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
Treatment for the disease itself is nonexistent, but there are options for most of the symptoms. For example, one suffering from hearing loss would be given hearing aids, and those with Hirschsprung’s disorder can be treated with a colostomy.
If the Hirschsprung's disease is treated in time, ABCD sufferers live otherwise healthy lives. If it is not found soon enough, death often occurs in infancy. For those suffering hearing loss, it is generally regressive and the damage to hearing increases over time. Digestive problems from the colostomy and reattachment may exist, but most cases can be treated with laxatives. The only other debilitating symptom is hearing loss, which is usually degenerative and can only be treated with surgery or hearing aids.
There is no cure for individuals with DES, but there are therapies to help them cope with their symptoms. DES can affect a number of functions in the brain and vary from person to person. Because of this variance, it is suggested that the most successful therapy would include multiple methods. Researchers suggest that a number of factors in the executive functioning need to be improved, including self-awareness, goal setting, planning, self-initiation, self-monitoring, self-inhibition, flexibility, and strategic behaviour.
One method for individuals to improve in these areas is to help them plan and carry out actions and intentions through a series of goals and sub-goals. To accomplish this, therapists teach patients a three-step model called the General Planning Approach. The first step is Information and Awareness, in which the patients are taught about their own problems and shown how this affects their lives. The patients are then taught to monitor their executive functions and begin to evaluate them. The second stage, Goal Setting and Planning, consists of patients making specific goals, as well as devising a plan to accomplish them. For example, patients may decide they will have lunch with a friend (their goal). They are taught to write down which friend it may be, where they are going for lunch, what time they are going, how they will get there, etc. (sub-goals). They are also taught to make sure the steps go in the correct order. The final stage, named Initiation, Execution, and Regulation, requires patients to implement their goals in their everyday lives. Initiation can be taught through normal routines. The first step can cue the patient to go to the next step in their plan. Execution and regulation are put into action with reminders of how to proceed if something goes wrong in the behavioural script. This treatment method has resulted in improved daily executive functioning, however no improvements were seen on formal executive functioning tests.
Since planning is needed in many activities, different techniques have been used to improve this deficit in patients with DES. Autobiographical memories can be used to help direct future behaviour. You can draw on past experiences to know what to do in the future. For example, when you want to take a bus, you know from past experience that you have to walk to the bus stop, have the exact amount of change, put the change in the slot, and then you can go find a seat. Patients with DES seem to not be able to use this autobiographical memory as well as a normal person. Training for DES patients asks them to think of a specific time when they did an activity previously. They are then instructed to think about how they accomplished this activity. An example includes "how would you plan a holiday". Patients are taught to think of specific times they went on a holiday and then to think how they may have planned these holidays. By drawing on past experiences patients were better able to make good decisions and plans.
Cognitive Analytic Therapy (CAT) has also been used to help those with DES. Because individuals with this syndrome have trouble integrating information into their actions it is often suggested that they have programmed reminders delivered to a cell phone or pager. This helps them remember how they should behave and discontinue inappropriate actions. Another method of reminding is to have patients write a letter to themselves. They can then read the letter whenever they need to. To help patients remember how to behave, they may also create a diagram. The diagram helps organize their thoughts and shows the patient how they can change their behaviour in everyday situations.
The use of auditory stimuli has been examined in the treatment of DES. The presentation of auditory stimuli causes an interruption in current activity, which appears to aid in preventing "goal neglect" by increasing the patients' ability to monitor time and focus on goals. Given such stimuli, subjects no longer performed below their age group average IQ.
Some researchers have suggested that DES is mislabelled as a syndrome because it is possible for the symptoms to exist on their own. Also, there is not a distinct pattern of damage that leads to the syndrome. Not all patients with frontal lobe damage have DES and some patients with no damage at all to the frontal lobe exhibit the necessary pattern of symptoms. This has led research to investigate the possibility that executive functioning is broken down into multiple processes that are spread throughout the frontal lobe. Further disagreement comes from the syndrome being based on Baddeley and Hitch's model of working memory and the central executive, which is a hypothetical construct.
The vagueness of some aspects of the syndrome has led researchers to test for it in a non-clinical sample. The results show that some dysexecutive behaviours are part of everyday life, and the symptoms exist to varying degrees in everyone. For example, absent-mindedness and lapses in attention are common everyday occurrences for most people. However, for the majority of the population such inattentiveness is manageable, whereas patients with DES experience it to such a degree that daily tasks become difficult.