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Georgia State Chapter Newsletter Fall 2005 |
Myasthenia Gravis: An Overview
Myasthenia Gravis and Speech/Voice Disorders
Everyone is invited to the first
Neurology EXPO sponsored by the American Academy of Neurology and its
Foundation on Saturday, October 22, from 10:00 a.m. to 4:00 p.m. Held at the
Georgia World Congress Center in hall A2, Neurology EXPO is aimed at providing
information to people with neurological diseases.
There will be something for
everyone at Neurology EXPO. A variety of healthcare providers will exhibit
their products and services. Patient advocacy groups, including the Georgia
State Chapter, MGFA, will provide information about their programs and
activities. The "Youth World" will offer interactive educational
programs where children from kindergarten to high school can learn about the
brain and the nervous system.
Neurologists will present
educational programs of interest on three stages, including the following
topics:
* Multiple Sclerosis
* Epilepsy and Women
* Stroke Prevention and Treatment
* Dementia and Alzheimer's Disease
* Parkinson's Disease
* Complementary and Alternative
Medicine in Neurology
* Caregivers
* New Medicare Legislation
Admission is free for children 18
and under. The cost for adults is $6. Discounted parking coupons and FREE
admission coupons are located on page 7 of this newsletter (make copies if you
like).
The Georgia World Congress Center is located at 285 Andrew Young International Blvd. NE in Atlanta. For directions and more information about Neurology EXPO, visit www.thinkneurologynow.org.
Established in 1948, the American
Academy of Neurology is an association of nearly 19,000
MYASTHENIA GRAVIS: AN OVERVIEW
Carol Ann Smith, RN
Definition:
Myasthenia Gravis is a neuromuscular
autoimmune disease.
It manifests itself by weakness of
muscles under voluntary control and fatigue.
Incidence:
1 in 40,000
Women to Men 2:1 All races
Presenting Symptoms:
Onset for women usually under 40;
Onset for men usually over 50
41% diplopia (double vision)
24% ptosis (drooping eyelid)
16% difficulty with voice
13% weak legs
11% difficulty swallowing
10% difficulty chewing
7% weak arms and hands
The Normal Neuromuscular
Junction
Nerve cell sends message -
acetylcholine (neurotransmitter) is released at the end of the nerve cell -
acetylcholine carries message over synapse - message arrives at muscle and
finds a receptor to latch on to - muscle receives message and muscle functions.
An acetycholinesterase enzyme "eats up" acetylcholine.
What's Your Thymus Gland For?
Serves an extremely important role
when you are a newborn in the development of your immune system. Manufactures
T-cells and B-cells, which are basis of our immune response. Serves a role
through childhood but diminishes with puberty and not really needed after that.
What's Different in MG?
Basically, your immune response is
different from people who do not have MG. You have an "autoimmune
disease" and in a way are allergic to yourself. Your immune system
recognizes a part of you as foreign and attacks it. In MG, it is the muscle
receptor site that is attacked.
In people with MG, a certain
portion of the thymus gland becomes "hyperplasic" or the tissue in it
becomes "abnormal". Some people get a tumor (about 15%) but most do
not. This hyperplasic tissue causes the immune system's T-cells and B-cells to
function in an unusual way, which causes an autoimmune response to occur. The
cells in the hyperplasic tissue manufacture the anti-AchR antibody and can also
teach other cells in the body to secrete it. This antibody is contained in the
IgG portion of our blood plasma and so circulates through the entire body.
The receptor sites on the surface
of the muscles are the targets of this antibody. The antibody attacks the
muscle receptor site and renders them totally or partially inactive and unable
to receive messages properly. It also changes the structure of the
neuromuscular junction by widening the synapse and flattening the muscle
receptor site membrane. If the muscle doesn't receive the message, it won't do
what it is supposed to do, such as keep your eyelid open, focus your eyes,
swallow, walk, etc.
Treatment of MG
Symptom Treatment -
acetylcholinesterase medications such as Mestinon
Immune System Suppression -
steroids such as Prednisone or cytoxic drugs such as Imuran
Decrease Antibody Titer -
thymectomy, plasmapheresis, IVIG
What Can I Do To Help Manage Mv
MG?
AMPS: Minnesota State
Chapter/Nutmeg
One of the first medications
prescribed for many myasthenics is prednisone, a drug used to suppress the
immune system and help to bring the symptoms of myasthenia gravis under control
in a timely manner.
Basically, prednisone is a
man-made version of a steroid normally produced in the human body. Steroids are
a group of hormones produced in the adrenal glands, which are located over the
kidneys. Naturally occurring steroids, as a group, help control metabolism,
inflammation, immune function, salt and water balance, and the ability to
withstand the stress of illness or injury.
One of the steroids produced by
the outer portion of the adrenal glands is cortisone which normally helps
regulate the body's salt and water balance and reduces inflammation. Prednisone
was developed in 1955, as a pharmaceutical replacement for cortisone. The
adrenal glands normally produce an amount of steroids equivalent to about 5 to
7. 5 mg. of prednisone a day. When prescribed in doses exceeding the natural
levels, prednisone suppresses inflammation and immune responses. This effect is
beneficial when treating autoimmune diseases like myasthenia. However, whenever
the immune system is weakened, the body's susceptibility to infection may be
increased. Prednisone does not
make an individual more likely to get a flu or catch a cold, but it may
increase susceptibility to herpes viruses and some other illnesses. Prednisone
is not the same as the dangerous anabolic steroids used by athletes to increase
muscle mass. It is not a sex hormone and is not addictive.
Myasthenics, unless instructed
otherwise, generally take all the prescribed dosage of prednisone once in the
morning. This mimics the natural release of cortisone in the body. Every effort
is made to minimize long term damage resulting from adrenal gland suppression.
To help in those patients who can tolerate it, prednisone can be given on
alternate days. This allows the body a brief reprieve from the drug.
Prednisone is best taken with
food, since it can irritate the stomach lining. It should be taken as
prescribed and the dose should not be altered without a physician's approval.
The goal is to control symptoms with the lowest effective dose possible, taken
for the shortest period of time. Prednisone should not be abruptly stopped. Over
time the adrenal glands may lose the ability to produce steroids naturally and
may shrink in size. Abrupt withdrawal after even one month may lead to severe
medical complications.
Prednisone is one of the
medications most effective in treating myasthenia. However, there can be many
potential problems. These negative side effects are usually dose-dependent.
This means the more prednisone taken over a longer period of time, the greater
the risk of side effects. Therefore, the goal is to control symptoms, and
gradually reduce, or "taper" the dose. Reducing the dose may result
in symptoms such as muscle soreness, joint pain, fatigue and depression. If any
unusual symptoms are experienced or if myasthenia symptoms worsen, as the
prednisone dose is reduced, the physician should be contacted. It may be
necessary to temporarily adjust the dose. For many myasthenics, prednisone is
used as a first line of immune suppression. It acts quickly for many to bring
symptoms under control. Another drug may be introduced to continue long term
control of immune responses. Among these drugs are Imuran and CellCept, which
are slower to take effect. Hence, prednisone is given early and tapered as the
new drug takes effect.
Prednisone has a number of side
effects which may require specific management. Taking the medication exactly as
prescribed may help control the negative aspects. Problems experienced should
be reported to the physician. Side effects vary from person to person, but
should be recognized and may be managed independently of the myasthenia.
Conditions that the myasthenic should note and the doctor will watch for and
help manage are:
Weight gain
Rise in blood sugar
Osteoporosis
Avascular necrosis (uncommon)
(bone death, especially in the hip joint)
Skin problems: acne, slow wound
healing, bruising, etc.
Digestive upset
Mood swings: euphoria, insomnia,
anxiety
Eye changes: cataracts, glaucoma
Myopathy
The decision to use prednisone to
treat myasthenia gravis should be one that is carefully considered and
discussed with the neurologist. Side effects may be reduced through careful
monitoring of the dosage, using the smallest effective dose and, in some cases,
using alternate day therapy, with no prednisone or a smaller dose on alternate
days.
AMPS: Upstate NY Jan. 2005
MYASTHENIA
GRAVIS AND SPEECH/VOICE DISORDERS
By: Frank B. Wilson, Ph.D. of the
American Speech Language Hearing Association
And Professor in the Department of
Speech Pathology and Audiology at the University of
Alberta, Edmonton, Canada
People in the early stages of
Myasthenia Gravis often consult a Speech-Language Pathologist
because they are concerned about a
change in their voice quality. Common, early voice symptoms of Myasthenia
Gravis are an increased nasal sound to the voice. This occurs because the onset
of MG often affects muscles of the neck and facial region. Resonance disorders
associated with MG develop during or after puberty begins; only rarely does MG
occur earlier.
As an example of nasality, the
pronunciation of the word "baby" may sound like "may be".
Besides excessive nasality, people
in the early stages of MG often have difficulty in chewing and swallowing, less
facial expression than they previously had, drooping of the eyelids, and
occasional episodes of liquid or food coming up through the nose. Patients
become excessively tired and frequently note their voice problems get worse
toward evening.
Because of muscle weakness, some
sounds may be made with less emphasis. The word "sit" may sound like
"it" for example.
When the medication is totally
effective in controlling, at least temporarily, some of these
symptoms a Speech-Language
Pathologist may provide help in several areas.
One area is use of speech
prosthesis. The prosthesis, or appliance, is constructed by a
prosthodontist, in conjunction
with a Speech-Language Pathologist. A correctly fitted appliance can reduce
hypernasality and improve the potential for better articulation.
Another area is swallowing. The person
with MG can have difficulty in swallowing when tired or all the time. A
Speech-Language Pathologist will take a complete history of the swallowing
disorder to deal with effective approaches to deal with it.
A third area is voice production.
Many MG patients have problems with voice production. In the advanced stages of
MG most patients will experience breathlessness, reduced loudness, two-pitched
voice and the intermittent loss of voice. A program of voice therapy
emphasizing good
vocal hygiene is helpful. It is
also helpful for the patient with MG to have training in listening skills to
better maintain adequate articulation.
In more severe cases of MG,
augmentative communication systems or devices ranging from a picture board to a
computer with synthesized speech, may be helpful.
AMPS: MG.Association of B.C.
Many prescription drugs can unmask
or worsen symptoms of MG.
These include:
When taking a new prescription
drug for the first time, it's a good idea to consult your doctor
about its possible effects on MG.
Also, you might want to keep a Medic Alert bracelet or card handy to inform
emergency medical personnel that you have MG and that certain drugs can be
harmful to you.
Overexertion, emotional stress,
infections (from tooth abscesses to the flu), menstruation and
pregnancy can also lead to increased
weakness in MG.
Myasthenic Crisis
Especially in people with bulbar
or respiratory symptoms. MG can sometimes worsen to the
point of myasthenic crisis, an
extreme episode of weakness that culminates in respiratory failure and the need
for mechanical ventilation. In some cases, the respiratory muscles themselves
give out, and in others, weakness in the throat muscles causes the airway to
collapse.
When MG is properly treated,
crisis is very rare. And when crisis does occur, it has a good rate of
recovery, thanks to the wide range of treatments for MG and the quality of
respiratory care at most hospitals.
Sometimes, myasthenic crisis can
occur without warning, but it often has an identifiable trigger, such as fever,
respiratory infection, traumatic injury, stress, or one of the drug types
mentioned.
If you have MG, you should have
these conditions monitored by a physician, and if you experience labored
breathing or unusual weakness, you should seek immediate medical attention.
Taken From the Muscular Dystrophy Association webpage at http://wwww.mdusa.org/publications/fa-mg-qa.html
In Memory of: By:
Tommy Harris Michelle Lewis
Morris
Weinstein Arva Weinstein
In Honor of:
By:
Dianne George
& Chapter Officers Jason
& Betty Hoffman