Alpers' Disease

Alpers' disease is a rare, progressive neurodegenerative disease of the brain. It is also know known as primary sclerosing poliodystropy. Alpers' disease affects infants and children.

CAUSES

Alpers' disease is an inheritated disorder and is passed from the parents to the child. Alpers' disease can occur in siblings.

SYMPTOMS

  • The first signs of Alpers' disease usually happen in infancy. Symptoms include:

  • Seizures (convulsions).

  • Failure to meet meaningful developmental milestones.

  • Progressive mental retardation.

  • Low muscle tone (hypotonia).

  • Stiffness of the limbs (spasticity).

  • Memory loss (dementia).

  • Loss of vision (blindness).

  • Loss of hearing (deafness).

  • Liver failure.

TREATMENT

There is no cure for Alpers' disease. Treatment is based on symptoms.

  • Antiseizure medicine (anticonvulsants) may be used to treat seizures.

  • Physical therapy may help to:

  • Relieve spasticity.

  • Maintain or increase muscle tone.

PROGNOSIS

The outcome (prognosis) for individuals with Alpers' disease is poor. Those with the disease usually die within the first 10 years of life. Death can occur from:

  • Continuous seizures.

  • Liver failure.

  • Heart and lung failure (cardiopulmonary failure).