Date: Mon, 6 Feb 95 01:53:59 -0500 From: Bob Broedel To: als@huey.met.fsu.edu Subject: ALSD#171 ALS-ON-LINE =============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest (#171, 06 February 1995) == == == == ------ Amyotrophic Lateral Sclerosis (ALS) == == ------ Motor Neurone Disease (MND) == == ------ Lou Gehrig's disease == == ------ Charcot's Disease == == == == This e-mail list has been set up to serve the world-wide == == ALS community. That is, ALS patients, ALS researchers, == == ALS support/discussion groups, ALS clinics, etc. Others == == are welcome (and invited) to join. The ALS Digest is == == published (approximately) weekly. Currently there are == == 470+ subscribers. == == == == To subscribe, to unsubscribe, to contribute notes, == == etc. to ALS Digest, please send e-mail to: == == bro@huey.met.fsu.edu (Bob Broedel) == == Sorry, but this is *not* a LISTSERV setup. == == == == Bob Broedel; P.O. Box 20049; Tallahassee, FL 32316 USA == =============================================================== CONTENTS OF THIS ISSUE: 1 .. re: leg/body cramps 2 .. Alternative treatment for ALS 3 .. Alleviating cramps 4 .. re: cramps 5 .. re: inquiry about anti-oxidants 6 .. ALS Drug Trial Announcement 7 .. MDA Research Report #55 (1) ===== re: leg/body cramps ========== Date : Fri, 03 Feb 1995 20:53:38 GMT >From : John@woodlea.demon.co.uk (John Stent) Subject: ALSD#170 Re: Vondas note about leg/body cramps Vitamin E may be worth trying. A friend of mine takes 800 - 1200iu/day and this stopped her cramps almost immediately. They have stayed away for almost a year now. Hope it helps. John. (2) ===== Alternative treatment for ALS ========== Date : Sun, 5 Feb 95 10:14:46 PST >From : Gen Baugher Subject: Alternative treatment for ALS A few months ago, we corresponded via e-mail regarding the ALS support group. At that time, I had a relative who had ALS, but he has since died. For about two years, I searched everywhere for some information on alternative healing methods for ALS and I could not find any book which mentioned this disease. Recently I came across an excellent book called Healthy Healing, an Alternative Healing Reference by Linda G. Rector-Page. Her book mentions ALS, some possible causes and also various herbs and foods used to treat it. One of the causes she mentions is heavy metal toxicity. My relative's doctor told him that his ALS was caused by exposure to heavy metals. He used to eat lots of shellfish, and I have since learned that shellfish, such as shrimp and crab, are very high in heavy metals. This book also mentions alternative treatment for heavy metal poisoning, including the use of aloe vera juice. In the course of my research on ALS, I spoke with a doctor in Texas who told me about 3 people he knew who are taking aloe vera juice and have been in remission for many years. Healthy Healing is available from: Stars Distributing; 20065B Highway 108: Sonora, CA 95370 Phone: 209-532-6474 Please pass this information along. Maybe it will help someone who is in the early stages of ALS and could still benefit. ----------------------------------- Gen Baugher P.O. Box 189279, Sacramento, CA 95818 Phone: 916-454-9880 Fax: 916-454-9350 ------------------------------------- (3) ===== Alleviating cramps ========== Date : Mon, 6 Feb 95 11:54:35 +1100 >From : paulf@tigger.mel.dbe.CSIRO.AU (Paul Failla ) Subject: Alleviating cramps My Father has MND and in fact the first indications looking back were the onset of cramps. These were mainly in the legs. He takes daily Quinate tablets and although he still occasionally gets cramps, it seems to be mainly under control. We have also found that his energy levels have increased after having had prescribed a series of vitamins by a Naturopath. He is taking Vitamins C and E, zinc, Baker's Yeast. As I said it has helped a little in that he is able to do a little more than previously. Hope this helps. Finally I would like to thank all those people that contribute to this group. It is a great source of information and strength. In particular I would like to thank Joe Springer who recently took some time to write to this interest group. His explanations were extremely helpful and the information he gave is the kind of thing we are looking for from people working in the area. (4) ===== re: cramps ========== Date: 05 Feb 95 16:15:13 EST >From: Barry Goldberg <71154.330@compuserve.com> Subject: ALSD#170 ALS-ON-LINE Response to Vonda: Vonda, I have had ALS going on six years now and originally suffered incredibly from cramping and fascinations (twitching). Originally I was put on a regimen of baclofen (20mg qid) and quinine sulfate (325 units qid). The baclofen was fine for the twitching other than at night so my doctor added 5mg of Valium (generic diazepam) and said I could also take another 5mg during the day if necessary. Everything worked great but the cramping increased geometrically and I often was in terrible pain. At that point my family doctor recommended using Slow-K, a prescription strength dosage of potassium. I began taking this twice a day and the difference was virtually miraculous! Today I only have severe cramping on occasion and that's certainly okay with me . I've recommended this to others but haven't had any direct feedback but I think this is definitely worth trying. Let all of us know how everything works out. Sincerely yours, Barry Goldberg (5) ===== re: inquiry about anti-oxidants ========== Response to Katherine from Barry Goldberg Katherine, I've been on an antioxidant program for almost a year now. The therapy has been 3,000 units of vitamin E, 3,000 units of vitamin C and 50,000 units of betacarotene. Naturally it's difficult to say that there has been any special benefit regarding progression of the disease since I had already been living with ALS for over 4 years. However, on the potential negative side, three weeks ago I found myself in terrific back pain which, after hospitalization, turned out to be a kidney stone. The urologist treating me gave me a full rundown on the possible reasons and the one which stood out was the high level of vitamin C. This may be only a sensitivity on my part but it's something to consider. Kidney stones just ain't no fun!! Hope this gives you some of the insight you were seeking. Best regards, Barry. MDA -- Working to find the cure for neuromuscular disease (6) ===== ALS Drug Trial Announcement ========== Date : 05 Feb 1995 23:11:29 -0500 (EST) >From : BROWN@HELIX.MGH.HARVARD.EDU Subject: Re: als drug trial announcement Thank you for the recent e-mailings. They have been very informative. I would like to take up your kind offer to post an announcement: ======================== = = SPORADIC and FAMILIAL ALS BLOOD DNA SAMPLES NEEDED = = Investigators at the Massachusetts General Hospital (Boston), = Northwestern University (Chicago) and Duke University (Durham) are = starting a collaborative study to identify risk factors for sporadic = ALS using methods which have recently proven effective in familial ALS. = Toward this end, we are soliciting blood samples from individuals with = sporadic ALS who have one or both parents surviving and willing to = donate as well. = Individuals with familial ALS can also assist importantly in this study. = We need only a standard blood sample obtained in your local doctor's = office. We will pay for shipping, handling and investigational expenses. = All samples are coded upon receipt to preserve anonymity. Please feel = free to call us if you have any questions. Samples can be provided = through: = = (1) Dr. Robert Brown and Ms. Diane McKenna-Yasek = Massachusetts General Hospital = Boston, MA = 617-726-5750 = = (2) Dr. Teepu Siddique = Northwestern University = Chicago, IL = 312-503-4737 = ========================= Many thanks, Bob Brown 617-726-5750 / 617-444-6125 ===== = If it has not already been done ... would one of the Prodigy = subscribers please post this important note to the Prodigy ALS BBS? = Also, Barry ... you will please post this to the CompuServe MDA/ALS = BBS. OK? rgds,bro ===== (7) ===== MDA Research Report #55 ========== Date : 05 Feb 95 23:07:51 EST >From : Barry Goldberg <71154.330@compuserve.com> Subject: MDA Research Report #55 Hi! Here's the latest research report from MDA. Please feel free to pass this along to the ALS Digest and MD membership. I hope they find it useful. Barry Goldberg (71154.330@compuserve.com) -- -- -- -- -- -- January 30, 1995, Muscular Dystrophy Association TO: MDA Clinic Directors FROM: Norine Stirpe, Ph.D. MDA Director of Research Development RE: Research Update #55 Deletions in genes located in the region known to contain the defect associated with SMA have been identified. Researchers in the SMA working group supported by MDA have independently reported findings that implicate two different genes. One gene is called NAIP and the other SMN. Due to the complicated nature of the genetic region where the SMA gene(s) are positioned, the results obtained thus far are not clear. It is yet to be determined whether both genes, only one gene or possibly neither gene are involved. NAIP has similarities to other known inhibitors of apoptosis. Apoptosis, or programmed cell death, is an important process during development when certain cells must die in a controlled fashion to allow the proper formation of the embryo. A defect in this process could result in the motor neuron destruction seen in SMA. The function of SMN must still be determined, however, deletions in this gene were found in 98% of the families examined. Both genes have strong cases in terms of their possible involvement in the disease process, however, we must await more definitive information as to their involvement in SMA. (Thompson, T. et al. Nature Genetics 1995; 9:56-62; Lefebvre, S. et al. Cell 1995; 80:155-165; Roy, N. et al. Cell 1995; 80:167-178). A gene defect has been found associated with nemaline myopathy (NEM1) in one large family affected by a dominant form of the disorder that has a clinical onset in childhood. Researchers in Australia determined that one form of a tropomyosin gene that is located on chromosome 1, contains a defect in the family examined. NEM is a muscle disease that can be inherited in a recessive or dominant manner, and it is characterized by the appearance in muscle fibers of rod bodies, which are mostly composed of a muscle protein called actin. The tropomyosin defect is positioned in an area of the protein that would interact with actin, suggesting that the defect causes a tighter interaction with actin leading to the formation of rod bodies. However, this is yet to be proven and not all families affected by NEM have the tropomyosin gene defect. There are a number of other structural muscle proteins that may be involved, including other versions of the tropomyosin gene. (Laing, N., et al., Nature Genetics 1995; 9:75-79). Individuals affected by chromosome X-linked Charcot-Marie-Tooth disease (CMTX) are known to have defects in their connexin 32 gene. The connexin 32 protein (Cx32) is a member of a family of proteins that are involved in forming channels that allow communication between cells through the exchange of certain compounds. In this way, a group of cells can coordinate its activities. Cx32 is expressed at high levels in myelinated peripheral nerve and MDA researchers have been studying the Cx32 gene defects in order to determine the basis of CMTX. The scientists have found that the Cx32 proteins produced from the defective gene are not active and have lost their ability to function. Apparently, other types of connexins cannot substitute for Cx32 and the loss of communication due to the defective channels most likely leads to the degeneration of peripheral nerves. (Bruzzone, R. et al. Neuron 1994; 13:1253-1260). Abnormal expression of PMP-22 is associated with Charcot-Marie-Tooth disease type 1A (CMT1A) and results in abnormal myelination -- insulation of nerve cells. The function of PMP-22 is not understood, however, it is a protein present in the Schwann cells that are responsible for myelination of peripheral nerves. Although axons -- the long, straight part of the nerve cell that conducts nerve impulses -- are also affected by the abnormal expression of PMP-22. MDA researchers have determined that in CMT1A a number of effects may be compromising the interaction of Schwann cells and axons as well as the mechanism by which materials are transported within axons. (Watson, D. F. et al. Neurology 1994; 44:2383-2387). Mitochondrial disease is characterized by the appearance of ragged red fibers (RRF) in muscle when the tissue is examined using certain histological techniques. However, RRFs may appear in other conditions where abnormalities in mitochondria are observed. MDA-supported researchers compared the presence of RRFs in muscle biopsies from healthy adults of various ages with biopsies from individuals affected by inclusion body myositis (IBM), polymyositis (PM) or dermatomyositis (DM). The study indicates that RRFs can be associated with mitochondrial disease as well as aging, and the frequency of RRFs is increased in IBM, although not in PM or DM. (Rifai, Z. et al. Annals of Neurology 1995; 37:24-29). Why only certain motor neurons are destroyed in amyotrophic lateral sclerosis (ALS) is not yet understood. MDA-supported researchers have evaluated the suggestion that deficiencies in specific calcium-binding proteins may contribute to the fact that motor neurons are vulnerable to degeneration in ALS. The scientists found that two types of calcium-binding proteins were not present in the motor neurons that degenerate early in the progression of the disease; however, the proteins were found in the motor neurons affected later or infrequently in the disease. In addition, motor neurons lacking the calcium-binding proteins and exposed to ALS IgG in a culture dish were killed. Their study implies that certain calcium-binding proteins have a role in the loss of motor neurons, or the proteins may signify neurons that are resistant to the degeneration associated with ALS. (Alexianu, M., et al. Annals of Neurology 1994; 36:846-858). IVIg has been considered a possible treatment for the autoimmune disease, myasthenia gravis (MG), and the inflammatory myopathies, dermatomyositis (DM) and polymyositis (PM). How IVIg can reduce the effects of these disorders is unclear although it may prevent or compete with certain immune responses that cause the diseases. There are several different therapies commonly used for blocking or reducing the immune response that results in MG. In many instances the trials studying IVIg have involved individuals who had already received or were simultaneously receiving at least one of these other therapies. Researchers believe that in order to establish when the use of IVIg in MG might be most successful, and to compare IVIg to the other therapies that are available, controlled, blind trials are necessary. Noted positive and negative aspects of therapy with IVIg are presented in a recent review. (Edan, G. and Landgraf, F. Journal of Neurology, Neurosurgery and Psychiatry 1994; 57[Supplement]:55-56). Other researchers have found that IVIg can be a useful therapy for PM and DM when other conventional therapies are not successful. They report that IVIg seems less effective when used early in the development of the diseases before conventional therapies such as corticosteroids are administered. IVIg given along with steroids appears to provide more benefit and, therefore, the two medications may complement one another. The research group has begun a randomized, multicenter, open trial comparing IVIg and immunosuppressive agents in adults with PM or DM that is resistant to steroids. Considering the cost of IVIg, the conclusion of the researchers at this time is that IVIg therapy for inflammatory myopathies can be administered when high doses of prednisone or other immunosuppressive agents do not work. (Cherin, P. and Herson, S. Journal of Neurology, Neurosurgery and Psychiatry 1994; 57[Supplement]:50-54). Hyperkalemic periodic paralysis (HYPP) and paramyotonia congenita (PC) are associated with defects located in the sodium channel gene. These channels are important in muscle for passing the signals necessary to trigger muscle contraction. In order to understand the clinical differences between HYPP and PC, MDA-supported scientists have been analyzing the exact types of sodium channel gene defects associated with each disorder. It appears that PC results from one type of alteration in the function of the channel, and HYPP results from a different functional alteration. Understanding the differences in how the sodium channel function is affected will help determine appropriate therapies for these diseases. (Yang, N. et al. Proceedings of the National Academy of Sciences, USA 1994; 91:12785-12789). In phosphofructokinase (PFK) deficiency there is an inability to use glycogen stored in muscle or glucose in the blood as energy sources. Administering glucose to individuals with PFK deficiency during exercise only enhanced muscle fatigue, whereas, muscle fatigue is relieved in individuals affected by phosphorylase deficiency who are given glucose. (Ono, A. et al. Neurology 1995; 45:161-164). Certain components that are produced by a metabolic cycle provide the fuel or energy needed for muscle contraction. Different steps in the cycle may be affected and lead to a metabolic muscle disorder. A new, noninvasive clinical technique to investigate fatigue in a number of these metabolic myopathies was recently described. (Bank, W. and Chance, B. Annals of Neurology 1994; 36:830-837). The location of the defective gene that is responsible for Fukuyama-type congenital muscular dystrophy (FCMD) has been linked to a certain area on chromosome 9. Japanese researchers recently determined that individuals affected by Walker-Warburg syndrome -- another CMD that is clinically much more severe than FCMD -- must also result from defects in the same gene. It is possible that different types of abnormalities in the same gene could result in diseases of varying severities, and the scientists are currently looking for the exact gene defect associated with the two disorders. (Toda, T. et al. Annals of Neurology 1995; 37:99-101). cc: "Ray Harwood -- Data Basix: (602)721-1988" [RHarwood@Data.Basix.Com] --- === MDA -- Working to find the cure for neuromuscular disease === === end of als 171 === ..