Alexia caused by a fusiform or posterior inferior temporal lesion

We evaluated the alexia and agraphia of three patients with different lesions using Japanese kanji (morphograms) and kana (phonograms) and made a lesion-to-symptom analysis. Patient 1 (pure alexia for both kanji and kana and minor agraphia for kanji after a fusiform lesion) made more paragraphic errors for kanji, whereas patient 2 (alexia with agraphia for kanji after a posterior inferior temporal lesion) showed severe reading and writing disturbances and more agraphic errors for kanji. Brodmann Area 37 was affected in both patients, but in patient 2 the lesion was located lateral to that in patient 1. Patient 3 showed agraphia without alexia after restricted lesion to the angular gyrus. We believe that pure alexia (patient 1) results from a disconnection between the medial fusiform gyrus and posterior inferior temporal area (the lateral fusiform and inferior temporal gyri), whereas alexia with agraphia for kanji (patient 2), corresponding to lexical agraphia in Western countries, results from damage to the posterior inferior temporal area, in which whole-word images of words are thought to be stored. Furthermore, restricted lesion in the angular gyrus (patient 3) does not produce alexia; the alexic symptom of "angular" alexia with agraphia may be the result of damage to the adjacent lateral occipital gyri.     

A case of alexia with agraphia caused by the re-infarct in left lateral occipital gyrus]

The angular gyrus has been proposed as the key area of reading and writing function. In recent PET (positron emission topography) activation studies, role of angular gyrus in the reading and writing has been reestimated. Whether the angular gyrus is necessary for reading and writing or not is now under discussion and should be clinically revised. We experienced a case that presented classical alexia with agraphia of kana (Japanese syllabogram) caused by the re-infarct in left lateral occipital gyrus. This case showed the alexia with agraphia more apparent in Japanese kana than in kanji characters. Interestingly, no higher cortical dysfunction was revealed at the first cerebral infarction in left angular gyrus which was assumed as the key area for alexia with agraphia. This case supported the opinion which pointed out the importance of left occipital gyrus on Japanese kana reading.     

Dorsal type letter-by-letter reading accompanying alexia with agraphia due to a lesion of the lateral occipital gyri

ABSTRACT

We report a patient with alexia with agraphia accompanied by letter-by-letter reading after hemorrhage in the left middle and inferior occipital gyri that spared the angular gyrus and the fusiform gyrus. Kanji (Japanese morphograms) and kana (Japanese phonetic writing) reading and writing tests revealed that alexia with agraphia was characterized by kana-predominant alexia and kanji-predominant agraphia. This type of “dorsal” letter-by-letter reading is discernable from conventional ventral type letter-by-letter reading that is observed in pure alexia in that (1) kinesthetic reading is less effective, (2) kana or literal agraphia coexists, and (3) fundamental visual discrimination is nearly normal.     

Fusiform type alexia: pure alexia for words in contrast to posterior occipital type pure alexia for letters

OBJECTIVE: To clarify the behavioral differences between patients with pure alexia from different lesions. METHODS: Two patients with pure alexia caused by damage to the fusiform or posterior occipital gyri were given reading and writing tests including kanji (Japanese morphograms) and kana (Japanese phonetic writing). RESULTS: Patient 1 (pure alexia from a fusiform gyrus lesion) had difficulty reading both kanji and kana, with kanji reading more impaired, and imageability and visual complexity effects (imageable or less complex words/characters were read better than nonimageable or more complex words/characters), whereas patient 2 (pure alexia from a posterior occipital gyri lesion) showed selective impairment of kana reading. CONCLUSION: Pure alexia for kanji (and kana; fusiform type) is characterized by impairments of both whole-word reading, as represented in kanji reading, and letter identification, and is different from pure alexia for kana (posterior occipital type) in which letter identification is primarily impaired. Thus, fusiform type pure alexia should be designated pure alexia for words, whereas posterior occipital type pure alexia should be designated pure alexia for letters.     

Neural mechanism of reading]

We conducted positron emission tomography studies on reading and found that two distinct areas were activated, i.e. the left fusiform/inferior temporal gyri (posterior inferior temporal cortex, Area 37) by kanji words and the fusiform/inferior occipital gyri (posterior occipital gyri, Area 18/19) by kana words. Clinically, alexia and agraphia for kanji is caused by a posterior inferior temporal cortex lesion. Moreover, pure alexia more impaired for kanji results from a fusiform gyrus lesion, whereas pure alexia for kana occurs because of damage to the posterior occipital gyri. These experimental and clinical findings suggest that impaired letter identification in Area 18/ 19 causes pure alexia for kana, disrupted visual images of words in Area 37 results in alexia with agraphia for kanji, and impaired access to the visual image storage (Area 37) yields pure alexia dominantly disturbed for kanji.     

Pure alexia in Japanese and agraphia without alexia in kanji. The ability dissociation between reading and writing in kanji vs kana

A 60-year-old right-handed Japanese man with infarction of the left occipital lobe and inferior temporal gyrus initially showed pure alexia in kana and kanji. Later, though pure alexia in kana persisted, his kanji reading improved markedly, but with little improvement of kanji writing. We speculate that different pathways are involved in kanji reading and writing. Wernicke's area and its surrounding left middle temporal lobe might play the most important role for kanji reading when visual information is transmitted by any pathway. The pathway from Wernicke's area to the left occipital lobe via the middle and inferior temporal pathway may be indispensable for kanji writing. We postulate "agraphia without alexia in kanji" due to left inferior temporal subcortical damage.     

Pure alexia for kana. Characterization of alexia with lesions of the inferior occipital cortex

OBJECTIVE: To characterize reading impairments caused by lesions in the posterior occipital cortices. METHODS: We gave six patients with these lesions reading and writing tests and located a critical site for alexia using MRI and SPECT. RESULTS: The patients read three-character kana (Japanese syllabograms) nonwords, and five-character kana nonwords significantly or at a near significant level more poorly and slowly than normal subjects, whereas they read kanji (Japanese morphograms) almost correctly but more slowly. Letter-by-letter reading with a single-kana character identification impairment (in five patients), a word-length effect, kinesthetic facilitation, a lexicality effect, and minor to mild agraphia for kanji (in three patients) were observed. These deficits were characteristic of pure alexia. Alexia disappeared within a few months except in one patient who had extensive hypoperfusion in the left occipital lobe. A shared lesion was located in the left posterior fusiform/inferior occipital gyri (Area 18/19) on MRI, and there was blood flow reduction around this area on SPECT. This area coincided with the activation site for kana word covert reading in our previous study. CONCLUSIONS: These results suggest that pure alexia particularly for kana, or more generally pure alexia for letters, is caused by a lesion in the posterior inferior occipital cortex, characterized primarily by impaired kana character or letter identification, with relatively preserved kanji or word recognition.     

Temporal lobe epilepsy manifesting as alexia with agraphia for kanji

Alexia with agraphia results from lesions of the left angular gyrus or the left posteroinferior temporal lobe. In Japanese or Korean persons, lesions of the latter cause alexia with agraphia for ideograms. We describe a case of alexia with agraphia for kanji (Japanese ideograms) caused by temporal lobe epilepsy. After generalized convulsions, a 32-year-old man noticed that he had difficulty in reading and writing kanji, although he could read and write kana (Japanese syllabograms). His EEG showed frequent sharp waves on the left occipitotemporal region. MRI of the brain revealed a hyperintense lesion on the left hippocampus. Single-photon-emission computed tomography revealed marked hyperperfusion at the left hippocampus and the left posteroinferior temporal cortex. Antiepileptic drugs improved his alexia with agraphia for kanji. This is the first report describing alexia with agraphia for kanji caused by temporal lobe epilepsy.     

Pure alexia caused by separate lesions of the splenium and optic radiation

Pure alexia is severe difficulty in reading and understanding written language but with normal oral language and writing abilities. We report a patient with pure alexia caused by two different infarct lesions in the left lateral thalamus and the left splenium of the corpus callosum. A 56-year-old right-handed man was admitted to hospital with right homonymous hemianopia associated with pure alexia. He could write kana characters but not kanji. His cranial magnetic resonance imaging revealed two different infarct lesions in the left optic radiation and the left splenium of the corpus callosum. Magnetic resonance angiography showed mild stenosis at the origin of the right vertebral artery and stenosis of the left distal posterior cerebral artery. The mechanism of developing pure alexia can be simply explained by disconnection. We assumed that agraphia of kanji was caused by the effect of ischemia and edema following transient obstruction in branches from the distal posterior cerebral artery.     

Alexia with agraphia of kanji (Japanese morphograms).

The case of the right-handed young Japanese woman with alexia with agraphia of kanji (the Japanese morphograms) due to a small circumscribed haematoma in the left posterior inferior temporal gyrus is described. Her chief complaint was the inability to read and write kanji. Detailed examination showed that her alexia with agraphia was much more predominant for kanji than kana (the Japanese syllabograms). These facts suggest that the processing of kanji and kana involves different intrahemispheric mechanisms.     

Kanji-predominant alexia with agraphia in opticospinal multiple sclerosis]

Alexia with agraphia is very rare symptom in multiple sclerosis. We present a patient of opticospinal multiple sclerosis with kanji-predominant alexia with agraphia. A 55-year-old, right-handed man was admitted to our hospital because of difficulty in reading and writing in August 2001. The patient had been diagnosed as having relapsing-remitting opticospinal multiple sclerosis eight years prior to admission. Language examination showed alexia with agraphia predominantly affecting kanji and also mild naming difficulties, but a good comprehension and a normal repetition. T2-weighted MRI demonstrated hyperintensity area in the left temporo-parietal lobe, involving the white matter beneath the postero-inferior temporal lobe and inferior parietal lobule. On brain SPECT, low blood perfusion was observed in the left temporo-parietal regions. Although agraphia for kana and alexia for both kana and kanji improved after steroid therapy, agraphia for kanji did not improve. After the treatment, high intensity area of inferior parietal lobule was disappeared on MRI, and the hypoperfusion of inferior parietal lobule on brain SPECT was also improved, but the lesion of left postero-inferior temporal lobe did not show any remarkable changes. We considered that the kanji-predominant alexia with agraphia was due to the lesions of left inferior parietal lobule and postero-inferior temporal lobe, and agraphia for kanji was due to the lesion of left postero-inferior temporal lobe.     

Pure alexia from a posterior occipital lesion

The authors report a patient with pure alexia (letter-by-letter reading) selectively impaired for kana (Japanese phonograms), cerebral achromatopsia, and right lower quadrantanopsia after hemorrhage in the left posterior occipital lobe, mainly under the lateral occipital gyri. The patient also could not recognize some single-character kana, nor could he discriminate between two shapes of a similar size. The authors believe that the posterior occipital lobe, including the lateral occipital gyri, is specialized to recognize kana characters in this patient.     

Progressive aphasia and surface Alexia in Japanese

Abstract

We report the reading performance of a patient, NK, with selective left-temporal atrophy and progressive aphasia. NK showed surface alexlc reading with the following pattern: flawless oral reading of kana words; a deficit in reading of two-character kanji words that was particularly severe for lower frequency words with an unpredictable correspondence between the component characters and their pronunciations; a predominance of kanji-word reading errors In which characters were assigned pronunciations appropriate to other words containing these characters. These features were predicted and are interpreted on the basis of (a) recent studies of patients with progressive aphasia in English, (b) recent analyses of surface alexia in terms of an interaction between word frequency and neighbourhood-based consistency of spelling-sound correspondences, and (c) characteristics of the Japanese kana and kanji writing systems.     

Alexia with agraphia produced by localized infarction in the inferior posterior region of the left temporal lobe

There have been several reports on alexia with agraphia due to hemorrhage or trauma in the inferior posterior region of the left temporal lobe since Yamadori (1982) first reported a case of cerebral hemorrhage. We presented the first case of alexia with agraphia due to a circumscribed infarct in the inferior posterior region of the left temporal lobe. A 65-year-old right-handed man had an acute onset of inability to read Japanese "kana" letters and to write any letters. He had no difficulty in speaking or understanding. Neurological examination on admission was normal except for homonymous quadrantanopia in the right upper field. Neuropsychological findings: Spontaneous speech was fluent without dysprosody. There were no disturbances in auditory understanding or in repetition. He could read Japanese "kanji" characters correctly, however, he could not read any "kana" letters. Writing was also disturbed severely even as to his address, name or telephone number. Calculation was also difficult. There were no disturbances in naming colors, no ideomotor or constructional apraxia nor visual agnosia. X-ray computed tomography (CT) scan showed a faint low density area with ring enhancement suggesting a cerebral infarct in the inferior posterior region of the left temporal lobe. Patient's hospital course was characterized by a marked improvement of alexia, especially of "kana" letters as compared with that of "kanji" characters. Dissociative improvement of alexia compared with agraphia in this case could be explained by the fact that the lesion was in close contact with the occipital lobe and that he also had pure alexia in the early stage.     

Neural mechanism of reading and writing in the Japanese language

Three Japanese patients presenting with pure alexia showed agraphia for Kanji in addition. A left angular gyrus lesion caused agraphia for both Kanji and Kana, but Kanji reading was preserved. A left posterior inferior temporal (PIT) lesion resulted in alexia and agraphia for Kanji, while the Kana function was preserved. These results imply that the semantic processing of reading Kanji words depends upon the left PIT area, while the phonological reading of Kana is mediated by the left angular gyrus. The PIT area also plays an important role in writing Kanji words.     

Naming difficulties seen in a case of alexia with agraphia caused by a left postero-inferior temporal lesion]

A 71-year-old right-handed man presented writing and reading difficulties as well as naming difficulties. Neuropsychological examinations revealed mild fluent type aphasia accompanied by alexia and agraphia predominantly affecting kanji and also severe naming difficulties. Brain MRI showed cerebral subcortical hemorrhage extending from the anterior one-third of the left temporal lobe to the temporo-occipital junction involving the fusiform gyrus. The analysis of the several reported cases with severe alexia with agraphia for kanji including ours revealed a close correlation between the severity of kanji writing disturbances and that of naming difficulties. It was also shown that cases with severe naming difficulties had lesion extending anteriorly to the anterior middle temporal gyrus or medially to the parahippocampal gyrus, suggesting that the disconnection between the parahippocampal gyrus and other cortices including the temporal lobe was essential for production of naming difficulties.     

Isolated thalamic agraphia with impaired grapheme formation and micrographia

Two patients with isolated thalamic agraphia are described. Both showed kanji (Japanese morphograms) agraphia due to impaired character recall, grapheme deformity and micrographia (progressive reduction in character size during writing) after a lesion that involved the ventral lateral and ventroposterolateral nuclei. Single photon emission computed tomography with a ^99mTc-ethylcysteinate dimer revealed hypoperfusion in the left precentral gyrus (Brodmann Area 6) and anterior supramarginal gyrus in both. Six months later, the extent of blood flow reduction decreased in the supramarginal gyrus in both patients and the precentral gyrus in patient 1. By this time, the writing impairment improved to nearly the normal range. Our study suggests that kanji agraphia (corresponding to lexical agraphia in Western countries) with poor grapheme formation and micrographia arises from a lesion in the ventral lateral and ventroposterolateral nuclei in the left thalamus. The accompaniment of poor grapheme formation and micrographia may reflect disruption of the cortico-subcortical motor circuit involving the putamen, thalamus, premotor cortex and sensorimotor cortex. It is also suggested that multiple cortical sites can be a target for secondary dysfunction that yields agraphia in a thalamic lesion, and that the recovery of reduced cortical blood flow does not always proceed in parallel with that of agraphia.     

A case of alexia with agraphia following left occipital lobe

Since Dejerine reported cases of alexia with agraphia in 1891 and of pure alexia in 1892, it is generally said that the former may occur due to the lesion of the left angular gyrus and the latter due to that of the medial inferior area of the left occipital lobe. In this article, we reported a case of alexia with agraphia who had the main lesion in the medial inferior area of the occipital lobe of the left hemisphere. A 62-year-old right-handed male showed alexia with agraphia. CT scan and single photon emission CT revealed the main lesion in the medial occipital area on the left side. Alexia with agraphia of the patient was characterized as follows: with regard to reading, though his recognition of forms as letter was nearly spared, he could neither read letters or words nor differentiate Kana- from Kanji-letters. Paralexic errors included confusion of Kana and Kanji. He manifested no kinesthetic facilitation in reading. Regarding writing, his disturbances were more severe in Kanji-writing, but there were paragraphia and difficulty of letter-form evocation even in Kana-writing. He could not write spontaneously or to dictation. His copying of letters was also disturbed. Since it is said that there is no difference between Kana- and Kanji-disturbance in Japanese pure alexics, an aspect of alexia of the patient may be common to pure alexia.(ABSTRACT TRUNCATED AT 250 WORDS)     

Central visual fields in pure alexia "without hemianopsia"--visual dysfunction in the right hemifield, and alexia for "kana" words in the left]

Following a left occipito-temporal subcortical hematoma, a 57-year-old, right-handed man developed pure alexia that was more prominent in kana words, especially in kana nonwords, than in kanji letters. Although a kinetic perimetry with a Goldmann perimeter showed his visual fields to be full, a static perimetry with a Humphrey visual field analyzer disclosed decreased visual sensitivity in the right visual field in its central 30 degrees. In addition, a tachnistoscopic examination with Landolt rings revealed his visual acuity (the ability of two points discrimination) to have decreased in the right half of his central visual filed in its 3 degrees. In the right central vision, he was unable to recognize the letters, pictures or colors presented by the tachistoscope. Concerning the reading, the more letters in kana words or the higher the number of strokes in kanji letters, the more difficulty he experienced in orally reading both kana and kanji. On the contrary, in the left central vision, kanji reading was not so affected by an increased number of strokes as the kana-word reading which became difficult when the number of the letters increased. CT scan showed subcortical hematoma in the left occipitotemporal region. Magnetic resonance imaging 3 months after onset revealed a localized injury in the region between the left postero-inferior temporal lobe and the infero-lateral occipital lobe, including the fusiform gyrus. None of the splenium, the lingual gyrus or the optic area were affected. The spared structures also included the angular gyrus, the Wernicke area and their subcortical white matter.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anomic alexia of kanji in a patient with anomic aphasia

The ability to read aloud kanji (logogram) words and to comprehend their meaning was systematically examined to clarify the underlying mechanism of kanji alexia in a patient with anomic aphasia. Confrontation naming, reading aloud and reading comprehension tasks were performed using 110 words from 11 semantic categories written in kanji or kana. Performance in oral reading of kanji words was significantly worse than oral reading of the same words transcribed into kana words. In addition, for kanji words reading aloud was much worse than reading comprehension. Oral reading of kanji words had a significant correlation with naming pictures corresponding to the words, but no correlation with comprehension of kanji words. Qualitative analyses demonstrated that errors in oral reading and naming tasks had many features in common. Our results indicated that some common mechanisms underlie both naming and oral reading of kanji words. We propose calling this type of alexia "anomic alexia of kanji", which should be distinguished from kanji alexia with difficulty in both reading aloud and comprehension. Lesions in our patient were located in the middle part of the left middle temporal gyrus and its subcortical area, which could be important for access to the phonological lexicon from semantics.