335 Journal of Neurology, Neurosurgery, and Psychiatry 1990;53:335-338 Pure agraphia of kanji due to thrombosis of the Labbe vein Takanori Yokota, Sumio Ishiai, Tetsuo Furukawa, Hiroshi Tsukagoshi Abstract A case is described of a 56 year old Japanese male with pure agraphia of kanji (the Japanese morphograms) due to haemorrhagic infarction of the left temporal lobe caused by the rare condition of cortical vein thrombosis of Labbe. Writing kanji was severely impaired without disturbed consciousness, aphasia or apraxia. On the other hand, writing kana (the Japanese syllabograms), and reading kanji and kana were almost intact. This suggests that the process of writing kanji involves a different pathway from that of reading kanji in the left temporal lobe. Pure agraphia of kanji is considered to be similar to lexical agraphia in Indo-European languages, in that the writing system with a poor or irregular. phoneme-grapheme transformation is impaired by the left temporal lesion. This case indicates the necessity for considering thrombosis of the Labbe vein when a subcortical haematoma is detected in a temporal lobe on computed tomography of the brain. Department of Neurology, Tokyo Medical and Dental University, Tokyo, Japan T Yokota S Ishiai T Furukawa H Tsukagoshi Correspondence to: Dr Takanori Yokota, Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu-shi Tokyo 183, Japan. Received 10 April 1989 and in revised form 31 July 1989. Accepted 21 August 1989 The Japanese language has two different writing systems, kana and kanji, and Japanese sentences usually consist of combinations of both. Generally, kanji are used for writing most nouns and roots of verbs, adverbs and adjectives, while kana are mainly used for inflectional endings, conjunctions and postpositions. Kana are the simple syllabograms with unique phonetic readings. Kanji are the structurally complex morphograms introduced from China, which have several phonetic readings. All kanji can be represented by kana. Japanese can be written exclusively with 104 kana, which are learnt in the first year of primary school. On the other hand, the 2000 commonly used kanji should be learnt by the end of the ninth grade. It has been suggested that the processing of kanji and kana involves a different intrahemispheric mechanism, as judged mainly through studies on patients with alexia and agraphia.'" We report a patient with thrombosis of the left Labbe vein who developed pure agraphia of kanji and compare it with agraphia in IndoEuropean languages. Case report A 56 year old right handed man, educated through high school, was admitted to another hospital for convulsion and disturbed consciousness following dysarthria and slight right hemiparesis lasting for a few hours on 24 August 1984. He soon recovered consciousness, and his dysarthria and right hemiparesis improved during the next four days. On 7 September 1984, he was admitted to our hospital having developed agraphia of kanji for the first time. On admission, neurological examination disclosed slightly increased deep tendon reflexes on the right side with an extensor plantar reflex. Consciousness, orientation, cranial nerve functions, including ocular movements and visual fields, muscle strength, sensation and gait were normal. Neuropsychological examinations disclosed slightly disturbed immediate ability to recall, name and read kanji and kana but with severely disturbed ability to write kanji. Brain computed tomography (CT) on 7 September 1984, disclosed 'a high density area around the posterior portion of the left temporal lobe and angular gyrus (fig 1A). In three weeks, neuropsychological problems other than the writing of kanji improved rapidly when the haematoma was absorbed leaving a low density area in the posterior portion of the left temporal lobe on brain CT (fig IB). Cerebral angiography on 22 October, revealed a filling defect of contrast medium in the portion in which the left Labbe vein descends between the junction of the transverse and sigmoid sinuses (fig 2). He had a second attack of consciousness disturbance and right hemiparesis, which improved in a few days in March 1987, and he was admitted to our hospital for a second cerebral angiography in June 1987. The neurological findings were normal. The neuropsychological findings were as follows: his Wechsler Adult Intelligence Scale was average (Verbal IQ = 118, Performance IQ = 114, Full Scale = 117). Neither hemineglect (bisection of a line, Albert's line-crossing test and copying the picture of a flower) nor constructional apraxia were noted. Limb and facial praxis to command, facial recognition, left-right discrimination and finger naming were intact. His spontaneous speech was fluent and well articulated. On spontaneous writing he used more kana instead of kanji than would be expected from his intelligence level (fig 3). His speech characteristics were normal on the Western Aphasia Battery (WAB).' He made no mistakes in the WAB tests of spontaneous speech, auditory comprehension, repetition and naming. In reading tasks the scores were maximum except for the following two sections: in Japanese-translated WAB, the "spelled word recognition" and "spelling" sections were changed for kanji and kana. 336 Yokota, Ishiai, Furukawa, Tsukagoshi Figure)I CT scan (1 4 days after the first attack) (A), axial view. A high density area with a vague margin was detected around the angular gyrus (top) and left posterior temporal area (bottom). Two months after the attack (B), a low density area was recognised in the left posterior temporal area (arrow) without abnormality in the angular gyrus (top). Tested kanji letters consisted of two elements, which had their own meanings and phonetic readings. "Spelled word recognition" section was changed to the recognition of kanji by hearing the structure of them, for example, the names of the elements and their positional ..j :. . * , / a :. , - Az,+~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ ... Figure 2 Angiography of the left carotid artery, venous phase, lateral view. Afilling defect of contrast medium (arrow) was recognised in the Labbe vein (L) falling into the junction between transverse ( T) and sigmoid (S) sinuses. relation between the elements. The "spelling" section was changed to the test of explaining the structure of kanji. In these two sections the scores were low (50o/, each). In writing tasks the scores for writing kanji was low (50-62 5 % ) except for copying kanji. On the other hand, the scores for writing kana and numbers were maximum. We performed special examinations for reading and writing kanji and kana. He was asked to read and write on dictation 218 kanji and 104 kana, which he had learnt in the first two years of primary school. He could not write 82 kanji out of 218 trials (37 6%). He tended to fail kanji characters which were more complex and had been learnt later. There was neither partial omissions, simplification nor confusion with other characters. On the other hand, he made no mistakes in reading kanji or in reading and writing kana letters. In contrast to the prompt responses in writing kana, and reading kana and kanji, he often took some time to write kanji, even when he eventually proved able to write them. When he could not write some kanji, he reported that he knew but could not recall them. In fact, he was able to write 337 Pure agraphia of kanji due to thrombosis of the Labbi vein Discussion Writing is a very complex function involving linguistic, acoustic, praxic, kinesthetic, visual sv and motor components. Chedru and Gesch-7. ,-o_ wind6 first indicated that writing was first II disturbed in general brain dysfunction. It has 1: 9 been suggested that agraphia was of no clinical value in the localisation of brain damage.7 3 *1 However, recently, much evidence has accumulated indicating that isolated writing 5 disturbances do occur in cases of localised brain * damage, which could not be explained by the '/ 1) -1, disturbance of any other higher functions. ('l -t Three specific areas for pure agraphia have been reported: the left posterior frontal lobe,8 9 4i~ the left superior parietal lobe'"'2 and the left i'~) temporal lobe.""'6. A? d Our case showed no disturbance of cons4 ciousness or intelligence, or aphasic or apraxic errors in the chronic phase even after a k^J l *tt thorough examination. The pure agraphia in . 5.0 it our case was therefore considered to be a focal cerebral sign. The lesion was clearly demonstrated in the left temporal lobe on CT and MRI, which was probably due to the thrombosis of the Labbe vein. Mochizuki and Ohtomo'7 reported a case of Figure 3 Spontaneous writing. He was requested to use kanji as much as po ssible. He wrote the corresponding kana (circle) instead of kanji, which were learnt in* primary infarction of the left occipital lobe and inferior school. On the other hand, kana were written correctly. There were no parag raphSerrors. temporal gyrus, who initially had the difficulty t The meanings of the sentences are; "On 17 November I went to Shinjuku and visited the in kanji reading and writing and kana writing, I dropped into my in and on the way home grave a temple with my wife, chihd's house, and I arrived at Seibu-Shinjuku station, and Ifelt ill when I got on the trai zn,and I was but later kanji reading improved with little brought to Tokyo Medical and Dental University Hospital by ambulance." improvement in kanji writing. They proposed that the left inferior temporal lobe might be complex kanji immediately after Iglancing at indispensable for writing kanji. Recently Soma them, but could not recall them spo ntaneously. et al" first described "pure agraphia of kanji" When he wrote kanji and kana w,ith his left in three cases with haematoma, infarction and trauma. All these cases had the lesion in the left hand, the results did not change maLrkedly. Magnetic resonance imaging (MR{I) revealed posterior temporal area probably extending to the lesions in the posterior part of le ft temporal the angular gyrus on CT. Although they had cortex (fig 4A) and the adjacent white matter amnesic aphasia, alexia and agraphia in the (fig 4B) with dilatation of the inferior horn of acute phase, disturbances other than agraphia the left lateral ventricle. There wer4 e no abnor- of kanji disappeared in a few months. The malities in the left angular gyrus. Oni the second clinical course, symptoms and lesion of our case cerebral angiography, the same findling, that is, was very similar to those of Soma's cases. Our the filling defect of the left Labb4 e vein, was case, however, clearly demonstrated that amnesic aphasia and alexia, associated with seen as before. 'It f- 0 ,) -I 0 le Figure 4 MRI (3 months after the second attack), T2-weighted image (TR = 2500 ms, TE = 105 ms). (A) Axial view. Low signal area in the posterior portion of left middle temporal lobe (arrow). (B) Frontal view. High signal area in the white matter of left temporal lobe (arrow head). 12 Yokota, Ishiai, Furukawa, Tsukagoshi 338 agraphia of kanji in the acute phase, was due to the lesion in the left angular gyrus, and that the remaining symptom of pure agraphia of kanji was caused by the left posterior temporal lesion. The haematoma, seen around the posterior temporal area and angular gyrus in the acute phase, was absorbed leaving the localised lesion in the posterior temporal area with no abnormality in the angular gyrus on CT and MRI. In reviewing reported Japanese cases with alexia and agraphia, Iwata' proposed the following pathway for the writing of kanji: Wernicke's area(W)-posterior inferior temporal acute phase, two cases2223 had sensory aphasia and the other2" had motor aphasia. However, there was no detailed description about agraphia in the reports. Cambria's case2' had carbon monoxide poisoning, and Komiyama's22 case was deficient in anti-thrombin III. In our report and that of Kawase23 the cause of the thrombosis could not be determined. All the reported cases including ours were accompanied by lobar subcortical haemorrhages, which were located around the left posterior temporal lobe and angular gyrus. Therefore, in such situations, it is necessary to consider Labbe vein thrombosis. area(T)-occipital lobe(O)-angular gyrus(A)anterior speech area of Broca and motor association area of hand(M). This is the pathway involved in selecting the correct kanji We thank Drs Ryoko Okusa, Yoshinori Ito and Kyoko graphemes according to the meaning of the Eguchi for their help. words, and thus recalled visual engrams of letters are sent to the angular gyrus which mediates the transformation of them into the 1 Iwata M. Kanji versus Kana. Neuropsychological correlates of the Japanese writing system. Trans Neurosci 1984; motor engrams of letters. On the other hand, 7:290-3. was postulated 2 Oka H, Asano T, Hattori S, Mochio S, Sugishita M. Pure the pathway for reading of kanji alexia and agraphia caused by the left inferior temporal to be O-T-W, for the writing of kana, W-A-M, and fusiform gyri. Neurol Med (Tokyo) 1985;23:73-6. and for reading kana, O-A-W. Our case sugges3 Kawahata N, Tagawa K, Hirata Y, Nagata K, Shishido F. A case of alexia with agraphia due to the infarction in the left ted that the process of writing kanji is different inferior-posterior temporal lobe. Clin Neurol (Tokyo) from that of reading kanji or kana, or writing 1987;27:420-7. 4 Kawamura M, Hirayama K, Hasegawa K, Takahashi N, kana. According to Iwata's hypothesis, in our Yamaura A. Alexia with agraphia of Kanji (Japanese case, the writing pathway for the kanji from W morphograms). J Neurol Neurosurg Psychiatry 1987; 50:1125-9. to T was selectively disturbed by the lesion in 5 Kertesz A. The Western Aphasia Battery. (Translated into the white reading the temporal matter, sparing Japanese, Sugishita Mj et al, Tokyo: Igaku-Shoin, 1986) New York: Grune and Stratton, 1982. pathway for kanji from T to W. 6 Chedru F, Geschwind N. Writing disturbances in acute In Indo-European languages two different confusional states. Neuropsychologia 1972;10:343-53. A: The agraphias. In: Vinken PJ and Bruyn GW agraphias, lexical and phonological, have 7 Leischner (eds): Handbook of Clinical Neurology, Amsterdam, 1969; been discussed.'6 1820 Beavois and Derouesne'9 4:141-80. W, Roberts L. Speech and brain mechanisms, argued that writing a word can be performed 8 Penfield Princeton: Princeton University Press, 1959. either by using a direct lexical method of 9 Dubois J, Hecaen H, Marcie P. L'agraphie "pure". Neuropsychologia 1969;7:271-86. obtaining its spelling (the "lexical route") or by 10 Russel R, Espir MLE. Traumatic aphasia. Oxford: Oxford sounding-out the word into phonemes and University Press, 1961. DB. Agraphia selective for M, Rosenfield employing phoneme-grapheme transforma- 11 Kinsboume written spelling: an experimental case study. Brain Lang tions. Lexical agraphia consists of a specific 1974;l:215-26. SH, Alexander MP. Pure agraphia and unilateral difficulty in writing those words which use 12 Auerbachataxia associated with a left superior parietal lobule optic any irregular or ambiguous phonemelesion. J Neurol Neurosurg Psychiatry 1981;44:430-2. Ein Fall von reiner motorischer Agraphie. Arch grapheme transformations, while the writing 13 Boettiger A. Nervenkr 1922;65:87-103. Psychiatr of regular words and nonsense syllables is 14 Rosati P. Pure agraphia: a discrete form of Bastiani G, Neurol J Psychiatry 1979;42:266-9. Neurosurg agraphia. preserved. Kana are the simple syllabograms 15 Soma A, Sugishita M, Maruyama S, Kitamura K, Tsubaki with unique phonetic reading, and the words T. "Pure agraphia of Kanji" associated with the left posterio-inferior temporal lesions. Neurol Med (Tokyo) using kana are orthographically regular. On the 1988;29: 172-8. other hand, kanji are the structurally complex 16 Croisile B: Agraphies pures, agraphies parietales, agraphia lexicales? A propos de trois observations. These de morphograms with several phonetic readings. Medicine, Lyon, 1987. Kanji cannot be read aloud without knowing 17 Mochizuki H, Ohtomo R. Pure alexia in Japanese and agraphia without alexia in Kanji. Arch Neurol 1988; how they should be pronounced, even when 45:1157-9. their meanings are understood. Therefore, 18 Shallice T. Phonological agraphia and the lexical route in writing. Brain 1981;104:413-29. in agraphia of kanji is similar to lexical agraphia M-F, Derouesne J. Lexical or orthographic Indo-European languages in that the writing 19 Beauvois agraphia. Brain 1981;104:21-49. 20 Roelgen DP, Sevush S, Heilman KM. Phonological system with a poor or irregular phonemeagraphia: Writing by the lexical-semantic route. The is transformations impaired. grapheme Neurology 1983;33:755-65. 21 Cambria S. Infarctus cerebral hemorragique par thrombose case reported by Croisile'6 had the lesion in the de la veine de Labbe. Rev Neurol 1980;136:321-6. left posterior temporal area, which was similar 22 Komiyama A, Hirayama K, Watanabe Y. Cerebral cortical vein thrombosis presenting with lobar hemorrhage in a to those in our case and those reported by with familial antithrombin III deficiency. Clin patient Soma. Neurol (Tokyo) 1988;28:493-6. So far as cerebral vein thrombosis is concer- 23 Kawase T, Tazawa T, Mizukami M.andCerebral venous operative findthrombosis-Computed tomography ned, only three cases with thrombosis of the ings in 4 cases of simulated subcortical hemorrhage. CT Study (Tokyo) 1983;5:19-27. Labbe vein2"23 have been reported. In their