So let's think about how we approach headache with vision disturbances, what's the overview?
How do we think about these patients?
They commonly present with some type of vision complaint
and those complaints can be a myriad of different ocular and visual pathology
and we need some schema, some algorithm for evaluating that.
Vision disturbances may not be universal and can happen early or late in the presentation.
So here's one algorithm for approaching patients with vision complaints in addition to their headache.
We can categorize those based on the eye of involvement,
and so the vision complaints may be in one eye and be monocular,
and that helps us to localize where those symptoms may be;
or in both eyes and be homonymous, the right side of the visual fields on both eyes,
the left side of the visual field on both eyes; or by temporal,
again the same visual field in both eye.
We can also categorize those with the type of vision involvement.
The part of vision that's involved may be the central vision
and we'll think about a macular problem or a problem with the eye,
the orbit, the ocular apparatus itself; or the peripheral fields, the fields outside of the macula.
And so, what are some of the symptoms of headaches with vision disturbances?
What's some - what are some of the ways patients may present with the visual disturbance?
The vision loss may be monocular and that points us to a problem that is prechiasmatic,
in front of the optic chiasm, and involving either the left or the right eye.
The patient in this case, presenting with transient monocular vision loss
is a typical pattern for monocular vision dysfunction vision loss that presents only in one eye.
And here we would think of amaurosis fugax,
and that is thromboembolism, a clot that develops in the carotid artery dislodges
and travels up the common carotid to the internal carotid
and one of the first branches of the internal carotid artery
is the ophthalmic branch, the ophthalmic artery.
Blood clots that travel through that course
and go down the ophthalmic artery can lodge
and cause monocular vision loss, loss of vision in one eye.
This is often sudden in onset and often wanes overtime
and may present with stuttering monocular vision loss
before others complete ischemia to the optic nerve head.
A presentation with amaurosis is a medical emergency
and those patients need to be evaluated urgently for the presence of a clot in the carotid artery.
So, transient monocular vision loss points us to look at the carotid artery for the pathology.
Vision loss may be a monocular altitudinal defect.
And we describe vision loss that is above the meridian or below the meridian,
as an altitudinal defect and here you see a inferior altitudinal defect.
This implies pathology to the central retinal artery.
That retinal artery comes out along the optic nerve and divides into a superior division
and an inferior division, and arthritis or a vasculitis,
a vasculopathy affecting the central retinal artery often affects just the superior
or just the inferior division and will present with an altitudinal defect
or other causes of optic neuropathy.
So, again, the presence of an altitudinal defect in the presence of headache
points us to looking for an arthritis which is something we would consider in this case.
Monocular vision loss with papilledema suggest possible anterior ischemic optic neuropathy
and may present with varying degrees of visual field loss.
And then visual field defects may also be seen in these typically arise from problems postchiasmatic,
after the chiasm, affecting the optic radiations as they travel through the temporal lobes
and the parietal lobes or the occipital cortex and occipital lobes themselves.
So the presence of the eye of involvement
and whether it's the central vision or the peripheral vision points us in the direction
of where we need to look for the pathology that's contributing to this patients headache.
So what are some of the causes of headaches with vision disturbances?
Well, the first is glaucoma.
Glaucoma is an abnormality, a problem with the - do with the - either the development of the aqueous humor,
the fluid that is in the eye, or reduction in the outflow of that aqueous humor.
If you have too much and you can't drain it out, there's too much pressure in the eye
and that's what glaucoma is, too much pressure.
Here we see a schematic of that occurring,
so normally you see the aqueous humor is able to flow out of the anterior chamber of the eye in the angle.
That angle is that critical area where the aqueous humor is able to flow out of the anterior chamber
and obstruction of the outflow results in increase pressure within the anterior chamber.
This is transferred back to the posterior chamber of the eye
and there's increase aqueous humor pressure in the eye and over time,
that will result in damage to the optic nerve and death of the retinal cell ganglion and vision loss.
That process, that glaucoma process, can develop slowly over time
and ultimately headache can be one of the initial symptoms,
as well as redness of the eye and other changes to the eye
and orbit that would tip us off to pathology of glaucoma.
The second cause of headaches with vision disturbances is giant cell arteritis.
This is an arteritis, it's a vasculopathy.
When we think about vasculopathies we categorize them as the type of blood vessel,
the size of the blood vessel that is affected.
There are small blood vessels, medium sizes blood vessels and large blood vessels,
and we also see small blood vessel vasculitides, medium size vasculitides, and large vessel vasculitides,
and giant cell arteritis falls in that middle category of middle to large vessel vasculitides.
Being a vasculitis we see inflammation within the blood vessels.
The temporal artery and the ophthalmic artery
as well as other arteries travelling to the eye and external aspect of the face,
off the external carotid artery are often affected.
So we see narrowing constriction ischemia to those arteries that leave from the external carotid artery
and that results in the patient's symptoms.
We see vision loss but it can also see jaw claudication as a result of ECA stenosis and vasoconstriction
and rarely, giant cell arteritis can cause vasoconstrictions to blood vessels in the brain
and causes cerebral vasculitis.
And here you can see the normal temporal artery within outer, middle, and inner membrane
and a lumen that's nice and open.
And we see in the setting of temporal arteritis, there's inflammation in the tunica intima,
that inner and middle aspect of the artery, resulting in narrowing and ischemia
which presents with pain, headache, vision loss, jaw claudication
and some of the symptoms that we see with giants cell arteritis.
The third condition that may present with headache
and vision disturbances are carotid dissection or thromboembolism.
Here we're looking at a lateral aspect of a patient's neck.
The white is the dye, that's the IV contrast that's travelling up in the carotid artery,
and we can see a small area of irregularity, a dark spot in that blood vessel and that's a thrombus.
It may even be a thrombus that's recently embolized leading to a clot
that's travelled up the carotid artery and could lodge in the ophthalmic artery
causing vision loss or vision disturbance and may present with amaurosis fugax,
and this is a medical emergency that must be evaluated and treated,
or it will present with a subsequent stroke and complete vision loss.
And then the last condition that I'd like for you to know
and associate with headache and visual disturbances
is called posterior reversible encephalopathy syndrome or PRES.
This is a condition of altered cerebral autoregulation,
so the brain isn't regulating blood flow and fluid flow well,
and the posterior circulation is particularly susceptible to this.
This may be induced by high blood pressure or sepsis or maybe seen in the postpartum period
and it results in swelling of the brain, swelling of the areas of the posterior circulation.
Here on the MRI, you see a T2-weighted MRI that's showing white areas,
hyperintensities on T2 that are seen in the bi-occipital regions sometimes in the cerebellum
and extending up into the parietal lobe.
Those imaging changes are posterior predominant, that's why it's called posterior.
They are readily reversible by fixing the inciting event, fixing the high blood pressure
by lowering the patient's blood pressure, treating the sepsis and treating infection,
or stopping an offending agent.
It presents with encephalopathy so patients are confused as well as headache and vision disturbances,
so it falls into this category of headache with vision disturbances.
Let's talk a little bit about giant cell arteritis because that's what's going on for the patient in our case
and I think it's really important for us to understand.
Giant cell arteritis is a vasculidity, so what presents with headache and vision loss,
it can affect many of the organ systems in the body and present with a range of symptoms.
And it's important to diagnose because it must be treated quickly,
otherwise patients can develop permanent vision loss.
Giant cell arteritis or GCA, is an immune-mediated inflammatory disease,
again, of those medium to large vessels, arteries, that are in and around the head.
It has a predilection for the vessels off the external carotid artery,
but can also cause internal carotid artery arteritis and present with a cerebral arteritis or vasculitis.
It affects about 15 in a 100,000 people aged 50 years or older.
So, we think about this and people who have headache and vision disturbances who are older than 50.
There is a female to male predominant, so females get this 4x as much as men.
We can see it in men and we could- should consider it,
but we think about that female association with this condition.
Smoking increases the risk by six-fold so female smokers over the age of 50,
are patients that we need to consider this in as well as others who may not fit that common criterion.
Median age of the disease onset is 75 years,
so it's really uncommon to see new onset headaches in elderly patients
or new vision disturbances in association with headaches in elderly patients
and we should think about giant cell arteritis in those patients.
And three or four patients have headache,
so this is a condition we think about in that bucket of headache differential.
Fatigue and malaise is seen in one to two patients because this is a systemic process
that can affect all blood vessels, medium and large-sized blood vessels.
Other symptoms include fever, neck pain, jaw pain, and weight loss.
And that neck pain or jaw pain can present with jaw claudication.
So, when patients eat, they get more pain and actually, eating can induce,
can incite the onset of headache which is very typical for this syndrome
and uncommon for other migraine or tension type or other headache syndromes.
This is diagnosed by blood test and we'll talk about the role of the ESR and CRP as well as biopsy.
And the artery we liked a biopsy is the temporal artery,
it runs right here superficially in front of the ear.
Unfortunately, sometimes the biopsy is negative.
This is a vasculitis, it only affects specific areas of the blood vessels
and it can skip one place on the vessel to the next,
so we typically do multiple biopsies to evaluate for this condition,
and a patient who presents with very classic features
who has a negative biopsy we would treat anyway.
Treatment is usually with steroid therapy and other immune suppressant agents
and that really needs to begin prominently because complication
include blindness, sometimes paralysis or heart attack if other organ systems are involved.
So how do we evaluate all of these conditions,
giant cell arteritis and other causes of headache and vision disturbances?
Well, if you're worried about glaucoma,
those patients need to be seen emergently by an ophthalmologist
to evaluate the type of glaucoma that it is and manage that increase pressure
behind the eye before there's damage to the retinal ganglion cells contributing to permanent vision loss,
so patient should be referred to ophthalmology emergently.
For giant cell arteritis we look for signs of increased inflammation within the body.
This is an arteritis, there's inflammatory processes going on in the blood vessels.
There's increase inflammatory markers in the blood, and we can test for those.
We look at the erythrocyte sedimentation rate and the C-reactive protein,
and we see that both of those are typically elevated in patients with giant cell arteritis.
Ultimately, the gold standard for diagnosing GCA is a temporal artery biopsy,
and here you can see a representative example of a temporal artery biopsy.
The blood vessel on the far left is obstructed for seeing reduced flow,
ischemia in that area is a result of small purple and immune
and inflammatory cells that are around that blood vessel resulting in fibrosis
and inflammation around this vessel, and that contributes to the patient's symptoms.
Again, we can see skip areas, so a negative temporal artery biopsy
in someone who has classic symptoms should not negate proceeding with initiation of treatment.
Carotid thromboembolism, blood clots on the carotid artery
that can embolize causing amaurosis fugax or headache
and monocular vision loss should be evaluated by CT angiography.
We're looking at the blood vessels to look for signs of a clot or obstruction.
Carotid ultrasound is also beneficial - ultrasound is a noninvasive test,
it can be used to evaluate the same blood vessels again,
looking for thrombosis or thromboembolism,
and then MRI of the brain with and without contrast is important for evaluating for stroke.
Blood clots which can distally embolize clots to the ophthalmic artery can also embolize to the brain
and we can look for other findings that indicate that proximal thrombus.
And then, PRES, posterior reversible encephalopathy syndrome.
We look at the characteristic imaging and MRI of the brain with and without contrast
is how we evaluate for that condition.
Again, those are patients who may have headache, can have visual field disturbances,
but also present with encephalopathy and that prominent confusion,
altered mental status, reduced alertness or level of arousal,
should tip us off to think PRES as opposed to the other three conditions
and we would perform MRI of the brain
and see the swelling, this increased T2 signal edema in the posterior regions often symmetrically.
Here, we can see in the bi-occipital regions,
though there are many exceptions to those characteristic rules for PRES.
And then what about treatment?
How do we treat each of these conditions that present with headache and visual disturbances?
Well, going back to our case, our patient with presumed giant cell arteritis,
the first step would be to initiate prednisone.
This is an arteritis, there is inflammation within the blood vessels
and we treat that anti-inflammatories and prednisone is our go to first line agent,
typically starting at 40-60 mg per day and tapering slowly
as we monitor the patient in consultation with ophthalmology.
For patients that present with prominent visual loss,
we may consider intravenous corticosteroids
and that would be methylprednisolone, 1000 mgs daily for 3-5 days for more severe cases.
We typically taper steroids slowly over weeks and start corticosteroids prior to temporal artery biopsy.
I said temporal artery biopsy is the gold standard for diagnosing this condition,
but patients can develop vision loss very quickly,
so a patient who has a typical clinical scenario, elevated ESR and CRP,
we would initiate steroids prior to referring to ophthalmology for the temporal artery biopsy,
so that we can maintain the patient's vision or rescue vision that may be affected.