,the patients have right
upper quadrantanopia and right lower achromatopsia, but not color anomia or verbal amnesia. The
lesion involves the paraventricular white matter, the
inferior contingent of the optic radiation and the
inferior visual association cortex (figures 7 and 8). A
variety of type 111is found in patients with full visual
fields, without color anomia and with or without
right hemiachromatopsia. The lesion is occipitotemporal, invdyingthe paraventricular white matter,
and may or may not extend caudally to involve the
mesial and inferior visual association cortex. The
splenium itself may or may not be affected. In vascular cases, the lesion results from infarcts in the territory of the posterior cerebral artery. The posterior
temporal and calcarine branches are involved, as in
the case of Dejerine. The splenial branch may be
spared, although a small lesion may appear in the
callosum, as in Dejerines or Bensons cases (such

1576 NEUROLOGY 33 December 1983

Figure 2. CT of patient 9, who had pure aleria, RHH,

and color anomia, but no memory defect. The lesion
involves the mesial occipital lobe (arrow 2) and extends
into the mesial occipitotemporal junction (arrow 3). I t
also involves the paraventricular white matter (arrow 1)
and the forceps major (arrow 4).

Figure 3. Postmortem of patient 9. T h e infarcted area

involves the cortex and underlying white matter of the
mesial occipital lobe and mesial occipitotemporal area
(arrows 2 and 3) as well a.s the paraventricular white
matter in the left hemisphere (arrow 1). The forceps
major also shows signs of infarct (arrow 4). T h e
midportion of the corpus callosum is intact. O n the
second slice, an infarcted occipital lobe was lost during
the photographing procedure. T h e black line indicates
previous contour.

Figure 4. Standard template of the anatomic localization

of the lesion i n patients with pure alexia, RHH, and
color anomia. There is involvement of the mesial and
lateral occipital lobe and the mesial occipitotemporal
area as well as the paraventricular white matter and the
forceps major. T h e callosum proper may or may not be
involved.

lesions may either be small border zone infarcts or an

autopsy artifact from pressure of the falx).

Discussion. Role of right hemianopia in pure

alexia. In practically every instance known to the
authors, references to t.he original case of Dejerine2
state that right hemianopia was the sole neurologic
sign associated with alexia. References to the case of
Geschwind and FusilloL3also stress the right hemianopia that was associated with pure alexia and color
anomia. Emphasis had been placed on the blindness
of the right visual fieldl, because it guaranteed that
visual information was confined to the right hemisphere. Consequently, Greenblatts patient with
pure alexia and no hemianopia14 appeared exceptional and constituted an important test of the disconnection theory. Even if visual information from
the right visual field could arrive in the left hemisphere of his patient, it obviously was not used for
reading, as there existed a block in the processing of
information beyond its arrival in the left occipital
cortex, that is, beyond the point at which the experience of seeing in the right visual field took place,
and before the stage at which the experience of
recognizing was organized.

Figure 5. C T of patient 13, who had pure alexia and

RHH, but not color anomia or memory defect. T h e lesion
involves the lateral aspect of the occipital lobe (arrow 2)
and the paraventricular white matter (arrow l),and
undercuts the forceps major (arrow 4).

We agree entirely with Greenblatts interpretation, but we believe the argument was implicit in
Dejerines original report, because neither patient
had a true right hemianopia. To prove that point, we
should refer to Dejerines text.* On page 64, Dejerine
indicates that the patient had a right homonymous

December 1983 NEUROLOGY 33 1577

Figure 6. Standard template of the anatomic localization

of lesion i n patients with pure alexia and RHH, but
without color anomia. T h e lateral and mesial occipital
lobe may be involved as well as the paraventricular white
matter and the outflow of the callosum, through the
forceps major. T h e mesial occipitotemporal area is
intact.

hemianopia with hemiachromatopsia of the same

side (line 28: Hemianopsie homonyme lathale
droite avec hemiachromatopsie du meme cot6). On
page 67, transcribing the detailed observation of the
ophthalmologist Landolt, who had referred the
patient to him, the article reads (line 3): That
(right) hemianopsia is not absolute, the visual sensation of the left half of the retinas being not entirely
abolished but merely diminished. When placed in the
right half of the visual fields, the objects appear more
obscure and less clear than in the other half; furthermore, we must add that the extreme limits of the
visual fields are normal. (Original text: Cette
hemianopsie nest pas absolue, la sensation visuelle
de la moiti6 gauche des deux retines n6tant pas
compliitement abolie, mais seulement diminu6e.
Plac6s dans la moitib droite des champs visuels, les
objects y semblent plus obscurs, moins nets que dans
lautre moiti6; ajoutons dailleurs que les limites
extremes des champs visuels sont normales.) Further on (line 14), describing the patients right hemiachromatopsia, Landolt states: No color can be
perceived after the colored object has gone beyond
the vertical line passing through the center of the
visual field. The brightest colors produce only an
impression of gray. The objects are, however, recognized in their shapes and dimensions. Colored
objects placed at the 0 degree level appear to be
colored only on their left even if we illuminate their
right halves with greater intensity. (Original text:
Aucune couleur nest percue d8s que lobjet color6 a

Figure 7. C T of
patient 3, who had
pure alexia and right
superior
quadrantanopia as
well as
achromatopsia i n
the right inferior
quadrant. T h e lesion
involves mesial
occipital lobe (arrow
2) as well as
paraventricular
white matter (arrow
19. T h e mesial
occipitotemporal
area is intact (arrow
3). T h e lesion also
extends into the
inferior
occipitotemporal
area (arrow 7).

1678 NEUROLOGY 33 December 1983

Figure 8. Standard template of the anatomic localization

of lesion in patients with pure alexia, right superior
quadrantanopia, and achromatopsia in the right inferior
quadrant. T h e paraventricular white matter is involved
as well as the mesial (and lateral) occipital lobe in its
infracalcarine region. T h e mesial occipitotemporal
occipital area and the supracalcarine mesial occipital
area are spared.

depassk la ligne verticisle passant par le centre du

champ visuel. Les objets sont reconnus dans leurs
formes et leurs dimensions. Les objets colorks fixes
au niveau du 0 degrk sernblent nbtre colorks que dans
leur moitik gauche, m&mesi Yon vient a eclairer avec
plus dintensitk leur moitik droite.)
These descriptions leave no room for doubt.
Dejerines patient had EL right hemianopia only in the
sense that his vision in the right visual field was not
intact. This concept of hemianopia was generally
used at the time and is bound to be misinterpreted
today, when the term hemianopia is literally taken to
mean blindness. The mere mention of both right
hemianopia and right hemiachromatopsia would
have been a contradiction, were it not for the fact
that Dejerine and Landolt only meant hemianopia in
the sense of imperfect vision, not blindness.
Both Dejerine and Landolt were aware of Verreys description of lhemiachromatopsia in 1888.
Verreys patient had right hemiachromatopsia also;
the lesion was confined to the infracalcarine cortex of
the lingual gyrus and the immediately underlying
white matter. The lesion did not extend, as in
Dejerines case, to the paraventricular white matter;
hence, her lack of alexia. Unfortunately, the concept

of hemiachromatopsia fell into disuse and came upon

hard times when Gordon Holmes denied the existence of disturbances of color vision due to central
lesions. Although this error was corrected later,18-21
it
made many authors unaware that vision in the right
field of the renowned patient was not lost, but merely
colorless and dimmer than the left.
Dejerine himself, it must be said, did not make
things easy for readers. As he attempts to interpret
alexia (page 88, line 32), he confuses matters further.
He probably would have liked to suggest that letters
might not be seen in the right visual field, but sensing
the contradiction, he had second thoughts that he
had confided in a footnote (marked 3): I should
remark, along with Landolt, that in this patient the
right hemianopsia was not a completely negative
hemianopsia, because vision had not been anulled in
the right half of the visual fields and there was,
rather, a sensation of obscure vision. He was, in
reality, more hemiachromatopsic than hemianopsic,
in the proper sense of the word. (Original text: Je
ferai remarquer avec Landolt, que chez ce malade
lhemianopsie droite nktait pas une hemianopsie
completement negative, car il navait pas une vision
nulle dans la moitik droite de chacun de ses champs
visuels, mais bien une sensation de vision obscure. I1
ktait, en realitk, plus hemiachromatopsique quhemianopsique proprement dit.) Dejerines patient
could see letters in the colorless and dim, but otherwise visually intact right field.
As Landolt indicated, and as further investigation
of hemiachromatopsia in other cases has demonstrated, central vision as well as panoramic vision
were intact. In our experience, hemiachromatopsic
patients can recognize letters (even in tiny print) as
well as objects in the hemianopsic field. Thus,
Greenblatt~~
explanation for alexia in the absence
of right hemianopia was appropriate for his own case
and also for Dejerines. In both, visual information
from two operational fields and two operational
visual cortices was prevented from proceeding to
cerebral structures capable of its further handling, ie,
the necessary visuoauditory linkage.
Though generally regarded as a replica of
Dejerines case, Geschwind and Fusillos13differed.
The patient had a dense right hemianopia. No visual
information could arrive in the left occipital cortex,
and alexia was attributed to disconnection of visual
information arriving in the right occipital cortex
from the language processing structures of the left
parietal and temporal lobes. Clearly, alexia can occur
with or without hemianopia, and the disconnection
mechanism holds for both types of cases. Dejerines
case is the prototype of alexia without hemianopia;
Geschwind and Fusillos is the standard for alexia
with hemianopia. We will argue later that the distinction may help predict another possible concomitant of alexia, color anomia: The patients of
Dejerine and Greenblatt had neither hemianopia nor
color anomia; Geschwind and Fusillos had both.

December 1983 NEUROLOGY 33 1579

The role of the callosal lesion. Dejerines patient

had lesions in both the left occipital lobe and
splenium. Although often cited incorrectly, Dejerine
was convinced that the small callosal lesion was of no
importance and that the lesion in the white matter of
the occipital lobe could account for visual disconnection. On page 89, line 10, Dejerine states: The lesion
of the white matter subjacent to the left occipital lobe
was extensive enough, since it reached the ventricle,
to compromise the fibers which connect the left
angular gyrus to the two occipital lobes, and that
frees us from having to bring in the small lesion of the
splenium of the corpus callosum. Unfortunately, he
adds: Symptoms caused by lesions of the corpus
callosum are, in effect, too obscure, and above all still
too little known. It seems, to me, inappropriate to
insist on them. (Original text: La lesion des masses
blanches sous-jacentes au lobe occipital gauche Btait
assez Btendue, puisquelle atteignait lependyme ventriculaire, pour que nous puissions admettre que les
fibres qui relient le pli courbe gauche aux deux lobes
occipitaux aient Qt6 interessbes, et sans que nous
ayons besoin de faire intervenir une seconde lBsion,la
petite lesion siegeant dans le bourrelet du corps calleux.) Unquestionably, Dejerine defended a disconnection hypothesis, indistinguishable from
Geschwinds. But Dejerine thought that all fibers
traveling to the angular gyrus from both left and right
occipital cortices would funnel through the white
matter damaged in the occipital lobe and that the
lesion of the callosum was unnecessary to produce
that effect. His comment on the function of the
callosum had little logic. If a white matter lesion in
the depth of the left occipital lobe could disrupt
visual information coming from the right visual cortex, it would do so by interrupting the continuation
of callosal fibers from the splenium.
The question, then, is whether interhemispheric
fibers connecting the visual cortices are damaged,
and, if so, where. They can be damaged in the midsplenium or in its extension into the occipital lobe
(the forceps major), or even beyond, as different fiber
components travel toward parts of the visual association cortices. Fiber systems interconnect the visual
association cortices of areas 18 and 19.22In violation
of Flechsigs rule, there may even be fibers interconnecting the two areas 17.**It is less likely that there is
any connection from areas 18 and 19 directly to the
angular gyrus of the contralateral hemisphere, as
Dejerine hypothesized. On the other hand, there are
probably connections from the association cortex
(where interhemispheric fibers terminate) forward
to the temporoparietal cortex (contained in the
occipital component of the inferior longitudinal fasciculus, in which fibers from the fusiform and lingual
gyri course in the direction of the language and limbic cortices of the parietal and temporal lobes).
Rockland and Pandya have found these connections in the rhesus monkey, joining areas 18and even
17 with a specific portion of the superior temporal
1580 NEUROLOGY 33 December 1983

sulcus (which is, at least in part, a probable homologue of the human superior temporal/inferior parietal
region), confirming other^.^^-^^ There is no evidence
that such direct connections between area 17 and
areas 22,37, or 39 exist in man, but they are possible.
There is a final issue concerning the role of callosal
fibers. Only one group, of those that terminate in
inferior visual association cortices, seems to be pertinent for reading. That corresponds roughly to the
middle component of fibers of the splenium, considered on the dorsoventral axis. The most ventral fibers
connect the hippocampal regions, and the dorsalmost fibers connect the superior visual cortices.27
Accordingly, alexia is associated with damage to the
inferior area of the forceps major. In many of our
alexic patients, the superior arm of the forceps major,
which carries fibers from the dorsal splenium to the
higher occipitoparietal cortices, was intact.
Dejerines disciple Vialet28 reiterated the interpretation of alexia on the basis of the deep left occipital lesion and even failed to mention the splenial
lesion at all when she described the microscopic and
macroscopic pathology of Dejerines case. Vialet also
emphasized the subventricular location of the lesion.
Damage extended not only to the tip of the occipital
horn, but under it, interrupting all the white matter
pathways traveling in the occipitotemporal junction
area. B r i s ~ a u ddescribed
~~
a similar case and again
gave short shrift to the callosal lesion. The role of the
callosal lesion itself, as opposed to a lesion in interhemispheric pathways, was first emphasized by Foix
and H i l l e m a ~ ~They
d . ~ ~described a patient with right
hemianopia but without alexia. The pathologic findings, so they said, resembled those of the Dejerine
case minus the splenial lesion. An infarct involved
the fusiform and lingual gyri, the banks of the calcarine fissure, and the cuneus. Unfortunately, they
did not describe the extent of the lesion into the
white matter-the crucial issue, as we see it. We
suspect the lesion was limited and that the patient
had hemianopia without alexia, a syndrome previously documented pathologically by Vialet in two
cases.28Foix was right in thinking that an additional
callosal lesion in his case would have produced alexia.
But so would extension of the lesion into the paraventricular area, with or without splenial damage.
We think Foix was in error when he attributed value
to the splenial lesion in Dejerines case, considering
the position and extent of the principal lesion.
Color naming defect. As can be gathered from the
survey of single cases previously published, color
anomia is not a necessary accompaniment of pure
alexia. In the only other extant series of patients with
pure alexia, Gloning et al3Iconcluded that 50 to 70%
of the patients had color anomia. Dejerines original
patient obviously could name colors without difficulty in his left, nonachromatopsic field, as could
Greenb 1a tt s.I4 But Ge sc h w i n d and Fu s ill0 s
patientl3 could not name colors. On the other hand,
color anomia can be seen in the absence of pure

alexia, as Mohr et all5 demonstrated.

Let us analyze the major behavioral features of
those four cases. Dejerines patient had right hemiachromatopsia, but not right hemianopia or
amnesia. Greenblatts patient did not have right
hemianopia, right herrdachromatopsia, or amnesia.
Geschwinds patient had both right hemianopia and
amnesia. Mohrs patient had both right hemianopia
and amnesia, but not alexia (table 4).In our own
cases, color anomia was strongly associated with
right hemianopia and was present in the two patients
who had memory disturbance (table 1).Color anomia
will probably not OCCUI without right hemianopia.
The anatomic underpinning of the historic cases
differed (table 3). In Greenblatts patient, the lesion
spared the left occipital cortices, but the white matter of the occipitotemporal region was compromised,
as in Dejerines case. The hippocampus was also
spared. In Geschwinds patient, the lesion involved
both white matter and cortex of the occipital lobe,
but in addition the splenium was destroyed (dorsally
as well as ventrally), and so was the left hippocampus. In Mohrs case, the lesion compromised most of
the left mesial occipitotemporal cortex; namely, the
hsiform and lingual gyri and the hippocampus-a
feature shared with (Geschwinds case. But the
splenium was intact, and so was the remainder of the
occipital cortex and underlying occipitotemporal
white matter. Unlike the cases of Dejerine or Geschwind, the lesion in Mohrs case was shallow and
spared the region beneath and behind the occipital
horn, with extensive damage to the left lateral geniculate ganglion. As for the lesion in Dejerines patient,
we should emphasize the absence of damage to the
left hippocampus, anterior portion of the lingual
gyrus, and dorsal fibers in the splenium. Based on
that material, it is legitimate to conclude that
damage to mesial occipitotemporal cortices, in a
patient with right hemianopia, is the major anatomic
correlate of color anomia.
Geschwind attributed color anomia to visuoverbal
disconnection, the same mechanism that accounted
for alexia. The explanation was appropriate for his
patient, because the left occipital cortex could not
receive any visual information, and the left language
cortex could not avail itself of any right visual field
input, in view of the extensive splenial damage. The
explanation may also be appropriate for other
patients if it can reconcile the following facts: (1)On
some patients, visuoverbal disconnection causes
alexia but not color anomia; (2) The visuoverbal
disconnection that caused color anomia in Mohrs
patient did not cause alexia. Thus, the anatomophysiologic locus of disconnection must be different for reading and color naming.
Aware of this necessary dissociation, some
authors have tried to establish its possible anatomic
basis. Cumming et a13zconjectured that spared fibers
in the dorsal splenium of their patient would convey
visual information froin right visual cortex to left

language cortex. OthersI4J3J4were of similar opinion, emphasizing that color naming would depend on
both the dorsal splenial fibers and dorsal occipital
cortex, which had remained intact in cases of alexia.
The hypothesis accounted for the absence of color
anomia in those patients, but did little to explain
color anomia in Mohrs patient, who had no lesion in
the splenium or dorsal occipital cortex. All callosal
pathways from the right visual cortex were patent in
that case, and dorsal fibers of the splenium could
have joined the dorsal sector of the left occipital
cortex; ie, the callosal fibers that Cumming et a1
considered crucial for color naming were intact. So
were the dorsal occipital pathways with which
Greenblatt justified the preservation of color naming
in his patient.
Our conclusion is different. We think it is likely
that, in Mohrs patient, interhemispheric fibers
arriving in the mesial occipitotemporal transition
cortex were destroyed or, more to the point, had
nowhere to end. This kind of lesion occurs in those
patients with pure alexia who have concomitant
damage to the hippocampal region, which is contiguous with the rostral portion of the lingual gyrus.
CT, in our cases of pure alexia, revealed that color
anomia was seen only when there were lesions in the
mesial occipitotemporal cortex in addition to the
paraventricular region (figure 5). If Mohrs case is
not an exception, the paraventricular component is
not necessary for the appearance of color anomia.
But the mesial occipitotemporal lesion is both necessary and sufficient. Color anomia probably appears
only when three conditions are satisfied: (1)damage
to left lingual gyrus, (2) damage to left hippocampal
region (hippocampus plus parahippocampal region),
and (3) presence of right hemianopia. Damage to the
splenium is not necessary if (l),(2), and (3) are
satisfied. Geschwinds patient and several of ours
had splenial damage, but also the three prerequisites.
Left hippocampal damage is not sufficient, and even
bilateral hippocampal lesions will fail to cause color
a n ~ m i a . ~ Damage
. ~ to the posterior part of the lingual gyrus is not sufficient, as Dejerines patient
proved. The essential lesions affect the anterior portion of the lingual gyrus, in the vicinity of the hippocampal region. Finally, lack of callosal damage does
not prevent color anomia.
Since all patients with color anomia have right
hemianopia, visual information arriving in the right
hemisphere fails to evoke color names in that hemisphere and, after transfer to the left hemisphere, that
information will still fail to evoke color naming when
lingual gyrus and hippocampus are damaged. The
visuoverbal disconnection mechanism for color operates there-a different site from where disconnection affects reading. Connections from the right
hemisphere, crucial for color naming but not for
reading, may course in the most mesial aspect of the
left occipitotemporal junction (in the border zone
between rostral lingual gyrus and caudal parahip-

December 1983 NEUROLOGY 33 1681

pocampal gyrus) and would be damaged by lesions

bearing on the reading disorder. Patients develop
alexia without it (Dejerines case) or with it (Geschthat involve both lingual and parahippocampal corwinds). Damage to the left hippocampal system
tices. In patients with right hemianopia, this would
alone does not cause alexia (Mohrs patient).
cause color anomia, as in Mohrs patient. In the
Damage to both hippocampal regions does not cause
patients of Dejerine, Greenblatt, Vincent et al, and
alexia either (as Scoville and Milnerspatient HM
many of ours, this area was apparently spared, and
and his counterparts demonstrate). Achromatopsia
color naming was intact.
Achromatopsia. T h e anatomic correlates of
also appears regardless of hippocampal lesions. But
left hippocampal damage seems to be associated with
achromatopsia were hinted at by Verrey, Dejerine,2
and MacKay and Dunlop.37 Unilateral lesions of
color anomia, even though solitary hippocampal
fusiform and lingual gyrus, in either the left or right
lesions cannot cause the symptom. One of our
h e m i s p h e r e , produced c o n t r a l a t e r a l h e m i patients with profound verbal amnesic syndrome
had a lesion in the left hippocampal region, but not
achromatopsia. Bilateral lesions in the same location
the left mesial occipital cortex: he could name colors
caused complete achromatopsia (and also prosopagand read normally. Patients with an amnesic synnosia). When the lesion in the left hemisphere
drome and bilateral hippocampal damage do not
extended to the paraventricular area, especially in its
inferior aspect, pure alexia would be added, as in
have color anomia. Our explanation for the associaDejerines case.
tion between left hippocampal damage and color
Hemiachromatopsic patients can read correctly
anomia is the following: Given the anatomic conbut slowly in the colorless field. Patients with hemitiguity of the parahippocampal and lingual cortices,
hippocampal lesions also affect the most anterior
achromatopsia-even those with right hemiportion of the lingual gyrus.
achromatopsia due to left occipital lesions-name
Visual agnosia, optic ataxia, and visual disoriencolors correctly in the intact left visual field. They are
tation (simultanagnosia) in pure alexia. Only one of
aware of the colorless hemifield, but they can name
our alexic patients had visual agnosia. The agnosia
colors or point to colors when given their names. In
other words, they are not color anomic.
was of the associative type, and the patient could not
recognize familiar faces or objects. That patient had
Modern s t u d i e s c o n f i r m t h e s e views.
Achromatopsia is associated with lesions in the
bilateral lesions that resembled the lesions described
by Benson et a145in a patient with alexia and visual
fusiform and lingual gyri that spare the calcarine
agnosia. The finding supports the notion that visual
cortex and a significant part of the optic radiaagnosia requires bilateral lesions of the occipitotemt i o n ~ . The
~ ~ ,presence
~ ~ , ~ of
~ color-related cells in the
poral region.
visual cortex of nonhuman primates has been docuBilateral optic ataxia was not seen in our patients.
mented, and recording from cell columns in the
However, unilateral optic ataxia was seen in six, all of
monkeys visual cortex, Zeki40s41suggested that a cirwhom had right hemianopia. Optic ataxia was seen
cumscribed area of visual association cortex is priin the intact left field and with the right hand. The
marily concerned with the processing of color, in
probable mechanism for optic ataxia was as follows:
contradistinction to other areas of visual c.ortexthat
Left hemisphere control of the right hand was disare either insensitive to color stimuli or respond to
connected from visual information arising from the
both color and other stimuli. Some authors have
field. Motor control of the left hand could
questioned the focal distribution of those ~ e l l s , ~ *left
~ ~visual
~
appropriately draw on visual information from the
and others have confirmed it.44If such areas do exist,
it is possible that they are homologues of the human
left visual field, which was available through the
mesial occipitotemporal cortex.
intact right visual cortex and its preserved connecAmnesia in pure alexia. The memory defect of
tions to parietal and frontal lobe structures of the
right hemisphere. Bilateral optic ataxia, as well as
patients with pure alexia is probably caused by
lesions in the left hippocampal region (hippocampus
visual disorientation (simultanagnosia), requires
proper and parahippocampal gyrus). Concomitant
bilateral occipitoparietal lesions, and none of our
lesions in the fornix and some thalamic nuclei are
patients had such lesions.46
unlikely culprits, but they could contribute. At first,
the defect resembles the amnesic syndrome that follows bilateral mesial temporal ablati0n,3~although in
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December 1983 NEUROLOGY 33 1583

The anatomic basis of pure alexia

Antonio R. Damasio and Hanna Damasio
Neurology 1983;33;1573
DOI 10.1212/WNL.33.12.1573
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