Visual agnosias …

Victoria S Pelak MD

Neuro-Ophthalmology & Neuro-Otology

Updated 07.17.2024
Released 04.07.1999
Expires For CME 07.17.2027

Visual agnosias

Author: Victoria S Pelak MD

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Editor: Jonathan D Trobe MD

Visual agnosias

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Introduction

Overview

Visual agnosia is the inability to recognize an entity based on its visual features alone despite adequate visual acuity. There are general visual agnosias and selective visual agnosias. General visual agnosias encompass form agnosia, integrative agnosia, and transformative agnosia. Selective agnosias include prosopagnosia (agnosia for faces), pure alexia (agnosia for written words), and topographagnosia (agnosia for locale). General and selective agnosias may be subdivided into apperceptive and associative types. Apperceptive agnosias are characterized by a lack of adequate visual perception of the basic features of an entity to allow for recognition. Associative agnosias are characterized by adequate visual perception but an impairment in associating the visual perception with stored knowledge and meaning of the entity. This article reviews the clinical characteristics, pathogenesis, localization, differential diagnosis, diagnostic evaluation, and rehabilitation of the visual agnosias.

Key points

	• Visual agnosias comprise a rare group of impairments characterized by an inability to recognize objects by sight despite the preservation of normal visual acuity and the ability to recognize the objects by sound or touch.
	• The two main visual processing pathways that extend from the primary visual cortex include the dorsal (“where or action”) stream and the ventral (“what”) stream. Visual agnosia occurs because of a problem of the ventral stream.
	• Visual agnosias are classified into two broad categories: general and selective. Further divisions in both categories include apperceptive visual agnosias (the inability to adequately perceive the basic visual properties of an entity) and associative agnosias (the failure to associate adequate perception with stored knowledge and meaning).
	• Rehabilitation programs for agnosia remain primarily experimental.

Historical note and terminology

Descriptions of visual agnosia have existed for over a century. One of the earliest experimental observations involved a dog with partial ablations of both occipital lobes which had lost its usual responses to familiar objects but could still navigate around them, suggesting that it could see but could not recognize (82). In 1890, Lissauer described this “mind-blindness” in humans (72). He distinguished between an apperceptive disorder, in which subjects did not perceive objects well, and an associative disorder, in which subjects could not link what they saw with their stored knowledge about objects (93). In 1891, Freud introduced the term "agnosia," which was later defined more specifically as a failure to recognize objects that could not be attributed to poor visual acuity, cognitive impairment, or aphasia. Despite these early descriptions, controversy regarding whether agnosia was a true entity continued until single-unit recordings and case studies emerged in the 1960s and 1970s (46).

Visual agnosias are divided into two broad categories: general and selective. General and selective agnosias can be further subdivided into apperceptive and associative types. Agnosias can also be divided into those that are developmental and those that are acquired.

The distinction between apperceptive and associative visual agnosia

The distinction between apperceptive and associative agnosia emerged from descriptions by Lissauer in 1890 (72); he introduced terms that have remained valid ever since. However, the understanding of visual perception and visual recognition has grown considerably since 1890, and discoveries have allowed for arguments suggesting that not all patients can be neatly categorized into apperceptive or associative agnosia (05).

Apperceptive visual agnosia is defined as the inability to visually perceive basic or elemental visual features of an object that results from errors at early visual processing stages. Patients with apperceptive visual agnosia cannot draw or copy viewed objects.

Associative visual agnosia is defined as an inability to visually recognize despite appropriate perception. In associative visual agnosia, recognition fails because it cannot be associated with past knowledge or meaning. Thus, patients with associative agnosia have trouble linking what they see with what they know (62). The stored knowledge may relate to what objects look like (their structural representations) or general semantic facts about objects (their function and where they are found). Patients with associative visual agnosia can often produce a faithful drawing or copy of a viewed object.

Over the years, additional subtypes of each type of visual agnosia have been described, and a more refined taxonomy has emerged (62; 44).

Visual agnosia taxonomy

(Contributed by Dr. Jason J S Barton.)

General visual agnosias

Form agnosia. Patients with this type of apperceptive agnosia have difficulty recognizing even basic shapes, which are the building blocks of object structure (20). However, they can identify certain visual properties, such as brightness, contrast, and motion.

Integrative agnosia. In this type of agnosia, which is usually classified as apperceptive, the integration of shapes into the more complex structures of real objects is impaired (62). For example, a patient might see two round wheels and two triangles but not realize they form a bicycle. This deficit can be shown in their struggles to identify the individual objects within an overlapping figure drawing (62; 56).

Transformation agnosia. Transformation agnosia is a rare apperceptive condition in which patients cannot recognize objects shown from unusual (“non-canonical”) viewpoints. One interpretation is that this deficit reflects a problem with deriving a viewpoint-independent perception of an object’s three-dimensional structure (109; 62).

Category-specific agnosia. In this type of associative agnosia, patients’ recognition of living things like plants and animals might be more deficient than recognition of man-made objects like tools, furniture, or vehicles (45; 70; 24). This category specificity may be explained by the greater visual complexity and similarity of natural objects (63).

Selective visual agnosias

Prosopagnosia. In this type of visual agnosia, patients cannot identify familiar faces (07; 35). The deficit is not always confined to faces, extending sometimes to difficulty identifying other objects, such as cars, bicycles, or animals (53; 08). There are apperceptive, amnestic, and associative variants of prosopagnosia (33). In the apperceptive variant, patients cannot see differences in the facial structures of different people. In the amnestic variant, patients cannot recall particular faces. In this amnestic variant, the deficit can be probed with a questionnaire inquiring what patients remember about famous faces (09). In the associative variant, patients have good facial feature perception and preserved facial memories but cannot identify or match a face to a prior memory. There are developmental and acquired forms of prosopagnosia (99).

Pure alexia or word blindness. Pure alexia is a selective associative agnosia also known as “alexia without agraphia.” At the severe end are patients with global alexia who cannot read words at all (21), a deficit that may extend to letters and numbers and other forms of visual symbolic communication, such as musical notation or map symbols (61; 18). At the milder end of pure alexia are patients who can read letters but not words. They recognize words only by reading “letter-by-letter,” reading slowly and taking even longer when word length increases (13).

Topographagnosia. In this selective associative visual agnosia, patients cannot recognize locales or environmental surroundings. A special form includes landmark agnosia, which is the inability to recognize landmarks, such as buildings and scenes (100). Although most topographagnosia is acquired, there is a developmental form (64).

Clinical manifestations

Presentation and course

	• Patients with visual agnosia may have vague complaints, such as “not able to see properly,” or very specific complaints, such as being “unable to recognize faces.” They typically have insight into the visual nature of their dysfunction by observing that their ability to recognize people depends on nonfacial cues.
	• Most patients with visual agnosia will attribute the deficit to poor vision and seek help from an optometrist or ophthalmologist.
	• Patients with visual agnosias can identify objects and entities with tactile or auditory information.

Diagnosing visual agnosia requires diligence and time, as well as the use of directed questions and specialized testing to discover that a patient’s visual struggles are due to impaired recognition. Many patients will not have insight into the fact that their visual problems are due to brain dysfunction and assume that their perceptual issues stem from an ocular disorder. Those with developmental forms of agnosia may have had the deficit forever and seek the attention of a caregiver only when they realize that other people see things that they are not seeing (84).

General visual agnosias

Patients with general visual agnosias often report being unable to focus on viewed objects. They mishandle most daily tasks, being unable to recognize coins or paper currency and not knowing which buttons to press on their telephones. They typically attribute their deficit to their eyes and repeatedly consult providers for replacement spectacles. Many will be labeled as having psychogenic disorders.

Selective visual agnosias

Prosopagnosia. Patients with the apperceptive variant of prosopagnosia may complain that all faces look similar, like pebbles on a beach. When presented with a sequence of faces spanning various ages, they will have difficulty discerning the youngest and the oldest faces. Patients with the associative variant can discern facial features that enable the detection of age and differences between faces, but if shown a series of famous or familiar faces, they will be unable to recognize them. In either form of prosopagnosia, patients will base recognition on other visual cues, such as hairstyle, distinctive facial features, gait, mannerisms, and voice.

Pure alexia. Patients with pure alexia report that they read slowly or that reading is so laborious or error-prone that they have given it up. They may even report that they cannot read their own writing. Print size and distance to printed material do not influence the reading deficit.

Topographagnosia. Patients with agnosia for locales will get lost in familiar surroundings, including their neighborhood or even their own home. Some patients can compensate by relying on devices with a global positioning system.

Prognosis and complications

The prognosis of agnosia depends on its cause. Patients with small strokes may partially recover. Those with carbon monoxide poisoning, herpes encephalitis, or post-temporal lobectomy often have persistent deficits. Unrelenting slow deterioration is the rule with neurodegenerative diseases that lead to dementia, including those with the posterior form (visual variant) of Alzheimer disease.

Developmental agnosias are stable throughout life, although some have fluctuating psychosocial consequences. For instance, those with developmental prosopagnosia might suffer with the impression that they are aloof and unsociable (31) when starting a new job or interacting with a new group of acquaintances.

Clinical vignette

Pure alexia. A 41-year-old mechanical engineer who owned an automotive repair shop awoke with numbness in the right face and leg 1 week after an airplane trip. MRI showed a left medial occipital and fusiform gyrus stroke, attributed to paradoxical emboli through a patent foramen ovale. His memory of events was poor initially but quickly returned to normal. He had experienced trouble naming objects, but this quickly normalized, as did the numbness. He reported persistent symptoms of trouble seeing to the right and problems reading. On examination, he had a right homonymous hemianopia. Reading was very slow, proceeding one letter at a time. He could identify single letters and write well. His partner had researched rehabilitative reading strategies, and he had tried using scrolling text and manually tracing letters. He considered that improvement had been modest.

He knew which faces were familiar to him but initially had trouble recalling their names or identities. Formal neuropsychological testing showed difficulties with verbal memory but normal performance on the visual object and space perception battery. On the Warrington Recognition Memory Test, he scored 37/50 for words and 44/50 for faces, and he had normal car recognition. Measures of his reading of single words showed a markedly elevated word-length effect of 1.5 seconds per letter. He was diagnosed with alexia without agraphia (pure alexia) and right homonymous hemianopia.

Experimental studies showed that his problem with visual processing of language impacted written words and the ability to lip-read from faces even though his ability to recognize the identity of faces was intact (94; 03).

Stroke in a patient with pure alexia and right hemianopia

Coronal T2-weighted MRI image of a left inferior occipital stroke in a patient with pure alexia and right hemianopia. (Contributed by Dr. Jason J S Barton.)

Biological basis

The processes associated with visual perception and recognition involve cerebral networks with simultaneous parallel and sequential processing. After visual information reaches the striate cortex in the occipital lobe, information proceeds to extrastriate areas V2 and V3. Thereafter, visual processing is distributed to various cortical regions by means of two main streams (106).

Visual regions and visual pathways of the human brain

The image identifies the location of the striate cortex (V1), early occipital visual regions (V2, V3), and two parallel pathways. One that leads dorsally to the posterior parietal regions for the "where and visually-guided acti...

The first pathway is the dorsal (occipitoparietal, “where”) stream, which is dedicated to spatial processing for action and temporal aspects of vision (78). The second pathway is the ventral (occipitotemporal, “what”) stream, which is dedicated to visual recognition. Both pathways have an intermediate level of processing. For the dorsal stream, intermediate areas include V5 and V5a (or V5/MT complex), which are involved in motion processing (114). For the ventral stream, intermediate areas include V4 and V4a, which are involved in color analysis (115).

Identification of any object, visual attribute, or entity involves a network of regions (16) that can be demonstrated using functional magnetic resonance imaging and lesion analysis. As information moves from the retina to cortical networks, more complex representations of the visual information are extracted and associated with nonvisual sensory perceptions, memories, emotions, and behaviors, including limb and body motor responses.

Anatomic localization

General visual agnosias.

Form agnosia. Form agnosia is associated with widespread bilateral occipital lesions, particularly damage to medial structures, such as the lingual gyri (66). Deficits are especially likely to occur with widespread but nonfocal damage, as occurs after carbon monoxide poisoning, mercury poisoning, or anoxia (02; 17; 71; 23; 79).

Integrative agnosia. Integrative agnosia occurs following bilateral lesions of the lateral and inferior occipital cortex (67), most commonly from peri-striate infarcts or in the visual variant of Alzheimer disease (62; 56). Damage to the right cerebral hemisphere alone can also cause a milder version with prolonged reaction times for naming objects in line drawings (51).

Transformation agnosia. Knowledge of what objects look like from different viewpoints may be stored in both hemispheres, perhaps with a greater “part-based” representation of features in the left hemisphere and more of a “whole-object” representation in the right hemisphere (45; 68).

Category-specific agnosia. Lesions that cause category-specific associative visual agnosia may be related to anterior occipital and posterior temporal damage (88), possibly bilaterally. This type of agnosia may occur when left-sided lesions disconnect posterior occipital areas from more anterior temporal areas (25) or involve the left parahippocampal, fusiform, and lingual gyri (48).

The selective visual agnosias.

Prosopagnosia. Right occipitotemporal or bilateral occipitotemporal lesions can result in prosopagnosia. There are rare cases with left-sided lesions, but in these instances, some subjects were left-handed and may have had atypical hemispheric dominance (105; 76; 06), and others had subtle right-sided anomalies and perhaps should have been considered as having bilateral damage (113).

A network of face areas has been demonstrated by functional MRI (59; 54), including the occipital face area, the fusiform face area, and the superior temporal sulcus, and there is evidence for an anterior inferotemporal area (69; 104). Beyond this core network, an extended network, including the precuneus and inferior frontal gyrus, is sometimes activated by faces (59).

Lesions that cause the apperceptive variant of prosopagnosia are in the inferior occipitotemporal cortex, with loss of the occipital face area, fusiform face area, or both, on the right or both sides (12; 92; 35). Lesions that cause the amnestic variant localize to the right or bilateral anterior temporal cortex. Some patients have both occipitotemporal and anterior temporal damage. In contrast, a lesion of the superior temporal sulcus may impair facial expression perception but leave face identification relatively intact (49).

There is not yet a consensus about the structural basis of developmental prosopagnosia. These subjects do not have gross structural lesions on clinical scans. Some investigators have found decreased fusiform face area activation (57), but others have not (58; 04). Some investigators have reported decreased cortical thickness of the fusiform gyrus (50). Others have argued more for reduced connectivity or white matter damage either in the vicinity of the fusiform gyrus or in the inferior longitudinal fasciculus, which may carry connections between the occipitotemporal face areas and those in the anterior temporal cortex (55; 96; 97; 74; 116; 117).

Pure alexia. Pure alexia has been explained by two hypotheses. The first hypothesis is that it is a disconnection of visual information from the dominant language hemisphere (39; 52). Damage to the left occipital cortex and to callosal fibers in the splenium would cause a homonymous hemianopia and disconnect the left language regions from visual information coming from the intact right occipital lobe. The second hypothesis is that cortical damage to the left fusiform gyrus causes pure alexia (111; 19). In that hypothesis, there is no need to invoke a callosal disconnection, and the left cerebral hemispheric lesion could spare the visual pathway. Indeed, cases of pure alexia without homonymous hemianopia have been reported.

Topographagnosia. In right-handed people, the bilateral parahippocampal gyri and parieto-occipital junctions are involved in processing scene perception. In functional MRI studies, the region activated during scene perception is known as the parahippocampal place area (43). Dysfunction of this region likely explains the association of right occipitotemporal damage with topographagnosia (26).

Differential diagnosis

Confusing conditions

Vision loss. Vision loss due to ocular or retro-ocular visual pathway disorders is an important cause of an inability to recognize by sight. Although homonymous visual field loss can accompany several selective visual agnosias, the recognition deficit should not be attributed to the field loss. A macular-splitting homonymous hemianopia can reduce reading speed if it involves the central 5 degrees, but such a deficit should be called “hemianopic dyslexia” rather than pure alexia (118; 103). Hemianopic dyslexia has a modest word-length effect of, at most, 120 ms per letter. By contrast, the word-length effect may reach 1 second or more per letter in pure alexia (94). As a precaution, however, patients with impaired reading should not be diagnosed with a recognition disorder until perimetry with special attention to the central 10 degrees of the visual field has excluded small homonymous paracentral scotomas typically caused by infarction of the occipital pole.

Anomia. Naming deficits due to language dysfunction can be mistaken for visual agnosias. Patients with anomia should be able to describe what they see and how to use the object and show its use with gestures. But they cannot name it.

Associated or underlying disorders

The classic “ventral visual syndrome” is the association of prosopagnosia with a right or bilateral superior homonymous quadrantanopia (77; 32), central dyschromatopsia (the inability to distinguish hues) (81), and topographagnosia (28). This combination of signs is caused by right or bilateral inferior occipitotemporal lesions. When the lesions are bilateral, pure alexia may be present as well.

Prosopagnosia can also be associated with phonagnosia, which is an impaired recognition of familiar voices, and amusia, also known as tone deafness (73; 30).

In developmental prosopagnosia, the patient may fall within the autism spectrum, and face processing can be impaired (107; 10; 65; 101).

Diagnostic workup

Basic visual examination. The examination begins by testing visual acuity and visual fields to ensure that the patient has adequate basic visual functions to allow for visual recognition. If there is impaired recognition of letters or shapes, patients might be unable to identify symbols on the Snellen visual acuity chart. The examiner must then rely on testing visual acuity with the letter E displayed in various orientations (“tumbling E”). The patient indicates the E’s orientation by positioning the fingers of one hand.

The visual field must also be tested formally to rule out loss of the central 5 to 15 degrees, which could impair the identification of objects, faces, or letters.

Cognitive examination. The Mini-Mental State Examination and Montreal Cognitive Assessment aid in screening for cognitive deficits. However, one often needs a formal neuropsychological assessment to identify language deficits or loss of semantic knowledge that does not rely on vision. A thorough evaluation of memory functions can also be completed in a formal neuropsychological assessment, which is particularly important when assessing whether associative visual agnosia is present.

Assessment of general visual agnosias. A diagnostic evaluation for the presence of general visual agnosia can begin by showing patients various items in the room and asking them to name the items, describe what they are, or mime how they are used. Identifying objects in line drawings may be more sensitive because they provide fewer visual cues than real objects. Difficulties on these tests should be compared to the ability to identify the same objects by hearing or touch. If the identification is impaired using nonvisual presentations, the patient has a multimodal problem rather than isolated visual agnosia.

To evaluate for the presence of form agnosia, a patient should be asked to identify simple shapes like squares, triangles, and circles. If they cannot identify these items, a form of agnosia may be present (42; 80). To differentiate a loss of semantic knowledge that does not depend on vision, a patient can be asked about the differences between shapes (“what is the difference between a square and a circle?”). Some patients with form agnosia can identify objects in drawings if they can trace their outlines, translating a visual percept into a kinesthetic one (01; 71).

If patients can identify simple shapes, one can use an overlapping figures test, a robust method of detecting integrative agnosia (62; 56). Patients with this type of visual agnosia also have trouble recognizing the impossibility of images like Escher drawings. Doing so requires an appreciation that the local elements do not integrate into a correct three-dimensional structure (40).

To test for knowledge of object structure, patients may be asked to describe what specific objects look like or to draw them from memory. Their visual access to semantic stores can be probed by showing them several objects and asking them which ones are related and which are not. For example, patients can be asked, “Which is the odd one out?” while viewing pictures of a hammer, wrench, and eggbeater. To ensure that the problem is specific to vision, this performance should be compared to the ability to do the same task with the names of objects rather than pictures.

Other neuropsychological batteries can supplement these bedside tests to distinguish between associative and apperceptive visual agnosias. The Visual Object and Space Perception Battery (VOSP) assesses various elements of object recognition and visuospatial perception (110). In contrast, the Birmingham Object Recognition Battery (BORB) assesses low-level, intermediate, and high-level processing in object recognition (91). Other useful tests include the Cortical Vision Screening Test (CORVIST) (87; 86) and the Leuven Perceptual Organization Screening Test (L-POST) (102).

Assessment of selective visual agnosias. Most of the visual processing necessary for object perception is relatively intact in patients with selective visual agnosias. One or more of the above tests should be completed before assessing for the selective agnosias.

Prosopagnosia. Assessment in the clinic can begin by using photographic albums available on smartphones to assess for familiarity. If the telephone of a family member or spouse is used, the correctness of the patient’s responses can be confirmed.

The Famous Faces test is a useful tool, bearing in mind the patient’s age and culture. Alternatively, short-term familiarity with faces can be assessed by showing several faces in a learning phase, presenting those faces along with others in a recall phase, and asking the patient to indicate which face in the larger set is recalled from the initial set. Formal tests include the Cambridge Face Memory Test (41) and the Warrington Recognition Memory Test (108). With the latter, poor performance on the face half of the test can be contrasted with better performance on the word half. The diagnosis of prosopagnosia is based on a lack of facial recognition but intact voice recognition.

Pure alexia. Pure alexia can be assessed by having the patient read single words and paragraphs in a magazine or book and displaying slowed reading of longer-length words. (13). If patients cannot read words, they can be assessed for reading of numbers and single letters to determine the severity of the deficit (98).

Patients with pure alexia can write much better than they can read. However, some patients have “surface dysgraphia,” an impaired ability to spell and write words with irregular spelling (yacht, colonel). Such a finding denotes a deficit in accessing an internal lexicon (95). Patients with both reading and writing impairment have “alexia with agraphia,” a language domain deficit rather than a visual perceptual deficit. In that circumstance, speech and auditory comprehension should be tested to ensure that poor reading is not simply part of an aphasia. Even without aphasia, reading and writing deficits may be part of a dementia.

Topographagnosia. An initial assessment includes the patient’s ability to recognize familiar landmarks, scenes, or buildings. A collection of pictures from a local city or from famous landmarks (the Eiffel Tower, Niagara Falls) can be used. The photographic library on a family member’s smartphone may serve.

Management

	• Strategic adaptations to help patients with impaired object recognition in daily life involve a greater emphasis on tactile or auditory cues or the use of visual cues that are not affected by their disorder.
	• Various types of computer software can substitute for the deficient skills, but adaptive technology has not reached its full potential.

Occupational therapists can help patients learn strategies to compensate in part for their deficits. However, there are very few studies to guide the approach. Mobile devices with recognition software have been steadily improving in accuracy and speed, but most patients find these applications and devices cumbersome.

General visual agnosia. Patients with general visual agnosia should be encouraged to use tactile and auditory cues to help recognize objects. The value of putting verbal labels on items in the environment depends on whether reading is spared. Organizing the environment to maintain the same location for items used frequently can reduce the need to search for items using touch. This approach will also allow a person to predict which objects they will encounter in a given place, making recognition easier. Patients should be advised not to drive and counseled about safety concerns in operating heavy machinery.

Prosopagnosia. Patients with prosopagnosia often benefit from learning which individuals will be present in a room before they enter. Patients can also be instructed to depend on voice recognition or on companions aware of their deficits. Patients should be encouraged to explain their deficits to friends and acquaintances when they are comfortable doing so. Nametags can be helpful in the right setting.

Topographagnosia. The widespread availability of devices with GPS capability has made route-finding much easier for patients with topographagnosia, although navigating inside buildings can be difficult. Advanced planning and excursions to familiar locations to discover compensatory methods are also helpful.

Alexia. Substitutive strategies include audiobooks and text-to-speech software applications available on many electronic devices or within audible text pages.

Outcomes

There is no clinical consensus on effective rehabilitative treatments or strategies for visual agnosias. The efficacy of restorative approaches is limited, and a recent systematic review highlights the paucity of rehabilitative investigations and their less-than-impressive results (60).

There is a long history of case reports describing attempts to improve face recognition in prosopagnosia (14; 38; 34). More recently, studies involving small cohorts of patients with prosopagnosia have shown that perceptual learning can lead to modest improvements in face-shape perception (37; 34; 29). Only anecdotal evidence supports the notion that this finding translates into benefits in daily life. One trial found benefits related to oxytocin treatment (14).

None of the treatments used for pure alexia has been accepted as a standard of care. These include increasing the spacing between words or phrases (15; 75), oral articulation during reading (27), repetitive oral reading of the text (15), and finger tracing of letters (75; 83).

There is sparse information about therapies for topographagnosia. Over-learning of familiar routes may help the navigation of some patients in daily life, although this ability may not generalize to new situations.

In the appropriate clinical context, assistive technology has considerable potential to support patients with visual agnosias, particularly those with stable deficits and minimal cognitive impairment. There have been few advances in the practical use of technologies for visual agnosia during the past decade (112). The use of assistive technologies for those with vision loss due to ocular disease is closer to becoming an everyday reality with recent progress in the field (89). This progress gives hope that what is gained and learned using assistive devices for ocular visual impairment can one day be translated into research and development of devices for visual brain dysfunction.

Media

References

01: Adler A. Disintegration and restoration of optic recognition in visual agnosia: analysis of a case. Arch Neurol Psychiatry 1944;51:243-59.
02: Adler A. Course and outcome of visual agnosia. J Nerv Ment Dis 1950;111(1):41-51. PMID 15402617
03: Albonico A, Barton JJ. Face perception in pure alexia: complementary contributions of the left fusiform gyrus to facial identity and facial speech processing. Cortex 2017;96:59-72. PMID 28964939
04: Avidan G, Hasson U, Malach R, Behrmann M. Detailed exploration of face-related processing in congenital prosopagnosia: 2. Functional neuroimaging findings. J Cogn Neurosci 2005;17(7):1150-67. PMID 16102242
05: Aviezer H, Landau AN, Robertson LC, et al. Implicit integration in a case of integrative visual agnosia. Neuropsychologia 2007;45(9):2066-77. PMID 17339044
06: Barton JJ. Prosopagnosia associated with a left occipitotemporal lesion. Neuropsychologia 2008a;46(8):2214-24. PMID 18374372
07: Barton JJ. Structure and function in acquired prosopagnosia: lessons from a series of 10 patients with brain damage. J Neuropsychol 2008b;2(1):197-225. PMID 19334311
08: Barton JJ, Albonico A, Susilo T, Duchaine B, Corrow SL. Object recognition in acquired and developmental prosopagnosia. Cogn Neuropsychol 2019;36(1-2):54-84. PMID 30947609
09: Barton JJ, Cherkasova M. Face imagery and its relation to perception and covert recognition in prosopagnosia. Neurology 2003;61(2):220-5. PMID 12874402
10: Barton JJ, Cherkasova MV, Hefter R, Cox TA, O'Connor M, Manoach DS. Are patients with social developmental disorders prosopagnosic? Perceptual heterogeneity in the Asperger and socio-emotional processing disorders. Brain 2004;127(Pt 8):1706-16. PMID 15215211
11: Barton JJS, Corrow SL. The problem of being bad at faces. Neuropsychologia 2016;89:119-24. PMID 27312748
12: Barton JJ, Press DZ, Keenan JP, O'Connor M. Lesions of the fusiform face area impair perception of facial configuration in prosopagnosia. Neurology 2002;58(1):71-8. PMID 11781408
13: Barton JJ, Hanif HM, Eklinder Bjornstrom L, Hills C. The word-length effect in reading: a review. Cogn Neuropsychol 2014;31(5-6):378-412. PMID 24665973
14: Bate S, Bennetts RJ. The rehabilitation of face recognition impairments: a critical review and future directions. Front Hum Neurosci 2014;8:491. PMID 25100965
15: Beeson PM, Insalaco D. Acquired alexia: lessons from successful treatment. J Int Neuropsychol Soc 1998;4(6):621-35. PMID 10050367
16: Behrmann M, Plaut DC. Distributed circuits, not circumscribed centers, mediate visual recognition. Trends Cogn Sci 2013;17(5):210-9. PMID 23608364
17: Benson DF, Greenberg JP. Visual form agnosia. A specific defect in visual discrimination. Arch Neurol 1969;20(1):82-9. PMID 4303441
18: Beversdorf DQ, Heilman KM. Progressive ventral posterior cortical degeneration presenting as alexia for music and words. Neurology 1998;50(3):657-9. PMID 9521252
19: Beversdorf DQ, Ratcliffe NR, Rhodes CH, Reeves AG. Pure alexia: clinical-pathologic evidence for a lateralized visual language association cortex. Clin Neuropathol 1997;16(6):328-31. PMID 9401800
20: Biederman I. Recognition-by-components: a theory of human image understanding. Psychol Rev 1987;94(2):115-47. PMID 3575582
21: Binder JR, Mohr JP. The topography of callosal reading pathways. A case-control analysis. Brain 1992;115(Pt 6):1807-26. PMID 1486462
22: Bowles DC, McKone E, Dawel A, et al. Diagnosing prosopagnosia: effects of ageing, sex, and participant-stimulus ethnic match on the Cambridge Face Memory Test and Cambridge Face Perception Test. Cogn Neuropsychol 2009;26(5):423-55. PMID 19921582
23: Campion J, Latto R. Apperceptive agnosia due to carbon monoxide poisoning. An interpretation based on critical band masking from disseminated lesions. Behav Brain Res 1985;15(3):227-40. PMID 4005031
24: Caramazza A, Shelton JR. Domain-specific knowledge systems in the brain the animate-inanimate distinction. J Cogn Neurosci 1998;10(1):1-34. PMID 9526080
25: Carlesimo GA, Casadio P, Sabbadini M, Caltagirone C. Associative visual agnosia resulting from a disconnection between intact visual memory and semantic systems. Cortex 1998;34(4):563-76. PMID 9800090
26: Claessen MH, van der Ham IJ. Classification of navigation impairment: A systematic review of neuropsychological case studies. Neurosci Biobehav Rev 2017;73:81-97. PMID 27993606
27: Conway TW, Heilman P, Rothi LJ, et al. Treatment of a case of phonological alexia with agraphia using the Auditory Discrimination in Depth (ADD) program. J Int Neuropsychol Soc 1998;4(6):608-20. PMID 10050366
28: Corrow JC, Corrow SL, Lee E, et al. Getting lost: Topographic skills in acquired and developmental prosopagnosia. Cortex 2016;76:89-103. PMID 26874939
29: Corrow SL, Davies-Thompson J, Fletcher K, Hills C, Corrow JC, Barton JJ. Training face perception in developmental prosopagnosia through perceptual learning. Neuropsychologia 2019a;134:107196. PMID 31541661
30: Corrow SL, Stubbs JL, Schlaug G, et al. Perception of musical pitch in developmental prosopagnosia. Neuropsychologia 2019b;124:87-97. PMID 30625291
31: Dalrymple KA, Fletcher K, Corrow S, et al. "A room full of strangers every day": the psychosocial impact of developmental prosopagnosia on children and their families. J Psychosom Res 2014;77(2):144-50. PMID 25077856
32: Damasio AR, Damasio H, Van Hoesen GW. Prosopagnosia: anatomic basis and behavioral mechanisms. Neurology 1982;32(4):331-41. PMID 7199655
33: Damasio AR, Tranel D, Damasio H. Face agnosia and the neural substrates of memory. Annu Rev Neurosci 1990;13:89-109. PMID 2183687
34: Davies-Thompson J, Fletcher K, Hills C, Pancaroglu R, Corrow SL, Barton JJ. Perceptual learning of faces: a rehabilitative study of acquired prosopagnosia. J Cogn Neurosci 2017;29(3):573-91. PMID 28139958
35: Davies-Thompson J, Pancaroglu R, Barton J. Acquired prosopagnosia: structural basis and processing impairments. Front Biosci (Elite Ed) 2014;6(1):159-74. PMID 24389150
36: De Renzi E. Prosopagnosia in two patients with CT scan evidence of damage confined to the right hemisphere. Neuropsychologia 1986;24(3):385-9. PMID 3736820
37: DeGutis J, Cohan S, Nakayama K. Holistic face training enhances face processing in developmental prosopagnosia. Brain 2014a;137(Pt 6):1781-98. PMID 24691394
38: DeGutis JM, Chiu C, Grosso ME, Cohan S. Face processing improvements in prosopagnosia: successes and failures over the last 50 years. Front Hum Neurosci 2014b;8:561. PMID 25140137
39: Dejerine J. Contribution à l'étude anatomo-pathologique et clinique des différentes variétés cécité verbale. Comptes Rendus Hebdomaladaires des Séances et Mémoires de la Société de Biologie 1892;4:61-90.
40: Delvenne JF, Seron X, Coyette F, Rossion B. Evidence for perceptual deficits in associative visual (prosop)agnosia: a single-case study. Neuropsychologia 2004;42(5):597-612. PMID 14725798
41: Duchaine B, Nakayama K. The Cambridge Face Memory Test: results for neurologically intact individuals and an investigation of its validity using inverted face stimuli and prosopagnosic participants. Neuropsychologia 2006;44(4):576-85. PMID 16169565
42: Efron R. What is perception? Boston Studies Philosoph Sci 1968;4:137-73.
43: Epstein R, Harris A, Stanley D, Kanwisher N. The parahippocampal place area: recognition, navigation, or encoding? Neuron 1999;23(1):115-25. PMID 10402198
44: Farah MJ. Visual agnosia: once more, with theory. Cognitive Neuropsychol 1988;5(3): 337-46.
45: Farah MJ. Patterns of co-occurrence among the associative agnosias: Implications for visual object representation. Cog Neuropsychol 1991;8(1):1-19.
46: Farah, MJ. Visual Agnosia. 2nd ed. Cambridge, Mass: MIT Press, 2004.
47: Farah MJ, McMullen PA, Meyer MM. Can recognition of living things be selectively impaired? Neuropsychologia 1991;29(2):185-93. PMID 2027434
48: Feinberg TE, Schindler RJ, Ochoa E, Kwan PC, Farah MJ. Associative visual agnosia and alexia without prosopagnosia. Cortex 1994;30(3):395-411. PMID 7805382
49: Fox CJ, Hanif HM, Iaria G, Duchaine BC, Barton JJ. Perceptual and anatomic patterns of selective deficits in facial identity and expression processing. Neuropsychologia 2011;49(12):3188-200. PMID 21807006
50: Garrido L, Furl N, Draganski B, et al. Voxel-based morphometry reveals reduced grey matter volume in the temporal cortex of developmental prosopagnosics. Brain 2009;132(Pt 12):3443-55. PMID 19887506
51: Gerlach C, Marstrand L, Habekost T, Gade A. A case of impaired shape integration: Implications for models of visual object processing. Vis Cog 2005;12(8):1409-43.
52: Geschwind N. Disconnexion syndromes in animals and man. Brain 1965;88:17-294.
53: Geskin J, Behrmann M. Congenital prosopagnosia without object agnosia? A literature review. Cogn Neuropsychol 2018;35(1-2):4-54. PMID 29165034
54: Gobbini MI, Haxby JV. Neural systems for recognition of familiar faces. Neuropsychologia 2007;45(1):32-41. PMID 16797608
55: Gomez J, Pestilli F, Witthoft N, et al. Functionally defined white matter reveals segregated pathways in human ventral temporal cortex associated with category-specific processing. Neuron 2015;85(1):216-27. PMID 25569351
56: Grossman M, Galetta S, D'Esposito M. Object recognition difficulty in visual apperceptive agnosia. Brain Cogn 1997;33(3):306-42. PMID 9126398
57: Hadjikhani N, de Gelder B. Neural basis of prosopagnosia: an fMRI study. Hum Brain Mapp 2002;16(3):176-82. PMID 12112771
58: Hasson U, Avidan G, Deouell LY, Bentin S, Malach R. Face-selective activation in a congenital prosopagnosic subject. J Cogn Neurosci 2003;15(3):419-31. PMID 12729493
59: Haxby JV, Hoffman EA, Gobbini MI. The distributed human neural system for face perception. Trends Cogn Sci 2000;4(6):223-33. PMID 10827445
60: Heutink J, Indorf DL, Cordes C. The neuropsychological rehabilitation of visual agnosia and Balint's syndrome. Neuropsychol Rehabil 2019;29(10):1489-508. PMID 29366371
61: Horikoshi T, Asari Y, Watanabe A, et al. Music alexia in a patient with mild pure alexia: disturbed visual perception of nonverbal meaningful figures. Cortex 1997;33(1):187-94. PMID 9088731
62: Humphreys GW, Riddoch MJ. To See But Not To See: A Case Study Of Visual Agnosia. London: Lawrence Erlbaum Associates, 1987.
63: Humphreys GW, Riddoch MJ, Quinlan PT. Cascade processes in picture identification. Cog Neuropsychol 1988;(1):67-104.
64: Iaria G, Barton JJ. Developmental topographical disorientation: a newly discovered cognitive disorder. Exp Brain Res 2010;206(2):189-96. PMID 20431873
65: Jemel B, Mottron L, Dawson M. Impaired face processing in autism: fact or artifact? J Autism Dev Disord 2006;36(1):91-106. PMID 16477517
66: Karnath HO, Rüter J, Mandler A, Himmelbach M. The anatomy of object recognition--visual form agnosia caused by medial occipitotemporal stroke. J Neurosci 2009;29(18):5854-62. PMID 19420252
67: Konen CS, Behrmann M, Nishimura M, Kastner S. The functional neuroanatomy of object agnosia: a case study. Neuron 2011;71(1):49-60. PMID 21745637
68: Kosslyn SM. Image and brain: The resolution of the imagery debate. Cambridge, MA, US: The MIT Press, 1994.
69: Kriegeskorte N, Formisano E, Sorger B, Goebel R. Individual faces elicit distinct response patterns in human anterior temporal cortex. Proc Natl Acad Sci U S A 2007;104(51):20600-5. PMID 18077383
70: Kurbat MA. Can the recognition of living things really be selectively impaired? Neuropsychologia 1997;35(6):813-27. PMID 9204487
71: Landis T, Graves R, Benson DF, Hebben N. Visual recognition through kinaesthetic mediation. Psychol Med 1982;12(3):515-31. PMID 7134311
72: Lissauer H. Einfall von Seelenblindheit nebst einem Bintrag zur Theorie derselben. Arch Psychiatr Nervenkr 1890;2:22.
73: Liu RR, Pancaroglu R, Hills CS, Duchaine B, Barton JJ. Voice recognition in face-blind patients. Cereb Cortex 2016;26(4):1473-87. PMID 25349193
74: Lohse M, Garrido L, Driver J, Dolan RJ, Duchaine BC, Furl N. Effective connectivity from early visual cortex to posterior occipitotemporal face areas supports face selectivity and predicts developmental prosopagnosia. J Neurosci 2016;36(13):3821-8. PMID 27030766
75: Maher LM, Clayton MC, Barrett AM, Schober-Peterson D, Gonzalez Rothi LJ. Rehabilitation of a case of pure alexia: exploiting residual abilities. J Int Neuropsychol Soc 1998;4(6):636-47. PMID 10050368
76: Mattson AJ, Levin HS, Grafman J. A case of prosopagnosia following moderate closed head injury with left hemisphere focal lesion. Cortex 2000;36(1):125-37. PMID 10728902
77: Meadows JC. The anatomical basis of prosopagnosia. J Neurol Neurosurg Psychiatry 1974;37(5):489-501. PMID 4209556
78: Milner A, Goodale M. The visual brain in action. Oxford: Oxford University Press, 1995.
79: Milner AD, Cavina-Pratesi C. Perceptual deficits of object identification: apperceptive agnosia. Handb Clin Neurol 2018;151:269-86. PMID 29519462
80: Milner AD, Perrett DI, Johnston RS, et al. Perception and action in 'visual form agnosia'. Brain 1991;114(Pt 1B):405-28. PMID 2004249
81: Moroz D, Corrow SL, Corrow JC, Barton AR, Duchaine B, Barton JJ. Localization and patterns of cerebral dyschromatopsia: a study of subjects with prospagnosia. Neuropsychologia 2016;89:153-60. PMID 27312747
82: Munk H. Über die Functionen der Grosshirnrinde: Gesammelte Mittheilungen aus den Jahren 1877-1880. Berlin: Hirschwald, 1881.
83: Nitzberg Lott S, Friedman RB. Can treatment for pure alexia improve letter-by-letter reading speed without sacrificing accuracy? Brain Lang 1999;67(3):188-201. PMID 10210630
84: Palermo R, Rossion B, Rhodes G, et al. Do people have insight into their face recognition abilities? Q J Exp Psychol (Hove) 2017;70(2):218-33. PMID 26935244
85: Pelak VS. The clinical approach to the identification of higher-order visual dysfunction in neurodegenerative disease. Curr Neurol Neurosci Rep 2022;22(4):229-42. PMID 35320467
86: Plant GT, Galton MJ, Warrington EK. The CORVIST. 2020.
87: Plant GT, Warrington EK. The cortical vision screening test (CORVIST). Bury St Edmunds: Thames Valley Test Company, 2001.
88: Ptak R, Lazeyras F, Di Pietro M, Schnider A, Simon SR. Visual object agnosia is associated with a breakdown of object-selective responses in the lateral occipital cortex. Neuropsychologia 2014;60:10-20. PMID 24863251
89: Ricci FS, Boldini A, Ma X, et al. Virtual reality as a means to explore assistive technologies for the visually impaired. PLOS Digit Health 2023;2(6):e0000275. PMID 37339135
90: Riddoch MJ, Humphreys GW. A case of integrative visual agnosia. Brain 1987;110(Pt 6):1431-62. PMID 3427396
91: Riddoch MJ, Humphreys GW. The Birmingham Object Recognition Battery. London: Erlbaum; 1993.
92: Rossion B, Caldara R, Seghier M, Schuller AM, Lazeyras F, Mayer E. A network of occipito-temporal face-sensitive areas besides the right middle fusiform gyrus is necessary for normal face processing. Brain 2003;126(Pt 11):2381-95. PMID 12876150
93: Shallice T, Jackson M. Lissauer on agnosia. Cogn Neuropsychol 1988;5(2):153-6.
94: Sheldon CA, Abegg M, Sekunova A, Barton JJ. The word-length effect in acquired alexia, and real and virtual hemianopia. Neuropsychologia 2012;50(5):841-51. PMID 22326623
95: Sheldon CA, Malcolm GL, Barton JJ. Alexia with and without agraphia: an assessment of two classical syndromes. Can J Neurol Sci 2008;35(5):616-24. PMID 19235446
96: Song S, Garrido L, Nagy Z, et al. Local but not long-range microstructural differences of the ventral temporal cortex in developmental prosopagnosia. Neuropsychologia 2015a;78:195-206. PMID 26456436
97: Song Y, Zhu Q, Li J, Wang X, Liu J. Typical and atypical development of functional connectivity in the face network. J Neurosci 2015b;35(43):14624-35. PMID 26511251
98: Starrfelt R, Behrmann M. Number reading in pure alexia--a review. Neuropsychologia 2011;49(9):2283-98. PMID 21554892
99: Susilo T, Duchaine B. Advances in developmental prosopagnosia research. Curr Opin Neurobiol 2013;23(3):423-9. PMID 23391526
100: Takahashi N, Kawamura M. Pure topographical disorientation--the anatomical basis of landmark agnosia. Cortex 2002;38(5):717-25. PMID 12507041
101: Tanaka JW, Sung A. The "eye avoidance" hypothesis of autism face processing. J Autism Dev Disord 2016;46(5):1538-52. PMID 24150885
102: Torfs K, Vancleef K, Lafosse C, Wagemans J, de-Wit L. The Leuven Perceptual Organization Screening Test (L-POST), an online test to assess mid-level visual perception. Behav Res Methods 2014;46(2):472-87. PMID 24190065
103: Trauzettel-Klosinski S, Brendler K. Eye movements in reading with hemianopic field defects: the significance of clinical parameters. Graefes Arch Clin Exp Ophthalmol 1998;236(2):91-102. PMID 9498119
104: Tsao DY, Moeller S, Freiwald WA. Comparing face patch systems in macaques and humans. Proc Natl Acad Sci U S A 2008;105(49):19514-9. PMID 19033466
105: Tzavaras A, Merienne L, Masure MC. Prosopagnosie, amnésie et troubles du langage par lésion temporale gauche chez un sujet Gaucher [Prosopagnosia, amnesia and language disorders caused by left temporal lobe injury in a left-handed man]. [French.] Encephale 1973;62(4):382-94. PMID 4794621
106: Ungerleider L, Mishkin M. Two cortical visual systems. In: Ingle DJ, Mansfield RJW, Goodale MS, editors. The Analysis Of Visual Behaviour. Cambridge, MA: MIT Press, 1982:549-86.
107: Volkmar FR, Sparrow SS, Rende RD, Cohen DJ. Facial perception in autism. J Child Psychol Psychiatry 1989;30(4):591-8. PMID 2768360
108: Warrington E. Warrington Recognition Memory Test. Los Angeles: Western Psychological Services, 1984.
109: Warrington EK, James M. Visual object recognition in patients with right-hemisphere lesions: axes or features? Perception 1986;15(3):355-66. PMID 3797207
110: Warrington EK, James M. The visual object and space perception battery. London: Pearson, 1991.
111: Warrington EK, Shallice T. Word-form dyslexia. Brain 1980;103(1):99-112. PMID 6244876
112: Worthington A, Sudol J. Assistive technology for disorders of visual perception. In: O'Neill B, Gillespie A, editors. Assistive Technology For Cognition: A Handbook For Clinicians and Developers. Psychology Press, 2015: 65-80.
113: Wright H, Wardlaw J, Young AW, Zeman A. Prosopagnosia following nonconvulsive status epilepticus associated with a left fusiform gyrus malformation. Epilepsy Behav 2006;9(1):197-203. PMID 16777488
114: Zeki S. Cerebral akinetopsia (visual motion blindness). A review. Brain 1991;114(Pt 2):811-24. PMID 2043951
115: Zeki S. A century of cerebral achromatopsia. Brain 1990;113(Pt 6):1721-77. PMID 2276043
116: Zhao Y, Li J, Liu X, et al. Altered spontaneous neural activity in the occipital face area reflects behavioral deficits in developmental prosopagnosia. Neuropsychologia 2016;89:344-355. PMID 27475965
117: Zhao Y, Zhen Z, Liu X, Song Y, Liu J. The neural network for face recognition: Insights from an fMRI study on developmental prosopagnosia. Neuroimage 2018;169:151-61. PMID 29242103
118: Zihl J. Eye movement patterns in hemianopic dyslexia. Brain 1995;118(Pt 4):891-912. PMID 7655887

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Visual agnosias

Differential Diagnosis

simultanagnosia
anomia
prosopagnosia
macular degeneration
Parkinson disease
- Alzheimer disease
- autism spectrum disorders
- hemianopic dyslexia
- homonymous paracentral scotoma
- amblyopic dyslexia
- topographic disorientation

Visual agnosias

Introduction

Overview

Key points

Historical note and terminology

The distinction between apperceptive and associative visual agnosia

General visual agnosias

Selective visual agnosias

Clinical manifestations

Presentation and course

General visual agnosias

Selective visual agnosias

Prognosis and complications

Clinical vignette

Biological basis

Anatomic localization

Epidemiology

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Management

Outcomes

Media

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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