Left Unilateral Agraphia and Tactile Anomia Disturbances Seen After Occlusion of the Anterior Cerebral Artery Atsushi Yamadori, MD; Yukio Osumi, MD; Hiroto Ikeda, MD; Yasuhisa Kanazawa, MD \s=b\ A 53-year-old right-handed Japanese had a callosal disconnection syndrome associated with an occlusion of the left pericallosal artery. Computerized tomography scan confirmed a left medial lesion affecting the cingulate gyrus, the paracentral lobule, the precuneus of the left hemisphere, and most probably the corpus callosum. This anterior cerebral man artery syndrome was characterized by right hemiparesis with predominant crural involvement, unilateral left-sided "disconnection" agraphia, and left unilateral "disconnection" tactile naming deficit. To our knowledge, this is the first case in the literature of the anterior cerebral artery syndrome in which unilateral left\x=req-\ sided apraxia was not associated with agraphia. The case is also the first in which Kanji-Kana dissociation of an aphasic nature has been reported as a feature of isolated left agraphia. (Arch Neurol 37:88-91, 1980) TTThen one anterior cerebral artery " ' is occluded, several characteristic signs may develop, depending on the exact site of occlusion. According to Critchley,1 the most frequently en¬ countered signs are contralateral pa¬ ralysis of the lower extremity, forced grasping or groping of the ipsilateral upper limb, and ideomotor apraxia of the left arm, regardless of the side of the infarction. Left-sided ideomotor apraxia was first described by Liepmann and Accepted for publication Feb 7, 1979. From the Departments of Neurology and Psychiatry (Drs Yamadori, Osumi, and Ikeda), and Neurosurgery (Dr Kanazawa), Kobe University School of Medicine, Kobe, Japan. Reprint requests to Department of Neurology and Psychiatry, Kobe University School of Medicine, Kusunokicho 7-chome, Ikutaku, Kobe 650, Japan (Dr Yamadori). Maas--' in a patient with left anterior cerebral artery occlusion. They postu¬ lated that it was due to involvement of the corpus callosum, the anterior four fifths of which is nourished by the anterior cerebral artery.1·4 According to Liepmann, apraxia of the left hand is a result of disturbed impulse trans¬ mission from the leading sensorymotor cortex of the left hemisphere to the right hemisphere. The role of the callosal lesions as a cause of the left ideomotor apraxia accepted by Liepmann's con¬ temporaries. Tumor cases50 as well as right-sided anterior cerebral artery occlusion7 have been reported. was soon 8 In addition to the left-sided aprax¬ ia, left-sided agraphia and left-sided astereognosis had also been observed and were attributed to a lesion of the corpus callosum. The agraphia had been explained as a partial symptom of the left-sided ideomotor apraxia.The astereognosis had been explained as a loss of the dominant left hemi¬ sphere influence of stereognostic ca¬ pacity over the right hemisphere because of callosal disconnection." Geschwind and Kaplan,1"" howev¬ er, presented a consistent and persua¬ sive interpretation of these three uni¬ lateral left-sided signs. They ex¬ plained them as a manifestation of disconnection of the left hemisphere language zones from the right hemi¬ sphere due to callosal destruction. Thus the left-sided apraxia to verbal command is a result of disconnection of the verbal order from the right motor cortex. The left-sided agraphia is not simply a partial phenomenon of apraxia, but a sign of disturbed flow of linguistic information from the left hemisphere to the right. The leftsided astereognosis is not an agnosia, Downloaded From: http://archneur.jamanetwork.com/ by a University of Pittsburgh User on 06/19/2015 but a failure to name resulting from failure of tactile information to be transmitted from the right to the left hemisphere. The hypothesis based on Geschwind and Kaplan's case of left anterior cerebral artery infarction associated with a left frontal tumor has since been confirmed in patients with callosal section,12"1"1 with tumor,"1 and after trauma.17 We report a case of the left anterior cerebral artery syndrome with leftsided agraphia and left-sided tactile naming deficit but without left-sided apraxia. Agraphia not accompanied by apraxia has not so far been reported in the anterior cerebral artery syndrome. In addition, the writing behavior observed in this Japanese patient handling Kanji (ideogram) and Kana (phonogram) was aphasie in nature and thus pre¬ sented further evidence that the leftsided writing disorder has definite linguistic abnormalities. REPORT OF A CASE The patient was a 53-year-old righthanded businessman. He had been hyper¬ tensive for 18 years. There was diminished hearing and tinnitus in the right ear, resulting from acoustic trauma in child¬ hood. For the past ten years, he had had transient episodes of nausea accompanied by transverse streaming of visual images lasting about 30 minutes and occurring once or twice a month. In December 1976, he experienced sudden loss of power of the right half of the body, which cleared rap¬ idly. There was no loss of consciousness. In July 1977, he experienced general weak¬ ness for several minutes. The next day, weakness of the right side appeared. He has continued to drag the right leg since. In the early morning of Dec 14th, 1977, brief painful clonic convulsion of the right leg occurred. Standing was impossible because of weakness of the right leg; in the after- noon the right arm became weak. The right hemiparesis soon worsened and he became dysarthric. Drooling from the right corner of the mouth and inappropriate laughing were noted. He was admitted to the Department of Neurosurgery, Kobe Uni¬ versity School of Medicine, on Jan 11, 1978. On Jan 24, 1978, detailed neuropsycho¬ logical testing was performed. The patient was observed closely for two weeks until his discharge, but it was not possible to perform subsequent examinations. The patient was attentive, cooperative, and oriented to time, place, and person. Digit span was five forward. Recent mem¬ ory was not impaired. Speech compre¬ hension, naming, and repetition were with¬ in normal range but with slight slurring and hesitancy. There were no paraphasic or grammatical errors. Reading and calcula¬ tion were good. Serial sevens were per¬ formed without error. Copying of a cube and drawing of a human face were done well with either hand. No apraxia to verbal command was detected in buccofacial, limb, and whole body movements, or in object handling. He could imitate with eyes closed and with either hand postures imposed on the fingers of the other hand by the examiner. There was right-sided weakness with predominant involvement of the lower extremity, which was almost immobile. In the right upper extremity, there was pre¬ dominantly proximal weakness, with the hand least affected. The right hand showed a strong grasp response and he had diffi¬ culty releasing objects placed in that hand. Often he had to release the grasp byextending each finger with the help of the left hand. Muscle stretch reflexes were hyperactive on the right with a Babinski sign. Sensation to touch and pain was slightly impaired in the right lower extremity (30% decreased, by the patient's description), but there was no loss in the upper extremity. Position sense of both toes and fingers was uncertain and often incorrect on the right side. Two-point dis¬ crimination was normal in both hands. Point localization was intact on the right side. In the left arm and hand, there was wandering of the pointing right index fin¬ ger, although it reached the correct spot eventually. Double simultaneous stimula¬ tion was correctly perceived on both sides. Vibration sense was reduced slightly on the right side. Cranial nerve functions were intact except for slight right central facial weakness. Details of specific find¬ ings are described below. SPECIAL EXAMINATION RESULTS Writing Despite the mild paresis and strong grasp reflex, the right hand made no linguistic errors in spontaneous writ¬ ing or writing to dictation. There was a tendency for letters to become smaller and for the space between the letters to get narrower as the patient continued to write. In contrast, writing with the left hand was markedly impaired. There were many unintelligible letters. Some letters could be made out but were not graphically correct because of added or missed strokes. Also, graphically correct letters were ar¬ ranged in wrong order, substituted for by different letters, or perseverated. There was a definite tendency for Kanji (ideograms or logograms, nonphonetic representations of word meaning) to be correctly produced more often than Kana (phonograms, words written phonetically). For in¬ stance, an ideogram kumo (cloud) was written correctly, but the two phono¬ grams of ku and mo were written incorrectly. (Note: Substantive words such as kumo can be written either f Fig 1.—Kanji writing. Right, Correct writing by right hand of Kanji kumo. Left, Writing by left hand of Kanji "thunder" instead of "cloud" (kumo). Second attempt by left hand (center) was correct. way.) He wrote a complex Kanji graphem of kumo correctly after he had written a different character, "thun¬ der," which resembles the former character in shape and meaning. On the other hand, when he was asked to write these characters in Kana he produced two unintelligible Kana-like patterns and two correct Kana mo and ku (ie, in reversed order). Copying of letters and sentences was correctly performed with either hand, although left-handed copying was very clumsy. Written Calculation Written calculations were tested using both hands. Unilateral (left hand) acalculia was present. For example, multiplication of 126 x 41 was correctly performed with the right hand. However, the left hand produced many perseverative Is and 6s and could not produce a correct answer. The patient kept saying that he could not help producing "6." It seemed that incorrect production of digits by the left hand made a correct operation impossible. Tactile Object Identification Objects were placed in either hand for identification. The patient was blindfolded. The right hand could not manipulate objects, which usually remained tightly held in the location in which they had been placed by the examiner. Naming of objects placed in this hand, however, was prompt and correct without exception. In contrast, the left hand could manipulate an object appropriately and adroitly. But naming of objects held in this hand was slow, hesitant, and often incor¬ rect; 52 objects were presented over three sessions, with 38 correct re¬ sponses and 13 incorrect. Incorrect answers were characterized by per- Downloaded From: http://archneur.jamanetwork.com/ by a University of Pittsburgh User on 06/19/2015 Fig 2.—Kana writing. Right column in descending order, ku and mo were correctly written by right hand. Left column in descending order, unintelligible character, correct mo, correct ku, and unintelligible character were produced by left hand. severation of the same name or by gross confabulation. For example, "toothbrush" was cor¬ rectly used on the first occasion and was then used for such dissimilar objects as scissors, pencil, and wire. The patient described a handkerchief as "something like a toothbrush," or "something is attached on top." When naming was incorrect, the patient was given the opportunity to choose the object from five objects using the same left hand. With vision shielded, this was easily done, demon¬ strating good preservation of stereognostic capacity. Graphesthesia Arabic numerals and Kana letters were traced on the palms of both hands for naming. Surprisingly, all numerals written on the left hand were identified correctly and prompt¬ ly. However, the patient showed defi- Fig 7—Left carotid arteriogram demon¬ strates occlusion of left pericallosal artery (white arrowhead). Fig 5.—Computerized tomography scan with contrast enhancement demonstrates a marked high-density area along medial aspect of left hemisphere, surrounded by low-density area. Fig 3.—Copying with left hand. Characters clumsily but are correct in their essential pattern. First and third lines are are written model sentences; second and fourth lines show patient's performance. Fig 4.—Right, correctly performed multipli¬ cation by right hand. Left, unsuccessful performance by left hand. Note perseverative tendency. nite difficulty in naming numerals written on the right palm; he was slow and hesitant in response and very often stated that he did not know the answer. Of 20 digits tested on the two occasions, only five were correct. The· pattern of failure is a reversal of that seen on the test of tactile naming, but it should be noted that there was not a single perseveratory or confabulatory in contrast to what was observed in the naming test. In reading of Kana no such distinct differences between the two hands were observed, but those Kana pre¬ sented on the left palm tended to be identified more frequently than ones on the right. Of eight Kana written on each palm, none was named on the right, while four were correctly named on the left. Again, there were no perseverative, confabulatory, or paraphasic errors. Kanji was not tested error, Fig 6.—Schematic representation of Fig 5. Hatched area represents extent of infarc¬ tion. because of graphic complexity. Thus, the patient showed poor per¬ formance of the right hand in identi¬ fying Kana and numbers. But the lack of aphasie features in the errors seems to distinguish this performance from the poor naming of objects held in the left hand. This is very probably due to impairment of the discriminative sense of the right hand and may be attributable to a possible left parietal lobe lesion. Computerized Tomography Computerized tomography (CT) low-density area in the parasagittal region of the left hemi¬ sphere. The lesion showed enhance¬ scan showed a ment with contrast. There was no deviation of the ventricular system. The lesion most probably involved part of the cingulate gyrus, the paracentral lobule, and the precuneus of Downloaded From: http://archneur.jamanetwork.com/ by a University of Pittsburgh User on 06/19/2015 the left hemisphere. The state of the corpus callosum could not be discerned from the CT scan but it is possible that the structure was involved. A left carotid arteriogram showed diffuse sclerotic change of the intra¬ cranial arteries. The anterior cerebral artery showed stenosis of a modest degree at its origin from the internal carotid artery, and the pericallosal artery was occluded just after the callosomarginal artery branched off. A right brachial arteriogram showed the same diffuse sclerotic change of the intracranial arteries as the other side. The right anterior cerebral artery was not visualized from its origin. The peripheral portion of the right anterior cerebral artery territo¬ ry was partially visualized by collater¬ als from the middle cerebral artery. It was concluded from these find¬ ings that the lesion responsible for the symptomatology of the present case was produced by infarction of the territory supplied by the left pericallo¬ sal artery. Other areas of the brain intact, despite sclerotic change of the intracranial arteries. were COMMENT Foix and Hillemand4 classified the anterior cerebral artery syndrome into three subtypes: simple crural monoplegia, hemiplegia with predom¬ inant crural involvement, and crural monoplegia (or predominantly crural hemiplegia) with left-sided unilateral ideomotor apraxia. The third type, which has a callosal lesion, is most relevant to the present discussion, although apraxia was absent in our case. Reported cases of this type of anterior cerebral artery syndrome are few. Foix and Hillemand collected three autopsy verified cases of the third type-71" and added another of their own. Critchley in 1930 added two more cases from the literature.1 But reports of such cases have almost dis¬ appeared from the literature since then. Only Sweet11' reported a case of aneurysm of the anterior cerebral artery with this type of symptoms. In the 1960s, interest was revived by Geschwind and Kaplan,1"·" but even after their work, reports of the syn¬ drome have been very rare. The case reported by Balaceanu et al in 1970-" is perhaps the only one. The left-sided agraphia in the pres¬ ent case had some interesting fea¬ tures that were manifested by clum¬ siness, production of occasional unin¬ telligible scribbles, substitution of letters, displacement of letter order, neologistic letter formation, and su¬ perior performance of Kanji writing over Kana. The patient could also copy letters well. These symptoms cannot be explained as a partial symptom of apraxia, as has been repeatedly attempted, because the present case had no accompanying ideomotor apraxia of the left side. On the other hand, all of the features enumerated are essentially the same as those usually observed in agraphia of apha¬ sie patients. Dissociation of Kanji and Kana writing of this kind is a charac¬ teristic phenomenon found in some types of Japanese aphasies.-1"' This finding is in accordance with Gesch¬ wind and Kaplan's observation10 as well as Bogen 's.14 This type of agraphia is aphasie in nature but cannot be identified with aphasie agraphia. In our patient, the left hemisphere speech zone was not affected and aphasia in the strict sense was not present. The essential mechanism lay in the failure of trans¬ mission of the programs of writing from the left speech hemisphere to the right hemisphere across the corpus callosum. Thus, as Geschwind sug¬ gested,11 it would be appropriate to call this type of agraphia "disconnec¬ tion agraphia," to emphasize the dis¬ sociative nature of the disturbance. Apart from writing, the patient responded well and without signs of apraxia to various tasks involving the left hand. Why should this happen? According to Geschwind,11'-3 callosal fibers responsible for praxis probably arise from the motor association cortex of one hemisphere and termi¬ nate in the same area of the other hemisphere. These fibers are concen¬ trated in the anterior portion of the corpus callosum. The absence of leftsided apraxia in our patient might be due to sparing of the anteriormost part of the callosum, since the portion of the pericallosal artery thought to serve this region was visualized, as already mentioned. Sugishita and oth¬ ers24 reported a remarkable case in relation to the independence of left agraphia from left ideomotor apraxia. Their patient underwent an operation for an arteriovenous malformation of the corpus callosum. Part of the poste¬ rior half of the trunk of the callosum was coagulated, and about 2 cm was subsequently sectioned. The patient was left with isolated left agraphia without other neuropsychological ab¬ normalities. The agraphia was charac¬ terized by aphasie errors only. This case and ours suggest the existence of independent functional channels of linguistic information for writing from channels of linguistic informa¬ tion for other motor performance within the corpus callosum. The difficulties in naming objects placed in the left hand observed in the present case were aphasie in nature. The linguistic nature of this left-sided difficulty was again made clear by Geschwind and Kaplan1" for the first time and has since been confirmed by many workers, especially by the observation of commissurotomized pa¬ tients1-lil5 and tumor patients.1" Balaceanu's patient also had similar diffi¬ culty.-0 In the present case as well as in these cases, tactile recognition itself was not impaired, nor was speech mechanism affected. The pa¬ tients' abnormal responses are a man¬ ifestation of failure of tactile infor- mation of the right hemisphere to reach the intact speech mechanism of the left hemisphere across the corpus callosum. Thus the left speech hemi¬ sphere tries to fill this information blank by confabulation or by repeat¬ ing a previous response (perseveration). This is an associative difficulty, a failure to name because no stimulus to naming reaches the left hemi¬ sphere. Here also "disconnection tac¬ tile naming deficit" would be a more accurate term. In summary, our patient apparently had a purely linguistic disconnection syndrome that was represented by difficulty of the left hand in coordi¬ nating linguistic information. This disconnection agraphia and disconnec¬ tion naming deficit can be explained as a result of a callosal lesion that disturbs the flow of linguistic infor¬ mation from the left hemisphere speech area to the right hemisphere. Transfer of other motor, auditory, and visual information between the two hemispheres seemed to be grossly undisturbed. Norman Geschwind, MD, Department of Neu¬ rology, Harvard Medical School, aided us consid¬ erably in highlighting the issues involved and in correcting the text. We are extremely grateful for his invaluable help. References 1. Critchley M: The anterior cerebral artery and its syndromes. Brain 53:120-165, 1930. 2. Liepmann H, Maas O: Fall von linksseitiger Agraphie und Apraxie bei rechtseitiger L\l=a"\hmung. J Psychol Neurol 10:214-227, 1907. 3. Liepmann H: Die linke Hemisph\l=a"\reund das Handeln, in Drei Aufs\l=a"\tzeaus dem Apraxiegebiet. Berlin, S Karger, 1908. 4. Foix C, Hillemand P: Les syndromes de l'art\l=e'\rec\l=e'\r\l=e'\braleant\l=e'\rieure.Enc\l=e'\phale20:209\x=req-\ 232, 1925. 5. Van Vleuten CF: Linksseitige motorische Apraxie. Allg Z Psychiatr 64:203-239, 1907. 6. Hartmann F: Beitr\l=a"\gezur Apraxielehre. Monatsschr Psychiatr Neurol 21:97-118, 248-270, 1907. 7. Goldstein K: Zur Lehre von der motorischen Apraxie. J Psychol Neurol 11:169-187, 270-283, 1908. 8. Goldstein K: Der makroskopische Hirnbefund in meinem Falle von linksseitiger motorischer Apraxie. Neurol Centralbl 28:898-906, 1909. 9. Hoff F: Balkentumor mit linksseitiger Astereognosie und Apraxie. Dtsch Z Nervenheilkd 123:89-100, 1931. 10. Geschwind N, Kaplan E: A human cerebral deconnection syndrome. Neurology 12:675-685, 1962. 11. Geschwind N: Disconnexion syndromes in animals and man. Brain 88:237-294, 585-644, 1965. 12. Gazzaniga MS, Bogen JE, Sperry RW: Some functional effects of sectioning the cerebral commissures in man. Proc Natl Acad Sci 48:1765-1769, 1962. 13. Gazzaniga MS, Sperry RW: Language after section of the cerebral commissures. Brain 90:131-148, 1967. 14. Bogen JE: The other side of the brain: 1. Dysgraphia and dyscopia following cerebral Downloaded From: http://archneur.jamanetwork.com/ by a University of Pittsburgh User on 06/19/2015 commissurotomy. Bull Los Angeles Neurol Soc 34:73-105, 1969. 15. Sperry RW, Gazzaniga MS, Bogen JE: Interhemispheric relationships: The neocortical commissures; syndromes of hemisphere diaconnection, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam, North Holland Publishing Co, 1969, vol 4, pp 273-290. 16. Brion S, Jedynak CP: Troubles du transfert interh\l=e'\misph\l=e'\rique(callosal disconnection). Rev Neurol 126:257-266, 1972. 17. Rubens AB, Geschwind N, Mahowald MW, et al: Posttraumatic cerebral hemispheric disconnection syndrome. Arch Neurol 34:750-755, 1977. 18. Claude H, Loyez M: Etude anatomique d'un d'apraxie avec h\l=e'\mipl\l=e'\giedroite et c\l=e'\cit\l=e'\ verbale. Enc\l=e'\phale8:289-307, 1913. 19. Sweet WH: Seeping intracranial aneurysm simulating neoplasm. Arch Neurol Psychiatr 45:86-104, 1941. 20. Balaceanu C, Gheorghiu M, Lupu A, et al: Syndrome de dysconnexion interh\l=e'\misph\l=e'\rique dans un cas de thrombose du tronc art\l=e'\riel brachio-c\l=e'\phalique.Rev Neurol 123:44-48, 1970. 21. Sasanuma S, Fujimura O: Selective impairment of phonetic and non-phonetic transcription of words in Japanese aphasic patients: Kana vs kanji in visual recognition and writing. Cortex 7:1-18, 1970. 22. Sasanuma S, Fujimura O: An analysis of writing errors in Japanese aphasic patients: Kanji versus kana words. Cortex 8:265-282, 1972. 23. Geschwind N: The apraxias: Neural mechanisms of disorders of learned movement. Am Sci 63:188-195, 1976. 24. Sugishita M, Yamada R, Yoshioka M: Agraphia of the left hand after section of the posterior half of the truncus of the corpus callosum. Clin Neurol 15:218-225, 1975. cas