Olivopontocerebellar Atrophy

Definition

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

Symptoms

Symptoms of Olivopontocerebellar Atrophy
Symptoms tend to start sooner at a younger age in people with the inherited form. 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:

1. Abnormal eye movements
2. Abnormal movements
3. Bowel or bladder problems
4. Difficulty swallowing
5. Lightheadedness when standing
6. Muscle spasms
7. Muscle stiffness or rigidity
8. Nerve damage (neuropathy)
9. Tremor
10. Sexual function problems

Causes

Causes of Olivopontocerebellar Atrophy

1. Olivopontocerebellar atrophy can be passed down through families (inherited form), or it may affect people without a known family history (sporadic form).
2. Researchers have identified certain genes that are involved in the inherited form of this condition.
3. The cause of olivopontocerebellar atrophy in those without a history of the disease is not known. The disease slowly gets worse (is progressive).

Diagnosis

Diagnosis of Olivopontocerebellar Atrophy

1. A thorough medical and neurological examination, as well as a symptom and family history, are needed to make the diagnosis.
2. There are no specific tests for this condition. An MRI of the brain may show changes in the size of affected brain structures, especially as the disease gets worse. This is helpful in making the diagnosis, but does not necessarily rule out the condition.
3. Other tests may be done to rule out other conditions. This may include swallowing studies to see if a person can safely swallow food and liquid.

Treatment

Treatment of Olivopontocerebellar Atrophy
There is no specific treatment or cure for this disease. Therapy is aimed at treating symptoms and preventing complications. This may include:

1. Tremor medications, such as those used to treat Parkinson's disease
2. Speech and physical therapy
3. Techniques to prevent choking
4. Walking aids to help with balance and prevent falls

Prognosis

Prognosis of Olivopontocerebellar Atrophy
The disease slowly gets worse, and there is no cure. The outlook is generally poor. However, it may be years before someone becomes significantly disabled.

Prevention

Prevention of Olivopontocerebellar Atrophy
Consult with your doctor.