Recently diagnosed, facing surgery next week

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) 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 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 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", 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 noting 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 (decompression with duraplasty) 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 & 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 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 caution, 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.


Jason, I honestly don't know whats going on and don't have an opinion. What I would say is I would be highly suspicious of a neurosurgeon (any surgeon) who claims a sucess rate 3 times better than everybody else and schedules elective brain surgery with in a week. Now while your chiari is small there could be other factors. What is your csf flow rate, the results of your cine mri and is their a syrinx? It may be just me, but Im not sure I'd be letting anyone open my skull without a second opinion...... I understand your need everybodies need for answers and relief. But if you have real doubts do not even consider surgery until they are put to rest. And do not decide on desperation. You have time.

I would definitely have the csf flow rate tested before surgery.

Jason, my tonsils are only descended little over 6mm. The problem is that they are chubby and act like a clog around my brain stem. Also back of my head (posterior fossa) is too small to fit this kind of herniation. I Need surgery the kind that they first burn of my tonsils and they completely cut them out and extract them. Do you have something similar???
My csf flow is not obstructed but it's very tight there. I have up to 85% chance that surgery will help me.
I'm 35 and you r even younger than me. Cross your T's and dot your I's. I wouldn't trust that doctor!
I know you want to get better and you want the fix now. But slow down. This is the big deal and the surgery can mess you up even more if it's not done by a chiari specialist.