Ettlinger Revisited: the Relation Between Agnosia J Neurol Neurosurg Psychiatry: First Published As 10.1136/Jnnp.58.3.350 on 1 March 1995
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35030ournal ofNeurology, Neurosurgery, and Psychiatry 1995;58:350-356 Ettlinger revisited: the relation between agnosia J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.3.350 on 1 March 1995. Downloaded from and sensory impairment Edward H F De Haan, Charles A Heywood, Andrew W Young, Nicki Edelstyn, Freda Newcombe Abstract tinction between apperceptive and associative The concept of agnosia as a higher order "Seelenblindheit", where the first results from functional impairment, which can occur visuosensory deficits and the second was in the absence of low level visual percep- thought to represent a difficulty in associating tual deficits, continues to provoke debate. meaning with a relatively intact percept. A few This controversy is complicated by the years later, Freud4 coined the term "agnosia" fact that, on close examination, agnosic to describe these recognition disturbances. It patients do tend to have some perceptual is the viability of the concept of associative difficulties. Thus the issue centres agnosia or as Teuber5 put it "a normal per- around the question as to whether these cept stripped of its meaning"-that has been deficits play a causal part in the aetiology questioned. Notably, Bay67 claimed that the of agnosia or whether they are function- socalled higher order recognition deficits are ally independent, with both impairments secondary to sensory impairment, general resulting from the substantial cerebral intellectual loss, language problems, or a com- lesions involved in agnosia. In 1956, bination of these factors. Ettlinger published a study' in which he More recent investigations have convinc- compared the performance of patients ingly shown that visual recognition disorders with visual recognition deficits and can occur in patients with normal or even patients with posterior brain lesions above average intelligence.8 10 Also, it has now whose recognition abilities were intact. been clearly established that language difficul- He argued that visual perceptual prob- ties are not instrumental in causing associative lems could not explain the recognition agnosias.I112 The question whether subtle sen- deficit in agnosia as he saw far worse per- sory impairment (Bay's "Funktionswandel"), ceptual impairments in patients who did or a certain constellation of sensory deficits, not experience any problems in visual can produce the clinical symptoms of associa- recognition. Although the logic of tive agnosia, however, remains controversial. Ettlinger's argument is not disputed, The debate is fuelled by the fact that even the some criticisms have arisen concerning most pure cases of agnosia often show some Russell-Cairns Unit, the study, such as the fact that his experi- mild problems on tasks of visual perception. Radcliffe Infirmary, In a seminal study, Ettlinger' set out to test http://jnnp.bmj.com/ Woodstock Road, mental group did not include a truly Oxford OX2 6HE, UK object agnosic patient. In addition, Bay's hypothesis. He carried out a careful E H F De Haan Ettlinger's visual-sensory assessment can assessment of sensory status in patients with N Edelstyn no longer be considered comprehensive and patients without recognition deficits, and F Newcombe in the light of present day knowledge of he argued that sensory status alone could not Psychology Department, Durham the cerebral visual apparatus. This study explain the presence or absence of recognition University, Science therefore investigated three disorder. The crux of his argument was that Laboratories, Durham with a deficit (prosop)agnosic patients and five although patients recognition on October 1, 2021 by guest. Protected copyright. DH1 3LE, UK have other C A Heywood patients with unilateral brain lesions may sensory impairments, patients on an exten- who do not experience recognition problems MRC Applied without recognition deficits Psychology Unit, 15 sive battery of visual sensory tests. The can show equal or worse impairments on the Chaucer Road, results support Ettlinger's original claim sensory tests. Cambridge CB2 2EF, that some cases) agnosia cannot be Recently, Campion'3 has reopened the dis- UK (in A W Young explained as resulting from lower level cussion, pointing out that Ettlinger's study include a Department of visual impairments. did not truly object agnosic patient, Psychonomics, and that Ettlinger's tests might not have cov- Utrecht University, (7NeurolNeurosurg Psychiatry 1995;58:350-356) ered all the relevant sensory abilities for visual Heidelberglaan 2, recognition. It is noted, however, that one Utrecht, The Netherlands patient in Ettlinger's population was fully E H F De Haan Keywords: agnosia; recognition deficits; lower level prosopagnosic (see Pallis14). Although visual impairments; visual sensory tests prosopagnosia the inability to recognise Correspondence to: familiar faces'- is often considered function- Dr Edward De Haan, Psychological Laboratory, Visual recognition disorders as a result of ally separate from object agnosia,'6 17 the Utrecht University, brain disease or injury have created theoretical dichotomy of apperceptive v associative is still Heidelberglaan 2, 3584 CS Utrecht, The Netherlands. controversies since they were first described in applicable. Therefore, we suggest that there is Received 25 April 1994 and the second half of the 19th century.2 a prima facie case that Ettlinger's line of rea- in final revised form Regarding the more selective impairment of soning also applies to object agnosia. 2 August 1994. Accepted 5 August 1994 object recognition, Lissauer3 suggested a dis- Campion's second criticism is more serious, Ettlinger revisited: the relation between agnosia and sensory impairment 351 as with hindsight Ettlinger did not test for all name or the voice. His visual recognition J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.3.350 on 1 March 1995. Downloaded from sensory abilities that later knowledge has problems were not confined to faces. Other shown might possibly affect visual recogni- types of within class recognition," such as for tion. Also, Ettlinger used a composite sensory cars or flowers, were very poor, and on object score, and did not look for subtle problems on naming tasks he showed a mild degree of specific sensory abilities or constellations of object agnosia. The severity of his impairment sensory impairments. of face recognition is well illustrated by his Recent advances in neuropsychological and performance on a forced choice task in which neurophysiological understanding of the visu- he was required to choose which one of two osensory apparatus have put us in a much bet- simultaneously presented faces looked famil- ter position to consider this issue. There is iar. Each trial involved a famous and an unfa- now strong evidence for relatively indepen- miliar face presented on photographic slides, dent functional components in human visual and he was asked to guess if he was unsure. processing. 18-20 Neuropsychological research His performance on this task was not signifi- has shown highly selective disorders of colour cantly different from chance, whereas he per- (but not grey) discrimination,2' movement formed much better on a parallel task with perception,22 and luminance (not colour) dis- written names." Recent MRI showed bilateral crimination.2324 Moreover, Warrington2' has abnormal signal in the temporo-occipital shown the existence of dissociable impair- junction, mainly in the inferior surface. ments affecting visual acuity, shape discrimi- Patient MS-This patient was first exam- nation, location, and colour perception in a ined in 1971 and has been studied at regular small group of patients who had made a par- intervals thereafter.263' 3335 In 1970, MS was a tial recovery from blindness. This advance in 21 year old, left handed police cadet, when he knowledge not only indicates in a more had presumptive herpes encephalitis. The detailed way which sensory abilities to test for; permanent neurological and clinical features it has also resulted in much more refined include a left sided homonymous hemianopia, assessment techniques.26 central achromatopsia, amnesia, agnosia, and The aim of the present study is therefore to prosopagnosia. He has never presented with reconsider the issue raised by Ettlinger by sensorimotor loss, aphasia, or apraxia. His investigating sensory status in three severely verbal IQ has remained in the average range prosopagnosic or object-agnosic patients and and he is a regular reader of a daily newspa- five exservicemen with unilateral brain lesions per. He is interested in and better than aver- who showed no recognition deficits. age at mental arithmetic (WAIS-R scale score 14). His visual acuity is excellent on clinical examination. At the age of 42, he was still able Methods to read, uncorrected, with either left or right SUBJECTS eye, the smallest print (N5) of ophthalmologi- Case descriptions cal text. His difficulty in recognising objects Patient PH-In 1982, at the age of 19, PH (and faces) was very severe and had not was involved in a road traffic accident in changed significantly on formal testing since which he sustained a severe closed head 1971. Using subsets of the Snodgrass and injury. The physical, ophthalmological, and Vanderwart36 line drawings, he only recog- neuropsychological sequelae have been nised seven from a sample of 72 on one occa- http://jnnp.bmj.com/ described in detail by De Haan and cowork- sion and 25 out of 80 pictures on another ers,27-29 and only the essential points will