BRAIN AND LANGUAGE 19, 346-356 (1983) Ideogram Writing in a Disconnection Syndrome ATSUSHI YAMADORI, TATSUYA Kobe University NAGASHIMA, School AND NORIHIKO TAMAKI of Medicine 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. INTRODUCTION One of the prominent effects of destruction of the corpus callosum in human beings is that linguistic information of the left hemisphere (in the majority of cases of right-handers) stops arriving at the right hemisphere. One sign of this effect is agraphia confined to the left hand (Geschwind & Kaplan, 1962; Bogen, 1969). The linguistic nature of this disconnectiontype agraphia has been made clear in recent years (Geschwind, 1965; Zaidel & Sperry, 1977), but its exact nature still remains unsettled. Reports of cases from countries where nonalphabetic writing systems are being practiced might contribute to further interests in this difficult issue. In a report of left-sided disconnection agraphia from Japan these authors have pointed out that Kanji (ideogram) and Kana (phonogram) writing were differentially affected, the latter being more disturbed than the former (Yamadori et al., 1980). Sugishita and others reported a similar phenomenon (Sugishita et al., 1980). This is another case report of dissociated agraphia of the disconnection type for Kanji and Kana letters. A complex combination of lesions caused destruction of the posterior half of the corpus callosum and the left medial Send requests for reprints to Atsushi Yamadori, M.D., Neurology Service, Hyogo Brain and Heart Center at Himeji, 520 Saisho-Ko, Himeji, 670, Japan. 346 0093-934X/83 $3.00 Copyright All rights 0 1983 by Academic Press, Inc. of reproduction in any form reserved. IDEOGRAM WRITING 347 occipital lobe. As a result a unique selective affection of written symbols resulted. Analysis of the symptom complex led us to a hypothesis that the neural substrate of both Kanji and Kana graphemes necessary for writing is stored bilaterally, while the neural substrate for ordering these graphemes into a meaningful sequence is confined to the left hemisphere. REPORT OF A CASE An 18-year-old right-handed female high school student suddenly developed a headache and lost consciousness on a road while traveling on August 5, 1980. In the hospital she was in a coma and anisocoria (left larger than right) was noted. Immediate arteriography revealed a huge arteriovenous malformation in the area of the posterior portion of the corpus callosum. Computed tomography revealed a high-density area in the posterior portion of the body of lateral ventricle on both sides and in the white matter neighboring the left trigone. Craniotomy was immediately performed to evacuate the hematoma. Slightly left of the corpus callosum, the roof of the body of the left lateral ventricle was excised in its posterior portion for about 3 cm. The blood in the ventricle was then evacuated. Resection of the arteriovenous malformation was not tried. Postoperative course was complicated by development of meningitis. She was transferred to the Department of Neurosurgery, Kobe University School of Medicine, for further treatment on September 29, 1980. On neurological examination, mild right-sided weakness and slight rightsided hyperreflexia were present. Plantar reflex was extensor on the right. Sensation was decreased for all modalities on the right. Two-point discrimination and point localization, as well as stereognosis, were also defective on the right. Perimetry revealed dense congruent right homonymous hemianopsia with macular split. The neuropsychological findings remained essentially unchanged up to the day of her operation on December 1, 1980. She was alert, attentive, and cooperative. Recent memory was moderately impaired. Recall of three items after 5 min was faulty. Remote memory for events prior to the onset of the present illness was well preserved. Impoverishment of spontaneous speech was noted but articulation as well as grammatical forms was normal. Repetition was done easily. Comprehension of spoken language was intact. Mild anomia was present. Neither ideomotor nor ideational apraxia was present bilaterally. Visual recognition was well preserved for common objects, pictures, and colors. Ability of tactile recognition of the left hand was intact. Details of major defects are described below. Reuding Performance The patient had difficulty reading. She read presented materials slowly and hesitantly, pointing at each letter with the hand. Paralexia was present 348 YAMADORI, NAGASHIMA, AND TAMAKI for single letters as well as for words. She was able to read about 80% of the test material correctly. Performance of oral reading for Kanji and Kana letters was essentially at the same level. Comprehension of written material was also defective. But meaning of Kanji was recognized better than Kana word. This was well manifested by the abundant semantic paralexia for Kanji word. Number reading was also defective. Kinesthetic cueing (Schreibendes Lesen) was effective. Writing Performance Writing performance was repeatedly tested with each hand. The ability was different depending upon which hand was tested. The RIGHT-hand writing was completely normal for Kana usage. Letters, words, and sentences were flawlessly written to dictation. However, for Kanji letters, many incomplete graphemes were produced. Most often only a portion of a Kanji grapheme was produced. Unnecessary iteration or omission of stroke was also noted. Thus only half of the tested Kanji letters were correctly written. LEFT-hand writing performance was opposite that of the right hand. This hand was totally unable to produce correct words or sentences in Kana. However, Kana grapheme itself was correctly produced most of the time. For instance, a four-letter word, SU-I-E-I (swimming) was written correctly by the right hand but the left hand produced a fourletter jargon, SU-SE-I-YA for the same word. Letters produced were often perseverative. On the contrary Kanji writing by the left hand was much superior to that by the right. Often the left hand was able to write the very letter the right hand had failed. For instance, a Kanji letter UMA (horse) which was not written by the right hand was correctly written by the left hand with ease. It has to be noted that Kanji writing by the left hand was not perfect as was the case with Kana by the right hand. There were many errors. But the nature of errors was different from that of the right hand. In contrast to graphic errors of the right hand, they consisted of perseveration of previously produced Kanji or semantic substitution of a required Kanji letter. For example, a word meaning foreign country made of two Kanji letters was substituted by a two-letter Kanji word meaning overseas. The difference in writing performance between the two hands was also noted in dictation of digit series. Thus the right hand could produce the five-digit series 1 3 8 4 0 with ease but the left hand produced a wrong sequence, 1 3 6 5 4 0. Copying and Drawing Performance COPYING OF LETTERS by the right hand was defective. She would give up copying after having tried a few strokes. No difference was present between Kanji and Kana letters. On the contrary copying of letters by the left hand was quite good. But success of copying did not IDEOGRAM WRITING 349 FIG. I. Example of Kana writing. Right column in descending order, a correct fourletter word HO,U,SO,U (broadcasting) by the right hand. Left column in descending order, a jargon JI,YU.U,MA and an unidentifiable letter by the left hand for the same target word. necessarily lead to restoration of reading. The same dissociated performance between the two hands was also seen in copying geometrical figures. For example, she could not copy a cube with the right hand but could copy it correctly with the left hand. DRAWING WITHOUT A MODEL was also dissociated. The command “draw a square and then draw a circle in it” was beyond the ability of the right hand. Only perseverative squares were produced. But the left hand drew the pattern easily. If only the right hand was tested, this result might have been taken as a sign of comprehension difficulty. To sum up, the patient showed alexia of the right visual field, dissociated difficulty of Kanji writing between the two hands, left unilateral difficulty of Kana writing, right unilateral difficulty of copying letters and geometrical figures, and right unilateral difficulty of drawing geometrical figures without a model. Neurorudiological Evaluation Computed tomography (CT) scan of the brain with enhancement showed a high-density area in the region of the posterior two-thirds of the corpus FIG. 2. Example of Kanji writing. Left column in descending order. a two-letter word HO-U, SO-U (broadcasting) by the left hand. For the same word the right hand produced only a portion of the frst letter HO-U. as shown to the right. 350 YAMADORI, NAGASHIMA, AND TAMAKI FIG. 3. Writing of digit series. Right column was written by the right hand and all are correct. The left column was written by the left hand. Only the second series (8 5 6) is correct. The columns should be identical. callosum mainly on the left side. The medial part of the left occipital lobe showed irregular enhancement coupled with a low-density area. The left trigone was enlarged. The coronal section showed an enhancing lesion in the posterior corpus callosum protruding into the left lateral ventricle. The deep white matter of the left cerebral hemisphere neighboring the posterior end of the body of the lateral ventricle showed a low-density area. Arteriographic study confirmed a large arteriovenous malformation situated at the posterior part of the corpus callosum on the left side. Main feeders consisted of the left and right pericallosal arteries, left FIG. 4. Copy of a cube. The upper one is a model. The lower left cube was copied by the left hand and is essentially correct. The lower right was copied by the right hand. 1DEOGRAM WRITING TABLE SUMMARY Kanji writing Kana writing Copying of letters Copying and drawing of geometrical figures OF RIGHT-LEFT 3.51 I DISSOCIATION Right hand Left hand Graphic difficulty Normal Faulty Faulty Paragraphia Jargon Good Good posterior choroidal artery, and left posterior cerebral artery. They drained to veins of Galen and Labbe. Thus lesions were composed of destruction of the posterior part of the corpus callosum, medial left occipital lobe, and deep left parietal white matter. In addition, the posterior 3 cm of the medial white matter on the roof of the left lateral ventricle was sectioned in the first operation, adding damage to callosal fibers. Also at the later operation destruction of the splenium by hematoma was confirmed. Hospitul Course On December 1. 1980, the patient underwent an operation and most of the mass was resected. On February 2, 1981, the last operation was FIG. 5. CT scan with enhancement. Most of the corpus callosum and the left lateral ventricle is occupied by an enhancing mass. In addition, the medial portion of the left occipital lobe shows a low-density area. 352 YAMADORI, NAGASHIMA, AND TAMAKI FIG. 6. Coronal CT of the same patient. Enhancing callosal lesion is exclusively situated in the left side. A low-density area is seen in deep white matter around the left lateral ventricle. performed to remove the remainder of the AV malformation. Unfortunately her memory, language, as well as the right hemiparesis deteriorated after the operation. She became unable to maintain a long attention span. As a result examination became very sketchy, but the essential feature of the asymmetrical writing performance between the two hands remained unchanged. DISCUSSION Lesion distribution of the present case could be summarized very schematically as an addition of an anteriorly extended callosal lesion to the classical combination of splenial and left medial occipital lesion often seen in cases of left posterior cerebral artery infarction. Neuropsychologically it can be understood as a combination of left unilateral agraphia and alexia without agraphia. Thus the case is unique in a sense that symptoms are mostly limited to affection of written language. As has been described in several previous papers (see, for example, Yamadori, 1973, the Japanese script system is composed of two different types of letters, i.e., Kanji (Chinese characters or ideograms) and Kana (phonograms). Kanji in Japanese usage represents various substantive concepts ranging from abstract ideas, such as pleasure, to concrete objects, such as an egg. These concepts are expressed in single Kanji or plural IDEOGRAM WRITING 353 Kanji combinations. These Kanji words naturally have their own linguistic names, i.e., phonetic labels. But in usual silent reading these words are very often scanned only for meanings. As a matter of fact, an average Japanese would have many Kanji vocabulary words whose meanings are quite familiar but whose exact phonetic labels are not. Kana, on the other hand, represents only sound. A Kana letter represents a syllable. They are small in number (46). With Kana all Japanese expressions are possible. Kanji has no such function. In modern Japanese, Kanji is used for a substantive portion of a sentence and Kana is used for the remainder. Dissociation of Letter Writings between the TMY~Sides Kanu letters. The RIGHT hand showed the normal ability of language expression with Kana letters. All words and sentences produced in Kana by this hand were linguistically correct. Her LEFT hand, on the contrary, produced correct Kana letters but in nonmeaningful sequence. The same phenomenon was also noted in number dictation. These two aspects of the left Kana agraphia, correct grapheme production and incorrect grapheme sequencing, lead to the following speculation. The right hemisphere seems to possess neural substrate for motor output of letter graphemes just like the left hemisphere. What the right hemisphere lacks is the higher linguistic function. i.e., how to combine the available units into a meaningful series, which is a left-hemisphere property. This line of reasoning is consistent with the recorded performance of the left hand of the commissurotomized or the commissure-injured patients. Thus a patient of Levy et al. (1971) wrote “PI” and “PENCIL” in capital letters for a tobacco pipe. A patient of Geschwind and Kaplan (1962) wrote “yonti” for “yesterday.” Did neural impulses for these letters also come from the left hemisphere? It seems more natural to assume that these letters P,I,E,N,C,I,L,y,o,n,t, and i must be the property of the right hemisphere, while “pipe” or “yesterday” may not. The right hemisphere tries to mobilize these letter units, but without the correct plan for sequencing only jargon is produced. Kanji fetters. In Kanji writing both hands showed subnormal capacity but the underlying mechanism seemed to be qualitatively different. The RIGHT hand, having shown no difficulty in Kana writing, had difficulty in realizing Kanji graphemes. The Kanji graphemes produced were similar in their Gestalt to correct ones but not precise. This was not a linguistic defect. A similar kind of defect limited to Kanji letters has long been known to occur in cases of alexia without agraphia in Japanese patients (Miura, 1933). The features of this Kanji dysgraphia of pure alexics were summarized recently by Iwata (1980). Of these, partial production of a target grapheme and, duplication or omission of a constituent stroke are important. 354 YAMADORI, NAGASHIMA, AND TAMAKI This is exactly what happened in the present case. As in alexia without agraphia which results in damage to the left occipital lobe and the splenium, our patient also showed the same lesional distribution and the same clinical picture in addition to the left agraphia. The occipital lesion may be responsible for this Kanji dysgraphia of the contralateral hand. Because Kanji has more complicated graphic structures than Kana, an intact contralateral occipital visual system may be necessary for normal writing of Kanji. The LEFT hand showed remarkable capacity in that it could write the very Kanji word that the right hand failed to produce, which may reflect the intactness of the right occipital lobe. The fact is doubly interesting because this left hand could not produce the same word as a combination of Kana letters. Thus it is quite likely that the right hemisphere has the neural substrate for Kanji graphemes just as it does for Kana and digit graphemes. Since Kanji is an ideogram some meaning could easily be expressed even with a single letter realization. Surprisingly, many correct Kanji combinations were also produced. It is true that errors were also seen. However, these were paragraphic substitutions in the same semantic sphere and were not graphic errors. Thus the right hemisphere seems to possess a certain degree of semantic processing capacity at word level which is probably unrelated to phonetic sequencing capacity. This vague semantic capacity without phonetic exactness may have contributed to the surprising success of correct Kanji letter combinations by the left hand. From the above arguments these authors come to the conclusion that Kanji and Kana letters as an element of linguistic expression are bilaterally represented. It is a different mode of processing strategy which produced the apparent dissociation of Kanji and Kana writing in the present case. This conclusion is consistent with what was proposed in another paper (Yamadori, 1980a). Dissociation of Copying and Drawing between the Two Sides Copying of letters. The present case showed selective difficulty in copying letters with the right hand. The left hand copied them correctly. This unilateral dyscopia of letters was first observed in patients of alexia without agraphia and was explained as a result of isolation of normal visual perception of letters in the right visual cortex from the left hemisphere because of the combined lesions of the splenium and the left medial occipital lobe (Yamadori, 1980b). The same mechanism must be in operation in the present case. Copying and drawing of geometrical jigures. Difficulties in copying geometrical figures limited to the right hand have been observed in commissurotomized patients and have been attributed to the disconnection of the left hemisphere from the dominant visuospatial influence of the IDEOGRAM WRITING 355 right hemisphere (Gazzaniga et al.. 1965; Zaidel & Sperry, 1977). In the present case, defects in spontaneous drawing of figures can be explained by this hypothesis. But as far as copying is concerned, dyscopia of figures can be as easily explained by the same mechanism as letter dyscopia without recourse to the influence of the right parietal lobe. For instance, dyscopia of cubes limited to the right hand was reported in a case of alexia without agraphia (Yamadori, 1980b). Thus there is a possibility that unilateral dyscopia of figures can be caused by different mechanisms. Reading Perj2mnance Moderate alexia in the present case affected both Kanji and Kana to the same degree. Some semantic processing capacity for Kanji words was noted but not for Kana words. Based on CT findings, the alexia seems to represent a typical example of alexia without agraphia. Since she had a complete right hemianopsia and complete callosal destruction at least in the posterior portion, her reading capacity can be taken as a sign of right hemisphere function. Our evidence obtained from a single Japanese case favors the notion that the right hemisphere has linguistic capacity to a certain degree. First, the neural substrate for letter graphemes necessary for writing is stored. Second, some capacity of semantic processing at word level is present. This capacity may remain latent and can only be tapped if an appropriate strategy such as ideogram reading or writing is available. The conclusion is different from that obtained with commissurotomized patients by Gazzaniga and Sperry (1967). They concluded that linguistic expressive capacity seemed to be organized almost exclusively in the left hemisphere. But later studies (Levy et al., 1971; Gazzaniga et al., 1977) showed that there could be patients whose minor hemispheres have some capacity to express at least simple language through control of the left hand. Levy (1978) concluded that language comprehension is apparently quite good in the right hemisphere, for both spoken and written words and phrases in spite of its essentially total lack of phonetic analyzers. This comment seems to be quite true also for written expression at least for the present case. Since our experience lacks encounters with patients with complete callosal destruction, this conclusion remains tentative. SUMMARY A case of disconnection-type agraphia coupled with alexia was reported. Neuropsychologically 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 difficulty of copying letters and geometrical figures, and right unilateral difficulty of drawing geometrical figures without model. 356 YAMADORI, NAGASHIMA. AND TAMAKI Anatomically lesions involved most of the corpus callosum in its posterior portion including the splenium and the left medial occipital lobe. Analysis of the data led us to following conclusions. 1. The right hemisphere seems to possess neural substrate for letters regardless of its type. 2. The right hemisphere seems to have no capacity to organize Kana or digits into a correct sequence. 3. The right hemisphere seems to have some capacity to process words at the semantic level. 4. For correct Kanji writing, particularly visual support seems to be a prerequisite. 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