On October 23rd, 2014, we updated our
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This is not intended to be a substitute for your own notes. These are
the notes I have taken in lecture for my own learning. As such, the
content is NOT an exact copy of the powerpoint presentation or the
entirety of the lecture. On my distracted days, the notes may even be
pretty shoddy. That said, I have tried to provide links to reputable
websites where you can find more information. As always, refer to
your textbooks and your instructors for the final word.
I’ll try to sort things out a little more and organize the
information, but the lecture was so disjointed I had to give
up. Sorry. If you have any specific things you’d like me to
look up for you, let me know.
Thursday, February 08, 2007
Client assessment and care
Menstruation- estrogen increases
Non-compliance to meds
Based on history
Epilepsy patients can sometimes predict seizures
Thursday, February 08, 2007
Examples of neurological disorders that cause mental-emotional changes
Cerebral palsy (similar to CVA)
Depends on where the lesion is
Speech, vocabulary, language
Right vs Left brain
General knowledge, education
Abstract vs concrete reasoning
Judgement, planning, insight
Parietal lobe does judgement
Ability to learn and retain
Thursday, February 08, 2007
Affects all cranial nerves except 1, 2, 8
Seizures and epilepsy
Thursday, February 08, 2007
Cranial nerve quiz
On Old Olympus Towering Top A Famous Vocal German Viewed Some Hops.
(olfactory, optic, oculomotor, trochlear, trigeminal, abducens, facial,
vestibulocochlear, glossopharyngeal, vagus, spinal accessory, hypoglossal.)
Tutorial and video of cranial nerve neuro exam
Jaw movement, chewing, speaking
Motor and cerebellar dysfunction
Thursday, February 08, 2007
Description of different types of tremors
Innervated by C3,4,5
Also affected by Guillain-Barre syndrome
Check how many words they can say at a time
Have them sniff
Thursday, February 08, 2007
Myasthenia gravis is a chronic disorder characterized by weakness and rapid fatigue
of any of the muscles under your voluntary control. The cause of myasthenia gravis
is a breakdown in the normal communication between nerves and muscles.
The disorder affects only the function of your muscles, and the muscle weakness you
experience improves when you rest. Myasthenia gravis may cause double vision,
drooping eyelids, difficulties with speech, chewing, swallowing and breathing, as well
as weakness of your limbs.
Myasthenia gravis can affect people of any age, but it's more common in women
younger than 40 or older than 70, and in men older than 50. There's no cure for
myasthenia gravis, but treatments are available to help control the signs and
symptoms of the condition.
Signs and symptoms
Myasthenia gravis can affect any of the muscles that you control voluntarily. It most
commonly affects certain muscles, including those of your face, eyes, arms and legs,
and those muscles involved in chewing, swallowing and talking. Muscles that control
breathing and the movement of your head, arms and legs also can be involved.
Signs and symptoms may include:
• Facial muscle weakness, including drooping eyelids
• Double vision
• Difficulty in breathing, talking, chewing or swallowing
• Muscle weakness in your arms or legs
• Fatigue brought on by repetitive motions
The more often a muscle action is repeated, the worse the weakness becomes. In
myasthenia gravis, good days may alternate with bad days. Temporary remissions
may occur. In rare cases, breathing or swallowing problems worsen markedly,
requiring emergency medical care.
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Thymus gland and lymphatic system
When your neuromuscular system functions normally, the chemical acetylcholine
transmits nerve impulses to your muscles. At specialized areas of your muscles,
called neuromuscular junctions, receptor sites receive nerve impulses and signal your
muscles to contract, such as when you raise a spoon to your mouth.
In myasthenia gravis, there's a breakdown in communication between your nerves
and your muscles. The culprit is your immune system. For unknown reasons,
myasthenia gravis causes your immune system to produce antibodies that block or
destroy many of the receptor sites for acetylcholine in your muscles. With fewer
receptor sites available, your muscles receive fewer nerve signals, resulting in
It's believed that the thymus gland, a part of your immune system located in the
upper chest beneath the breastbone, may trigger or maintain the production of these
antibodies. Large in infancy, the thymus is small in healthy adults. But, in some
adults with myasthenia gravis, the thymus is abnormally large. Some people also
have tumors of the thymus. Usually, thymus gland tumors are noncancerous
Some factors can make myasthenia gravis worse, including fatigue, illness, stress,
extreme heat, and some medications, such as beta blockers, calcium channel
blockers, quinine and some antibiotics. Check with your doctor before taking any
new medications, even over-the-counter drugs.
When to seek medical advice
If you experience muscle weakness or have difficulty controlling the muscles of your
eyes, face and mouth, have trouble breathing or experience fluctuating weakness in
your arms and legs, see your doctor. These signs and symptoms could be indications
of myasthenia gravis.
Although there's no cure for myasthenia gravis, the outlook for managing its signs
and symptoms is good. The earlier you see your doctor, the sooner treatments can
be initiated to help you improve your muscle strength, and the sooner you can learn
about strategies to help you use your energy in the most efficient ways.
Screening and diagnosis
The key sign that points to the possibility of myasthenia gravis is muscle weakness
that improves with rest. Tests to confirm the diagnosis may include:
• Neurological examination. This may include testing of your reflexes,
muscle strength, muscle tone, senses of touch and sight, gait, posture,
coordination, balance and mental skills.
• Blood analysis. A blood test may reveal the presence of abnormal
antibodies that disrupt the receptor sites where nerve impulses signal
your muscles to move.
• Edrophonium test. Injection of the chemical edrophonium (Tensilon)
may result in a sudden, although temporary, improvement in your
muscle strength, an indication that you may have myasthenia gravis.
Edrophonium acts to block an enzyme that breaks down acetylcholine,
the chemical that transmits signals from your nerve endings to your
muscle receptor sites.
• Nerve conduction studies and single-fiber electromyography.
During the first part of this test, a small electrical impulse is applied to
your skin, stimulating your nerves in order to test the strength of your
muscle contraction. In the second part, a thin-needle electrode
inserted into one of your muscles helps measure patterns of electrical
activity in your muscle at rest and with slight muscle contraction.
Doctors use a variety of treatments, alone or in combination, to relieve symptoms of
• Medications. Drugs called cholinesterase inhibitors, such as
pyridostigmine (Mestinon) and neostigmine (Prostigmin), enhance
communication between nerves and muscles. These drugs don't treat
the underlying problem, but they do improve muscle contraction and
Corticosteroids inhibit the immune system, limiting antibody
production. Prolonged use of corticosteroids, however, can lead to
serious side effects, such as bone thinning, weight gain, diabetes,
increased risk of some infections and a redistribution of body fat.
Your doctor may also prescribe other medications that alter your
immune system, such as azathioprine (Imuran), mycophenolate
mofetil (CellCept), cyclophosphamide (Cytoxan) or cyclosporine
• Surgery. It's generally been believed that removal of the thymus
gland (thymectomy) brings relief to the majority of people with
myasthenia gravis. But only about 25 percent of those who have the
surgery go into remission within a year, and significant improvement is
often delayed for years. The surgery has generally been recommended
for people younger than 60.
Recent analysis has cast some doubt on the benefit of thymectomy in
people who have myasthenia gravis but do not have thymomas
(tumors in the thymus). A large international trial is being conducted
to assess whether thymectomy should be routinely recommended in
• Plasmapheresis (plaz-muh-fuh-RE-sis). This procedure can
remedy life-threatening stages of myasthenia gravis. Plasmapheresis
involves removal of antibodies from your blood that block transmission
of signals from your nerve endings to your muscles' receptor sites.
Blood is taken from your body, passed through a filter that removes
antibodies and then returned to your body.
This approach is expensive and time-consuming. Also, other forms of
therapy are necessary for long-term restoration of muscle strength.
Otherwise, the immune system soon makes new antibodies to replace
those that have been removed.
• Intravenous immune globulin. This therapy provides your body
with normal antibodies, which alters your immune system response. It
has a lower risk of side effects than do plasmapheresis and immune-
suppressing therapy, but it can take a week or two to start working
and lasts only several weeks to months.
This therapy is expensive, and it's not clear that intravenous immune
globulin offers clear advantages over other available therapies. For
those reasons, it's often reserved for people who are severely ill.
As part of your treatment, your doctor may suggest physical therapy and
occupational therapy to help you adjust to tasks you need to do around the house
and in your job.
Be sure not to take any medications without checking with your doctor. Some
medications, such as some heart medications and botulinum toxin, can worsen
numbness in your legs and arms characterize GBS. Loss of feeling and movement
(paralysis) may occur in your legs, arms, upper body and face.
GBS affects an estimated one to three in every 100,000 persons annually in the
United States. It can strike any race at any age, but its incidence increases with age.
GBS may occur within days or weeks after a viral infection such as influenza (flu) or
diarrhea. It may be triggered by pregnancy or a medical procedure, such as a
vaccination or minor surgery, or have no evident reason for developing. Because the
cause of GBS is unknown, there's no way to prevent the disease from occurring.
In its most severe form, GBS is a medical emergency and may require
hospitalization. Severe GBS may result in total paralysis, potentially dangerous
fluctuations in heart rate and blood pressure, and inability to breathe without
respiratory assistance. The muscles you use for eye movement, speaking, chewing
and swallowing also may become weak or paralyzed. People with severe GBS often
need long-term rehabilitation to regain normal independence, and as many as 15
percent experience lasting physical impairment. In some cases, GBS can be fatal.
Most people recover from even the most severe cases of GBS. Available treatments,
if started soon after signs and symptoms appear, may lessen the severity of GBS and
reduce recovery time.
Signs and symptoms
The signs and symptoms of GBS usually appear rapidly over the course of a single
day. These may include:
• Weakness, tingling or loss of sensation that often begins in your feet
and legs and spreads to your upper body and arms
• Moderate pain throughout your body
• Difficulty breathing
• Paralysis of your legs, arms, respiratory muscles and face
• Difficulty with eye movement, facial movement, speaking, chewing or
• Very slow heart rate or low blood pressure
• Difficulty with bladder control or intestinal functions
GBS progresses quickly, with most people experiencing the most significant
weakness in the legs, arms, chest and other areas within three weeks of the start of
this disorder. In some cases, the signs and symptoms of GBS may progress very
rapidly with complete paralysis of legs, arms and breathing muscles over the course
of a few hours.
If GBS is mild, the signs and symptoms may not extend beyond a feeling of general
weakness. GBS may improve on its own within a few weeks, and some people
initially may think the signs and symptoms are due simply to a common virus.
The signs and symptoms of GBS may last days, weeks or months before muscle
sensation begins to return. Regaining your pre-illness strength and functioning is
slow, sometimes requiring months or years. However, most people with GBS return
to normal within months.
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Nerve and myelin sheath
Doctors and scientists don't know what causes Guillain-Barre syndrome. However,
they believe that GBS is a disorder of the immune system. Research indicates that
the immune system may destroy the protective covering of the peripheral nerves
(myelin sheath), which disables the nerves from transmitting signals to the muscles.
When this happens, muscles become unable to respond to nerve commands.
Weakness, numbness or paralysis may occur. The brain also receives fewer sensory
signals, resulting in the inability to feel heat, cold, pain and other sensations.
The most recognized conditions that occur before GBS develops are bacterial or viral
infections. Approximately two-thirds of people affected by GBS have had a recent
infectious illness, such as sore throat, diarrhea, cold or flu, within one to four weeks
before the onset of GBS. The Epstein-Barr virus or Hodgkin's disease also may
A bacterial infection such as campylobacteriosis — caused by campylobacter,
bacteria commonly found in undercooked food, especially poultry — may be a
possible cause of GBS. This form of the disorder is usually more severe because it
directly attacks the nerves, instead of the nerve coverings.
GBS may also be triggered by medical procedures including surgery and, in rare
cases, influenza immunizations. However, the connection between the flu vaccine
and GBS is weak, and the risk the vaccine poses to your health is much less
significant than your risk of serious flu-related illness.
When to seek medical advice
GBS is a serious disease that requires immediate hospitalization because of the rapid
rate at which it worsens. Early treatment is important. The sooner appropriate
treatment is started, the better the chance of a good outcome. If treatment can
result in reducing the inflammation of your peripheral nerves, the chances of serious
permanent damage to the myelin sheath and nerve fibers may be substantially
If you experience a tingling sensation in your toes, feet or legs followed by muscle
weakness, seek medical attention immediately. Failure to see your doctor promptly
may lead to progression of the disorder and paralysis, which can leave you
incapacitated and unable to get help.
Screening and diagnosis
GBS can be difficult to diagnose in its earliest stages. Its signs and symptoms are
similar to those of other neurological disorders and may vary from person to person.
The first step in diagnosing GBS is for your doctor to take a careful medical history to
fully understand the cluster of signs and symptoms that you're experiencing. People
with GBS have a relatively sudden onset of signs and symptoms, and muscle
weakness usually occurs on both sides of the body as opposed to on one side only,
as in a stroke.
Doctors commonly use two tests to determine if GBS is present:
• Electromyography (EMG). An EMG helps establish a diagnosis and
the extent of a neurological problem by measuring the electrical
activity of a muscle in response to nerve stimulation. The test also
measures the nature and speed of impulse conduction along a nerve.
In an EMG, a technician inserts electrodes in fine needles into the
muscles being tested and places electrodes on your skin over
• Spinal tap (lumbar puncture). This procedure involves inserting a
needle into your spinal canal, usually at the low back (lumbar) level.
Your doctor can determine the pressure of the cerebrospinal fluid
(CSF), and a sample of fluid can be removed for laboratory analysis.
This analysis may include checking for evidence of bleeding, the
number and types of white blood cells, the levels of glucose and
protein, the types of proteins, and tests for bacteria and fungi.
The long-term outlook for most people with GBS is good. About 75 percent to 85
percent of those affected recover completely or have only minor, residual weakness
or abnormal sensations such as numbness or tingling.
Up to 15 percent of people with GBS experience more serious, permanent problems
with sensation and coordination, including some cases of severe disability. About one
in ten of those affected is at risk of experiencing a relapse.
GBS may cause severe damage to your muscles and nervous system, weakening
your heart and lungs. About one-third of people with GBS require assisted ventilation
while they are ill. Up to one in 12 people with GBS die of related complications such
as respiratory failure, pulmonary embolism and cardiac arrest. Older adults with
other medical problems are at greatest risk of death from GBS.
There's no cure for GBS. However, certain therapies can lessen the severity of the
illness and accelerate recovery for most people. The general treatment for GBS is
supportive care to help with activities of daily living, such as eating and using the
bathroom. For some, recovery can take a long time — from several months to a year
According to a guideline developed by the American Academy of Neurology (AAN),
treating GBS early, within two to four weeks after signs and symptoms first appear,
may speed recovery time. Two main treatments, both equally effective, have been
shown to speed the recovery from and reduce the severity of GBS in adults:
• Plasmapheresis (plaz-muh-fuh-RE-sis). This treatment — also known
as plasma exchange — is a type of "blood cleansing" in which
damaging antibodies are removed from your blood. Plasmapheresis
consists of removing the liquid portion of your blood (plasma) and
separating it from the actual blood cells. The blood cells are then put
back into your body, which manufactures more plasma to make up for
what was removed. It's not clear why this treatment works, but
scientists believe that plasmapheresis removes certain antibodies from
plasma that contribute to the immune system attack on the peripheral
• Intravenous immunoglobulin (IVIg). Immunoglobulin contains
healthy antibodies from blood donors. High doses of immunoglobulin
can block the damaging antibodies in your blood that may contribute
The AAN guideline also shows no benefit in combining plasma exchange and IVIg.
Mixing the treatments or administering one after the other is no more effective than
if either method is used alone.
Your treatment also may include pain medications including acetaminophen and
nonsteroidal anti-inflammatory drugs, possibly in combination with narcotic
Often before recovery begins, caregivers may need to manually move your arms and
legs to help keep your muscles flexible and strong. After recovery has begun, you'll
likely need physical therapy to help regain strength and proper movement to be able
to function on your own. Whirlpool therapy (hydrotherapy) may help relieve pain and
retrain the movement of your affected limbs. After your symptoms subside, your
rehabilitation team will prescribe an active exercise routine to help you regain muscle
In the United States, amyotrophic lateral sclerosis is often called Lou Gehrig's
disease, after Hall of Fame baseball player Lou Gehrig of the New York Yankees, who
died of the disease in 1941.
The cause of amyotrophic lateral sclerosis remains elusive, as does any treatment
that can reverse the relentless progression of the disease. Treatment of amyotrophic
lateral sclerosis typically focuses on efforts to relieve symptoms and maintain quality
of life in the years after diagnosis.
Signs and symptoms
Signs of amyotrophic lateral sclerosis are typically subtle at first. The disease
commonly begins in one part of the body and progresses to other areas. Gradual
death of nerve cells saps muscles of their control. Early signs and symptoms of ALS
• Difficulty lifting the front part of your foot (footdrop)
• Weakness in your leg, feet or ankles
• Hand weakness or clumsiness
• Slurring of speech or trouble swallowing
• Muscle cramps and twitching in your arms, shoulders and tongue
• Fatigue in combination with the above signs and symptoms
The disease frequently begins in your hands, feet or limbs, and then spreads to other
parts of your body. It eventually affects chewing, swallowing, speaking and breathing
— which are known as bulbar functions after the bulb-shaped part of the brainstem
that houses nerve cells controlling these functions. Less commonly, ALS begins with
these bulbar symptoms. As the disease advances, your muscles become
progressively weaker until they're paralyzed.
Eventually, amyotrophic lateral sclerosis paralyzes the muscles needed to breathe.
Most people with ALS die of respiratory failure, usually within three to five years
after symptoms begin. Sometimes, people with amyotrophic lateral sclerosis develop
pneumonia because they can't swallow and they inhale (aspirate) food and oral
secretions into their lungs.
ALS usually leaves intellect intact and spares the senses of sight, hearing, smell,
taste and touch. It doesn't affect involuntary muscles, such as the muscles that
control heartbeat and bladder and bowel function.
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The basic units of your central nervous system — which includes your brain and
spinal cord — are nerve cells (neurons). Each neuron consists of a cell body, a major
branching fiber (axon) and numerous smaller branching fibers (dendrites). Nerve
cells communicate with adjacent nerve cells at contact points called synapses. They
also send and receive signals throughout your body.
Nerve signals provide information to your brain through your senses. They
communicate with your involuntary muscles — the ones that control activities such
as digestion, sexual function and heartbeat. Nerve signals also activate your
Amyotrophic lateral sclerosis is a disease that involves death of motor neurons — the
nerve cells that control voluntary muscles. These are the muscles you use to move
your limbs, face, neck and torso and to talk, chew, swallow and breathe. In ALS,
both upper motor neurons, located in your brain, and lower motor neurons, located
in your spinal cord, gradually die. As a result, your voluntary muscles no longer
receive messages. These muscles become weak and begin to waste away (atrophy).
Researchers aren't sure what causes motor neuron deterioration, but amyotrophic
lateral sclerosis appears to fall into two categories:
• Inherited (familial) ALS. Up to one in 10 cases of ALS appear to be
inherited. About 20 percent of these cases result from a specific gene
mutation in an enzyme known as superoxide dismutase 1 (SOD1).
Scientists and researchers are still trying to identify other ALS gene
mutations that may be inherited.
• Sporadic ALS. More than 90 percent of cases of ALS appear to occur
randomly, with no identifiable cause and no obvious risk factors.
One possible cause of amyotrophic lateral sclerosis is excess glutamate — one of the
many chemicals (neurotransmitters) that neurons use to send signals to one
another. People with ALS appear to have too much glutamate in their nervous
systems, which can cause neurons to die.
Researchers are studying other factors that may contribute to amyotrophic lateral
sclerosis, including viruses, immune system abnormalities, environmental toxins, cell
suicide (apoptosis), defects in the energy-producing parts of a cell (mitochondria)
and the buildup of certain proteins known as neurofilaments. Some researchers
believe that a combination of more than one factor may trigger ALS.
Although most cases of amyotrophic lateral sclerosis have no apparent cause, a
small percentage of people who have ALS inherit it. Inherited ALS may strike at a
younger age and will affect consecutive generations. If you're the first person in your
family to have amyotrophic lateral sclerosis, you likely don't have an inherited form,
and your children aren't at a greater risk.
Noninherited ALS often develops between ages 40 and 60, although the disease
affects people of all ages. ALS affects more men than women.
When to seek medical advice
If you've noticed weakness in one of your limbs or twitching and cramping in your
muscles, see your doctor to determine if there may be a neurological cause such as
amyotrophic lateral sclerosis. Although there's no treatment to reverse the course of
ALS, other neurological conditions that may mimic some of the signs and symptoms
of ALS are treatable.
It's easy to ignore the early, sometimes subtle signs of amyotrophic lateral sclerosis.
In fact, it takes about a year before most people are diagnosed with ALS. But the
sooner you see your doctor, the sooner a diagnosis and treatment decisions can be
Screening and diagnosis
Amyotrophic lateral sclerosis is difficult to diagnose early because it may appear
similar to other neurological diseases. Your doctor may refer you to a doctor who
specializes in brain and nervous system disorders (neurologist).
Diagnosing amyotrophic lateral sclerosis first involves reviewing your family's
medical history and your signs and symptoms. You'll then have a physical
examination that may include some basic in-office testing of your nerves and
muscles. Additionally, you'll undergo a variety of tests, including tests to rule out
other conditions. These diagnostic tests may include:
• Electromyogram (EMG). Electromyography measures the tiny
electrical discharges produced in muscles. A thin-needle electrode is
inserted into the muscles that your doctor wants to study. An
instrument records the electrical activity in your muscle as you rest
and contract the muscle.
• Nerve conduction study (NCS). As a part of an electromyography
study, two electrodes are taped to your skin above a nerve or muscle
to be studied. A small shock is passed through the nerve to measure
the size and speed of nerve signals.
• Imaging. Your doctor may suggest magnetic resonance imaging
(MRI) or a computerized tomography (CT) scan to produce images of
your brain and spinal cord. These tests can help your doctor determine
if something other than ALS may be causing your signs and
• Spinal tap (lumbar puncture). This test analyzes the fluid
surrounding your brain and spinal cord (cerebrospinal fluid). You
typically lie on your side with your knees drawn up to your chest. A
local anesthetic is injected in an area over your lower spine to reduce
any discomfort from the procedure. Then a needle is inserted into your
spinal canal, and fluid is collected. Tests on this fluid indicate the
number and types of white blood cells, the level of sugar, and the
levels and types of proteins. Additional tests on this fluid can indicate
evidence of bleeding, infection and abnormal cells.
• Blood and urine tests. Analyzing samples of your blood and urine in
the laboratory may help your doctor eliminate other possible causes of
your signs and symptoms.
• Muscle biopsy. If your doctor believes you may have a muscle
disease rather than ALS, you may undergo a muscle biopsy. In this
procedure, a small portion of tissue is removed while you're under
local anesthesia and is sent to a lab for analysis.
Because there's no reversing the course of amyotrophic lateral sclerosis, treatments
mainly involve efforts to make you more comfortable and independent and to slow
the progression of symptoms. Treatments may involve many professionals, including
doctors, therapists, dietitians, social workers and hospice nurses, and may include:
• Medications. The drug riluzole (Rilutek) is the first and only
medication approved by the Food and Drug Administration for slowing
ALS. The drug appears to slow the disease's progression and prolong
life by a few months in some people, perhaps by reducing glutamate
levels. Your doctor may also prescribe medications to provide relief
from muscle cramps and constipation and to reduce fatigue, excessive
salivation and excessive phlegm. Some people with amyotrophic
lateral sclerosis also benefit from medications for pain or depression.
• Physical and occupational therapy. A physical therapist can
recommend low-impact exercises to maintain your muscle strength
and range of motion as long as possible, helping you preserve a sense
of independence. An occupational therapist can help you become
accustomed to a brace, walker or wheelchair and may be able to
suggest devices such as ramps that make it easier for you to get
• Speech therapy. Because ALS affects the muscles you use to speak,
communicating clearly becomes an issue as the disease progresses. A
speech therapist can help teach techniques to make your speech more
clearly understood. Later in the disease, a speech therapist can
recommend devices such as speech synthesizers and computers that
may help you communicate.
• Nutritional support. A dietitian can advise you on foods that are
nutritious but easy to swallow. As the disease progresses and you
have greater difficulty swallowing, you may consider a feeding tube
inserted into your stomach to receive nutrition and avoid choking.
• Breathing assistance. ALS progressively weakens the muscles you
need for breathing, making it more difficult to breathe. At first, you
may benefit from a device that makes it easier for you to breathe
during the night. Some devices, such as masks or nose inserts, fit over
your face and are easy to use or remove. The mask or nose insert is
attached to a portable machine that provides air pressure to inflate
your lungs. You can also use these devices during the day.
As breathing muscles weaken further, you may need to decide whether
or not to use the full-time help of a respirator that inflates and deflates
your lungs. A respiratory therapist or lung specialist (pulmonologist)
can help you choose devices that will work for you.
Researchers are investigating many other medications for their usefulness in treating
amyotrophic lateral sclerosis. Examples include the antibiotic minocycline (Minocin),
the breast cancer drug tamoxifen, the antioxidant coenzyme Q10 and a nerve-
nourishing drug called insulin-like growth factor (IGF-I). Increasingly, researchers
are studying drug "cocktails" — which are combinations of medications. Stem cell
therapy is another avenue of research. Early studies show that stem cells may have
the ability to repair or replace the motor neurons damaged by ALS, but clinical use or
clinical trials with stem cell therapy is still a long way off. Only time will tell if these
new treatments will be useful.
It's important to work closely with a doctor who specializes in amyotrophic lateral
sclerosis, who will be informed as new discoveries and treatments may become
available. Some health care centers offer ALS clinics where people with ALS can work
with physical therapists, occupational therapists, speech therapists, nurses, social
workers and other people with ALS — all in the same place.
No one chooses to live with amyotrophic lateral sclerosis. Yet many have managed to
do it with daring, dignity, humor and a hopeful attitude. If you have ALS, you can,
• Take time to accept your diagnosis and the changes it will
bring. Hearing that you have a fatal condition that will rob you of your
mobility and independence is often nothing less than shocking. If you
are newly diagnosed, you and your family will likely experience a
period of mourning and grief that is similar to the period after the
death of a loved one. Although the grieving process is different for
everyone, it's common to experience denial or disbelief, then sadness,
anger, bargaining and, finally, acceptance.
• Be hopeful. Some people with amyotrophic lateral sclerosis live much
longer than the three to five years typically associated with this
condition. Some live 10 years or more. Keeping hope alive is vital for
people with ALS.
• Think beyond the physical changes. ALS doesn't typically affect the
intellect or spirit. Many people with amyotrophic lateral sclerosis lead
rich, rewarding lives. Try to think of ALS as only one part of your life,
not your entire identity.
Signs and symptoms
Signs and symptoms of multiple sclerosis vary widely, depending on the location of
affected nerve fibers. Multiple sclerosis symptoms may include:
• Numbness or weakness in one or more limbs, which typically occurs on
one side of your body at a time or the bottom half of your body
• Partial or complete loss of vision, usually in one eye at a time, often
with pain during eye movement
• Double vision or blurring of vision
• Tingling or pain in parts of your body
• Electric-shock sensations that occur with certain head movements
• Tremor, lack of coordination or unsteady gait
In some cases, people with multiple sclerosis may also develop muscle stiffness or
spasticity, slurred speech, paralysis, or problems with bladder, bowel or sexual
function. Mental changes, such as forgetfulness or difficulties with concentration, also
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Your central nervous system contains millions of nerve cells that send their electrical
signals to and from your brain along wire-like extensions of the cells called axons, or
nerve fibers. Myelin is the fatty substance that coats and protects these fibers,
similar to the way insulation shields electrical wires.
In people with multiple sclerosis, the immune system mistakenly destroys the cells
that produce the myelin sheath. As a result, myelin becomes inflamed and swollen
and detaches from the nerve fibers. The detached myelin may eventually be
destroyed. Firm or hardened (sclerosed) patches of scar tissue form over the fibers.
When nerve impulses reach a damaged area, some impulses are blocked or delayed
from traveling to or from your brain. Ultimately, this process leads to degeneration of
the nerves themselves, which likely accounts for the permanent disabilities that may
develop in MS.
Doctors and researchers don't understand what causes this autoimmune reaction.
Something seems to trigger the condition in susceptible people.
Genetic factors may make certain people more susceptible to multiple sclerosis. But
genetic susceptibility is only part of the explanation. A number of researchers believe
the disorder is related to a protein that mimics the myelin protein, which may be
introduced into the body by a virus. Other researchers believe that the immune
system overreacts toward myelin proteins in people with MS, which leads to an
abnormal tendency to develop autoimmune disease.
A period of disease activity (exacerbation) may be triggered by a viral infection, such
as a cold or flu, or by changes in the immune system during the first six months
following a pregnancy.
Patterns of MS
Whatever the multiple sclerosis cause or trigger, the disease occurs in four main
• Relapsing remitting. This type of multiple sclerosis is characterized
by clearly defined flare-ups, followed by periods of remission. The
flare-ups typically appear suddenly, last a few weeks or months, and
then gradually disappear. Most people with MS have this form at the
time of diagnosis.
• Primary progressive. People with this less common form of multiple
sclerosis experience a gradual decline, without periods of remission.
People with this form of MS are usually older than 40 when signs or
• Secondary progressive. More than half the people with relapsing
remitting MS eventually enter a stage of continuous deterioration
referred to as secondary progressive MS. Sudden relapses may occur,
superimposed upon the continuous deterioration that characterizes this
type of multiple sclerosis.
• Progressive relapsing. This is primary progressive MS with the
addition of sudden episodes of new symptoms or worsened existing
ones. This form is relatively uncommon.
These factors may increase your risk of developing multiple sclerosis:
• Heredity. Multiple sclerosis is more common in people of Northern
European descent. There also appears to be a genetic component to
the condition, although the risk to children of people affected by MS is
less than 5 percent over their lifetime. Researchers suspect that the
tendency to develop multiple sclerosis is inherited, but the disease
manifests only when environmental triggers are present.
• Environmental factors. Environmental factors have some influence
on multiple sclerosis. Many viruses and bacteria have been suspected
of causing MS, most recently the Epstein-Barr virus, known also for
causing infectious mononucleosis. Some studies have suggested that
developing infection at a critical period of exposure may lead to
conditions conducive to the development of MS a decade or more
• Geographical factors. Multiple sclerosis is more common in countries
with temperate climates, including Europe, southern Canada, northern
United States, and southeastern Australia. The reason is unknown.
When to seek medical advice
One of the early indications of multiple sclerosis is numbness and tingling in your
arms, legs or elsewhere in your body. Although numbness most often doesn't
indicate MS, it's important to see your doctor if you experience numbness or tingling
that persists for days or weeks — especially if it spreads to involve large parts of
your body. Your doctor can determine whether MS or another neurological disorder
may be the cause. Also see your doctor if you experience persistent double vision,
persistent loss of balance or visual loss.
Screening and diagnosis
Multiple sclerosis can be difficult to diagnose. Many other conditions may produce
symptoms similar to multiple sclerosis, but with a different prognosis and treatment.
There are no specific tests for multiple sclerosis. Ultimately, the diagnosis relies on a
determination that the clinical symptoms, radiological studies and laboratory studies
suggest MS, and that no other condition provides a better explanation for them. Your
doctor may base a multiple sclerosis diagnosis on the following:
• Medical history. Your doctor reviews your signs and symptoms and
• Neurological examination. This examination systematically tests
various parts of your nervous system, including your reflexes, muscle
strength, muscle tone, and sensations of pain, heat, touch and
vibration. Your doctor may also observe your gait, posture,
coordination and balance, and ask you questions to help determine the
clarity of your thinking, judgment and memory.
• Magnetic resonance imaging (MRI) scan. The cylinder-shaped MRI
scanner creates tissue-slice images on a computer from data
generated by a powerful magnetic field and radio waves. Your doctor
can view these images from any direction or plane.
This imaging technique may reveal MS lesions, which are caused by
myelin loss. An intravenous dye, gadolinium, will highlight "active"
lesions that have developed within the past two months and this may
help doctors know whether the MS is in an active phase, even if no
symptoms are present indicating an attack of MS. Newer MRI
techniques can provide even greater detail about the degree of nerve
fiber injury or permanent myelin loss and recovery.
• Spinal tap (lumbar puncture). In this procedure, a doctor or nurse
removes a small sample of cerebrospinal fluid from within your spinal
canal for laboratory analysis. This sample can show abnormalities
associated with multiple sclerosis, such as abnormal levels of white
blood cells or proteins. This procedure can also help rule out viral
infections and other conditions that can cause neurological symptoms
similar to those of MS.
• Evoked potential test. This test measures the electrical signals sent
by your brain in response to stimuli. An evoked potential test may use
visual stimuli or electrical stimuli, in which short electrical impulses are
applied to your legs or arms.
If your attacks are mild or infrequent, your doctor may advise a wait-and-see
approach, with counseling and observation.
Medications for relapsing MS
If you have a relapsing form of the disease, your doctor may recommend treatment
with disease-modifying medications early in the course of disease. You can't take
these medications if you're pregnant or may become pregnant. These medications
for multiple sclerosis treatment include:
• Beta interferons. Interferon beta-1b (Betaseron) and interferon
beta-1a (Avonex, Rebif) are genetically engineered copies of proteins
that occur naturally in your body. They help fight viral infection and
regulate your immune system.
If you use Betaseron, you inject yourself under your skin
(subcutaneously) every other day. If you use Rebif, you inject yourself
subcutaneously three times a week. You self-inject Avonex into your
muscle (intramuscularly) once a week. These medications reduce but
don't eliminate flare-ups of multiple sclerosis. It's uncertain which of
their many actions lead to a reduction in disease activity and what
their long-term benefits are. Beta interferons aren't used in
combination with one another; only one of these medications is used
at a time.
The Food and Drug Administration (FDA) has approved beta
interferons only for people with relapsing forms of MS who can still
walk. Beta interferons don't reverse damage and haven't been proved
to significantly alter long-term development of permanent disability.
Some people develop antibodies to beta interferons, which may make
them less effective. Other people can't tolerate the side effects, which
may include symptoms similar to those of the flu (influenza).
Doctors generally recommend beta interferons for people who have
more than one attack of MS a year and for those who don't recover
well from flare-ups. The treatment may also be used for people who
have a significant buildup of new lesions as seen on an MRI scan, even
when there may not be major new symptoms of disease activity.
The FDA has approved the use of several beta interferons for people
who've experienced a single attack that suggests multiple sclerosis,
and who may be at risk of future attacks and developing definite MS.
Risk of MS may also be suggested when an MRI scan of the brain
shows lesions that predict a high risk of conversion to definite MS.
Controversy exists as to whether these people should take these
expensive and often inconvenient drugs for indefinite periods,
especially because some people do well both in the short term and
long term without therapy. Some doctors prefer to observe people at
high risk with follow-up examinations and MRI scans to document any
ongoing inflammatory disease activity before recommending long-term
therapies such as beta interferon.
• Glatiramer (Copaxone). This medication is an alternative to beta
interferons if you have relapsing remitting MS. Doctors believe that
glatiramer works by blocking your immune system's attack on myelin.
You must inject glatiramer subcutaneously once daily. Side effects
may include flushing and shortness of breath after injection.
• Natalizumab (Tysabri). This drug is administered intravenously once
a month. It works by blocking the attachment of immune cells to brain
blood vessels — a necessary step for immune cells to cross into the
brain — thus reducing the immune cells' inflammatory action on brain
During clinical trials, this drug was shown to significantly reduce the
frequency of attacks in people with relapsing MS. After receiving FDA
approval, however, the drug was withdrawn from the market because
of reports from three people who developed a rare, often fatal, brain
disorder called progressive multifocal leukoencephalopathy.
In 2006, after reconsideration of the drug's benefits for people with
multiple sclerosis, the FDA agreed to allow the drug to be marketed
again under specific conditions. Chief among these conditions is the
requirement that doctors, pharmacists and patients be involved in a
special distribution program known as TOUCH in order to prescribe,
dispense or receive the drug. Because of the drug's risks, it's generally
recommended only for people whose condition hasn't responded to
other forms of MS therapy. Furthermore, there has been no study
direct comparing natalizumab to existing treatments to prove whether
it's superior to existing treatments.
• Other medications. Mitoxantrone (Novantrone) is a chemotherapy
drug used for many cancers. This drug is also FDA-approved for
treatment of aggressive forms of relapsing remitting MS, as well as
certain forms of progressive MS. It's given intravenously, typically
every three months.
Mitoxantrone may cause serious side effects, such as heart damage,
after long-term use, so it's typically not used for longer than two to
three years. And it's typically reserved for people with severe attacks
or rapidly advancing disease who don't respond to other treatments.
Close monitoring is critical for anyone on this medication.
Some doctors are also prescribing other chemotherapy drugs, such as
cyclophosphamide (Cytoxan), for people with severe, rapidly
progressing MS. However, these medications aren't FDA-approved for
treatment of MS.
Medications for progressive MS
Some medications may relieve symptoms of progressive MS. They include:
• Corticosteroids. Doctors most often prescribe short courses of oral or
intravenous corticosteroids to reduce inflammation in nerve tissue and
to shorten the duration of flare-ups. Prolonged use of these
medications, however, may cause side effects, such as osteoporosis
and high blood pressure (hypertension), and the benefit of long-term
therapy in multiple sclerosis isn't established.
• Muscle relaxants. Baclofen (Lioresal) and tizanidine (Zanaflex) are
oral treatments for muscle spasticity. If you have multiple sclerosis,
you may experience muscle stiffening or spasms, particularly in your
legs, which can be painful and uncontrollable. This typically occurs in
people with persisting or progressive weakness of their legs. Baclofen
may temporarily increase weakness in your legs. Tizanidine controls
muscle spasms without causing your legs to feel weak, but can be
associated with drowsiness or a dry mouth.
• Medications to reduce fatigue. To help combat fatigue, your doctor
may prescribe an antidepressant medication, the antiviral drug
amantadine (Symmetrel) or a medication for narcolepsy called
modafinil (Provigil). All drugs prescribed for this purpose appear to
work because of their stimulant properties. One study has showed that
aspirin treatment may be effective in controlling MS-related fatigue;
further research is planned to address the benefits of aspirin on
• Other medications. Many medications are used for the muscle
stiffness, depression, pain and bladder control problems associated
with multiple sclerosis. Drugs for arthritis and medications that
suppress the immune system may slow MS in some cases.
MS treatments other than medications
In addition to medications, these treatments also may be helpful:
• Physical and occupational therapy. A physical or occupational
therapist can teach you strengthening exercises and show you how to
use devices that can ease the performance of daily tasks. Therapists
are usually supervised by doctors (physiatrists) who advise and
coordinate the therapy that you might receive. Therapists can assist
you in finding optimal mobility assistance devices such as canes,
wheelchairs and motorized scooters. These devices and exercises can
help preserve your independence.
• Counseling. Individual or group therapy may help you cope with
multiple sclerosis and relieve emotional stress. Your family members
or caregivers also may benefit from seeing a counselor.
• Plasma exchange (plasmapheresis). Plasma exchange may help
restore neurological function in people with sudden severe attacks of
MS-related disability who don't respond to high doses of steroid
treatment. This procedure involves removing some of your blood and
mechanically separating the blood cells from the fluid (plasma). Your
blood cells then are mixed with a replacement solution, typically
albumin, or a synthetic fluid with properties like plasma. The solution
with your blood is then returned to your body.
Replacing your plasma may dilute the activity of the destructive
factors in your immune system, including antibodies that attack
myelin, and help you to recover. Plasma exchange has no proven
benefit beyond three months from the onset of the neurological
These steps may help relieve some symptoms of multiple sclerosis:
• Get enough rest. Fatigue is a common symptom of multiple sclerosis,
and getting your rest may make you feel less tired.
• Exercise. Regular aerobic exercise may offer some benefits if you
have mild to moderate MS. Benefits include improved strength, muscle
tone, balance and coordination, and help with depression. Swimming is
a good option for people with MS who are bothered by heat.
• Be careful with heat. Extreme heat may cause extreme muscle
weakness. Although some people with multiple sclerosis aren't
bothered by heat and may enjoy warm baths and showers, be very
careful before exposing yourself to extreme heat until you know how
you'll react. Don't get into a hot tub or sauna unless there's someone
nearby who can pull you out if necessary. If you do experience heat-
related worsening of signs or symptoms, cooling down for a few hours
usually will return you to your normal state.
• Cool down. Many people with multiple sclerosis experience heat-
related worsening of MS symptoms. If you live in a hot and humid
area, consider having air conditioning in your home. Tepid or cool
baths also may provide some relief.
• Eat a well-balanced diet. Eating a healthy, well-balanced diet can
help keep your immune system strong.
As is true with other chronic diseases, living with multiple sclerosis can place you on
a roller coaster of emotions. Here are some suggestions to help you even out the ups
• Maintain normal daily activities as best you can.
• Stay connected with friends and family.
• Continue to pursue hobbies that you enjoy and are able to do.
If multiple sclerosis impairs your ability to do things you enjoy, talk with your doctor
about possible ways to get around the obstacles.
Remember that your physical health can directly impact your mental health. Denial,
anger and frustration are not uncommon when you learn life has dealt you
something painful and unexpected. Professionals such as therapists or behavioral
psychologists may help you put things in perspective. They can also teach you
coping skills and relaxation techniques that may be helpful.
Sometimes, joining a support group, where you can share experiences and feelings
with other people, is a good approach. Ask your doctor what support groups are
available in your community.
Many chronic illnesses, including multiple sclerosis, are associated with an increased
risk of depression. This isn't a failure to cope. Rather, depression may indicate a