Supranuclear Palsy

A discussion of the neurological etiology of Progressive Supranuclear Palsy Disorder

Progressive Supranuclear Palsy (PSP) is an unusual neurodegenerative situation that leads to severe executive dysfunction. The traditional findings in PSP reveal that executive functions are the most severely affected, including lack of initiative, increasing information processing time, decreased verbal fluency and loss of mental flexibility. PSP is often regarded as a typical parkinsonian disease, along with multiple systemic atrophy, and corticobasal damage. Onset of signs happens after age 40, with the optimum onset at age 63. It is uncertain whether men or women affected on the same magnitude. Although genetics may infer some vulnerability to the illness, PSP cases are usually infrequent and not usually inherited (Ghosh, Carpenter & Rowe, 2013).

Clinical results in PSP may be minor at initial presentation but become more noticeable as the illness advances. The executive dysfunctions in course of PSP require supranuclear efforts and consist of voluntary saccades, usually affected first, and impaired vigilance by recognizing fewer targets over time. Both the velocity and range of these motions are reduced, and motions in the vertical route are more impacted originally. Delayed latencies and eye-movements demanding only lower motor nerves, such as the vestibulo-ocular reflex, are saved. Patients may also display square trend jerks, which are brief, horizontally, saccadic uses into fixation (Burbank, 2006). Altered sensory processing often gives these patients a startled or surprised overall look and can cause dry sight. Additional ocular results consist of blepharospasm, photophobia, and apraxia in eyelid opening.

Though victims are most likely to show slowed information processing, key neurologic results are also obvious on evaluation and should be thoroughly analyzed. Decreased verbal fluency is typical, and victims can show loss of mental flexibility during the illness. In PSP, altered memory retrieval indicates memory problems caused by dysfunction in the frontostriatal system. Along with the lack of ability to look downwards, the gait apraxia causes frequent falls in patients with PSP. The falls are usually in reverse; victims often make little attempts to alter the fall. This makes them have a “toppling bowling pin” outlook. Rigidity of the throat and other axial muscle tissue is typical and can be recognized during the evaluation of the vestibulo-ocular reflex. Patients may also encounter changes in personality and behavior, most often anxiety, apathy or disinhibition (Olanow, Stocchi & Lang, 2011). Lastly, PSP victims may display frontal release symptoms, such as the applause sign and the grasp reflex, which is the lack of ability to clap only three times on command. All these suggest that the executive dysfunction of PSP patients is extremely severe.

Relate the impaired cognitive process to performance problems

In most situations, the visual performance is at least as affected as the walking performance, though it does not appear, on average until three to five years after the walking problem. Because the main problem with the sight is in aiming them effectively, reading often becomes challenging. The person discovers it is hard to move down to the beginning of the next content line instantly after reaching the end of the first line. This is very different from just requiring reading glasses. An eye physician unfamiliar with PSP may confuse the individual’s incapability to read a paper despite normal capability to read the individual letters on an eye graph. Some victims have their light cataracts extracted in a useless attempt to reduce such a visual issue. Another typical visual issue is the lack of capability to sustain eye contact during discussion (Josephs et al., 2005). This can give the wrong impression that the affected person is uninterested, hostile, or senile. The same eye activity issue can make the indication of “tunnel vision” and can cause trouble when driving a car.

The most typical eye activity issue in PSP is an affected capability to shift the eyes up or down. This can interfere with eating or descending the stairs, among other things. This issue is not usually as vexing for the individual and family as the lack of capability to sustain eye contact or to coordinate eye movements while studying. However, it is much simpler for the physician to identify. This decrease in vertical eye activity is usually the first hint to the physician that the diagnosed problem is PSP. Other circumstances, particularly Parkinson’s illness and regular ageing, can sometimes cause problems shifting the eyes up. However, PSP is nearly exclusive in also resulting in problems shifting the eyes down, hence interfering with normal performance (Hohler, et al. 2012).

Dysarthria (slurred or slow speech) is a very typical indication in PSP. Affected persons often find it challenging to carry discussions with others because of the delays of their reactions and their complications with diction. Gradually, complications with control of oral activities can advance to the point where taking meals, and particularly fluids, can be badly synchronized, resulting in the leakage of meals into the throat (dysphagia). This can lead to pneumonia, the most typical cause of loss of life in PSP. Some of the indicators that caregivers should consider include increased attempts in swallowing, food gathering in the mouth, weight loss, trouble talking and chest congestion (Egerton, et al. 2009).

PSP victims also experience behavioral and cognitive changes indicating irregular functioning in the front lobes. Cognitive changes in executive functions involve a decrease in front lobe functioning, such as slow information handling and recovery, altered reasoning, concrete thinking, difficulty planning and moving between tasks. Behaviorally, victims often display apathy, including withdrawal and decreased motivation, impulsivity and perseveration, lack of ability to change topic or switch tasks. Depressive disorders are also common.

Treatment methods

Medication

While medication for PSP is lacking, some medications can help control the symptoms. These include amantadine, levodopa, and other medications used in treating Parkinson’s disease. These medications can help improve stiffness and balance and reduce depression associated with PSP. However, these medications are only effective on short-term. Additionally, PSP patients are likely to be more sensitive to side effects of these medications, and experts might suggest stopping them (Donaldson & Marsden, 2011).

Speech and language therapist (SLT)

A speech and language therapist (SLT) can play a crucial role in enhancing the patient’s conversation and issues related to swallowing (dysphagia). They can also educate on a variety of methods to create the conversation function by making voice as clear as possible. As PSP advances, patients may need some assistive technology to help in communication. Various communication aids are available: the SLT will offer advice about the gadgets, especially on the most appropriate ones depending on the patient’s condition. SLT may be able to educate exercises that can activate the nerves used to induce the swallowing reflex, as well as building up the muscle tissues used during swallowing. There are also physical methods that can be used to make swallowing simpler (Fabio, Zampieri & Tuite, 2008). For example, some individuals complain that shifting their chin forward while swallowing allows avoiding any food from coming into their airways.

Physiotherapist

A physiotherapist advises on how to make the most of a patient’s remaining mobility by using exercise. Physical workout helps to enhance muscles, enhance posture and avoid stiffening of the joints. The physiotherapist will also be able to offer advice on aids that could be of advantage to the patient. This includes special shoes to minimize the risk of falling or slipping (Fabio, Zampieri & Tuite, 2008). A physiotherapist will also be able to educate on breathing exercises that a patient can use when eating to decrease the chances of developing aspiration pneumonia.

Critique of the treatment method

Currently, Pharmacologic providers are of little help in treating symptoms; in particular, inadequate reaction to levodopa is suggestive of PSP. Helpful care is of the biggest assistance to patients. Evidence-based techniques to recovery in PSP are missing. Today, a majority of research on the topic includes case studies only on some patients.

Case reviews focusing on rehabilitation programs for people with PSP often consist of limb-coordination exercises, gait coaching, tilt-board balancing, strength coaching with modern resistive workouts, and isokinetic workouts and stretching of the throat muscle. While some case reviews recommend that physiotherapy enhance stability and gait of PSP patients, it is impossible to generalize the results across all patients with PSP. This is because each case study was based on a limited number of patients. However, the results from these studies can be effective in assisting to guide upcoming research concerning the potency of stability and gait coaching programs in the control of PSP (Zareia, et al. 2010).

Patients suffering from PSP require occupational practitioners to help in handling their situation and enhancing their freedom. This may consist of being trained to use mobility aids. Because of the patients’ propensity to fall backwards, the use of walkers, is suggested over a stick. The use of mobility aids reduces the person’s risk of falling. It also enables to ambulate individually without support the society. Because of their irregular movements and balance issues, people will need to spend some time learning how to transfer safely in their homes and community (Donaldson & Marsden, 2011). This may consist of arising and seating in chairs securely. Following the progressive characteristics of this illness, all people gradually lose their capability simply to move and will need to advance and use a wheelchair.

References

Burbank, P.M. (2006). Vulnerable older adults: Health care needs and interventions. New York, NY: Springer Pub.

Donaldson, I.M., & Marsden, C.D. (2011). Marsden’s book of movement disorders. Oxford: Oxford Univ. Press.

Egerton, T., Williams, D. & Iansek, R. (2009). Comparison of gait in progressive supranuclear palsy, Parkinson’s disease and healthy older adults. Philadelphia: Lippincott Williams & Wilkins.

Fabio, R., Zampieri, C., Tuite, P. (2006). Gaze-shift strategies during functional activity in progressive supranuclear palsy. Received: 20 July 2006 / Accepted: 26 September 2006 / Published online: 8 November 2006. Springer-Verlag 2006.

Fabio, R., Zampieri, C., Tuite, P. (2008). Gaze Control and Foot Kinematics During Stair Climbing: Characteristics Leading to Fall Risk in Progressive Supranuclear Palsy.

Ghosh B., Carpenter R. & Rowe J. (2013). A Longitudinal Study of Motor, Oculomotor and Cognitive Function in Progressive SupranuclearPalsy. PLoS ONE 8(9): e74486.

Hohler, a., D. et al. (2012). Effectiveness of an InpatientMovement Disorders Program for Patients with Atypical Parkinsonism. Hindawi Publishing Corporation.

Josephs, K.A. et al. (2005). Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia. New York: Taylor & Francis Inc.

Olanow, C.W., Stocchi, F., & Lang, a.E. (2011). Parkinson’s disease: Non-motor and non-dopaminergic features. Chichester, West Sussex, UK: Wiley-Blackwell.

Zareia, Pouretemadb, H.R., Back, T. & Hodges, J.R. (2010). Autobiographical memory in progressive supranuclear palsy. European Journal of Neurology 2010, 17: 238 — 241