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The anatomic basis of pure alexia.

The behavioral and anatomic correlates of pure alexia were analyzed in 16 patients. Right homonymous hemianopia failed to appear in three patients, who had right achromatopsia instead. Color anomia and unilateral optic ataxia were seen in six patients. Memory defects were found in two patients. Visual agnosia was noted in two. No patient had visual disorientation. The crucial anatomic correlate of alexia was a lesion of the paraventricular white matter of the left occipital lobe, capable of compromising both interhemispheric and intrahemispheric visual pathways. The lesion associated with color anomia was in the mesial occipitotemporal junction of the left hemisphere.

Adolescent↗

Left hemisphere pathways in reading: inferences from pure alexia without hemianopia.

In pure alexia, reading is impaired despite almost normal speech, spelling, and writing. We studied a right-handed man with pure alexia, but no hemianopia. He had more difficulty reading longer words (word-length effect), but had no selective reading impairment in phonologic or semantic analysis. Clinical-CT correlation suggests that (1) left hemisphere visual pathways crucial for reading arise from or pass close to the left occipitotemporal or inferior temporal gyrus, and (2) relevant transcallosal fibers from the right hemisphere course inferior to the posterior horn of the left lateral ventricle before ascending to left hemisphere language areas.

Aged↗

Resolving metabolic abnormalities in a case of pure alexia.

We report resolving metabolic abnormalities corresponding to clinical improvement in a patient with pure alexia secondary to acute cerebral infarction. Local cerebral glucose metabolism (lCMRgl) was measured with positron emission tomography (PET) using 18F-fluorodeoxyglucose (18F-FDG) close to ictus and 4 1/2 months later. Serial CTs and a subsequent MRI demonstrated small, unchanging left-hemispheric lesions involving the area of the lateral geniculate body and the splenium of the corpus callosum. PET demonstrated the evolution of the metabolic abnormality resulting from intrahemispheric (lateral geniculate) and interhemispheric (splenium) disconnection in the absence of occipital lobe infarction. This case illustrates that cerebral disconnection can result in the syndrome of pure alexia. The factors accounting for focal hypometabolism in the absence of cerebral infarction are discussed.

Brain↗

Alexia without agraphia associated with spleniogeniculate infarction.

Pure alexia, following an infarction in the distribution of the left posterior cerebral artery, is attributed to damage of the left occipital lobe and the splenium of the corpus callosum. We describe a case of pure alexia in a 57-year-old woman with infarction of the left lateral geniculate body and the splenium of the corpus callosum, a variation on this classic disconnection syndrome.

Cerebral Infarction↗

Hanja alexia with agraphia after left posterior inferior temporal lobe infarction: a case study.

Korean written language is composed of ideogram (Hanja) and phonogram (Hangul), as Japanese consists of Kanji (ideogram) and Kana (phonogram). Dissociation between ideogram and phonogram impairment after brain injury has been reported in Japanese, but few in Korean. We report a 64-yr-old right-handed man who showed alexia with agraphia in Hanja but preserved Hangul reading and writing after a left posterior inferior temporal lobe infarction. Interestingly, the patient was an expert in Hanja; he had been a Hanja calligrapher over 40 yr. However, when presented with 65 basic Chinese letters that are taught in elementary school, his responses were slow both in reading (6.3 sec/letter) and writing (8.8 sec/letter). The rate of correct response was 81.5% (53 out of 65 letters) both in reading and writing. The patient's performances were beyond mean-2SD of those of six age-, sex-, and education-matched controls who correctly read 64.7 out of 65 and wrote 62.5 out of 65 letters with a much shorter reaction time (1.3 sec/letter for reading and 4.0 sec/letter for writing). These findings support the notion that ideogram and phonogram can be mediated in different brain regions and Hanja alexia with agraphia in Korean patients can be associated with a left posterior inferior temporal lesion.

Cerebral Infarction↗

[Alexia without agraphia due to the lesion in the right occipital lobe in a right-handed man. Detection of hemispheric lateralization of handedness and language in a right-handed patient].

INTRODUCTION AND CLINICAL CASE: A 65 year-old man, right-handed, without any family history of left handiness, suddenly developed a left homonymous hemianopia and incapacity for reading. Neurological and neuropsychological examinations showed the presence of a profound alexia with preservation of writing to dictation and spontaneously. He was unable to read what he had written. He could spell the words letter by letter but he was unable to read the complete word. MRI showed an extensive infarct in the territory of the right posterior cerebral artery. The infarct extended anteriously to the right thalamus and to the medial temporal fifth or fusiform gyrus. The splenius was spared. Brain SPECT disclosed the area of the infarct and an extensive area of decreased cerebral perfusion over the right parietal and temporal areas. CONCLUSION: Alexia without agraphia has been reported in right-handed patients with left occipital lesions and in right occipital regions in left-handed patients but rarely if ever in right occipital lesions in right-handed patients.

Aged↗

[Amyotrophic lateral sclerosis presented with alexia of kanji and word meaning aphasia].

We report a 63-year-old right-handed Japanese man with progressive bulbar dysfunction and alexia of kanji (Japanese morphograms). He was well until his 62 years of age, when he noted difficulty of reading kanji, which was followed by disturbances in his speech. Reading of kana (Japanese phonograms) was preserved. He also showed naming difficulties with semantic memory loss for words, which were characterized for word meaning aphasia or semantic dementia. He showed dysarthria and mild dysphagia with atrophy and fasciculations of the tongue. The electromyographic studies disclosed diffuse neurogenic pattern. He was diagnosed as having bulbar type amyotrophic lateral sclerosis. Cranial magnetic resonance imaging and single-photon emission computed tomography revealed bilateral involvements of the temporal lobes. Our patient appeared to meet the clinical criteria for frontotemporal degeneration of motor neuron disease type, and is the first case of amyotrophic lateral sclerosis showing alexia of kanji and word meaning aphasia.

Amyotrophic Lateral Sclerosis↗

[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.

Aged↗

[Preserved ability to read aloud kanji idioms in left handed alexia].

We report a 69-year-old left-handed man, who developed alexia after a right medial occipito-temporal lobe infarction. On admission to the rehabilitation department two months after the onset, neurological examination showed left hemianopia, left hemiparesis, decreased deep sensation on the left side, and alexia. A brain MRI demonstrated infarcts in the right medial occipito-temporal lobe and the splenium of the corpus callosum. Detailed neuropsychological examination was performed two months after the onset. The patient was alert and cooperative. His speech was fluent with some word-finding difficulty. Comprehension for spoken materials, repetition, and naming abilities were all preserved. Systematic examination for reading revealed that reading aloud was disturbed in both kanji and kana words. Reading comprehension was significantly better for kanji words than kana words. First, we examined the effects of number of characters in a word. The number of characters in a word didn't affect his reading performance. Second, his performance on reading aloud of usual kanji words was compared with that of kanji words representing idioms. A kanji idiom is different from usual kanji words, in which pronunciation of each character is selected from several options. Reading aloud kanji idioms was significantly better than usual kanji words. In addition, reaction time to complete reading a word was much shorter for kanji idioms than usual kanji. An analysis of qualitative features of errors revealed that most errors in kanji idiom reading were semantically similar to the correct answers, while many errors in usual kanji word reading were classified into "don't know" responses. These findings suggested that a kanji idiom was tightly connected to its pronunciation, which resulted in his much better performance for kanji idiom reading. Overlearning of a unique relationship between a kanji idiom and its pronunciation might modify neuronal organization for reading.

Aged↗

[The alexias].

The authors propose a review about Alexias according to the latest theories. Anatomoclinical and linguistic classification of alexia are reported. Some hypotheses about reading processes, according to linguistic classification, are discussed.

Agraphia↗

[Alexia without agraphia. Anatomical basis and physiopathological mechanisms].

Clinical, neuropsychological and radiological signs were studied in a patient suffering from pure alexia associated with right superior quadrantanopia. The lesion responsible for the defects was located in the periventricular white substance at the level of the left inferior occipitotemporal convolutions. These structures seem to constitute the pathway of the visual information channelled from the two hemispheres towards the language centres. The lesion therefore disconnects the angular gyrus from its visual information and gives rise to alexia without agraphia.

Aged↗

Alexia without agraphia and associated disorders: importance of recognition in the rehabilitation setting.

Alexia without agraphia is readily recognized in its pure (without other neurologic findings) but rare form. However, this deficit is more common when associated with other behavioral disturbances that result from trauma or cerebral infarction to the posterior cerebral hemispheres. Two cases presented with alexia without agraphia following infarction within the posterior cerebral artery territory. Because of initial confusion and disorientation the patients were diagnosed as demented and unsuitable for rehabilitation. Both subjects had a visual field deficit and color agnosia. Prosopagnosia, simultanagnosia, and ataxia of visual-motor control were also present. The second case was unusual because of pure word blindness associated with a right occipital lobe lesion. Only a few such cases have been reported in the literature worldwide. This paper demonstrates that careful delineation of cognitive deficits permits greater understanding of functional disorders with improved rehabilitation outcome.

Aged↗

[Remarks on the "pure alexia" syndrome--a case report].

The article describes a case with the "pure alexia" syndrome caused by an infarct of the arteria cerebri posterior sinistra during a diffuse cerebral vascular disease. The authors consider the alexia to be a consequence of difficulties in transcoding graphic into graphemic structures.

Aged↗

Alexia without either agraphia or hemianopia in temporal lobe lesion due to herpes simplex encephalitis.

We report a case of alexia without either agraphia or hemianopia following herpes simplex encephalitis. The patient had a temporal lobe lesion with involvement of the occipitotemporal gyrus. This is an unusual cause of alexia without agraphia. The location of the lesion supports the view that transcallosal fibers from the right hemisphere to the left angular gyrus course inferior to the posterior horn of the left lateral ventricle and pass close to the left occipitotemporal gyrus.

Acyclovir↗

Evolution of a form of pure alexia without agraphia in a child sustaining occipital lobe infarction at 2 1/2 years.

The progress of cognitive visual dysfunction over an 8-year period of a child who sustained bilateral occipital-lobe infarctions at the age of 2 1/2 years is described. She survived with normal intelligence and went on to attend mainstream school. She manifested many features of cognitive visual impairment and, in particular, developed a form of pure alexia without agraphia. She achieved some letter-by-letter reading but no sight vocabulary development, including to her own name. She learned to write imaginatively employing phonetically true spelling but cannot read what she has written. Her progress and the difficulties encountered during the management of her condition are discussed in this first case report of the evolution of pure alexia without agraphia in childhood. The features of this syndrome in the developing child who has never developed the capacity to read are contrasted with that seen in affected adults.

Agraphia↗

[Pure alexia: presentation of a case].

INTRODUCTION AND CLINICAL CASE: A case of pure alexia, secondary to an occipital cerebral infarct, is presented, which initially appearing with bilateral amaurosis of two hours of duration, following by a significant difficulty to read, and according to patient, was a deficit to near vision and by criteria of ophthalmologists and optometrists was a presbyopia. Authors identified a lack of P-300 wave in study of endogenous component of events related potentials (ERP), to task of phonetic discrimination, and its normal registry to task of graphic discrimination. CONCLUSION: Therefore, we confirm the cliniconeurophysiologic diagnosis of a pure alexia without agraphia, by means of this non-invasive method studying cerebral cognitive process.

Cerebral Infarction↗

Patterns of peripheral paralexia: pure alexia and the forgotten visual dyslexia?

The concept of visual dyslexia put forward by Marshall and Newcombe (1973) is assessed. After a long period of neglect it was resurrected in the late 1990s in a narrow form. In the current paper it is proposed that a wider form of the functional syndrome is useful to include amongst other conditions attentional dyslexia and neglect dyslexia. The variety of sub-forms would correspond to the behavioural effects of the different ways in which the orthographic processing systems can be impaired. What distinguishes the broader form from pure alexia is that the patient lacks the capacity to use a serial letter processing strategy, and so interpretation of visual dyslexia in terms of the impairment to the orthographic processing systems is not contaminated by the use of a compensatory strategy that results in processing operations which are qualitatively very different from the normal and highly opaque. The lack of a serial letter processing strategy makes visual dyslexia a much more transparent functional syndrome.

Alexia, Pure↗

Visual command hallucinations in a patient with pure alexia.

Around 25% of patients with visual hallucinations secondary to eye disease report hallucinations of text. The hallucinated text conveys little if any meaning, typically consisting of individual letters, words, or nonsense letter strings (orthographic hallucinations). A patient is described with textual visual hallucinations of a very different linguistic content following bilateral occipito-temporal infarcts. The hallucinations consisted of grammatically correct, meaningful written sentences or phrases, often in the second person and with a threatening and command-like nature (syntacto-semantic visual hallucinations). A detailed phenomenological interview and visual psychophysical testing were undertaken. The patient showed a classical ventral occipito-temporal syndrome with achromatopsia, prosopagnosia, and associative visual agnosia. Of particular significance was the presence of pure alexia. Illusions of colour induced by monochromatic gratings and a novel motion-direction illusion were also observed, both consistent with the residual capacities of the patient's spared visual cortex. The content of orthographic visual hallucinations matches the known specialisations of an area in the left posterior fusiform gyrus--the visual word form area (VWFA)--suggesting the two are related. The VWFA is unlikely to be responsible for the syntacto-semantic hallucinations described here as the patient had a pure alexic syndrome, a known consequence of VWFA lesions. Syntacto-semantic visual hallucinations may represent a separate category of textual hallucinations related to the cortical network implicated in the auditory hallucinations of schizophrenia.

Aged↗