Hallervorden-Spatz disease is an inherited, degenerative disease that begins in childhood and involves progressive muscle rigidity , weakness, spasticity, and dementia.

Causes, incidence, and risk factors

The majority of cases occur in families with a history of the disorder. The pattern of inheritance suggests that this condition is caused by a recessive gene (which means a child needs defective copies of the gene from both parents) located on chromosome 20p.

Signs and tests

Neurological examination shows evidence of muscle rigidity; weakness; and abnormal postures, movements, and tremors. The presence of other affected family members may help determine the diagnosis. There are no specific genetic tests yet for this condition, so other movement disorders and diseases must be ruled out. MRI findings may show iron deposits.

Treatment

There is no specific treatment for Hallervorden-Spatz disease. Treatment is aimed at the control of symptoms.

Expectations (prognosis)

Hallervorden-Spatz is a progressive, neurodegenerative illness with immobility early on and death by early adulthood.

Complications

Complications may result from medication used to treat symptoms.

Calling your health care provider

Call your health care provider if your child has symptoms of Hallervorden-Spatz disease.

Prevention

Genetic counseling is appropriate in families affected by this illness, as there is no known way to prevent it.