Therapies to manage dystonia can include:
  • Intramuscular botulinum toxin

    • Botox is injected in spastic, dystonic muscles to help them relax for a period of time

  • Oral Artane (trihexyphenidyl)

  • Baclofen (oral or intrathecal)

    • One of the main drugs used to treat dystonia, usually first taken orally and divided into several doses each day

    • In the intrathecal method, an implanted baclofen pump delivers medication directly into the spinal fluid

  • Deep brain stimulation

    • Used more often and has some evidence for benefit

    • It involves surgical implantation of a lead, extension and battery pack (IPG)

      • The lead contains 4 electrodes and is implanted in the globus pallidus region of the brain

      • The extension connects the lead to the battery pack (IPG)

      • The IPG is a battery-powered neurostimulator that is placed in the abdomen (or in some cases below the clavicle)

  • Physical and occupational therapy

    • May or may not be indicated for those who are only mildly symptomatic

Medication to manage parkinsonism:

The symptoms of parkinsonism can be treated with the same medications used in Parkinson’s disease. Treatment with dopamine agonist drugs (like Levodopa) must be started and monitored carefully. In the beginning, the dose is increased gradually until both the patient and doctor feel symptoms are under control. While taking dopaminergic drugs, individuals must be regularly monitored for adverse neuropsychiatric effects, psychiatric symptoms and worsening of parkinsonism. There is often short-term great benefit from Parkinsons medications. However, this usually only lasts a few years and is often eventually limited by the development of dyskinesias (a common side effect that creates unwanted movement).

Even after a diagnosis has been made and the appropriate therapies have been chosen, it is recommended to continue long-term surveillance to decrease the impact of BPAN symptoms and increase quality of life.

Long-term surveillance for BPAN can include:
  • Medication for spasticity, dystonia, and/or parkinsonism

  • Monitoring of individuals receiving dopaminergic drugs for parkinsonism for:

    • Adverse neuropsychiatric effects

    • Psychiatric symptoms

    • Worsening of parkinsonism

  • Nutrition

    • Monitoring of height and weight in children

    • Swallowing evaluation and regular dietary assessments

      • Assure adequate nutrition

      • Prevent aspiration

    • Gastrostomy tube placement (as needed)

  • Routine eye exams

  • Regular assessments of walking and speech abilities