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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
           
        
There are few neuropsychological assessments that can definitively diagnose prosopagnosia. One commonly used test is the famous faces tests, where individuals are asked to recognize the faces of famous persons. However, this test is difficult to standardize. The Benton Facial Recognition Test (BFRT) is another test used by neuropsychologists to assess face recognition skills. Individuals are presented with a target face above six test faces and are asked to identify which test face matches the target face. The images are cropped to eliminate hair and clothes, as many people with prosopagnosia use hair and clothing cues to recognize faces. Both male and female faces are used during the test. For the first six items only one test face matches the target face; during the next seven items, three of the test faces match the target faces and the poses are different. The reliability of the BFRT was questioned when a study conducted by Duchaine and Nakayama showed that the average score for 11 self-reported prosopagnosics was within the normal range.
The test may be useful for identifying patients with apperceptive prosopagnosia, since this is mainly a matching test and they are unable to recognize both familiar and unfamiliar faces. They would be unable to pass the test. It would not be useful in diagnosing patients with associative prosopagnosia since they are able to match faces.
The Cambridge Face Memory Test (CFMT) was developed by Duchaine and Nakayama to better diagnose people with prosopagnosia. This test initially presents individuals with three images each of six different target faces. They are then presented with many three-image series, which contain one image of a target face and two distracters. Duchaine and Nakayama showed that the CFMT is more accurate and efficient than previous tests in diagnosing patients with prosopagnosia. Their study compared the two tests and 75% of patients were diagnosed by the CFMT, while only 25% of patients were diagnosed by the BFRT. However, similar to the BFRT, patients are being asked to essentially match unfamiliar faces, as they are seen only briefly at the start of the test. The test is not currently widely used and will need further testing before it can be considered reliable.
The 20-item Prosopagnosia Index (PI20) is a freely available and validated self-report questionnaire that is able to identify individuals with prosopagnosia. It has been validated against the famous faces test and Cambridge Face Memory Test, with evidence that PI20 scores are correlated with performance on these objective measures of face recognition. It can be downloaded from the Royal Society's Open Science website and on . Alternatively, the questionnaire can be completed online on the official website.
The nature of the alleged mental representations that underlie the act of pointing to target body parts have been a controversial issue. Originally, it was diagnosed as the effects of general mental deterioration or of aphasia on the task of pointing to body parts on verbal command. However, contemporary neuropsychological therapy seeks to establish the independence of autotopagnosia from other disorders. With such a general definition, a patient that presents with a dysfunction of or failure in accessing one of four mental representation systems suffers from autotopagnosia. Through observational testing, the type of mental misrepresentation of the body can be deduced: whether "semantic", "visuospatial", "somatosensory", or "motor misrepresentations". Neuropsychological tests can provide a proper diagnosis in regards to the specificity of patient’s agnosic condition.
1) Test 1: Body Part Localization: Free vision and no vision conditions
2) Test 2: On-line positioning of body vis-à-vis objects
3) Test 3: Localization of objects on the body surface
4) Test 4: Body part semantic knowledge
5) Test 5: Matching body parts: Effect of viewing angle
"Aphasia is usually first recognized by the physician who treats the person for his or her brain injury. Most individuals will undergo a magnetic resonance imaging (MRI) or computed tomography (CT) scan to confirm the presence of a brain injury and to identify its precise location." In circumstances where a person is showing possible signs of aphasia, the physician will refer him or her to a speech-language pathologist (SLP) for a comprehensive speech and language evaluation. SLPs will examine the individual's ability to express him or herself through speech, understand language in written and spoken forms, write independently, and perform socially.
The American Speech, Language, Hearing Association (ASHA) states a comprehensive assessment should be conducted in order to analyze the patient's communication functioning on multiple levels; as well as the effect of possible communication deficits on activities of daily living. Typical components of an aphasia assessment include: case history, self report, oral-motor examination, language skills, identification of environmental and personal factors, and the assessment results. A comprehensive aphasia assessment includes both formal and informal measures.
Formal assessments:
- Boston Diagnostic Aphasia Examination (BDAE): diagnoses the presence and type of aphasia, focusing on location of lesion and the underlying linguistic processes.
- Western Aphasia Battery - Revised (WAB): determines the presence, severity, and type of aphasia; and can also determine baseline abilities of patient.
- Communication Activities of Daily Living - Second Edition (CADL-2): measures functional communication abilities; focuses on reading, writing, social interactions, and varying levels of communication.
- Revised Token Test (RTT): assess receptive language and auditory comprehension; focuses on patient's ability to follow directions.
Informal Assessments:
Informal assessments aide in the diagnosis of patients with suspected aphasia.
- Conversational Speech and Language Sample
- Family Interview
- Case History or Medical Chart Review
- Behavioral Observations
Diagnostic information should be scored and analyzed appropriately. Treatment plans and individual goals should be developed based on diagnostic information, as well as patient and caregiver needs, desires, and priorities.
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.
One treatment thought to be effective is the repeated exposure to a particular face or object, where impaired perception may be reorganized in memory, leading to improvement on tests of imagery relative to tests of perception. The key factor for this type of treatment to be successful is a regular and consistent exposure, which will lead to improvements in the long run. Results may not be seen right away, but are eventually possible.
Ideational apraxia is a difficult disorder to diagnose. That is because the majority of individuals who have this disorder almost always have some other type of dysfunction such as agnosia or aphasia. The tests used to make an IA diagnosis can range from easy single object tasks to complex multiple object tasks. When being tested a patient may be asked to view twenty objects. They then have to demonstrate the use of each single object following three different ways of presenting the stimuli. The patient must then perform complex test where the examiner describes a task such as making coffee and the patient must show the sequential steps that makes a cup of coffee. The patients are then scored on how many errors are seen by the examiner. The errors of the patients in performing the MOT were scored according to a set of criteria partly derived from De Renzi and Lucchelli.
Another method implemented to test for aprosodia involves having questionnaires filled out by those close to the patient. The doctors and nurses taking care of a patient are also requested to fill out a questionnaire if aprosodia is suspected. This diagnosis method occurs more as an indicator that the aprosodia battery should be administered rather than being used as a singular diagnosis tool. Implementation of the questionnaire is expected to become more widespread as aprosodia is revealed to be a side-effect of more diseases.
Assessment will usually include an interview with the child’s caregiver, observation of the child in an unstructured setting, a hearing test, and standardized tests of language and nonverbal ability. There is a wide range of language assessments in English. Some are restricted for use by speech and language professionals (therapists or SALTs in the UK, speech-language pathologists, SLPs, in the US and Australia).
A commonly used test battery for diagnosis of SLI is the Clinical Evaluation of Language Fundamentals (CELF).
Assessments that can be completed by a parent or teacher can be useful to identify children who may require more in-depth evaluation.
The Grammar and Phonology Screening (GAPS) test is a quick (ten minute) simple and accurate screening test developed and standardized in the UK. It is suitable for children from 3;4 to 6;8 years;months and can be administered by professionals and non-professionals (including parents) alike, and has been demonstrated to be highly accurate (98% accuracy) in identifying impaired children who need specialist help vs non-impaired children. This makes it potentially a feasible test for widespread screening.
The Children’s Communication Checklist (CCC–2) is a parent questionnaire suitable for testing language skills in school-aged children.
Informal assessments, such as language samples, may also be used. This procedure is useful when the normative sample of a given test is inappropriate for a given child, for instance, if the child is bilingual and the sample was of monolingual children. It is also an ecologically valid measure of all aspects of language (e.g. semantics, syntax, pragmatics, etc.).
To complete a language sample, the SLP will spend about 15 minutes talking with the child. The sample may be of a conversation (Hadley, 1998), or narrative retell. In a narrative language sample, the SLP will tell the child a story using a wordless picture book (e.g. "Frog Where Are You?", Mayer, 1969), then ask the child to use the pictures and tell the story back.
Language samples are typically transcribed using computer software such as the Systematic Analysis of Language Software (SALT, Miller et al. 2012), and then analyzed. For example, the SLP might look for whether the child introduces characters to their story or jumps right in, whether the events follow a logical order, and whether the narrative includes a main idea or theme and supporting details.
Individuals with pure alexia usually have difficulty reading words as well as difficulty with identifying letters. In order to assess whether an individual has pure alexia, tests of copying and recognition must be performed. An individual with pure alexia should be able to copy a set of words, and should be able to recognize letters.
Assessment will usually include an interview with the child’s caregiver, observation of the child in an unstructured setting, a hearing test, and standardized tests of language. There is a wide range of language assessments in English. Some are restricted for use by experts in speech-language pathology: speech and language therapists (SaLTs/SLTs) in the UK, speech-language pathologists (SLPs) in the US and Australia. A commonly used test battery for diagnosis of DLD is the Clinical Evaluation of Language Fundamentals (CELF).
Assessments that can be completed by a parent or teacher can be useful to identify children who may require more in-depth evaluation. The Children’s Communication Checklist (CCC–2) is a parent questionnaire suitable for assessing everyday use of language in children aged 4 years and above who can speak in sentences.
Informal assessments, such as language samples, are often used by speech-language therapists/pathologists to complement formal testing and give an indication of the child's language in a more naturalistic context. A language sample may be of a conversation or narrative retell. In a narrative language sample, an adult may tell the child a story using a wordless picture book (e.g. Frog Where Are You?, Mayer, 1969), then ask the child to use the pictures and tell the story back. Language samples can be transcribed using computer software such as the Systematic Analysis of Language Software, and then analyzed for a range of features: e.g., the grammatical complexity of the child's utterances, whether the child introduces characters to their story or jumps right in, whether the events follow a logical order, and whether the narrative includes a main idea or theme and supporting details.
Expressive aphasia is classified as non-fluent aphasia, as opposed to fluent aphasia. Diagnosis is done on a case by case basis, as lesions often affect the surrounding cortex and deficits are highly variable among patients with aphasia.
A physician is typically the first person to recognize aphasia in a patient who is being treated for damage to the brain. Routine processes for determining the presence and location of lesion in the brain include Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) scans. The physician will complete a brief assessment of the patient's ability to understand and produce language. For further diagnostic testing, the physician will refer the patient to a speech-language pathologist, who will complete a comprehensive evaluation.
In order to diagnose a patient who is suffering from Broca’s aphasia, there are certain commonly used tests and procedures. The Western Aphasia Battery (WAB) classifies individuals based on their scores on the subtests; spontaneous speech, auditory comprehension, repetition, and naming. The Boston Diagnostic Aphasia Examination (BDAE) can inform users what specific type of aphasia they may have, infer the location of lesion, and assess current language abilities. The Porch Index of Communication Ability (PICA) can predict potential recovery outcomes of the patients with aphasia. Quality of life measurement is also an important assessment tool. Tests such as the Assessment for Living with Aphasia (ALA) and the Satisfaction with Life Scale (SWLS) allow for therapists to target skills that are important and meaningful for the individual.
In addition to formal assessments, patient and family interviews are valid and important sources of information. The patient’s previous hobbies, interests, personality, and occupation are all factors that will not only impact therapy but may motivate them throughout the recovery process. Patient interviews and observations allow professionals to learn the priorities of the patient and family and determine what the patient hopes to regain in therapy. Observations of the patient may also be beneficial to determine where to begin treatment. The current behaviors and interactions of the patient will provide the therapist with more insight about the client and his or her individual needs. Other information about the patient can be retrieved from medical records, patient referrals from physicians, and the nursing staff.
In non-speaking patients who use manual languages, diagnosis is often based on interviews from the patient's acquaintances, noting the differences in sign production pre- and post- damage to the brain. Many of these patients will also begin to rely on non-linguistic gestures to communicate, rather than signing since their language production is hindered.
Management strategies for acquired prosopagnosia, such as a person who has difficulty recognizing people's faces after a stroke, generally have a low rate of success. Acquired prosopagnosia sometimes spontaneously resolves on its own.
As autotopagnosia is not a life-threatening condition it is not on the forefront of medical research. Rather, more research is conducted regarding treatments and therapies to alleviate the lesions and traumas that can cause autotopagnosia. Of all the agnosias, visual agnosia is the most common subject of investigation because it is easiest to assess and has the most promise for potential treatments. Most autotopagnosia studies are centered on a few test subjects as part of a group of unaffected or “controlled” participants, or a simple case study. Case studies surrounding a single patient are most common due to the vague nature of the disease.
Individuals with conduction aphasia are able to express themselves fairly well, with some word finding and functional comprehension difficulty. Although people with aphasia may be able to express themselves fairly well, they tend to have issues repeating phrases, especially phrases that are long and complex. When asked to repeat something, the patient will be unable to do so without significant difficulty, repeatedly attempting to self-correct ("conduite d'approche"). When asked a question, however, patients can answer spontaneously and fluently.
Several standardized test batteries exist for diagnosing and classifying aphasias. These tests are capable of identifying conduction aphasia with relative accuracy. The Boston Diagnostic Aphasia Examination (BDAE) and the Western Aphasia Battery (WAB) are two commonly used test batteries for diagnosing conduction aphasia. These examinations involve a set of tests, which include asking patients to name pictures, read printed words, count aloud, and repeat words and non-words (such as "shwazel").
Two classes of errors are used to develop a diagnosis:
Class I: Sequence errors
- Action addition (AA) is a meaningful action step that is not necessary for accomplishing the goal of the MOT action (e.g., removing the filter of the orange squeezer in order to pour the liquid);
- Action anticipation (A) is an anticipation of an action that would normally be performed later in the action sequence (e.g., blowing the match out before using it);
- Step omission (SO) is an omission of a step of the multiple-actions sequence (e.g., inserting the filter in the coffee machine without pouring some water);
- Perseveration (P) is a repetition of an action step previously performed in the action sequence.
Class II: Conceptual errors
- Misuse (Mis) errors that can be differentiated into two further types:
1. (Mis1) the first type of misuse involves a well-performed action that is appropriate to an object different from the object target (e.g., hammering with a saw);
2. (Mis2) the second type involves an action that is appropriate at a superordinate level to the object at hand but is inappropriately specified at the subordinate level (e.g., cutting an orange with a knife as if it were butter).
- Mislocation (Misl) which can be further differentiated into two error subtypes:
1. (Misl1) the first type is an action that is appropriate to the object in hand but is performed in completely the wrong place (e.g., pouring some liquid from the bottle onto the table rather than into the glass);
2. (Misl2) the second type involves the correct general selection of the target object on which to operate with the source object or instrument in hand but with the exact location of the action being wrong (e.g., striking the match inside the matchbox).
- Tool omission (TO) is an omission in using an obligatory tool where the hand is used instead (e.g., opening a bottle without using a bottle opener);
- Pantomiming (Pant) is where the patient pantomime show the object should be used instead of using it;
- Perplexity (Perpl) Is a delay or hesitation in starting an action or subcomponents of an action;
- Toying(T) consists of a brief but repeated touching of an object or objects on the table.
As the examiner observes the patient for each task they mark off which errors were committed. From this criteria the examiner will be able to focus on severity of the dysfunction. It is important to express that the motor movement is not lost in patients with IA. Yet, at first glance their movements may appear to be awkward because they are unable to plan a sequence of movements with the given object.
Specialists, like ophthalmologists or audiologists, can test for perceptual abilities. Detailed testing is conducted, using specially formulated assessment materials, and referrals to neurological specialists is recommended to support a diagnosis via brain imaging or recording techniques. The separate stages of information processing in the object recognition model are often used to localize the processing level of the deficit.
Testing usually consists of object identification and perception tasks including:
- object-naming tasks
- object categorization or figure matching
- drawing or copying real objects or images or illustrations
- unusual views tests
- overlapping line drawings
- partially degraded or fragmented image identification
- face or feature analysis
- fine line judgment
- figure contour tracking
- visual object description
- object-function miming
- tactile ability tests (naming by touch)
- auditory presentation identification
Topographical disorientation is usually diagnosed with the use of a comprehensive battery of neuropsychological tests combined with a variety of orientation tasks performed by the participants in both virtual and real surroundings. Performance on certain tests can identify underlying neurological disorders and verify the disorientation as a selective impairment. Brain imaging is used to determine regions of brain damage, if any. Navigational skills can be assessed by tests pertaining to memory, visual-perceptual abilities, object recognition, mental rotation, imagery abilities, and spatial abilities. More direct testing of navigation involves asking the patient to describe a route, read a map, draw a map, follow a route, or point out landmarks.
Sensory modality testing allows practitioners to assess for generalized versus specific deficits, distinguishing visual agnosias from optic aphasia, which is a more generalized deficit in semantic knowledge for objects that spans multiple sensory modalities, indicating an impairment in the semantic representations themselves.
Cases with integrative agnosia appear to have medial ventral lesions in the extrastriate cortex. Those who have integrative agnosia are better able to identify inanimate than animate items, which indicates processes that lead to accurate perceptual organization of visual information can be impaired. This is attributed to the importance of perceptual updating of stored visual knowledge, which is particularly important for classes of stimuli that have many perceptual neighbors and/or stimuli for which perceptual features are central to their stored representations. Patients also show a tendency to process visual stimuli initially at a global rather than local level. Although the grouping of local elements into perceptual wholes can be impaired, patients can remain sensitive to holistic visual representations.
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.
Epidemiological surveys, in the US and Canada, estimated the prevalence of SLI in 5-year-olds at around 7 percent. However, neither study adopted the stringent 'discrepancy' criteria of the Diagnostic and Statistical Manual of Mental Disorders or ICD-10; SLI was diagnosed if the child scored below cut-off on standardized language tests, but had a nonverbal IQ of 90 or above and no other exclusionary criteria.
Treatment for aphasias is generally individualized, focusing on specific language and communication improvements, and regular exercise with communication tasks. Regular therapy for conduction aphasics has been shown to result in steady improvement on the Western Aphasia Battery. However, conduction aphasia is a mild aphasia, and conduction aphasics score highly on the WAB at baseline.
Emotional Batteries consist of asking patients to read various sentences with specific emotional indicators. Their performance is subjectively analyzed by an expert to determine if they are aprosodic. The analysis is often performed by two experts independently, with one of the judges not being present during the interview in case the patient was still able to use facial cues.
DLD is defined purely in behavioural terms: there is no biological test. There are three points that need to be met for a diagnosis of DLD:
1. The child has language difficulties that create obstacles to communication or learning in everyday life,
2. The child's language problems are unlikely to resolve by five years of age, and
3. The problems are not associated with a known biomedical condition such as brain injury, neurodegenerative conditions, genetic conditions or chromosome disorders such as Down Syndrome, sensorineural hearing loss, or Autism Spectrum Disorder or Intellectual Disability.
For research and epidemiological purposes, specific cutoffs on language assessments have been used to document the first criterion. Tomblin et al. proposed the EpiSLI criterion, based on five composite scores representing performance in three domains of language (vocabulary, grammar, and narration) and two modalities (comprehension and production). Children scoring in the lowest 10% on two or more composite scores are identified as having language disorder.
The second criterion, persistence of language problems, can be difficult to judge in a young child, but longitudinal studies have shown that difficulties are less likely to resolve for children who have poor language comprehension, rather than difficulties confined to expressive language. In addition, children with isolated difficulties in just one of the areas noted under 'subtypes' tend to make better progress than those whose language is impaired in several areas.
The third criterion specifies that DLD is used for children whose language disorder is not part of another biomedical condition, such as a genetic syndrome, a sensorineural hearing loss, neurological disease, Autism Spectrum Disorder or Intellectual Disability – these were termed 'differentiating conditions' by the CATALISE panel. Language disorders occurring with these conditions need to be assessed and children offered appropriate intervention, but a terminological distinction is made so that these cases would be diagnosed as Language Disorder associated with ___, with the main diagnosis being specified: e.g. "Language Disorder associated with Autism Spectrum Disorder." The reasoning behind these diagnostic distinctions is discussed further by Bishop (2017).
Social-emotional agnosia is generally diagnosed through the use of two tests, the Faux Pas Test and the Strange Stories Test. Both of these tests are used to show deficits in theory of mind, the recognition of mental states of others. For people with social-emotional agnosia, it is mainly the emotional states that are difficult for them to recognize. Studies have shown that subjects with amygdala damage perform poorly on both the Faux Pas test and the Strange Stories test.
The Faux Pas test measures how socially adept one is in certain situations. For this test, a faux pas is considered a statement or action that accidentally offends another person. During the test, the subject or patient is told of various social situations and later asked if one of the people in the story would be offended in the situation. A person with impaired social skills would have difficulty in detecting the faux pas made by characters in the stories.