Olivopontocerebellar Atrophy

What is Olivopontocerebellar Atrophy?

Olivopontocerebellar atrophy (OPCA) refers to a group of ataxias characterised by progressive neurological degeneration affecting the cerebellum, the pons and the inferior olives. OPCA may be classified based on clinical, genetic, or neuropathological findings; thus, there are many classifications of the disorder. Among the different classifications there is wide variation in severity and age of onset. The symptoms of OPCA differ from person to person. Most patients experience difficulty with balance and coordination of the legs and arms (ataxia) and slurred speech (dysarthria). Other symptoms may include muscle spasms or weakness and stiffness of the muscles; numbness or tingling of the hands or feet; tremor (shaking) of the hand or arm; reduction or slowness of movements; loss of thinking and/or memory skills; difficulty controlling the bladder or bowels; and feeling faint when standing up. Some patients also have fatigue and/or trouble with sleep. Generally symptoms of OPCA begin in mid-adult life and progress slowly over the course of many years.

Is there any treatment?

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.

What is the prognosis?

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