Instruct: Given a question, retrieve passages that can help answer the question.
Query: Is there a cure for agnosia?
For all practical purposes, there is no direct cure. Patients may improve if information is presented in other modalities than the damaged one. Different types of therapies can help to reverse the effects of agnosia. In some cases, occupational therapy or speech therapy can improve agnosia, depending on its cause.
Agnosia is a rare occurrence and can be the result of a stroke, dementia, head injury, brain infection, or hereditary.
Apperceptive agnosia is a deficit in object perception creating an inability to understand the significance of objects.
Similarly, associative visual agnosia is the inability to understand the significance of objects; however, this time the deficit is in semantic memory. Both of these agnosias can affect the pathway to object recognition, like Marr's Theory of Vision. More specifically unlike apperceptive agnosia, associative agnosic patients are more successful at drawing, copying, and matching tasks; however, these patients demonstrate that they can perceive but not recognize.
Integrative agnosia(a subtype of associative agnosia) is the inability to integrate separate parts to form a whole image. With these types of agnosias there is damage to the ventral (what) stream of the visual processing pathway.
Object orientation agnosia is the inability to extract the orientation of an object despite adequate object recognition. With this type of agnosia there is damage to the dorsal (where) stream of the visual processing pathway.
This can affect object recognition in terms of familiarity and even more so in unfamiliar objects and viewpoints.
A difficulty in recognizing faces can be explained by prosopagnosia. Someone with prosopagnosia cannot identify the face but is still able to perceive age, gender, and emotional expression. The brain region that specifies in facial recognition is the fusiform face area. Prosopagnosia can also be divided into apperceptive and associative subtypes. Recognition of individual chairs, cars, animals can also be impaired; therefore, these object share similar perceptual features with the face that are recognized in the fusiform face area.
In order to assess an individual for agnosia, it must be verified that the individual is not suffering from a loss of sensation, and that both their language abilities and intelligence are intact. In order for an individual to be diagnosed with agnosia, they must only be experiencing a sensory deficit in a single modality. To make a diagnosis, the distinction between apperceptive and associative agnosia must be made. This distinction can be made by having the individual complete copying and matching tasks. If the individual is suffering from a form of apperceptive agnosia they will not be able to match two stimuli that are identical in appearance. In contrast, if an individual is suffering from a form of associative agnosia, they will not be able to match different examples of a stimulus. For example, an individual who has been diagnosed with associative agnosia in the visual modality would not be able to match pictures of a laptop that is open with a laptop that is closed.[3]
An agnosia that affects hearing, "auditory sound agnosia", is broken into subdivisions based on level of processing impaired, and a "semantic-associative" form is investigated within the auditory agnosias.
In 1928, Kleist suggested that the etiology of word deafness could be due either to impaired perception of the sound (apperceptive auditory agnosia), or to impaired extraction of meaning from a sound (associative auditory agnosia). This hypothesis was first tested by Vignolo et al (1969), who examined unilateral stroke patients. They reported that patients with left hemisphere damage were impaired in matching environmental sounds with their corresponding pictures, whereas patients with right hemisphere damage were impaired in the discrimination of meaningless noise segments. The researchers then concluded that left hemispheric damage results in associative auditory agnosia, and right hemisphere damage results in apperceptive auditory agnosia. Although the conclusion reached by this study could be considered over-reaching, associative auditory agnosia could correspond with the disorder word meaning deafness.
Agnosia can result from strokes, dementia, or other neurological disorders. It may also be trauma-induced by a head injury, brain infection, or hereditary. Additionally, some forms of agnosia may be the result of developmental disorders.[4] Damage causing agnosia usually occurs in either the occipital or parietal lobes of the brain. Although one modality may be affected, cognitive abilities in other areas are preserved.[4]
Although visual agnosia can be general, there exist many variants that impair recognition of specific types. These variants of visual agnosia include prosopagnosia (inability to recognize faces), pure word blindness (inability to recognize words, often called "agnosic alexia" or "pure alexia"), agnosias for colors (inability to differentiate colors), agnosias for the environment (inability to recognize landmarks or difficult with spatial layout of an environment, i.e. topographagnosia) and simultanagosia (inability to sort out multiple objects in a visual scene).
In a review of the auditory agnosia literature, Phillips and Farmer showed that patients with word deafness are impaired in their ability to discriminate gaps between click sounds as long as 15-50 milliseconds, which is consistent with the duration of phonemes. They also showed that patients with general auditory agnosia are impaired in their ability to discriminate gaps between click sounds as long as 100-300 milliseconds. The authors further showed that word deafness patients liken their auditory experience to hearing foreign language, whereas general auditory agnosia described speech as incomprehensible noise. Based on these findings, and because both word deafness and general auditory agnosia patients were reported to have very similar neuroanatomical damage (bilateral damage to the auditory cortices), the authors concluded that word deafness and general auditory agnosia is the same disorder, but with a different degree of severity.
Auditory verbal agnosia (AVA) is not the same as auditory agnosia; patients with (nonverbal) auditory agnosia have a relatively more intact speech comprehension system despite their impaired recognition of nonspeech sounds.
In contrast, individuals with associative visual agnosia experience difficulty when asked to name objects. Associative agnosia is associated with damage to both the right and left hemispheres at the occipitotemporal border.[22] A specific form of associative visual agnosia is known as prosopagnosia. Prosopagnosia is the inability to recognize faces. For example, these individuals have difficulty recognizing friends, family and coworkers.[22] However, individuals with prosopagnosia can recognize all other types of visual stimuli.[23]
The term "agnosia" refers to a loss of knowledge. Acquired music agnosia is the "inability to recognize music in the absence of sensory, intellectual, verbal, and mnesic impairments". Music agnosia is most commonly acquired; in most cases it is a result of bilateral infarction of the right temporal lobes. In his article, Satoh states "when pure word deafness, auditory sound agnosia, and receptive amusia occur simultaneously, the state is called auditory agnosia" (Satoh 2007). However, one must understand the subtle difference between auditory and music agnosia; the former refers to the inability to recognize environmental sounds while the latter refers to the inability to recognize music.
Musical agnosias may be categorized based on the process which is impaired in the individual. Apperceptive music agnosia involves an impairment at the level of perceptual analysis involving an inability to encode musical information correctly. Associative music agnosia reflects an impaired representational system which disrupts music recognition. Many of the cases of music agnosia have resulted from surgery involving the middle cerebral artery. Patient studies have surmounted a large amount of evidence demonstrating that the left side of the brain is more suitable for holding long-term memory representations of music and that the right side is important for controlling access to these representations. Associative music agnosias tend to be produced by damage to the left hemisphere, while apperceptive music agnosia reflects damage to the right hemisphere.
Social-emotional agnosia, also known as emotional agnosia or expressive agnosia, is the inability to perceive facial expressions, body language, and voice intonation. A person with this disorder is unable to non-verbally perceive others' emotions in social situations, limiting normal social interactions. The condition causes a functional blindness to subtle non-verbal social-emotional cues in voice, gesture, and facial expression. People with this form of agnosia have difficulty in determining and identifying the motivational and emotional significance of external social events, and may appear emotionless or agnostic (uncertainty or general indecisiveness about a particular thing). Symptoms of this agnosia can vary depending on the area of the brain affected. Social-emotional agnosia often occurs in individuals with schizophrenia and autism. It is difficult to distinguish from, and has been found to co-occur with, alexithymia.
Broadly, visual agnosia is divided into apperceptive and associative visual agnosia.
Cerebral deafness (also known as cortical deafness or central deafness) is a disorder characterized by complete deafness that is the result of damage to the central nervous system. The primary distinction between auditory agnosia and cerebral deafness is the ability to detect pure tones, as measured with pure tone audiometry. Using this test, auditory agnosia patients were often reported capable of detecting pure tones almost as good as healthy individuals, whereas cerebral deafness patients found this task almost impossible or they required very loud presentations of sounds (above 100 dB). In all reported cases, cerebral deafness was associated with bilateral temporal lobe lesions. A study that compared the lesions of two cerebral deafness patients to an auditory agnosia patient concluded that cerebral deafness is the result of complete de-afferentation of the auditory cortices, whereas in auditory agnosia some thalamo-cortical fibers are spared. In most cases the disorder is transient and the symptoms mitigate into auditory agnosia (although chronic cases were reported). Similarly, a monkey study that ablated both auditory cortices of monkeys reported of deafness that lasted 1 week in all cases, and that was gradually mitigated into auditory agnosia in a period of 3–7 weeks.
Auditory agnosia is a form of agnosia that manifests itself primarily in the inability to recognize or differentiate between sounds. It is not a defect of the ear or "hearing", but rather a neurological inability of the brain to process sound meaning. It is caused by bilateral damage to the anterior superior temporal gyrus, which is part of the auditory pathway responsible for sound recognition, the auditory "what" pathway . Persons with auditory agnosia can physically hear the sounds and describe them using unrelated terms, but are unable to recognize them. They might describe the sound of some environmental sounds, such as a motor starting, as resembling a lion roaring, but would not be able to associate the sound with "car" or "engine", nor would they say that it "was" a lion creating the noise. All auditory agnosia patients read lips in order to enhance the speech comprehension. It is yet unclear whether auditory agnosia (also called general auditory agnosia) is a combination of milder disorders, such auditory verbal agnosia (pure word deafness), non-verbal auditory agnosia, amusia and word meaning deafness), or a mild case of the more severe disorder, cerebral deafness. So far, 203 patients with auditory perceptual deficits due to CNS damage were reported in the medical literature, of which 183 diagnosed with general auditory agnosia or word deafness, 34 with cerebral deafness, 51 with non-verbal auditory agnosia-amusia and 8 word meaning deafness (for a list of patients see).
Agnosias are sensory modality specific, usually classified as visual, auditory, or tactile. Associative visual agnosia refers to a subtype of visual agnosia, which was labeled by Lissauer (1890), as an inability to connect the visual percept (mental representation of something being perceived through the senses) with its related semantic information stored in memory, such as, its name, use, and description. This is distinguished from the visual apperceptive form of visual agnosia, "apperceptive visual agnosia", which is an inability to produce a complete percept, and is associated with a failure in higher order perceptual processing where feature integration is impaired, though individual features can be distinguished. In reality, patients often fall between both distinctions, with some degree of perceptual disturbances exhibited in most cases, and in some cases, patients may be labeled as integrative agnostics when they fit the criteria for both forms. Associative visual agnosias are often category-specific, where recognition of particular categories of items are differentially impaired, which can affect selective classes of stimuli, larger generalized groups or multiple intersecting categories. For example, deficits in recognizing stimuli can be as specific as familiar human faces or as diffuse as living things or non-living things.
Since the early days of aphasia research, the relationship between auditory agnosia and speech perception has been debated. Lichtheim (1885) proposed that auditory agnosia is the result of damage to a brain area dedicated to the perception of spoken words, and consequently renamed this disorder from ‘word deafness’ to ‘pure word deafness’. The description of word deafness as being exclusively for words was adopted by the scientific community despite the patient reported by Lichtheim’s who also had more general auditory deficits. Some researchers who surveyed the literature, however, argued against labeling this disorder as pure word deafness on the account that all patients reported impaired at perceiving spoken words were also noted with other auditory deficits or aphasic symptoms. In one review of the literature, Ulrich (1978) presented evidence for separation of word deafness from more general auditory agnosia, and suggested naming this disorder “linguistic auditory agnosia” (this name was later rephrased into “verbal auditory agnosia”). To contrast this disorder with auditory agnosia in which speech repetition is intact (word meaning deafness), the name “word sound deafness” and “phonemic deafness” (Kleist, 1962) were also proposed. Although some researchers argued against the purity of word deafness, some anecdotal cases with exclusive impaired perception of speech were documented. On several occasions, patients were reported to gradually transition from pure word deafness to general auditory agnosia/cerebral deafness or recovery from general auditory agnosia/cerebral deafness to pure word deafness.
Apperceptive agnosia is a failure in recognition that is due to a failure of perception. In contrast, associative agnosia is a type of agnosia where perception occurs but recognition still does not occur. When referring to apperceptive agnosia, visual and object agnosia are most commonly discussed; This occurs because apperceptive agnosia is most likely to present visual impairments. However, in addition to visual apperceptive agnosia there are also cases of apperceptive agnosia in other sensory areas.
When determining whether a patient has form agnosia or integrative agnosia, an Efron shape test can be performed. A poor score on the Efron shape test will indicate form agnosia, as opposed to integrative agnosia. A good score on the Efron shape test, but a poor score on a figure-ground segmentation test and an overlapping figures test will indicate integrative agnosia. A patient with integrative agnosia will find it hard to group and segment shapes, especially if there are overlapping animate items or they can over segment objects with high internal detail. However, the patient should have and understand basic coding of shape.
In addition to verbal and nonverbal auditory agnosia, there are cases of auditory apperceptive agnosia where patients are unable to recognize music in the absence of sensory, intellectual, and verbal impairments. In these cases there may be a melodic or a memory basis established in the brain and damage to those areas lead to music agnosia. Agnosia occurs because of failure to re-encode melodic information properly. This tends to be associated with right-sided lesions interrupting the melodic route in the brain.
Auerbach et al considered word deafness and general auditory agnosia as two separate disorders, and labelled general auditory agnosia as pre-phonemic auditory agnosia and word deafness as post-phonemic auditory agnosia. They suggested that pre-phonemic auditory agnosia manifests because of general damage to the auditory cortex of both hemispheres, and that post-phonemic auditory agnosia manifests because of damage to a spoken word recognition center in the left hemisphere. Recent evidence, possibly verified Auerbach hypothesis, since an epileptic patient who undergone electro-stimulation to the anterior superior temporal gyrus was demonstrated a transient loss of speech comprehension, but with intact perception of environmental sounds and music.
Visual agnosia (both apperceptive and associative) is prevalent in Alzheimer’s Disease (AD) patients. Visual agnosia may be present in early stages of AD and can often act as an indicator of AD. Apperceptive agnosia results from diffuse cortical pathology of AD. There is early involvement in the hippocampus and the entorhinal cortex followed by a spread to adjacent areas with neurofibrillary tangles (NFT). Gradual extension of NFT throughout the occipital, parietal, and temporal regions devoted to vision occur resulting in visual agnosia.
The term auditory agnosia was originally coined by Freud (1891) to describe patients with selective impairment of environmental sounds. In a review of the auditory agnosia literature, Ulrich re-named this disorder as non-verbal auditory agnosia (although sound auditory agnosia and environmental sound auditory agnosia are also commonly used). This disorder is very rare and only 18 cases have been documented. In contradiction to pure word deafness and general auditory agnosia, this disorder is likely under-diagnosed because patients are often not aware of their disorder, and thus don’t seek medical intervention.
Phonagnosia (from Ancient Greek φωνή "phone", "voice" and γνῶσις "gnosis", "knowledge") is a type of agnosia, or loss of knowledge, that involves a disturbance in the recognition of familiar voices and the impairment of voice discrimination abilities in which the affected individual does not suffer from comprehension deficits. Phonagnosia is an auditory agnosia, an acquired auditory processing disorder resulting from brain damage, other auditory agnosias include cortical deafness and auditory verbal agnosia also known as pure word deafness.