JYournal ofNeurology, Neurosurgery, and Psychiatry 1993;56:1027-1032

SHORT REPORT

"Crossed homonymous hemianopia" and"crossed left hemispatial neglect" in a case ofMarchiafava-Bignami disease

Mutsuo Kamaki, Mitsuru Kawamura, Hirobumi Moriya, Keizo Hirayama

Abstract"Crossed homonymous hemianopia"and "crossed left hemispatial neglect"were observed in a woman withMarchiafava-Bignami disease. Twoforms of "crossed homonymous hemi-anopia" were observed. Initially,Goldmann perimeter testing showed aleft homonymous hemianopia with theright hand and vice versa. Later, con-frontation tests showed a left homony-mous hemianopia, whereas visual fieldtesting using the Goldmann perimeter(kinetic quantitative perimeter) and theOCTOPUS (Interzeag AG, static auto-mated perimeter) showed a righthomonymous hemianopia with eitherhand. "Crossed left hemispatial neglect"was not seen with the left hand, butneglect of the left hemifield was seen withthe right hand. CT and MRI showed alesion occupying almost the entire cor-pus callosum. PET showed no significantdifferences between comparable areas ofthe left and right cerebral hemispheres.These findings indicate that both signs ofinterhemispheric disconnection were dueto the callosal lesion. Moreover, the"crossed left hemispatial neglect" can beexplained as being a consequence of thedominance of the right cerebral hemi-sphere for visuospatial recognition.

(J7 Neurol Neurosurg Psychiatry 1993;56: 1027-1032)

Department ofNeuropsychiatry,Tokyo MetropolitanHiroo GeneralHospital, Tokyo, JapanM KamakiH MoriyaDepartment ofNeurology, School ofMedicine, ChibaUniversity, Chiba,JapanM KawamuraK HirayamaCorrespondence to:Dr Kamaki, Department ofNeuropsychiatry, TokyoMetropolitan Hiroo GeneralHospital, 2-34-10 Ebisu,Shibuya-ku, Tokyo 150,JapanReceived 20 May 1992 andin revised form21 September 1992.Accepted 11 December 1992

"Crossed homonymous hemianopia" and"crossed left hemispatial neglect" were

observed in a case of Marchiafava-Bignamidisease (MBD). MBD is a disease of chronicalcoholics caused by demyelination of thecorpus callosum leading to symmetricalnecrosis. The subject was a 43 year oldwoman with a history of alcohol abuse inwhich the callosal lesion was clearly diag-nosed by X ray CT and MRI. BothGoldmann and OCTOPUS perimetry were

used according to their specified conditions.Use of the non-dominant hand was a modifi-cation made for this study. Initially,Goldmann perimetry showed a left homony-mous hemianopia with the right hand push-ing the switch and a right homonymoushemianopia with the left hand pushing the

switch. Subsequently, visual field testingusing instruments such as the Goldmannperimeter and the OCTOPUS static auto-mated perimeter showed a right homony-mous hemianopia with either hand pushingthe switch. "Crossed left hemispatial neglect"was seen with the right hand when copyingfigures and during Albert's line-crossing test.Neither finding has previously been reported.

Case reportA 43 year old right handed housewife wasfound unconscious at home on 19 November1987. She did not respond to her name andhad been incontinent. She was taken toTokyo Metropolitan Hiroo General Hospital.The patient had been a heavy drinker ofJapanese rice wine for 20 years. She had com-plained of coldness and pain in the tips of hertoes for three years, and had been aware ofincreasing forgetfulness over the precedingtwo months.On admission, she was delirious, her

speech was abnormal and she was unable toobey verbal commands. There were noabnormalities of the ocular fundi, the pupilswere round and isocoric, and the light reflex-es were normal. Central type left facial paresisand mildly decreased muscle strength of theleft upper and lower limbs were found.General muscle tone was mildly hypertonic.Deep tendon reflexes were brisk, but bothplantar responses were flexor. The sensorysystem could not be assessed and there wasurinary incontinence.One week after onset, her level of con-

sciousness was nearly normal, but deficits intemporal and spatial orientation remained.Her memory was still defective and herspeech was dysarthric although improved. Onconfrontation tests a left homonymous hemi-anopia was found. The muscle strength of theleft side of the face and left upper and lowerlimbs had improved, and muscle tone hadreturned to normal. Subsequently extinctionof tactile stimuli of the left hand was found.There were digital disturbances of tempera-ture and pain in all four limbs indicating apolyneuropathy. She regained control of herbladder and bowels. One month after onset,her level of consciousness was normal and shewas cooperative enought to participate inneuropsychological testing.

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NeuroradiologicalfindingOne month after onset, sagittal TI-weightedMRI (TR = 2080 ms, TI = 500 ms; PickerInternational, VISTR-MR 0 5 Tesla super-conducting magnet showed abnormal signalintensity (SI) of the entire corpus callosumexcept for the extreme anterior portion. Verylow SI was seen to extend from the genu toslightly posterior of the centre of the trunk ofthe corpus callosum, around which there wasa border region with SI somewhat lower thanthat of the normal corpus callosum. Varyingdegrees of low SI were seen throughout thetrunk and splenium (fig 1).

Positron emission tomography (PET) wasperformed three and four months after onset.The cerebral metabolic rate for oxygen(CMRO,) and cerebral blood flow (rCBF)were measured using the '50-steady statemethod. No relevant differences between thecerebral hemispheres were found.

CROSSED HOMONYMOUS HEMIANOPIATwo months after onset, a left homonymoushemianopia was noted both on confrontationtests (verbal response) and on the Goldmannperimeter with the right hand pushing theswitch (fig 2A). Four months after onset, shewas assessed using the Goldmann perimeterwith the left hand pushing the switch. A defi-nite right homonymous hemianopia wasfound; that is, both hands showed a "crossedhomonymous hemianopia" (fig 2B). Fromeight months after onset, the Goldmannperimeter test and the OCTOPUS staticautomated perimeter test indicated a righthomonymous hemianopia with either hand(fig 2C). Confrontation tests conducted atthe same time consistently indicated a lefthomonymous hemianopia so that the initialcrossed homonymous hemianopia with theleft and right hands was found to be tran-

Figure 1 Tl-weighted sagittalMRI (Repetition time 2080 ms. Inversion time 500 ms,inversion recovery technique) on the 34th day. The most anterior portion of the corpuscallosum shows normal SI, whereas the SIfrom the genu to the anterior half of the trunkshows a long and thin very low SI, around which there is a border of tissue with SIsomewhat lower than that of normal callosal tissue. In other regions, the corpus callosumshows heterogeneous low SI.

sient. Later, results of confrontation tests andthose obtained by using more sophisticatedinstruments, such as the Goldmann perimetertest, continued to be contradictory.

At seven months, when instructed to imi-tate a simple finger pattern presented withone hand ipsilaterally, no deficit (34/36 withthe right hand, 36/36 with the left hand) wasfound. She rarely failed to imitate simple fin-ger patterns presented in the left visual field-where the examiner's hand should not havebeen visible in confrontation tests. Whenrequired to imitate a contralateral finger pat-tern, she failed to imitate any pattern witheither hand.The response to placing a rod in the left

visual field was also tested. When asked if shecould see the rod, she said she could not, butwhen asked to grasp it, she was able to do soswiftly and accurately with the left hand. Shewondered why she could grasp it, despite thefact that she couldn't see it. When asked tograsp the rod in the left visual field with theright hand, she would repeatedly grope inmid-air-eventually sometimes hitting it. Incontrast, when the rod was placed in the rightvisual field, she said she could see it and hadno difficulty in grasping it with the righthand, but clearly had difficulties with the lefthand.

Four years after onset, there is no changein the pointing pattern and she is still unableto grasp a rod placed in the contralateral visu-al field. However, she says she can now seeobjects in the left visual field and is able toindicate the number of fingers displayed toher in that field.

CROSSED LEFT HEMISPATIAL NEGLECTIn Albert's line-crossing test,' there wasmarked left-sided visual neglect with the righthand, but no indication of visual neglect withthe left hand (fig 3). It was evident that therewas neglect of the left hand side when copy-ing figures with the right hand. Fig 4A showsthe patient's drawings of a Greek cross. Shedrew a winding cross with the left hand, but astraight one omitting the left part with theright hand. Fig 4B shows her drawings of twoflowers in a pot. With the right hand, theflower on the left and the petals on the left ofthe other flower were omitted, indicating leftvisual neglect. However, although drawingwith the left hand was somewhat disordered,neglect was not evident. Fig 4C shows herdrawings of a house and two trees on bothsides of it. She failed to draw the left part ofthe house. When asked to read a Chinesecharacter (kanji) out aloud, she read out onlythe right half of the character and neglectedthe left half which in many cases modifies theright half, changing its meaning. The abovefindings were observed not only when theoriginal was placed directly in front of her,but also when it was placed on her right orleft side and she was instructed to draw withher right or left hand. The results were notconsiderably different from those foundwhen the paper was placed directly in front ofher. In drawing a woman's face without a

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"Crossed homonymous hemianopia" and "crossed left hemispatial neglect" in a case ofMarchiafava-Bignami disease

A2nd month

B

4th month

C8th month

Left hand Right hand

Figure 2 Goldmann perimeter tests (2, 4 and 8 months after onset). Results on the left (right) hand side of the figure are those obtained with the left(right) hand pressing the switch. After two months, a left homonymous hemianopia isfound with the right hand pressing the switch (A), whereas afterfour months a left homonymous hemianopia is found with the right hand pressing the switch while a right homonymous hemianopia is found when the lefthand did so, that is "crossed homonymous hemianopia" (B). After eight months, a pattern similar to that after 4 months is seen with the left hand, but,with the right hand, the visualfield defect has become a right homonymous hemianopia (C).

model, unilateral neglect was not seen witheither hand (fig 5). Slight "crossed left hemi-spatial neglect" still remains four years fromonset.

Other signs of the interhemispheric discon-nection syndrome, that is, apraxia and tactileanomia of the left hand, were also observed.Agraphia of the left hand was found whenattempting to write Chinese characters andJapanese script. The nature of the mistakesmade were not due to scrawling with the non-dominant hand, but mainly due to para-graphia with perseveration. In a dichoticlistening test there was clear extinction of the

left ear. Facial recognition, identification ofoverlapping figures (Poppelreuter), colournaming, left-right discrimination, finger nam-ing and simple verbal calculation were intact.The Wechsler Adult Intelligence Scale(WAIS) three months after onset, showed averbal IQ of 73, a performance IQ below 60and total score below 60. In the WesternAphasia Battery2 one year after onset, no sig-nificant abnormalities were seen concerningher ability to write except for her occasionalrefusal to do so. Construction tasks with theright hand were moderately impaired andthose with the left hand were slightly better.

Figure 3 Albert's line-crossing test. With the lefthand, no hemispatialneglect is found. But, withthe right hand, dramatichemispatial neglect isfound.

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Figure 4 Copying modelswith the left and righthands. A: Greek cross.With the left hand, thecross is curved. With theright hand, the left part isomitted. B: Twoflowers ina pot. The drawing withthe left hand is less precise,but there is no neglect,whereas with the righthand, thefigure is copiedshowing left hemi-neglect(complete omission of theflower on the left and alsothe left-half of thefloweron the right). C: A housewith 2 trees on both sides ofit. With the right hand, theleft part of the house is notdrawn.

No other abnormalities were found and therewas no aphasia.

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Figure 5 Drawings of awoman 'sface without amodel. Even with the righthand, hemispatial neglectis not clear.

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DiscussionThere was no difficulty in diagnosingMarchiafava-Bignami disease from the historyof alcohol abuse, the pathognomonic findingson X ray CT and MRI and the various signsof the interhemispheric disconnection syn-

Right hand drome, such as apraxia, agraphia and tactileanomia of the left hand.The most characteristic feature of this case

is the crossed homonymous hemianopia.After four months, a left homonymous hemi-anopia was found using the Goldmannperimeter with the right hand pushing theswitch, but a right homonymous hemianopiawas found with the left hand. This "crossedhomonymous hemianopia" can be explained

y9g by a failure of unilaterally-presented visualinformation travelling across the corpus callo-

(* M~'~ sum to the contralateral motor system, to ini-tiate a response. After eight months,

.i9 confrontation tests indicated a left homony-mous hemianopia, whereas the tests usinginstruments such as the Goldmann kinetic

Right hand perimeter or OCTOPUS static perimeterwith either hand indicated a right homony-mous hemianopia. These results may havebeen due to different levels of hemisphericactivation by the two kinds of tests. That is,in tests requiring verbal responses, the lefthemisphere may be more highly activated,whereas in tests requiring only the tracking ofa simple light source, the right hemispheremay be more highly activated. This mightthen explain why two distinct forms ofcrossed homonymous hemianopia wereobserved. When the crossed homonymoushemianopia was initially found, there mayhave been a complete lack of interhemispher-ic communication and the results would have

tight hand reflected the functions of the isolated left andright hemispheres. The crossed homonymoushemianopia observed later would then havebeen a result of the different hemispheric acti-vation induced by the different testing meth-ods. After four years, the left homonymoushemianopia observed on confrontation andthe right homonymous hemianopia observedwith Goldmann perimeter testing are bothless distinct, suggesting that there has beensome recovery of interhemispheric communi-cation, and that the crossed homonymoushemianopia will continue to improve. Despitethe fact that for three years the patient saidshe "could not see" objects in the left visualfield, she could readily and accurately graspthem with the left hand, and she was able toimitate finger patterns presented by the exam-iner to the left visual field with the left hand.From these results, we believe that thispatient is neither able to verbalise objects

L , seen in the left visual field nor is she con-scious of them, in spite of the fact that thereis essentially no disturbance of her visualfield.We have been unable to find any previous

Right hand reports of crossed homonymous hemianopia

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"Crossed homonymous hemianopia" and "crossed left hemispatial neglect" in a case ofMarchiafava-Bignami disease

detected by visual field tests using theGoldmann perimeter or comparable instru-ments, but several related syndromes havebeen reported. Brion and Jedynak3 reported acase of a callosal vascular lesion, due to a leftparacallosal angioma fed by the posteriorcerebral artery, showing a so-called "pseudo-hemianopsie". Notably, they found that whentwo objects were placed one each in the leftand right visual fields, the object on the leftwas ignored. The presence of the stimulus onthe left was sometimes denied, but theirpatient could readily and, simultaneously,grasp the objects with the left and right handsrespectively, indicating that the object in theleft visual field was seen. Our patient consis-tently denied that she could see the object inthe left visual field, but was easily able tograsp it. This phenomenon was not due toextinction. Lhermitte et a! 4 reported threecases of suspected MBD, in which verbalresponses could not be elicited following thepresentation of a stimulus in the left visualfield or placement of an object in the lefthand, but when instructed to raise the lefthand to indicate a response, the instructionwas obeyed. They called this syndrome"pseudo-extinction", because it differed fromthe case reported by Brion and Jedynak3which involved two stimuli in separate fields.However, Lhermitte's case and our case aresimilar as correct responses could be elicitedby non-verbal means. Neither of thesereports, however, mentions crossed visualfield deficits. In a study of a case of MBD atnecropsy, Lechevalier et al I reported a"crossed avoiding reaction", in which a handwould actively avoid an object placed in thecontralateral field. Their case was, however,different from our case as no abnormalitieson the Goldmann perimeter test could befound. Bogen6 noted "double hemianopia" asone of the signs of the IDS. He described thissyndrome as the ability of the subject sittingopposite the examiner to point to the examin-er's finger when it was placed individually inthe visual field of the subject's pointing hand,but the inability to point if the examiner's fin-ger was placed in the visual field contralateralto the subject's pointing hand; at which timethe subject would completely ignore it, as inhomonymous hemianopia. Moreover, whenthe examiner used both hands in both visualfields, the subject was able to point simulta-neously to both fingers. Similar results wereobtained in this case using the same examina-tion technique, but such results leave unclearwhat differences there may be with callosalcrossed visual ataxia. Bogen6 referred to thissyndrome as being similar to homonymoushemianopia, but he reported neither theresults of confrontation tests nor the results ofGoldmann perimeter tests. Irrespective of thefact that she could not see objects in the lefthemifield, her ability to grasp each objectaccurately resembles cases of blindsightincluding the DB case described byWeiskrantz.7One of the most notable characteristics of

our case is that, despite an absence of neglect

when copying simple pictures, figures orChinese characters with the left hand, there isa notable left hemispatial neglect with theright hand. Even in Albert's line-crossing test'with either hand, only the right hand showedclear left hemispatial neglect. When readingChinese characters, she omitted the left partentirely and read only the right half of thosecharacters whose right halves could be read asindependent entities. Neither hand showedabnormalities during spontaneous drawing.These findings suggest that the deficit is inthe process of visual input. This "crossed lefthemispatial neglect" is not mentioned in neu-ropsychology texts89 or in reviews of inter-hemispheric disconnection syndrome.610However, similar signs were reported by Sineet al," in a case of left parasagittal frontopari-etal haemorrhage with spasm of the anteriorcerebral artery and by Goldenberg et al 1213 ina case of pericallosal haemorrhage of theanterior two thirds of the corpus callosum,both cingulate gyri and the white matterunderlying both medial frontal lobes sec-ondary to bleeding from an aneurysm of theanterior cerebral artery. Recently, Kashiwagiet al 14 noted a similar case secondary toinfarction of the anterior cerebral artery.Furthermore, Costello and Warrington'5reported both a right-sided visuospatialneglect and left-sided neglect dyslexia (mis-reading the beginning of a word) in a case oflymphoma occupying mainly the left occipitallobe including the splenium.

"Crossed left hemispatial neglect" can beexplained as follows: in our case, neitherhemispace is neglected in copying with theleft hand, although the right hemispherealone is functioning; in copying with the righthand, however, attention is paid exclusivelyto the contralateral right hemispace and theipsilateral left hemispace is neglected becauseonly the left hemisphere which is disconnect-ed from the right, is functioning. "Crossedleft hemispatial neglect" has not previouslydrawn attention as one of the signs of theinterhemispheric disconnection syndrome.

In our case, PET was performed. No sig-nificant lateralisation, however, was found interms of either rCBF or oxygen metabolicrate. PET and MRI results also indicate thatthe "crossed homonymous hemianopia" and"crossed left hemispatial neglect" observedare not due to lesions of the cerebral hemi-spheres.

1 Albert ML. A simple test of visual neglect. Neurology1973;23:658-64.

2 Kertesz A. The Western Aphasia Battery. New York:Grune and Stratton, 1982.

3 Brion S, Jedynak CP. Troubles du transfert interhemis-pherique (callosal disconnection). A propos de troisobservations de tumeurs du corps calleux. Le signe de lamain etrangere. Rev Neurol (Paris) 1972;126:257-66.

4 Lhermitte F. Marteau R, Serdaru M, Chedru F. Signs ofinterhemispheric disconnection in Marchiafava-Bignamidisease. Arch Neurol 1977;34:254.

5 Lechevalier B, Andersson JC, Morin P. Hemispheric dis-connection syndrome with a 'crossed avoiding' reactionin a case of Marchiafava-Bignami disease. J NeurolNeurosurg Psychiatry 1977;40:483-97.

6 Bogen JE. The callosal syndrome. In: Heilman KM,Valenstein E, eds. Clinical neuropsychology. Oxford:Oxford University Press 1979:308-59.

7 Weiskrantz L. Blindsight - a case study and implications.

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Oxford: Oxford University Press 1990:20-46.8 Hecaen H, Albert ML. Disorders of visual perception. In:

Hecaen H, Albert ML, eds. Human Neuropsychology.New York: John Wiley, 1978:217-24.

9 Heilman KM, Valenstein E, Watson RT. The neglect syn-drome. In: Frederiks JAM, eds. Handbook of clinicalneurology, Vol 1 (45): Clinical neuropsychology.Amsterdam: Elsevier Science 1985:153-83.

10 Sperry RW, Gazzaniga MS, Bogen JE. Interhemisphericrelationships: the neocortical commissures; syndromesof hemisphere disconnection. In: Vinken PJ, BruynGW, eds. Handbook of clinical neurology, Vol 4.Amsterdam: North-Holland 1969:273-90.

11 Sine RD, Soufi A, Sham M. Callosal syndrome:

Implications for understanding the neuropsychology ofstroke. Arch Phys Med Rehabil 1984;65:606-10.

12 Goldenberg G, Wimmer A, Holzner F, Wessely P.Apraxia of the left limbs in a case of callosal disconnec-tion: The contribution of medial frontal lobe damage.Cortex 1985;21:135-48.

13 Goldenberg G. Neglect in a patient with partial callosaldisconnection. Neuropsychologia 1986;24:397-403.

14 Kashiwagi A, Kashiwagi T, Nishikawa T, Tanabe H,Okuda J. Hemispatial neglect in a patient with callosalinfarction. Brain 1990;113:1005-23.

15 Costello A, Warrington EK. The dissociation of visuo-spatial neglect dyslexia. Neurol Neurosurg Psychiatry1987;50:1110-6.

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