Introduction, Photophobia/Hemidystonia/Cavernoma

[Beka]

Hi, my name is Beka.

2 years ago I was diagnosed with a cavernous angioma on the right side of my brain, frontal lobe, basal ganglia and on my optic nerve.
It has been a long journey for me and continues to be. Around eight years ago, I began to have random spasms in my left hand, starting with my small finger jetting out to the left and holding it's awkward position for a few seconds. Three years ago I got to the point where my left hand was unusable. It had become rigid whenever it had a spasm. Each finger contorted in strange ways. I went to a few doctors over the years, but my symptoms were so random in their timing that I had no visual example to give them.
I decided to seek out a hand surgeon for help and that is where my active journey began. The journey where I decided that my quality of life was important enough and I was suffering.
It was difficult, my son was 1 1/2 years old at the time. The 1st hand surgeon I saw noticed that I had neuropathy in my hands and we went on a detour to discover that I have Charcot Marie Tooth Disease (CMT1X), a genetic neuropathy that affects the nerves in my feet and hands. It explained some of my symptoms, but not the spasms on my left hand, which was starting to affect my left foot too.
During that time, I had two hand surgeries, one after the other. My surgeon had informed me that my tendons on my knuckles had been shifted off, locking my hand in between those same knuckles. Some sections needed to be tightened, some loosened and in the case of my thumb, shortened altogether. The spasms had such great tone that 2 months after the 1st surgery all the work that had been done on my hand had to be done again.
My surgeon and his hand therapy staff had suspected that there was something else going on. They thought it was dystonia and some kind of brain issue, not just CMT.
I sought out help from my CMT neurosurgeon and after being sent to the neurology movement department, I got my first MRI. That's when they found my cavernoma.
I had a laser ablation surgery in August. My surgeons were cautious about the deep seated nature of my mass. They thought the laser ablation surgery would be less risky than a craniotomy, so that's what they recommended. They reminded me that there was no way to know for sure if the cavernoma was causing the spasms. By that point I was getting desperate, my right hand had started spasming just like the left. I couldn't imagine a life where both my hands would be unusable. They had no idea why it was happening, doubting that it was related, even though the symptoms were similar.
Recovery for the first two weeks was amazing! No spasms! But they came back. I got lucky though, I had a few months there where my hand was calmer than it had ever been. With that break, I started seeing a pattern. I had begun to notice that my spasms were being triggered by natural shifting light, LED lights and bright reflective surfaces. At my 6 month post op/MRI appointment I showed up with red tinted, glare resistant glasses, telling my neurosurgeon how much better I felt in the dark. The results from the MRI showed that there was only a 4mm decrease in the size of my cavernoma, which was 18mm to begin with, not what my surgeon was hoping for.
Those first two weeks, post surgery, I got plenty of rest in a dark room, now I wonder if it was the lack of light and not decrease in my cavernoma's size which had improved things.
Now, fast forward to today. I am so sensitive to light that it's hard to grocery shop without having at least a dozen spasms. I have increased spasms in our bathrooms with all of the reflective surfaces and same goes for the kitchen. My husband got major points when he switched out all our LED lights for incandescent and I now wear those red tinted glasses with polarized sunglasses on top and sunhat as well.
I had my third hand surgery last month and the spasms have not helped in the healing process. Though, now that I know the trigger, I have the ability to calm them down. It's a glass half full feeling for me.
I scheduled a functional eye exam at a facility I've chosen to help understand what is going on. My neurosurgeon has referred me to a Neurology Ophthalmologist in the same hospital and my eye doctor has referred me to the best eye doctor in Washington state. I need answers and I'm thinking it might not be bad to get a second opinion as to what to do next, neurosurgery-wise and eye-wise.

It all seems like a guessing game. My world is feeling smaller and smaller as I go. My husband is concerned and my 5 year old son is resilient, but still grasps everything that's going on. I'm an artist, who can't work without spasms. it's all too much.

Do any of you have any advice? Have you seen or heard of anyone like me? Any recommendations for Neurologists? I'm seeing a team at UW Medical Center.

[Elizabeth]

I responded to your other post but this one strikes me differently...have you been evaluated for seizures? Does anyone think the spasms are seizures? Have you tried seizure medication? My eeg didn't show seizures but abnormal waves...I was having seizures weird ones. I took keppra and they didn't stop but they reduced greatly. I had surgery and they went away, thats not always the case and they told me mine wouldn't go away but I was lucky. Get checked for seizures for sure.

[Beka]

Thanks Elizabeth, your message was so helpful.
I thought of seizures too early on, but dismissed them when they put my symptoms down as dystonia.
I just set up an appointment to talk to my neurology movement dr to see about getting a full work up. More information is needed.
Looking online I came across a form of epilepsy called Focal photosensitive epilepsy. It is when you have seizures caused by flashing or flickering light. I'm curios to see if that's similar to what's going on with me.
I chatted with another angioma alliance poster and she suggested I get my vitamin d3 levels checked. Maybe I'm deficient. I realized that my symptoms got worse when I took a break from my multivitamin that has 400 iu per dosage. Maybe it'll calm things down for me if I have the correct dosage for me?

Off on another adventure!

[Elena]

Hi Beka, I was diagnosed with a cavernoma pontine hemorrhage just about a year ago. I had all the brainstem symptom including dizziness, facial droop and left side weakness, nausea, severe headache. All started to resolve and by 2 months, but I started having funny spasms in my left hand and left foot. Over the course of a month, these spasms lasting about 30 seconds or so started taking over my whole left side, including face, arms and legs and became severe. Eventually, I made it to a movement disorder neurologist who also thinks it is a hemidystonia. Medications have been challenging to tolerate but I have had a lot of success with acetazolamide. My triggers are also light related and worse with fatigue, hunger or when I stop exercising. I do seem to feel worse with the cold and dark days but it has been a long winter, and I will see if the sunny weather will help my symptoms. The medication has helped me go back to working a little and has helped me to be able to care for and keep up with my kids and life. Good luck!

[Beka]

Elena,

Nice to meet you! I wasn't sure that I'd find someone with similar symptoms. While it is refreshing to not be alone in this strange brain journey, I hope you get some peace in all of this. It sounds like you've been able to take back your life a little bit, with your kids and work. I hope to get there too . I'm glad that the medication you're on is working for you!
Some differences between us are that my symptoms get worse when I excercise, worse in the sunlight (sun coming throught the trees, shifting light, sun relecting off of cars windshields on the road), I feel better when it's a cloudy day (something I'd never thought I'd say, given I live in Washington.). It does get worse with hunger and fatigue, for sure. If I'm too tired, the sound of a plane flying by or the heater turning on, will send me right into a spasm. Also, the sensation of touch will send me into spasm if I haven't had enough rest. My little boy, getting all wiggly next to me and pushing into me . . . automatic spasm.
My spasms have kept to my hand and have almost stopped in my foot since brain surgery and since figuring out the light connection. I was starting to have spasms in my right hand, but they seemed to have died down. I can't imagine what it must be like to have them over the entire left side of your body. Sorry you had to go through that and still are.
I see my movement disorder doc at the end of next month. They had me on caverdopa/levodopa for a while, but it wasn't really helping. We'll see what they say, seizure or spasm. Good luck to you too!
All the best,
Beka

[Beka]

I just got my Vitamin D levels checked and I am very low. My GP has put me on a 12 week regimen of 50,000 ui (once a week). I am supposed to go to 2,000 ui/day indefinitely from then on. Will see if this helps!

[Elizabeth]

Low hit D is a risk factor so its good u got checked and are supplementing.

[Beka]

After 2 doses of the 50,000 iu vitamin D pills my doctor prescribed me, my photosensitivity is almost gone and my spasms have decreased!
Could it be this simple?
I will be sure to post an update around the 60 day mark, that's when my levels should be consitant!

[Elizabeth]

Great news!!! Congratulations... Update us later please! I hope its that easy!

[Beka]

Thanks Elizabeth, I'll be sure to!