>i was reading a womans magazine lately and a man from south australia asked >for information on >multiple system atrophy >it was diagnosed firstly as parkinsons and he is asking for information for >himself and his wife who has the disease now correctly diagnosed >i think i saw a refernece to this some weeks ago but deleted as i wasnt of >interest to me >i have faxed him this website but would appreciat any help for him >________________________________________________________________________ >Get Your Private, Free E-mail from MSN Hotmail at http://www.hotmail.com Judy--- Here's some info that may help him--- You are invited to join the Multiple System Atrophy electronic mail discussion list. You'll find patients and caregivers sharing information on symptom management and coping strategies for dealing with the various forms of MSA which include Shy-Drager Syndrome (SDS), non-hereditary Olivopontocerebellar atrophy (OPCA) and Striatonigral Degeneration (SND). Anyone is welcome to join regardless of diagnosis. This list is sponsored by David Robertson, GCRC at Vanderbilt University Medical Center. The list manager is Sylvia Dickinson, RN. To subscribe to the list: Send an email to: [log in to unmask] and in the body of the message write subscribe Shy-Drager We hope that this list will serve as a forum to exchange information and support for those who are diagnosed with various forms of Multiple System Atrophy, their families and friends as well as anyone who has an interest in this disorder. Please send a message to the list (if you want to) introducing yourself and any MSA related topic you would like to discuss. The introductory message as well as all other "public" messages should be sent to the following Internet address: [log in to unmask] Your message will be automatically sent to all subscribers. Conversely, you will receive all messages sent to the above address. If you want to REPLY *PUBLICLY* to the sender of a message, use the "REPLY" feature of your mail system. (In other words, the message will be returned to the list server and will be distributed in that way.) If you want to REPLY *PRIVATELY* to the sender of a message, you must "SEND" to that person's email address. To LEAVE the list at any time, address an email message to [log in to unmask] and in the body of the message write: unsubscribe Shy-Drager It is a good idea to leave the list if you are going to be away from your computer for a couple of months. To resubscribe, simply address mail to: [log in to unmask] and in the body of the message write subscribe Shy-Drager Please do not write subscribe and unsubscribe messages to the [log in to unmask] address. They will go out to everyone on the list. *********** Parkinson's Report National Parkinson's Foundation, Inc. VOLUME XIX - ISSUE 2 / Spring 1998 Multiple System Atrophy By members of the National Parkinson Foundation Center of Excellence at Vanderbilt University, including David A. Robertson, Director, Nathan S. Blaser Shy-Drager Research Laboratories; Thomas L. Davis, Director, Movement Disorder Clinic; and Ariel Y. Deutch, Director, NPF Center of Excellence Although the cause of idiopathic Parkinsonís disease is unknown, Parkinsonís disease is probably the best characterized of the neurodegenerative disorders. The loss of dopamine in the striatum is the major contributor to the disorder. However, there are several other neurodegenerative disorders involving several different systems in the brain, in which striatal dopamine loss is also found. Among these other neurodegenerative disorders is multiple system atrophy (MSA), in which degeneration in diverse brain regions leads to problems in the control of movement, balance, blood pressure, and sexual and urinary tract function. MSA is often accompanied by some striatal dopamine loss and in certain patients typical parkinsonian symptoms are either the first noted or the most prominent. A number of areas of the brain are involved by MSA. This has led to different varieties of MSA receiving different names, depending on which area of the brain has predominant involvement. When MSA begins with imbalance, incoordination, and difficulties in speaking (dysarthria), it is often called olivopontocerebellar atrophy; as the name suggests, this form of MSA is marked by degeneration in the cerebellum, a structure involved in balance and learned motor tasks. When a patient initially has rigidity (stiffness) and slowness in initiating movements (bradykinesia) that is out of proportion to tremor, this MSA form has been called striatonigral degeneration, involving communication between nerve cells in the striatum and midbrain. In patients in whom changes in autonomic function dominates the initial presentation, particularly changes in blood pressure regulation, the MSA form is often called Shy-Drager syndrome. Between 25,000 and 100,000 American have multiple system atrophy. However, many will not receive the correct diagnosis during their lifetime. This is due to the difficulty in differentiating MSA from other disorders (including relatively common degenerative disorders such as Parkinsonís disease and more rare ones such as pure autonomic failure). MSA usually occurs after age 50, with a slightly higher incidence in males. Patients usually have autonomic nervous system dysfunction first. Genitourinary dysfunction (difficulty with urination) is the most frequent initial complain in women, while impotence is the most frequent initial complaint in men. Orthostatic hypotension (a large drop in blood pressure upon standing) is common and may cause dizziness, dimming of vision, head or neck pain, yawning, temporary confusion, slurred speech, and if the hypotension is severe, the patient may "faint" upon arising from a recumbent position. In spite of low blood pressure while standing, it is common for MSA patients to have high blood pressure when lying down. A fall in blood pressure following meals or in hot weather or following infection is quite common. When MSA begins with non-autonomic features, imbalance is the most common feature. This difficulty in maintaining balance may be due to either cerebellar or Parkinsonian abnormalities. Some patients complain of stiffness, clumsiness, or a change in handwriting at the onset of MSA. The concurrent involvement in MSA of multiple brain systems subserving movement, including the striatum, cerebellum, and cortex, leads to the movement disorder as often being the most profound disability. Hoarseness or even vocal paralysis are relatively common, as are sleep disturbances, including snoring and sleep apnea. The ability to swallow foods and liquids may be impaired. The initial diagnosis of MSA is usually made by carefully interviewing the patient and performing a physical examination. However, more testing is often needed to confirm the diagnosis. Among the tests that are helpful in determining the presence of MSA are several types of brain imaging including computerized tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET). Pharmacological challenge tests (administering certain drugs in the presence of various types of movements of the patient) may also be of help. In those patients with typical parkinsonian signs, an incomplete and relatively poor response to dopamine replacement therapy (such as l-dopa [Sinemet]) may be a clue that MSA is present. The characteristic involvement of multiple brain systems is a defining feature of MSA, and one that on autopsy confirms the diagnosis. Recently, several groups have reported the presence of unusual inclusions in certain types of brain cells. These glial cytoplasmic inclusions are, as the name indicates, typically found in glial cells, which are the structural and metabolic support elements of the brain but are not neurons (nerve cells). Glial cells are central to maintaining the correct balance of ions in the brain, without which neurons cannot survive. Moreover, glial cells express certain proteins that accumulate and thereby limit extracellular excitatory amino acids that can be toxic to neurons. These functions of glial cells, coupled with the presence of glial cytoplasmic inclusions in MSA but not Parkinsonís disease, have sparked considerable research interest. It is noteworthy that a different type of intracellular inclusion in nerve cells, the Lewy body, is present in Parkinsonís disease but not MSA. In MSA, there is loss of function in the two divisions of the peripheral nervous system: the sympathetic and parasympathetic nervous systems. Although the autonomic nerves themselves are largely intact, the brain loses its capacity to properly engage them to control the autonomic function. Consistent with the involvement of many brain regions in MSA, the concentrations of many neurotransmitters in the brain are reduced in MSA. As with Parkinsonís disease, the cause of MSA remains unknown. Antibodies in the spinal fluid of patients with MSA have been shown to react with a specific area in an experimental animal brain, raising the possibility that MSA may be related to an abnormality of the immune system. It is also possible that MSA is due to abnormal folding of some unknown protein. At this time, however, these observations require independent confirmation in large groups of patients, and the relationship of such changes to specific symptoms in MSA remains unclear. What is clear is that there is a compelling need for research into the causes, and hence treatment and cure, of MSA and Parkinsonís disease. MSA is a rare and sporadic disorder and available evidence does not support a hereditary component to the disorder. Among more than 400 patients evaluated at Vanderbilt University Medical Centerís Autonomic Dysfunction Center during the past 20 years, not one had a family member with MSA, although a number of them had family members with Parkinsonís or Alzheimerís disease. While it is possible that a few of these family members diagnosed with Parkinsonís or Alzheimerís disease might have actually had MSA, available data strongly suggests that MSA is not inherited. In Parkinsonís disease there is a similar but not identical situation, with hereditary forms of the disease representing only a small minority of the patients; even in these patients, the disease process differs somewhat from idiopathic Parkinsonís disease. There is no evidence that MSA is contagious; we have never observed people in the same house who developed the disease. Given the relative rareness of MSA and the frequent misdiagnosis of the disorder, it is not surprising that there is a paucity of careful epidemiological investigations of MSA that allow one to identify predisposing environmental factors. Although one report raised the possibility of a small effect of exposure to environmental toxins and another report suggested a slight correlation with prior head injury, these claims have not yet been supported by other studies. In particular, MSA does not appear to be related to or caused by prior alcohol or drug abuse, poor nutrition, or other disease process earlier in life. MSA may progress rapidly. Patients survive an average of nine years following onset of illness; some patients live as much as twice this long. Current treatment of MSA is symptomatic. The most valuable agents to increase blood pressure are fludrocortisone and midodrine. In addition, most patients with MSA derive some benefit from typical antiparkinsonian medications such as levodopa (Sinemet), dopaminergic agonists (pergolide and bromocriptine), and anticholinergic drugs. In summary, MSA is a severe neurodegenerative disorder of unknown cause. There is currently no cure for MSA, nor is there any therapy available that stops or slows the progression of the disease. At this time, treatment is aimed at treating problems as they arise, and thus requires careful monitoring of the patient by a skilled and experienced clinician with expertise in MSA. The lack of specific treatments to cure or slow the progression of MSA is disheartening to patients and their loved ones and caregivers. However, exensive research efforts aimed at advancing our understanding of MSA, Parkinsonís disease and other neurodegenerative disorders are in place, and we have enjoyed a period of very rapid advances in understanding of the pathophysiology of neurodegenerative disorders. We can expect such advances to culminate in a better understanding and treatment for MSA and Parkinsonís disease over the next decade. Multiple System Atrophy Olivopontocerebellar Atrophy Striatonigral Degeneration Shy-Drager Syndrome Symptoms of MSA difficulty with urination impotence orthostatic hypotension gastric fullness loss of sweating frequent nighttime urination imbalance incoordination hoarseness/snoring muscle weakness Parkinsonís Disease vs. Multiple System Atrophy: Observations Suggestive of MSA Poor response to Sinemet Low blood pressure on standing Difficulty with urination Use of a wheelchair Loud snoring or loud breathing Frequent nighttime urination Treatment of MSA Fludrocortisone (blood pressure) Midodrine (blood pressure) Sinemet (movement disorder) Dopaminergic Agonists (movement disorder) Anticholinergics (movement disorder) Erythropoietin (anemia) Camilla Flintermann, CG for Peter 82/70/55 Oxford, Ohio http://www.newcountry.nu/pd/members/camilla/one.htm <[log in to unmask]> also, on PDWebring at http://members.tripod.lycos.nl/genugten/flinterm.htm "Ask me about the CARE list for Caregivers of Parkinsonians ! " And visit the CARE webring at http://www.crosswinds.net/~caregivers/index.html