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What is Neuro-Ophthalmology? |
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Neuro-ophthalmology is an ophthalmic subspecialty that addresses
the relationship between the eye and the brain, specifically disorders
of the optic nerve, orbit, and brain, associated with visual symptoms.
Neuro-ophthalmologists provide comprehensive clinical care to a
broad spectrum of patients with visual disturbance from optic nerve
diseases, central nervous system disorders, ocular motility dysfunction,
and pupillary abnormalities. Over 50 percent of all intracranial
lesions involve the visual or oculomotor pathways. Shroff Eye Centre
offers you a dedicated Neuro-Ophthalmology Service for the diagnosis
and treatment of these neuro-ophthalmic disorders.
Click for Appointment
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The Neuro-Ophthalmology Service at Shroff Eye Centre
Learn about neuro-ophthalmic disorders.
What are the common symptoms of neuro-ophthalmic diseases?
What are the common types of neuro-ophthalmic diseases?
What is Ischemic Optic Neuropathy (AION)? |
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This is the most common cause of sudden decreased vision in patients
older than 40 years. We do not see with our eyes. We see with part
of our brain that is capable of interpreting visual signals sent
back from the eyes. This is located at the back of our head (the
occipital lobes). Information is transmitted from the eyes to the
brain via the optic nerves. These nerves are composed of the long
tube extensions (axons) of cells (ganglion cells) located within
the inner lining of the eye (the retina) that exit the back of the
eye at the optic disc. Each of the optic nerves receives blood supply
from branches of the ophthalmic artery within each eye socket. The
optic disc has a unique blood supply (the posterior ciliary arteries).
Loss of blood supply within the posterior ciliary arteries deprives
the optic nerve tissue of oxygen and results in damage to part or
all of the optic nerve. This is a small "stroke" in the optic nerve.
It is painless. Patients may become aware of decreased vision or
difficulty seeing above or below the center of gaze. Loss of the
blood supply results in swelling of the optic disc, often associated
with hemorrhages. The hemorrhages and swelling will go away leading
to the development of a pale disc (optic atrophy). As the swelling
resolves, some of the axons will be permanently lost. We do know
that this happens more often in patients who are born with small
optic discs. These episodes may occur when there is a sudden drop
in blood pressure (following an operation or associated with blood
loss after an accident). Patients who smoke, or who have diabetes
or high blood pressure, may be at higher risk for AION.
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Learn about neuro-ophthalmic disorders |
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What are the symptoms
and tests for AION? |
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Will I get back my vision? |
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What is the treatment
for AION? |
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What is Cranial Nerve
Palsy? |
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How do I know that I
have cranial nerve palsy? |
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What tests are done for
nerve palsies? |
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What is the outcome
of nerve palsies? |
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What do I do about the
double vision? |
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What is Optic Neuritis? |
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What are the symptoms
of optic neuritis? |
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How does the ophthalmologist
know that I have optic neuritis? |
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What will happen to
the vision over time? |
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How is optic neuritis
treated? |
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Do I have (Multiple
Sclerosis) MS? |
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What is a Pituitary
Tumor? |
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How would I know if
I have a Pituitary tumor? |
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How is a pituitary
tumor detected? |
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How is a pituitary
tumor treated? |
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Will my vision get
better? |
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What is papilledema? |
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How will I know if
I have papilledema? |
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How is the cause for
papilledema detected? |
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How is papilledema
treated? |
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What is Thyroid Eye
Disease? |
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How will I know if
I have thyroid Eye Disease? |
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What tests are done
to detect thyroid eye disease? |
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What is the prognosis
of thyroid eye disease? |
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What is the treatment
for thyroid eye disease? |
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The steroids made my
eyes much more comfortable. Can't I just continue taking them? |
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What are the common
symptoms of neuro-ophthalmic diseases? |
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Symptoms that are more common in neuro-ophthalmic disease include
visual loss, visual disturbance, diplopia, unequal pupils and eyelid
and facial spasms.
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What are the common
types of neuro-ophthalmic diseases? |
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A few of the most common neuro-ophthalmic conditions
are optic neuritis, ischemic optic neuropathy, compressive optic neuropathy
(including pituitary tumors), papilledema, inflammatory and infectious
optic neuropathies, cerebrovascular disorder involving vision, tumors
involving vision, blephrospasm & hemifacial spasm, thyroid eye disease,
myasthenia gravis, ocular motor disorders (including cranial nerve
palsies), pupillary abnormalities, hereditary optic neuropathies and
patients who have unexplained visual loss. |
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to the top » |
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What is Ischemic
Optic Neuropathy (AION)? |
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This
is the most common cause of sudden decreased vision in patients
older than 40 years. We do not see with our eyes. We see with part
of our brain that is capable of interpreting visual signals sent
back from the eyes. This is located at the back of our head (the
occipital lobes). Information is transmitted from the eyes to the
brain via the optic nerves. These nerves are composed of the long
tube extensions (axons) of cells (ganglion cells) located within
the inner lining of the eye (the retina) that exit the back of the
eye at the optic disc. Each of the optic nerves receives blood supply
from branches of the ophthalmic artery within each eye socket. The
optic disc has a unique blood supply (the posterior ciliary arteries).
Loss of blood supply within the posterior ciliary arteries deprives
the optic nerve tissue of oxygen and results in damage to part or
all of the optic nerve. This is a small "stroke" in the optic nerve.
It is painless. Patients may become aware of decreased vision or
difficulty seeing above or below the center of gaze. Loss of the
blood supply results in swelling of the optic disc, often associated
with hemorrhages. The hemorrhages and swelling will go away leading
to the development of a pale disc (optic atrophy). As the swelling
resolves, some of the axons will be permanently lost. We do know
that this happens more often in patients who are born with small
optic discs. These episodes may occur when there is a sudden drop
in blood pressure (following an operation or associated with blood
loss after an accident). Patients who smoke, or who have diabetes
or high blood pressure, may be at higher risk for AION.
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What are the symptoms
and tests for AION? |
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Most patients with AION notice a sudden painless disturbance
in their vision. Because of the decreased optic nerve function, however,
the pupils may not react as well when light is directed into the affected
eye. Swinging a flashlight between the two eyes will then show an
"afferent pupillary defect." Visual field testing can identify the
area of optic nerve dysfunction. Blood pressure should be checked
and if there are any unusual features other blood studies may be done.
In elderly patients a blood test (sedimentation rate or c-reactive
protein) can help assess the risk of giant cell arteritis.
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Will I get back my
vision? |
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Most patients with ischemic optic neuropathy will have
relatively stable vision. A recent study suggests that 40% of patients
may expect to have some improvement in central vision. A very small
number of patients can have worsening of vision. In patients who have
had AION there is a possibility of this happening in the other eye.
Fortunately, this is not common (approximately 20% chance). Probably
the best news is that it is very rare for a second episode of ischemic
optic neuropathy to occur in the same eye. |
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What is the treatment
for AION? |
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Unfortunately, at this time there is no proven treatment
for patients with AION. It has been suggested that aspirin (regular
size or baby aspirin once a day) may decrease the chance of an episode
in the opposite eye. It is important that the blood pressure be followed
by your doctor (elevated pressure increases risk). On the other hand
it is important that there be no sudden drop in blood pressure (overly
aggressive treatment). Smoking should be stopped. |
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What is Cranial Nerve
Palsy? |
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This
is one of the most common causes of acute double vision in the older
population. It occurs more often in patients with diabetes and high
blood pressure. These will get better and essentially always resolve
without leaving any double vision. However, compressive masses,
infections, inflammation and injury can also cause cranial nerve
palsy. The eyes are moved by 6 extra-ocular muscles. Four of these
are rectus muscles (superior, inferior, medial, and lateral) that
attach to the front part of the eye (just behind the iris, the colored
portion of the eye). Two muscles (the superior and inferior oblique)
attach to the back of the eye. These 6 muscles receive their signals
from 3 cranial nerves (the IIIrd [oculomotor], IVth [trochlear],
and VIth [abducens]). These nerves originate in the brain stem (at
the base of the brain) and enter the eye socket through a fissure
in the bone of the skull behind the eye.
Pressure on or Interruption of the blood supply to one of the cranial
nerves causes it not to work. If there is interruption of signal
to the VIth nerve (which innervates the lateral rectus muscle) the
affected eye will not be able to move to the outside. The patient
will be aware of side-to-side double vision that will be worse (further
separation) when the patient looks towards the affected sideWhen
the IIIrd nerve (which goes to multiple muscles) is involved the
eye may be limited in up, down, and gaze toward the nose. The patient
is usually aware of combined vertical and side to side double vision
although there may be no double vision at all since the lid droops
and may block the second image. The nerves are not permanently injured
and over a period of 6 to 12 weeks the function should recover.
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How do I know that
I have cranial nerve palsy? |
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Dysfunction of one cranial nerve will produce weakness
in one or more muscles. If the eyes aren't moving together the patient
will experience blurred or double vision. If only the VIth nerve (innervating
the lateral rectus) is affected the double vision will be side to
side. If the IIIrd or IVth nerve is affected there will most commonly
be some vertical ("one on top of the other") double vision. This will
vary depending on the direction of gaze. |
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What tests are done
for nerve palsies? |
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While it is possible for multiple cranial nerve palsies
to have a microvascular cause all patients with more than a single
nerve palsy or with other neurologic findings must have a work up
(neurologic examination and imaging study) before the diagnosis is
accepted. If the cranial nerve palsy fails to resolve completely over
3 months additional work-up is indicated. All patients with presumed
microvascular cranial nerve palsies should have their blood pressure
and blood sugar checked to make sure they do not have diabetes or
hypertension. Additional work up such as CT or MRI scans or even an
angiogram to rule out an aneurysm may be necessary. |
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What is the outcome
of nerve palsies? |
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There is no known means of accelerating the natural
recovery characteristic of microvascular cranial nerve palsy. It is
important to make sure that blood pressure and blood sugar are adequately
controlled. The double vision may be treated acutely with patching
either eye. It is very important that patients report any new symptoms
or failure of the double vision to resolve. |
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What do I do about
the double vision? |
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Since we expect the double vision to clear up on its
own any treatment will hopefully be necessary for only a few weeks
or months. The easiest way to get rid of the double vision is to wear
a patch. Alternatively one lens of your glasses may be fogged using
frosted cellophane tape on the inside. |
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What is Optic Neuritis? |
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This is the most common cause of sudden visual loss
in a young patient. It is often associated with discomfort in or around
the eye, particularly with eye movement. The optic nerve fibers are
coated with myelin to help them conduct the electrical signals back
to your brain. In the most common form of optic neuritis, the optic
nerve has been attacked by the body's overactive immune systemA viral
infection that may have occurred years, or even decades, earlier may
have set the stage for an acute episode of optic neuritis. The inflammation
associated with optic neuritis can result in discomfort (particularly
with movement of the eye). |
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What are the symptoms
of optic neuritis? |
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The most common symptom of optic neuritis is sudden
decrease in vision. In mild cases, it may look like "the contrast
is turned down" or that colors appear "washed out." This may vary
and, not infrequently, will progress from the time it is first noticed.
The second most common symptom associated with optic neuritis is discomfort
in or around the eye often made worse by movement of the eye. |
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How does the ophthalmologist
know that I have optic neuritis? |
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A few patients with optic neuritis have swelling of
the optic disc (the beginning of the optic nerve) at the back of the
eye. This is referred to as papillitis. One sign usually detected
by your eye doctor is the presence of an afferent pupillary defect.
This indicates that there is less light being sensed by the affected
eye than the opposite eye. This is found by swinging a bright light
back and forth between your two eyes while observing how your pupil
reacts. |
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What will happen
to the vision over time? |
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The pain will go away, usually in a few days. The vision
problems will improve in the majority (92%) of patients. There are
rare patients who have continued progressive loss of vision. Frequently
colors look different or "washed out." Visual recovery usually takes
place over a period of weeks to months, although both earlier and
later improvement is possible. Optic neuritis can recur involving
the same eye, the other eye or other parts of the central nervous
system (brain and spinal cord). This may result in recurrent episodes
of decreased or loss of vision or problems with weakness, numbness
or other signs of brain involvement. An MRI scan can give us a rough
guess as to the likelihood of recurrence. Other testing techniques
include visual evoked potentials (a test where you are shown a checkerboard
of light and signals are recorded from electrodes on your scalp) that
can show a delay in conduction due to the damage to the myelin. |
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How is optic neuritis
treated? |
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Patients treated with oral (pills) steroids seem to
have a higher chance of recurrent episodes. Therefore, steroid pills
alone are not recommended as treatment. Patients who were treated
with intravenous (given by needle) steroids did have a slightly more
rapid recovery of their vision, although the final visual outcome
was not better than in those who were not treated. Thus, IV steroids
can be recommended for patients with severe involvement or involvement
of both eyes. |
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Do I have Multiple
Sclerosis (MS)? |
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Multiple
sclerosis (MS) is a disease process where the body's immune system
attacks multiple areas in multiple episodes. An episode of optic neuritis
may be the first indication of multiple sclerosis. With a single episode,
without other evidence of involvement, we usually cannot make the
diagnosis at that time. An MRI scan may be helpful in dividing those
patients into high and low risks. Finding evidence of other areas
of inflammation on MRI scanning suggests you may be at higher risk
for recurrent episodes and thus MS. |
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What is a Pituitary
Tumor? |
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Pituitary tumors are benign (non-cancerous) overgrowth of cells
that make up the pituitary gland (the master gland that regulates
other glands in the body). Tumors that grow large enough to produce
symptoms are less common but still are one of the most common tumors
occurring within the head. These tumors may often be present for
years without diagnosis or even symptoms.
The optic nerves coming from each eye meet just above the pituitary
gland in the optic chiasm. An abnormal growth of cells within the
pituitary gland may produce an excess of signal to the other endocrine
glands leading to overproduction of thyroid, cortisone, or sex hormones.
If the pituitary tumor extends out of the sella it may produce symptoms
due to compression of surrounding structures including the optic
nerves, chiasm, and cranial nerves in the cavernous sinus (controlling
eye movement and facial sensation).

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How would I know
if I have a Pituitary tumor? |
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Patients with pituitary tumors often have no symptoms
at all. Occasionally these tumors may produce headaches. Head pain
may be sudden and severe if there is a bleed into the tumor. Endocrine
symptoms are most common including alterations in menstruation, lactation
(milk from the breast), impotence, or loss of sex drive. Less commonly,
tumors may produce growth hormone causing gigantism in young patients
or enlargement of hands, feet, and facial features (acromegaly) in
older patients. The most common of these symptoms is due to compression
of the optic nerves or chiasm. Patients with involvement of one optic
nerve may notice dim, dark, or blurred vision. If the chiasm is affected,
vision will be lost off to the outside in both eyes. |
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How is a pituitary
tumor detected? |
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Pituitary tumors are usually suspected based on endocrine
changes but are confirmed with imaging studies. A CT scan can reveal
a pituitary tumor and may be especially sensitive to hemorrhage. MRI
scanning may be more sensitive and better define the relationship
of the tumor to the optic nerves and surrounding structures. Blood
studies to check pituitary function are essential. Other lesions around
the sella and pituitary may produce similar symptoms and may be confused
with a pituitary tumor. These include meningiomas, craniopharyngiomas,
germ cell tumors, and aneurysms. |
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How is a pituitary
tumor treated? |
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When
the patient is symptomatic, some form of treatment is usually indicated.
The most common approach is surgery usually performed through the
nose (or up under the lip). Occasionally (especially when the tumor
is larger or extending to the side) a surgical approach may be best
through a scalp incision. With large tumors, some tumor cells are
almost always left behind following surgery. Additional treatment
may be needed. This may include repeat surgery or radiation therapy.
It is very important that hormone levels be checked periodically and
replaced as necessary. It is also important to follow vision, visual
fields, and imaging studies (MRI scan) to make sure that there is
no re-growth of the tumor. These should probably be checked at least
once every one to two years. |
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Will my vision get
better? |
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In patients with visual loss due to compression of the
optic nerve or chiasm by a pituitary tumor the chance of visual improvement
is best predicted by the duration of the damage. The presence of changes
in the back of the eye (optic atrophy) may suggest long duration.
Surgery also can result in rapid relief of compression. |
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What is papilledema? |
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This is a condition in which high pressure inside your
head can cause problems with vision and headache. In papilledema cerebrospinal
fluid outflow is blocked. The pressure is transmitted to the back
of the eye via the optic nerve sheath (surrounding each of the optic
nerves) producing the swelling seen at the disc (papilledema). |
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How will I know
if I have papilledema? |
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The most common symptoms of high intracranial pressure
are headache and visual loss. The headache may be located anywhere;
frequently in the back of the neck. It is usually steady but may be
pounding. It may be very severe, and unlike migraine, it may awaken
the patient in the middle of the night. It also may worsen with bending
or stooping. The optic nerve swelling may eventually lead to loss
of vision seen as dimming, blurring or graying of vision. Patients
may be aware of difficulty seeing to the side. Frequently patients
notice visual disturbance lasting for a few seconds (often associated
with bending or stooping). High pressure may cause damage to the nerves
that move the eyes resulting in double vision. Patients may also be
aware of a rushing noise in their ears. Nausea and vomiting may occur
if the pressure is high and especially with a severe headache. Peripheral
vision (detected on visual field testing) is usually abnormal and
is one of the most important means of judging both the necessity for
and effectiveness of treatment. |
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How is the cause
for papilledema detected? |
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The
patient requires a MRI scan. The diagnosis also requires a spinal
tap. This will document elevated pressure inside your head and make
sure there are no other abnormalities in the CSF. The finding of abnormal
cells, inflammatory cells, or elevated protein may indicate a previous
infectious, inflammatory, or tumor related cause of elevated intracranial
pressure. In rare cases, an angiogram, where a catheter is placed
in the arteries and veins going to the head, may be necessary to exclude
an abnormality of the blood vessels. To determine whether there is
further damage to the optic nerve acuity and visual field testing
is necessary. |
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How is papilledema
treated? |
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Reduction in CSF production or increase in its outflow
may reduce intracranial pressure. Weight reduction programs (in overweight
patients) may be effective. Continuous drainage may be surgically
accomplished by placing a catheter between the spinal canal and the
abdomen (lumbo-peritoneal shunt). In patients with worsening visual
fields or decrease in central acuity, who do not have severe headaches,
an optic nerve sheath fenestration may protect the optic nerve from
further damage. A small hole or multiple slits are placed in the optic
nerve sheath just behind the eye using an operating microscope. |
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What is Thyroid Eye Disease? |
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This
is an autoimmune condition where your body's immune system is producing
factors that stimulate enlargement of the muscles that move the eye.
This can result in bulging of the eyes, retraction of the lids, double
vision, decreased vision, and ocular irritation. This is often associated
with abnormalities in thyroid gland function (either too much thyroid
(Graves' disease) or too little). The eye findings of thyroid orbitopathy
may be independent of treatment of your thyroid abnormalities and
may not resolve in spite of the fact that the thyroid is now "controlled."
These symptoms may be present even if your thyroid has no apparent
problems. |
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How will I know if I have thyroid Eye
Disease? |
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With muscle enlargement the globe (eyeball) is pushed forward leading
to the characteristic "stare." In addition, the muscles become stiff
and the upper lid tends to retract, pulling away from the colored
portion of the eye. The eyes may become red due to difficulty closing
as well as increased prominence of the blood vessels. This often
results in double vision with one image seen on top of the other.
If the muscles get large enough, they may press on the optic nerve
causing damage to the nerve.
This dysfunction within the optic nerve, which transmits information
from the eye to the brain, results in decreased vision. This, fortunately,
occurs only in about 5% of the patients with thyroid orbitopathy
and may be reversible if the pressure on the optic nerve is relieved.
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What tests are done to detect thyroid
eye disease? |
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This is detected by carefully checking vision, pupillary
reactivity, visual fields, and the appearance of the optic nerve head.
Most frequently this makes the thyroid gland over produce thyroid
hormone that in turn can lead to tremors, shakes, weight loss, rapid
heart beat or palpitations, nervousness, and sensitivity to heat.
Less commonly the attack on the thyroid gland leads to low thyroid
production or even normal thyroid levels. We may see antibodies in
your blood that can be identified as attacking thyroid tissue. |
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What is the prognosis of thyroid eye
disease? |
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Thyroid orbitopathy, like other autoimmune diseases,
often comes and goes on its own. There is frequently only one acute
inflammatory episode but unfortunately the effects may persist for
years or even permanently. Although there may be some reduction of
the prominence of the globe, eye movements will often not return to
normal. Lid position will also likely remain elevated, possibly with
persistent problems with closure. |
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What is the treatment for thyroid eye
disease? |
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In patients with mild involvement, irritation and foreign body
sensation may improve with artificial tears and the use of lubricating
ointment at night. If the lids are not closing completely, they
may be taped closed at night. With more severe corneal problems,
lid surgery to help partially close the lids or to raise the lower
lids may be necessary. In severe retraction of the upper or lower
lid, surgery to reduce the effects of the lid retractors, either
without or with spacer placement (such as a piece of tissue removed
from the roof of the mouth) can help the lids to close. Smoking
may worsen symptoms and should be discontinued.
There is no medicine that improves the ability of muscles to move
(and thus relieves double vision). Covering one eye immediately
relieves double vision. It may be possible to optically realign
eyes with the use of prisms either applied to glasses or ground
into the lens although this may not be effective until things stabilize.
When double vision cannot be corrected with prisms, eye muscle surgery
may be necessary. Often multiple muscle operations are necessary.
It is sometimes not possible to completely remove double vision,
but the goal is to remove double vision looking straight ahead and
in reading position, as these are the most important directions
of sight.
Fortunately, optic nerve problems resulting in decreased vision
are uncommon. When it occurs, treatment is aimed at shrinking the
muscles, usually by the use of high dose steroids (prednisone).
For those patients who will not tolerate steroids radiation therapy
may be of benefit. If the muscles cannot be made small enough to
relieve the compression of the optic nerve (resulting in decreased
visual acuity) then the orbit can be made larger. This is usually
done surgically by removing one or more of the bony walls of the
orbit. Since the optic nerve is usually compressed at the very back
of the orbit, removing the posterior medial wall of the orbit is
most critical. This may be done directly (through the soft tissues
or skin around the eye), through the sinus under the eye, or through
the nose. To further reduce the eye bulge the floor, lateral wall,
or even the roof of the orbit may be removed. One of the problems
with surgical decompression is that this often affects eye movements,
thus changing the pattern of double vision (if it already exists)
or potentially producing double vision in those patients who don't
have it before surgery.
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The steroids made my eyes much more
comfortable. Can't I just continue taking them? |
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Steroid therapy may be effective in halting the inflammatory
phase of thyroid orbitopathy and partially shrinking the muscle swelling.
Steroid side effects are very common with continued treatment. If
there are still problems with eye movements (double vision), exposure
problems (irritation and foreign body sensation), or decreased vision
then surgery should be considered. |
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For further information consult The Neuro-Ophthalmology Service at Shroff Eye Centre |
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