Made by DATEXIS (Data Science and Text-based Information Systems) at Beuth University of Applied Sciences Berlin
Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
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.
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.
Visual agnosia is a broad category that refers to a deficiency in the ability to recognize visual objects. Visual agnosia can be further subdivided into two different subtypes: apperceptive visual agnosia and associative visual agnosia.
Individuals with apperceptive visual agnosia display the ability to see contours and outlines when shown an object, but they experience difficulty if asked to categorize objects. Apperceptive visual agnosia is associated with damage to one hemisphere, specifically damage to the posterior sections of the right hemisphere.
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. 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. However, individuals with prosopagnosia can recognize all other types of visual stimuli.
Associative visual agnosia is a form of visual agnosia. It is an impairment in recognition or assigning meaning to a stimulus that is accurately perceived and not associated with a generalized deficit in intelligence, memory, language or attention. The disorder appears to be very uncommon in a "pure" or uncomplicated form and is usually accompanied by other complex neuropsychological problems due to the nature of the etiology. Afflicted individuals can accurately distinguish the object, as demonstrated by the ability to draw a picture of it or categorize accurately, yet they are unable to identify the object, its features or its functions.
The two main categories of visual agnosia are:
- Apperceptive visual agnosia, impaired object recognition. Individuals with apperceptive visual agnosia cannot form a whole percept of visual information.
- Associative visual agnosia, impaired object identification. Individuals with associative agnosia cannot give a meaning to a formed percept. The percept is created, but it would have no meaning for individuals who have an associative agnosia.
There are three main types of anomia:
- Word selection anomia occurs when the patient knows how to use an object and can correctly select the target object from a group of objects, and yet cannot name the object. Some patients with word selection anomia may exhibit selective impairment in naming particular types of objects, such as animals or colors. In the subtype known as color anomia, the patient can distinguish between colors but cannot identify them by name or name the color of an object. The patients can separate colors into categories, but they cannot name them.
- Semantic anomia is a disorder in which the meaning of words becomes lost. In patients with semantic anomia, a naming deficit is accompanied by a recognition deficit. Thus, unlike patients with word selection anomia, patients with semantic anomia are unable to select the correct object from a group of objects, even when provided with the name of the target object.
- Disconnection anomia results from the severing of connections between sensory and language cortices. Patients with disconnection anomia may exhibit modality-specific anomia, where the anomia is limited to a specific sensory modality, such as hearing. For example, a patient who is perfectly capable of naming a target object when it is presented via certain sensory modalities like audition or touch, may be unable to name the same object when the object is presented visually. Thus, in such a case, the patient's anomia arises as a consequence of a disconnect between his/her visual cortex and language cortices.
Symptoms generally include memory or learning impairments, with the inability to integrate parts coherently.There is a big range to the severity of this disease and often the symptoms that are shown in each patient vary as well. As ambiguous as the general symptoms may be, patients are often treated of their respective symptoms as they appear and how critical the conditions are.
Broadly, visual agnosia is divided into apperceptive and associative visual agnosia.
Apperceptive agnosia is failure of object recognition even when the basic visual functions (acuity, color, motion) and other mental processing, such as language and intelligence, are normal. The brain must correctly integrate features such as edges, light intensity, and color from sensory information to form a complete percept of an object. If a failure occurs during this process, a percept of an object is not fully formed and thus it cannot be recognized. Tasks requiring copying, matching, or drawing simple figures can distinguish the individuals with apperceptive agnosia because they cannot perform such tasks.
Associative agnosia is an inability to identify objects even with apparent perception and knowledge of them. It involves a higher level of processing than apperceptive agnosia. Individuals with associative agnosia can copy or match simple figures, indicating that they can perceive objects correctly. They also display the knowledge of objects when tested with tactile or verbal information. However, when tested visually, they cannot name or describe common objects. This means that there is an impairment in associating the perception of objects with the stored knowledge of them.
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).
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.
Integrative agnosia is a sub-disease of agnosia, meaning the lack of integrating perceptual wholes within
knowledge. Integrative agnosia can be assessed by several experimental tests such as the Efron shape test, which
determines the specificity of the disease being Integrative.
This disease is often caused by brain trauma, producing medial ventral lesions to the extrastriate cortex. Affecting this region of the brain produces learning impairments: the inability to
integrate parts such as spatial distances or producing visual images from short or long-term memory.
Speech agnosia, or auditory verbal agnosia, refers to "an inability to comprehend spoken words despite intact hearing, speech production and reading ability". Patients report that they do indeed hear sounds being produced, but that the sounds are fundamentally unrecognizable/untranslatable.
1. EXAMINER: What did you eat for breakfast?
2. PATIENT: Breakfast, breakfast, it sounds familiar but it doesn't speak to me. (Obler & Gjerlow 1999:45)
Despite an inability to process what the speaker is saying, some patients have been reported to recognize certain characteristic information about the speaker's voice (such as being a man or woman).
Anomic aphasia (anomia) is a type of aphasia characterized by problems recalling words, names, and numbers. Speech is fluent and receptive language is not impaired in someone with anomic aphasia. Subjects often use circumlocutions (speaking in a roundabout way) in order to avoid a name they cannot recall or to express a certain word they cannot remember. Sometimes the subject can recall the name when given clues. Additionally, patients are able to speak with correct grammar; the main problem is finding the appropriate word to identify an object or person.
Sometimes subjects may know what to do with an object, but still not be able to give a name to the object. For example, if a subject is shown an orange and asked what it is called, the subject may be well aware that the object can be peeled and eaten, and may even be able to demonstrate this by actions or even verbal responses – however, they cannot recall that the object is called an "orange". Sometimes, when a person with this condition is multilingual, they might confuse the language they are speaking in trying to find the right word (inadvertent code-switching).
Auditory verbal agnosia can be referred to as a pure aphasia because it has a high degree of specificity. Despite an inability to comprehend speech, patients with auditory verbal agnosia typically retain the ability to hear and process non-speech auditory information, speak, read and write. This specificity suggests that there is a separation between speech perception, non-speech auditory processing, and central language processing. In support of this theory, there are cases in which speech and non-speech processing impairments have responded differentially to treatment. For example, some therapies have improved writing comprehension in patients over time, while speech remained critically impaired in those same patients.
The term "pure word deafness" is something of a misnomer. By definition, individuals with pure word deafness are not deaf – in the absence of other impairments, these individuals have normal hearing for all sounds, including speech. The term "deafness" originates from the fact that individuals with AVA are unable to "comprehend" speech that they hear. The term "pure word" refers to the fact that comprehension of verbal information is selectively impaired in AVA. For this reason, AVA is distinct from other auditory agnosias in which the recognition of nonspeech sounds is impaired. Classical (or pure) auditory agnosia is an inability to process environmental sounds. Interpretive or receptive agnosia (amusia) is an inability to understand music.
Patients with pure word deafness complain that speech sounds simply do not register, or that they tend not to come up. Other claims include speech sounding as if it were in a foreign language, the words having a tendency to run together, or the feeling that speech was simply not connected to the patient's voice.
The following are common symptoms seen in patients with Wernicke's aphasia:
Impaired Comprehension: deficits in understanding (receptive) written and spoken language. This is because Wernicke's area is responsible for assigning meaning to the language that is heard, so if it is damaged, the brain cannot comprehend the information that is being received.
Poor Word Retrieval: ability to retrieve target words is impaired. This is also referred to as Anomia.
Fluent Speech: individuals with Wernicke's aphasia do not have difficulty with producing connected speech that flows.. Although the connection of the words may be appropriate, the words they are using may not belong together or make sense (see Production of Jargon below).
Production of Jargon: speech that lacks content, consists of typical intonation, and is structurally intact. Jargon can consist of a string of neologisms, as well as a combination of real words that do not make sense together in context.
Awareness: Individuals with Wernicke's aphasia are often not aware of their incorrect productions, which would further explain why they do not correct themselves when they produce jargon, paraphasias, or neologisms.
Paraphasias:
- Phonemic (Literal) Paraphasias: involves the substitution, addition, or rearrangement of sounds so that an error can be defined as sounding like the target word. Often, half of the word is still intact which allows for easy comparison to the appropriate, original word.
- Ex: "bap" for "map"
- Semantic (Verbal) Paraphasias: saying a word that is related to the target word in meaning or category; frequently observed in Wernicke's aphasia.
- Ex: "jet" for "airplane" or "knife" for "fork"
Neologisms: nonwords that have no relation to the target word.
- Ex: "dorflur" for "shoe"
Circumlocution: talking around the target word.
- Ex: "uhhh it's white...it's flat...you write on it…" (when referencing paper)
Press of speech: run-on speech.
- If a clinician asks, "what do you do at a supermarket?" And the individual responds with "Well, the supermarket is a place. It is a place with a lot of food. My favorite food is italian food. At a supermarket, I buy different kinds of food. There are carts and baskets. Supermarkets have lots of customers, and workers…."
Lack of Hemiparesis: typically, no motor deficits are seen with a localized lesion in Wernicke's area.
Reduced Retention Span: reduced ability to retain information for extended periods of time.
Impairments in reading and writing: impairments can be seen in both reading and writing with differing severity levels.
How to Differentiate from Other Types of Aphasia.
- Expressive Aphasia (non-fluent Broca's Aphasia): individuals have great difficulty forming complete sentences with generally only basic content words (leaving out words like "is" and "the").
- Global Aphasia: individuals have extreme difficulties with both expressive (producing language) and receptive (understanding language).
- Anomic Aphasia: the biggest hallmark is an individuals poor word finding abilities; their speech is fluent and appropriate, but full of circumlocutions (evident in both writing and speech).
- Conduction Aphasia: individual can comprehend what is being said and is fluent in spontaneous speech, but they cannot repeat what is being said to them.
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 a neurological inability of the brain to process sound meaning. It is a disruption of the "what" pathway in the brain. 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. Auditory agnosia is caused by damage to the secondary and tertiary auditory cortex of the temporal lobe of the brain.
There are three primary distinctions of auditory agnosia that fall into two categories.
There is no uniform performance among patients with auditory verbal agnosia; therefore it is not possible to attribute specific phonetic or phonological deficits to the syndrome. In order to diagnose AVA, two intact abilities need to be established:
- Words that are heard must have undergone adequate acoustic analysis as evidenced by correct repetition;
- The semantic representation of the word must be intact as evidenced by immediate comprehension of the word when presented in written form.
If both of these criteria are met "and" lack of auditory verbal comprehension is apparent, a diagnosis of AVA may follow.
In at least one instance, the Boston Diagnostic Aphasia Examination has been used to profile AVA. This method was able to show that the patient experienced marked difficulty in speech perception with minor to no minor deficits in production, reading, and writing, fitting the profile of AVA. While this provides a well-known example, other verbal-audio test batteries can and have also been used to diagnose pure speech deafness.
The syndrome rarely presents itself the same way in every patient. Some symptoms that occur may be:
- Constructional apraxia: difficulty in constructing: drawing, copying, designs, copying 3D models
- Topographical disorientation: difficulty finding one's way in the environment
- Optic ataxia: deficit in visually-guided reaching
- Ocular motor apraxia: inability to direct gaze, a breakdown (failure) in starting (initiating) fast eye movements
- Dressing apraxia: difficulty in dressing usually related to inability to orient clothing spatially, and to a disrupted awareness of body parts and the position of the body and its parts in relation to themselves and objects in the environment
- Right-left confusion: difficulty in distinguishing the difference between the directions left and right
Wernicke's aphasia, also known as receptive aphasia, sensory aphasia, or posterior aphasia, is a type of aphasia in which individuals have difficulty understanding written and spoken language. Patients with Wernicke's aphasia demonstrate fluent speech, which is characterized by typical speech rate, intact syntactic abilities, and effortless speech output. Writing often reflects speech in that it tends to lack content or meaning. In most cases, motor deficits (i.e. hemiparesis) do not occur in individuals with Wernicke's aphasia. Therefore, they may produce a large amount of speech without much meaning. Wernicke's aphasia was named after Carl Wernicke who is credited with discovering the area of the brain responsible for language comprehension. Individuals with Wernicke's aphasia are typically unaware of their errors in speech and do not realize their speech may lack meaning. They typically remain unaware of even their most profound language deficits.
Like many acquired language disorders, Wernicke's aphasia can be experienced in many different ways and to many different degrees. Patients diagnosed with Wernicke's aphasia can show severe language comprehension deficits; however, this is dependent on the severity and extent of the lesion. Severity levels may range from being unable to understand even the simplest spoken and/or written information to missing minor details of a conversation. Many diagnosed with Wernicke's aphasia have difficulty with repetition in words and sentences, and or working memory.
Patients with autotopagnosia exhibit an inability to locate parts of their own body, the body of an examiner’s, or the parts of a representation of a human body. Deficiencies can be in localizing parts of a certain area of the body, or the entire body.
Some patients demonstrating the symptoms of autotopagnosia have a decreased ability to locate parts of other multipart object. Patients are considered to suffer from “pure” autotopagnosia, however, if their deficiency is specific to body part localization. Patients suffering from “pure” autotopagnosia often have no problems carrying out tasks involved in everyday life that require body part awareness. Patients have difficulty locating body parts when directly asked, but can carry out activities such as putting on pants without difficulty. Patients can describe the function and appearance of body parts, yet they are still unable to locate them.
Damage to the left parietal lobe can result in what is called Gerstmann syndrome. It can include right-left confusion, a difficulty with writing Agraphia and a difficulty with mathematics Acalculia. In addition, it can also produce language deficiencies Aphasia and an inability to recognize objects normally Agnosia.
Other related disorders include:
- Apraxia: an inability to perform skilled movements despite understanding of the movements and intact sensory and motor systems.
- Finger agnosia: An inability to name the fingers, move a specific finger upon being asked, and/or recognize which finger has been touched when an examiner touches one.
Topographical disorientation, also known as topographical agnosia and topographagnosia, is the inability to orient oneself in one's surroundings as a result of focal brain damage. This disability may result from the inability to make use of selective spatial information (e.g., environmental landmarks) or to orient by means of specific cognitive strategies such as the ability to form a mental representation of the environment, also known as a cognitive map. It may be part of a syndrome known as visuospatial dysgnosia.
Central agraphia occurs when there are both impairments in spoken language and impairments to the various motor and visualization skills involved in writing. Individuals who have agraphia with fluent aphasia write a normal quantity of well-formed letters, but lack the ability to write meaningful words. Receptive aphasia is an example of fluent aphasia. Those who have agraphia with nonfluent aphasia can write brief sentences but their writing is difficult to read. Their writing requires great physical effort but lacks proper syntax and often has poor spelling. Expressive aphasia is an example of nonfluent aphasia. Individuals who have Alexia with agraphia have difficulty with both the production and comprehension of written language. This form of agraphia does not impair spoken language.
- Deep agraphia affects an individuals' phonological ability and orthographic memory. Deep agraphia is often the result of a lesion involving the left parietal region (supramarginal gyrus or insula). Individuals can neither remember how words look when spelled correctly, nor sound them out to determine spelling. Individuals typically rely on their damaged orthographic memory to spell; this results in frequent errors, usually semantic in nature. Individuals have more difficulty with abstract concepts and uncommon words. Reading and spoken language are often impaired as well.
- Gerstmann syndrome agraphia is the impairment of written language production associated with the following structural symptoms: difficulty discriminating between one's own fingers, difficulty distinguishing left from right, and difficulty performing calculations. All four of these symptoms result from pathway lesions. Gerstmann's syndrome may additionally be present with alexia and mild aphasia.
- Global agraphia also impairs an individuals' orthographic memory although to a greater extent than deep agraphia. In global apraxia, spelling knowledge is lost to such a degree that the individual can only write very few meaningful words, or cannot write any words at all. Reading and spoken language are also markedly impaired.
- Lexical and structural agraphia are caused by damage to the orthographic memory; these individuals cannot visualize the spelling of a word, though they do retain the ability to sound them out. This impaired spelling memory can imply the loss or degradation of the knowledge or just an inability to efficiently access it. There is a regularity effect associated with lexical agraphia in that individuals are less likely to correctly spell words without regular, predictable spellings. Additionally, spelling ability tends to be less impaired for common words. Individuals also have difficulty with homophones. Language competence in terms of grammar and sentence writing tends to be preserved.
- Phonological agraphia is the opposite of lexical agraphia in that the ability to sound out words is impaired, but the orthographical memory of words may be intact. It is associated with a lexicality effect by a difference in the ability to spell words versus nonwords; individuals with this form of agraphia are depending on their orthographic memory. Additionally, it is often harder for these individuals to access more abstract words without strong semantic representations (i.e., it is more difficult for them to spell prepositions than concrete nouns).
- Pure agraphia is the impairment in written language production without any other language or cognitive disorder.
Agraphia can occur separately or co-occur and can be caused by damage to the angular gyrus
Peripheral agraphias occurs when there is damage to the various motor and visualization skills involved in writing.
- Apraxic agraphia is the impairment in written language production associated with disruption of the motor system. It results in distorted, slow, effortful, incomplete, and/or imprecise letter formation. Though written letters are often so poorly formed that they are almost illegible, the ability to spell aloud is often retained. This form of agraphia is caused specifically by a loss of specialized motor plans for the formation of letters and not by any dysfunction affecting the writing hand. Apraxic agraphia may present with or without ideomotor apraxia. Paralysis, chorea, Parkinson's disease (micrographia), and dystonia (writer's cramp) are motor disorders commonly associated with agraphia.
- Hysterical agraphia is the impairment in written language production caused by a conversion disorder.
- Reiterative agraphia is found in individuals who repeat letters, words, or phrases in written language production an abnormal number of times. Preservation, paragraphia, and echographia are examples of reiterative agraphia.
- Visuospatial agraphia is the impairment in written language production defined by a tendency to neglect one portion (often an entire side) of the writing page, slanting lines upward or downward, and abnormal spacing between letters, syllables, and words. The orientation and correct sequencing of the writing will also be impaired. Visuospatial agraphia is frequently associated with left hemispatial neglect, difficulty in building or assembling objects, and other spatial difficulties.
Impaired verbal comprehension can be the result a number of causes such as failure of speech sound discrimination, word recognition, auditory working memory, or syntactic structure building. When clinically examined, patients with TSA will exhibit poor comprehension of verbal commands. Based on the extent of the comprehension deficiency, patients will have difficulty following simple commands, e.g. “close your eyes.” Depending on the extent of affected brain area, patients are able to follow simple commands but may not be able to comprehend more difficult, multistep commands, e.g. “point to the ceiling, then touch your left ear with your right hand." Verbal commands as such, that require the patient to cross over the midline of their body are typically more taxing than commands that involve solely the right or left side. When increasing the complexity of verbal commands comprehension is often tested by varying the grammatical structure of the command to determine whether or not the patient understands different grammatical variations of the same sentence. Commands involving the passive voice or possessive, e.g. "If the snake killed the mouse, which one is still alive," usually result in comprehension problems in those who can understand simple questions.
Autotopagnosia from the Greek "a" and "gnosis," meaning "without knowledge", "topos" meaning "place", and "auto" meaning "oneself", autotopagnosia virtually translates to the "lack of knowledge about one's own space," and is clinically described as such.
Autotopagnosia is a form of agnosia, characterized by an inability to localize and orient different parts of the body. The psychoneurological disorder has also been referred to as "body-image agnosia" or "somatotopagnosia." "Somatotopagnosia" has been argued to be a better suited term to describe the condition. While autotopagnosia emphasizes the deficiencies in localizing only one's own body parts and orientation, "somatotopagnosia" also considers the inability to orient and recognize the body parts of others or representations of the body (e.g., manikins, diagrams).
Typically, the cause of autotopagnosia is a lesion found in the parietal lobe of the left hemisphere of the brain. However, it as also been noted that patients with generalized brain damage present with similar symptoms of autotopagnosia.
As a concept, autotopagnosia has been criticized as nonspecific; some claim that this is a manifestation of a greater symptomatic complex of anomia, marked by an inability to name things in general—not just parts of the human body.
Naming involves the ability to recall an object. Patients with TSA, as well as patients with all other aphasia subtypes, exhibit poor naming. Clinical assessment of naming involves the observer first asking the patient to name high frequency objects such as clock, door, and chair. TSA patients who name common objects with ease generally have difficulty naming both uncommon objects and specific parts of objects such as lapel, or the dial on a watch.