=============================================================== == == == ----------- ALS INTEREST GROUP ----------- == == ALS Digest (#90, 30 March 1994) == == == == ------ Amyotrophic Lateral Sclerosis (ALS) == == ------ Motor Neurone Disease (MND) == == ------ Lou Gehrig'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. Currently there are == == 210+ 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. == == == == All interested people may "broadcast" messages to == == ALS Digest subscribers by sending to: == == als@huey.met.fsu.edu == == But be advised that if there are too many messages per == == day, researchers, international readers, etc. will be == == dropping out because of cost & time considerations. == == == == The ALS Digest is not a peer reviewed journal and it is == == not edited by an MD. It comes out (usually) weekly. == == == == Bob Broedel; P.O. Box 20049; Tallahassee, FL 32316 USA == =============================================================== == Back issues are available via e-mail from: == == LISTSERV@mailer.fsu.edu == == send an e-mail message that says INDEX ALS == == on a separate line say, HELP == == Also available via anonymous FTP at mailer.fsu.edu == == directory /pub/als == == Hardcopy versions (with extensive indexing) available == == from Grant Nicholas (gnicholas@pcgate.lanl.gov) == == A Global Index of issues 1-75 (created by G. Nicholas) == == is available via FTP. The 150K file is named als.index == =============================================================== CONTENTS OF THIS ISSUE: 1 .. seeking ALS discussion group 2 .. recent ALS paper 3 .. CNTF Efficacy 4 .. re: female voice synthesizer 5 .. motor neuron disease 6 .. another recent ALS article 7 .. CNTF drug trials 8 .. internet for medical librarians 9 .. hereditary aspects of ALS 10 . CNTF side effects 11 . ALS fund raising effort (1) ===== seeking ALS discussion group ========== Date : Mon, 14 Mar 1994 13:02:14 UNDEFINED Sender : Multiple Sclerosis Discussion/Support : >From : Dave Kirby Subject : looking for ALS support group (Lou Gehrig's) Hi, I'm looking for an online group that would discuss ALS (Lou Gehrig's disease) for a friend whose spouse has been diagnosed. Can anyone give me some guidance on finding such a group? (2) ===== recent ALS paper ========== Date: 15 Mar 1994 14:56:55 -0500 (EST) >From: ROSEN@HELIX.MGH.HARVARD.EDU Subject: Recent ALS paper Here is the abstract of a recent paper: Cu/Zn Superoxide Dismutase Activity in Familial and Sporadic Amyotrophic Lateral Sclerosis W. Robberecht et al., J. Neurochemistry 62; 384-387 (1994) Abstract: Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease that is inherited as an autosomal dominant trait in ~10% of cases. Recently we and others identified several single-base mutations in the Cu/Zn superoxide dismutase (SOD1) gene in patients with familial ALS (FALS). Using single-strand conformational polymorphism, we studied the C to G mutation in exon 2 of the SOD1 gene (resulting in a leucine to valine substitution in position 38) in affected and unaffected members of a large Belgian family with FALS. We measured the SOD1 activity in red blood cell lysates in 14 members of this family, including the only surviving clinically affected patient. SOD1 activity of the family members carrying the mutation was less than half that of members without the mutation. In addition, in 11 patients with sporadic ALS and 11 age- and sex-matched controls, red blood cell SOD1 activity was normal. These studies indicate that SOD1 activity is reduced in these FALS patients but not in sporadic ALS patients. Moreover, this SOD1 enzyme abnormality is detectable years before onset of clinical ALS in carriers of this FALS mutation. (3) ===== CNTF Efficacy ========== Date: Mon, 21 Mar 94 07:21:15 MST >From: "gnicholas" Subject: CNTF Efficacy The recently announced law suit against Regeneron has been followed closely but Synergen contacting people in their CNTF test program, asking for volunteers to extend their contract with that company. My Dad was asked if he would be willing to extend his contractual agreement with Synergen for a period of nine MORE months. He is waiting to see the terms of the contract, but is inclined to look favorably on the idea. Based on his reaction to the injections, we believe that he is receiving CNTF, and not the Placebo. About one week into the injections he developed a cough, and began getting the flu every day, about four to five hours after the injection. We decided to move the time of his injection from the am to the pm, about three hours before his bed time. Now he sleeps thru the flu (not flu like), and generally feels a great deal better. The coughing was a little harder to control. He found that 'RICOLA' brand cough drops helped a great deal, as did 'ROBITUSSIN DM'. He seems to have a lot of these symptoms behind him. Perhaps his body has adjusted to what ever is being injected into it. On a very positive note, he has recently found that he can suck in his tummy muscles, something that has not been the case in a long time. He noticed this the other day when he was putting on his pants (one leg at a time). The natural instinct of those of us with a middle that is bigger is to suck it in while zipping and buttoning the fly. He hasn't had any control or response in to this reflex since last summer. Now he can do it at will. Maybe, just maybe .... Grant (4) ===== female voice synthesizer ========== >From : vitale@speech.enet.dec.com Date : Fri, 25 Mar 94 10:19:19 EST Subject: RE: Female voice synthesizer I recently saw Michael's notes re a voice synthesizer. I work in the field of speech synthesis. Digital Equipment Corp. has what is considered by many to be the best speech synthesis technology out there today. It's called DECtalk. It has a choice of nine voices including both male and female voices. Also, rumor is that there may be one coming out in a couple of months which is a serial line version which can attach to any laptop or notebook and which weights only 15 oz. and which is slightly less expensive than the PC card. In addition, many companies offer Voice Output Communication Aids (VOCAs). These include Prentke-Romich Co. out of Wooster, Ohio, Sentient Systems, Inc. out of Pittsburg, Phonic Ear from the West Coast, and a number of others. MultiVoice from the Communication Enhancement Clinic at Children's Hospital-Boston is another good one. Many of these license high quality synthesis. The TRACE Catalog from the TRACE Center at the University of Wisconsin, Madison has a list of all of such devices. If anyone has specific questions about speech synthesizers, I'll be more than happy to answer them. I've been working in the fields of speech science and linguistics since 1971 and am currently President of the American Voice Inpput/Output Society, a large international organization based in San Jose. I just returned from Los Angeles where I gave a 3-hour seminar on Speech Synthesis Technology for Persons with Disabilities. I work in the Assistive Technology Group at Digital where we develop devices for individuals with vocal and visual impairments as well as license speech synthesis technology to other companies who make VOCAs. I'd be more than happy to serve as a resource for anyone who has questions about speech synthesizers. Hope this is helpful. \tony (5) ===== motor neuron disease ========== Date : Wed, 30 Mar 1994 10:00:54 EST Sender : L-HCAP List >From : Bill McGarry Subject: Handicap Digest # 3432 ****** To subscribe to the Handicap Digest mailing list or have your thoughts in the next issue, please send electronic mail to Bill McGarry at any of the following addresses: UUCP: uunet!bunker!wtm INTERNET: wtm@bunker.afd.olivetti.com BITNET: l-hcap@ndsuvm1.bitnet Fidonet: The Handicap News BBS (141/420) 1-203-926-6168 (300 - 14,400 baud, 24 hours) NVN: ABILITY Compuserve: 73170,1064 Bill McGarry (Moderator) (203) 926-6187 ------ Subject: Motor Neuron Disease >From : L.H.Webb@lut.ac.uk (Lynda Webb) Is there anyone who could give help or advice, especially on communication aids for a man with Motor Neuron disease. His voice has now gone so any help on that would be welcome. Also are there any support groups, literature that would help his wife and family in understanding the illness. He is based in Scotland. Has anyone got anything to offer? Thank you in advance. Marion (6) ===== another recent ALS article ========== =============================================== Title : SOD1 missense mutation in an Italian family with ALS Author : Rainero I.; Pinessi L.; Tsuda T.; Vignocchi M.G.; : Vaula G.; Calvi L.; Cerrato P.; Rossi B.; Bergamini L.; : McLachlan D.R.C.; St. George-Hyslop P.H.; Source : 1994 44/2 (347-349) NEURA Neurology Abstract : We have discovered a new Italian pedigree with autosomal- : dominant ALS. The pedigree, at present, comprises 75 : members distributed in five generations. ALS was : diagnosed in eight patients. The mean +/- SD age of : onset of the disease was 46.8 +/- 13.5 years, with a : range of 29 to 63 years. The mean +/- SD duration of : the disease was 11.6 +/- 1.7 months. Molecular genetic : studies showed a missense mutation (Gly-Ser, codon 41) : in exon 2 of the Cu/Zn superoxide dismutase gene (SOD1) : on chromosome 21 in the available affected member and : in 45% of the at-risk subjects of the pedigree. This : study confirms the presence of SOD1 point mutations in : families with autosomal- dominant ALS and suggests that : additional genetic or environmental factors may be : involved in the full expression of the disease. =============================================== (7) ===== CNTF drug trials ========== Topic 986 CNTF drug trials bionet.neuroscience 5:02 pm Mar 20, 1994 >From: maryr@magnus1.com) Could someone please fill me in on how the CNTF was administered and what the side affects were? Thanks, Mary (8) ===== internet for medical librarians ========== Date : Thu, 17 Mar 1994 18:31:35 PST Sender : Medical Libraries Discussion List : >From : Nelson Gilman Subject: Internet for Medical Librarians: A Syllabus INTERNET FOR MEDICAL LIBRARIANS A SYLLABUS Internet for Medical Librarians: A Syllabus, an 83-page publication prepared by information specialist Dudee Chiang, is distributed by the University of Southern California Norris Medical Library. The syllabus emphasizes medical resources on the Internet including electronic mail, telnet, gopher, FTP, listservs and news groups, and related online search tools. Price $12. Orders, accompanied by a check payable to the University of Southern California, should be sent to: Internet Syllabus, Reference Section USC Norris Medical Library 2003 Zonal Avenue Los Angeles, CA 90033 For additional information, call (213) 342-1483. (9) ===== hereditary aspects of ALS ========== >From : sherm_sowby@csufresno.edu (Sherm Sowby) Subject: ALS Info To : bro@huey.met.fsu.edu Date : Fri, 18 Mar 1994 11:34:51 -0800 (PST) I am interested in obtaining some information about the hereditary aspects of ALS. Do you know of any publications that may summarize that information? Thanks Sherm Sowby (10) ===== CNTF side effects ========== According to BIOTECHNOLOGY NEWSWATCH (Monday March 21, 1994) in a copyrighted article entitled, REGENERON STOCKS TUMBLE ON WORD OF SIDE EFFECT OF LOU GEHRIG'S DISEASE DRUG: The side effects eventually subsided, and when those patients receiving CNTF were retested, they showed less weakening of muscles than did the control group. But, the analysis of the data showed that a longer, modified trial will be needed to give statistically valid and significant information. Regeneron added that there "can be no assurance that the study as proposed to be modified and extended will either demonstrate the safety and efficacy of CNTF or provide sufficient data to support a product license application to the FDA." Regeneron anticipated that the 720-patient, 36-site trial would be completed in June, 1994, said Chaisson. Now, she said that Regeneron cannot anticipate when the trial will end. Chaisson would not comment on the proposed modifications because Synergen also has CNTF in clinical trials. (11) ===== ALS fund raising effort ========== According to PR NEWSWIRE (Wednesday March 23, 1994) in an article entitled APRIL FOOL'S DAY 'GRASS CUTTER'S BALL' No Foolin' -- Lawn Mower Racing Season Begins April 1; Lawn mower racing was featured on the 1993 season finale of the ABC-TV comedy "Home Improvement" and on "CBS This Morning." Parade magazine also named lawn mower racing "Worst New Sport of 1992" in its annual "Best and Worst" issue (Dec. 27, 1992). Admission proceeds from The STA-BIL Nationals benefit the Les Turner ALS Foundation, the nation's largest independent organization dedicated to finding a cause and cure for amyotrophic lateral sclerosis, or Lou Gehrig's disease. < parts deleted> /CONTACT: Bruce Kaufman or Karin Janessa, 708-729-7363, for U.S. Lawn Mower Racing Association/ == end of als 90 ==