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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)
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There are clinical trials being done to further research for treatments. At the National Institute of Neurological Disorders and Stroke (NINDS) they support research for rare diseases like agnosia. Some organizations that are recruiting for trials are using clincaltrials.gov and give status updates on the trials.
These strategies elicit the use of an unaffected modality. For example, visual agnosics can use tactile information in replacement of visual information. Alternatively, an individual with prosopagnosia can use auditory information in order to replace visual information. For example, an individual with prosopagnosia can wait for someone to speak, and will usually recognize the individual from their speech.
The affected individual may not realize that they have a visual problem and may complain of becoming "clumsy" or "muddled" when performing familiar tasks such as setting the table or simple DIY.
Anosognosia, a lack of awareness of the deficit, is common and can cause therapeutic resistance. In some agnosias, such as prosopagnosia, awareness of the deficit is often present; however shame and embarrassment regarding the symptoms can be a barrier in admission of a deficiency. Because agnosias result from brain lesions, no direct treatment for them currently exists, and intervention is aimed at utilization of coping strategies by patients and those around them. Sensory compensation can also develop after one modality is impaired in agnostics
General principles of treatment:
- restitution
- repetitive training of impaired ability
- development of compensatory strategies utilizing retained cognitive functions
Partial remediation is more likely in cases with traumatic/vascular lesions, where more focal damage occurs, than in cases where the deficit arises out of anoxic brain damage, which typically results in more diffuse damage and multiple cognitive impairments. However, even with forms of compensation, some afflicted individuals may no longer be able to fulfill the requirements of their occupation or perform common tasks, such as, eating or navigating. Agnostics are likely to become more dependent on others and to experience significant changes to their lifestyle, which can lead to depression or adjustment disorders.
Following are some precautions that should be taken to avoid aphasia, by decreasing the risk of stroke, the main cause of aphasia:
- Exercising regularly
- Eating a healthy diet
- Keeping alcohol consumption low and avoiding tobacco use
- Controlling blood pressure
There is no method available to completely cure anomic aphasia. However, there are treatments to help improve word-finding skills.
Although a person with anomia may find it difficult to recall many types of words such as common nouns, proper nouns, verbs, etc., many studies have shown that treatment for object words, or nouns, has shown promise in rehabilitation research. The treatment includes visual aids, such as pictures, and the patient is asked to identify the object or activity. However, if that is not possible, then the patient is shown the same picture surrounded by words associated with the object or activity. Throughout the process, positive encouragement is provided. The treatment shows an increase in word-finding during treatment; however, word identifying decreased two weeks after the rehabilitation period. Therefore, it shows that rehabilitation effort needs to be continuous for word-finding abilities to improve from the baseline. The studies show that verbs are harder to recall or repeat, even with rehabilitation.
Other methods in treating anomic aphasia include Circumlocution Induced Naming therapy (CIN), wherein the patient uses circumlocution to assist with his or her naming rather than just being told to name the item pictured after given some sort of cue. Results suggest that the patient does better in properly naming objects when undergoing this therapy because CIN strengthens the weakened link between semantics and phonology for patients with anomia, since they often know what an object is used for but cannot verbally name it.
Anomia is often burdensome on the families and friends of those suffering from it. One way to overcome this burden is computer-based treatment models, effective especially when used with clinical therapy. Leemann et al. provided anomic patients with computerized-assisted therapy (CAT) sessions, along with traditional therapy sessions using treatment lists of words. Some of the patients received a drug known to help relieve symptoms of anomia (levodopa) while others received a placebo. The researchers found that the drug had no significant effects on improvement with the treatment lists, but almost all of the patients improved after the CAT sessions. They concluded that this form of computerized treatment is effective in increasing naming abilities in anomic patients.
Additionally, one study researched the effects of using "excitatory (anodal) transcranial direct current stimulation" over the right temporo-parietal cortex, a brain area that seems to correlate to language. The electrical stimulation seemed to enhance language training outcome in patients with chronic aphasia.
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.
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 only way to treat aphasia is with speech and language therapy (SLT). It will not completely restore the person’s prior level of communication, but SLT can lead to a massive improvement of jargon aphasia. Recipients of this treatment typically achieve better use of residual language abilities, improved language skills, and the ability to communicate in a different way by making up for missing words in their speech[2].
One specific method that has shown to lead to improvements with certain symptoms is “phonological component analysis”, or PCA for short. Participants in PCA therapy tend to improve in the ability to name specific items that they are test on, as well as the decrease in use of nonwords to describe said items. Seeing promising results from this type of therapy has led to much optimism in hopes of developing more treatment methods for jargon aphasia[6].
When addressing Wernicke’s aphasia, according to Bakheit et al. (2007), the lack of awareness of the language impairments, a common characteristic of Wernicke’s aphasia, may impact the rate and extent of therapy outcomes. Klebic et al. (2011) suggests that people benefit from continuing therapy upon discharge from the hospital to ensure generalization. Robey (1998) determined that at least 2 hours of treatment per week is recommended for making significant language gains. Spontaneous recovery may cause some language gains, but without speech-language therapy, the outcomes can be half as strong as those with therapy.
When addressing Broca’s aphasia, better outcomes occur when the person participates in therapy, and treatment is more effective than no treatment for people in the acute period. Two or more hours of therapy per week in acute and post-acute stages produced the greatest results. High intensity therapy was most effective, and low intensity therapy was almost equivalent to no therapy.
People with global aphasia are sometimes referred to as having irreversible aphasic syndrome, often making limited gains in auditory comprehension, and recovering no functional language modality with therapy. With this said, people with global aphasia may retain gestural communication skills that may enable success when communicating with conversational partners within familiar conditions. Process-oriented treatment options are limited, and people may not become competent language users as readers, listeners, writers, or speakers no matter how extensive therapy is. However, people’s daily routines and quality of life can be enhanced with reasonable and modest goals. After the first month, there is limited to no healing to language abilities of most people. There is a grim prognosis leaving 83% who were globally aphasic after the first month they will remain globally aphasic at the first year. Some people are so severely impaired that their existing process-oriented treatment approaches offer signs of progress, and therefore cannot justify the cost of therapy.
Perhaps due to the relative rareness of conduction aphasia, few studies have specifically studied the effectiveness of therapy for people with this type of aphasia. From the studies performed, results showed that therapy can help to improve specific language outcomes. One intervention that has had positive results is auditory repetition training. Kohn et al. (1990) reported that drilled auditory repetition training related to improvements in spontaneous speech, Francis et al. (2003) reported improvements in sentence comprehension, and Kalinyak-Fliszar et al. (2011) reported improvements in auditory-visual short-term memory.
Most acute cases of aphasia recover some or most skills by working with a speech-language pathologist. Recovery and improvement can continue for years after the stroke. After the onset of Aphasia, there is approximately a six-month period of spontaneous recovery; during this time, the brain is attempting to recover and repair the damaged neurons. Improvement varies widely, depending on the aphasia's cause, type, and severity. Recovery also depends on the person's age, health, motivation, handedness, and educational level.
There is no one treatment proven to be effective for all types of aphasias. The reason that there is no universal treatment for aphasia is because of the nature of the disorder and the various ways it is presented, as explained in the above sections. Aphasia is rarely exhibited identically, implying that treatment needs to be catered specifically to the individual. Studies have shown that, although there is no consistency on treatment methodology in literature, there is a strong indication that treatment in general has positive outcomes. Therapy for aphasia ranges from increasing functional communication to improving speech accuracy, depending on the person's severity, needs and support of family and friends. Group therapy allows individuals to work on their pragmatic and communication skills with other individuals with aphasia, which are skills that may not often be addressed in individual one-on-one therapy sessions. It can also help increase confidence and social skills in a comfortable setting.
Evidence dose not support the use of transcranial direct current stimulation (tDCS) for improving aphasia after stroke.
Specific treatment techniques include the following:
- Copy and Recall Therapy (CART) - repetition and recall of targeted words within therapy may strengthen orthographic representations and improve single word reading, writing, and naming
- Visual Communication Therapy (VIC) - the use of index cards with symbols to represent various components of speech
- Visual Action Therapy (VAT) - typically treats individuals with global aphasia to train the use of hand gestures for specific items
- Functional Communication Treatment (FCT) - focuses on improving activities specific to functional tasks, social interaction, and self-expression
- Promoting Aphasic's Communicative Effectiveness (PACE) - a means of encouraging normal interaction between people with aphasia and clinicians. In this kind of therapy the focus is on pragmatic communication rather than treatment itself. People are asked to communicate a given message to their therapists by means of drawing, making hand gestures or even pointing to an object
- Melodic Intonation Therapy (MIT) - aims to use the intact melodic/prosodic processing skills of the right hemisphere to help cue retrieval of words and expressive language
- Other - i.e. drawing as a way of communicating, trained conversation partners
Semantic feature analysis (SFA) -a type of aphasia treatment that targets word-finding deficits. It is based on the theory that neural connections can strengthened by using using related words and phrases that are similar to the target word, to eventually activate the target word in the brain. SFA can be implemented in multiple forms such as verbally, written, using picture cards, etc. The SLP provides prompting questions to the individual with aphasia in order for the person to name the picture provided. Studies show that SFA is an effective intervention for improving confrontational naming.
Melodic intonation therapy is used to treat non-fluent aphasia and has proved to be effective in some cases. However, there is still no evidence from randomized controlled trials confirming the efficacy of MIT in chronic aphasia. MIT is used to help people with aphasia vocalize themselves through speech song, which is then transferred as a spoken word. Good candidates for this therapy include people who have had left hemisphere strokes, non-fluent aphasias such as Broca's, good auditory comprehension, poor repetition and articulation, and good emotional stability and memory. An alternative explanation is that the efficacy of MIT depends on neural circuits involved in the processing of rhythmicity and formulaic expressions (examples taken from the MIT manual: “I am fine,” “how are you?” or “thank you”); while rhythmic features associated with melodic intonation may engage primarily left-hemisphere subcortical areas of the brain, the use of formulaic expressions is known to be supported by right-hemisphere cortical and bilateral subcortical neural networks.
According to the National Institute on Deafness and Other Communication Disorders (NIDCD), involving family with the treatment of an Aphasic loved one is ideal for all involved, because while it will no doubt assist in their recovery, it will also make it easier for members of the family to learn how best to communicate with them.
Visual agnosia is an impairment in recognition of visually presented objects. It is not due to a deficit in vision (acuity, visual field, and scanning), language, memory, or low intellect. While cortical blindness results from lesions to primary visual cortex, visual agnosia is often due to damage to more anterior cortex such as the posterior occipital and/or temporal lobe(s) in the brain. There are two types of visual agnosia: apperceptive agnosia and associative agnosia.
Recognition of visual objects occurs at two primary levels. At an apperceptive level, the features of the visual information from the retina are put together to form a perceptual representation of an object. At an associative level, the meaning of an object is attached to the perceptual representation and the object is identified. If a person is unable to recognize objects because they cannot perceive correct forms of the objects, although their knowledge of the objects is intact (i.e. they do not have anomia), they have apperceptive agnosia. If a person correctly perceives the forms and has knowledge of the objects, but cannot identify the objects, they have associative agnosia.
Treating auditory verbal agnosia with intravenous immunoglobulin (IVIG) is controversial because of its inconsistency as a treatment method. Although IVIG is normally used to treat immune diseases, some individuals with auditory verbal agnosia have responded positively to the use of IVIG. Additionally, patients are more likely to relapse when treated with IVIG than other pharmacological treatments. IVIG is, thus, a controversial treatment as its efficacy in treating auditory verbal agnosia is dependent upon each individual and varies from case to case.
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.
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.
Speech and language therapy is typically the primary treatment for individuals with aphasia. The goal of speech and language therapy is to increase the person’s communication abilities to a level functional for daily life. Goals are chosen based on collaboration between speech language pathologists, patients, and their family/caregivers. Goals should be individualized based on the person’s aphasia symptoms and communicative needs. In 2016, Wallace et al. found the following outcomes were commonly prioritized in therapy: communication, life participation, physical and emotional well-being, normalcy, and health and support services. However, available research is inconclusive about which specific approach to speech and language therapy is most effective in treating global aphasia.
Therapy can be either group or individual. Group therapies that integrate the use of visual aids allow for enhanced social and communication-skill development. Group therapy sessions typically revolve around simple, preplanned activities or games, and aim to facilitate social communication.
One particular therapy designed specifically for treatment of aphasia is Visual Action Therapy (VAT). VAT is a non-verbal gestural output program with 3 phases and 30 total steps. The program teaches unilateral gestures as symbolic representations of real life objects. Research on the effectiveness of VAT is limited and inconclusive.
One important therapy technique includes teaching family members and caregivers strategies for more effectively communicating with their loved ones. Research offers such strategies including, simplifying sentences and using common words, gaining the person's attention before speaking, using pointing and visual cues, allowing for adequate response time, and creating a quiet environment free of distractions.
Another approach to speech and language treatment is constraint-induced language therapy (CILT). CILT involves teaching the patient to use speech in small segments but avoid using gestures and familiar words . The speech language pathologist provides positive feedback throughout and ignores any mistakes made by the patient. The intensity with which this treatment is provided has been debated in the literature. One study, performed in 2015, compared the outcomes of patients with aphasia who received CILT for either 30 hours total over 2 weeks or 30 hours distributed over 10 weeks. Results showed that both groups made significant speech and language improvements. Overall, CILT is an effective treatment at a variety of intensities.
Research supporting the efficacy of pharmacological treatments for aphasia is limited. To date, no large scale clinical trials have proven benefits of pharmacological treatment.
As autotopagnosia arises from neurological and irreversible damage, options regarding symptom reversal or control are limited. As of April 2010, there are no known specific treatments for autotopagnosia.
No medications or pharmaceutical remedies have been approved by the U.S. Food and Drug Administration to treat or cure autotopagnosia. There have been cases in which extensive rehabilitation has been beneficial following restitution, repetitive training to correct the impaired function, and compensation of other skills to make up for the deficit. Rehabilitation is not a definitive treatment and only shows signs of slight improvement in a small percentage of autotopagnosia patients. The condition of the disease can be monitored with continued neurological examination and using a CT scan to note the progression of the parietal lesion.
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.
Transient paraphasias (as well as other language defects such as speech arrest) can be generated by artificially activating the brain's language network with Transcranial magnetic stimulation (TMS). With navigated TMS (nTMS), nodes of the language network can be located presurgically so that critical areas can be saved when performing tumor or epilepsy surgery. Marketed by Nexstim, this method has received Food and Drug Administration (FDA) clearance in the United States.
In incidents where tumors and their pressure effects are the cause of pure word deafness, removal of the tumor has been shown to allow for the return of most auditory verbal comprehension.
Many language impairments, including paraphasic errors, are reduced in number through spontaneous recovery of neurological function; this occurs most often with stroke patients within the first three months of recovery. Lesions associated with ischemic strokes have a shorter spontaneous recovery time, within the first two weeks, and lesions associated with hemorrhagic strokes, on the other hand have a longer period for spontaneous recovery, four to eight weeks. Whether spontaneous recovery occurs or not, treatment must begin immediately after the stroke. A traditional approach requires treatment beginning at the level of breakdown - in the case of paraphasia, at the level of the phoneme. There are commercially available workbooks that provide various activities such as letter, word-picture, or word-word matching, and sentence completion, among other things. The difficulty of these activities varies with the level of treatment. However, these treatments have not been proven to be clinically productive. Functional magnetic resonance imaging is the most widely used technique to study treatment-induced recovery, looking at activation of particular areas of the brain. There are many different ways to process fMRI scans, beginning with the pre-scanning process. Data must be normalized. There is also no consensus on whether or not single subject scans are more helpful than group scans to determine a general pattern of treatment. However, fMRI scans have a few disadvantages.
A 1988 study by Mary Boyle proposed a method focused on oral reading to treat phonemic paraphasias was partially successful, resulting in fewer phonemic paraphasias but a slower rate of speech. Treatments lasted for 50 minutes and occurred once a week. During these treatment sessions, the patient was instructed to look at twenty different phrases -each of these phrases consisted of one to three syllables - then read the phrase. If the patient failed to read the phrase, the process was repeated. If the patient failed to read the phrase again, the process was abandoned. To progress from a set of one syllable phrases to two syllable phrases and two syllable phrases to three syllable phrases, an 80% success rate was necessary. This treatment was partially successful. Although fewer phonemic paraphasias were produced due to this treatment, speaking efficiency was not improved by this study. This is partially because the focus of the treatment was on sound production rather than semantic content. Improvements lasted for six weeks before the patient regressed.
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.
Due to advances in modern neuroimaging, scientists have been able to gain a better understanding of how language is learned and comprehended. Based on the new data from the world of neuroscience, improvements can be made in coping with the disorder.
Therapists have been developing multiple methods of improving speech and comprehension. These techniques utilize three general principles: maximizing therapy occurrences, ensuring behavioral and communicative relevance, and allowing patients to focus on the language tools that are still available in his or her repertoire.
Many of the following treatment techniques are used to improve auditory comprehension in patients with aphasia:
- Use common words
- Using concrete nouns is more effective than using adjectives, adverbs, or verbs
- Using action verbs that are easily imagined
- Concise and grammatically simple sentences as opposed to lengthy sentences
- Speaking slowly, repeating oneself several times when conversing with patients who are aphasic
- Using gestures
A relatively new method of language therapy involves coincidence learning. Coincidence learning focuses on the simultaneous learning of two or more events and stipulates that these events are wired together in the brain, strengthening the learning process. Therapists use coincidence learning to find and improve language correlations or coincidences that have been either damaged or deleted by severe cases of aphasia, such as transcortical sensory aphasia. This technique is important in brain function and recovery, as it strengthens associated brain areas that remain unaffected after brian damage. It can be achieved with intensive therapy hours in order to maximize time where correlation is emphasized.
Through careful analysis of neuroimaging studies, a correlation has been developed with motor function and the understanding of action verbs. For example, leg and motor areas were seen to be activated words such as "kick", leading scientists to understand the connection between motor and language processes in the brain. This is yet another example of using relationships that are related in the brain for the purpose of rehabilitating speech and comprehension.
Of huge importance in aphasia therapy is the need to start practicing as soon as possible. Greater recovery occurs when a patient attempts to improve their comprehension and speaking soon after aphasia occurs. There is an inverse relationship between the length of time spent not practicing and level of recovery. The patient should be pushed to their limits of verbal communication in order for them to practice and build upon their remaining language skills.
One effective therapy technique is using what are known as language games in order to encourage verbal communication. One famous example is known as "Builder's Game", where a 'builder' and a 'helper' must communicate in order to effectively work on a project. The helper must hand the builder the tools he or she may need, which requires effective oral communication. The builder succeeds by requesting tools from the assistant by usually using single word utterances, such as 'hammer' or 'nail'. Thus, when the helper hands the tool to the builder, the game incorporates action with language, a key therapy technique. The assistant would then hand the builder the requested tool. Success of the game occurs when the builder's requests are specific to ensure successful building.
Ultimately, regardless of therapy plan or method, improvement in speech does not appear overnight; it requires a significant time investment by the patient as well as a dedicated speech therapist seeking to ensure that the patient is focusing on the correct speech tasks outside of the clinic. Furthermore, the patient must collaborate with friends and family members during their free time in order to maximize the efficacy of the treatment.
Anomic aphasia (also known as dysnomia, nominal aphasia, and amnesic aphasia) is a mild, fluent type of aphasia where an individual has word retrieval failures and cannot express the words they want to say (particularly nouns and verbs). Anomia is a deficit of expressive language. The most pervasive deficit in the aphasias is anomia. Some level of anomia is seen in all of the aphasias. Individuals with aphasia who display anomia can often describe an object in detail and maybe even use hand gestures to demonstrate how the object is used but cannot find the appropriate word to name the object.
There are three primary distinctions of auditory agnosia that fall into two categories.
"Developmental prosopagnosia" (DP), also called "Congenital prosopagnosia" (CP), is a face-recognition deficit that is lifelong, manifesting in early childhood, and that cannot be attributed to acquired brain damage. A number of studies have found functional deficits in DP both on the basis of EEG measures and fMRI. It has been suggested that a genetic factor is responsible for the condition. The term "hereditary prosopagnosia" was introduced if DP affected more than one family member, essentially accenting the possible genetic contribution of this condition. To examine this possible genetic factor, 689 randomly selected students were administered a survey in which seventeen developmental prosopagnosics were quantifiably identified. Family members of fourteen of the DP individuals were interviewed to determine prosopagnosia-like characteristics, and in all fourteen families, at least one other affected family member was found.
In 2005, a study led by Ingo Kennerknecht showed support for the proposed congenital disorder form of prosopagnosia. This study provides epidemiological evidence that congenital prosopagnosia is a frequently occurring cognitive disorder that often runs in families. The analysis of pedigree trees formed within the study also indicates that the segregation pattern of hereditary prosopagnosia (HPA) is fully compatible with autosomal dominant inheritance. This mode of inheritance explains why HPA is so common among certain families (Kennerknecht et al. 2006).
There are many developmental disorders associated with an increased likelihood that the person will have difficulties in face perception, of which the person may or may not be aware. The mechanism by which these perceptual deficits take place is largely unknown. A partial list of some disorders that often have prosopagnosiac components would include nonverbal learning disorder, Alzheimer's disease, and autism in general. However, these types of disorders are very complicated, so arbitrary assumptions should be avoided.
In 2012, it was shown that developmental prosopagnosia cases show poor integration of low and high spatial frequency information.
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.