Wilson's Disease: A Case Study: Difference between revisions

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== Outcome  ==
== Outcome  ==
[[File:UWDRS 1.png|thumb]]


== Discussion  ==
== Discussion  ==

Revision as of 01:10, 11 May 2023

Original Editor -Yuka Abe

Top Contributors -

Abstract[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]

Outcome[edit | edit source]

Discussion[edit | edit source]

References[edit | edit source]

  1. 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
  2. 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
  3. 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
  4. 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
  5. 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