Alexia with agraphia due to the left posterior inferior temporal lobe lesion--neuropsychological analysis and its pathogenetic mechanisms

We report three cases of alexia with agraphia due to the left posterior inferior temporal lesions. In Case 1, the reading disability was more prominent in the use of Kana than in the use of Kanji, which is similar to previously reported cases of alexia with agraphia due to angular gyrus lesion. In Cases 2 and 3, by contrast, the reading disability was more prominent in the use of Kanji than in the use of Kana. In spontaneous writing and dictation, the disability was more pronounced in the use of Kanji compared with the use of Kana. In each of the three cases, the CT scan and positron emission tomography showed a localized lesion in the lower part of the left posterior temporal lobe. A typical form of an alexia with agraphia could be caused not only by the left angular lesion but also by the left posterior inferior temporal lesion. We discuss the neuropsychological analysis and pathogenetic mechanisms of alexia with agraphia due to the left posterior inferior temporal lesion in the comparison of alexia with agraphia caused by the left angular lesion.     

Varieties of alexia from fusiform, posterior inferior temporal and posterior occipital gyrus lesions

Reading impairments of three alexia patients, two pure alexia and one alexia with agraphia, due to different lesions were examined quantitatively, using Kanji (Japanese morphogram) words, Kana (Japanese phonetic writing) words and Kana nonwords. Kana nonword reading was impaired in all three patients, suggesting that widespread areas in the affected occipital and occipitotemporal cortices were recruited in reading Kana characters (corresponding to European syllables). In addition, the findings in patient 1 (pure alexia for Kanji and Kana from a fusiform and lateral occipital gyri lesion) and patient 2 (pure alexia for Kana from a posterior occipital gyri lesion) suggested that pure alexia could be divided into two types, i.e. ventromedial type in which whole-word reading, together with letter identification, is primarily impaired because of a disconnection of word-form images from early visual analysis, and posterior type in which letter identification is cardinally impaired. Another type of alexia, alexia with agraphia for Kanji from a posterior inferior temporal cortex lesion (patient 3), results from deficient whole-word images of words per se, and thus should be designated "orthographic alexia with agraphia". To account for these impairments, a weighted dual-route hypothesis for reading is suggested.     

Pure agraphia and Gerstmann's syndrome as a visuospatial-language dissociation: an experimental case study

A right-handed man suffered a left parieto-occipital cerebral infarction, causing agraphia with Gerstmann's syndrome but without major aphasia, alexia, or apraxia. Oral spelling was superior to written spelling. Experiments were performed involving (1) analysis of errors in writing, (2) tasks of visual imagery, and (3) identifying letters drawn without leaving a visual trace. The results suggest that the agraphia and Gerstmann's syndrome are due to a dissociation of language skills and visuospatial skills caused by a dominant parieto-occipital lesion.     

Unilateral agraphia after section of the posterior half of the truncus of the corpus callosum

A Japanese patient who had undergone section of the posterior half of the truncus of the corpus callosum in the process of removing an arteriovenous malformation was examined with a battery of special tests. He showed agraphia only with the left hand (unilateral agraphia). The agraphia was more prominent for the words written in Kana characters (phonograms) than for the words written in Kanji characters (ideograms). The patient showed neither unilateral apraxia, nor tactile alexia, nor tactile anomia.     

Ideogram writing in a disconnection syndrome

A case of disconnection-type agraphia coupled with alexia was reported. The patient showed several asymmetrical manual capacities between the two hands, i.e., dissociated difficulty of Kanji (ideogram) writing between the two hands, left unilateral difficulty of Kana (phonogram) writing, right unilateral dyscopia of letters as well as geometrical figures, and right unilateral difficulty in drawing without a model. Anatomically, lesions involved most of the corpus callosum in its posterior portion including the splenium and the left medial occipital lobe. From these data, a possible liguistic capacity of the right hemisphere was suggested.     

Iconic reading in a case of alexia without agraphia caused by a brain tumor: a tachistoscopic study

The lack of nonverbal reading comprehension in the clinical disconnection syndrome, alexia without agraphia, has been contradictory to the relative reading comprehension of the right hemisphere in split brains. We report a 39-year-old patient with verbal alexia without agraphia caused by brain tumor. On rapid tachistoscopic presentation of object names, he denied seeing anything but showed nonverbal reading comprehension by pointing to the corresponding objects. He lost this ability when he recovered ability to name individual letters of the object names. Our results suggest that even partial verbal reading such as the naming of single letters makes demonstration of iconic reading impossible and that total functional disconnection from verbalization, as initially noted in this case or in split brain studies, is necessary to show nonverbal reading comprehension.     

Auditory repetition priming is impaired in pure alexic patients

Alexia without agraphia, or "pure" alexia, is an acquired impairment in reading that leaves writing skills intact. Repetition priming for visually presented words is diminished in pure alexia. However, it is not possible to verify whether this priming deficit is modality-specific or modality independent because reading abilities are compromised. Hence, auditory repetition priming was assessed with lexical decision and word stem completion tasks in pure alexic patients with lesions in left inferior temporal-occipital cortex and the splenium. Perceptually based, modality-specific priming models predict intact auditory priming, since auditory association cortex is spared in the patients. Alternatively, modality-independent models, which suggest that priming reflects the temporary modification of an amodal system, might predict impairments. Baseline performance was matched in the patients and controls, although lexical decision priming measures showed an interaction between group and repetition lag. The patients showed intact immediate priming but significantly less priming than controls at longer delays. Furthermore, word stem completion priming was abolished in the patients. One explanation for the deficit is that left inferior temporal-occipital cortex supports amodal aspects of priming, as suggested by recent neuroimaging results. Another possibility is that long-term auditory priming relies on covert orthographic representations which were unavailable in the patients. The results provide support for interactive models of word identification.     

Event-related potentials in neuropsychological studies of language

Experiments are described in which event-related potentials (ERPs) are employed to study the specialization of functions between and within the cerebral hemispheres during the performance of language and nonlanguage tasks by normal adults. Similar studies of deaf subjects suggest that the functional organization of the brain may be altered after different early language and sensory experiences. Studies of patients with alexia without agraphia suggest that the ERP may be a valuable tool with which to study cerebral reorganization after brain damage.     

Cognitive resource limitations during sentence comprehension in Parkinson's disease

We report a single-case study of peripherally acquired dyslexia that meets the clinical criteria of "alexia without agraphia." The patient, AA, has a large infarct involving the left posterior cerebral artery. The most striking feature is a severe impairment in recognizing single visually presented letters that precludes explicit or implicit access to reading, even in a letter-by-letter fashion. AA can, however, differentiate letters from similar nonsense characters and digits, and he is also able to identify alphanumeric signs when the visual channel is bypassed (through somesthesic or kinesthesic presentation). Spelling tasks are also well performed. Since there is a breakdown in mapping a visually presented letter to its abstract graphemic representation, we propose the term "visuographemic alexia" for this kind of reading disorder. The pattern of deficits is interpreted following theoretical models previously developed in cognitive neuropsychology. An alexia for arabic numerals with preserved comprehension lends additional support for the crucial processing of different notational systems (e.g., phonographic vs logographic). More general perceptive disorders do not seem to account for these patterns; they are material-specific. Finally, we attempt to specify functional correlations with the implied neural networks.     

Optic aphasia, optic apraxia, and loss of dreaming

A 47-year-old man with a left temporo-occipital infarct in the area of the posterior cerebral artery is presented. The neuropsychological examination did not reveal aphasia or gross mental deficits. The patient presented with alexia without agraphia, color agnosia, but few visual perceptual deficits. The main impairment was in confrontation naming; he was incapable of naming objects and pictures, not from lack of recognition (excluding visual agnosia) but from lack of access to the appropriate word (optic aphasia). The patient also exhibited a deficit in the evocation of gesture from the visual presentation of an object (optic apraxia) and a difficulty in "conjuring up" visual images of objects (impaired visual imagery) and loss of dreams. The fundamental deficit of this patient is tentatively explained in terms of visuoverbal and visuogestural disconnection and a deficit of mental imagery.     

Letter-by-letter reading: natural recovery and response to treatment

The present investigation provides a longitudinal study of an individual (RB) with acquired alexia following left posterior cerebral artery stroke. At initial testing, RB exhibited acquired alexia characterized by letter-by-letter (LBL) reading, mild anomic aphasia, and acquired agraphia. Repeated measures of reading accuracy and rate were collected for single words and text over the course of one year, along with probes of naming and spelling abilities. Improvements associated with natural recovery (i.e., without treatment) were documented up to the fourth month post onset, when text reading appeared to be relatively stable. Multiple oral reading (MOR) treatment was initiated at 22 weeks post-stroke, and additional improvements in reading rate and accuracy for text were documented that were greater than those expected on the basis of spontaneous recovery alone. Over the course of one year, reading reaction times for single words improved, and the word-length effect that is the hallmark of LBL reading diminished. RB's response to treatment supports the therapeutic value of MOR treatment to in LBL readers. His residual impairment of reading and spelling one-year post stroke raised the question as to whether further progress was impeded by degraded orthographic knowledge.     

Is lesion of Exner's area linked to progressive agraphia in amyotrophic lateral sclerosis with dementia? An autopsy case report

Agraphia, as a neuropsychological symptom of ALS, especially ALS with dementia (ALS-D), has recently attracted more attention. However, the brain lesion responsible has not been identified. Here we present an autopsy case of ALS-D of a patient with obvious agraphia, without aphasia, that also presented cerebrospinal degeneration with TDP-43-pathology compatible with ALS-D. Of the pre-motor frontal lobe cortices, degeneration and immuno-histochemical pathology were most obvious in the caudal area of the left middle frontal gyrus, or Exner's area. Assuring this area plays a pivotal role in the kanji and kana formation used in writing the Japanese language, this case of ALS-D showed both agraphia and Exner's area stressed pathological lesions. It may thus be the first case to indicate an intimate relationship between the neuropsychological symptoms and an associated lesion for ALS-D.     

Alexia and agraphia with spared spelling and letter recognition abilities

A patient with alexia and agraphia had intact spelling and comprehension of spelled words and used a letter-naming strategy to read and write. We propose that there is a graphemic area important for distinguishing graphemic features and for programming movements used in writing. In this patient this area was not functioning or did not have access to the area of visual word imagés. Therefore, he used an ideographic letter-naming strategy to verbally circumvent his disability and gain access to the area of visual word images.     

A case of literal alexia: evidence for a disconnection syndrome

We describe a patient with literal alexia, agraphia, and only minimal aphasia, resulting from a left occipito-parietal ischemic infarction. Neither impaired visual processing nor impaired oral output could explain the patient's letter reading deficit, since he retained the ability to match letters of mixed case, and remained able to spell aloud and to pronounce and comprehend orally spelled words. His residual word reading was characterized by better performance for nouns and highly imageable words than for functors , poorly imageable words, and nonwords. We propose that this patient's letter reading deficit was due to a partial disconnection of his visual from his linguistic systems, with residual word reading accomplished by a semantically mediated compensatory mechanism. We suggest, further, that the dissociations between word and letter reading observed in some alexic individuals may be attributed, in part, to a difference in the relative contributions of dorsal and ventral occipital structures to the reading process.     

Impaired activation of the phonological lexicon: effects upon oral reading

The role of the phonological lexicon in oral reading is examined in a patient with a small focal left hemisphere lesion. Impaired access to the patient's phonological lexicon is suggested by a number of findings, including the production of phonemic errors across a variety of tasks; increasing difficulty in word production with increasing word length; and difficulty on tests of homophone and rhyme judgments. Two competing models of reading are tested: the nonlexical ("rules") and the lexical ("no-rules") models. The rules model predicts that a disturbance in the phonological lexicon will result in surface alexia; the no-rules model predicts phonological alexia. Results indicate that the patient's reading is most similar to phonological alexia, providing support for the no-rules model. The applicability of the no-rules model to other forms of acquired alexia is explored.     

Errors resembling semantic paralexias in Spanish-speaking aphasics.

Forty-one Spanish-speaking left-hemisphere-damaged patients were selected and divided into seven groups (transcortical, Broca's aphasia, conduction aphasia, Wernicke's aphasia, anomic aphasia, alexia without agraphia, and global aphasia). A reading battery composed of eight different subtests was given to each patient (reading of letters, reading of syllables, reading of pseudowords, reading of words, reading of sentences, understanding commands, reading and comprehension of texts, and logographic reading). Different types of reading errors were analyzed. Only in the logographic reading subtest were some word-recognition errors found, resembling semantic paralexias. It is proposed that semantic paralexias in English (and other languages) depend upon the partial logographic nature of the reading system. The importance of cross-linguistic analysis of reading errors, taking into account reading system idiosyncracies, is emphasized.     

Pure agraphia in Romaji after left inferior frontal gyrus infarction: A case of selective deficit in syllable-to-grapheme conversion in Japanese

The Japanese writing system employs two distinct categories of characters: Kana and Kanji. The difference between Kana and Kanji writing corresponds roughly to that between phonological and lexical systems in Western languages. When typing, most Japanese use alphabetical characters based purely on phonological rules. In particular, the Romaji system is used, in which a syllable consists of a single vowel, a consonant–vowel compound (e.g., ka, ki), or a sequence of consonant–y(semivowel)–vowel (e.g., kya, kyu). We describe a right-handed Japanese patient who developed pure agraphia that affected Romaji writing but preserved Kana and Kanji writing and who had a lesion in the left pars opercularis of the inferior frontal gyrus that extended to the anterior precentral gyrus. The patient demonstrated literal paragraphia in spelling Romaji across modalities. Our findings suggested that the patient’s agraphia in Romaji after a confined left frontal infarction was manifested by a selective impairment in syllable-to-grapheme conversion.     

Cerebellar-induced apraxic agraphia: a review and three new cases

Apraxic agraphia is a writing disorder due to a loss or lack of access to motor engrams that program the movements necessary to produce letters. Clinical and functional neuroimaging studies have demonstrated that the neural network responsible for writing includes the superior parietal region and the dorsolateral and medial premotor cortex. Recent studies of two cases with atypical lesion localisations in the left thalamus and the right cerebellum support the hypothesis that the written language network is larger than previously assumed. The aim of this study is twofold: (1) to provide a survey of cases of apraxic agraphia published between 1973 and June 2010, and (2) to provide further evidence for a role of the cerebellum in writing via three additional cases who presented with apraxic agraphia after ischemic damage in the cerebellum. Functional neuroimaging studies by means of brain perfusion SPECT showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, syntax, naming, writing and executive functioning.     

Phonological alexia with optic and tactile anomia: a neuropsychological and anatomical study

We describe a patient with phonological alexia caused by a small hemorrhage in the posterior-inferior portion of the left temporal lobe. The lesion induced a highly selective impairment of phonological reading without concomitant oral language deficits other than anomia for objects presented in the visual and tactile modalities. We propose that an intact dorsal pathway from inferior visual association areas to Wernicke's area via the angular gyrus could mediate reading by the lexical route, while damage to a ventral pathway disrupted the patient's ability to read nonwords. We suggest further that although visually and tactually presented objects could be recognized and both verbally and nonverbally identified, they could not be named because of a disconnection from the area of word representations.