Olivopontocerebellar atrophies


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.


Many symptoms are associated with olivopontocerebellar atrophy, but 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: * Tremor * Abnormal movements * Abnormal eye movements * Sexual function problems * Bowel or bladder problems * Muscle stiffness or rigidity * Muscle spasms * Nerve damage * Difficulty swallowing


Olivopontocerebellar atrophy can be passed down through families (inherited form), or it may affect people without a known family history (sporadic form). The cause of olivopontocerebellar atrophy in those without a history of the disease is not known. The disease slowly gets worse (is progressive). Symptoms tend to start sooner in people with the familial form. This disease is slightly more common in men than in women.


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.


There is no specific treatment or cure for this disease. Therapy is aimed at treating symptoms and preventing complications. This may include techniques to prevent choking, speech and physical therapy, and walking aids to help with balance and prevent falls.