Physiotherapy in Wilson's Disease
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Wilson's Disease & its Symptoms[edit | edit source]
Wilson's disease is a rare autosomal recessive disorder which causes excess copper build up in the body[1]. It mainly affects the brain, liver and cornea. It's incidence is 1 in 30,000 individuals, from ages 4 to 40 years[2].
Symptoms are varied and depend on the degree of involvement of organs damaged due to copper accumulation. Earlier signs and symptoms seen are hepatic (involving liver) in ~40% of patients, neurological (involving brain) in ~40%–50% and psychiatric in ~10% of patients[3]. The symptoms of interest from physiotherapy treatment point of view are the neurological and to some extent psychological. The neurological ones are chiefly tremor, dystonia, parkinsonism that can be seen clinically along with dysarthria, gait and posture disturbances, drooling and dysphagia[4].
may severely affect the activities of daily living
Physiotherapy management[edit | edit source]
The symptoms of this disease, especially neurological will affect the activities of daily living of the patient to a great extent. Physiotherapy is of value in improving the affected individual's quality of life by treating these symptoms. The aim is to preserve optimum functional level of patient rather than rehabilitate or produce drastic improvements in symptoms. Thus in case of Wilson's disease, maintenance therapy is more important.
The mainstay management of Wilson's disease is copper-chelating therapy. It requires a time period of six months to take effect[2]. In the mean time physiotherapy can help cope with ataxia, dystonia and tremors.
Physiotherapy-
Relaxation technique is more important to maintain the position and posture. To reduce hyper-activeness use sedative otherwise due to hyper activeness rigidity will increase and result will be dislocation/fracture/injury of some joints/bones or muscle/ligament injury. To avoid torticollis maintain the position of neck and do the proper exercise and stretching.
Use cock-up splint to maintain hand position and use L-splint or night splint to maintain ankle position, proper positioning of spine to avoid scoliosis and kyphosis. Maintain sitting position 2-3 hours twice daily.
References[edit | edit source]
- ↑ Bull PC, Thomas GR, Rommens JM, Forbes JR, Cox DW. The Wilson disease gene is a putative copper transporting P–type ATPase similar to the Menkes gene. Nature genetics. 1993 Dec;5(4):327-37.
- ↑ 2.0 2.1 Chaudhry HS, Anilkumar AC. Wilson Disease. [Updated 2021 Aug 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441990/#
- ↑ Hedera P. Update on the clinical management of Wilson’s disease. The application of clinical genetics. 2017;10:9.
- ↑ Członkowska A, Litwin T, Dusek P, Ferenci P, Lutsenko S, Medici V, Rybakowski JK, Weiss KH, Schilsky ML. Wilson disease. Nature reviews Disease primers. 2018 Sep 6;4(1):1-20.
