Olivopontocerebellar Atrophy

Olivopontocerebellar atrophy (OPCA) is a term that describes the degeneration of neurons in specific areas of the brain – the cerebellum, pons, and inferior olives.

Olivopontocerebellar atrophy can be passed down through families, or it may affect people without a known family history (sporadic form). The cause of OPCA in those without a history of the disease is not known. This disease is slightly more common in men than in women.

OPCA is present in several neurodegenerative syndromes, including inherited and non-inherited forms of ataxia (such as the hereditary spinocerebellar ataxia known as Machado-Joseph disease) and multiple system atrophy (MSA), with which it is primarily associated.

OPCA may also be found in the brains of individuals with prion disorders and inherited metabolic diseases.

Symptoms start sooner in people with the inherited form rather than the sporadic form of OPCA. The main symptom is clumsiness (ataxia) that slowly gets worse. There may also be problems with balance, slurring of speech, and difficulty walking.

Other symptoms may include:

  • Abnormal eye movements
  • Abnormal movements
  • Bowel or bladder problems
  • Difficulty swallowing
  • Tremor
  • Muscle spasms
  • Muscle stiffness or rigidity
  • Nerve damage (neuropathy)
  • Sexual function problems
  • Sleep disorders
  • Depression

The characteristic areas of brain damage that indicate OPCA can be seen by imaging the brain using CT scans or MRI studies.

There is no cure for OPCA. The disorder is slowly progressive with death usually occurring approximately 20 years after onset.

There is no specific treatment for OPCA. Physicians may try different medications to treat the ataxia, tremor, and rigidity that are associated with the disorder. Other treatments are directed at specific symptoms. Stiffness, spasms, sleep disorders, depression, and tremor may be improved with medication. A physical therapist may be helpful in establishing a routine of exercise and stretching, and in obtaining devices or appliances to assist in walking and other daily activities.