Physiotherapy in Wilson's Disease
Wilson's disease is an inherited condition in which copper is not excreted properly from the body. The excess copper can build up in the liver and/or brain causing liver damage and/or neurological problems. It can also collect in other parts of the body including the eyes and the kidneys. Copper begins to accumulate immediately after birth but the symptoms usually appear in the 2nd to 3rd decade. The first signs are hepatic (liver) in about 40% of cases, neurological (brain) in about 35% of cases and psychiatric, renal (kidney), haematological (blood), or endocrine (glands) in the remainder.
Read more about the Pathophysiology of Wilson's Disease here
About half the people with Wilson's disease have neurological or psychiatric symptoms. Most initially have mild cognitive deterioration and clumsiness, as well as changes in behavior. Specific neurological symptoms usually then follow, often in the form of parkinsonism with or without a typical hand tremor, masked facial expressions, slurred speech, ataxia or dystonia. Seizures and migraine appear to be more common in Wilson's disease. A characteristic tremor described as "wing-beating tremor" is encountered in many people with Wilson's; this is absent at rest but can be provoked by extending the arms. Cognition can also be affected in Wilson's disease. This comes in two, not mutually exclusive, categories: frontal lobe disorder (may present as impulsivity, impaired judgment, promiscuity, apathy and executive dysfunction with poor planning and decision making) and subcortical dementia (may present as slow thinking, memory loss and executive dysfunction, without signs of aphasia, apraxia or agnosia). It is suggested that these cognitive involvements are related and closely linked to psychiatric manifestations of the disease.
Psychiatric problems due to Wilson's disease may include behavioral changes, depression, anxiety and psychosis. Psychiatric symptoms are commonly seen in conjunction with neurological symptoms and are rarely manifested on their own. These symptoms are often poorly defined and can sometimes be attributed to other causes. Because of this, diagnosis of Wilson's disease is rarely made when only psychiatric symptoms are present.
Physiotherapy is beneficial for patients with the neurologic form of the disease. The copper chelating treatment may take up to six months to start working, and physical therapy can assist in coping with ataxia, dystonia, and tremors, as well as preventing the development of contractures that can result from dystonia.
Maintenance therapy is more important than rehab.
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.