Question Title: FAMILIAR CEREBELLUM DEGENERATION

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There are a variety of cerebellar degenerative diseases which run in families. The common symptom produced by damage to the cerebellum (of whatever cause) is ataxia, that is, incoordination. Ataxia can affect gait, causing people to walk like they're drunk (alcohol affects the cerebellum, so that's actually why you walk like that if you drink too much). Ataxia can also affect limb function, so that use of the hands is incoordinated.

Familial cerebellar system degenerative diseases can be inherited in dominant or recessive patterns, depending on the specific disease. Dominant is when you have it, a parent has it, and a grandparent has it, as well as assorted brothers, sisters, aunts, uncles, and cousins. Recessive is when two siblings have it but no one else in the family has it.

Making a specific diagnosis rests on the recognition of the ataxia, to be sure, but also on other signs and symptoms and laboratory tests or MRI results. One of my colleagues suggests that there are over 600 diseases which cause ataxia, so it isn't that helpful in narrowing down what the problem is. An experienced neurologist (usually a movement disorders specialist) should be able to narrow it down for you. For a small fraction of cerebellar diseases, there are genetic tests (for example, Freidreich's ataxia, SCA-1, SCA-2, and SCA-3). We usually don't order a genetic test until signs and symptoms have helped us narrow down the possibilities.

Treatment is very primitive, and very frustrating. Sometimes people with tremor resulting from cerebellar damage may respond to clonazepam or related medications, but we've not found any medications yet which help ataxia. More, we have no currently available agent which has been demonstrated to halt or even slow down the progression of cell loss (degeneration). It certainly is an issue in neurologic/neuroscience research, but nothing is yet available even for clinical trials, let alone in commercial preparations.

On the other hand, some devices can help make walking easier and safer. One we use is called a 3-wheeled walker. If your dad walks better with less effort and fatigue by using a shopping cart (have him try this), then he may do better with a 3-wheeled walker or similar device.

Ask your doctor more about this. If you are not certain the diagnosis is specific, then I'd advise seeing a movement disorders specialist. I do know that Toronto (the university medical center) has strength in this field, but I don't know about other parts of Canada. Of course, if you wish you are welcome to come to Cleveland. Call 800 223-2273 and ask for neurology appointments, 4-5559.

I hope this helps. Good luck. CCF MD mdf.