Aspire Educational Series: OlivoPontoCerebellar Atrophy (OPCA) – Part 22

The term olivopontocerebellar atrophy (OPCA) has historically been used to describe a group of disorders that affect the central nervous system and are termed neurodegenerative diseases because they result in a progressive nerve tissue degeneration and atrophy in the brain. These conditions are characterized by progressive balance problems (disequilibrium), progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia), and difficulty speaking or slurred speech (dysarthria).

Doctors believe that OPA is similar to a multiple system atrophy (MSA) disorder. Different MSA disorders occur in different sites within the brain. OPA shares many symptoms with MSA disorders. One common symptom is ataxia. Ataxia is a difficulty in controlling muscle movements for gait. Diagnosing OPA can be challenging because so many of the symptoms mirror those of MSA disorders.

Some of the more common symptoms of OPA may also include:

Olivopontocerebellar atrophy can be passed down through families (inherited form), or it may affect people without a known family history (sporadic form). Researchers have identified certain genes that are involved in the inherited form of this condition. In this disease brain cell of the particular part of brain namely the pons, cerebellum and inferior olive begin to degenerate and die. Genetic forms of OPCA may be inherited in an autosomal dominant, autosomal recessive, or X-linked manner. The cause of olivopontocerebellar atrophy in those without a history of the disease is not known. In such cases, the prime suspected causative factor is exposure to certain harmful chemicals and environmental toxins. This disease is slightly more common in men than in women. The average age of onset is 54 years old.

Hereditary Olivopontocerebellar Atrophy can be diagnosed through:

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.

The prognosis for Hereditary Olivopontocerebellar Atrophy is generally poor, as the condition slowly gets worse with time and there is no cure. It can take years for the individual to become fully disabled, but it has been shown that death occurs approximately 20 years after the onset of symptoms. In general, the outcome of OPCA depends upon the progression and severity of the signs and symptoms. Individuals with milder symptoms that show slower disease progression have better outcomes than those with rapidly progressing and severe symptoms

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