Eye ptosis severe thoracic neck arm pain and previous tethered cord release

[KarenLee2023]

Hi has anyone had a ptosis of one of their eyes related to chiari ? I had surgery for a tethered spinal cord that took 10 years to dx. When I turn  my head to right my left eye which has the ptosis opens up but to the left is worse. Also improves when i bend knees but worse when i stand tall. Started with a twitch 14 years ago then progressed to droopy eye. Is also helped by iopodine eyedrops and weirdly enough a steroid injection in my neck for stenosis or ice applied. Neg to MG.Have bad thoracic/neck and arm pain burning in nature Has all specialists stumped.  Caffeine and alcohol aggravate muscle pain and I have weakness attacks with low potassium and then severe spinal pain tremors nausea frequent urinatation slurred speech.  Feel like I am intoxicated. MRIs keep showing my spinal cord in different positions.  It's now back L4 despite surgery to release in 2009. I also have large arachnoid cyst in sacrum and small one in back of brain. Also has a genetic finding for ducheene muscular dystrophy both sons with same gene but ok so far. I had uncle with limb girdle muscular dystrophy. I have large muscles but don't do weights. Anyone out there relate to any of this? On my brain mri it looks very similar to a chiari but they haven't mentioned it. I feel like my cord has retethered but they say no yet it's back where it was prior surgery.  Regards

[Red_Star]

I have generalised MG. Not officially diagnosed and general doctor tested for achr antibodies which came back normal. Regardless I have MG 100%.   I record all my MG symptoms on video. Cogan’s eyelid twitch, enhanced ptosis (curtain sign), ice test, voice counting test etc.  My symptoms improved to some degree on a course of steroids (taken for another condition).

I really need to see a specialist to check for the other MG antibodies (musk, lrp-4) but I could be seronegative (low-affinity antibodies that cannot be detected on standard labs).

Anyway, I’m glad I read your post as you mentioned eye drops so I had a look online and found this recent study on MG and Apraclonidine (also known under the brand name Iopidine)…

“Apraclonidine may alleviate ptosis secondary to MG and may be an effective alternative treatment for this group of patients.”

Hmm interesting. The mechanism of action…

“Apraclonidine elevates the eyelid by activating alpha-2 receptors on Muller's muscle, an accessory eyelid elevator muscle.”

Agha M, Ismail H, Sawaya R, Salameh J. Efficacy of apraclonidine eye drops in treating ptosis secondary to myasthenia gravis: A pilot clinical trial. Muscle Nerve. 2023 Aug;68(2):206-210. doi: 10.1002/mus.27851. Epub 2023 May 31. PMID: 37259693.

[KarenLee2023]

Thats really interesting about the steroids. The Ophthalmologist thought that it may have been from the local anaesthetic used for the steroid injection that interacts with the muscle channels. Curious because I had some issues with different locals used at times. I can also press on certain spots on my face or spine that make the eye open. Its the bottom lid that contracts which seems to cause it all. When I had the injection in my neck the result really was astonishing. Though the pupil was bigger the eye was more open than the normal one. I also read about some eyedrops called "upneeq" that people use for ptosis. Have you any experience with these? There is a Pubmed article called " Chiari Type 1: A mimicker of Myasthenia Gravis" which I found interesting also.

[Red_Star]

MG is an autoimmune disease and steroids reduce autoimmune activity. I find fatigue, heat, stress and illness all worsen my symptoms. My face falls on one side too in my case; MG is also a stroke mimic.

You mentioned ice and I thought you had tested positive to the ice test (thought to be sensitive and specific for MG whereas it has no effect on ptosis from other causes) but re reading your post looks like you meant icing your neck. I can’t press any spots to make my eyes open (although that is a cool party trick lol) and haven’t tried upneeq.

[KarenLee2023]

Hi  
The ice I apply to my eye and it fixes it but Ive been tested 3 times for MG and its negative. I have a lot of muscle burning pain and they say MG is painless???. Cold makes me worse but also cant do hydrotherapy in a too warm a pool as it makes me weak. I also find illness sets things off. Weird

[selmaS]

Wondering if you have POTS as well as EDS it could explain the heat and Ice issues.....and possibly the pain that is not from your other DX.

[Red_Star]

MG can cause pain due to muscle weakness. If my neck is weak and leaning to the side I can have a neck ache. There is a video on YouTube: “Neurology - Topic 21 - Ice test” that has two tests, one for ocular MG and one for generalised MG.  I thought I would try out the arm test. What a mistake that was.  My arm burned for an hour after that test lol.  There is a quote in that video on a positive ice test...”there really is no need for any other test..we know it’s Myasthenia Gravis.” Definitely worth a watch!

This is some info from the Myasthenia Gravis website...

“These factors can make MG worse:
Fatigue, insufficient sleep
Stress, anxiety
Illness
Overexertion, repetitive motion
Pain
Sudden fear, extreme anger
Depression
Extreme temperatures (hot or cold weather, hot showers or baths, sunbathing, saunas, hot tubs)
Humidity
Sunlight or bright lights (affects eyes)
Hot foods or beverages (affects mouth and throat)
Some medications, including beta blockers, calcium channel blockers, and some antibiotics
Alcoholic beverages
Quinine or tonic water
Low potassium levels or low thyroid levels
Some chemicals, including some household cleaners, insecticides and pet flea sprays
Exposure to chemical lawn treatments

Infections and respiratory illnesses can produce increased weakness that lasts for a while after the illness is gone. The stress of surgery can make MG temporarily worse. The disease may intensify during certain times of a woman’s menstrual cycle.

Drugs to avoid
Commonly-used medications like ciprofloxacin or certain other antibiotics, beta-blockers like propranolol, calcium channel blockers, Botox, muscle relaxants, lithium, magnesium, verapamil and more, can worsen the symptoms of myasthenia gravis.”

[Red_Star]

Oops! The full YouTube video which includes the arm test is actually “Neurology - Topic 20 - Myasthenia gravis and Ptosis”.  That video I mentioned previously is just a short segment of this full video.

[selmaS]

Hi and welcome to the Chiari forum.
May I ask, have you had ALL related conditions ruled out ?
Next, Are you saying the MRI indicated your spine is tethered again? If so, that is very possible for it to retether...
Everyone is different in how we are affected and much depends on what other conditions related or non related you have and your pain levels.

What we all tend to have in common is similar symptoms, but then it can get specialized again depending on what else is going on.And in addition to symptoms is Drs not understanding how we feel or recognizing what is actually going on to what they feel or think is going on, for this reason it is very important to have a true Chiari specialist...they should rule out ALL related conditions as well as non related before offering surgery.
Do you know if you have a syrinx?

[KarenLee2023]

Hi
I have had dozens of scans and tests last month. They said that my cord wasnt tethered but cant understand how its now back at L4 when it was at L2 in 2018 and all my old issues are back. I am seeing my old surgeon Friday week to get his opinion on the scans as the last 6 MRIs all report it in different locations and I have all the same symptoms as before but now with the worsening of the ptosis that no one seems to have answers for. I've been tested for MG multiple times as ice also temporarily stops the ptosis but it continues to comes back neg. I had a cataract when I was 19 that has also stumped them all and was neg to myotonic dystrophy and a few others that symptoms matched with. One of the eye specialists took a video of my eye and took it to a conference in America but no one could shed any answers. I asked AI about chiari and eye ptosis and it said there was a connection. Also an article called ' Chiari Type 1 Malformation a mimicker of myasthenia Gravis" on a PubMed site. I dont really get a lot of headaches so that part doesnt fit with the Chiari though when I had the TSC surgery the surgeon thought the Ptosis was been caused by the brain being pulled down. Two months ago I was swimming 60 laps a day in the pool and started getting neck and shoulder symptoms till one of these weakness episodes happened and havent been able to get rid of this thoracic/neck arm pain since nor get back in the pool :(. I was just curious if anyone out there with Chiari also had eye issues like mine and similar symptoms. Ill let you know what surgeon says. I certainly wont be rushing in. I had lot complications with the TSC surgery and it took me a good 2 years to have any sort of normal gait. Regards

[selmaS]

I was scheduled for The tethered cord release surgery and my Drs decided to do the PFD for Chiari insteada and that was in '09. My recovery was slow, but I also have EDS and that is why  I am a slow healer.I do not have the eye issues you have but my sister who has not been DX'd with EDS also has similar eye issues as you and my dad had them as well and had surgery to lift his eyelids....
So EDS or having a syrinx and the cord to retether are all possibilities. Please do keep me posted on what you find out, prayers you get answers soon that help you.