Presentation and interview

1. Ocular Myasthenia Gravis: As Seen Through My
Mother’s Eyes
Nicole Levina – Maria Demos
By PresenterMedia.com

2. Explain in detail the ways in which this course has allowed you to better analyze
the events and phenomena around you.
This course has allowed me to learn about the brain, and more specifically to
understand its function in detail. I was further intrigued to uncover the various
issues that arise as a result of strokes, lesions and other diseases such as
Myasthenia Gravis. This new learning has allowed me to be more empathic towards
those whose lives have changed as a result of damages to the brain. In some way, I
no longer take the role of the brain and its various parts for granted.
I felt challenged at times with my lack of prior knowledge and exposure but I am
proud of what I learned, read and saw.
In my efforts to further explore Myasthenia Gravis, specifically Ocular Myasthenia, I
have interviewed my mother who has had the disease for over thirty years.
Although she is currently in remission. I am in continued awe of her strength and
determination. I am thankful for her and for this opportunity to continue learning.

3. Origins of Myasthenia Gravis
 Myasthenia gravis, which is Latin and Greek in origin, literally means "grave muscle
weakness”.
 Myasthenia gravis is a chronic autoimmune neuromuscular disease characterized
by varying degrees of weakness of the skeletal (voluntary) muscles of the body.
 Thomas Willis, a 17th Century Oxford physician was the first to recognize it is a
disease.
 The first modern description was made by Samuel Wilkes in 1877.
 Indian Chief Opechankanough (died 1644), may represent the first recognized case
of MG in the United States.
 Occurs in all ethnic groups and in both genders. Usually females are diagnosed
between the ages of 25-40, while it may occur in men over 60.

4. Origins of Myasthenia Gravis
 Neonatal myasthenia , given to the child via mother’s immune antibodies, is temporary
with symptoms disappearing within the first two to three months.
 Myasthenia rarely occurs in juveniles.
 It is not directly inherited or contagious
 Children may show signs of congenital myasthenia or congenital myasthenic syndrome.
These are not autoimmune disorders, but are caused by defective genes that produce
abnormal proteins.
 Myasthenia gravis can be classified according to which skeletal muscles are affected.
Within a year of onset, approximately 85–90% of patients develop generalized
myasthenia gravis, which is characterized by weakness in the trunk, arms, and legs.
 About 10–15% of patients have weakness only in muscles that control eye movement.
This type is called ocular myasthenia gravis.

5. •ClinicalOverviewof
MyastheniaGravis
 Myasthenia is caused by a defect in the transmission of nerve
impulses to muscles.
 It occurs when normal communication between the nerve and
muscle is interrupted at the neuromuscular junction.
 Normally as impulses travel down the nerve, the nerve endings
release a neurotransmitter substance called acetylcholine.
 Acetylcholine travels from the neuromuscular junction and binds
to acetylcholine receptors which are activated and generate a
muscle contraction.
 Antibodies block, alter, or destroy the receptors for acetylcholine
at the neuromuscular junction, which prevents the muscle
contraction from occurring.
 These antibodies are produced by the body's own immune system
 As a result the immune system, whose role is to protect the body
attacks itself.

6. •Normal Receptors vs Myasthenia Gravis

7. The Role of the Thymus Gland in Myasthenia Gravis
 Located beneath the breastbone may be responsible for sending
incorrect instructions to developing immune cells.
 This may lead to the production of the acetylcholine receptor
antibodies, thereby setting the stage for the attack on
neuromuscular transmission.
 Some patients with MG may develop thymomas (benign tumors)
which need to be removed.
 In severe MG cases a complete thymectomy is recommended to
alleviate symptoms.

8. Immunosuppressants -Alter your immune system, such as azathioprine
(Imuran), mycophenolate mofetil (CellCept), cyclosporine (Sandimmune,
Neoral) or tacrolimus (Prograf).
Cholinesterase inhibitors-Such as pyridostigmine
(Mestinon) enhance communication between nerves
and muscles, may improve muscle contraction and
muscle strength.
Corticosteroids-Such as prednisone inhibit the immune system,
limiting antibody production.

9. Plasmapheresis– Removes the antibodies in the blood
that block the transmission of signals from nerve
endings to the muscle receptor sites.
Intravenous immunoglobulin (IVIg)- Provides the body with normal antibodies
Robot-assisted thymectom or video assisted thymectomy-
Removal of thymus may allow for symptoms and the taking of
medications to stop

10. Muscle weakness worsens as
the affected muscle(s) is used
repeatedly
Ptosis – drooping of one or
both eyelids
Diplopia – double vision
Issues with speaking
 Difficulties swallowing,
chewing and choking
Limited facial expressions –
“lost your smile”
Trouble walking
Use of arms, or hands limited
Holding up head is a
challenge
•Common Symptoms of Myasthenia Gravis

11. Fatigue
Illness
Extreme heat
Stress
 Medications – beta blockers,
quinine, quinidine gluconate,
quinidine sulfate for example
Certain anesthetics and
antibiotics
FACTORS THAT MAY ENHANCE
SYMPTOMS OF MYASTHENIA
GRAVIS

12. •ClinicalOverviewofOcular
MyastheniaGravis
 Form of MG in which the muscles that move the eyes and control the
eyelids are easily fatigued and weakened.
 Patients have trouble with ptosis or diplopia, the symptoms can range
from mild to severe, and can change from day to day.
 Problems with double vision and drooping eyelids are often the first
symptoms of MG.
 One hypothesis is that patients may simply notice eye weakness more
often than mild weakness in other muscle groups in the body.
 Another hypothesis is that the eye and eyelid muscles are structurally
different from muscles in the trunk and limbs, for example, they have
fewer acetylcholine receptors.
 The final hypothesis may be that eye muscles respond differently to
immune attack.

13. Using eyelid tape, or eyelid crutches
Thymectomy is usually not considered for people with ocular MG, unless
symptoms are severe or debiltating
Wearing dark glasses in bright light,which some pa-
tients find helpful.
Agents that improve neuromuscular transmission, such
as Mestinon®, may be helpful for ptosis, but not for
diplopia.

14. • How old were you when your first symptoms appeared? I was thirty eight years old
and had been perfectly healthy before them.
• What were your symptoms? I experienced double vision, both my eyelids drooped
especially in bright sunlight or from lights in the house. I often choked on my own
saliva, which was very frightening.
• What was your initial diagnosis? My initial diagnosis after my first experience was
general Myasthenia Gravis. Later on it was narrowed down to Ocular Myasthenia
Gravis.
• How did you feel after hearing this diagnosis? I felt scared, not being fully aware of
what to expect. English is not my native language, and my doctor was Greek
Australian so I needed to make sure that we could understand and communicate with
each other. I wanted to learn as much as I could about this disease which was a
challenge because we had to rely on books and articles instead of the internet or
support groups.
Interview with my Mom:

15. • How was your Myasthenia diagnosed? First the doctor tested my reflexes, muscle
strength and tone, my balance and coordination. I had a blood test as well as an
Edrophonium test which was an injection of edrophonium chloride to see if I had MG.
The most painful was the repetitive nerve simulation test where small pulses of
electricity were sent through electrodes that were over my muscles.
• What happened next? We decided to get a second opinion and traveled to the US
where I underwent the same battery of tests. My doctor talked about removing my
thymus as a possibility if my symptoms persisted over time and if they worsened. At
that point we decided to try Mestinon which I took twice a day.
• You have lived with Ocular Myasthenia Gravis for over thirty years, can you share
your symptoms at their worst? Crossing the street, needing to focus ahead while my
eyelid drooped and I had double vision was the worst. Trying to do daily tasks when
both natural and artificial light made it hard to see. I often wore dark sunglasses
inside. Sudden bouts of choking were dangerous, especially during meals. When I
was tired or stressed, sometimes my speech was slurred.
Interview with my Mom:

16. • You have lived with Ocular Myasthenia Gravis for over thirty years, can you share
your symptoms at their best? Over the years, I have been lucky to be in remission for
years. During this time, my ptosis and diplopia have disappeared and I have not taken
any medication. I have recognized when I am tired or stressed and then carefully
monitor my eyes. I also am aware of what to do when the sunlight is too bright or too
low on the horizon. I am aware of my immediate environment in order to ‘prevent’
symptoms from emerging stronger. Usually after a very stressful time, the symptoms
come back. Often in the Fall or Spring, when the sun is lower and shines in my eyes,
they may come back.
• How are you today? Today I am still sensitive to bright sunlight and/or artificial light
and so I take prednisone, 10mg a day. I am not experiencing ptosis, diplopia, slurred
speech or choking incidents. I try and stay physically healthy and eat a balanced diet.
• How has this affected your personal life? Overall this disease has been challenging and
scary for me and for my family, especially when my symptoms are at their worst. My
family has been supportive and we are able to have honest discussions about my
symptoms and treatments.
Interview with my Mom:

17. • How has this affected your professional life? When I have been symptom free, I have been
able to work in the school library without any problems. I always made sure that my desk
was in front of the windows and that the natural light was behind me. When my
symptoms returned, I took my medication and made sure that I could focus to do my job. I
was lucky that my colleagues and my bosses were supportive.
• What advice would you give to someone with this same condition? Stay positive and
informed, ask questions and live your life as fully as you can. Don’t let other people tell
you what you can do, and what you can see because you need to be in control of your
vision and your behaviors.
• Thanks Mom, for your interview Thank you for letting me share my experiences.
Interview with my Mom:

18. Focused MGFA Research
Priorities
 Etiology- what is the basic cause of the
MG?
 Detection and early diagnosis: Would
earlier diagnosis lead to improved
treatment?
 Genetics of MG.
 Enhance understanding and application
of current treatments of MG
 Thymus: how exactly does the thymus
gland promote the production of
acetylcholine receptor antibodies?
 New therapeutic targets identified for
MG, with the goal of improved
treatment responses and fewer
adverse events
 New strategies for treatment including
development of potential vaccine
therapies.
 Quality of Life: develop better
strategies to improve quality of life and
develop rigorous research methods to
study quality of life in MG.
 Collateral effects of MG.
RESEARCH HIGLIGHTS

19. Resources
http://www.mayoclinic.org/diseases-conditions/myasthenia-gravis/basics/symptoms/con-
20027124
http://www.mayoclinic.org/diseases-conditions/myasthenia-gravis/basics/causes/con-
20027124
http://www.mayoclinic.org/diseases-conditions/myasthenia-gravis/basics/treatment/con-
20
http://www.ninds.nih.gov/disorders/myasthenia_gravis/detail_myasthenia_gravis.htm
http://www.nmd-journal.com/article/S0960-8966(05)00256-7/abstract

20. Resourceshttp://www.webmd.com/brain/understanding-myasthenia-gravis-basics
http://myasthenia.org/Home.aspx
http://myasthenia.org/LinkClick.aspx?fileticket=efzk1NMTJBk%3d&tabid=84
http://www.ncbi.nlm.nih.gov/pubmed/3277598
http://myasthenia.org/WhatisMG/ResearchHighlights.aspx
ttp://intranet.tdmu.edu.ua/data/kafedra/internal/vnutrmed2/classes_stud/en/med/lik/ptn/Clinical%20imunology/5%20c
ourse/04.%20The%20immunological%20aspects%20of%20autoimmune%20diseases.files/image016.jpg
Class notes from video lectures
Interview with my Mother, conducted July 2014