=============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest #849 (06 May 2001) == == == == ------ Amyotrophic Lateral Sclerosis (ALS) == == ------ Motor Neurone Disease (MND) == == ------ Lou Gehrig's disease == == ------ maladie de Charcot == == == == 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 == == 4700+ subscribers in 70+ countries. Please be advised, == == the editor is not a medical doctor and the Digest is == == not peer reviewed. This newsletter is not intended to == == provide medical advice on individual health matters. == == Any such advice should be obtained personally from a == == physician. == == To subscribe, to unsubscribe, to contribute notes, == == etc. to ALS Digest, please send e-mail to: == == bro@met.fsu.edu (Bob Broedel) == == == == Bob Broedel; P.O. Box 20049; Tallahassee, FL 32316 USA == =============================================================== == Back issues of the ALS Digest are available on-line at: == == http://www.glnicholas.com/ == == http://www.alslinks.com == == http://www.alssurvivalguide.com == == http://cc4144-a.ensch1.ov.nl.home.com/~digest == == http://health.oldeman.net == =============================================================== CONTENTS OF THIS ISSUE: 1 .. re: The ice flow sounds better 2 .. 4th Annual STRATEGIES FOR QUALITY CARE 3 .. re: Pseudobulbar palsey 4 .. First Annual Harrisburg Walk to D'Feet ALS 5 .. Key Signaling Mechanism ... 6 .. re: professor vs. homeopathic? 7 .. Mattinglys disease 8 .. re: ALS information in Spanish language 9 .. Dick Spitzer, my father 10 . Galectin-1 (1) ===== re: The ice flow sounds better ========== >From : HollyHoffy@aol.com Date : Sun, 6 May 2001 Subject: Re: The ice flow sounds better >C.) DRUGS (Pills) Really don't have to tell American Seniors that they >are being ripped off by the major Pharmaceutical organizations.. and by >the FDA (who allows a 65% higher price on most drugs, over the prices >being charged in neighbor countries..FOR THE SAME PILLS.) Wife and I >paying over $700.00 per month... NOW THINK ON THIS!! >> Certainly, I have no answer for this but I do have a suggestion. Check with your doctors to see if your meds can be doubled and then split in half. Many pills cost only a few dollars more for the double strength. Splitting them in half will yield two month's supply for roughly half the price of your original script. I do this with Zoloft and it's really helped my pocketbook. As Bobby said, "NOW THINK ON THIS!" You'll be pleasantly surprised. Holly (2) ===== 4th Annual STRATEGIES FOR QUALITY CARE ========== >From : "Moore, Myrna" Subject: 4th Annual STRATEGIES FOR QUALITY CARE Date : Thu, 3 May 2001 I wonder if you would please include a note on our symposium to be held in Toronto Canada at Sunnybrook&Women's Health Sciences Centre in conjunction with the ALS Society of Toronto on June 8, 2001. The speakers and topics: ALS 101: Dr. Marek Gawel, neurologist Breathing isuues in ALS: Dr. Peter Webster, respirologist Determining and respecting patients' wishes: Dr. Tony Newall, physiatrist. Living Will-power of attorney for personal care: Wendy Moore, Johns lawyer Panel: multidisciplinary approach to care: ;OT;PT;SLP;Society. Caregiver's Journey: A young spouse's story. Looking back, Moving forward: Dr. Neil Cashman, a noted scientific researcher and clinician. This will be held at Sunnybrook & Women's College Health Sciences Centre, 2075 Bayview Avenue, Toronto, Ontario, Canada in the McLaughlin Education Centre in E-Wing. To register please call the ALS Society of Toronto & Area. Tel # (416) 497-2267x217 or 220 Toll Free # 1-800-267-4257 Fax# (416) 497-1256 E-Mail: alstor@idirect.com Registration fees (including lunch) in Canadian Funds are as follows: Health Care Professional (after May 30/01-$65) ............ $60.00 Person with ALS .................. No Charge Family members or caregivers accompanying ALS person .......... $35.00 (3) ===== re: Pseudobulbar palsy ========== >From : "Anne Blacher" Subject: Pseudobulbar palsy Date : Thu, 3 May 2001 The following response on pseudobulbar palsy taken from MDA's new ALS Web site at www.als.mdausa.org might be helpful to you. www.mdausa.org/experts/ask_als-dis.html SUBJ (12/00): PSEUDOBULBAR PALSY, PLS AND ALS My father has recently been diagnosed with pseudobulbar palsy by one physician and primary lateral sclerosis (PLS) by another. My father's symptoms are with his speech and started about three years ago. Otherwise, he says he feels fine. His EMG of the muscles in his arms, trunk and legs were normal. His tongue has started to "atrophy"; it moves constantly from side to side and he has difficulty forming words (speech is labored and slow but understandable). The doctor who diagnosed him with pseudobulbar palsy does not want to start him on any drug therapy because he feels that he doesn't have amyotrophic lateral sclerosis (ALS) yet. Can a person with these symptoms develop ALS at a later time? Are pseudobulbar palsy and PLS the same disease? REPLY from MDA: Maura Del Bene, M.S.,R.N., NP-P, The Eleanor and Lou Gehrig MDA/ALS Center, Columbia Presbyterian Medical Center, New York, NY This is a difficult question in that motor nerve diseases are clinically diagnosed. This essentially means that there is no one test to say yes you do, or no you don't, have this specific disease. It is a combination of both diagnostic tests (EMG) and clinical examination that determines one's diagnosis. This may vary depending upon the symptoms a person has and the interpretation of the examining clinician. To help you understand more, I have listed below a brief description of each component of motor nerve disease. ALS is one of many disorders affecting motor neurons. The general term that one hears for this group of diseases is motor neuron disease. While this term is correct, it can be somewhat misleading because it is imprecise. The diagnosis ALS is given when motor neurons in both the brain and spinal cord are affected. When just the motor neurons in the brain are affected, the diagnosis can be primary lateral sclerosis (PLS), and when motor neurons solely in the spinal cord are affected, the diagnosis is progressive spinal muscular atrophy (SPMA). Progressive bulbar palsy (PBP) is a diagnosis given when primarily cranial motor neurons are affected. The cranial motor neurons control the muscles of the face, neck, and head, including the tongue and throat. The individual diagnosed with PBP most frequently has changes in speech and swallowing. In most ALS centers patients with PBP, SPMA and PLS are offered by their physician the same treatment options for ALS that are available through the FDA or over the counter. These individuals, though, would not necessarily be eligible for ALS clinical trials. The rationale behind this treatment approach is that the effect on the motor nerves is essentially the same yet the progression may be somewhat different in that it is usually slower than that of ALS. The progression of any motor nerve disease is unique from person to person. Approaching the symptoms in an active way to decrease discomfort and to maximize function and quality is the best overall approach on a daily basis. Speak with the medical team (a complement of many disciplines) working with your dad to further explain the management, and possible changes that may occur over time. Anne Blacher MDA Online Services ablacher@mdausa.org (4) ===== First Annual Harrisburg Walk to D'Feet ALS ========== Date : Thu, 03 May 2001 >From : "Julie Morrison" Subject: New Event First Annual Harrisburg Walk to D'Feet ALS Sunday, June 10, 2001 Registration 9am Walk begins at 10 Riverfront Park, City Island-3miles The ALS Association Greater Philadelphia Chapter's Walk to D'Feet ALS is part of a National effort that raised more than $1,500,000 in 2000- it's inaugural year- in support of local patient services programs and nationally directed research. This is our first Harrisburg Walk and we are thrilled to be participating this year. Our goal is to raise $50,000 for local programs and services. We need your help to accomplish that goal. If you would like to get involved, please call Julie Morrison at the chapter office at 215-643-5434 to request more information. Or email at: julie@alsphiladelphia.org. Please get in step and join us as we Walk to D'feet ALS! Sincerely, Scott E. Zimmerman Jody Z. Laverty Event Chairman Family Chairman Julie A. Morrison Special Events Coordinator The ALS Association-Greater Philadelphia Chapter 500 Office Center Drive Suite 340 Fort Washington, PA 19034 215-643-5434 (5) ===== Key Signaling Mechanism ========== Date : Sun, 6 May 2001 >From : Ian Stewart Subject: Key Signaling Mechanism Could Point to Muscular Dystrophy Treatment HONG KONG (Reuters Health) Apr 27 - A research group at the University of Science and Technology in Hong Kong has shown that an enzyme critical in controlling the gene expression of acetylcholine receptors may induce the formation of neuromuscular synapses. The finding may hold promise for the treatment of muscular dystrophy. The Cdk5 protein was first identified by team member Dr. Jerry H. Wang, and is well known for playing an important role in neuronal development and maturation. However, it is only recently that Dr. Wang and his colleagues made the "unexpected discovery" that Cdk5 is localized at the nerve-muscle junction. Their paper is published in the April issue of Nature Neuroscience. In it, Dr. Amy kit-yu Fu and her colleagues write that "inhibition of Cdk5 activity not only blocked the neuregulin-induced acetylcholine transcription but also attenuated the ErbB activation in cultured myotubes." "In light of our finding that overexpression of p35 alone led to an increase in acetylcholine receptor promoter activity in muscle, Cdk5 activation is sufficient to mediate the upregulation of acetylcholine receptor gene expression. Taken together, these results reveal the unexpected involvement of Cdk5/p35 in neuregulin signaling at the neuromuscular junction," they add. Said Dr. Fu in an interview with Reuters Health: "The next step of our research is to replicate these findings from our in-vitro studies in knockout mice. We want to identify the details of Cdk5 involvement in the Erb signaling pathway." In the long-term, she predicts that the identification of Cdk5 and its role at the neuromuscular junction could lead to the development of therapeutic agents to combat different kinds of neuromuscular disorders, specifically muscular dystrophy. Nat Neurosci 2001;4:374-381. Copyright ) 2000 Reuters Ltd. (6) ===== re: professor vs. homeopathis? ========== >From : "Pat & Amie Thornburg" Subject: professor vs. homeopathic? Date : Sun, 6 May 2001 I normally choose not to respond/offer comment to Mr. Sampsons negative posts, as it only gives him the opportunity for more negative comments. For those of you who choose to keep hope alive, and believe that hope offers more than science has to offer, I would like to give other definitions to the term "alternative". Please read the below definitions I found from Merriam-Webster and an on-line medical dictionary. Thank you. Amie Thornburg The definitions I have found: >From Merriam- Webster Collegiate Dictionary Main Entry: alternative medicine Function: noun Date: 1977 Any of various systems of healing or treating disease (as chiropractic, homeopathy, or faith healing) not included in the traditional medical curricula taught in the U.S. and Britain 2nd definition: >From Source: On-line Medical Dictionary, 1997-98 Academic Medical Publishing & CancerWEB Alternative Available in place of something else. (18 Nov 1997) (7) ===== Mattinglys disease ========== Date : Sat, 05 May 2001 >From : jay Subject: Mattinglys disease Title : Geneticists hunt illness afflicting Maryland family; Researchers closer: to finding the cause of debilitating disease. Need to find more Mattingly descendants with this disease for the research project. Gene hunters have moved a step closer to finding the mutation responsible for a rare inherited illness that has crippled scores of people in a single Maryland family, the descendants of Thomas Mattingly, an English colonist who died in St. Mary's County in 1664. A team of scientists says they have narrowed their search to one tip of chromosome No. 9, one of the 23 that contain the human genetic blueprint. In doing so, they've winnowed the number of suspect genes from the 100,000 that comprise the human genome to 10. Pinpointing the gene could one day yield therapies for the Mattingly family illness. And it could shed light on the causes of other neurological ailments. The plan at this point will be to continue mapping studies with Mattingly family decedents to try to narrow the region to be studied even further. Dr. Phillip Chance of the University of Washington, who leads the hunt for the gene would like to identify more people involved in the project. It is in this regard that we put this message on the internet. We are looking for descendants of Thomas Mattingly or those that have the Mattingly Family disease, that is, a disease that first strikes its victims in their teens, and typically surfaces as a sudden weakness in the ankles and hands, and over the course of decades, it paralyzes hands, feet, arms and legs. Its effects resemble those of amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig's disease. Unlike ALS, Mattingly disease has never been found outside of one branch of the Mattingly clan. It starts much earlier in life and advances much more slowly. And it is not known to be fatal. Scientists have identified this disease as Juvenile amyotrophic lateral sclerosis. If you are affected by this disease or know someone who is you are encouraged to contact Jay Lane at jaylane@erols.com, or Andy Jackson at ajack10@bellatlantic.net. (8) ===== re: ALS information in Spanish language ========== >From : "Pat Conway" Subject: Re: ALS information in Spanish language needed Date : Sat, 5 May 2001 You can find a lot of resources in Spanish by going to an English search engine and searching for "Esclerosis Lateral Amiotrofica" I found this Spanish ALS Association, http://www.advernet.es/adela/ , by doing such a search at Yahoo. The other returns I got are these: http://google.yahoo.com/bin/query?p=%22Esclerosis+Lateral+Amiotrofica%22 &hc=0&hs=0 (If you double click on those addresses, you should go right to the page.) Alternatively, you can go to advanced/power English search engines pages (like Hotbot, AltaVista, or Northern Lights) and search for "amyotrophic lateral sclerosis" but specifying that you only want Spanish hits returned. Hope that helps. Pat (9) ===== Dick Spitzer, my father ========== >From : "Maribeth Salge" Subject: Re: Date : Sun, 29 Apr 2001 Hi, As a coordinator of an ALS support group, a physical therapist, and a daughter of an ALS survivor, I would like to share some tidbits with you. After fighting with everything he had, my father finally had to give his body a rest on April 14, 2001. Until that fateful day, I always hoped that Dad's stregth would pull him through each and every challenge. Instead, he contracted pseudomonis, a bacterial infection, about 9 months ago, and had felt not so good since. When an ulcer that he had had for many years started to bleed profusely 3 weeks ago, the infection took over, shut down his organs and took Dad from us within hours of stopping all the heroic measures. I didn't even have the courage to be there. Seeing Dad in a wheelchair was no big deal for me. I see people in wheelchairs every day. I don't however, have to watch all the dying moments of a person that I loved and respected greatly pass before my eyes. I hope he forgives me for not being there, but I just could not do it. At my ok, compassionate care methods only were instated, and within 30 minutes, Dad was gone. I know he had gotten tired of fighting because he felt so bad. While he felt good, he was up for anything! I hope all the PALS will take a page from my father, Dick Spitzer and keep fighting!!! Just be sure to get medical care at the first sign of infection, and have the bacteria cultured so you know what you're dealing with!! In closing, I'll pass on to you one of Dad's favorite witticisms. "As we travel the road of life, two rules that will not bend....never whittle towards yourself, nor pee into the wind!"--Dick Spitzer You go, Dad!!! I love you! Maribeth Salge, MA, PT, ATC, CSCS, RCEP (10) ===== Galectin-1 ========== Date : Wed, 2 May 2001 >From : Will Hubben Subject: Galectin-1 is a component of neurofilamentous lesions in ALS Galectin-1 is a component of neurofilamentous lesions in sporadic and familial amyotrophic lateral sclerosis. Biochem Biophys Res Commun 2001 Mar 23;282(1):166-72 (ISSN: 0006-291X) Kato T; Kurita K; Seino T; Kadoya T; Horie H; Wada M; Kawanami T; Daimon M; Hirano A Third Department of Internal Medicine, Yamagata University School of Medicine, Yamagata, Japan. E-mail: tkato@med.it.yamagata-u.ac.jp. In amyotrophic lateral sclerosis (ALS), abnormal accumulation of neurofilaments induces pathological changes such as axonal spheroids, cord-like neurite swellings, and perikaryal conglomerate inclusions in degenerating motor neurons of the spinal cord, and the accumulation seems to cause motor neuron degeneration in this disease. Such ALS lesions were intensely labeled with HepSS-1, a monoclonal antibody to heparan sulfate. Since the identification of HepSS-1-immunoreactive substance seems to be an important step for understanding the molecular pathology of ALS, we purified the substance from human spinal cord tissue to homogeneity. Amino acid sequence of the protein was consistent with that of galectin-1. Immunohistochemistry using antibodies against recombinant human galectin-1 showed that galectin-1 was accumulated in these lesions in ALS. Although HepSS-1 was believed to be specific for heparan sulfate, it reacted with recombinant human galectin-1 which has no heparan sulfate moiety. The results show that galectin-1 is a component of the neurofilamentous lesions in ALS. Since galectin-1 has axonal regeneration-enhancing activity, the abnormal accumulation of galectin-1 to the lesions seems to be related to the pathological process of ALS. Copyright 2001 Academic Press. MEDLINE Indexing Date: 200104 PreMedline Identifier: 0011263987 --------------------------- Will Hubben whubben@earthlink.net http://www.steveholly.com/Will.html === end of alsd 849 ===