Neurological Sciences (2022) 43:709–711
https://doi.org/10.1007/s10072-021-05678-1
LETTER TO THE EDITOR
Alexia without agraphia: a disconnection syndrome or a primary
involvement of a visual language center?
Halil Onder1   · Selcuk Comoglu1
Received: 15 August 2021 / Accepted: 15 October 2021 / Published online: 31 October 2021
© Fondazione Società Italiana di Neurologia 2021
Letter to Editor,
A 55-year-old male patient with a history of hypertension
(using aldactazide 35 mg daily) applied to emergency ser-
vice due to complaints of disturbance in reading compre-
hension which had started abruptly the previous day. On
neurological exam, the patient was orientated and coopera-
tive. The cranial nerve exams were normal, except for right
homonymous hemianopsia. The motor, sensory, and cerebel-
lar examinations were also within normal limits. Besides,
the detailed aphasia investigations including speech fluency,
auditory comprehension, repeating, naming, writing, and
automatic speech were evaluated as normal which excluded
aphasia. On the other hand, the patient could decipher words
letter-by-letter. He had difficulty reading fluently and pro-
nounce the words. There was severe impairment in read-
ing aloud and reading comprehension. However, the patient
could easily write the verbal statements and he could also
easily write spontaneously. The remarkable point was that
the patient could read the statements he had written more
comfortably; however, he rather suffered difficulty in reading
the statements instructed to write and particularly those he
had not written. The cranial CT and MRI showed oedema-
tous lobar hemorrhage in the left middle and inferior tem-
poral gyrus, the left lateral occipitotemporal gyrus (fusiform
gyrus) (Fig. 1). Antihypertensive therapy and antiedema
therapy were administered for four days. The follow-up
neurological exam showed persistence of alexia and right
homonymous hemianopsia. The patient was discharged with
adding amlodipine therapy for resistant hypertension. The
neurological exam three weeks later also showed persistence
of alexia and homonymous hemianopsia.
* Halil Onder
halilnder@yahoo.com
1
Neurology Clinic, Diskapi Yildirim Beyazit Training
and Research Hospital, Şehit Ömer Halisdemir Street. No:
20 Altındag, Ankara 06110, Turkey
Discussion
Alexia without agraphia is a clinical disconnection syn-
drome that was first described by Dejerine in 1892 followed
by Geschwind in 1965. The clinic is characterized by diffi-
culties in rapid word recognition and fluent reading abilities.
Besides, the visual field defects like right-sided homony-
mous hemianopia usually accompany the clinic. This syn-
drome is exceptionally rare as a clinical manifestation in
stroke patients [1] and most cases reported are those caused
by left posterior cerebral artery (PCA) occlusion [1, 2]. The
splenium is also affected by the lesion that leads to disrup-
tion of the connection between the intact right visual cortex
and the left angular gyrus, the cortical center for reading.
Taken together, the individual suffers from reading prob-
lems albeit intact right occipital lobe, that is why the authors
rather describe this clinic as a disconnection syndrome [3].
The preserved writing ability is attributed to the intact left
angular gyrus [3]. On the other hand, in our patient, the
splenium was not affected by the hemorrhage suggesting a
distinct mechanism. However, the detailed phenomenologi-
cal investigations showed pure alexia. He suffered particu-
larly difficulty in reading the statements he had not written;
however, he was more comfortable in reading the sentences
he had written spontaneously in comparison to those he was
instructed to write. This considered that he displayed better
performance in reading the statements he had written using
his short-term memory, to compensate for the disturbed read-
ing skills. In our patient, the affected site involved the left
middle and inferior temporal gyrus, the left fusiform gyrus.
The middle temporal gyrus is on the temporal lobe, located
between the superior temporal gyrus and inferior temporal
gyrus. Besides its function in audio-visual emotional rec-
ognition, this gyrus is also associated with accessing word
meaning while reading [4]. Some studies also indicate that
lesions of the posterior region of the left middle temporal
gyrus may result in alexia and agraphia. The hemorrhage in
our patient also involved the left fusiform gyrus and inferior
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Fig. 1  The brain CT (A,B,C)
and MRI images (D, E, F)
showing the oedematous lobar
hemorrhage in the left middle
and inferior temporal gyrus
(jagged arrows), the left fusi-
form gyrus (jagged arrows)
temporal gyrus where is highly associated with impaired
reading acquisition ability [5]. Of note, the basal tempo-
ral language areas [3] were not affected. One explanation
may be that although the splenium was not affected by the
hemorrhage, the splenial function might be disturbed by the
increased ICP effect of the hemorrhage that is rather com-
mon in comparison to the ischemic brain lesions. Accord-
ing to this hypothesis, the pressure effect of the hemorrhage
would be disappeared in the subacute period. However, the
follow-up exam of the patient three weeks later showed the
persistence of alexia which conflicts with the hypothesis. On
the other hand, the pure alexia in association with the lesion
in our patient may be explained by a mechanism of direct
participation of basal temporal and occipital cortices in the
cognitive process of reading which is also shown in recent
functional imaging studies [3, 6, 7]. In light of these data,
Mani et al. reported three patients with a syndrome of pure
alexia who were induced by high-frequency electrical corti-
cal stimulation of the left mid fusiform and inferior temporal
gyrus [3]. In these patients, spontaneous speech, auditory
comprehension, writing, calculation, right-left orientation,
praxis, and gnosis were also preserved as in our patient. The
high-frequency electrical stimulation of the cortex, used in
this study, deactivates discrete areas of the cortex and per-
mits elucidation of cognitive function of discrete areas of
cortex [8]. Supporting these results, other fMRI and ERP
studies also remark on the function of the inferior tempo-
ral and occipitotemporal lobe in visual language processing
[6, 7]. Such that, based on the evidence of its function in
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Neurological Sciences (2022) 43:709–711
processing the letter strings, the left inferior temporal gyrus
and the lateral occipitotemporal gyrus have been emphasized
as the anatomical regions to represent visual word form area
(VWFA) [6]. In addition, some clinicopathologic studies
also support the existence of a gray matter relay center in
the pathways connecting the visual association areas and the
dominant angular gyrus [9]. As discussed by Mani et al. [3]
the basal temporal language areas, known to function in also
understanding simple spoken commands, repeating words
orally, or following written commands, were not affected by
the hemorrhage in our index case. Up to our knowledge, in
a unique clinical report, two cases of pure alexia developing
due to the left inferior temporal and occipitotemporal gyri
hematoma were also illustrated in detail [10]. In this report,
the authors explained the underlying mechanism of alexia
with direct damage to the VWFA that was shown to exist
in the functional MRI studies in the lateral occipitotempo-
ral gyrus [6]. The illustration of our patient, supporting the
above-mentioned experimental study results [3, 6, 7] and the
discussions in the clinical report by Rodríguez-López et al.,
is critical in this sense. We also think that our case may be
classified under the head of the ‘cortical alexia type’ which
has been hypothesized to develop due to direct damage to
this VWFA. [6, 10].
In conclusion, our case constitutes a crucial sample for
alexia without agraphia at whom the lesion involved the fusi-
form gyrus and the inferior temporal gyrus; however, the
splenial corpus callosum was sparred. Besides, the clinic
was associated with hemorrhage which was strictly rare.
Neurological Sciences (2022) 43:709–711
The presentation of acute onset reading problems should
consider the syndrome of ‘Alexia without agraphia’ and the
clinical suspicion should prompt early cranial screening for
timely diagnosis and appropriate management. The illustra-
tion of these rare cases may also contribute substantially to
our classical understanding of the pathways processing in
the reading network.
Abbreviations  fMRI: Functional MRI; ERP: Event-related potentials;
ICP: Intracranial pressure
Author contribution  Concept—H.O.; design—H.O.; supervision—
S.C.; materials—H.O, S.C.; data collection and/or processing—H.O;
analysis and/or interpretation—HO., S.C.; literature search—H.O;
writing manuscript—H.O., critical review—S.C.
Declarations  trical stimulation of the human cerebral cortex. Electroencepha-
Conflict of interest  The authors declare no competing interests.
Ethical approval and Informed consent  Informed consent has been
obtained from the patient.
References
1. Sharma B, Handa R, Prakash S, Nagpal K, Bhana I, Gupta PK,
Kumar S, Sisodiya MS (2014) Posterior cerebral artery stroke pre-
senting as alexia without agraphia. Am J Emerg Med 32(12):1553
e3 4
711
2. Quint DJ, Gilmore JL (1992) Alexia without agraphia. Neurora-
diology 34(3):210–214
3. Mani J, Diehl B, Piao Z, Schuele SS, Lapresto E, Liu P, Nair
DR, Dinner DS, Luders HO (2008) Evidence for a basal temporal
visual language center: cortical stimulation producing pure alexia.
Neurology 71(20):1621–1627
4. Acheson DJ, Hagoort P (2013) Stimulating the brain’s language
network: syntactic ambiguity resolution after TMS to the infe-
rior frontal gyrus and middle temporal gyrus. J Cogn Neurosci
25(10):1664–1677
5. Kronbichler L, Kronbichler M (2018) The importance of the left
occipitotemporal cortex in developmental dyslexia. Curr Dev Dis-
ord Rep 5(1):1–8
6. Cohen L, Lehericy S, Chochon F, Lemer C, Rivaud S, Dehaene
S (2002) Language-specific tuning of visual cortex? Func-
tional properties of the Visual Word Form Area. Brain 125(Pt
5):1054–1069
7. Nobre AC, Allison T, McCarthy G (1994) Word recognition in
the human inferior temporal lobe. Nature 372(6503):260–263
8. Nathan SS, Sinha SR, Gordon B, Lesser RP, Thakor NV (1993)
Determination of current density distributions generated by elec-
logr Clin Neurophysiol 86(3):183–192
9. Beversdorf DQ, Ratcliffe NR, Rhodes CH, Reeves AG (1997)
Pure alexia: clinical-pathologic evidence for a lateralized visual
language association cortex. Clin Neuropathol 16(6):328–331
10. Salio CRLMPGMAM (2018) Pure alexia: two cases and a new
neuroanatomical classification. J Neurol 265:436–438
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