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.     

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.     

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.     

Progressive agraphia can be a harbinger of degenerative dementia

By investigating three patients with progressive agraphia, we explored the possibility that this entity is an early sign of degenerative dementia. Initially, these patients complained primarily of difficulties writing Kanji (Japanese morphograms) while other language and cognitive impairments were relatively milder. Impairments in writing Kana (Japanese syllabograms), verbal language, executive function, visuo- and visuospatial cognition and memory were identified by neuropsychological testing. The agraphia was compatible with a peripheral type, based on deficits at the interface between the central letter selection and the graphemic motor execution (Patient 1) or at the stage of central letter selection as well (Patients 2 and 3). Agraphia was generally more prominent, although not exclusive, for Kanji probably because of later acquisition and larger total number of Kanji letters leading to lower frequency of use and familiarity per letter. Concurrent or subsequent emergence of non-fluent aphasia, ideomotor apraxia, executive dysfunction and asymmetric akinetic-rigid syndrome in two patients suggested degenerative processes involving the parietal-occipital-temporal regions, basal ganglia and striato-frontal projections. We propose that progressive agraphia may be one of the early symptoms of degenerative dementia such as corticobasal degeneration.     

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.     

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.     

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.     

Anatomy of posterior pathways in reading: a reassessment

Contemporary accounts of the neurology of reading stem from Dejerine's original visual-verbal disconnection hypothesis of pure alexia. Reassessment of Dejerine's traditional formulations for posterior left hemisphere pathways in reading suggests that the occipital cortex-left angular gyrus-Wernicke's area scheme is undoubtedly oversimplified. The role of left angular gyrus cortex in reading is unsettled, and although clinically undefined, more inferior portions of the left temporal lobe may also contribute to the reading process. Nevertheless, to a surprising extent, the neuroanatomic edifice erected by Dejerine remains largely intact.     

Can treatment for pure alexia improve letter-by-letter reading speed without sacrificing accuracy?

An experimental treatment study designed to improve both the accuracy and the speed of reading was administered to a patient with pure alexia and impaired letter naming. The study focused on the use of letter-by-letter reading. A two-stage approach was employed. The first stage implemented a tactile-kinesthetic strategy to improve accuracy. The second stage concentrated on speed. At the end of the treatment, patient DL was reading both trained and untrained words more accurately and with considerably greater speed than prior to treatment. Accuracy and speed of reading at the sentence level improved as well.     

Kanji reading by a prekindergarten language-disabled child: A pilot study

A , severely language-disabled boy was taught 250 words made up of one, two, three, or four Japanese/Chinese kanji. Many kanji words were learned and some ability to process these in simple sentences was observed. Acquisition of a word for meaning was not dependent on its visual complexity, but on the child's ability to grasp the meaning of the word. Results suggest that a whole-word method may facilitate very early reading acquisition and a possible neurophysiological correlate of this is proposed. Kanji or whole words may prove useful for language-handicapped individuals unable to process words phonetically.     

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.     

A case study of an English-Japanese bilingual with monolingual dyslexia

We report the case of AS, a 16 year-old English/Japanese bilingual boy, whose reading/writing difficulties are confined to English only. AS was born in Japan to a highly literate Australian father and English mother, and goes to a Japanese selective senior high school in Japan. His spoken language at home is English. AS's reading in logographic Japanese Kanji and syllabic Kana is equivalent to that of Japanese undergraduates or graduates. In contrast, his performance in various reading and writing tests in English as well as tasks involving phonological processing was very poor, even when compared to his Japanese contemporaries. Yet he has no problem with letter names or letter sounds, and his phoneme categorisation is well within the normal range of English native speakers. In order to account for our data that show a clear dissociation between AS's ability to read English and Japanese, we put forward the ‘hypothesis of granularity and transparency'. It is postulated that any language where orthography-to-phonology mapping is transparent, or even opaque, or any language whose orthographic unit representing sound is coarse (i.e. at a whole character or word level) should not produce a high incidence of developmental phonological dyslexia.     

Subangular alexia without agraphia or hemianopsia

A 40 year old right-handed woman underwent resection of an unruptured vascular malformation which was located just deep to the left posterior insula. Postoperatively she demonstrated a transient syndrome of alexia without agraphia or hemianopsia. Analysis of her postoperative findings, in conjunction all available anatomical data, let to the conclusion that the responsible lesion was in the white matter of the left occipitotemporal region, below the angular gyrus and lateral to the lateral ventricle. Hence the lesion in the present case was truly subangular, and is therefore distinctly differentiated from the classical lesion in alexia without agraphia, which may be described as splenio-occipital.     

Rapid word identification in pure alexia is lexical but not semantic

Following the notion that patients with pure alexia have access to two distinct reading strategies-letter-by-letter reading and semantic reading-a training program was devised to facilitate reading via semantics in a patient with pure alexia. Training utilized brief stimulus presentations and required category judgments rather than explicit word identification. The training was successful for trained words, but generalized poorly to untrained words. Additional studies involving oral reading of nouns and of functors also resulted in improved reading of trained words. Pseudowords could not be trained to criterion. The results suggest that improved reading can be achieved in pure alexia by pairing rapidly presented words with feedback. Focusing on semantic processing is not essential to this process. It is proposed that the training strengthens connections between the output of visual processing and preexisting orthographic representations.     

Selective alexia and agraphia sparing numbers-a case study

We report a patient (MT) with a highly specific alexia affecting the identification of letters and words but not numbers. He shows a corresponding deficit in writing: his letter writing is impaired while number writing and written calculation is spared. He has no aphasia, no visuo-perceptual or -constructional difficulties, or other cognitive deficits. A similar pattern of performance has to our knowledge only been reported once before [Anderson, S. W., Damasio, A. R., & Damasio, H. (1990). Troubled letters but not numbers. Domain specific cognitive impairments following focal damage in frontal cortex. Brain, 113, 749-766]. This study shows that letter and number reading are dependent on dissociable processes. More interestingly, it points to a common mechanism subserving the perception and production of letters. We suggest that a deficit in a visuo-motor network containing knowledge of the physical shape of letters might explain the pattern of performance displayed by MT.     

Letter imagery deficits in a case of pure apraxic agraphia

Pure apraxic agraphia is a condition in which motor writing is impaired but limb praxis and nonmotor writing (typing, anagram letters) are preserved. This condition is believed to result from disruption of a parietal lobe graphemic area which generates grapheme representations used to program motor-writing patterns. We report a single case of pure apraxic agraphia in which defective letter imagery was evident. Results of writing and imagery evaluations suggest that the graphemic area may be responsible for generating letter images. In this respect, clinical evaluation of letter imagery abilities in cases of apraxic agraphia may be useful in identifying subtypes of the disorder resulting in a better understanding of the neuropsychological process involved in writing.     

Age-of-acquisition effects in pure alexia

Pure alexia is an acquired reading disorder in which previously literate adults adopt a letter-by-letter processing strategy. Though these individuals display impaired reading, research shows that they are still able to use certain lexical information in order to facilitate visual word processing. The current experiment investigates the role that a word's age of acquisition (AoA) plays in the reading processes of an individual with pure alexia (G.J.) when other lexical variables have been controlled. Results from a sentence reading task in which eye movement patterns were recorded indicated that G.J. shows a strong effect of AoA, where late-acquired words are more difficult to process than early-acquired words. Furthermore, it was observed that the AoA effect is much greater for G.J. than for age-matched control participants. This indicates that patients with pure alexia rely heavily on intact top-down information, supporting the interactive activation model of reading.     

Age-of-acquisition effects in pure alexia

Pure alexia is an acquired reading disorder in which previously literate adults adopt a letter-by-letter processing strategy. Though these individuals display impaired reading, research shows that they are still able to use certain lexical information in order to facilitate visual word processing. The current experiment investigates the role that a word's age of acquisition (AoA) plays in the reading processes of an individual with pure alexia (G.J.) when other lexical variables have been controlled. Results from a sentence reading task in which eye movement patterns were recorded indicated that G.J. shows a strong effect of AoA, where late-acquired words are more difficult to process than early-acquired words. Furthermore, it was observed that the AoA effect is much greater for G.J. than for age-matched control participants. This indicates that patients with pure alexia rely heavily on intact top-down information, supporting the interactive activation model of reading.