Brain (1974) 97, 251-262

CUING AND MEMORY DYSFUNCTION IN ALEXIA WITHOUT AGRAPHIA
A CASE REPORT 1
BY

DEJERINE (1892) described the clinical syndrome of alexia without agraphia (pure
word blindness) occurring in a man with infarction in the territory of the left
posterior cerebral artery. Geschwind (1965) and Geschwind and Fusillo (1966) have
recently called attention to and supported the explanation of the syndrome as a
disconnexion between the visual input in the right hemisphere and the language
region of the left hemisphere. The present case report concerns a patient with alexia
without agraphia studied clinically over a three-year period, shown at post-mortem
to have an extensive unilateral infarction in the territory of the left posterior cerebral
artery. Her capabilities with visual symbols when naming was not required or when
prompting or cuing was introduced, may shed some additional light on the nature of
the visual defect in alexia without agraphia. Furthermore, this is the first known
documented example of a severe memory disturbance lasting for six months related
to a unilateral infarction.

Case
In November 1969 a 68-year-old right-handed lady suddenly developed numbness of the right
arm. She had had rheumatic fever as a child, and had been admitted to the Massachusetts General
Hospital in June 1967, with ankle oedema and dyspnoea, at which time the diagnosis of rheumatic
heart disease with mitral insufficiency and congestive heart failure was made. There were no
previous neurological symptoms, and her deficit appeared abruptly and without warning after
rising. Blood pressure was 140/85, pulse was 90 and regular. There was a pan-systolic mitral
apical murmur. No signs of congestive heart failure were present. Neck pulses were normal. No
bruits were audible. There was a complete, congruous right hemianopsia without macular sparing.
Optokinetic nystagmus was absent to the right. The right corneal reflex was reduced and pain,
temperature, and touch perception were diminished on the right face. Pupils and eye movements
were normal. Her face was symmetrical. Movements of the right limbs were slow and clumsy but
good strength was gradually mobilized at all joints. Deep tendon reflexes were brisker on the right,
and both plantar reflexes were extensor. No response to pin, touch, cold, vibration, or alteration
•Read in part at the American Academy of Neurology, St. Louis, Missouri, April 27, 1972.
*Present Address: Neurology Service, Beth Israel Hospital, Boston, Massachusetts 02215.

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LOUIS R. CAPLAN2 AND TESSA HEDLEY-WHYTE
(From the Stroke Service, Massachusetts General Hospital and the Departments of Neurology and
Pathology, Beth Israel Hospital, Harvard Medical School)

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LOUIS R. CAPLAN AND TESSA HEDLEY-WHYTE

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of joint position could be elicited in the right limbs. A severe pinch on the right evoked a poorly
localized weak tingling sensation.
Cortical function: She was alert and spoke normally. She could accurately repeat sounds,
words and phrases; spelled normally and identified words spelled to her readily. She wrote slowly
and clumsily to dictation with her left hand. Her writing was accurate except for occasional
misspellings and mirror writing errors; she correctly copied words, numbers and symbols. She had
no difficulty reciting lists or series from memory, e.g. months, colours, flowers, animals. The
following higher cortical function deficits were consistently demonstrated while she was in hospital:
(1) Reading: She could read no words and incorrectly named written numbers, letters and
musical and arithmetical symbols. She wrote but could not later read what she had written nor
identify it as her own writing.
(2) Memory: She had a profound recent memory disturbance. She could recall having seen the
examiner before, but never recalled his name nor where she had seen him even after scores of
meetings. At best, she was able to recall one or two facts in a ten-pait story after three minutes,
but seldom recalled any of the story after ten minutes. Even when given alternative choices in
relationship to a story told her ten minutes previously, she could only on rare occasion select one or
two facts correctly. She was given repeated batteries of tests in a specially prepared room on
twelve different days, but never recalled being in the room before nor doing any of the tests
previously.
(3) Colour naming: She named correctly only one-fourth of the colours presented to her yet was
able to match colours, to name colours of familiar objects, and to name objects that characteristically
are of a given colour.
(4) Visual naming: She failed to name approximately one-half of objects or pictures of objects
presented to her, yet she was able to quickly name the same objects presented tactually to her
left hand and to find names for objects described verbally. She used objects in her environment
normally. The following are representative responses to large pictures shown her:
A. Bus—A map, no, I know it, of course, ought to be able to say it. Not a map.
What do you do with it ? It's a road, you use it on a road. Is it a chair, is it a car, is it a horse, is it
a bus ? No. Is it a bus ? It could be a bus, yes, yes, that's what it is, a bus.
B. Belt.—Like a map, no, I know what it is, I can't say it. What do you do with it ? You measure
something with it. Is it a tape measure P No. Is it a belt ? Yes, more like a belt.
C. Banana—Fruit, it's a measure, no. What do you do with it? Eat it. Is it an apple, a prune, a
pear, a banana ? No. Is it a banana ? No, more like a pineapple.
D. Bathtub—It's for water, you measure water with it, a sink, no you have it near a sink like a
faucet, a drain, it has another name, I know. What do you do with it ? Take a bath in it, wash. 'Is
it a bathtub ? Yes, it could be, yes.
E. Cane—It's a drain, a sink, no like a sink. What do you do with it ? It's a . . .Is it a cane, a bed,
a chair ? No. Is it a cane ? No.
F. Cigarette—A water, it's a water, it's a shadow, a shade. What do you do with it ?\ can't tell you.
Is it a horse, a cigarette, a chair ? No. Is it a cigarette ? It might be a cigarette.
G. Clock—A shade, it's a shade, I know what it is, a shade. Is it afloor,a table, a clock ? No, it's
a shade.
Though she could not name some objects when visually presented, she was able to name the
same objects on verbal questioning. For example, to the question what objects would you use to
cut your lawn? she replied "lawnmower" or "shears." What would you cut with? "A knife or
scissors." Yet she did not name scissors when the object or a picture of the object was shown to her.
(5) Right-left confusion, dyscalculia, and finger agnosia were present. Spontaneous drawings
were poorly formed with her left hand, and she copied pictures and matchstick constructions poorly.
Laboratory tests: An EEG showed left-sided theta and delta slowing. CSF pressure 130 mm;
28 RBC per c.mm, protein 27 mg per cent, sugar 77 mg per cent. BUN 41, electrolytes normal.
Skull X-ray was normal.

ALEXIA WITHOUT AGRAPHIA

253

Clinical course: The constructional dyspraxia, right-left confusion, finger agnosia, dyscalculia,
and left Babinski sign cleared within the first month. The profound recent memory disturbance
gradually improved. Three months after her stroke, she still could not recall the name of the
examiner (L. R. C.) after five minutes, and recalled no facts of a ten-part story after ten minutes.
By seven months, she spontaneously reported all four parts of her breakfast ingested hours before,
could repeat my name after five minutes, and could repeat seven of ten facts in a story given ten
minutes before. She was able to spontaneously recall two out of three names of objects told her
five minutes before, and could readily choose the third object if given alternative choices. Subsequent'
memory testing over the three-year period did not change. She never regained memory of the onset
of her illness nor of herfirstmonths in hospital. After six months, optokinetic nystagmus responses
were symmetrical, though there was no improvement in her complete right homonymous
hemianopsia. The remainder of her deficit did not substantially change after the original stroke.
She remained at home after discharge from the hospital and was able to do minor household
chores. In December 1972 she suddenly became dyspnoeic and had cardiac arrest.

At the time of autopsy, which was limited to the cranial vault, the brain was of normal size with
minimal atrophy of superior anterior aspects of both frontal lobes. The arteries of the circle of
Willis were moderately atherosclerotic but otherwise were within normal limits. The brain was
sectioned horizontally and sections were stained with hsmatoxylin and eosin, luxolfast blue,
Bodian's stain for axons and phosphotungstic acid-hsematoxylin. A large old infarct in the
distribution of the left posterior cerebral artery extended from the mid-portion of the temporal
lobe into the occipital lobe around the calcarine fissure, including the white matter, the splenium,
posterior hippocampus and geniculate bodies (fig. 1). The left optic radiation was 1 mm in

FIG. 1.—Horizontal section of brain viewed from the base with an old infarct involving the medial
aspect of the left occipital and posterioi temporal lobe. Note the atrophic left hippocampus and
mammillary body and lack of pigmentation of the posterior left substantia nigra. The occipital
horn of the left lateral ventricle is maikedly dilated. The atherosclerosis of the internal carotid
arteries is representative of that seen in the other vessels of the circle of Willis.

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Neuropathology

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LOUIS R. CAPLAN AND TESSA HEDLEY-WHYTE

greatest width whereas the right optic radiation was 3 mm. A similar aged infarct involved part
of the posteroventral lateral nucleus of the left thalamus (fig. 2). The remainder of the left thalamus
was unremarkable. The left fornix and mammillary body were markedly atrophic as was the right
forceps major of the corpus callosum (figs. 1 and 2). The right thalamus, hippocampus, fornix and
mammillary body were grossly and microscopically normal.
No Alzheimer-like changes were found either in the hippocampus or in the multiple sections of
cerebral cortex. Other small old infarcts were found in the right parietal (angular gyrus) and occipital
lobes and a small recent (week to ten days old) infarct involved the anterior limb of the left internal
capsule and putamen at the level of the anterior commissure (fig. 2). The posterior third of the left
substantia nigra was pale (fig. 1) and microscopically numerous macrophages were filled^with
melanin. The remainder of the brain-stem and cerebellum was unremarkable.

Results of Special Cortical Function Investigations

(1) Capabilities with visual symbols: When presented a letter, word or symbol, she invariably
gave a letter name. When given a number, she would also give a letter name unless directed to name
a number, in which case she would correctly name approximately 50 per cent of single digits. If the
number had a dollar or cents sign, she would then usually give a price name. Repeated testing over
a thirty-month period demonstrated that despite her inability to name letters, the patient could:
A. count the number of letters or words present;
B. correctly position individual plastic letters or numbers when these were aligned incorrectly;
for example fig. 3:

A

FIG. 3.

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FIG. 2.—Dorsal view of same horizontal section as fig. 1 showing the discoloration and atrophy
of the splenium of the corpus callosum extending into the right occipital lobe (thick arrow). The
old infarct in the left thalamus is seen together with a more recent infarct in the right parietal lobe
and a small acute infarct in the left putamen (thin arrow). Note the absent left fornix and the
atrophic left temporal lobe.

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TABLE I.—PATIENT'S RESPONSE TO A LIST OF WORDS

Red
Hot
Cold
Brown
Warm
Pink
Yellow

Spontaneous
reading
H
O
M
Boy
W
C
E

Red
Rose
Chair
Tulip
Boy
Daisy

Spontaneous
R
R
AH
B
HT
DAT

Any alike ?
No
No
No
No
No
No
No

Any colours ?

Any
Select word
temperatures ?
I say
Warm
+
Cold

Tan
Hot
Tan
Brown

TABLE II.—PATIENT'S RESPONSES TO A LIST OF WORDS

Any alike?
No
No
No
No
No
No

Any flowers? Any persons?
Hyacinth

+
—
+
—

Select word
I say

+
+

Boy
Flower

+

+
+

spontaneously proceed down the list giving each word a letter name or series of letter names or
volunteering an incorrect word name. She would then be asked a general question, e.g. Are any
alike? Later more specific queries, for example, are there any colours? would be directed to her.
On rare occasions, this degree of prompting would be followed by a correctly read word. With
the more diiect queries, she was often able to point to a word in the correct category despite the fact
that she often incorrectly named the word. For example, when seeking a colour, she would point
to brown and utter the word tan. When she was then asked even more directly to find the spoken
word, she would correctly identify about two-thirds of the words.
DISCUSSION

Holmes in 1950, and more recently Benson and Geschwind (1969), have reviewed
the morbid anatomy in cases of alexia without agraphia. Lesions in the left occipital

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C. correctly order letters alphabetically when a limited number were given Oess than 10) despite
frequently misnaming aloud the letters as she proceeded with the ordering. For example,
PN QOSR M
->-she aligned M N O P Q R S;
D. identify extra letters in a word without being given the word name; for example:
C A T A T. Patient covered the last AT, saying "Boy." "This part doesn't belong."
X E D O G . Covering the XE as being wrong, yet not being able to name the remainder.
E. identify misaligned letters or non letters in words without being given the word name. For
example,
B O O M , "The P (the patient pointing to the backward K) is backward."
G O • D. The patient said the word dog and pointed to the • , saying "That is not right."
G O J D. While pointing to the J, she stated "This is backwards."
F. Complete letters in a word. For example,
E A T=EAT
R A C F - should be RACE (though she read the word as "dog").
(2) Capabilities with words when cuing was introduced: the patient was presented with a list of
single words printed in capital letters one below the other (see Tables I and II). She would

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lobe, generally in the lingual or fusiform gyri or their subjacent white matter were
universally found. In addition, the splenium of the corpus callosum, thalamus,
and mesial temporal lobe were often infarcted in the distribution of the left posterior
cerebral artery. In our present case, a severe persistent hemisensory loss with
incoordination indicated a thalamic lesion in addition to the expected occipital lobe
infarction. The clinical diagnosis, an embolus from her rheumatic heart to the
proximal left posterior cerebral artery, was consistent with the post-mortem
observations. A large infarction had virtually destroyed the left occipital lobe, left
mesial temporal lobe, left hippocampal region, and splenium of the corpus callosum.
The left fornix and mammillary body were atrophied. No pathological changes
were identified in the right temporal lobe or in the right thalamus.
Several aspects of the case merit further comment:
Gerstmann's syndrome.—Gerstmann (1930) identified the angular gyrus and
occipital convexity as sites where damage might produce finger agnosia, right-left
confusion, agraphia, and dyscalculia. He listed the following as symptoms frequently
associated with the Gerstmann's syndrome: right hemianopsia, diminished right
optokinetic nystagmus, amnestic aphasia, impairment of reading capacity, colour
recognizing disturbance, dyspraxia, and disordered equilibrium. Potzl (1937)
and Critchley (1953) also have cited cases of parieto-occipital damage producing
the Gerstmann's syndrome. It should be emphasized that Gerstmann's syndrome
does occur with lesions of the posterior cerebral artery as well as with the more
commonly recognized infarction of the angular gyrus occurring with middle cerebral
artery disease, producing the alexia with agraphia syndrome. In our case the
Gerstmann's syndrome was transient. Posterior lesions may undercut the angular
gyrus ,and produce a more transient Gerstmann's syndrome.
Amnestic disturbance.—Bilateral damage in the mesial temporal lobe clearly may
produce a severe memory disturbance which is characterized by failure to evoke
memories, lack of improvement in performing a new task even after continued
practice, and retrograde amnesia with retention of otherwise normal intelligence and
verbal skills. Bechterew (1900) was the first to report amnesia as a sign of medial
temporal lobe damage produced in his case by bilateral infarction in hippocampus
and subjacent white matter. Victor et al. (1961) and Van Buren and Borke (1972)
report examples of severe, permanent memory disturbance in three patients with
bilateral mesial temporal lobe infarctions.
Geschwind and Fusillo (1966) reported a case of alexia without agraphia in which
unilateral infarction involving the left occipital lobe, left hippocampal region, and
left thalamus had been associated with a transient disturbance of memory lasting
seven weeks. Van Buren and Borke (1972) further described and diagrammed the
pathology of this case. Mohr et al. (1971) reported an example of left hippocampal
region infarction producing a severe recent memory disturbance which lasted until
death approximately twelve weeks after onset. Geschwind (1965), Geschwind and
Fusillo (1966), and Mohr et al. (1971) each suggested the possibility that unilateral
dominant temporal lobe ischsemia could produce a severe memory disturbance. Our

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17

BRAIN—VOL. XCVII

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patient is a further example of severe temporary memory disturbance related to a
unilateral temporal infarction; the duration of this disturbance (six months) is the
longest reported to date in unilateral temporal infarction unassociated with significant
disease of the contralateral temporal lobe.
The thalamus has been thought by some to play an important role in memory
function. Victor et al. (1961) have argued for the importance of bilateral thalamic
lesions in Korsakoff's syndrome. Stimulation in the left lateral thalamus may have
an effect on verbal recall performance (Ojemann, Blick and Ward, 1969) giving
weight to the speculation that the thalamus may have an alerting function in regard
to verbal memory. The cases of Mohr et al. (1971), Geschwind and Fusillo (1966),
and the present case are all examples of left thalamic dysfunction co-existent with left
temporal lobe infarction. Might the unique summation of thalamic and dominant
temporal lobe pathology produce a memory disturbance of a degree of severity not
found in cases of unilateral temporal lobectomy or unilateral damage limited to the
temporal lobe (Milner, 1966)?
Optokinetic nystagmus.—Cogan's rule states that in the presence of a hemianopsia
produced by an occipital lobe mass lesion, optokinetic nystagmus is reduced to the
side of the lesion; but, if the occipital lobe lesion is vascular, symmetrical optokinetic
nystagmus is found (Smith, 1963). Ling and Gay (1968) in their analysis of anatomical
pathways responsible for optokinetic nystagmus, point out that a lesion in one
occipital lobe which extends forward into the corpus callosum to involve fibres from
the other occipital lobe would produce asymmetrical optokinetic nystagmus. The
reduced optokinetic nystagmus found in our patient may be related to callosal
infarction. The patient initially provided an exception to Cogan's rule.
Visual agnosia.—An unusual feature of this case was the presence of the syndrome
of "associative visual agnosia" as described originally by Lissauer (1890). In this
syndrome, in the absence of a severe generalized intellectual disturbance or
encephalopathy, the patient has normal visual perception of an object but fails to
appreciate its character and meaning; but when appeal is made to another sensory
modality such as touch, the patient names and employs the object correctly. The
term associative was used by Lissauer to refer to a disorder interfering with connexions
between cortical regions as contrasted to "apperceptive visual agnosia" in which
there was an interference with the processing of primary visual information. The
intactness of our patient's visual perception was demonstrated by normal picture,
face, and object matching, and normal diagram copying. Tactile naming of objects
was immediate despite the fact that the same objects could not be identified when
shown visually. Naming of objects described to her verbally was also intact. Rubens
and Benson (1971) have recently reported a case of associative visual agnosia and
have reviewed the literature. Their patient had difficulty recognizing faces and
visual objects after a hypotensive episode. As in our patient, alexia without agraphia,
right hemianopsia, and colour naming deficits were also present. The syndrome
which has been called associative visual agnosia thus can be associated with the
syndrome of alexia without agraphia, though this is rare. Much recent debate has

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centred about the validity of the concept of visual agnosia. Bay (1953) has
emphasized the difficulties in assessing "primary visual perception". Teuber (1965,
1968) and Bender and Feldman (1972) have been impressed with the combination of
intellectual defects, naming disorders, and altered primary perception in patients
with "so-called visual agnosia." Our patient certainly had difficulties with visual
objects disproportional to her defect in visual perception, but did not have a pure
naming difficulty or a generalized severe intellectual disturbance.
Number reading.—The reading of numbers is often preserved in cases of alexia
without agraphia. In Dejerine's (1892) original case, number reading was normal.
Symonds (1953) and Geschwind (1965) emphasize the sometimes striking preservation
of the capability of reading complex numbers in patients with severe letter and word
naming defects. Geschwind (1965) speculated that use of somaesthetic features of
number concepts (in early life numbers are learned by counting on ringers) could
allow the patient to utilize alternate non-visual somatosensory pathways for
identification of written numbers. Our patient's inability to name numbers is unusual
in the alexia without agraphia syndrome. Could the absence of available somEesthetic
pathways in relation to her severe right hemianeesthesia be related?
Non-verbal visual capabilities with letters and numbers.—Numerous examples
have been given of our patient's non-verbal capabilities with letters, words and
numbers. Is this performed by the virtually intact right hemisphere? Data regarding
the right hemisphere handling of linguistic material is fragmentary. Dimond,
Gibson and Gazzaniga (1972) examined normal patients' capabilities of matching
four letter nouns given sequentially in either visual field, and found matching in the
left visual field (right hemisphere) equal to that in the right visual field. They
speculated that word matching might be done by physical characteristics independent
of language. In commissurotomized patients, Teng and Sperry (1973) found
essentially correct verbal identification of 20 per cent of letters and 35 per cent of
digits presented to the left visual field. Gazzaniga and Hillyard (1971) demonstrated
that some commissurotomized patients, when a single noun was flashed to the left
visual field, could select with the left hand the correct object from a series of objects
placed out of view. In addition, if a simple picture of a boy were shown in the left
visual field, some subjects could retrieve a card with the word Boy printed on it, or
correctly choose the spoken word "Boy" when a series of alternatives was read
aloud. One of their patients could name aloud numbers in the left visual field,
though in a delayed fashion. This patient's explanation gives insight into his method:
"What I do is to count up until I hit a number that sticks out—then I stop and tell
you what it is." Thus this "speech function", i.e. verbal identification, may be done
by using matching strategy. The right hemisphere may have some non-verbal
capabilities with visual symbols. In addition, the left hemisphere may be able to
generate, with the aid of cues, pictures of visual symbols which subsequently can be
matched to visual percepts analysed by the right hemisphere.
Cuing.—Kreindler and Ionasescu (1961), using a technique they called auditory
and visual unblocking, analysed the performance of their patient with alexia without

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agraphia who had normal picture naming. Their patient when shown a single picture
of an object could correctly choose the correct single written word description from a
written list of words, but could not choose the correct picture from a group, when
given only a single written word description. Their patient could also select the
correct sentence from a group, when the sentence was spoken aloud. The authors
suggested that "the pictures of the symbols of language were not destroyed but
merely could not be evoked visually".
Kreindler and Ionasescu's patient could read (i.e. select a correct written word from
alternatives) when given a verbal language input. Since he was also able to accurately
name and describe pictures and give synonyms for spoken words a picture input was
similar to giving him a word name. One could interpret the data from their patient
and ours, in the language of the disconnexion hypothesis, as demonstrating a
unidirectional type of disconnexion, i.e. visual language input was disconnected
from spoken language output but spoken language input was not disconnected from
visual selection output. It is possible that many cortical pathways are not
representable by equidirectional equation equilibria (Geschwind and Fusillo, 1966;
Mohr, 1972).
The capabilities of patients when cuing is introduced bring up the important
question of why patients are able to perform a task under certain circumstances and
not under others. Fatigue, perseveration, lack of co-operation, or other non-specific
factors are applicable but may be partially avoided by altering the timing and pace
of the examination, and by testing on multiple occasions. Cuing seems to have
improved our patient's and Kreindler and Ionasescu's patient's performance reliably
and reproducibly. This would be difficult to explain if verbal behaviour was
anatomically disconnected from visual behaviour bidirectionally. A unidirectional
disconnexion is one explanation as outlined above. An alternative idea is that the
visual-verbal disconnexion is one of interference with the general process of obtaining
visual information.
Ramon y Cajal and Polyak (Brindley, 1970) identified anatomically fibres in the
optic nerve going to the retina from the central nervous system. Granit (1955,
1962), Brindley (1970) and others have shown that stimulation within the central
nervous system can potentiate or inhibit responses of the retinal ganglion cell to
light; that is, that there are centrifugal pathways to the primary visual receptor
effecting or regulating the stream of afferent impulses. This would be an example of
how the brain may be able to help set, tune or filter the mass of incoming sensory
data from primary receptors, to help answer specific queries or interests. In the
visual sphere, what one sees depends to a great extent on what one "looks for".
Tyler (1968) has recently reviewed the interactions between "looking" and "seeing":
Luria (1966) has divided the process of visual perception into a step process, the
first step of which is scanning of a visual object for a "dominant cue". This cue then
allows a definite visual hypothesis to form in which the object is classified into a
particular category. Perceived cues continue to evoke a number of alternatives
which are explored visually by palpitatory eye movements allowing perception of

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SUMMARY

A 68-year-old female with rheumatic heart disease suddenly developed a right
hemianopsia, right hemisensory loss, and complete alexia. An embolic occlusion of
the left posterior cerebral artery had caused a large infarction involving the left
temporal and occipital lobes and their subjacent white matter. A small infarction
was seen in the left thalamus. Unusual features of this case included faulty number
naming, disordered visual object and picture identification, and a severe defect in
recent memory function which lasted six months. Emphasis is placed on the patient's
capabilities with visual symbols when naming was not required, and on her behaviour
when cuing or prompting was introduced. The defect in alexia without agraphia may
involve faulty visual exploration of a written word symbol, and not merely a pure
disconnexion between visual input in the right hemisphere and the language region of
the left hemisphere.
Performed in the Behavior Research Laboratory, Massachusetts General Hospital, under the
direction of Dr. M. Sidman.

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visual stimuli with continuous checking of changing hypotheses until an answer is
reached.
Our patient seemed to have a defect in her spontaneous approach to visual objects,
especially written symbols, which at times was helped by verbal cuing. Kreindler
and Ionasescu's patient had a similar defect with written symbols. A patient of
Albert et al. (1973) had a similar defect in spontaneous naming of a written word yet
could correctly match words to pictures, choose a word correctly within a spoken
semantic category, and could eliminate the single odd word in a group of written
words. An insight into our patient's difficulties is given by the patient's own reports
early in her course. She had just successfully completed matching a picture of an
object in the centre box to its identical counterpart on the periphery. Now instead of
a picture, a simple word like "man" or "hen" appeared in the centre of the screen,
the task being to match the word with the appropriate picture.1 She failed on 20 such
matches, each time repeating that "nothing looks like the thing in the centre". She
did not acknowledge that the thing in the centre was a word, a symbol for something.
A patient of Casey and Ettlinger (1960) with alexia without agraphia is quoted as
saying, "The words didn't mean anything—the shapes definitely seem familiar.
Given Greek and Latin letters mixed, I would have known which were which—the
sound belonging to the letters did not get confused, I got stuck on the visual side."
It seems clear that the disorder in alexia without agraphia is a modality specific
visual disturbance. The defect in this syndrome may be one of faulty visual tuning, of
not "knowing what to look for". In Luria's terms, there may be difficulty in isolating
the initial "dominant cue". The fault may be helped by cuing or prompting the
patient to seek specific words, sentences, or categories of words. These cues evoke
visualized symbols from the left hemisphere speech region which are correctly
matched to percepts visualized by the right hemisphere. The left calcarine or
paracalcarine cortex, or the thalamus may take part in setting or tuning the visual
analyser.

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ACKNOWLEDGMENT

We acknowledge the advice of Drs. C. Miller Fisher, N. Geschwind, M. Ginsburg, H. R. Tyler,
M. Sidman and J. P. Mohr.
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{Received September 19,1973)