RECOVERY FROM ALEXIA WITHOUT AGRAPHIA:
REPORT OF AN AUTOPSY
Masayoshi Kurachi, Nariyoshi Yamaguchi, Tohru Inasaka and Hosaku Torii
(Department of Neuropsychiatry and the First Internal Medicine,
Kanazawa University, and Department of Neuropsychiatry, Kanazawa
Medical University)

Since the first detailed description of clinical and pathological findings
of pure alexia, i.e., alexia without agraphia, by Dejerine in 1892, about
30 autopsy cases have been reported in the medical literature. Several
features of this syndrome were explained by the callosal disconnexion theory
(Geschwind, 1965; Geschwind and Fusillo, 1966), but underling impairment of the neuropsychological processing is not yet clearly defined. In this
respect, our two previous reports (Kurachi, Fukuta, Jibiki, Koyama and
Torii, 1975; Kurachi, Fukuta, Jibiki, Enokido and Torii, 1977) confirmed
that the script form recognition in alexia without agraphia is well preserved in
spite of severe alexia and copying disturbance ameliorates when the patient
uses his left hand.
The present case is a 58-years-old Japanese teacher of German literature
who suffered twice from cerebrovascular accidents and had pure alexia;
this symptom almost disappeared, however, in 10 weeks and 2 weeks
respectively. This case is milder than any other hitherto reported autopsy
case of alexia without agraphia. We will describe the recovery process and
examine the correlation between this good recovery and the pathological
findings, and compare this with other autopsy cases.

CASE

REPORT

Case K. T. A 58-year old right-handed Japanese teacher of German literature
had been hypertensive for ten years fell backward in the lavatory wihout loss
of consciousness on the evening of December 23, 1969. He complained next
morning of double and triple vision, and he watched the face of his wife intently,
saying she appeared to have no face. On that day he came to the clinic of
Ophthalmology at Kanazawa University Hospital. His visual acuity was 5/24
and funduscopic examination showed arteriosclerosis retinae Scheie HISI - 2. Blood
pressure was 170/110 mmHg. He gave evidence of disorientation for time,
memory disturbance, complete alexia, and weakness of the right extremities. On
Cortex (1979) 15, 297-312.

M. Kurachi, N. Yamaguchi, T. Inasaka and H. Torii

298

December 26, he developed difficulty in walking and was admitted to another
hospital.
During this period he complained that, "The pillar looks inclined and the
cup looks distorted." He could write but the line slanted upward to the right.
He felt as if his right extremities were not his own. Thereafter memory impairment
and motor disturbance of the right extremities improved and he could eat and
walk by himself. On January 12, he was referred to the Department of Internal
Medicine at Kanazawa University Hospital.
On admission, consciousness was clear. Blood pressure was 190/94 mmHg.
Neurological examination showed right homonymous hemianopsia (Figure 1),
L

Fig. 1 -

R

Visual field on February 25, 1970, showing right homonymous hemianopsia.

exaggerated jaw reflex, and slight weakness and increased tonus of the right
extremities. There was slight hyperreflexia of the upper extremities on both
sides. Knee jerks were exaggerated but more pronounced on the right side. Plantar
reflex was diminished on the right side but pathological reflexes were not
elicited. There was only slight hypesthesia and hypalgesia on the right side of
the body, but deep sensation was preserved.

Laboratory examination
EEG on January 30, 1970, showed paucity of alpha aCtiVity in the left
occipital lead. The cerebrospinal fluid was clear and colourless, with a cell count
of 4/3, Pandy + +, Nonne-Apelt +, protein level 107 mg/dl, sugar 96 mg/dl,
and chloride 129 mEq/L.

General intellectual assessment
He was slightly disoriented for time. Digit span did not go beyond
6 figures. Remote memory was preserved but recent memory was impaired. Even
on the 36th hospital day, he could not remember his attending doctor's name.
Using the Japanese version of the Wechsler Adult Intelligence Scale on March 5,
1970, the patient had a full IQ of 110, verbal IQ 124 and performance IQ 94
points. The each scaled score of the subtest was as follows: Information 11,
Comprehension 17, Arithmetic 10, Similarities 11, Digit Span 10, Vocabulary 17,
Digit Symbol 3, Picture Completion 5, Block Design 6, Picture Arrangement 7,
Object Assembly 12.

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299

Neuropsychological findings
Speech

There was no disturbance of spontaneous speech but he occasionally showed
hesitance and paraphasia in naming objects. Comprehension was normal, and
there was no disturbance in repetition.
Reading

On admission, reading was severely impaired and he could not read words
even written by himself. On the 8th hospital day, he could read only numbers
but scarcely read the alphabet, Kana script, and Kanji script. On the 19th hospital
day, reading of most of the alphabet and Katakana script became possible, but
he could read only one-third of the Hiragana script. He read words by using
his finger to trace the outline of each letter (somesthetic reading). Reading of
sentences was impossible. English and German words were read by individual
letters. On his 30th hospital day, he could read almost all Hiragana script, and
reading of words gradually became possible. On the 36th hospital day the words
in Kana and Kanji script· could be read but occasionally he showed paralexia and
perseveration. He could write spontaneous sentences fluently but had difficulty
in reading them himself, and there were many instances of paralexia because
of confusing similar written forms. In reading certain expression, he also used
different words having the same meaning. On the 48th hospital day, in reading
a German sentence: "Es war einmal ein reicher Mann, der trug trotz seines
ungeheuren Reichtums nur einen alten schmutzigen Mantel," he showed paralexias
such as seines ~ eines, ungeheuren ~ ungeneuren, Reichtums ~ Reichtuns, einen
~ einer, and it took ten minutes to read this short sentence. On the 57th hospital
day, several lines could be read, though slowly and supplemented by somesthetic
reading.
Writing

Writing ability was almost preserved at the time of his admission. He
could dictate the sentence but could not read it later. He could write sentences
not only in Katakana, Hiragana, and Kanji script, but also in English and
German. There were occasionally some omissions and mistakes and the form
of the letters and ideographs was often irregular. After the 35th hospital day,
the form was excellent, and he wrote several lines of sentences fluently. Copying
was performed awkwardly and often with errors.
Calculation

Mental arithmetic and calculation on paper were moderately disturbed on
admission. On the 22nd hospital day, dyscalculia became minimal and on the
46th hospital day, it disappeared.
Colour-naming

On the 43rd hospital day, he correctly named red, green, black, and white,
but misnamed dark blue as light-blue, orange as brown, violet as brown ... green ...
violet. Selection of colours on oral command was performed correctly. On his
57th hospital day, he named correctly brown, yellow-green, dark blue, yellow,

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M. Kurachi, N. Yamaguchi, T. Inasaka and H. Torii

red, and pink; the last of these was identified with hesitation. For violet he
said, "It isn't green ... nor violet... it is green."
Somatognosis

There was slight confusion in finger gnosis and right-left discrimination
on admission, but on the 8th hospital day, finger recognition was correct. The
hand-eye-ear test (Head) on the 38th hospital day was performed correctly in
response to oral commands and also by confronted imitation.
Praxis

There was no apraxia but spontaneous drawing of an automobile was not
well executed.
Visual gnosis

There was no object agnosia, prosopagnosia, or metamorphopsia, although
the last of these seems to have existed before his admission. In examinations
from the 36th to 48th hospital day, he read clock times without difficulty and
explained correctly the situational picture. Test for topographical memory showed
slight impairment. In Poppelreuter's hidden figure test, he could find a hammer,
a pot, a kitchen knife, but pointing to the iron, he said, "There exists one thing
more. I wonder what this is?"
In summary, neuropsychological examination showed striking alexia with
preserved writing ability, recent memory impairment, and slight impairment in
word-finding, calculation, right-left discrimination, colour naming, constructional
tasks, and topographical memory. These alexic symptoms improved gradually,
and on discharge (March 11, 1970) there were only a few paralexias in reading
a sentence.
After discharge he continued to practice reading, and from April 1971 he
resumed his lectures in German. In October 1971 he complained, "It takes more
time to read than before the attack. Comparing Japanese and German, it takes
more time to read German and is not as easy to understand the meaning of
German sentences." When tested, however, he could read both languages aloud,
and understand German as well as Japanese. There remained right homonymous
hemianopsia, slight colour naming disturbance, and hyperactive deep tendon
reflexes, but other symptoms were improved.
On the morning of October 28, 1971, while he was reading a newspaper
he became unable to read it although he could see the script clearly, and he
lost consciousness for 30 minutes. After he recovered consciousness, although
spontaneous speech and comprehension were normal, he showed global alexia,
i.e., he could read neither letters nor words except numbers. Spontaneous writing and dictation were performed easily, but copying was greatly impaired and
he copied both Japanese and German words as if they were meaningless forms.
In addition, there were disorientation for time, impairment of recent memory,
and dyscalculia. Neurological examination revealed hyperactive deep tendon reflexes,
ankle clonus and extensor plantar reflex on the right side. On the next day,
he was admitted to the Department of Internal Medicine of Kanazawa University
Hospital.
Ophthalmologically, visual acuity was 5/24 on the right, 12/24 on the

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301

left. Visual field was almost the same as February 25, 1970. EEG on November
6, 1971, showed occipital dominant alpha rhythm of 10 Hz, but there was
asymmetry of amplitude (righ > left) in the parietal and occipital leads. At
the time of admission disorientation for time disappeared but there remained
severe impairment of recent memory. On the 6th hospital day, he did not
remember examinations such as the 50g glucose tolerance test performed the day
before. But 7 figures digit span was possible.
Neuropsychological findings were as follows. Spontaneous speech was fluent
and intelligent. There was no difficulty in word-finding and comprehension.
Reading disability improved more rapidly than in the case of the former
admission. On the 3rd hospital day he could read several lines of sentences in
Kanji and Kana script without difficulty. But it took about four times longer
to read the newspaper than normal, and he also omitted some words in sentences.
By the 15th hospital day, reading ability had almost recovered to the state before
the second attack. During this period of hospitalization he wrote a diary in
German. There were also no errors in copying, and he could write texts after
reading them. Colour naming: On the 3rd hospital day, he correctly named black,
red, blue, green and white, but to orange he said, "It is not light-brown but..."
Selection of all these colours to oral command was performed correctly. On the
6th hospital day, red was called "pink" and yellow was called "light blue."
There was no difficulty in calculation, spontaneous drawing, constructional praxis,
somatognosis, and topographical memory.
After his discharge on November 13, 1971, he received anticoagulant
therapy. He planned to begin lectures in April 1972, but on March 8 a series
of convulsive seizures occurred suddenly and his eyes were deviated to the
right. Three hours later, these convulsions ceased but he was comatose. Deep
tendon reflexes of the right leg were hyperactive and there were bilateral
extensor plantar reflexes. Blood pressure remained low and he died on March 10,
1972. The total clinical course was 26 months.

PATHOLOGICAL FINDINGS

Aorta showed moderate atherosclerotic changes, and there was excessive
bleeding into the right retroperitoneal space with peritonitis due to perforation of a duodenal ulcer.

Nervous system
Macroscopic findings
Brain weighed 1330g. The basilar and vertebral arteries showed striking
atherosclerotic changes and there was severe stenosis of the left posterior
cerebral artery proximally. The right vertebral artery was hypoplastic, and
its diameter was only one-fifth of the left one which had almost equal size to
the basilar artery. Gross appearance of the brain showed slight atrophy of
the left occipital lobe, and there were two old yellow infarcts in the left
fusiform gyrus, extending into the lingual gyrus. The left mammillary body
was atrophic (Figure 2).

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M. Kurachi, N. Yamaguchi, T. Inasaka and H. Torii

Fig. 2 - Basal sur/ace of the brain showing two old infarcts in the left fusiform and
lingual gyrus. Note atrophy of the left mammillary body. Demarcated area represents the extent
of the subcortical infarct. Numbers of the right side indicates the number of the coronal section
and the arrow indicates calcarine fissure.

The brain was sectioned coronally at 1 cm intervals. Anterior sections
revealed slight dilatation of the left lateral ventricle. There was an old
cavitated infarct in the left fusiform, lingual and parahippocampal gyrus,
sparing the calcarine cortex.
The left external and internal sagittal stratum was pale and atrophic,
and its lower part was also involved by the above-mentioned infarct. The
lower third of the splenium of the corpus callosum showed several linear
brown foci with a round defect of 1 mm diameter in its mesial part.
The left fornix was atrophic and there was a small linear infarct in the
left hippocampus. The left thalamus showed a small old infarct in the
medial nucleus and a wedge-shaped old infarct in the pulvinar.
There were no pathological findings in the right hemisphere. Brain
stem: There were two small old infarcts in the medial lemniscus and in
the pontine longitudinal fasciculus in the right side of the pons. Cerebellum
showed no pathological changes.

Histopathological findings
The brain was embedded in celloidin and cut serially at intervals of 20
microns. Each lOOth section was stained by the methods of Woelcke, Kluver-Barrera, haematoxylin and eosin, and Holzer.

Alexia without agraphia

303

Left hemisphere
There was a cavitated infarct of the white matter in the posterior twothirds of the fusiform gyrus, the lingual gyrus, and the posterior region of
the parahippocampal gyrus. This lesion reached to the floor of the inferior
and posterior horns of the lateral ventricle. The cortices of the fusiform and
the lingual gyrus were also involved but their summits were partially preserved. In all sections, the cortices facing the inferior temporal sulcus and
the calcarine fissure were preserved.
The necrotic lesion were cavitated and bundles of connective tissue fibres
extended from the walls. There were many phagocytes and hyalinized arterioles. Adjacent to this lesion there were scattered hypertrophic astrocytes and
perivascular cell infiltration.
A part of the cortex of the parahippocampal gyrus, and the pyramidal
cell layer of the subiculum were also involved by the lesion. The hippocampus showed complete loss of pyramidal cells in Sommer's sector (h 1) with
relative preservation in h 2• A small linear infarct was present in the region
from the endofolium (h5) to the fimbria of the hippocampus. The alveus,
the fimbria and the fornix were severely demyelinated. The left anterior
nucleus of the thalamus was also infarcted and the nerve cells of the left
mammillary body were moderately decreased in number (Figure 3). In the
pulvinar of the thalamus there was an old cavitated infarct with neuronal
loss in its adjacent medial and ventrolateral region (Figure 3 and 4).

Fig. 3 - Coronal section through the mammillary body. The left mammillary body is atrophic
and there is an infarct in the anterior nucleus
of the left thalamus. Woelcke myelin stain
(Section 4-251).

Fig. 4 - Coronal section through
the pulvinar showing old infarct
of the left hippocampal and fusiform gyri and the left pulvinar.
Note degeneration of the left
fornix. Woelcke myelin stain
(Section 6).

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M. Kurachi, N. Yamaguchi, T. Inasaka and H. Torii

The splenium of the corpus callosum showed band-like demyelination
and dense gliosis in the lower third (Figure 5). The bulbus of the posterior
horn was atrophic and there was similar demyelination and gliosis in the
radiation of the corpus callosum (Figures 6 and 7). Posterior to the splenium,

B
Fig. 5 - Coronal section through the splenium of the corpus callosum. The splenium is
degenerated in the lower third. A. Woelcke myelin slain (Section 7-104). B. Holzer stain
(S ection 7-103).

A
B
Fig. 6 - Coronal section about 0.1 em posterior to the splenium. Note myelin loss and
gliosis of the right bulb of the posterior horn. A. Woelke myelin stain (Section 7-303). B. Holzer
stain (Section 7-302).

A
B
Fig. 7 - Coronal section about 0.8 em posterior to the splenium. Note degeneration of the
occipital radiation of the corpus callosum on both sides. A. Woelcke myelin stain (Section
8-1 10). B. Holzer stain (Section 8-111).

Alexia without agraphIa

305

the tapetum showed band-like demyelination and gliosis (Figure 8). In the
coronal sections anterior to the splenium, however, the tapetum was preserved
except its lower border.

A
B
Fig. 8 - Coronal section about 2.8 em posterior to the splenium. Note myelin loss of the
left tapetum. A. Woelcke myelin stain (Section 10-110). B. Holzer stain (Section 10-111).

The internal and external sagittal stratum along the lateral wall of the
inferior horn showed demyelination and gliosis. This secondary degeneration
could be traced to the lateral geniculate body (Figure 9).

Fig. 9 - Coronal section through the lateral geniculate body. Note degeneration of the
left internal and external sagittal layer up to the lateral geniculate body. Woelcke myelin stain
(Section 5-251).

There was laminar rarefaction in the 3rd layer of the cingulate cortex.
Neurons in the 3rd and 4th layer were decreased in numbers and remaining
cells showed ischaemic cell change. Similar changes were also found in the
precuneus and the anterior part of the upper lip of the calcarine cortex but
not in the cuneus. In the more posterior section, however, the both lips
of the calcarine cortex were well preserved. The cortices in the convexity of

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M. Kurachi, N. Yamaguchi, T. Inasaka and H. Torii

the occipital lobe and the parietal lobe were also preserved. In the white
matter of the occipital lobe, perivascular spaces were dilated and contains
a few hemosiderin granule cells.

Right hemisphere
Demyelination and gliosis of the splenium was extended to the bulbus
of the posterior horn (Figure 5). This degeneration, though thinner than
that of the left, continued to the radiation of the corpus callosum and the
tapetum (Figures 6 and 7). There were scattered fatty macrophages and
perivascular cell infiltration.
There was no primary lesion in the right hemisphere, except a small
perivascular infarct in the subcortical white matter of the precentral gyrus.

DISCUSSION

Clinical course
Clinically the present case showed severe alexia and memory impairment
without agraphia. Reading ability was facilitated by somesthetic reading and
there was disturbance in copying, associated with slight difficulty in naming
colours, and right homonymous hemianopsia. An important characteristic of
this case was good recovery: After the 1st attack reading ability almost
returned in about 10 weeks and thereafter the patient taught German at
school. After the 2nd attack, the symptoms reappeared but he recovered
in only 2 weeks. The recovery was as follows: Initially he showed global
alexia, i.e., he was able to read numbers, but not letters or words. Then
reading of single letters and the Katakana script became possible. In this
stage there was still severe disturbance in reading words and Hiragana script.
Then reading of the Hiragana script returned. Finally he showed "alexie
de la phrase." But at discharge, he could read long sentences without much
difficulty.
Dejerine's patient (1892) remained globally alexic for 4 years from
onset to death. Even in Bonvicini and Potzl's case (1907) having the smallest
lesion among the autopsy cases, the alexic symptoms seem to have continued
at least 5 months. In clinical reports, several cases showed good clinical
recovery such as the second case of Dejerine and Pellisier (1914), but among
the cases verified by autopsy our case shows the most excellent restoration.
In the following section we examine the reason as suggested by the
pathological findings.

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307

Pathological findings

Pathological examination showed an old infarct in the posterior twothirds of the fusiform, in the almost whole lingual and in the posterior
border of the parahippocampal gyrus of the left hemisphere. The transverse
fasciculus of Vialet was also involved but the left cuneus and the calcarine
cortex was preserved. There was degeneration of the lower third of the
splenium of the corpus callosum, its occipital radiation and the tapetum
on both sides. The left cingulate cortex and the precuneus showed laminar
rarefaction with ischemic cell change but this change was relatively recent.
He had suffered twice from apoplectic attacks of alexia without agraphia.
But it is difficult to distinguish the first from the second lesion. Considering that the symptoms of the second attack were milder and he recovered
rapidly, it is reasonable to assume the above-described old infarct occurred
at the first attack.
Thirty-one autopsy cases of alexia without agraphia have been reported
to our knowledge. The extent of necessary and sufficient lesion to produce
this sympthom was formulated by Hoff, Gloning and Gloning (1962):
(1) The white matter of the lingual gyrus. (2) The splenium of the corpus
callosum. (3) The external sagittal stratum, though the macular bundle in
its dorsal part is spared in pure cases. (4) The basal part of the internal
sagittal stratum. (5) The transverse fasciculus of Vialet which belongs
to the lingual gyrus and usually the fasciculus of Sachs, i.e. the transverse
association system of the cuneus. (6) In the typical case, the basal temporal
lobe and Ammon's horn as well as the fornix were often involved. In
all cases, the lesion existed in the dominant hemisphere.
Most of the autopsy cases showed this localization except for Poussepp's
(1923) one. The lesion in our case is almost identical to the lesion described
by Hoff et al. But in our case, the lesion of the splenium was confined to the
lower third, and the left cuneus was preserved. The significance of
involvement of the splenium in alexia was indicated by Foix and Hillemand
in 1925 and recently supported by Geschwind (1965) and Geschwind and
Fusillo (1966). Foix and Hillemand's patient showed right homonymous
hemianopsia without alexia. Autopsy confirmed that there was an old infarct
in the left lingual and fusiform gyrus but the corpus callosum was intact.
Certainly, almost all the autopsy cases of alexia without agraphia related
to cerebrovascular disease showed lesions in the splenium. Scheller (1966)
reported an alexic case with optic aphasia in which there was no lesion
in the splenium, but his case showed extraordinary marked atrophy in the
left occipital lobe, so callosal fibres may have been impaired near the
splenium. In Dide and Botcazo's case (1902), the corpus callosum appeared
completely normal. Their case, however, showed infarcts bilaterally in the

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M. Kurachi, N. Y amaguchi, T. Inosaka and H . Torii

lingual and fusiform gyri, and bilateral lesions may have the same meaning
as involvement of the splenium.
In view of the correspondence between the clinical symptoms and the
extent of the lesion in the splenium and the left occipital lobe, an important
case may be the one reported by Lissauer (1890), of which the pathological
findings were given by Hahn (1895). This case is famous as a typical case
of psychic blindness of Lissauer's type, but it also accompanied alexia
without agraphia. In Lissauer's case, the main lesion occupied the left
cuneus, extending to the left lingual and calcarine gyrus. The splenium
showed softening in its lower two-thirds: two times larger than that of
Dejerine's case, and there was no lesion in the right hemisphere, except
for secondary degeneration of the forceps following softening of the splenium.
Similar findings were observed in the surgical excision of the splenium.
When the excision was confined to the splenium, the patient showed alexia
in his left visual field, but when the excision was extended to the truncus
of the corpus callosum the patient showed, in addition to alexia, objectnaming disturbance in his left visual field (Trescher and Ford, 1937; Maspes,
1948; Iwata, Sugishita, Toyokura, Yamada and Yoshioka, 1974).
Hecan, Ajuriaguerra and David (1952) reported that even after total
resection of the left occipital lobe, patients were able to read short texts
in 4-6 weeks. The reason for this may be attributed to sparing of the
splenium. So the good recovery of our case seems to be due mainly to the
fact that the lesion of the splenium was partial.
The second characteristic of our case was sparing of the left cuneus and
the transverse fasciculus of Sachs which connects the upper lip of the calcarine
cortex to the convexity of the occipital lobe, although the white matter of
the lingual and the fusiform gyri was almost completely destructed. Recently
Greenblatt (1973) reported an interesting case of alexia without agraphia
or hemianopsia. This right-handed patient showed severe alexia, but no visual
field defect on confronting test, nor colour naming disturbance. Pathological
examination revealed the glioblastoma multiforme occupying the inferomedial
white matter of the left occipital lobe invading grossly into the splenium
of the corpus callosum. The transverse fasciculus of the lingual gyrus (Vialet)
and the vertical occipital fasciculus were also involved . The author proposed
that, "within each occipital lobe, the inferior association tracts and the ventromedial (lingual and fusiform) gyri are necessary for reading." This conclusion
is more likely than Dejerine and Vialet's (1893) view that involvement of
the inferior part of the inferior longitudinal fasciculus is responsible for
alexia without agraphia, and it may well explain the appearance of alexia
also in our case. However, another factor is necessary, in our view, for
persistence of alexic symptoms. As stated above, Dejerine's patient did not

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309

recover from alexia in spite of enduring exercises. Comparing Dejerine's to
our case, the splenium in the latter was nearly the same as the former,
but the major difference is that the cuneus as well as the lingual gyrus was
involved in the former. In Bonvicini and Potzl's case, there were two small
lesions in the medial surface of the left occipital lobe, i.e. in the cuneus
and the lingual gyrus. So preservation of the cuneus and the fasciculus
of Sachs seems also to be related to recovery from alexia.
Alexia without agraphia occasionally accompanies object agnosia or optic
aphasia; this is described in 9 of 31 autopsy case. As stated before, the main
lesion of Lissauer's case occupied the left cuneus and similarly, in other
autopsy case of alexia without agraphia which accompanied object agnosia
(e.g. Muller, 1892; Souques, 1907; Niessl von Mayendorf, 1935) the left
cuneus was extensively involved as well as the lingual gyrus. In Dejerine's
case which did not show optic aphasia, the cuneus was also involved, but
the lesion was restricted to its posterior part. On the basis of the abovedescribed evidence, it seems likely that when the cuneus is partially involved,
in addition to the spleno-lingual region, alexia becomes persistent and when
the cuneus is destroyed more extensively it accompanies object agnosia
or optic aphasia (Table I).
TABLE I

Clinico-Pathological Correlation of Alexia and Object Agnosia
Foix and
Hillemand

Present
case

Dejerine

Lissauer

Alexia

(-)

Recovered

Persistent

Persistent

Object agnosia

(-)

(-)

(-)

(+)

Lingualis

Involved

Involved

Involved

Involved

Splenium

Intact

Involved
lower 1/3

Involved
lower 1/3

Involved
lower 2/3

Cuneus

Intact

Intact

Partially
involved

Extensively
involved

Clinically

Pathologically

Contradictory findings were reported in regard to the lesion which
produces object agnosia. Nielsen (1937) concluded from 15 autopsy cases
that within the left occipital lobe the cortex of the 2nd and 3rd convolutions
represents an area essential for the recogllltlOn of objects. Nevertheless
object agnosia was not observerd in Hecaen's 7 patients who underwent

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occipital lobectomy (Hecaen et aI., 1952). According to Hecaen's observation, the extent of occipital lesion appears less significant than the splenium
lesion, but there remains possibility that occipito-temporal infarction exerts
somewhat different effect from the occipital lobectomy on the visual-verbal
processing.
We postulate the restricted lesion in the splenium and sparing of the
cuneus as the reason for good recovery of our case. These two points
can be understood uniformly, considering the fibre distribution of the corpus
callosum.
From the study of secondary degeneration of the splenium following
a lesion of the occipital lobe, Dejerine (1892, 1895) divided the splenium
into three parts: superior, inferior, and posterior. According to him, the
superior part is essentially the posterior edge of the truncus of the corpus
callosum and contains no fibres from the occipital lobe. The inferior part
contains fibres from the 2nd and 3rd occipital gyrus, and the lingual and
fusiform gyrus; these fibres form the forceps major. The posterior part
is the true posterior genu of the corpus callosum and contains fibres from
the first occipital gyrus and the cuneus.
The lesions in our case were mainly in the inferior part of the splenium,
as well as the lingual and fusiform gyrus. The left intact cuneus seems
to maintain connexion with the right cuneus. Owing to the right homonymous
hemianopsia, visual input reached only the right occipital lobe. But this
information may have been transported to the left cuneus and the convexity
of the left occipital lobe. Preservation of this system may explain the good
recovery.
Memory impairment

Memory impairment in patients was noticed in several autopsy cases with
alexia without agraphia (Dide and Botcazo, 1902; Souques, 1907; Boudin,
Brion, Pepin and Barbizet, 1968; Geschwind and Fusillo, 1966; Caplan and
Hedley-Whyte, 1974). Delay's first case (Delay, Brion, Escourolle and Marques, 1961) may be included in this group.
Our case showed disturbance of recent memory for 55 days after the
first apoplectic attack and six days after the second admission. Pathological
examination revealed infarcts in the left parahippocampal gyrus, the hippocampus and also the left thalamus. There was secondary degeneration
in the alveus, fimbria and fornix, and the left mammillary body was atrophic.
Recently the left hippocampus is considered to be related to verbal
memory and the right hippocampus to non-verbal memory (Milner, 1968a,
1968b, 1972). Geschwind and Fusillo (1966) also noted the possibility
that an infarct of the medial temporal region, particularly on the dominant

Alexia without agraphia

311

side, might cause transient recent memory disturbance despite complete
normalcy of the nondominant side. Victor, Adams and Collins (1971)
mentioned the diencephalon, especially the medial dorsal nuclei and medial
pulvinar as the responsible lesion for amnesic deficit in the WernickeKorsakoft syndrome. It is thus reasonable to assume that recent memory
disturbance in our case is related to the lesion in the hippocampus and
the thalamus. The symptom was transient probably because the lesion was
confined only to the left side.

SUMMARY

The patient is a 58-year-old Japanese teacher of German literature who
suffered twice from cerebrovascular accidents, showing alexia without agraphia.
Pathological examination showed an old infarct in the posterior two-thirds of
the fusiform and almost the whole lingual gyrus, involving the posterior border
of the parahippocampal gyrus in the left hemisphere. The left cuneus and
the calcarine cortex were preserved. There was degeneration of the lower third
of the splenium of the corpus callosum, extending to its occipital radiation
and tapetum on both sides.
Comparing clinico-pathological findings of the 31 known autopsy cases,
it was proposed that the lesion of the left spleno-lingual system produces alexia
without agraphia but it may ameliorate. In addition, when spleno-cuneate system
is also involved alexia becomes persistent and it may accompany object agnosia
or optic aphasia.

Acknowledgements. We are grateful to Professor M.G. Netsky, M.D.,
and Associate Professor J. Dower, Ph.D., for reading the manuscript and
help with English, to Mr. M. Saeki and T. Honda for technical assistance
and to Mr. T. Ikeda for the photography.
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Masayoshi Kurachi, M.D., and Nariyoshi Yamaguchi, M.D., Department of Neuropsychiatry, Kanazawa University
School of Medicine, 13·1 Takara·machi, Kanazawa 920, Japan.
Tohru Inasaka, M.D., Department of Internal Medicine (I), Kanazawa University School of Medicine, 13·1
Takara·machi, Kanazawa 920, Japan.
Hosaku Torii, M.D., Department of Neuropsychiatry, Kanazawa Medical University, Uchinada 920-02, Japan.