Wilson's Disease: A Case Study
Original Editor -Yuka Abe
Top Contributors - Courtney Lim
Abstract[edit | edit source]
Introduction[edit | edit source]
Client Characteristics[edit | edit source]
Examination Findings[edit | edit source]
The Unified Wilson’s Disease Rating Scale (UWDRS) would be appropriate for this patient in measuring the body structure and function limitations. The UWDRS consists of 3 subscales – neurological, hepatic, and psychiatric. The neurological subscale consists of items including rigidity, chorea, tremors, as well as facial expression and oromandibular dystonia. The UWDRS has been shown to be a reliable and valid tool to monitor disease progression in patients with Wilson’s disease [1]. Items on the UWDRS were adapted from scales that have already been established in measuring neurological impairments, including the Unified Parkinson’s Disease Rating Scale (UPDRS), Barthel index, and the Unified Huntington’s Disease Rating Scale, which suggests a high degree of content validity[1].
To evaluate the activity limitation, the 2 Minute Walk Test (2MWT) can be used. The 2MWT measures the distance walked in 2 minutes, and can be used to quantify walking limitations and fatigue. This measure has been proven to be reliable and valid in patients with various neuromuscular diseases[2]. In addition, the 2MWT is well correlated with the 6 Minute Walk Test, which is a widely used and validated tool in measuring walking capability. For this case, the patient is currently unable to walk without assistance, so the 2MWT would be administered when the patient is able to walk independently.
For participation limitations, the Tremor Research Group Essential Tremor Rating Scale (TETRAS) can be used to measure essential tremors. The TETRAS consists of ADL (12 items) and performance (9 items) sections[3]. Each item is scored on a scale of 0 to 4, where a higher score indicates more severe tremors. It is a well-validated and reliable tool to assess the severity of essential tremors, which is commonly present in those with Wilson’s Disease[4][5] . Furthermore, the TETRAS assesses wing-beating tremors which is characteristic of Wilson’s Disease.
Clinical Hypothesis[edit | edit source]
Intervention[edit | edit source]
Virtual reality (VR) is a new and upcoming innovative technology that has been shown to help facilitate and improve the rehabilitation process. Virtual reality uses principles of neuroplasticity and motor learning and utilizes computer algorithms to make decisions towards progressing the difficulty of a task. It has the ability to track neuroplastic changes and can be used to track recovery. Studies have shown VR to have great ecological validity and researchers have been steadily improving its technology (e.g higher resolution 3D systems are more prevalent). For instance, new research has worked to improve head mounted displays, allowing these displays to produce realistic, high resolution augmented visual feedback. This visual feedback can help with sensorimotor integration and thus help the patient improve their gait. Our patient, Chris Brown, presents with gait impairments. VR technology can be used to help in the treatment process of our patient’s gait patterning. Another applicable example is that VR can be used to improve upper extremity functioning and fine motor control by providing hepatic feedback to the patient. This can also help to facilitate Chris Brown's rehabilitation process. VR is a fresh new rehabilitation approach that can be engaging for patients and could help with motivation.
Despite the benefits that VR can bring, it does come with a few challenges. Two of the most pressing issues include accessibility issues due to expensive equipment costs and safety issues and considerations.
To mitigate costs barriers associated with VR systems, facilities/clinicians can work with researchers in pilot programs. This is a great way for the clinician and facilities to see if VR is suitable for their patient population while assessing if VR would be a reasonable investment that ultimately will save money in the long-term. Cost barriers can also be addressed by using alternative materials such as a cardboard headset. Some VR training content could also be used in a more accessible form such as on a desktop or mobile device.
To mitigate issues with safety, proper clinician training and education would be appropriate. To use VR, there could be requirements such as completion of a training course or passing a specific exam. The patient also needs to be given proper comprehensive education and informed consent. Side effects of VR can include cybersickness, perceptuomotor after-effects, headaches and eye-strain. The patient is also at risk for addiction to VR-based technologies. The patient should be informed of both benefits and drawbacks of the treatment, as well as alternative solutions. This can help to ensure safety and proper usage of VR systems by both patient and practitioner.
Outcome[edit | edit source]
UWDRS Outcome:
Discussion[edit | edit source]
References[edit | edit source]
- ↑ 1.0 1.1 Leinweber, B., Möller, J. C., Scherag, A., Reuner, U., Günther, P., Lang, C. J. G., Schmidt, H. H. J., Schrader, C., Bandmann, O., Czlonkowska, A., Oertel, W. H., & Hefter, H. (2008). Evaluation of the Unified Wilson’s Disease Rating Scale (UWDRS) in German patients with treated Wilson’s disease. Movement Disorders, 23(1), 54–62. https://doi.org/10.1002/mds.21761
- ↑ Andersen, L. K., Knak, K. L., Witting, N., & Vissing, J. (2016). Two- and 6-minute walk tests assess walking capability equally in neuromuscular diseases. Neurology, 86(5), 442–445. https://doi.org/10.1212/WNL.0000000000002332
- ↑ Elble, R. J. (2016). The Essential Tremor Rating Assessment Scale. Journal of Neurology & Neuromedicine, 1(4). https://www.jneurology.com/articles/the-essential-tremor-rating-assessment-scale.html
- ↑ Ondo, W., Hashem, V., LeWitt, P. A., Pahwa, R., Shih, L., Tarsy, D., Zesiewicz, T., & Elble, R. (2018). Comparison of the Fahn-Tolosa-Marin Clinical Rating Scale and the Essential Tremor Rating Assessment Scale. Movement Disorders Clinical Practice, 5(1), 60–65. https://doi.org/10.1002/mdc3.12560
- ↑ Ortiz, J. F., Morillo Cox, Á., Tambo, W., Eskander, N., Wirth, M., Valdez, M., & Niño, M. (n.d.). Neurological Manifestations of Wilson’s Disease: Pathophysiology and Localization of Each Component. Cureus, 12(11), e11509. https://doi.org/10.7759/cureus.11509
