Multiple Sclerosis and Demyelinating Disease
About the Author
Patricia Marx, RN, BSN, MS has worked in nursing since 1979 with a certification in neuroscience nursing, oncology, and hospice and palliative care. She was diagnosed with multiple sclerosis in 1993 and writes this course from a professional and personal level. She has struggled for many years with relapsing-remitting MS that has now become secondary progressive. Ms. Marx teaches nursing at Penn State and is a PhD student in nursing. She has been awarded a grant from the Hartford Foundation for Excellence in Geriatric Nursing. She has a master's degree in psychology from The State University of New York at Binghamton where her primary focus was on research of neurological diseases.
Excerpts prepared by Shelda Hudson, RN, BSN, PHN. Ms. Hudson's responsible for directing the activities of this department, selecting qualified, credentialed authors for the courses offered by the National Center as well as advising staff of required course design and criteria. Ms. Hudson has over 18 years of extensive experience.
Purpose and Goals
The goal of this course material is to educate nurses and other healthcare professionals in the nature and management of Multiple Sclerosis and demyelinating diseases. Topics include: types of MS, pathophysiology, symptomatology, pain, MS alternative treatments and more.
- Recall the pathophysiology of the demyelinating disorders.
- Outline the role of myelin in the transmission of neural signals within the central nervous system.
- List the demographics and peculiar geographical distribution of multiple sclerosis.
- Name the characteristic types of multiple sclerosis.
- Describe the primary symptoms of multiple sclerosis.
- Identify the four types of pain common to multiple sclerosis.
- Recognize the potential cognitive changes that may occur in individuals with multiple sclerosis.
- Identify paroxysmal symptoms of multiple sclerosis.
- State the major drugs used to treat multiple sclerosis.
- List types of complimentary and alternative therapies used for people with multiple sclerosis.
Multiple sclerosis is a chronic autoimmune disorder that can affect movement, sensation and many bodily functions. It is caused by the destruction of the coating of myelin that surrounds the nerves of the central nervous system. This myelin helps the neurons to transmit signals from the brain and spinal cord to the rest of the body. When it is destroyed, signals are transmitted erratically or not transmitted at all. Scar tissue, called plaque, is deposited over the neuron causing an even more severe barrier to communication.
Multiple sclerosis has a very broad range of symptoms that can affect nearly every system in the body, including vision, sensation, coordination and bodily functions that most of us take for granted.
The History of Multiple Sclerosis
It appears that the earliest description of multiple sclerosis comes from the late fourteenth century. At the age of 16, a young woman named Ludwina who lived in Holland fell while skating because of weakness in her legs. She recovered strength in her legs but after that had recurrent episodes of loss of balance, weakness, and visual problems. This progressed over decades until she was unable to walk. Very specific records were kept by the Vatican on this woman because she later was declared to be a saint.
Three hundred years later in England, an illegitimate grandson of George III kept a diary for 26 years in which he recorded the progression of his symptoms which we now believe to be those of multiple sclerosis.
In 1868, a French neurologist named Jean-Martin Charcot provided a definitive description of what we now know as multiple sclerosis (MS). When he did autopsies on individuals with this disorder, he found the presence of "plaque" in the brain and spinal cord. He was a meticulous artist and published his drawings of these lesions.
From that time until now, scientists and physicians have been trying to better understand this disease in order to help patients who suffer from this debilitating illness. Yet, there is still speculation about the cause of the disease. New diagnostic tools and new drugs have been developed, yet the cause of MS remains a mystery.
An Introduction to Demyelinating Diseases
Demyelinating diseases are a category of pathologies that affect the white matter of the central nervous system. This white matter is called "myelin" and it is a protective white coating that surrounds the nerves in the brain and spinal cord. This allows a smooth passage of electrical discharges (action potentials) within the nervous system. Just as wires in an electrical system require an "insulator" so that electricity can flow smoothly, our central nervous system requires this coating too, so that electrical signals do not jump from one place to another and are able to reach their final destination.
Most of the symptoms of demyelinating diseases occur because of the destruction of the myelin. As the myelin is stripped away, scar tissue, called plaque, forms where the myelin originally had been. The scar tissue is not conductive and impedes the smooth-travelling of the signals from the brain to their final destination.
In this course we will examine three major demyelinating diseases: multiple sclerosis, amyotrophic lateral sclerosis (also called "Lou Gehrig's Disease") and adrenoleukodystrophy (ALD). They have many factors in common, but we will discuss them separately in the course, because they have a different diagnostic perspective and different drugs are used for treatment.
Specifics on Multiple Sclerosis
Multiple sclerosis is a major cause of chronic disability in young adults with the onset of the illness occurring between the ages of 20 and 40 years old. Onset of the disease is rare before puberty, reaches a peak at age 30, and after the age of 60, the onset of multiple sclerosis is negligible.
The disease affects more women than men at a ratio of 3:2. Five times as many Caucasians have the disease than Blacks. The disease afflicts 350,000 people in the United States. Approximately 200 people are newly diagnose each week. However, according to a recent report in the journal "Neurology" (11/2007), the incidence of multiple sclerosis is grossly under-reported. The journal criticizes the Center for Disease Control for reporting such a low number. Autopsy reports alone show that thousands of cases of MS go undiagnosed. A more reasonable estimate is that there are over 600,000 people in the United States with multiple sclerosis. In this same journal, the opinion is also voiced that the incidence of multiple sclerosis has skyrocketed in the past fifty years and more attention and resources are needed to find the cause of this disease. MS is a very financially and emotionally draining chronic illness.
Potential Contributing Factors
There is a striking difference in the number of cases of multiple sclerosis according to geographic distribution. This is well known and has been thoroughly researched. The incidence of MS in countries north of the 45-degree parallel latitude, including the northern part of the United States, Canada, and Northern Europe is extremely high. Scientists speculate that perhaps a climatic variable, such as temperature or the amount of sunlight a particular area receives each day influences the development of multiple sclerosis. Others believe that a particular virus may affect people living in temperate climates but this virus does not find tropical climates hospitable.
Genes may play a role in creating a susceptibility to multiple sclerosis. Certain populations have a higher incidence of autoimmune disease. It is clear that MS is an autoimmune disease. The body's own defense mechanisms found in the T-cells begin to attack not foreign invaders (like bacteria and viruses), but the person's own body. In the case of MS, T-cells attack the myelin and as the body attempts to heal itself, plaque is formed. Individuals with the human leukocyte antigen(HLA) have a much higher incident of, than those without this antigen. Newer studies with identical twins reveal that if one of the twins has MS, there is a 30% chance the other twin will also develop the disease.
Another potential factor in MS is some sort of infectious agent. A team of researchers found that individuals with MS have a higher titer for the measles antibody than a control group. Others implicate the Epstein-Barr virus, the herpes virus (HHV-6), and a retrovirus. However, there is no clear evidence for this viral/bacterial theory.
Speculation about diet is one of the mysteries that surrounds MS. The countries where there is the highest prevalence of MS are some of the wealthiest nations in the world. A characteristic of these nations is that the people in them consume much more animal fats and dairy products than in poorer nations. A diet containing no meat or milk products, called "The Swank Diet" has been circulating for about 20 years. No research has been done to understand the impact of this diet on the progression of MS.
As can be seen from this discussion, there are many theories about the cause of MS, but the disease still mystifies researchers. No etiology for MS has been found. It does not fit into a neat little box, so trying to find a cure for the disease remains difficult. A combination of factors (geographical area, genetic predisposition, a slow-acting virus) may be responsible.
Types of Multiple Sclerosis
There are several types of multiple sclerosis. These different types mandate a various drug treatment, and most-likely, a different prognosis.
It is customary to classify MS according to the diseases progression such as: remitting-relapsing, chronic progressive, secondary progressive, or progressive and aggressive from onset.
The most common form of MS is the relapsing-remitting variety. It is characterized by an initial attack, a remission that gives the person an almost complete return of all functional losses, and then periodic relapses that may occur a few months or even decades later. After the relapse, the person can have significant recovery of function. If the initial symptom was loss of vision (optic neuritis), the vision may return completely. As a person experiences more relapses over the years, there may be a much poorer chance that they would regain complete functional ability and they are left with a "residual" of the symptoms.
As the person ages, the course of the disease may evolve into "secondary progressive." The person then experiences more permanent symptoms without further remissions.
In the chronic progressive type of MS, the person will have an initial attack followed by a gradual worsening of symptoms over a period of years without any sign of a remission. This type of MS is usually found in those individuals who were diagnosed after the age of 43.
A rare malignant form of MS involves death within five years of diagnosis. Severe and intense demyelination and the development of demyelination in the parts of the brain responsible for vital bodily functions, particularly the brain stem, may result in death because of the inability of the brain to control respiration and heart rate.
The Pathophysiology of Multiple Sclerosis
Multiple sclerosis is not an easy disease to find a cause for, categorize, or treat. Many variables confuse the picture of how MS can affect a person throughout their lifespan.
Despite this medical professionals and research scientists need to understand the pathophysiology of the disease in order to help the patient better understand what to expect and how to cope with this chronic and often debilitating illness.
We know from basic anatomy and physiology that the neuron is the basic unit of structure of the central nervous system, responsible for all bodily activity. The heart beats because of a complex signaling system that begins in the brain and is transmitted via neurons to the heart. When we become frightened or anxious, a complex network of hormones affects these neurons and they tell the heart to speed up. Every movement is initiated by the working of neurons which can be finely tuned in an Olympic athlete or damaged as in a child with cerebral palsy who struggles with walking, talking, and even controlling bodily movements.
When any damage occurs to neurons, specific functions are impaired. So, using a common example, when a person has a cerebrovascular accident (CVA), if the neurons in the speech center are damaged, the person will have problems with talking.
Neurons in the central nervous system of adults are thought to be incapable of regeneration. In very young children, it has been shown that neurons will actually regenerate and a child may regain function much more easily than an adult. This degree of regeneration is called "neural plasticity." Very young children have a high degree of plasticity.
In multiple sclerosis, the damage does not initially occur to the actual body of the neuron but the covering surrounding that neuron, called the "myelin." So, to review once again this process: the myelin is attacked by the body's own immune system. Scar tissue forms (plaque) and this scar tissue is termed "lesions". These lesions impair the conductivity of the nervous system.
Symptomatology of Multiple Sclerosis
Multiple sclerosis can present with a legion of symptoms, which are often puzzling for the physician who must attempt to diagnose the problem a person is having. These symptoms at first may "come-and-go", making it seem at times as though the doctor is looking at a "mental condition". Many individuals with MS become angry and frustrated. They return again-and-again to a doctor with vague symptoms such as transient visual loss, periods of vertigo, and numbness that can occur for a period of a few hours and then disappear. Because the lesions of MS can occur anywhere in the brain or spinal cord, one individual may have the presenting symptom of facial pain. , while another may have trouble lifting a foot or odd sensations of burning or tingling in a leg.
There are six characteristic initial symptoms found in most individuals with multiple sclerosis (Figure 1).
These symptoms can occur for a period of several hours or days and then seem to disappear. Many people diagnosed with MS may have had the disease for many years and just ignored the symptoms until they became more alarming. At this point, the person may have first visited a physician about them.
Because they tend to "come and go", it is difficult to diagnose MS after only one episode. The diagnostic criteria for MS mandates that at least two episodes must occur before there can be absolute certainty that the person has MS.
As an individual experiences recurrent relapses (or in the chronic progressive form, has no remission but only worsening symptoms), specific symptoms become more prominent including increasing weakness, spasticity, ataxia, sensory loss, and the loss of bladder control.
Weakness in one or more extremities is a predominate feature of multiple sclerosis. It usually begins with one leg. In one case study, a woman reported that she was driving her car and each day it seemed as if something was wrong with the brake pedal because it became more difficult to press. Her husband realized it was not the brake pedal, and the woman was subsequently diagnosed with multiple sclerosis.
Patients may complain of a heavy feeling in a leg or the inability to move a leg properly after a period of exertion. As the disease progresses, the weakness may result in a total inability to use that leg. The arms may also be affected as the disease progresses. This may first be noticed by a tremor that occurs when the person goes to reach for a coffee cup.
In rare cases, weakness of the muscles of the diaphragm lead to the inability to fill the lungs completely with each breath. As a result patients may develop chronic respiratory infections. Pneumonia can be quite deadly for MS patients. A health care professional must be on alert for shallow breathing and the restriction of the muscles used for respiration.
Spasticity is another cardinal feature of multiple sclerosis and is most commonly seen in the lower extremities. The examiner may see increased extensor tone and hyperactive reflexes in a leg. This may first be noticed by the patient as they lay down in bed for the night. Their legs become very rigid and, at times, painful. As the disease becomes chronic, flexor tone begins to take control of the leg. This can be seen in an unsteady gait and result in sudden falls. The person feels that their "legs have gone out from under them." These spasms are painful because of the severe muscle rigidity, even worse, may become disabling. The force of them can actually make it difficult for a person to remain in a wheelchair when the spasms occur in the lower back and buttocks. Many people with advanced MS are at a high risk for falls in health care facilities and at home.
Fatigue is one of the most insidious symptoms and may begin very early in the course of the disease. Some patients with MS describe it as feeling like they are walking around with a heavy wet blanket draped over them. Fatigue is not the same as "tiredness." Patients report that even after a complete night's rest, they wake up feeling exhausted. Fatigue may interfere with a person's job performance, their ability to have a social life, and relationships with a spouse and children. The Social Security Administration has recently added fatigue to their list of symptoms for multiple sclerosis.
Fatigue can be one of the most frustrating symptoms for the MS patient because it may be "invisible" to others. People may remark to a person with the fatigue of MS "Oh, you look so good, I would not even know you have MS." Of course, this remark is to encourage, but can instead be frustrating. The other person cannot experience what the individual with the illness experiences.
This fatigue is believed to be primarily cause by the poor conduction of nerve signals from the brain to parts of the body. However, it may also be caused by the inflammatory process that occurs as the central nervous system is attacked by the person's immune cells. It is well know that people suffering from more common ailments, such as a cold or the flu, experience fatigue because the body responds to an immune attack with a feeling of exhaustion. This might be the best way to explain to family and friends why you "look so good" but feel "rather lousy."
- unsteady gait
- muscle rigidity
- muscle spasms
- loss of sense of position
- girdle pain
Fatigue has been reported to be a crucial element in 78% of individuals struggling with MS (Figure 2). Within the past three years, a new medication called "Provigil" has been developed which seems to help some individuals with this fatigue. In other cases, Ritalin has been prescribed in order to increase the person's energy output.
Many people with MS unknowingly increase the amount of caffeinated beverages they drink in order to "get through the day."
In addition to the symptoms listed above, there is an entire array of sensory symptoms that may accompany MS. In the early stages of the disease, many people only have sensory symptoms that are difficult to describe to a physician. Those physicians unfamiliar with MS may attribute these symptoms to "hypochondria" which frustrates the patient even more. They may begin to wonder "Are these symptoms all in my head?" Physicians who have little expertise in MS may then prescribe an antidepressant or anti-anxiety drug.
This further increases the patient's level of fatigue as they struggle now with the symptoms AND the side effects of these unnecessary drugs. One patient with MS stated:
"The third time I went to my doctor and told him I felt like bees were stinging my leg, he put me on Valium. Then I still felt the pains in my leg and began to walk into walls because the Valium made me so 'loopy'."
This "feeling of bee stings" is yet another symptom of MS, called paresthesia (numbness and cold sensations) and hyperesthesias (a feeling of burning, electric shocks on the extremities). Some people describe a strange vibratory sensation either in the extremities or abdomen. One patient stated "I felt like my cell phone was in my pocket vibrating until I realized I did not have my cell phone..it was the MS."
Neurologists often use pinprick testing to determine how much sensory loss has occurred. This test would be quite painful to a healthy person. For many with MS, there is no feeling as the physician digs a needle into a sensitive area of the leg. Some people report feeling a slight pressure, but not pain. Others, who suffer from hyperesthesia (extreme sensitivity to even a bed sheet being placed over them) report excruciating pain from the pin. Theses sensory symptoms worsen as more demyelination occurs.
Another sensory symptom is the loss of "position sense" in the entire body, especially in the arms and hands. This symptom may interfere with fine motor skills, and is obvious in the handwriting of MS patients with this problem. Those who had beautiful penmanship may gradually drift into handwriting that is sloppy and at times illegible.
The patient's family may notice this symptom first and may consider it to be "clumsiness". A person may go to pick up a glass, and not realize that it is not completely in the hand and drop it on the floor. Patients may begin to trip going up or down steps and even when walking on a flat and even surface, may lose their footing easily.
A very disturbing sensation, described by the talk-show host Montel Williams in his recent books about his struggles with MS, is what has been described as "the MS hug". Using the word "hug" certainly is an understatement of what actually happens.
The "MS hug" symptom is technically referred to as "girdle pain." It actually feels like a boa constrictor has begun to wrap itself around the thorax and abdomen of the person, becoming tighter and tighter. Some feel as though they cannot take a breath because of this tightness. Others describe just trying to find a comfortable position until the sensation temporarily goes away. It can be triggered by drinking hot beverages, exercise, or an increase in environmental temperature.
It is believed this symptom may be caused by the demyelination of the spinal cord but clearly, no one is absolutely sure why this very uncomfortable symptom occurs. It can be so painful that some individuals with MS must take pain medication and antispasmotics.
An old saying concerning multiple sclerosis was that "If there is pain, then it is not MS." Unfortunately, some medical practitioners continue to believe this, even with countless reports of MS related pain.
The assessment and treatment of pain in any disease has become the focus of the Institute of Medicine (IOM) the Joint Commission for the Accreditation of Hospitals (JCAHO), and the National Institutes of Health (NIH)Because it has become so important as a factor in the quality of life in MS patients, the entire segment below is devoted to the treatment of pain in MS.
Pain and Multiple Sclerosis
Pain was thought to be a rare and minor symptom in patients with MS but studies conducted from 2007 to the present show that 86% of patients with MS complain of pain. Patients with multiple sclerosis may experience more than one type of pain syndrome. This pain is classified into four categories: (1) neuropathic pain (2) pain indirectly related to MS (3) MS treatment related pain (4) pain unrelated to MS.
The most common pain of MS is headache (40%), neuropathic limb pain (19%), back pain (17%), and painful spasms (11%). Dissatisfaction with the listening skills and care by the physician in regard to this pain was reported in 68% of the patients.
Chronic pain has a drastic influence on quality of life. It affects recreational activities, exercise, and work. Chronic pain can become the source of clinical depression and a dramatically increases the rate of suicide. The reader may recall the infamous Dr. Kevorkian, and those who went to him for physician-assisted suicide; forty percent were individuals with multiple sclerosis.
One source of pain in MS is neuropathic pain, which is described as any pain that has its source within the nervous system. Heat, pressure, overwork, and lack of sleep may aggravate this symptom. The pain is described as extremely intense and intolerable during the night.
The drug gabapentin (Neurontin) has been used for this pain with a dose range from 100 mg./day up to 3600 mg/day. More recently, a drug from the same class as gabapentin has been developed called pregabalin (Lyrica) using 350-600 mg a day.
These drugs may often be used with an opioid analgesic, although this continues to be a source of controversy. Some physicians are opposed to the use of opioids for any type of chronic pain. Tramadol, morphine, and oxycodone as well as fentanyl transdermal application have been used successfully, often in combination with gabapentin or pregabalin.
In patients who suffer from painful neuropathies, intrathecal baclofen, an antispasmodic agent, is used for treatment. This involves the implantation of a pump or injection into the spinal cord via a lumbar puncture.
Lately, the use of cannabinoids (THC, Marinol) has been tried for neuropathic pain of MS but studies are inconclusive at this point.
Pain associated with muscle spasticity affects 40-70% of all patients with MS. The drugs of choice to control this is baclofen and Valium. However, both are associated with increased weakness in the affected limb. While trying to relax a very tight spastic muscle, the drugs have the negative effect of causing a flaccid muscle that is weaker.
Headaches are seen in 60% of people receiving one of the classic treatments used for MS (Interferon-beta, to be discussed later in this article) and can be controlled with NSAIDS (non-steroidal anti-inflammatory drugs). Migraine headaches were reported to be the first symptom of MS in 16% of patients.
Back pain is severe in 39% of patients with MS because of spasticity that occurs in the legs, and uneven and awkward ambulation. Treatment for this may consist of physical therapy and/or TENS (transdermal electrical nerve stimulation, to be discussed under "Complimentary Therapies")
A very odd type of pain experienced at least once by most MS patients is the Lhermitte's Sign. This can be elicited in a person by having them stand up straight and then lower their chin to the chest. This may elicit a feeling of electric shock that begins in the head and shoots down to the base of the spinal cord. It is described as "very unpleasant" by most MS patients. They try to avoid this motion for fear it will elicit this pain.
Persistent pain is one of the most difficult problems encountered in pain management clinics because of the diverse and diffuse nature of the pain. Some individuals may have spasticity, with a painful neuropathy and migraine headaches. Finding the right combination of drugs and alternative therapies is vital to a good quality of life with minimal depression.
Cognitive Changes in Multiple Sclerosis
A common question many people with MS ask is "Will this disease affect my mind?". Physical symptoms are troubling enough but patients often greatly fear that they will lose memory and the ability to learn, or even worse, that they may become "insane" from the disease.
The discipline of cognitive neuropsychology is just beginning to search for answers to these questions. However, it is known that measurable cognitive deficits are common in patients with multiple sclerosis. In advanced MS, memory can be affected and cognitive abilities, such as word-finding, creative thinking, and sharp mental problem solving may be blunted. It is difficult to directly link structure and function. Even though a person may have a lesion in the area of the brain responsible for short-term memory, they may not be affected. It is believed that widespread lesions across the cerebrum are responsible for cognitive deficits, not just an isolated lesion in a specific area of the brain.
People with MS have a fear that they may develop a psychiatric disorder due to the effects of lesions in the brain. Studies continue to show a high rate of depression among MS patients. But the question that needs to be asked is this: "Is the high rate of depression from a lesion in the brain or is it because the person is coping with a severe and debilitating illness?"
Perhaps it is a combination of the physiological factor of the lesion AND the stress of coping that is responsible for a high depression rate among MS patients. Certainly, if a person with MS was to take a "Stress in My Life Inventory", many factors would emerge that would cause depression in anyone healthy. Such as loss of health, loss of an income, strain on a marital relationship, and uncertainty about the future.
On the other hand and in the extreme opposite direction from depression is the presence of euphoria in MS. Euphoria is a personality change and mood disorder characterized by unusual and seemingly bizarre optimism in the face of very bad circumstances. It is usually seen in MS patients with long-standing disease and severe debilitation. The patient may smile and laugh inappropriately and dismiss any concerns about even life-threatening health problems.
Paroxysmal Symptoms of Multiple Sclerosis
In addition to the classic symptoms of MS, there is a category of symptoms referred to as "paroxysmal symptoms". These may occur during a relapse or even for very brief periods of time while a person is in remission. They may last from several minutes to several weeks and then disappear.
Trigeminal neuralgia is a symptom involving the demyelination of the fifth cranial nerve and/or its nucleus in the pons. Trigeminal neuralgia can cause severe pain on one side of the face so that even a breeze can cause severe pain.
MS patients with trigeminal neuralgia often go to a dentist believing they may have severe dental problems and often find themselves in the emergency room because the pain is so severe. The treatment for this disorder is carbamazepine.
Other paroxysmal symptoms include the occurrence of seizures in 4% of MS patients, uncontrollable itching, and double vision.
Optic Neuritis and Visual Problems
In most parts of the world, demyelinating optic neuritis is the most common cause of painful unilateral visual loss and often one of the first signs of multiple sclerosis. It can become worse over a period of a few days to two weeks. There is periocular eye pain, partial loss of vision in one eye, a recurrence of symptoms with increased body temperature (Uhthoff's phenomenon), and pallor of the optic disc. Approximately 50% of the cases go on to develop other symptoms of multiple sclerosis within 15 years.
Within 2-3 weeks, an 80% recovery of visual loss occurs but with subsequent attacks, the visual loss increases.
The treatment for optic neuritis is often the same as for multiple sclerosis: the use of corticosteroids as anti-inflammatory agents.
The optic nerves and the optic chiasm may be the first to be vulnerable in the onset of MS because they lie so close to the periventricular area of the brain, where most initial lesions occur, where the "blood-brain barrier" is crossed. This fact alone has led to speculation that some sort of latent virus, as it breaks through this barrier, begins the course of MS. This remains a theory, not a fact.
The blurred vision of this disease is often referred to as a "mist over the eyes"
A recent biography of a CNN journalist tells of his struggle with optic neuritis and his subsequent development of multiple sclerosis in the book "Blind-Sighted". In it, he tells of his new outlook on even trying to walk up steps and his near total blindness when he was sent on an assignment into a very hot climate.
G.I. and Urinary Disturbances
Difficulty with bladder control is a frequent symptom of multiple sclerosis and is often associated with leg weakness and the demyelination of the spinal cord. Common symptoms include urgency (60%), frequency (50%), incontinence (10%) and retention (2%). These symptoms can be the most distressing for a patient because of a fear of having "an accident" in a public place.
Patients who have sudden new symptoms of bladder dysfunction should be seen at a clinic for urinalysis and culture to be certain they do not have a urinary tract infection.
People with MS develop a neurogenic bladder. It must be determined whether the problem is failure to empty the bladder adequately or the ability to stop the flow of urine.
In patients with incontinence the drugs oxybutynin (Ditropan) and tolterodine (Detrol) are effective.
Drug treatment for inability to empty the bladder completely are usually ineffective. Trials have been done with Flomax, a drug normally given to men with urinary problems caused by an enlarged prostate.
For urinary retention, the patient needs to be taught self-catheterization, a very frightening procedure for those not familiar with medical technique. A nurse with the ability to keep the patient calm and still able to express fears will be of great help to a patient who may have to self-catheterize for many years. Assurance that many people do this procedure daily, even at work, will give the patient hope that this does not mean they will be "chained to a bathroom."
Lately, problems have arisen because some health insurance providers will not pay for a new catheter for each catheterization. This could cause an increase in urinary tract infections. Just this year, Medicare has begun to pay for a fresh catheter for each use. Unfortunately for those not yet eligible for Medicare. insurers are unwilling to provide for any disposable catheters.
Patients with the bladder problems of MS report that they "know every bathroom along their route to work". Many take a change of clothes because frequent accidents may occur.
Constipation and Bowel Problems
Constipation is defined as the passage of stool less than three times a week. MS patients exhibit a slowed transit time through the colon. In addition to this, they have a poor volumetric squeeze pressure. The pelvic floor may suffer from spasticity. This inhibits the proper emptying of the rectum.
The use of stimulant laxatives may help with this slowed transit time. The addition of more fiber to the diet may also help. The frequent use of enemas may be necessary to prevent impacted stool. Constipation is a problem for 73% of MS patients.
Because multiple sclerosis can affect not only the spinal nerves but also the autonomic nervous system, erectile dysfunction may be a symptom encountered by the male patient. Lack of sensitivity or paresthesia throughout the female genitalia may cause inability to produce or sustain arousal. Sexual dysfunction in women is less documented. When questioned, women with multiple sclerosis frequently tell the physician of a loss of libido associated with this loss of sensation in the genitalia as well as a loss of vaginal moisture. Many patients are hesitant to mention sexual symptoms but they are troubled by them because it has an impact on intimacy with their partner and can be the source of relational discord. There are no medications that assist in the return of sensation in the sexual organs.
Diagnosis of Multiple Sclerosis
Many advances have occurred in medical technology in the past ten years most notably the increased resolution of magnetic resonance imaging (MRI). The diagnosis of MS is based on the presence of central nervous system lesions that are disseminated in time and space. This means that these lesions occur in different parts of the brain or spinal cord at least three months apart.
NO single test is reliable in diagnosing multiple sclerosis and a variety of conditions can mimic the disease, including Lyme disease, lupus, a tumor, and Vitamin B12 deficiency.
Findings on the MRI scan on two different occasions may give a tentative diagnosis of MS. MS lesions appear as areas of high signal intensity on T-2 weighted images. The most common area of first lesions is the periventricular area. These lesions are often enhanced by an injection of gadolinium prior to the scanning.
Sensory evoked potentials may be used for diagnosis, especially the visually evoked potential which may demonstrate evidence of an optic nerve lesion that may not be evident on the MRI study. For this testing, the patient is seen by an ophthalmologist. They are placed in a darkened room and asked to look onto a screen where various bursts of light occur. The person presses a button if they are able to see the burst of light. The pattern of the visual field is then assessed by the physician.
Auditory evoked potentials are conducted by a specialist in hearing. These may demonstrate damage to the auditory-vestibular nerve as manifested by tinnitus, hearing loss, or problems with vertigo and balance.
Cerebrospinal fluid analysis in 90% of MS patients shows an increased IgG concentration and oligoclonal bands. However, it is not always diagnostic for MS and is used to rule out infection or a neoplasm.
Even with modern technology, the most important tools for the diagnosis of multiple sclerosis are physical examination and the patient interview. The diagnosis of MS relies heavily on clinical findings over a specific period of time.
The diagnosis of "clinically definitive multiple sclerosis" is made in patients who have their first neurological symptoms as an episode between the ages of 20 and 50 years of age and the symptoms are referable to the white matter of the central nervous system. These symptoms are then correlated with objective neurological signs and an exacerbation of the disease that occurs at a later time.
For this reason, it is important that the patient be able to maintain an excellent relationship with the physician, being able to ask any question that is bothersome. Many neurologists are accustomed to dealing with abnormalities such as stroke and diseases of the elderly. It is imperative to find a neurologist who frequently sees MS patients in his practice.
Treatment and Pharmacology
In the past 15 years, the treatment of MS has developed at lightning speed with the use of "the ABC drugs" (Avonex, Betaseron, Copaxone) used for relapsing-remitting forms of MS.
However, clinically significant acute relapses are treated with high dose intravenous corticosteroids (methylprednisolone 1 g/day for 3-5 days) which has been the standard of treatment for at least the past 20 years. This will shorten the relapse duration but does not improve the degree of recovery or the long-term course of the disease.
The A-B-C drugs do alter the course of the disease. It has been shown that the lower the relapse rate, the less lesions that will develop over the course of a lifetime and thus, the less disability a person will experience.
The first approved disease-altering drug was interferon-beta called "Betaseron" which was approved in 1993 by the FDA. Betaseron is usually given by a subcutaneous injection every other day. It works by altering and suppressing immune activity in the body. Another interferon medication for MS is Avonex which is given by a 30 mcg intramuscular injection. The third drug used is Copaxone (glatiramer acetate) which has a slightly different mode of action. It is a synthetic protein injection composed of four of the amino acids found in myelin. Its action is based on the similarity of the drug to the actual myelin. Some people using Copaxone experience chest tightness and severe anxiety attacks following injection.
Clinical data has shown that all of the A-B-C drugs reduce relapse rate by 30%. Tysabri (natalizumab) emerged on the scene in 2006. It is from a new class of monoclonal antibodies and is used for people with more serious cases of relapsing-remitting MS. A two year clinical trial called the AFFIRM study involved 942 patients. Those treated with the drug showed a 42% reduced risk of the progression of a disability. Unfortunately, safety issues surfaced about the drug when some patients began to develop progressive leukoencephalopathy, a very destructive disease of the brain.
These therapies are all for individuals with RRMS (relapsing remitting MS) and have been shown to be ineffective in progressive disease. It is believed that neuroprotective and neurorestorative therapies will be required for those who have chronic progressive MS. Lipoic acid, a fatty acid present in certain foods and available as a supplement may protect the myelin from further destruction.
Newer drugs are being tested in clinical trials but most of them cause a decrease in the immune response, opening the door for infection and the potential for the future development of cancer.
The effectiveness of a drug is difficult to ascertain because most people with MS have relapses and remissions that occur spontaneously. It takes a difficult statistical analysis to ascertain if the remission occurred from the drug or the spontaneous nature of the remission. None of these A-B-C drugs work for the chronic progressive form of MS.
Side effects of the interferon-based drugs include flu-like symptoms, headaches and gastrointestinal symptoms such as diarrhea and vomiting.
In an experimental trial, T-cell vaccination was attempted. The myelin-attacking T-cells were isolated from the blood, irradiated and then injected as a vaccine into that person which stimulated the immune system to recognize and eliminate them. This clinical trial was performed in Belgium but only on a small scale.
As mentioned at the beginning of this section, corticosteroids remain the mainstay of treatment for a relapse of MS. For 50 years they have been used as an immunosuppressant and anti-inflammatory. ACTH is normally secreted by the anterior pituitary gland in the brain and then stimulates the adrenal cortex (the small organ that sits like a cap directly over the kidney) to secrete cortisol. This continues to cause a rapid improvement in many patients with chronic symptoms. Possible effects include fluid retention, the loss of bone mass, and at very high doses, the may initiation of a condition know as "steroid psychosis" in which a person may have hallucinations. With the withdraw of this drug, these mental symptoms almost always disappear.
Complementary and Alternative Therapies
Complementary and alternative medicine has been a controversial field in the area of neurology. Much progress has been made in other medical disciplines. On the National Cancer Institute website, there is a large section on alternative therapies. Grant money has become available to explore such things as Eastern medicine, herbs, and anything non-traditional.
Research in these areas has been very slow in coming because it has been difficult to obtain a sample size large enough to demonstrate a consistent pattern.
Complementary and alternative medicine is defined as interventions that are not taught in medical schools and not available in hospitals in the United States. In 1998, the US Congress established The National Center for Complementary and Alternative Medicine.
These therapies include mind-body therapies (prayer, yoga, meditation), biological-based therapies (herbs, supplements), manipulative therapies (massage, chiropractic), energy therapies (therapeutic touch), and alternative medicine systems (naturopathy, traditional Chinese medicine).
The use of Complementary and Alternative therapies (CAM) is now sought after by 43% of the American public. Although most are not covered by traditional health insurance policies and people are willing to pay out-of-pocket for them.
Up to 81% of multiple sclerosis patients use CAM therapies, a percentage that far-exceeds that for any other disease. This seems to be attributed to the fact that the patient has had an unfavorable treatment from orthodox medicine.
Another reason for this very high number is that people with MS are pragmatic and are willing to try whatever works for them. They also enjoy being a part of their own health care decisions. Many MS patients are young and intelligent. They report feeling valued by the so-called unconventional practitioners who are willing to work with them so that they can maintain optimum health even within the disease process.
In modern Western medicine, the relationship between stress, the life of the spirit, and diet and exercise has been poorly understood. In general, people with multiple sclerosis feel they have just been given "another medication" each time they visit the physician's office.
Some of the modalities of CAM that have been helpful to people with multiple sclerosis include chiropractic care (26% of patients) Careful manipulation of the spine and work with the muscles involved in spasticity has been of great help to those suffering from chronic pain. Massage is also used by 24% of MS patients.
Acupuncture (19% of patients) has been used for the relief of pain and spasticity. This involves the placement of very thin, long sterile needles into various dermatomes of the body. It is a science that has been used in the Far East for thousands of years and practitioners often study in excess of seven years to become proficient in its use.
Many individuals have consulted with homeopaths or naturopaths (27% of patients) in order to understand the delicate balance needed to maintain health. They have used herbs and vitamins as well as natural, unprocessed foods and found these very beneficial to their health.
Nutritional Approaches to MS
Proper nutrition has been studied in maintaining the well-being of MS patients. The elimination of food allergens has been proposed in order to halt the over-active immune system found in MS patients. One popular diet is the non-dairy products, low fat diet. The diet suggests that daily fat intake should be kept under 15 grams a day which necessitates the avoidance of red meat and dairy products. Omega-3 fatty acids are important for regulating the immune response and inflammation and eating fish and using fish oil capsules is advocated.
In addition to adequate vitamin D intake and maintaining a proper balance between omega-3 and omega-6 fatty acids, a number of other nutrients have been studied in the context of MS. These include:
- Coenzyme Q10 (CoQ10) is another antioxidant of potential usefulness in treating MS and, while there is some debate, its levels may be low in patients with MS. Although CoQ10 has not been investigated specifically for the treatment of MS, it is generally recognized as safe, well tolerated, and potentially useful in the treatment of neurodegenerative disorders. CoQ10 is a naturally occurring, lipid-soluble antioxidant that serves as a co-factor in the mitochondrial respiratory chain. Mitochondria are subcellular organelles present in all cells. They are responsible for the production of cellular energy. As an added bonus, CoQ10 is capable of regenerating the antioxidant capacity of spent vitamin E in the body. Decreased levels of CoQ10 are associated with many disease states, including heart disease, cancer, and neurodegenerative diseases.
- Vitamin B12. Vitamin B12 plays a key role in the generation of myelin. Studies have shown that patients with MS often have abnormally low levels of vitamin B12 in their cerebrospinal fluid and/or blood serum. In fact, clinical vitamin B12 deficiency and MS share remarkably similar characteristics, occasionally rendering correct diagnosis difficult. For more than three decades, many physicians have prescribed vitamin B12 injections for patients who have MS. Patients who have received vitamin B12 supplements have reportedly experienced consistent clinical improvements in their symptoms.
Vitamin D Deficiency: An MS Risk Factor
Recent research has suggested a high correlation between MS and Vitamin D deficiency. Vitamin D is emerging as a far more important immune system component than was previously appreciated. Long known to play a key role in the regulation of calcium and in the formation and maintenance of healthy bones, vitamin D is now known to influence cell differentiation, function, and survival. In fact, the most bioactive form of vitamin D acts as a hormone in the body, and receptors for it have been discovered in a wide range of tissues.
Vitamin D may also be involved in preventing MS. This was originally inferred from epidemiological data. Scientists noted that MS is more prevalent in people living at higher latitudes (in either the Northern or Southern hemispheres) where sunlight is weaker, particularly in winter. The most bioactive form of vitamin D is generated in the body through a biosynthetic process that begins with, and is dependent on, exposure of the bare skin to sunlight.
In 2004, scientists from the Harvard School of Public Health published the results of two long-term studies on women's health and nutrition. Researchers looked at dietary and supplemental intake of vitamin D as it related to the incidence of MS. Gleaned from the Nurses' Health Study (more than 92,000 women followed from 1980 to 2000) and the Nurses' Health Study II (more than 95,000 women followed from 1991 to 2001), the data support a protective effect for vitamin D against MS, especially for women who consume more than 400 international units (IU) daily of vitamin D from supplements, but not from food sources.
Scientists now believe that vitamin D (commonly depleted in people who have MS) may play a crucial role in preventing the disease. Low vitamin D levels are also an emerging risk factor for other diseases and disorders such as type 1 diabetes, heart disease, and rheumatoid arthritis.
The optimal level of vitamin D varies, but many experts agree that supplemental vitamin D is required, even up to 1000 IU daily. By contrast, a whole-body exposure to peak summer sun will rapidly cause the release of up to 20,000 IU into the circulation. Other experts suggest that anyone with a blood level of less than 80 nanomoles per liter (nmol/L) of circulating 25-hydroxyvitamin D is at risk of a vitamin D deficiency.
Supplements that have been studied in animals and people with MS include:
- Vitamin D 1000 international units (IU) daily
- EPA/DHA3000 to 4000 milligrams (mg) daily of fish oil concentrate
- GLA 1000 to 3000 mg daily of high GLA oil
- DHEA 15 to 75 mg daily (Have blood tested in 3 to 6 weeks to maintain optimal levels.)
- NAC 600 mg daily with 1800 mg of vitamin C
- Vitamin E 400 IU daily
- CoQ10 100 to 300 mg daily
- Lipoic acid (preferably R-dihydro lipoic acid)300 mg daily
- Vitamin B12 5 to 40 mg daily in the form of sublingual methylcobalamin tablets
Hormonal therapy with bioidentical hormones may also be considered, especially in women. Numerous studies have shown that hormone levels that approximate late pregnancy can reduce the severity of MS, although there is controversy surrounding this idea, and studies have shown a rebound effect in MS symptoms after pregnancy. Before bioidentical hormonal therapy is initiated, a complete Female Hormone Panel is recommended.
Supplement Safety Caveats
An aggressive program of dietary supplementation should not be launched without the supervision of a qualified physician. Several of the nutrients suggested in this protocol may have adverse effects. These include:
- See your doctor and monitor your blood glucose level frequently if you take CoQ10 and have diabetes. Several clinical reports suggest that taking CoQ10 may improve glycemic control and the function of beta cells in people who have type 2 diabetes.
- Statin drugs (such as lovastatin, simvastatin, and pravastatin) are known to decrease CoQ10 levels.
- Do not take DHEA if you could be pregnant, are breastfeeding, or could have prostate, breast, uterine, or ovarian cancer.
- DHEA can cause androgenic effects in woman such as acne, deepening of the voice, facial hair growth and hair loss.
- Consult your doctor before taking EPA/DHA if you take warfarin (Coumadin). Taking EPA/DHA with warfarin may increase the risk of bleeding.
- Discontinue using EPA/DHA 2 weeks before any surgical procedure.
- Consult your doctor before taking GLA if you take warfarin (Coumadin). Taking GLA with warfarin may increase the risk of bleeding.
- Discontinue using GLA 2 weeks before any surgical procedure.
- GLA can cause gastrointestinal symptoms such as nausea and diarrhea.
Consult your doctor before taking lipoic acid if you have diabetes and glucose intolerance. Monitor your blood glucose level frequently. Lipoic acid may lower blood glucose levels.
- NAC clearance is reduced in people who have chronic liver disease.
- Do not take NAC if you have a history of kidney stones (particularly cystine stones).
- NAC can produce a false-positive result in the nitroprusside test for ketone bodies used to detect diabetes.
- Consult your doctor before taking NAC if you have a history of peptic ulcer disease. Mucolytic agents may disrupt the gastric mucosal barrier.
- NAC can cause headache (especially when used along with nitrates) and gastrointestinal symptoms such as nausea and diarrhea.
Vitamin B12 (methylcobalamin)
- Do not take cyanocobalamin if you have Leber's optic atrophy
- Do not take vitamin D if you have hypercalcemia.
- Consult your doctor before taking vitamin D if you are taking digoxin or any cardiac glycoside.
- Only take large doses of vitamin D (2000 international units or 50 micrograms or more daily) if prescribed by your doctor.
- See your doctor frequently if you take vitamin D and thiazides or if you take large doses of vitamin D. You may develop hypercalcemia.
- Chronic large doses (95 micrograms or 3800 international units or more daily) of vitamin D can cause hypercalcemia.
- Consult your doctor before taking vitamin E if you take warfarin (Coumadin).
- Consult your doctor before taking high doses of vitamin E if you have a vitamin K deficiency or a history of liver failure.
- Consult your doctor before taking vitamin E if you have a history of any bleeding disorder such as peptic ulcers, hemorrhagic stroke, or hemophilia.
- Discontinue using vitamin E 1 month before any surgical procedure.
In addition to these therapies, studies find that people who rely on their faith to get through difficult times and who use prayer and meditation for anxiety or depression fare better than those who have no hope in the afterlife and have not formulated a real meaning in their lives.
Psychosocial Factors in Multiple Sclerosis Patients
As with any chronic and debilitating disease, people with multiple sclerosis and their families must cope with a variety of key decisions regarding their present lifestyle and their very uncertain future. Multiple sclerosis is primarily a disease that begins in young adulthood and approximately 12 years after diagnosis, many people have at least one disability. The financial and social implications are tremendous. There is a high unemployment rate among MS patients who very sadly give up fulltime careers for less stressful part time work. They may even resort to Social Security Disability, a lengthy and tedious process with frequent long waits and rejections. There is a higher divorce rate among people with a spouse who has MS.
Women who develop multiple sclerosis in their 20's or 30's wonder if they should have children, even though there is not a direct correlation between pregnancy and relapse. Career questions evolve around the amount of sick time a person is able to take and how their illness will impede the advancement of their careers.
Despite the passage of The American's With Disabilities Act", many people fear coming to a job interview with an assistive device or even mentioning their disease. Employers become anxious, wondering if they will be hiring an "impaired" individual.
Support groups are available for people with MS and their families through the National Multiple Sclerosis Foundation. Even with this help, families need to be ever-aware of the changes in mobility, independence, and health that occur in their loved-one with MS. Even if that person "looks great", that is not an indication of how they feel. Many people with MS are high-achievers and overcompensate for their disease by doing much more than a "normal" person might do.
Fatigue can be overwhelming. Numbness and motor weakness can cause fear and frustration and the mood swings caused by some of the MS drugs can turn a person's world upside-down. Families need to talk realistically about the future in regard to disability, retirement, and even vacations to tropical spas where the heat may totally disable the person with MS. If ambulation becomes a problem, the "old homestead" may no longer be the house of choice and plans for a handicapped-accessible apartment may be necessary. Making plans for a son's wedding or a child's birthday may look good at the time, but just as the occasion approaches, a relapse may occur.
Too often, physicians do not talk about the psychological consequences of MS because they may feel they are under a time constraint or they do not want to "upset" the patient. Actually, patients are relieved when they realize they can speak with their physician about their frustration with the disease.
Adrenoleukodystrophy (ALD) was mentioned previously in regard to The Myelin Foundation. It is a demyelinating disease with onset in childhood and symptoms beginning to show from the ages of 4-8 years of age. It is a sex-linked genetic disease and so is only found in males, yet the trait is carried only on the X-chromosome.
This is a very rare disease that came to light with the movie "Lorenzo's Oil." Lorenzo had this disease and went from being a very bright and active child to a paralytic within 18 months. The parents were told even though many young boys had this disease throughout the world, it was considered to be an "orphan disease" and no drug companies would invest in research and development for a disease that had only a few hundred thousand victims.
Through endless library research (even before the days of the Internet) and vital communication with primary research chemists, this family was able to develop "Lorenzo's Oil" (erucic acid), which blocked the formation of the very long chain fatty acids at C24 and C26 (carbon chains) that cause the disease.
ALD begins with sudden behavior changes such as hyperactivity, difficulty in concentration, and changes in personality, such as inappropriate screaming and crying. These symptoms are followed by neurological deficits, such as problems with gait and a gross tremor. The disease progresses to cortical blindness, deafness, and impairment of all higher intellectual functions.
These symptoms are caused by the rapid and massive demyelination of he brain. The adrenal glands are also destroyed by these abnormal lipid deposits and Addison's Disease develops (complete lack of the production of the adrenal hormone cortisol).
Since 1989, the addition of erucic acid to the diet of affected children has helped them to be normal. The discovery was too late for Lorenzo. He had already suffered irreversible brain damage.
Now, in 2010, a team at the University of Paris has found a way to remove the causative gene from the X-chromosome and replace it with a normal gene. The Myelin Foundation gives grants to scientists who wish to work on the cutting edge in finding the cure for demyelinating diseases.
The Myelin Project
In 1989, Augusto and Michaela Odone created the Myelin Project when their son, Lorenzo, was diagnosed with adrenoleukodystrophy and they were told there was nothing that could be done for him. They were told to take him home (at the age of 7) and just wait for him to die. They were unwilling to accept that prognosis and created The Myelin Project.
The project began as an attempt to get scientists to work together to share, collaborate and critique their research on demyelinating diseases. They also wanted to unite these scientists with the families that suffered from these diseases. The goal was not "science for science's sake" but science to preserve human life.
In 1992, the movie "Lorenzo's Oil" was produced with major Hollywood actors and was seen by millions across the United States. This brought an awareness of demyelinating diseases to common people. In 2009, their website www.myelin.org had over 176,000 visitors from 185 countries.
They use a very different approach to research in actually trying to regenerate myelin, omega 3 and omega 6 fatty acids and the progression of multiple sclerosis. Efforts to transplant glial cells and the use of umbilical cord or stem cells of that person to regenerate myelin are now being done through this foundation.
Demyelinating Diseases of the Peripheral Nervous System
The peripheral nervous system includes all nervous structures lying outside of the pial membrane of the spinal cord and brain. These nerves emerge from the spinal cord at various levels of the spinal nerve roots. It is possible that the myelin of these structures can degenerate in a disease process. These nerves, unlike those in the central nervous system, are capable of regeneration.
The most predominant disease in this category is acute idiopathic polyneuritis, also know as Guillain-Barre Syndrome. It is found throughout the world and affects children and adults. In 70% of the cases, the onset of the disease is preceded by a viral infection that occurred 1-3 weeks prior to the onset of the disease. A major outbreak of the Guillain-Barre Syndrome occurred in 1976 after the administration of the Swine Flu vaccine.
The first major clinical symptom is weakness that evolves over a period of several hours to several days and includes all extremities and frequently the muscles used in respiratory function. This weakness can progress to total motor paralysis and death from respiratory failure.
Weakness is followed by a loss of the deep tendon reflexes. The cerebrospinal fluid is normal for the first week but then shows high protein levels for the next six weeks, indicative of the breakdown of myelin within the peripheral nervous system.
Most research shows that Guillain-Barre is a result of the cell-mediated immunological reaction directed against the peripheral nervous system and specifically, the myelin. It seems that having had a viral infection in the weeks preceding the onset of the disease somehow activates the immune system to actually attack "self-tissue", much like what happens in multiple sclerosis.
Guillain-Barre Syndrome goes into remission naturally and results in complete recovery in the majority of cases. The dangerous period is in the first few days, when the respiratory system can actually shut down from the loss of movement of the diaphragm. Within hours, a person can deteriorate with no compensation for pulse oxygen levels that dive into the 70% range or lower. At the first sign of dyspnea, intubation and ventilation should begin.
About 3% of patients do not survive the illness and death may occur without a rational explanation. In 10% of the patients, residual disability can be severe, requiring ongoing mechanical ventilation. Recovery can occur in a few weeks although some people take 18 months to achieve recovery.
A controlled study using plasmapheresis demonstrated a much better prognosis for those suffering from Guillain-Barre Syndrome. Strangely, corticosteroids seem to be ineffective in the treatment of this disease.
A classic account of a woman who went through the entire course of this disease and her very bad experience with uncaring medical professionals is recorded in the book "Bed Number Ten" (which is required reading for all my nursing students).
Amyotrophic Lateral Sclerosis (ALS) aka Lou Gehrig's Disease
Although not technically a "demyelinating disease", it is covered within the scope of this review paper because of similarities it has with the demyelinating process.
This disease takes its non-medical name from the Yankee first baseman who acquired it in 1939 and died in 1941. Many people are familiar with it for that reason.
ALS is a motor-neuron disease of unknown etiology that causes a progressive degeneration of the corticospinal tracts and cells in the anterior horn of the spinal cord. The average age of onset is 55 years of age. It is more common in males. The patient may begin to have slight neurological impairments, such as speech that sounds slurred. Later, he will experience problems with ambulation and then movement of the upper body.
Patients usually die within 3 years of onset from respiratory failure. In most, all voluntary movement stops and the patient is totally dependent on family or other caregivers for survival.
Most ALS patients do not lose their ability to think and at times, can only blink an eye in order to get caregivers to understand what they need.
In many cases, those with ALS have decided they do not want to be on a ventilator. They opt for conscious sedation around the time of death so that there efforts to breathe are not a torture. They gradually die because they can no longer breathe without assistance.
Less is know about ALS than MS. Researchers have made every effort to discover the cause of the disease with the hope that this will help aid in finding a treatment that works.
There are many neurological diseases not covered within the scope of this course. Other diseases involve specific parts of the brain and neurotransmitters (such as in Parkinson's Disease) or they have no known direct cause. They may cause brain atrophy (Alzheimer's Dementia) rather than demyelination.
Demyelinating diseases require the professional to have excellent assessment skills in all areas of pathophysiology. In particular, the professional caring for a patient with MS needs to understand why bodily functions are affected because of structural changes in the brain and spinal cord. She needs to understand that all body systems are affected by the disease and as a patient advocate, she must be able to help the patient to get the proper information needed to live a good quality of life. She must be prepared to listen to the patient and the family. She must recognize that caregiving for an MS patient can be a 24-hour a day obligation in the end-stages of the disease.
Although progress has been made in the development of pharmaceutical agents for use in these diseases, much still needs to be done. If you think about it, we are not even sure of the cause of MS. The role of the health care professional is of vital importance in assisting the patient to live a high quality of life. Recent advances in the understanding and treatment of MS have improved the prognosis and quality of life of MS patients. People with MS have a substantial ability to affect the course of their illness. Adhering to a proper diet; avoiding toxins (such as solvents or heavy metals) and known triggers; getting mild, regular exercise; taking recommended supplements; and working with qualified physicians, are proactive choices which positively affect the course of this frustrating disease.
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