BALINT’S SYNDROME IN A MAN WITH BORDER-ZONE INFARCTS CAUSED BY ATRIAL FIBRILLATION A. HIJDRA* andJ.D. MEERWALDP INTRODUCTION SUMMARY Hypotension is an uncommon cause of stroke and transient ischaemic attacks (TIA’s).‘+~ Border-zone infarcts may occur during (cardiac) surgery3, due to the combined effects of hypotension and loss of autoregulation from anaesthetic agents.* Sometimes this type of infarct is caused by acute and severe h~otension from other causes5+j, such as cardiac arrest, myocardial infarction, sepsis and adverse reactions to intravenously administered contrast agents, but to our knowledge it has never been reported in relation with the transient fall in blood pressure sometimes associated with the onset of cardiac tachy-arrhythmias.7 If, in such cases, there is no severe stenosis of the neck arteries and the circle of Willis is normal, the ischaemia is the most severe in the parieto-occipital border-zones between the posterior and the anterior circulatiom5 and is always combined with global cerebral ischaemia leading to unconsciousness.1,4 We report a patient with atria1 fibrillation who developed bilateral posterior border-zone infarcts without losing consciousness. Among other signs he showed the triad of simultanagnosia, optic ataxia and defective fixation of objects in the periphery of the visual fields, known as Balint’s syndrome, which disappeared with recovery from the left-hemisphere damage while there were still typical righthemisphere defects in spatial perception. A 70-year-old man showed Balint’s syndrome and other signs of bilateral posterior-hemisphere dysfunction of acute onset without initial unconsciousness. A computerized tomographic scan showed bilateral infarcts in the posterior arterial border-zone areas. Clinical evidence suggests that hypotension at the onset of atria1 fibrillation caused these infarcts. There was a marked difference in the time course of recovery between the left and the right infarct which demonstrated that the elements of Balint’s syndrome disappeared together with the left-hemisphere symptoms while the right-hemisphere symptoms were still present. words: Cerebrovascular Key hypotension, atria1 fibrillation. accidents, CASE REPORT A 70-year-old right-handed man had experienced paroxysms of palpitations and tightness of the chest for a few years. He could sometimes end these sensations by pounding upon his breastbone, The day before admission he was found in the kitchen by his daughter. He was standing at the sink holding a teapot in his right hand, he did not speak and appeared to be “confused”. He was put to bed where he remained behaviourally responsive but mute until the next morning. He then spoke in an unintelligible manner and was taken to hospital. Department ofNeurologY,University Hospital Rotterdam-Dijkrigt, 40 Dr. Molbvaterpt’ein, 3015 GD Rotterdam, The Netheriands Present address qf.4. Hydra: Department of Neurology,Academisch Medisch Centrum, II05 AZ Amsterdam, The Netherlands Clin New-01 Neurosurg 1984: Vol. 86-l. (Accepted 15.9.83) 51 Figure 1. CT scan on admission (upper row) shows hypodensity of both temporo-parieto-occtpital border-zones which IS more pronounced 16 months later (lower row). The estimated volumes” of the infarcts on the second scan are: left 17 ml and right 35 ml. The other slices were normal. The angutation is different in the two investigations. On examination he was an alert-looking man in no obvious distress. His blood pressure was 180/ 110 mm Hg (later normal); the pulse rate was 124 beats per minute and irregular. His speech was non-fluent with paraphrasic errors; naming and comprehension of spoken language were disturbed. The visual fields were difficult to examine, which was initially attributed to comprehension problems. No other abnormalities were found. Routine la~rato~ studies, including thyroxin and cardiac enzymes, were normal. An electrocardiogram showed atria1 fibrillation with a ventricular response of 125 per minute. On echocardiography normal atria1 and ventricular dimensions were found and no valvular Doppler-haematota~hography abnormalities. demonstrated a normal flow in both carotid and vertebral arteries. A computerized tomographic temporo-parietobilateral scan showed occipital hypodense areas (Fig. 1, upper row). The patient was treated with heparin intravenously and with digoxin and phenprocoumon. The next day his dysphasia had considerably improved. He was slightly dis52 orientated in time and did not remember the first 24 hours of his illness. His speech had near-normal fluency, was without paraphrasic errors and his responses were adequate. Naming and repetition were without errors, and verbal comprehension seemed good but this could not be formally tested due to other disturbances. He had reading and writing disturbances of both spatial and symbolic character. He could easily perform motor commands but could not draw a cube or a clock and he did not notice that his attempts at drawing were made across other drawings. He could not make calculations from memory or on paper. There was a left-right disorientation and a finger agnosia. He could not name the colours of objects shown to him and could not match coloured objects. When asked to point to certain large objects in the room (clock, vase with flowers) he scanned the room with his eyes but could not discover the objects. He could not name the letters on a Snellen chart (although he recognized them as letters), could not count fingers at whatever distance, but could name small objects at distances of 50 cm to 2 meters. If 16 DISCUSSION 0 TACTILE HORIZONTAL 0 TACTILE VERTICAL DEVIATION IN DEGREES 1 Bilateral border-zone infarcts caused by atria1 fibrillation. FOLLOW UP IN MONTHS 1 2 3 4 NUMBER OF ERRORS 18 Figure 2. Time course of disturbances in spatial perception as tested by the Rod Orientation Test (above abcissa) and the Line Orientation Test (below abcissa). Normal values have been marked with N. two objects were shown to him at the same distance, just 1 cm apart, he only saw the right one, and when asked to search for another one he scanned the visual space but did not see the left object; he did see it, however, when the right object was removed (simultanagnosia). He could not point to an object wherever held in the visual fields, could not grasp it (optic ataxia) and when asked to look at it made helpless searching saccades and then sometimes accidentally found it. Testing of the visual fields, which was possible only in a very crude manner, demonstrated extinction in the left visual field. There was no nystagmus. The saccadic eye movements were normal, spontaneously and on command, and pursuit was normal when the object was moved very slowly. No optokinetic nystagmus could be elicited. His condition gradually improved and one week after admission no abnormalities could be demonstrated on clinical examination, but spatial perception was still disturbed as shown by testing with the Rod Orientation Test8yg and the Line Orientation Test.lO The test results continued to improve and were within normal limits after two weeks, but further improvement continued for at least two months (Fig.2). A CT scan 16 months after the event showed bilateral temporo-parieto-occipital infarcts (Fig. 1, lower row). He remained free of symptoms while on digoxin and phenprocoumon. Our patient suffered bilateral focal ischaemia presumably at the onset of atria1 fibrillation (AF). Brain infarcts with AF are usually caused by arterial embolism which seems unlikely in our patient because of the simultaneous onset of bilateral lesions, the absence of valvular lesions and of atria1 enlargement, and the bilateral localization of the infarcts in the posterior border-zone areas. Strokes and TIA’s seldom occur in cardiac arrhythmias” but have been described in patients with chronic hypertension and severe carotid artery stenosis during hypotensive episodes from other causes, and ischaemia was then restricted to the vascular area of the stenotic vessel.12 Our patient had no chronic hypertension and his neck arteries were not or only moderately stenotic. Moreover, the ischaemia was restricted to the posterior border-zone areas which suggests that systemic hypotension was the main cause.5 The onset of AF can lead to a sudden and shortlasting decrease of cardiac output.’ The history of our patient strongly suggests paroxysmal AF, and we believe that the brain infarcts were caused by a sudden fall in blood pressure at the onset of AF. It is unusual for this type of ischaemia to occur without loss of consciousness due to global cerebral ischaemia.l One of the eleven patients of Adams et al.6 remained conscious during a hypotensive episode but later deteriorated and died; she had bilateral posterior border-zone infarcts, but it is not clear whether these were caused by the previous hypotension, as neurologic signs were absent while she was responsive. Fisher’s and McQuillen’s13 report does not mention the level of consciousness at onset. Rapid amelioration of Balint’s syndrome Our patient showed a great diversity of neuropsychological signs, of which the defects in visual attention, spatial perception and perceptually guided eye movements were the most striking. Another prominent feature was the dissociation between the time courses of 53 recovery from these different defects. The signs of left-hemisphere damage (aphasia, agraphia. acalculia and left-right disorientation) disappeared first and with them most of the visual and visuospatial symptoms. The disorder of spatial perception - as assessed by the Rod Orientation Test and the Line Orientation Test - was the last to disappear. This defect can be regarded as a sign of a right-posterior lesion and one of us (JDM) has shown that the rapidity of recovery after right-posterior infarction with regard to this sign is dependent on the extent of the lesioni Although such a relationship has not been demonstrated for other neurological signs, it seems reasonable to assume that the difference in volumes between our patient’s right and left infarction (35 and 17 millilitres respectively) accounts for the dissociation in recovery of the right- and the left-hemisphere signs. The syndrome of simultanagnosia, optic ataxia and defective fixation of objects in the periphery of the visual fields was first described by Balint in 190915 in a patient with bilateral parieto-occipital brain lesions, and since then bears his name. These signs can either be submerged into other signs (such as hemiparesis, aphasia, Gerstmann’s syndrome) or be only fragmentarily discernible.‘a Balint’s triad was part of the clinical spectrum of signs in our patient. but with the disappearance of the specific left-hemisphere defects it disappeared as well and only the disturbance in spatial perception remained. Though at the same time there was probably some recovery of the righthemisphere damage, this observation may be in accordance with the view that Balint’s syndrome should be regarded as a complex perceptual disorder without a unitary neuropsychological defect, always on the basis of biEateral posterior brain damage.*6,17 Many patients with this syndrome had extensive brain damage from multiple metastases, primary brain tumour or multiple infarcts caused by eclampsia or cortical vein thrombosis and in them the defects were either constant or progressive. r5-i8 In more recent reports3*1Q-‘2, however, it has been stressed that posterior arterial border-zone infarcts caused by acute hypotension (mostly during surgery) are most commonly associated with this syndrome or 54 uf its xymptoms. Many of these paticntx show rapid amelioration. though some t RENZI I:.I.AGLIONI p. SCOT~I G. Judgement of spatial orientation in patients with focal brain damage. 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