Re: Hereditary Spastic Paraparesis

Posted By ccf neuro M.D. on April 18, 1997 at 14:10:34:

In Reply to: Hereditary Spastic Paraparesis posted by Denis P. on April 11, 1997 at 14:17:59:







: Question:
I have what doctors think to be Hereditary Spastic Paraparesis. They are reluctant due to no family history of the disease. It possibly is sporadic. I have moderate to severe spascticity according to my neurologist. I battle fatigue on a daily basis with no improvement after numerous medications to counteract it. When does fatigue become a concern and does it compromise other body function besides the neuromuscular system? Also do you have any suggestions on treatment of fatigue?
Thank you for taking our questions!
Denis




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Common causes of spastic paraparesis include multiple sclerosis, slowly growing spinal cord tumors, thoraccic spine herniated discs, and other rarer diseases such as tropical spastic paraparesis, caused by a virus called HTLV-1 (human T-cell lymphocyte virus 1), and another called primary lateral sclerosis. Rarely, certain vitamin deficiencies such as vitamin B12 deficiency and, very rarely (but hardly ever diagnosed early on) vitamin E deficiency, ususally secondary to an absortion or transporter defect--- but important since it is a treatable cause. Presumably you have had MRI scans with contrast of your cervical and thoraccic spinal cord to look for tumors, herniated discs, and multiple sclerosis; and presumably you've had one or more spinal taps done to look for HTLV-1 and other infectious or inflammatory causes for your symptoms. If you have not had a vitamin E blood level (alpha-tocopherol) done, it's worth it despite the miniscule chance again because it is treatable. For the treatment of spasticity
itself, in addition to mainstay druge like baclofen (Lioresal) and diazepam (Valium), there is a relatively new drug called tizanadine that you should try if you haven't already. All of these drugs promote fatigue because they are sedating, although tizanadine may cause less fatigue after time in some patients. For treatment of the fatigue itself, there is a drug known as amantidine (Symmetrel) that is often effective at reducing it and is relatively side-effect free and often greatly liked by our multiple sclerosis patients. I would certainly recommend giving it a try as it is often helpful. As to why people with neurologic problems get fatigue, to be honest with you no one really knows, and there is no simple or obvious explanation--- if there was, some drug company by now would have been very rich!!! I hope these comments and suggestions are useful to you.    P.S. Although one, of course, ideally likes to have a family history of any "hereditary" disease, there is always a very low but ever present rate of spontaneous mutations in people's DNA as they are conceived, and it may be that you are such a case.