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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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Since pseudobulbar palsy is a syndrome associated with other diseases, treating the underlying disease may eventually reduce the symptoms of pseudobulbar palsy.
Possible pharmacological interventions for pseudobulbar affect include the tricyclic antidepressants, serotonin reuptake inhibitors, and a novel approach utilizing dextromethorphan and quinidine sulfate. Nuedexta is an FDA approved medication for pseudobulbar affect. Dextromethorphan, an N-methyl-D-aspartate receptor antagonist, inhibits glutamatergic transmission in the regions of the brainstem and cerebellum, which are hypothesized to be involved in pseudobulbar symptoms, and acts as a sigma ligand, binding to the sigma-1 receptors that mediate the emotional motor expression.
Medications that impede the release of excitatory neurotransmitters have been used to control or prevent spasms. Treatment with intrathecal baclofen, a gamma-aminobutyric acid (GABA) agonist, decreases muscle tone and has been shown to decrease the frequency of muscle spasms in ADCP patients. Tetrabenazine, a drug commonly used in the treatment of Huntington's disease, has been shown to be effective treating chorea.
Physical therapy and Occupational Therapy are staple treatments of ADCP. Physical therapy is initiated soon after diagnosis and typically focuses on trunk strength and maintaining posture. Physical therapy helps to improve mobility, range of motion, functional ability, and quality of life. Specific exercises and activities prescribed by a therapist help to prevent muscles from deteriorating or becoming locked in position and help to improve coordination. Occupational therapy interventions for children with CP can include feeding, dressing, bathing, toileting, grooming, pencil grasp and handwriting skills, play, and use of adaptive equipment.
CBPS is commonly treated with anticonvulsant therapy to reduce seizures. Therapies include anticonvulsant drugs, adrenocorticotropic hormone therapy, and surgical therapy, including focal corticectomy and callosotomy. Special education, speech therapy, and physical therapy are also used to help children with intellectual disability due to CBPS.
"For many years, it was thought that postural and balance disorders in cerebellar ataxia were not treatable. However, the results of several recent studies suggest that rehabilitation can relieve postural disorders in patients with cerebellar ataxia...There is now moderate level evidence that rehabilitation is efficient to improve postural capacities of patients with cerebellar ataxia – particularly in patients with degenerative ataxia or multiple sclerosis. Intensive rehabilitation programs with balance and coordination exercises are necessary. Although techniques such as virtual reality, biofeedback, treadmill exercises with supported bodyweight and torso weighting appear to be of value, their specific efficacy has to be further investigated. Drugs have only been studied in degenerative ataxia, and the level of evidence is low."
One approach is that it can be ameliorated to varying degrees by means of Frenkel exercises.
One main objective of the treatment is to re-establish the physiological inhibition exerted by the cerebellar cortex over cerebellar nuclei. Research using Transcranial direct-current stimulation (TCDCS) and Transcranial magnetic stimulation (TMS) shows promising results.
Additionally, mild to moderate cerebellar ataxia may be treatable with buspirone.
It is thought that the buspirone increases the serotonin levels in the cerebellum and so decreases ataxia.
Depending on subtype, many patients find that acetazolamide therapy is useful in preventing attacks. In some cases, persistent attacks result in tendon shortening, for which surgery is required.
Physiotherapy intervention aims to improve balance and gait of OPCA patients, by stimulating neuroplastic changes in the atrophied neural structure. A challenge-oriented treatment program has previously been shown to be beneficial for individuals with ataxia from OPCA. The treatment program was composed of repetitive training with task challenges (e.g. obstacle course) and/or novel motor skills acquisition over a 12-week period under the supervision of a physiotherapist. Task challenges were progressed only when the patient showed mastery of a task.
Overground harness systems may be used to allow OPCA patients to challenge their balance without chance of falling. Furthermore, home exercise programs and/or aquatic exercises are used to allow more repetitions to facilitate balance learning. Treatment programs should be frequently monitored and adjusted based on a patient's progress. Outcome measures such as the Berg Balance Scale, Dynamic Gait Index and activities-specific balance confidence scales are useful to assess patient’s progress over time.
Individuals with cerebellar ataxia have full cognitive awareness: it is usually only the physical deterioration that prohibits them from participating in activities of daily living and any other relevant or desired interests. One of the most significant barriers in the lives of these individuals is dysarthria. Due to their cognitive stability, it is important that people who spend time with individuals with this disease are able to communicate as fully as possible with them. This is necessary in order to improve their day-to-day interactions.
Behavioral intervention is successful when it involves engaging knowledge of the interests and general backgrounds of individuals with cerebellar ataxia. Communication maximizing strategies are also useful, such as exaggeration of articulatory gestures, giving full attention to their responses, repeating where necessary, and slowing down speaking rate. Another intervention technique for speech is to focus on optimizing respiratory and vocal resources as well as training compensatory strategies.
These listed intervention techniques can improve quality of life in individuals with this disease and can be helpful for professionals/clinicians in the field as well as loved ones of those affected.
The most common drug used to treat AHC is flunarizine. Flunarizine functions by acting as a calcium channel blocker. Other drugs, in order of frequency of use are benzodiazepines, carbamazapine, barbiturates, and valproic acid. Flunarizine is prescribed for the purpose of reducing the severity of AHC attacks and the number of episodes, though it rarely stops attacks altogether. Minimizing the attacks may help reduce damage to the body from hemiplegic attacks and improve long-term outcomes as far as mental and physical disabilities are concerned.
Experts differ in their confidence in flunarizine's effectiveness. Some studies have found it to be very effective in reducing the duration, severity, and frequency of hemiplegic attacks. It is generally considered the best treatment available, but this drug is thought by some to be of little benefit to AHC patients. Many patients suffer adverse effects without seeing any improvement. Flunarizine also causes problems because it is difficult for patients to obtain, as it is not readily available in the United States.
Articulation problems resulting from dysarthria are treated by speech language pathologists, using a variety of techniques. Techniques used depend on the effect the dysarthria has on control of the articulators. Traditional treatments target the correction of deficits in rate (of articulation), prosody (appropriate emphasis and inflection, affected e.g. by apraxia of speech, right hemisphere brain damage, etc.), intensity (loudness of the voice, affected e.g. in hypokinetic dysarthrias such as in Parkinson's), resonance (ability to alter the vocal tract and resonating spaces for correct speech sounds) and phonation (control of the vocal folds for appropriate voice quality and valving of the airway). These treatments have usually involved exercises to increase strength and control over articulator muscles (which may be flaccid and weak, or overly tight and difficult to move), and using alternate speaking techniques to increase speaker intelligibility (how well someone's speech is understood by peers). With the speech language pathologist, there are several skills that are important to learn; safe chewing and swallowing techniques, avoiding conversations when feeling tired, repeat words and syllables over and over in order to learn the proper mouth movements, and techniques to deal with the frustration while speaking. Depending on the severity of the dysarthria, another possibility includes learning how to use a computer or flip cards in order to communicate more effectively.
More recent techniques based on the principles of motor learning (PML), such as LSVT (Lee Silverman voice treatment) speech therapy and specifically LSVT may improve voice and speech function in PD. For Parkinson's, aim to retrain speech skills through building new generalised motor programs, and attach great importance to regular practice, through peer/partner support and self-management. Regularity of practice, and when to practice, are the main issues in PML treatments, as they may determine the likelihood of generalization of new motor skills, and therefore how effective a treatment is.
Augmentative and alternative communication (AAC) devices that make coping with a dysarthria easier include speech synthesis and text-based telephones. These allow people who are unintelligible, or may be in the later stages of a progressive illness, to continue to be able to communicate without the need for fully intelligible speech.
There is currently no cure for SCA 6; however, there are supportive treatments that may be useful in managing symptoms.
Current research at the University of Utah is investigating whether sodium oxybate, also known as Gamma-Hydroxybutyric acid is an effective treatment for AHC. Thus far, only a small number of patients have been sampled, and no conclusive results are yet available. While some success has been had thus far with the drug, AHC patients have been known to respond well initially to other drugs, but then the effectiveness will decline over time. Currently, sodium oxybate is used as a narcolepsy-cataplexy treatment, though in the past it has been used controversially in nutritional supplements. This drug was chosen to test because of a possible link between the causes of narcolepsy-cataplexy and AHC.
Idebenone, an antioxidant, was recently removed from the Canadian market in 2013 due to lack of effectiveness. A Cochrane review on antioxidants and other pharmacological treatment of patients with Friedreich ataxia concluded that there is limited but not persuasive evidence of efficacy.
Nicotinamide administration on patients was associated with a sustained improvement in frataxin concentrations towards those seen in asymptomatic carriers during 8 weeks of daily dosing. The daily oral administration of 3.8 g nicotinamide resulted in a 1.5-times increase, whereas 7.5 g resulted in a doubling of frataxin protein concentration.
Individuals with paraplegia can range in their level of disability, requiring treatments to vary from case to case. From a rehabilitation standpoint, the most important factor is to gain as much functionality and independence back as possible. Physiotherapists spend many hours within a rehabilitation setting working on strength, range of motion/stretching and transfer skills. Wheelchair mobility is also an important skill to learn. Most paraplegics will be dependent on a wheelchair as a mode of transportation. Thus it is extremely important to teach them the basic skills to gain their independence. Activities of daily living (ADLs) can be quite challenging at first for those with a spinal cord injury (SCI). With the aid of physiotherapists and occupational therapists, individuals with an SCI can learn new skills and adapt previous ones to maximize independence, often living independently within the community.
There is currently no cure for Costeff syndrome. Treatment is supportive, and thus focuses on management of the symptoms. The resulting visual impairment, spasticity, and movement disorders are treated in the same way as similar cases occurring in the general population.
There have been no major breakthroughs in the treatment of PKAN, with most pharmacologic treatments focusing on the easing or temporary relieving of PKAN’s symptoms. Iron chelating agents have been used somewhat successfully in retarding the disorder, but they have not been a significant success.
Current research focuses on the future use of high dose pantothenate, the PANK2 enzyme substrate, in possibly alleviating symptoms as well as the further development of iron chelating agents that may be better aimed at reaching the central nervous system and working to better remove excess iron from the individual’s system.
Complications may result from the medication used to treat symptoms. Immobility from the disease can also lead to skin breakdown, respiratory infections, and blood clots, among others.
Flaccid dysarthria is caused when damage occurs to the motor unit (one or more cranial or spinal nerves). Processes that can cause this include:
- Congenital disorders
- Demyelinating disorders
- Infectious/Inflammatory
- Degenerative disorders
- Metabolic
- Neoplastic
- Traumatic
- Vascular Diseases
- Flaccid Paralysis
In cases of acute AOS (stroke), spontaneous recovery may occur, in which previous speech abilities reappear on their own. All other cases of acquired AOS require a form of therapy; however the therapy varies with the individual needs of the patient. Typically, treatment involves one-on-one therapy with a speech language pathologist (SLP). For severe forms of AOS, therapy may involve multiple sessions per week, which is reduced with speech improvement. Another main theme in AOS treatment is the use of repetition in order to achieve a large amount of target utterances, or desired speech usages.
There are various treatment techniques for AOS. One technique, called the Linguistic Approach, utilizes the rules for sounds and sequences. This approach focuses on the placement of the mouth in forming speech sounds. Another type of treatment is the Motor-Programming Approach, in which the motor movements necessary for speech are practiced. This technique utilizes a great amount of repetition in order to practice the sequences and transitions that are necessary in between production of sounds.
Research about the treatment of apraxia has revealed four main categories: articulatory-kinematic, rate/rhythm control, intersystemic facilitation/reorganization treatments, and alternative/augmentative communication.
- Articulatory-kinematic treatments almost always require verbal production in order to bring about improvement of speech. One common technique for this is modeling or repetition in order to establish the desired speech behavior. Articulatory-kinematic treatments are based on the importance of patients to improve spatial and temporal aspects of speech production.
- Rate and rhythm control treatments exist to improve errors in patients’ timing of speech, a common characteristic of Apraxia. These techniques often include an external source of control like metronomic pacing, for example, in repeated speech productions.
- Intersystemic reorganization/facilitation techniques often involve physical body or limb gestural approaches to improve speech. Gestures are usually combined with verbalization. It is thought that limb gestures may improve the organization of speech production.
- Finally, alternative and augmentative communication approaches to treatment of apraxia are highly individualized for each patient. However, they often involve a "comprehensive communication system" that may include "speech, a communication book aid, a spelling system, a drawing system, a gestural system, technologies, and informed speech partners".
One specific treatment method is referred to as PROMPT. This acronym stands for Prompts for Restructuring Oral Muscular Phonetic Targets, and takes a hands on multidimensional approach at treating speech production disorders. PROMPT therapists integrate physical-sensory, cognitive-linguistic, and social-emotional aspects of motor performance. The main focus is developing language interaction through this tactile-kinetic approach by using touch cues to facilitate the articulatory movements associated with individual phonemes, and eventually words.
One study describes the use of electropalatography (EPG) to treat a patient with severe acquired apraxia of speech. EPG is a computer-based tool for assessment and treatment of speech motor issues. The program allows patients to see the placement of articulators during speech production thus aiding them in attempting to correct errors. Originally after two years of speech therapy, the patient exhibited speech motor and production problems including problems with phonation, articulation, and resonance. This study showed that EPG therapy gave the patient valuable visual feedback to clarify speech movements that had been difficult for the patient to complete when given only auditory feedback.
While many studies are still exploring the various treatment methods, a few suggestions from ASHA for treating apraxia patients include the integration of objective treatment evidence, theoretical rationale, clinical knowledge and experience, and the needs and goals of the patient
Flaccid dysarthria is a motor speech disorder resulting from damage to peripheral nervous system (cranial or spinal nerves) or lower motor neuron system. Depending on which nerves are damaged, flaccid dysarthria affects respiration, phonation, resonance, and articulation. It also causes weakness, hypotonia (low-muscle tone), and diminished reflexes., Perceptual effects of flaccid dysarthria can include hypernasality, imprecise consonant productions, breathiness of voice, and affected nasal emission.
The treatment for seizures may include antiepileptic medications, diet, and vagus nerve stimulator.
Any number of medications may be used to both prevent and treat seizures.
Generally after three medications are tried, different treatment should be considered. It should also be noted that some medications are harmful to those with this syndrome and can increase seizures.
Treatment for MSS is symptomatic and supportive including physical and occupational therapy, speech therapy, and special education. Cataracts must be removed when vision is impaired, generally in the first decade of life. Hormone replacement therapy is needed if hypogonadism is present.
Parkinson-plus syndromes are usually more rapidly progressive and less likely to respond to antiparkinsonian medication than PD. However, the additional features of the diseases may respond to medications not used in PD.
Current therapy for Parkinson-plus syndromes is centered around a multidisciplinary treatment of symptoms.
These disorders have been linked to pesticide exposure.
At the time of the report there was no known treatment for the disease; specifically, it was not established whether steroids were helpful or harmful. Other techniques such as plasmaphoresis, intravenous immunoglobulin, and experimental antiviral drugs have been attempted on a trial basis, but have not been reported to be effective. On November 7 the CDC issued "Interim Considerations for Clinical Management of Patients with Acute Flaccid Myelitis", based on "consensus guidance drawn from experts in infectious diseases, neurology, pediatrics, critical care medicine, public health epidemiology and virology." Mark Sawyer of the American Academy of Pediatrics, who contributed to the guidance, was quoted by the organization's newsletter: The most important issue summarized in the document is that there is no clear evidence that therapies intended to modify the immune system (e.g., corticosteroids, immune globulin, plasmapheresis) have a beneficial effect in this condition. Plasmapheresis is specifically not recommended because the potential for harm is significant in the absence of any evidence of benefit.