I was fortunate to be referred to a large tertiary center, which was Mayo Clinic in Rochester MN. My first appointments were scheduled with the neurology department. During this appointment, the neurologist who specializes in idiopathic intracranial hypertension was not convinced my eye issues were related to the results on the lumbar puncture and MRI; however, to ensure this he ordered a repeat lumbar puncture and a MRV (magnetic resonance venography, which is the same techniques as a MRI but the dye allows them to see the veins of the brain better). During this fist visit with neurology, he highly recommended seeing neuro-ophthalmology and he saw that the request from my Vegas physician was denied with no rationale so he re-requested for that department to see me. Furthermore, he tested my neurologic responses and I had no neurologic defects.
The next day, I had my MRV which was a similar experience to my previous two MRIs. And the following the day, I had the lumbar puncture preformed. This time during the lumbar puncture, they had me lie on my side in the fetal position while they took the fluid from my spine. Additionally, I was to continue to lie down for 20 - 30 minutes after completing to ensure that the spinal fluid redistributed. With these changes, I had no headache!
Meanwhile, through a handful of connections (old colleagues but mostly my brother-in-law who works for Mayo Clinic), I was finally able to get an appointment at the end of the week with neuro-ophthalmology department. Initially, it sounded like I would have to fly back a few weeks to months later to be seen.
After the MRV and lumbar puncture were completed, I saw the neurologist for my second appointment with him. My opening pressure during the lumbar puncture was at the upper limit of normal, but not elevated and there were no abnormalities on the cell counts. Interestingly, my MRV showed extensive signs of intracranial hypertension with nearly attenuated transverse venous sinus (nearly collapsed blood vessels on the back of my brain). So again, the results were incongruent.
Furthermore, my neurologist who is specialized in intracranial hypertension had no idea what this meant other than at the time, I did not have intracranial hypertension. He stated he thought about my case for an entire evening (and likely through the weekend), because it didn't fully make sense. He stated that I may be at risk for developing intracranial hypertension at sometime in my lifetime or perhaps I just had more spinal fluid volume causing the changes on MRI and MRV. Also, he was adamant that if they ever wanted to do neurosurgery for this, that I come back to see him at that time. To that end, I do need to get my eyes checked for a swollen optic nerves (papilledema) every 6 months to monitor for intracranial hypertension for the rest of my life since I have those changes on imaging.
Ultimately, my neurology appointments were helpful as they ruled out a condition that was questionable and I didn't need neurosurgery!!! On the other hand, it was disappointing to not know what was causing my vision loss.
Next up, I will describe my neuro-ophthalmology appointments at Mayo Clinic.
When evidence of intracranial hypertension was found on my MRI, my neuro-ophthalmologist ordered a stat lumbar puncture. With not driving, not knowing the date of the procedure and my husband out of town, I had a lot of anxiety how I was going to get to this procedure and back. To that end, outpatient stat means something very different than inpatient stat and it was ordered on Thursday and not performed until Monday when my husband was back so there was no reason to worry.
A lumbar puncture is also referred to as a spinal tap and a needle is inserted into your spinal fluid via your low back to test the fluid. This procedure was done by using an X-ray to find a good position. Unfortunately, I have scoliosis and needed to be an a relatively awkward position for them to get the needle in easily. Luckily, there were no issues once they found the correct position. Normally, you would wait 15 - 30 min after they removed the fluid for it to redistribute along your spinal cord and in your brain. Since this was an outpatient setting, they put the bandage on, gave me instructions on managing the injection site, and sent me on my way less than 5 minutes later.
I ended up having the worst headache of my life (and I have had viral meningitis and migraines in the past). For the first 2 days, I couldn't lift my head off of my pillow without my head pounding. The next day, I could sit up but no quick movements. Each day it slowly slowly got better, but it took me a complete week to be back to my baseline.
Around the same time as the lumbar puncture, I started having excessive exhaustion. It was persistent and some of the worst exhaustion I have experienced. One day after work, I slept 18 hours and only waking for a brief moment when my husband checked on me to ensure I was still alive. Most days, the exhaustion set in sometime during the midmorning (2 - 3 hours after waking) and often, I couldn't physically stay awake. I went from working out (high intensity interval training) three days a week or more to not being able to walk half a mile without a nap. This exhaustion continued for about a month and a half and nothing seemed to improve it. After some slow improvements and a change in meds, I began to improve a little each day. To this day, I still get overtly tired if I do too much computer work but not usually if I do anything physical.
My lumbar puncture showed I had elevated intracranial (brain) pressure and an extremely low glucose in my spinal fluid. With my neuro-ophthlamologist appointment 2 weeks after the lumbar puncture was completed and having my results, one of my other physicians prescribed a medication that can help with idiopathic intracranial hypertension, acetazolamide. This medication is a diuretic that can help in both intracranial hypertension and altitude sickness. Additionally, she referred me to Mayo Clinic neurology and ran a few more laboratory exams.
Once I saw my neuro-ophthlamalogist, my vision hadn't improved despite treating the potential intracranial hypertension for nearly 2 weeks. We discussed my results and although my brain pressure was elevated, it was considered indeterminate levels for intracranial hypertension. I had a pressure of 24 mmHg and 25 mmHg is diagnostic and 10-18 mmHg is considered normal. Also, it was unclear why my glucose was so low in my spinal fluid as it didn't follow a typical idiopathic intracranial hypertension picture. She began to doubt whether the intracranial hypertension was causing the vision issues since the pressure was not excessively high nor did the medication help. She had me stop the medication, which ended up getting restarted that weekend after my vision began to oscillate. Nonetheless, she wanted to refer me to a tertiary center which she added neuro-ophthalmology specialty to my Mayo Clinic visit. Typically, losing your vision due to intracranial hypertension is a neurologic emergency and neurosurgery should be conducted, but my picture was unclear and the risks may outweigh the benefits thus the referral was necessary.
More to come regarding my Mayo Clinic visit.
When there is sudden vision loss, the brain may be imaged. There are a number of different pathologies (causes) that they will be using imaging to rule out, but common ones include brain tumors, strokes, and multiple sclerosis. While a CT and MRI have similarities, each have advantages to be able to view different disease states. Depending on your underlying diseases and presentation, they may order one or both of these.
A CT of the brain (also called a CAT scan) uses special X-rays to get multiple views of the brain in order to get more information about the tissues and brain structure. During the CT, an X-ray beam moves in a circle around the body (or body part) to get many views. A CT can be done with or without an iodine-based contrast. CT contrast dye can be given orally and/or injected into a vein. Contrast dye can help certain structures be more evident; however, the decision if you receive contrast dye will be dependent on a number of factors including your renal function. The contrast dye is iodine-based so allergic reactions are rare but can occur.
To prepare for a CT of the brain, they will have you change into a hospital gown and remove all metal objects including piercings and jewelry. You will complete a screening tool to ensure that the CT is safe for you and there are no contraindications. If you are receiving IV contrast, they will place a peripheral (arm) IV in order to give you the contrast. You will lie on a table which will be moved into a circular machine, which is the CT machine. After you are positioned correctly in the machine, the technicians in the room will move behind a window to where the scanner controls are, but they will be able to speak to you via speakers. Once the exam begins, you will lie still for the duration of the exam and the machine will circle around you. Throughout the exam, there will be a number of whirling and clicking sounds which are normal. If they use IV contrast dye, they will either inject it at the beginning of the CT scan or midway through the exam after they have images without contrast. When it is injected, you may feel a sensation of flushing, nausea, a salty taste in your mouth, or even like you peed your pants. This will only be brief and will reside quickly. Typically, a CT of the brain takes about 15 - 30 minutes with most of this being the prep time and the actual exam only lasting around 5 minutes. Once the CT scan is complete, you will be removed from the machine and if you have an IV line, this will be removed.
A MRI of the brain uses a magnetic field and radio waves to get multiple views of the brain to get a 3D image of your brain. During a MRI, your body will be on a table that may move back and forth in the MRI machine which contains the magnets. A MRI can be done with or without IV contrast dye. This contrast dye is gadolinium which helps to visual blood vessels better; however, the decision if you receive contrast dye or not will depend on a few factors but primarily what structures they need to see. Since a MRI is a smaller space and is tubular, it is important to note if you are claustrophobic or not. If you are, they may give you a relaxing medication to help ease your anxiety during the exam.
To prepare for a MRI of the brain, they will have you change into a hospital gown and remove all metal objects including piercings and jewelry. You will complete a screening tool to ensure that the MRI is safe for you and there are no contraindications. If you are receiving IV contrast, they will place a peripheral (arm) IV in order to give you the contrast. You will lie on a table which will be moved into a tubular machine, which is the MRI machine. Since the sounds of the machine is loud, they may give you ear plugs or headphones to listen to music. After you are positioned correctly in the machine, the technicians in the room will move behind a window to where the scanner controls are, but they will be able to speak to you via speakers. Once the exam begins, you will lie still for the duration of the exam and the table may move back and forth a little to get images of different parts of the brain. Throughout the exam, there will be loud banging sounds which are normal. If they use IV contrast dye, they will either inject it at the beginning of the MRI scan or midway through the exam after they have images without contrast. When it is injected, you may feel a sensation of flushing, nausea, or a salty taste in your mouth. This will only be brief and will reside quickly. Typically, a MRI of the brain takes about 45 - 60 minutes with most of this being the prep time and the actual exam lasting around 30 - 45 minutes. Once the MRI scan is complete, you will be removed from the machine and if you have an IV line, this will be removed.
The optic nerve is a bundle of nerves that carry vision information from the retina to the brain thus optic nerve atrophy is a breakdown of that nerve. Ultimately, the retina can not send the impulses to the brain and this can lead to a loss of central, peripheral, and color vision and potentially blindness if the underlying condition is not addressed. Optic nerve atrophy is not a disease of in of itself, but it is a sign of a more serious condition.
Causes of Optic Nerve Atrophy
The causes of optic neuropathy are numerous. Causes include but are not limited to glaucoma, a stroke of optic nerve (anterior ischemic optic neuropathy), a tumor pressing on the optic nerve, inflammation of the nerve due to multiple sclerosis or other autoimmune conditions, infections, nutritional deficiencies, hereditary conditions, congenital defects, or medications and local radiation treatment.
Diagnosis of Optic Nerve Atrophy
Initially, your eyes will be dilated and inspected with an ophthalmoscope (in instrument) to examine the optic nerve. If optic nerve atrophy is present, the optic nerve will look pale due to a lack of blood flow to the area. Depending on what they expect the cause to be, different tests from there will be used. Often, due to the extensive differential diagnosis, a myriad of exams and laboratory tests will be conducted. This may include the aforementioned vision tests, a MRI of the brain, and blood tests may be considered depending on the likelihood of each of the potential causes.
There is no specific treatment for optic nerve atrophy since it is a sign of another disease. Once the cause is determined, that underlying condition will be addressed. Depending on the condition, vision may return back to normal or continue to progress. During this time, it is important to have regular vision exams conducted in order to monitor changes and if any sudden vision changes occur, to see the ophthalmologist as soon as possible.
During the second week of September (right after my first neuro-ophthalmology appointment), I was driving to work, which was only about a mile away, on a three lane road. I was in the middle lane and was paying extra attention due to my visual issues. I ended up slamming my brakes because I thought the car on my left was merging into my lane and was going to sideswipe my vehicle. Once I slowed to almost a stop, I noticed that the person was 100% in their lane. Luckily for me and everyone else, there was no one directly behind me.
What probably actually happened is they were coming up on the left and I was unable to see them approach. When I noticed them, they suddenly appeared from my blind spot thus they appeared to be moving to the right. Everyone was fortunate that day that I didn't harm anyone by suddenly braking on a normally busy road.
I am able to legally drive since my visual acuity is still good (multiple doctors have confirmed); however, the visual disturbances and blind spots make me hesitant to drive and are the reason since that day, I have chosen not to drive. Did I ever expect that at the age of 36 I would forgo my right to drive? Never. Was it an easy decision? Absolutely not; however, I was not willing to put others at risk due to my ego and desires.
Giving up the freedom to go where you want, need or to respond to situations is extremely difficult and aggravating. You can ask any elderly person who refuses to give up their license. Furthermore, if you have been to Las Vegas, you are probably aware that we are a completely car centric city. It is a sprawling metropolitan city with limited public transportation. Crossing the city using public transportation can take 2+ hours in one direction. Furthermore, we have limited bike lanes which are often shared-use with car traffic. With or without limited vision, biking for daily transportation is probably not the safest mode of transportation in Las Vegas.
In the first few months of not driving, I had moments of anger, despair, and frustration. When my husband didn't arrive when I thought he should, I would dissolve into anger instead of gratitude that he was willing to rearrange his schedule to help. To this day, 8 months later, I still struggle with this at times probably because I have always hated asking for help for any reason. Additionally, I have been driving since I was 14 years old (grew up in ND and it was legal at that time to get a license with special driving classes) and have always been on the go.
Will I ever fully accept this is my new normal? I don't know and the unknown can be scary. I renewed my drivers license so I could get the real ID and honestly, I will probably continue to renew it as long as I am able to pass the vision test just in case there is an emergency and I absolutely need to drive. Despite renewing my license, I rely on my husband, friends, family when they are in town, and Lyft or Uber when none of the aforementioned are available. Being reliant on others for daily transportation is a new experience that will likely continue to evolve.
After continued left peripheral blurriness, I was sent to one of the two neuro-ophthlamologists in Las Vegas. This doctor appointment occurs in two portions, which typically occur on two separate days. The first appointment consists of a number of visual tests and the second, the actual visit with the physician.
The vision testing included a typical vision acuity exam, color blindness exam, a depth perception exam, eye pressure test, visual field exam (Humphrey Visual Field - HCF), and an OCT (optical coherence test). Interestingly, during this appointment, there was a fire on the first floor of the building and we were evacuated. I was rescheduled later that week to complete my examinations.
During my first visit at the neuro-ophthalmologist, the majority of my initial examinations were normal at first glance; however, with testing in the future, it became evident that my visual field exam had early signs of an enlarged left blind spot. Important to note, that blind spots are normal and we have one on each eye; however, the other eye is able to see in that area so it does not cause issues. Additionally, my first OCT (which takes a picture of the retina) showed a decrease in the optic nerve thickness which was below normal at 65 microns (normal is 70-100) and significantly lower than my other eye which had a thickness of 104 microns.
During my visit with the physician, she listened carefully, took what I was experiencing seriously, and gave words of encouragement. If anyone is reading from Las Vegas or the surrounding area and would like a recommendation, please message me and I will forward you her office info. Due to the reduced optic nerve thickness, she ordered a ton of bloodwork to add to the bloodwork that I had during my hospitalization to help elucidate the cause of the optic nerve atrophy. Ultimately, they took 14 vials of blood and I was tested for the most common causes of optic nerve atrophy including: infectious, autoimmune, immune related, hematologic, metabolic, cancer, and vitamin deficiencies. As well as, a MRI of the eye orbit was ordered.
When I returned in two weeks, my eye exam showed an enlarged blind spot on the left, which with foresight was minimally evident at the first visit. For the most part the blood work was normal. I already knew that I had latent TB (tuberculosis) which was detected again (travel to India yearly), but additionally, I had a folic acid deficiency and some mild leukocytosis (indicative of inflammation) with high lymphocytes (antibodies) and eosinophils (allergy responders). Of note, latent TB just means that you have been exposed to the bacteria and are at risk for a future infection if your immune system is decreased.
The MRI of the orbit showed that I had tortuous optic nerves (zig zagged nerves) and fluid buildup around them. This indicated that the pressure in my brain (intracranial pressure) was likely too high so she ordered a stat lumbar puncture (spinal tap) in the outpatient setting. At this point in time, my physician was concerned that the TB was active in my brain causing the increased pressure and the optic nerve atrophy. The other possibility was idiopathic intracranial hypertension (also called pseudotumor cerebri), which is an increased pressure in the brain for an unknown reason but would account for the enlarged blind spot and the MRI results.
More to come on these tests, optic nerve atrophy, and the next steps.
Although ocular and visual migraines are used interchangeably, they are different entities. If the symptoms occurs in both eyes it is more likely a visual migraine; whereas if it occurs in one eye it is more likely an ocular migraine; however, if there is sudden new onset of vision loss, you should seek medical care.
Ocular or Retinal Migraine
An ocular migraine is a rare condition and is also called a retinal migraine. It is thought to be due to reduced blood flow or spasms of the blood vessels of the retina (back of the eye). This leads to temporary vision loss or blindness of one of the eyes. The person experiences a blind spot which can become enlarged during the episode. Typically, the symptoms resolve within an hour from onset. This can be pain-less or can occur with or be followed by a migraine. Complications may include irreversible vision loss and the exact incidence of irreversible vision loss is unknown.
Visual Migraine or Migraine Aura
This is a much more common and is considered a more harmless condition. It is more commonly called a migraine aura and is characterized by temporary visual loss that lasts less than 30 minutes. It is thought to occur due to abnormal electrical activity in the cortex of the brain. This typically affects both eyes unlike the ocular migraine. Symptoms vary much more and may include flickering blind spot, flashing lights, wavy or zigzagged ring of colored light, or a blind spot that moves across the vision. A migraine headache may occur shortly there after or no migraine can occur.
Treatment is highly individualized depending on frequency of migraines; however, those with either ocular or visual migraines are recommended to receive medications to reduce incidence of migraines (migraine prophylactic medications). These medications may include anti-seizure medications or antidepressants. Additionally, they are recommended to avoid potential triggers of migraines, which may inclue bright light, stress, hormonal changes, nitrates, fasting, and wine among many others.
Image from: https://www.medicalnewstoday.com/articles/314917
Most people are familiar with an ultrasound being utilized during pregnancy or for viewing abdominal disorders; however, you may not have heard of it being used to view the eye.
What is an ultrasound?
It is a machine that utilizes high frequency sound waves (also called sonography) to create images of the inside of the body. The reflections (or echoes) of the sounds waves create the picture. Typically, this would be performed at an ophthalmology office with a specialized ultrasound; however mine was performed at a hospital ER with an ultrasound that can be used on any body part.
How is an ultrasound performed?
You will be in a seated position and asked to close your eyes. Once your eyes are closed, they will place a gel on the top of your eyelids, which will be cold. From there they place the ultrasound wand (transducer) over your eyelid where the gel was placed and ask you to look in specific directions to view all areas of the eye.
There is another specialized eye ultrasound machine in which the eye is numbed with special anesthetic eyedrops and a small probe is placed against your eye to visualize.
What should I do to prepare for an eye ultrasound?
Nothing really. The procedure is non-invasive and only takes minutes if checking for a retinal detachment, but could be 15-30 min if viewing the whole eye. Just recognize the gel will be cool on your eyelid. If they use anesthetic eye drops, this may sting a little when getting administered and lead to extra eye dryness afterwards.
What can an eye ultrasound detect?
This imaging can detect quite a few different abnormalities and this by no means is comprehensive. An eye ultrasound is helpful to detect retinal detachments, cancer of the eye (retinoblastoma or melanoma), bleeding within the eye (vitreous hemorrhages), damage to the bones around the eye, foreign bodies in the eye, or significant swelling (inflammation). Additionally, when patients have cataracts it may be difficult for the back of the eye to be visualized normally due to the cloudiness of the eye so ultrasound may be used.
Image from: https://radiopaedia.org/cases/normal-eye-ultrasound
I have been wanting to write this blog since I found myself able to speak about my vision loss. As with many things in life, I set the project to the side for unknown reasons. This blog will follow my journey, discuss diagnostic exams for vision loss, and treatments that may be beneficial. Perhaps it can help just one person in their own vision loss journey or describe what someone you know may be going through.
On a Saturday in August, I attended a going-away party for a sweet friend at a coffee shop. Afterwards, I drove home and was chatting with my husband. All of a sudden, I couldn't focus my vision on anything. Objects were moving and blurry. It seemed as though everything was oscillating (moving up and down rapidly). I waited nearly 15 minutes before stating anything to my husband as I thought it would just go away. After letting him know, I laid down, rested my eyes, and decided to meditate because I figured it was stress even though I was having a great day which felt stress free. Nothing really changed but I convinced myself that I was okay and my vision wasn't improving because I was so focused on what I was seeing.
I had my own going-away since I had just completed my last day of full-time job as I transitioned to being an adjunct professor and part-time pharmacist. My husband asked if we should cancel, but I insisted we go. At my going-away party, I tried to act as though nothing was wrong; however, I couldn't read the menu (which is a huge problem when you have celiac disease and have to be extra cautious) and the whole world seemed to be moving. My husband helped secretly read the menu to me and assisted me when I wasn't quite sure how to do anything without my eyes. After we got home, we decided I should go to the ER to ensure I did not have a detached retina since intervention for that is extremely time sensitive.
At the hospital, they did not have an ophthalmologist available on a Saturday night and doubted that the one on call would come in on a weekend so they decided to do an eye ultrasound (more to discuss this in the future). The eye ultrasound showed no retinal detachment so they were moving forward with blood work, a CT scan of the brain, and a MRI of the brain. Due to the time of evening, they admitted me since the MRI would not be accomplished until 1am. Nonetheless, all the tests came back essentially normal and I was discharged the next day.
By the second day, the oscillating movement had minimized, but my left peripheral vision was extremely blurry. I got an "emergency" retinologist appointment for that coming Tuesday, which I had a few diagnostic exams completed with no obvious abnormalities. My retinologist thought that it could be an ocular migraine and told me if it didn't improve to come back in a few weeks.
Needless to say, it didn't improve and continued to progress each month, each week, and even each day from there. I will be writing soon to discuss more regarding the progressing of vision loss, diagnostic exams, and information about all of the potential diagnoses that were high probability at one time.