Chiari Malformation Community
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12417243 tn?1425412164

Newly Diagnosed, facing imminent surgery with doubts.

Hi Everyone,

Firstly, I just wanted to thank the community here as a whole, specifically the Neuro/Vision focused societies. Your insight, knowledge, and shared experiences have been a huge source of support and strength for me the last few months. So really, thank you!

The basic rundown is I'm a 24 year-old, formerly healthy guy. For the past 2 1/2 years I've been experiencing progressive degradation in visual acuity of both eyes, hearing issues, and various other Neruological/Optical symptoms. Before this incident, I'd always had 20/15 vision, an optometrist once remarked that I could "fly planes with those eyes".

This all started while doing volunteer work in Japan (May 2013) I suddenly became very ill, with a high fever and terrible stomach pains. The 104 F degree (40 C) fever wouldn't break, so after 2 days in bed I got on a train and went to the ER. The doctors did blood work with results coming back indicative of a viral/bacterial infection. I was given some general anti-inflammatory medicine and told come back if things didn't subside within the week. After another ER visit and about three weeks of feeling awful, the symptoms seemed to finally subside. I then left to work with another organization in a nearby city, assuring myself it was just some weird viral thing that my body had fought/was fighting off. A week or so later I first began to notice alarming visual phenomena.

Artificial lighting (florescent store lights, neon signs, computer screens, tv's) all began to have this strange blurring/shimmering effect, as if my eyes kept focusing, de-focusing, then focusing again. A static-like haze began to set in across my entire field of vision, as if I could actually see the machinations of the brain/nerve/eye system doing it's thing when I obviously shouldn't be able to (I later came to understand this phenomena as "visual snow"). I began to have severe photophobia (sensitivity to light) and red, painful eyes. My balance/equilibrium would suddenly become totally altered, with my head feeling like it was "swimming", causing a sensation that I might pass out at any moment. My ears would begin ringing randomly, sometimes for minutes, other times for hours. Text on paper/computer screens/subway signs would bend and melt into itself, making it almost illegible unless I really strained to focus on it. Shooting nerve pain would radiate from my eyes and pierce into the center of my head then across my scalp. Finally, the headaches. Merciless aches where every blood vessel in my brain felt like it could burst at any second. I was terrified.

Within less than a month it had become noticeably worse. I could hardly get out of bed, and going out into sunlight was an excruciating and frustrating ordeal. I finally decided to see an ophthalmologist. After a series of tests which all came back normal, the eye doc said she could not find anything conclusively wrong, noting only a "low light accommodation level". She prescribed some b12 and atropine drops, and sent me home. I took the drops (which did nothing except give me crazy dreams due to the atropine) for two weeks while the symptoms persisted, worsening steadily. I went back, and the doctor again said my eyes were healthy, and that it's likely a neurological issue. She recommended I go home immediately, advice I reluctantly took (loved Japan, not so keen on my home country).

The two years since I came home had been the most frustrating, terrifying period of my life. I'd seen numerous doctors of varying specialties, gone through MRI's, CT scans, a lumbar puncture (I feel for anyone else who's had to do one of these), and countless eye exams-all coming back with a universal response of "inconclusive". Each doctor has said something in the manner of "Yes, there's definitely something wrong with you, but evidence sufficient to make a formal diagnosis is simply not there. Best of luck." It was  terribly disheartening. All the while the symptoms have gotten more & more intense, to the point now where I can't drive, have lost my job, and been forced to take 1 (soon to be 2) semesters off school.

Before the Chiari diagnosis I was diagnosed with persistent silent migraine with aura and possible occular migraine. I was systematically put through a trial with each other major medications used to treat this condition, with no success.

So, while being worked up by a new Neurologist who specialises in Multiple-Sclerosis and other neurological disorders, I came back positive on a brain & cervical spine MRI for Chiari 1 malformation/cerebral tonsillar ectopia. The distention is 3.68mm. Now, it's worth nothing that I had an MRI done a year & a half ago at the same testing center which showed no sign of this. The Neurologist refered me to a Neurosurgeon, who after a (very) brief consult, said surgery was my best & essentially only option to get alleviation from these debilitating symptoms. The surgery is scheduled a few days from now, and with the initial diagnosis being less than a month ago, this is all just happening really fast for me.

The Neurosurgeon claimed a "90%" success rate, saying risk of infection is very low and in general seemed very confident in performing the procedure. However, after doing some research about The Chiari Institute & also the Neurology department at John Hopkins (I'm in California, US), I'm unsure if I should head East and explore other options/get another opinion. In general I'm just a bit overwhelmed, and don't want to let myself rush into something as serious as surgery without taking logical steps. A measured reaction is important here, I keep trying to remind myself of this despite feeling like "I finally know what's wrong, forget pragmatism, let's get better NOW"

Anyways, sorry if this got long-winded. Anyone who reads even a bit of this, it means a lot. Thanks for being a source of courage in knowing I'm not alone.

2 Responses
620923 tn?1452915648

  Hi and welcome to the Chiari forum.

First let me address the MRI taken a yr prior and not showing Chiari....it is possible to take different angles or slices when doing a MRI that could not reflect what is going on, and by taking different slices with each MRI can make something appear to grown or even disappear when there have been no changes....let me explain it this way...imagine the area of the brain where the cerebral tonsils are as an inverted pear....cut into 3rds the 2 most outer slices are small....but the middle is larger....so if the slice is different it can change the outcome or appearance of the outcome of a MRI.

Chiari can remain dormant or un noticed for yrs as it is a congenital condition those with it have  adapted to the symptoms and accept them as normal as we do not know anything else as we have always felt a certain way....but, it is possible with stress, or an injury, or just a hard cough to cause  the symptoms to flare and we sit up and take notice...once we have  stress can add into it and the flare may last longer....Chiari symptoms can also cycle...come and go...so we chalk up symptoms to many things instead of being able to see they (symptoms) are all due to one thing....

There is a very good Dr in CO...if you do not want to come across the country...Dr Oro....check him out....and if you do TCI is rated the same as Dr O....they are tops in the field.....

What surgery is being done, as there are different types of decompression for Chiari and some do not have a prolonged success rate.

90% is higher then what I have heard any Chiari specialist offer....???

If in doubt listen to your gut....
5792451 tn?1390934690
I always support people who want a second opinion, but please keep in mind that the visual symptoms you're experiencing can be a symptom of intracranial hypertension, which can get very bad very fast.

I backed out of my Chiari surgery last summer because I didn't think my symptoms were worth brain surgery, I felt like I could manage headaches and POTS since I had been dealing with them quite a while. Then out of the blue at my annual eye exam my eye dr told me I had developed hemorrhagic papilledema, which means the pressure in my brain is so high that it's cutting off the blood supply to my eyes.

I was hospitalized shortly afterwards and I asked the neuro ophthalmologist how bad the condition of my eyes was, he said on a scale on 0-10 I was at 7.5. So I'm only 2.5 points away from going blind.

Please, keep in mind that your surgery needs to be done soon if you're already experiencing the amount of symptoms that you are. And don't be like me, because I regret that appointment that I turned my back on every day.
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