"Ataxia Or What?".

Another title for this talk might be, How Do You Know l Don t Have ...? That is probably the most common question he is asked: "How do you know I don't have this? How do you know I don't have that? How is this different and how is that different?" Ataxia is a symptom, it is incoordination. Ataxia can be caused by many different things.

Multiple sclerosis is a very common disorder of young people, and it definitely can cause ataxia. The ataxia from multiple sclerosis is really no different in its appearance than what a hereditary ataxia patient might look like. It is very commonly misdiagnosed either one way or the other. What is the difference? Contrary to popular opinion, not all tests are accurate enough to diagnose multiple sclerosis or hereditary ataxia. Sometimes it depends on the history. MS patients have had an up and down course. They are ataxic for a month or so, and all of a sudden it goes away and comes back later for a progressive period of a week, two weeks, or a month, and then it goes away. Multiple sclerosis has a lot of weakness involved, sometimes one side, sometimes the other. Patients complain of having a lot of numbness. Multiple sclerosis is usually not hereditary. Multiple sclerosis is unpredictable. In ataxia you have a much better idea of what the natural progression of the disease will be.

Parkinson's Disease: Usually the onset is in the the age group of 40 and 50, sometimes even later. Parkinson's Disease has tremors, sometimes on one side or the other. They have a lot of stiffness and slow movements. Are they ataxic? Not by the true definition but they have progressive difficulty walking, but it is not really a true ataxia.

Alzheimer's is not usually hereditary. Alzheimer's is a problem with a loss of memory, personality changes, patients become confused, and usually ataxia is not a symptom of Alzheimer's. However, if patients are having difficulty thinking, are very slow, often depressed, uncooperative, they frequently look like they have gait disorders or problems walking.

How do you know that you don't have a brain tumor? The MRI Scans and CAT Scans will be normal and there won't be any evidence of lesions in the brain. Usually a brain tumor is going to be a lot more rapidly progressive than the ataxias. If someone has been having trouble with walking, balance, and coordination for a progressive period of eight to ten years, it is unlikely that they have a brain tumor. There are some slow growing brain tumors, but it is unlikely. Brain tumors usually start on one side or the other, whereas the hereditary ataxias are usually a fairly symmetrical disease--it is the same on both sides. Brain tumors commonly cause bad headaches which doesn't mean that every headache is a brain tumor. Cerebellar tumors do cause ataxia and do cause problems with balance usually one side or the other is more affected.

Cerebral Palsy starts at birth because something happens during the period around birth such as lack of oxygen or trauma. Problems start very early in childhood but do not progress. They continue to have the same difficulty throughout their life.

Charcot-Marie-Tooth (CMT) or hereditary motor sensory neuropathy causes ataxia, but it is more of a sensory ataxia or an ataxia that is due to the peripheral nervous system as opposed to problems with the brain or the spinal cord. Symptoms are an unsteady gait, numbness, a loss of feeling, (similar to Friedreich's) a lot of weakness in the muscles, atrophy or wasting in the muscles. One big difference between CMT and Friedreich's is that Charcot-Marie-Tooth is a dominantly inherited disorder and commonly has a pattern of anticipation. Usually Charcot-Marie-Tooth is much more slowly progressive and the onset can be at a very young age or it can be at an older age.

Hereditary spastic paraparesis is a disorder that causes ataxia, but it is not a true ataxia. It is more what we call spasticity or stiffness in walking. There is more spinal cord involvement, isolated to a particular areas of the spinal cord causing muscle spasms and spasticity. A whole spectrum of different problems can occur. It is usually dominantly inherited but there are some recessive families so it is a much more complicated disease. There is a big continuum of what hereditary spastic paraparesis looks like from one end of the spectrum to the other. It is not a true ataxia, but patients do have a lot of difficulty walking.

Hereditary tremor is often called benign essential tremor. It is dominantly inherited with definite anticipation. It is a progressive tremor with no other neurological problems. It is most commonly confused with Parkinson's Disease. There is a difference in the type of tremor. Essential tremor or hereditary tremor is more prominent when you hold your hand out or when you try to do something as opposed to Parkinson's tremor which is more prominent when you are resting. There are medications to treat these tremors, but they are difficult to treat.

Huntington's Chorea is a dominantly inherited disease that is very rapidly progressive. Does ataxia affect your mental ability, in other words do you have problems thinking? You might have a little but most of the problems are not so much with thinking as they are with other areas of the nervous system. Huntington's Chorea starts out very early with a brain syndrome or dementia where there are personality changes very similar to Alzheimer's, but much worse. They have movement disorders that sometimes can be confused with ataxia. One of the most common presentations is called, "dancing gait".

Muscular Dystrophy has hundreds of different patterns, just like the ataxias. There are different genetic patterns, different types, different ages of onset, but one of the keys is there is no brain involvement. They don't have the particular patterns that we see with the ataxias. The ataxia that accompanies muscular dystrophy is due to weakness.

Stroke: There is no progression in a stroke. Usually what you see is what you get right from the very start. There is frequent recovery of varying degrees.

Head Trauma is similar to the stroke pattern. It is usually on one side or the other. The person may have trouble with coordination in one arm or may have trouble with walking, but no trouble with the arms. They may have trouble with their speech, but not with their walking. The key with head trauma is you have an injury--it is not progressive. The other key with head trauma is it is very similar to ataxia in that a lot of other neurologic deficits can occur. The next problem is how to differentiate between multiple problems because a lot of ataxia patients have fallen and have had head injuries. That becomes difficult so it needs to be watched over time and see if there is progression..

"In summary, I think the point that I would like to make is all ataxias are not hereditary."

Michael Wilensky, MD is currently in private practice in the greater New Orleans area. He serves as the Medical Director for the Louisiana Chapter of NAF and has been working with ataxia for over ten years. He is also a member of NAF's Medical and Research Advisory Board.