Date: Mon, 16 Jan 95 02:29:39 -0500 From: Bob Broedel To: als@huey.met.fsu.edu Subject: ALSD#165 ALS-ON-LINE =============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest (#165, 15 January 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 == == 450+ 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: sleep problem 2 .. re: Ricky & ALS 3 .. re: burning skin 4 .. animal models in ALS and general symptoms 5 .. SMA 6 .. Experimental NIH Web Server 7 .. Myotrophin & ALS 8 .. BDNT & ALS (1) ===== Re: sleep problem ========== Date: 10 Jan 95 18:30:05 EST >From : Mike Ward <72567.400@compuserve.com> Subject: Re: sleep problem Bro, Concerning Wayne Phillips sleep problem. My solution was to buy an adjustable bed. From a local manufacter we bought two adjstable beds with pillow top mattresses. We even talked the insurance company into paying for it since the $2000 cost was less than a hospital bed. Of couse they didn't know that was two beds! The only change from a standard bed was we put a 6" lift kit so a hoyer would fit under it. I put a foot board on it and use 6 pillows. I have stayed in bed for three days with no turning so it works well. I use a sheepskin under me. I put one pillow under my feet leaving the heels floating, one pillow goes against the foot board to prevent pain from foot drop. Two go under my head with the bottom one moved in or out to adjust my head angle. The last two go under my arms to raise them to a comfortable height. I put a tash pillow switch by my head so I can call my aides. I raise the foot of the bed a little to reduce foot swelling and raise my head for comfort. I hope this helps. I have used this for four years and it works well. Regards, Mike Ward (2) ===== re: Ricky & ALS ========== Date : Fri, 13 Jan 1995 04:08:40 -0500 (EST) >From: RICKY998@delphi.com Hi Kate- My EMG test came back abnormal. That was the only test that came back abnormal. Thank the good man above I was not positive for the HIV virus so the GP that suggested such a thing was QUITE wrong. There is no one else in my family that has ALS. Thank the good man above I am not married and I have no offspring so, I have eliminated the possibility the transferring this to any other person. I know what I have gone thru with this diease and I wouldn't want anyone to go thru what I have and am going thru, The good news is that I missed an appointment so there was two months since my last visit. And I told the Doctor that I thought I was not doing very well in that I had lost control of the right foot toes. But she tested every muscle group and everything turned out to be just about the same as it was 2 momths ago. I told the Doctor that I had just signed a years lease for a handicapped apartment and asked her if that was a sound judgement on my part and she said to me "Why not?" You are doing great. I will write a prescription for you to get filled for a brace like on the left leg for the right one. In that you will begin to stumble over that foot. So far so good I have not been fitted yet for the brace. To me.. it is a tad early to be doing this.. If I was going over on that foot, I could see the point of it. But for now I think it can wait. And please keep writing to me in that I know other people with ALS that come to my third Thursday of each month ALS meeting. But I do not have the opportunity to compare notes as it were. Ricky998 (3) ===== re: burning skin ========== Date : Fri, 13 Jan 1995 20:52:28 -0500 >From : KeyOfF@aol.com Subject: Re: Lyall Winlaw/burning skin Lyall, I meant to respond to your post of 11/24/94 quite some time ago, but better late than never. I don't know how widespread the feeling of burning skin is among ALS patients, but my mother experienced this. She used to complain that her feet burned like fire. She died twenty years ago, but if I remember correctly the neurologist told her the sensation was caused by the nerve endings. We used to put cool compresses on her feet; this would provide a little relief. My mother was put on a mild dose of elavil, a tricyclic antidepressant. Taken at night, this helps relieve pain and discomfort, and may also help the sleeping difficulty. I would advise talking to your wife's neurologist about the burning sensation and the sleeping problem, if you haven't already done so. I hope that you may have already found some help for these problems since you posted your message. Best wishes to you and your wife in coping with these symptoms. Faye (4) ===== animal models in ALS and general symptoms ========== Date : Thu, 12 Jan 1995 11:12:15 -0500 >From : "Cynthia Goodfellow" Subject: animal models in ALS and general symptoms I am currently working on a paper for a thesis prep. course and my topic is ALS. I have been asked to define the disease and discuss it in terms of genetics - how it is inherited, are there any genetic based therapies being developed or in current use, are there any animal models that are currently being used for research eg. the sod gene in Drosophila. I have seached various abstracts and come up with very little information that I can actually use for instance rats with ALS-like symptoms are not considered to be animal models. If you could send me any information or could tell me where I could look or who I might contact I would be very appreciative. Thanking you in advance, Cynthia Goodfellow 3317 Grassfire Cres. Mississauga, ON L4Y 3J8 Canada Tel: 905-238-0719 FAX:905-238-3518 (5) ===== SMA ========== PR NEWSWIRE Thursday January 12, 1995 TUCSON, Ariz., Jan. 12 /PRNewswire/ -- Two genes lying very close to each other on chromosome 5 may work together or separately to cause spinal muscular atrophy (SMA), the Muscular Dystrophy Association (MDA) announced today. Two research teams, one comprised of Canadian, American and Japanese scientists and the other a French group funded by the Association Francaise Contre Les Myopathies (the French MDA), each identified a gene they say may play a major role in causing SMA, a disease in which people become paralyzed as muscle-controlling nerve cells in the spinal cord die. The most severe form of SMA is the leading genetic cause of death among infants in the United States. "We've never seen a genetic disease discovery quite like this before," said Don Wood, MDA's director of science technology. "The two proteins (coded for by the newly discovered genes) may normally work together to make a healthy nerve cell, so that a defect in either one causes the same nerve disease." Wood said he was fascinated by the findings, but clearly much more work needs to be done. "There's no question that we're close to understanding this disease. The immediate implication is the development of better genetic tests for SMA treatmenthe disease must be better understood." MDA grantee Alex MacKenzie of Children's Hospital of Eastern Ontario in Ottawa, Canada, was on the international team, which found a gene they're calling NAIP (neuronal apoptosis inhibitory protein). The investigators say the gene may carry instructions for a protein that protects muscle- controlling nerve cells (motor neurons) from a type of normally occurring cell death that is an important part of fetal development. A flawed or absent protective protein might allow this type of cell death to continue into childhood, leading to SMA, they report. The French team identified a gene it has named the SMN (motor neuron survival) gene. The gene's protein hasn't yet been studied. The findings of both teams are published in today's issue of the journal Cell. < parts deleted > Facts About Spinal Muscular Atrophy Group/Type Usual Age of Onset Disease Characteristics Type I - Birth to three months Generalized muscle weakness, Infantile weak cry, trouble swallowing Progressive and sucking, and breathing Spinal distress, usually leading to Muscular paralysis of legs and arms Atrophy within three months. Life span rarely exceeds age 2. Type II - Six months to Weakness in arms, legs, upper Intermediate 3 years and lower torso, often with Spinal skeletal deformities. Muscular Disease progresses rapidly. Atrophy While most patients survive to early childhood, respiratory problems can further shorten life. Type III - 1 to 15 years Weakness in leg, hip, Juvenile shoulder, arm and Spinal respiratory muscles. Calf Muscular muscles are often enlarged. Atrophy Disease progresses slowly. Wheelchair is often required by age 30. Life span is unaffected. X-Linked 18 to 50 years Weakness in the tongue, hands Spinal and or feet which slowly spreads Bulbar to other parts of the body. Muscular A relatively mild form of Atrophy spinal muscular atrophy, it has little impact on life expectancy. -- In spinal muscular atrophy (SMA), motor neurons -- the nerve cells controlling the movement of voluntary muscles -- in the base of the brain and the spinal cord gradually disintegrate, making them unable to deliver signals that muscles need to function. -- Type I SMA is the leading genetic cause of death in infants. The three childhood forms of SMA together form the second most common lethal, autosomal recessive disease, after cystic fibrosis. -- SMA is caused by a genetic defect on chromosome 5. Scientists believe that various mutations in the same gene account for the four types of the disease. -- The SMA defect is a recessive genetic trait, meaning that if both parents carry the defect, a child of theirs has a one in four chance of inheriting the disease and a one in two chance of becoming an unaffected carrier. -- Estimates of the number of SMA carriers range from one in 40 to one in 80. -- SMA affects approximately one in 10,000 births. -- Some 20,000 Americans are affected by SMA. -- There is currently no known treatment or cure for SMA. Physical therapy and orthopedic devices can prolong walking ability. -- MDA's worldwide research program is aggressively pursuing a cure for SMA. Pinpointing the gene and genetic defect responsible makes more accurate diagnosis possible and provides a crucial step toward developing a treatment. CONTACT: Jim Brown of MDA, 602-529-5317 (6) ===== Experimental NIH Web Server ========== >From : stewartw@helix.nih.gov (Walter W. Stewart) Newsgroups: comp.infosystems.www.users Subject : Announcement: NIDDK/NIH (National Institutes of Health) : Experimental Web Server Date : Wed, 28 Dec 1994 16:29:13 -0500 The NIDDK is trying an experimental web server. It contains patient information on a number of diseases. We would be grateful for comments, which can be directed to stewartw@helix.nih.gov. The URL is: http://www.niddk.nih.gov/ (7) ===== Myotrophin & ALS ========== SAN FRANCISCO -DJ- (10 JAN 95) Cephalon Inc. (CEPH) President and Chief Executive Officer Frank Baldino said he expects data analysis to begin by March of from Phase II/III clinical trials for myotrophin used in the treatment of amyotrophic lateral sclerosis, or Lou Gehrig's disease, to begin by March. Speaking at the Hambrecht & Quist Life Science Conference here, Baldino said 449 patients have participated in the clinical trials for myotrophin, and to date no safety concerns have developed. Baldino said the company also has begun Phase II trials for myotrophin for indications in peripheral neuropathy. < rest of article deleted > (8) ===== BDNT & ALS ========== Business Editors TARRYTOWN, N.Y. HEALTHWIRE -Jan. 9, 1995--P. Roy Vagelos, M.D., former chairman and chief executive officer of Merck & Co. has been elected chairman of the board of Regeneron Pharmaceuticals Inc. (NASDAQ:REGN). Leonard S. Schleifer, M.D., PhD will retain the positions of chief executive officer and president of Regeneron. Dr. Vagelos, who retired from Merck in November 1994, will assume the non- executive position effective immediately. < parts deleted > Regeneron currently has two products in clinical trials and is engaging in a series of research programs and collaborations that have positioned the company as a leader in the discovery and development of biotechnology- based compounds focused on the treatment of neurodegenerative diseases. Brain-derived neurotrophic factor (BDNF), being developed by Regeneron in a joint venture with Amgen (NASDAQ:AMGN), is in Phase I/II clinical trials with patients with amyotrophic lacteral sclerosis (ALS, commonly known as Lou Gehrig's disease.) Amgen is conducting the trials on behalf of Amgen-Regeneron Partners. Last year, Amgen, on behalf of Amgen-Regeneron Partners, began conducting Phase I clinical trials of NT-3 for peripheral neuropathies. Regeneron is also developing BDNF in Japan in collaboration with Sumitomo Pharmaceuticals Ltd. < parts deleted > Regeneron is a leader in the discovery and development of biotechnology- based compounds focused on the treatment of neurodegenerative diseases, peripheral neuropathies, and nerve injuries, including such conditions as Parkinson's disease, Alzheimer's disease, ALS and spinal cord injuries. CONTACT: Regeneron Pharmaceuticals Inc. Paul Lubetkin, TEL: 914/347-7000 === end of als 165 ===