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Wilson Disease (Neurologic Involvement)

Neurologic manifestations of an inherited disorder caused by copper accumulation.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Wilson Disease (Neurologic Involvement)?

Wilson disease is an autosomal recessive disorder caused by ATP7B gene mutations, in which copper cannot be excreted from the liver and accumulates in tissues. The neurologic form may emerge in young adulthood with movement disorders and behavioral changes.

Typical findings include tremor, dystonia, dysarthria, swallowing difficulty, and gait disturbances. Psychiatric symptoms such as depression, behavioral changes, and cognitive decline may also be observed.

Diagnosis is supported by serum ceruloplasmin level, 24-hour urinary copper, liver biopsy, and ophthalmologic examination for the Kayser-Fleischer ring. Treatment is lifelong.

Symptoms

Tremor of the arms and head
Dystonia of the neck and face
Speech disturbance and dysarthria
Swallowing difficulty
Unsteady gait
Behavioral and personality changes
Kayser-Fleischer ring on eye examination

Risk Factors

Family history of Wilson disease
Carrier status of ATP7B mutation
Consanguinity
Young age (10-40)
History of liver disease
Unexplained hepatitis
Psychiatric history

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Unexplained tremor and dysarthria at a young age
  • Liver enzyme elevation accompanied by neurologic symptoms
  • Family history of Wilson disease
  • Behavioral changes and cognitive decline added to the picture
  • Kayser-Fleischer ring detected on eye examination

Treatment Methods

01
Copper chelators (D-penicillamine, trientine)
02
Zinc salts
03
Low-copper diet
04
Liver and neurologic follow-up
05
Family screening and genetic counseling
06
Liver transplantation (in selected severe cases)

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.