Date: Fri, 20 Jan 95 17:28:18 -0500 From: Bob Broedel To: als@huey.met.fsu.edu Subject: ALSD#167 ALS-ON-LINE =============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest (#167, 20 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: cautiously optimistic 2 .. request 3 .. Computer for Communication 4 .. MND Iceland 5 .. L.A. Conf on Technology and Persons with Disabilities 6 .. Her Illness Brought Out His Goodness (1) ===== re: cautiously optimistic ========== Date : Wed, 18 Jan 1995 10:03:18 -0500 (EST) >From : SPRINGERJ@HAL.HAHNEMANN.EDU Subject: Re: ALSD#166 ALS-ON-LINE I read with interest David Wray's comments, in which he wrote that he has learned to be "cautiously optimistic" regarding any information on ALS. As a scientist working in the field, and a relative of someone who died of ALS last year, I can identify with David's comment. I must admit that I have not contributed responsibly to this digest and am taking this opportunity to comment and give my opinion on some of the basic research related to ALS. Soon, we are going to hear the outcome of two of clinical trials; one from Cephalon, Inc with Myotrophin, and the other from Rhone-Poulenc with Riluzole. Myotrophin is a growth factor that has been shown to stimulate the sprouting of nerves innervating the muscle. It is hope that this growth factor will slow the progression of clinical symptoms by stimulating motor neurons that are not yet affected by the disease process. Riluzole is reported to block the release of glutamate, an amino acid that serves as one of the major neurotransmitters in the nervous system. Neurotransmitters are molecules that are responsible for sending information from one neuron to another. In sufficiently high amounts, however, glutamate can over stimulate a neuron and deplete it of it's energy, which ultimately results in the death of the neuron. It is thought that this may be occurring in ALS (overstimulation of motor neurons by glutamate), and work from several labs support the idea that too much glutamate may be circulating around the motor neurons. One clinical trial has already been completed and some interesting results emerged. Riluzole was found to be beneficial in ALS patients with bulbar onset, but not limb onset. The reason for this is not clear, but it is hoped that the outcome of the larger, more recent trial will help to clarify this initial finding. On the basic science front, there are a number of findings that are helping us to understand the multitude of events that may contribute to motor neuron death in ALS. In David's commentary, he mentioned growth factors, the immune system, and mutant genes. A recent finding reported in Science is that a new growth factor, called GDNF, is currently the most potent growth factor for increasing motor neuron survival. On the surface this is a great finding, but there a number of issues that must be addressed in order to determine whether GDNF is destined to be used in ALS. One concern I have is that GDNF is found in almost every tissue and organ in the body. Does this growth factor have multiple effects in different tissues? If so, what will happen when we put this growth factor into the body? A second concern is that the studies to date have only looked at the effects of GDNF in developing motor neurons. The role of GDNF in the adult may be completely different. One study that should be done, if it is not already in progress, is to look at the ability of GDNF (and other factors like BDNF) to slow the progression of motor neuron loss in the SOD gene transgenic mice. This would provide better evidence that these growth factors can function in adult motor neurons. With respect to the SOD gene mice, these findings indicate that motor neuron loss can occur in mice when a mutated form of the human SOD gene is introduced into these mice. The key point here is that the mutations (there are different mutations in the SOD) occur in approximately 1 out of 3-4 familial ALS patients. This is certainly an interesting finding for those individuals with the mutation, especially if the recent positive report that treatment of a single familial patient with non-mutated, bovine SOD stands up to rigorous testing. On the other hand, how do these findings impact the other 90-95% of the ALS patient population who presumably do not have the SOD mutation? Is SOD still one of the culprits in these patients? If so, there may be opportunities to help correct abnormal SOD function. If not, we may be looking at a unique event that happens in only a few ALS patients. The immune system theory has had it's share of ups and downs. But recent studies in several labs have found that motor neurons grown in culture die when treated with antibodies isolated from the serum or spinal fluid of patients with ALS. The actual antibody responsible for this is not known, but the findings indicate that motor neurons (grown in culture) express some molecule that is recognized by the ALS antibody, and this recognition results in the death on the neuron. Finally, I have to make a comment about the focus of the field in general. Most of the popular scientific work (and the press it has received) involves studies that are looking at the survival of spinal cord or brainstem motor neurons. ALS is a disease where motor neurons die in the spinal cord, brainstem, and cortex. However, there are relatively few studies that are focusing on the motor neurons in the cortex. I am not condemning the efforts to understand why motor neurons die in the brain stem and spinal cord, but the disease process must be investigated in it's entirety. If not, there may come a time when we can treat one set on neurons, but have neglected to figure how to rescue the cortical motor neurons that play a role in controlling the motor neurons in the spinal cord. In closing, I hope that this contribution has been informative and I welcome any comments to clarify or address my statements. Sincerely, Joe E. Springer, Ph.D. Associate Professor of Anatomy and Neurobiology Medical College of Pennsylvania/Hahnemann University (2) ===== request ========== Date : Fri, 20 Jan 95 11:53:48 PST >From : jfee@jeeves.ucsd.edu (jfee) Subject: request I recently learned that a very dear friend of mine, who lives in a rural western area, has contracted a motorneurone disease that has been variously diagnosed all the way from ALS to pesticide poisoning, depending on who he talks to. I have some knowledge of basic research in the SOD field and told him that I would attempt to find an organization that could provide a definitive diagnosis of his condition. He agreed to let me pursue that. Therefore, I am asking for information about medical specialists in the Texas/New Mexico/Colorado/Arizona region that might provide help in this matter. There is some urgency to this matter, and any assistance would be greatly appreciated. James A. Fee Senior Research Scientist Department of Biology University of California at San Diego 9500 Gilman Drive La Jolla CA 92093 619/534-4424 email = jfee@jeeves.ucsd.edu (3) ===== Computer for Communication ========== Date : Thu, 19 Jan 1995 18:31:54 -0500 >From : BWRAYNSTRM@aol.com Subject: Computer for Communication I've read several inquiries about communication aids. The following information may be helpful to someone. The state of Maryland provides assistance to disabled individuals through its Department of Rehabilitative Services. With failing speech, I needed a voice synthesizer. In order to qualify, I had to be employed or employable. They evaluated my situation and provided a computer system to meet my needs. The system includes the following: - A Compaq laptop computer, 486x25mhz, full color screen, 200mb hard drive, 12mb memory, WINDOWS format I have the following key programs for work: - WORDPERFECT 5.2 - LOTUS 1-2-3 release 4.01 - DELRINA WINFAX PRO 3.0 - Headmouse with WIVIK 2.1 software (on-screen keyboard) - Modem, 9600 baud rate - Voice synthesizer with WIVOX 1.0 software Although I can't lift my arms, I have ample strength in my fingers to click a large round button (jellybean switch). This functions as a mouse button. The headmouse brings it all together. A reflective dot is mounted between my eyes on a cheap pair of sunglasses (lenses removed). The sensor module (about size of small cassette player), mounted above the computer screen, picks up the light reflected off the dot and moves the cursor around the screen according to my head movement. The Wivik software provides an on-screen keyboard. With fairly modest head movement, I place the cursor on the letters or numbers. With a click of the jellybean switch the spelling is displayed in any word processing program I'm running. The voice synthesizer (small unit mounted under the laptop) will speak whatever text I highlight with the cursor. The Wivik keyboard program can save 10,000 words of my vocabulary and complete each after only a few letters are typed (called Word Prediction). It can even remember and recall sentences with only a few keystrokes. There are a few drawbacks. The evaluation and approval process takes a long time (almost five months for me). The system is expensive ($10,000 for mine). The state pays for a portion based on income using a sliding scale (The less you make the more they pay) and they retain ownership. Finally, it helps to be computer literate, but my state provides training. I'm pleased with my system. I have many other programs "loaded" to keep me occupied. America Online allows access to this digest. I play chess with one of my PALS in Boston. I communicate regularly with friends and relatives. Don't procrastinate as I did. Check with the agency in your community that offers rehabilitative services. Contact me for more information about my system. David Wray (4) ===== MND Iceland ========== >From : jongus@rhi.hi.is (Jon E Gustafsson) Subject: MND Iceland Date : Sun, 15 Jan 1995 15:33:37 +0000 (GMT) My name is Jon Gustafsson and I am a member of the Icelandic MND Society. I am interested in keeping up with the development of MND research and forwarding that information to MND patients in Iceland. My e.mail address is "jongus@rhi.hi.is" Yours sincerely, Jon Gustafsson, Iceland. (5) ===== L.A. Conf on Technology and Persons with Disabilities ========== Date : Thu, 19 Jan 1995 13:37:49 -0500 >From : GlennB7490@aol.com Subject: L.A. Conf on Technology and Persons with Disabilities L.A. Conf on Technology and Persons with Disabilities, March 14-18, 1995 The Center on Disabilities at Cal State University - Northridge (18111 Nordhoff St, Northridge CA 91330-8340, 818-885-2578/voice/TDD/msg, 818-885- 4929/fax, ltm@csun.edu) has announced the above-titled conference. Fees range from $150 to $295 by March 1st, and $200 to $345 thereafter. (6) ===== Her Illness Brought Out His Goodness ========== Source: THE SACRAMENTO BEE 01/08/95 Title : HER ILLNESS BROUGHT OUT HIS GOODNESS Byline: Diana Griego Erwin Bill Barta was a rotten husband. He drank too much, stayed out on the town with his friends too late and generally neglected his wife, Marge, for much of their marriage. But now his family is declaring the man a saint. "You have never seen such a transformation," says his stepdaughter, Greenhaven resident Debby Puliz, 45. Eight years ago, Marge Barta, 62, was headed toward carpal tunnel syndrome surgery when her doctor decided to conduct one more test. The test revealed what the doctor had begun to suspect. Marge had amyotrophic lateral sclerosis, commonly known as Lou Gehrig's disease. Doctors said she'd be lucky to live six months. The family's reaction was textbook: "We all rushed around and fell apart," says Puliz, the younger of Marge's two daughters. But what happened to Bill Barta was amazing. "My dad became a totally different person from the day my mother was diagnosed," Puliz says. "He had always been kind of a playboy: He liked to drink, he liked to go to the races. People adored him, he had lots of friends, but he just wasn't a devoted, I'm-here-for-you kind of guy. You would never imagine him being someone's caretaker. He needed someone to take care of him." < parts deleted because of copyright > He experimented with new breakfasts to please her. He learned how to wash Marge's hair, recruiting a stylist to show him the best way to do it. Puliz recalls watching how he blotted it dry; applied mousse; styled it just the way her mother liked it. And every morning, he carefully outlined her mouth in Revlon's Soft Silver Rose, Marge's favorite lipstick. As her health worsened, especially the facial pain, he delicately flossed her teeth because it seemed to relieve her somewhat. < parts deleted > He never left the room without kissing her. < parts deleted > To the end, his devotion and dedication to Marge was unfailing and unbelievable. Puliz sees this as a love story that surprised everyone who thought they knew the couple well. It also proves that people sometimes have a goodness hidden deep inside that only adversity can draw out. During Marge's struggle, the couple talked as they never talked before. Puliz says she knows they said everything they wanted and needed to say. There were no regrets. < parts deleted > "If my mother had a voice right now," Puliz said of her mother, "she'd be telling people Bill was a saint." IT WAS DEC. 23 AND EDGING toward midnight. Bill told his wife he was moving to the couch to sleep because his back was bothering him. Those would be their last words. Shortly thereafter, Marge heard a thud in the living room of their Sacramento home. Lou Gehrig's disease saps the body, and particularly the muscles, of all strength. It took Marge two hours to reach for the phone and dial 911. Bill, meanwhile, lay on the floor, stricken by a massive stroke. After eight years of caring for his Margery, he passed away at Sutter General Hospital Dec. 28. He was 78. "I like to think," Puliz says, "that he's still paving the way -- making things easier for my mom." DIANA GRIEGO ERWIN'S column appears Sunday, Tuesday and Thursday. Write her at P.O. Box 15779, Sacramento, 95852, or call (916) 321-1057. === end of als 167 ===