Question Title: Olivio Cerebellum Atrophy

Forum: Neurology Forum
Topic: Neurology - General

Posted by CCF Neurology MD on November 05, 1997 at 22:47:04:

In Reply to: Olivio Cerebellum Atrophy posted by Sheila L. Hughes on October 15, 1997 at 09:18:31:

: Mother was diagnosed with this rare disease about three years ago. They were many months discovering what her problem was. Since being diagnosed we haven't been able to acquire much information with regards to treatment, stages,life expectancy, and most of all what could have the causes. It is very heartbreaking to watch her gradually loose all her muscles and will to fight. Could you please tell me what type of medication and if there are any clinics in the United States that might be able to help me out. The doctors she has seen are using her as a guinea pig and now have ceased to treat her disease. Will she loose her eyesight and have to be fed through tubes? Thank you in advance for any information you can give me. Sheila Hughes

Dear Sheila:

The term “Olivo-ponto-cerebellar atrophy” (OPCA) stands for a group of relatively rare degenerative disorders of the brain. The degeneration involves the small brain or the cerebellum, and also parts of the brainstem. The main symptoms are imbalance of gait, slurring of speech, and incoordination of the hands. Inconstant features are visual impairment, dementia, difficulty swallowing, neuropathy, and autonomic insufficiency (urinary retention, constipation, orthostatic hypotension, etc.), amongst others. While several heredofamilial forms of the disorders exist and have been recently characterised, sporadic OPCA (these case do not have other members of the family affected) is probably more common. The age of onset and the rate of progression differ according to the type of OPCA. Onset is later in life (median in the 6th decade) and progression in somewhat more rapid in patients with sporadic OPCA. On the average (and there is wide variation), the life expectancy of a patient with sporadic OPCA after diagnosis is about one decade; time to a chairbound state is reported to be about 5 years. Autonomic insufficiency is a very common and major problem in patients with sporadic OPCA.
At this time, there is unfortunately no treatment known to be effective in reversing/halting disease progression or treating motor symptoms. A variety of maneuvers may help ameliorate autonomic symptoms, if present.
If you wish to seek a second opinion, I suggest that your mother be seen by a specialist in movement disorders from an academic neurology department near you. If you live near Cleveland, I strongly recommend Dr. R. Stanley Burns and Dr. Erwin Montgomery at the Cleveland Clinic.
This information is provided for general medical education purposes only. Please consult your physician for diagnostic and treatment options of your specific medical condition.

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