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J Neurol Neurosurg : first published as 10.1136/jnnp.45.8.719 on 1 August 1982. Downloaded from

Journal of , Neurosurgery, and Psychiatry 1982;45:719-724

Alexia and in Wernicke's

HOWARD S KIRSHNER, WANDA G WEBB From the Departments ofNeurology and Hearing and Science, Vanderbilt University School of Medicine, Nashville, Tennessee, USA

SUMMARY Three patients. with otherwise typical Wernicke' s aphasia showed consistently greater impairment of than auditory comprehension. While this resembles alexia with agraphia, the of speech, repetition, and naming underline the aphasic nature of the disorder. Together with previous reports of isolated word deafness in Wernicke's aphasia, these cases suggest a relative independence of auditory and visual language processing.

Wernicke's aphasia is traditionally characterised' 2 repetition, ano oral reading, however, distinguish as a disorder of central language processes, affecting their deficit from without agraphia and the different modalities of language input and out- link them more closely to Wernicke's aphasia. These put more or less equally. Thus reading is impaired cases add to previous evidence that the auditory and along with auditory comprehension, and , visual modalities of language may be affected to dif- like spontaneous speech, is marred by paraphasic ferent degrees in Wemicke's aphasia and suggest at Protected by copyright. substitutions. Anomia and paraphasic substitutions least a partial independence of auditory and visual in naming and repetition complete the picture. The language processing. equivalence of deficits in reading and auditory com- prehension is often explained by the concept that Methods and report of cases reading is learned by transcoding of visual language stimuli to previously acquired auditory word associ- Three aphasic patients, all victims of acute left hemisphere ations, stored in Wemicke's area.3 Disruption of the , were studied within five days after onset and again auditory association area would thus interfere not four to six weeks later. All had at least a partial high school with auditory comprehension, but also with education and good premorbid reading skills. The tests only employed are the same as those described previously.4 reading. Case I This 67-year-old right-handed male, in good In a previous communication,4 we discussed cases health except for hypertension and prior myocardial infarc- of Wemicke's aphasia with spared reading ability tion, entered the hospital after an episode of blurred vis- and compared this auditory-predominant sub-type ion, lightheadedness, nausea, and left-sided weakness. Ini- of Wernicke' s aphasia to the traditional syndrome of tial examination disclosed only a left homonymous pure word deafness. The present report concerns hemianopsia and left hemisensory deficit, both of which three cases of Wernicke's aphasia, all secondary to resolved over the next few hours. A computed tomo- ischaemic , in which reading was more graphic (CT) scan was normal. Three days later, during http://jnnp.bmj.com/ both acutely cerebral arteriography, the patient became restless and impaired than auditory comprehension, "confused". The arteriogram showed nonstenosing plaque and after partial recovery. Whereas the auditory- formation at the right carotid bifurcation; the left carotid predominant cases of Wernicke's aphasia resemble bifurcation and intracranial vessels were unremarkable. pure word deafness, this second, visual-predominant On examination the patient repetitively uttered phrases sub-type resembles the classical syndrome of pure such as "let me out, . . . let me get over". He followed no alexia with agraphia, an acquired illiteracy in which commands, called his daughter "Mama", and made many . He had a dense right homonymous reading and writing are impaired in the absence of paraphasic on October 1, 2021 by guest. other language deficits. The paraphasic productions hemianopsia but no apparent motor or sensory deficits. By of our patients in spontaneous speech, naming, the next morning he followed some simple commands but not multistep directions. He manifested almost total anomia for objects and colours. Address for reprint requests: Howard S Kirshner, MD, Depart- The patient improved steadily over the next few days. ment of Neurology, Vanderbilt University School of Medicine, Five days after onset he named eight of ten pictures, five of Nashville, Tennessee 37232, USA. six colours, and eight of ten small letters. He correctly Received 21 November 1981 and in revised form 6 March 1982. followed seven of eight commands of increasing complex- Accepted 18 March 1982 ity, either spoken or presented in printed form. On a 719 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.45.8.719 on 1 August 1982. Downloaded from

720 Kirshner, Webb matching-to-sample test battery modified from Sidman et inferior-most , along with subjacent al,5 he performed with greater than 90% accuracy in white matter.8 The patient continued to improve in speech matching single, dictated or printed small letters to printed and language, with mild paraphasia of spontaneous speech capital letter choices and in matching single words pre- and continued reading difficulty when last seen three sented in printed, dictated, or dictated, spelled form to months after onset. printed word choices. He read aloud 10/10 3-letter words. Case 2 This 69-year-old hypertensive, diabetic right- On the Boston Diagnostic Aphasia Examination handed lady presented with several episodes of left mono- (BDAE),6 performed four days after onset, the patient's cular blurred vision and one of transient left oral expression was fluent but marked by both literal and associated with "slurred" speech. Examination revealed verbal and word-finding difficulty. Confronta- right carotid and left supraclavicular bruits. No fixed tion naming showed prolonged latencies and some errors neurologic deficits were found. CT scan of the head was on pictures, body parts, and colours. Auditory comprehen- normal. Cerebral arteriography revealed tight stenosis of sion subtests showed normal responses to multistep com- the right internal carotid artery, severe plaque formation mands and complex ideational material. Repetition was and 50-60% stenosis of the left internal carotid artery, and marked by paraphasic substitutions, especially on low stenosis of the proximal left vertebral and subclavian probability phrases. On reading tests he was unable to arteries. Following the arteriogram the patient was noted comprehend passages of more than two sentences, and oral to be "confused"; on examination, her speech was rambl- reading was slow, effortful, and contaminated by paralexic ing, with numerous spontaneous paraphasic errors and errors. He could write short sentences to dictation but . She did not respond to commands or yes/no made errors on longer words. Re-examination questions. She showed no apparent appreciation of visual with the BDAE four weeks after onset showed improve- stimuli and failed to count fingers or blink to visual threat. ment in all areas, but with continued paraphasic errors in Spontaneous movements and withdrawal to pinprick were spontaneous speech, repetition, and oral reading. His most strong and symmetric in all extremities. Reflexes were significant disability remained that of alexia, with inability symmetic, with flexor plantar responses. On the following to comprehend paragraph-length material. day she remained confused but reached out to grasp

The Token Test7 was performed four weeks after onset, objects with either hand, more consistently in the left field Protected by copyright. both in the standard, auditory form and with the instruc- of vision. She continued to speak paraphasically but ex- tions presented in printed form. He correctly performed pressed some appropriate phrases, including a complaint of 94% of the auditory and 82% of the visual instructions (by . She failed to identify objects or colours. By the weighted scores). fourth day she looked about the room, recognised family A repeat CT scan (fig 1), performed 6 days after onset, members, and spoke in full sentences, though with many showed an area of low density involving predominantly the word substitutions. Over the next few days she regained left , with extension into the supramarginal the ability to name some objects but not uncommon gyrus, posterior-most superior temporal gyrus, and objects or parts of objects. Her auditory comprehension http://jnnp.bmj.com/

Fig 1 Computed tomographic (CTscan), Case 1, showing an area ofreduced attenuation, consistent with infarction in the region ofthe left angular gyrus. on October 1, 2021 by guest. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.45.8.719 on 1 August 1982. Downloaded from

Alexia and agraphia in Wernicke's aphasia 721

Fig 2 CT scan, Case 2, showing - ; _ infarction in the vicinity ofthe left i4F angular gyrus. The small area of increased density represents haemorrhagic infarction. Protected by copyright. and following of commands recovered substantially, except letters, numbers, or words to dictation. Retesting six weeks for left-right errors. In contrast, she remained almost after onset revealed almost normal auditory comprehen- entirely unable to read or write. She could not read more sion and mild, persistent dysnomia and paraphasic speech. than single words aloud, follow the simplest printed com- Reading and writing, though improving, remained severely mands, or sign her name. She was unable to perform even deficient. The Token Test, performed three weeks after simple calculations, such as "7 + 5", to copy or construct onset, showed 90% correct for auditory and 39% correct geometrical figures, or to identify left and right. She was for visual instructions. able to point to specific fingers on her own and the A second Cf scan, 18 days after onset, revealed a low examiner's hand. density area involving the left angular gyrus and subjacent Speech and language evaluations were carried out 2-3 white matter, with slight extension into the supramarginal weeks after onset. She named 16/20 capital letters, 17/20 gyrus and superior parietal lobule. The entire temporal pictures, and read aloud 19/20 picture names. In contrast, lobe appeared spared." The patient's subsequent course she could not write the names of any of ten pictures. On was one of gradual improvement, with mild paraphasic the matching-to-sample battery, she correctly matched speech and word-finding difficulty, but persistent and frus- 19/20 dictated and 8/10 printed small letters to the correct trating impairments in reading and writing. capital letters. She also matched 20/20 printed, 20/20 dic- Case 3 This 60-year-old right-handed male was previ- tated, and 19/20 dictated, spelled words to pictures. Given ously in good health except for hypertension, amputation a series of word lists differing in length and frequency of of the right leg, and a progressive left ear hearing loss. http://jnnp.bmj.com/ occurrence, she read aloud the frequent words accurately, Audiometry disclosed a moderate conductive hearing loss whether short or long, but she made frequent errors on the on the left, with only high frequency loss on the right. unfamiliar words and was totally unable to pronounce non- While hospitalised for evaluation of the hearing loss he sense trigrams. At the time of retesting six weeks after suddenly developed a right hemiparesis and aphasia, with onset, the patient named 20/20 pictures orally and 10/20 in initial descriptions of his speech as "slurred" and "nonsen- writing. On the BDAE, spontaneous speech was fluent, sical". By day 5 he manifested a clearcut fluent aphasia, with frequent literal paraphasic errors. The patient exhi- while the hemiparesis had improved to mild weakness of bited moderate dysnomia for objects, actions, colours, the right face and arm. Visual fields were intact. Bedside on October 1, 2021 by guest. numbers, and body parts. Auditory comprehension was aphasia testing six days after onset revealed fluent speech impaired only for multistep commands and complex idea- with word-searching pauses, circumlocutions, and frequent tional material. Single word and sentence repetition literal and verbal paraphasic errors. He named 8/8 objects, showed literal paraphasic errors. Oral reading was severely 3/3 parts of objects, and 10/10 pictures. He repeated sim- impaired, with marked hesitancy and both literal and ver- ple and complex phrases with few errors. Despite the hear- bal paraphasic errors. She failed to read sentences or para- ing loss he followed 8/8 spoken commands of increasing graphs for meaning and made occasional errors even in complexity and answered appropriately to yes/no and non- single word recognition. Writing ability was limited to the sense questions. In contrast, he was able to carry out or copying of single letters and numbers; she failed to write read aloud only 5/8 printed commands. Writing was J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.45.8.719 on 1 August 1982. Downloaded from

722 Kirshner, Webb limited to his signature and single, dictated words; he could fluently prior to their strokes. The greater involve- not write even short sentences to dictation. Matching was ment of reading than of auditory comprehension nearly errorless for the single letter, word, and picture persisted over time and was apparent in bedside test- items. ing, on the BDAE, and on the auditory and visual On the BDAE, performed on days 5-7, spontaneous forms of the Token Test. Cases 2 and 3 showed speech was characterised by fluent phraseology with many cliches, few meaningful nouns, paraphasic errors, and some significant agraphia as well as alexia, suggesting a neologistic jargon. Naming was mildly impaired, and repe- primary diagnosis of pure alexia with agraphia. AlR tition was accurate but contaminated by paraphasic errors. three cases, however, had persistent paraphasic Auditory comprehension was impaired only on four and speech, repetition, naming, and oral reading, linking five part commands and complex ideational material. In them more closely to Wernicke's aphasia. contrast, reading comprehension was severely impaired for The anatomic substrate of this syndrome, as more than single word items. He correctly answered only judged by the CT scans of Cases 1 and 2, involved 20% of questions on sentences and paragraphs. Oral read- the left inferior parietal region, especially the angu- ing of sentences contained many paraphasic errors. Writing lar gyrus and subjacent white matter. In Case 1 the was poor even for dictated single words and copying of words and sentences. Retesting five weeks after onset posterior-most part of the superior temporal gyrus showed improvement in spontaneous speech and auditory also appeared involved. In Case 2 the aphasia comprehension, with persistent paraphasic errors and remained as a persistent focal deficit after the reso- word-finding difficulty. Reading of sentences, both aloud lution of a more diffuse postarteriography syndrome and for comprehension, remained impaired. The Token of confusion and . Cortical blind- Test was performed 16 days after onset, by which time ness is a recognised complication of cerebral comprehension had improved in both modalities. The arteriography and may represent a direct effect of patient correctly carried out 93% of the auditory and 88% the contrast material.'2 The focal lesion seen on CT of the visual instructions. however, suggests embolisation to posterior

scan, Protected by copyright. A cT scan, performed within five hours of onset, was artery, as does normal. A repeat study was not obtained. The clinical branches of the left middle cerebral course was one of substantial improvement in all aspects of the sudden onset of the in all language. Only mildly paraphasic speech and reading three cases. difficulty remained as clinically evident deficits. While Wernicke himself assigned the auditory word association area to the posterior part of the superior temporal gyrus,9 subsequent aphasiologists Discussion have differed in its localisation. The , or supramarginal and angular gyri, has been Since its original description in 1874,9 Wernicke's included by some authors.'3 Paraphasic speech and aphasia has been considered a disorder of central impaired auditory comprehension are thus not language processes, independent of specific lan- unexpected with lesions in this vicinity. The greater guage modalities. Disruption of the auditory word involvement of reading than of auditory comprehen- association area in the superior temporal gyrus sion, however, cannot be explained by involvement (Wernicke's area) interferes with auditory proces- of the auditory association area alone, but rather sing. Reading, by the traditional formulation,39 is must reflect the importance of the angular gyrus reg- learned by the transcoding of visual language stimuli ion to reading. to previously learned auditory word associations; Dejerine,'4 in 1892, described the syndrome of http://jnnp.bmj.com/ alexia would thus also be expected with lesions in pure alexia with agraphia in a patient with an infarct this region. of the left angular gyrus. He believed the angular In a previous communication,4 we discussed cases gyrus to be a centre for visual word images, analog- of Wernicke's aphasia with poor auditory com- ous to the superior temporal gyrus as a centre for prehension but relatively spared reading. This dis- auditory word images. Wernicke,9 and more order resembles the syndrome of pure word deaf- recently Geschwind,'5 emphasised the role of the ness, except for the paraphasic speech, repetition, angular gyrus in cross-modal sensory associations. and naming. The preserved reading ability in these Instead of removing a cortical word-image centre, a on October 1, 2021 by guest. cases appears to reside in the left parietal and occipi- lesion of the angular gyrus would disrupt reading by tal lobes,4 10 or possibly even in the right hemis- disconnecting visual to auditory transcoding. By phere."I either explanation, an angular gyrus lesion would The three cases discussed here suggest a second produce alexia without affecting either auditory modality-specific deficit pattern in Wernicke's comprehension or . Dejerinel4 aphasia, that of greater impairment of reading than stated that alexia and agraphia could either accom- auditory comprehension. All three patients had pany a Wernicke's aphasia or exist in pure form. completed at least ten years of school and read Some recent authors2 16 17 have attempted to distin- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.45.8.719 on 1 August 1982. Downloaded from Alexia and agraphia in Wernicke's aphasia 723 guish alexia with agraphia from "aphasic alexia", or global manner.'92526 In Wernicke's aphasia, single the alexia associated with Wernicke's aphasia. On letters may be processed well, as seen in our cases, the other hand, both Kertesz ' and '8 noted and difficulty increases with increasing complexity of that alexia with agraphia can remain as a residual words and phrases.'6 In alexia with agraphia, some deficit after recovery from Wernicke's aphasia. Ben- authors2 27 have found letter greater than word son'9 stated that only in rare instances does alexia alexia, much like that seen in Broca's aphasia. Other with agraphia occur without any signs of aphasia. authors,'6 however, have not found letter alexia in Modem classifications of alexia have emphasised alexia with agraphia, and indeed occasional cases of three principal groups: alexia with agraphia, alexia alexia with agraphia have been reported with intact without agraphia, and alexia associated with oral spelling and comprehension of dictated, spelled aphasia.3 Alexia with agraphia has already been dis- words.2830 Performance in letter versus word read- cussed. Alexia without agraphia is an easily separ- ing would thus not appear to be a reliable factor in able syndrome seen with lesions of the left occipital distinguishing alexia with agraphia from alexia lobe, often including the splenium of the corpus cal- related to Wemicke's aphasia, though certainly it losum. Dejerinel4 explained this syndrome by a dis- does distinguish alexia without agraphia, or "post- connection of visual information perceived by the erior alexia", from the alexia associated with frontal intact right from the left angular gyrus. lesions. Inability to perceive a word as a whole is the Aphasic alexia has included different groups of most consistent deficit in "posterior" alexia, while aphasic cases. Benson'9 has called attention to alexia single letter reading and phonetic processing of in Broca's aphasia, under the term "third alexia". may be intact. In both the "anterior" and The nonfluent speech of these patients, of course, "central" alexias, on the other hand, whole word should distinguish them readily from the other alexic perception may be the only preserved function, groups. Finally, the alexia of Wemicke's aphasia while letter reading and phonetic processing are 25 may be considered as a separate, fourth group, or it impaired.'9 26 Protected by copyright. may be combined with the first syndrome, alexia Cases of Wemicke's aphasia with alexia out of with agraphia. Benson'9 has suggested the terms proportion to auditory comprehension deficit have "central alexia" for the alexia with agraphia cases, not been generally recognised, though as discussed "posterior alexia" for alexia without agraphia, and above some cases of alexia with agraphia may "anterior alexia" for the reading disorder seen with belong to this category. H6caen'6 described a subset Broca's aphasia. of Wemicke's aphasics with auditory predominant An alternative explanation for the findings of deficits, but he related this to a deficit in the percep- fluent aphasia and alexia in our patients would be a tion of . His second subgroup, with combination of and alexia with deficient semantic comprehension, was said to have agraphia. Conduction aphasia, a syndrome in which equal impairment of auditory comprehension and paraphasic speech and impaired repetition occur in reading. Mohr, Hier, and Kirshner,'0 in a retrospec- the presence of intact auditory comprehension, is tive review of CT scan lesions, found two cases of associated with lesions of either the left supramargi- Wemicke's aphasia with greater impairment of read- nal gyrus or the left superior temporal gyrus.20 Fac- ing than of auditory comprehension. Both had tors against the diagnosis of conduction aphasia in parieto-occipital lesions. This compared to six cases our patients include the presence at least initially of of auditory-predominant deficit and 23 in which no

auditory comprehension deficit, the absence of a disproportion could be judged. The present three http://jnnp.bmj.com/ severe repetition disturbance out of proportion to cases are part of an ongoing investigation of selec- other language deficits, and the absence of dispro- tive involvement of specific language modalities in portionate literal as compared to verbal paraphasic aphasia secondary to ischaemic stroke. Of 18 cases errors. For these reasons we prefer the diagnosis of of Wernicke's aphasia studied, two showed the Wemicke's aphasia with alexia. The most important auditory and three the visual-predominant syn- point, however, is the linkage of alexia with agraphia drome. Selective impairment of either auditory to the fluent . comprehension or reading thus appears to be not

Numerous studies have attempted to distinguish uncommon in Wernicke's aphasia. on October 1, 2021 by guest. alexic by their relative impairments of The independence of deficits in reading and word versus letter reading. In alexia without auditory comprehension in Wernicke's aphasia sug- agraphia, preservation of individual letter reading gests at least partial independence of the brain has been amply documented.2'-24 The alexia of mechanisms for auditory and visual language pro- Broca's aphasia, on the other hand, produces a cessing. This independence belies the simple con- major deficit in the naming and reading of single cept that reading can proceed only via transcoding letters, even when some words can be read in a of visual to phonetic information. The evidence J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.45.8.719 on 1 August 1982. Downloaded from

724 Kirshner, Webb from Wernicke' s aphasia thus confirms evidence 13 Bogen JE, Bogen GM. Wernicke's region-Where is it? from other sources31 32 that reading can occur from Ann NY Acad Sci 1976;280:834-843. purely visual to semantic processing, without access 14 Dejerine MJ. Contribution a l6tude anatomo- to auditory or phonetic information. pathologique et clinique des differenctes varietes de This research was supported by NINCDS Teacher c6cit6 verbale. CR Soc Biol (Paris) 1892;4:61-90. Award No. .s Geschwind N. Disconnexion syndromes in animals and Investigator Development man. Brain 1965;88:237-94, 585-644. 5K07NS00429-03 to Dr Kirshner and also by the 16 H6caen H, Kremin H. Neurolinguistic research on read- Veterans Administration. The authors thank Mrs ing disorders resulting from left hemisphere lesions: Anne Morgan for technical assistance and Mrs Jane aphasic and "pure" alexias. In: Whitaker H, Whitaker S Smith for assistance with preparation of the H eds; Studies in , Vol 2, New York*, manuscript. Academic Press, 1977. 17Kertesz A. Aphasia and Associated Disorders. Tax- onomy, Localization, and Recovery, New York, Grune & Stratton, 1979. 18 Brain WR. Speech Disorders. London; Butterworths, References 1962. 19 Benson DF. The third alexia. Arch Neurol 1977; lBenson DF, Geschwind N. The aphasias and related dis- 34:327-31. turbances. In: Baker AB, Baker LH eds; Clinical 20 Benson DF, Sheramata WA, Bouchard R, et al. Conduc- Neurology. Hagerstown, Harper and Row Publishers, tion aphasia. A clinicopathological study. Arch Neurol 1977, Vol 1, Chapter 8, 3-9. 1973;28:339-46. 2 Brown J. Aphasia, and . Clinical and 21 Warrington EK, Rabin P. Visual span of apprehension in Theoretical Aspects. Springfield: Charles C. Thomas, patients with unilateral cerebral lesions. Q J Exp 1972, 56-71. Psychol 1971;23:423-31. 3Benson DF. Aphasia, Alexia, and Agraphia. New York: 22 Staller J, Buchanan D, Singer M, et al. Alexia without Churchill Livingstone, 1979, 117. agraphia: An experimental case study. Brain Lang Protected by copyright. 4 Kirshner HS, Webb WG, Duncan GW. Word Deafness 1978;5:378-87. in Wernicke's Aphasia. J Neurol Neurosurg 23 Levine DN, Calvanio R. A Study of the visual defect in Psychiatry, 1981 ;44:197-201. verbal alexia-simultanagnosia. Brain 1978;101:65- 5 Sidman M, Stoddard LY, Mohr JP et al. Behavioral 81. studies of aphasia. Methods of investigation and 24 Kirshner HS, Staller J, Webb WG, Sachs P. Transtentor- analysis. Neuropsychologia 1971;9: 119-40. ial herniation with posterior cerebral artery territory 6 Goodglass H, Kaplan E. The Assessment ofAphasia and infarction: a new mechanism of the syndrome of alexia Related Disturbances. Philadelphia: Lea and Febiger, without agraphia. Stroke, 1982;13:243-6. 1972. 2S Benson DF, Brown J, Tomlinson EB. Varieties of alexia: Spreen 0, Benton A. Token Test. From the Neurosen- Word and letter blindness. Neurology (Minneap) sory Center Comprehensive Examination for 1971 ;21:951-7. Aphasia, Neuropsychiatry Laboratory, University of 26 Kirshner HS, Webb WG. Word and letter reading and Victoria, 1969. the mechanism of the third alexia. Arch Neurol Gado M, Hanaway J, Frank R. Functional anatomy of 1982;39:84-7. the by computed tomography. J Com- 27Alajouanine T, Lhermitte F, Ribaucourt-Ducarne B. put Assist Tomogr 1979;3:1-19. Les alexies agnostiques et aphasiques. In: Ala- 9 Wernicke C. The symptom complex of aphasia. A jouanine T ed, Les grandes activit6s du lobe occipital. psychological study on an anatomical basis. Proc Bos-

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