Corticobasal Degeneration

Overview

Corticobasal degeneration is a rare progressive neurologic disorder that causes atrophy (deterioration or shrinkage) of specific areas of the brain, often the cerebral cortex and basal ganglia, due to an abnormal buildup of tau protein. The onset of this disease typically occurs around age 60. Corticobasal degeneration can cause involuntary movements, problems with walking, impaired cognition (trouble with memory and thinking) and behavioral changes.

Types

Corticobasal degeneration is a type of parkinsonism, a neurologic disorder that mimics symptoms of Parkinson’s disease. Other parkinsonisms include:

  • Dementia with Lewy bodies
  • Drug-induced parkinsonism, a movement disorder that occurs as a result of medications that interfere with dopamine transmission
  • Multiple system atrophy, which causes failure of involuntary body functions, including bladder function, blood pressure, breathing and motor control
  • Progressive supranuclear palsy, which is a deterioration of brain cells that causes balance, eye movement and walking problems
  • Vascular parkinsonism, which causes unsteadiness while walking but no tremors

Symptoms

  • “Alien limb syndrome,” a feeling that your limb has a mind of its own due to involuntary movements such as raising your arm involuntarily
  • Behavioral changes such as irritability or personality changes
  • Difficulty performing some physical or analytical actions
  • Loss of sensation on one side of the body or difficulty identifying objects by touch
  • Myoclonus, which are involuntary muscle jerks, spasms or twitches
  • Speech and language changes such as speaking more softly or having trouble finding the right words
  • Stiffness, slowness and sometimes pain in the limbs or neck
  • Swallowing difficulties
  • Walking difficulties or loss of balance

Prevention

The cause of corticobasal degeneration is not well understood, so there are no known preventive measures known to reduce the risk of developing corticobasal degeneration. However, physical activity may help patients maintain function longer.

Risk Factors

Corticobasal degeneration typically occurs spontaneously. There does not appear to be a significant genetic or environmental cause.

Diagnosis

  • Medical history and symptom review. The provider will review symptoms and inquire about the patient’s ability to perform daily tasks.
  • Physical examination. The doctor will perform a neurologic exam, including assessing for rigidity, gait abnormalities, tremor and abnormal reflexes, and may perform cognitive assessment testing.
  • Diagnostic tests. MRI or CT scans of the brain may show asymmetric (one-sided) atrophy of specific areas of the brain. An EEG test may show a slowing of certain areas of the brain.

Treatment

There is no known treatment to stop or slow the progression of corticobasal degeneration. However, the symptoms may be treated with medications and other therapies to help maintain function.

  • Although few patients with corticobasal degeneration respond well to Parkinson’s disease medications, a trial of levodopa or other Parkinson’s disease medications sometimes alleviates slow movements or rigidity.
  • Myoclonus (involuntary muscle jerks or twitches) may be treated with muscle relaxants or certain seizure medications.
  • Botulinum toxin may be injected to help relax muscles and reduce symptoms of spasticity or stiffness in the limbs and/or neck.
  • Physical or occupational therapy may be ordered to help improve mobility and function in daily activities when necessary.
  • Speech therapy may be ordered to improve speech or swallowing.

Follow-up Care

Your doctor may recommend periodic office visits to assess your need for medication adjustments or referrals.