What is Olivo-Ponto Cerebellar Atrophy?

There is a rare disease called Olivo Ponto Cerebellar Atrophy. I didn't know about it myself until a friend of mine who was diagnosed with this when he was 50 years of age and suffered for 5 years with an entire muscular degeneration over his body. Sadly, he passed away two months ago after years of debilitation! may God bless his soul.

What is Olivopontocerebellar Atrophy?


Olivopontocerebellar atrophy is a disease that causes certain brain areas to shrink.

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.


There is no specific treatment for OPCA (Olivoponto Cerebellar Atrophy). Neurologists 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.

Alternative Names:

OPCA; Olivopontocerebellar degeneration

Causes, incidence, and risk factors:

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.

Unfortunately there are little studies made about this disease and if you knew someone who has been diagnosed with OPCA please take note of the information below.


National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291