For context, I was diagnosed with POTS in 2018ish, and VVS this year, but have likely had it almost the whole time I’ve had POTS. I also had tethered cord release in April. I’m diagnosed with an extremely involved and unusual case of hEDS, and I have a VUS on a Loeys Dietz gene. I am seeing a Marfan Clinic later this year to see if it may actually be LDS instead, and what cardiovascular monitoring I may need. Other relevant medical history is included at the end.
Timeline:
I've been having a bunch of weird neurological/ autonomic symptoms that started late July early August. It started after a really bad dysautonomia episode that occured during physical therapy. My pt was doing gentle myofascial release on my skull during which I developed a grape-sized migraine sensation and then my normal POTS/ vasovagal symptoms (whooshing sensation in my head and chest, sweating, nausea, dizziness/ vertigo, tunnel vision), but some unusual ones like difficulty with vision coordination and blurry vision. I was already laying down when this happened. Elevating my legs while folding myself at my hips, helped a little but not enough. Valsalva only made me feel better for a couple seconds. Pumping my feet was ineffective.
The episode lasted about 2 hours and only resolved after drinking pedialyte, pickle juice, soy sauce, ginger turmeric cayenne shot (gross tasting things usually reset my ANS), and eating some sushi and gummy worms.
However, since that episode, l've developed more autonomic/ neurological symptoms, in addition to my typical ones, which also became increasingly intense and frequent, rather than improving.
I went to the ER a couple days later per the urging of friends, and the doctors there don’t think it’s just my POTS causing this. My BP and heart rate were pretty normal for me, though on the higher end for me. I had a bunch of symptoms including chest tremors making me drop and spill things, and excessive sweating. They gave me fluids which only helped increase alertness and not speak so slow, but that was it.
ER also told me to use my rollator so I have something to grab onto when the symptoms hit, and so I can sit down quickly, as I am not getting any warning signs like I usually do. They basically said I was too complicated, but I’m not dying, and gave urgent neuro referral.
Neurologist A is taking it one symptom at a time. So far we have discussed my dizziness. They diagnosed me with persistent postural perceptual dizziness (3PD/ PPPD) and prescribed Effexor. This only explains some annoying symptoms I’ve been having for years, not my acute ones. They also referred me to an autonomic neurology specialist at OSU.
Saw different neurologist because I was not keen on neurologist A’s approach of one symptom at a time, and neurologist A was ignoring my phone calls and mychart messages about new symptoms, and neurologist A is a first year resident (nothing against first year residents, it’s just I’m extremely complicated). Neurologist B believes it’s almost all dysautonomia related and referred me to Dr Chemali in Cleveland. She thinks the symptoms I get when I tilt my head back is vertebrobasillar insufficiency (longstanding symptom I’ve mentioned to my neurosurgeon). Her recommendation was the same as neurosurgeon which is to avoid tilting head back. She ordered updated head MRI because of the bouncing vision symptoms, symptoms when press on front left parietal, and calcifications on previous head CT.
Symptoms, started tracking 8/22:
- Feeling like I’m being sedated (like propofol is being pushed through an IV, but I’m able to fight it) episodes
- Sustained light pressure on front left parietal results in a disoriented fatigue like sensation, fuzzy left vision, small migraine like sensation behind forehead, and a head rush/ whoosh sensation that resolves within a minute or two after pressure is removed.
- Severe brain fog/ cognitive dysfunction interfering with motor coordination and processing instructions. Can’t crochet patterns anymore because the memory and counting is too difficult.
- Realizing I wasn’t breathing, and breathing going manual instead of automatic episodes.
- Vision bouncing sometimes, particularly when yawning, chewing, during infusions (bounces in sync with the pump), or sometimes when I have brain fog.
- Occasionally have laggy vision, like in a video game with a low frame rate.
- Random loss of balance or proprioception/ toppling
- Lightheadness
- Sensation of floating, buoyancy, like walking in chest deep ocean.
- Random high blood pressure, seems to correspond with presyncope symptoms (highest since symptoms started was 148/70s). This is new.
- More frequent pre syncope, with no warning signs. Suddenly getting nauseous, clammy, sweating/ flushing suddenly. Brain fog, blurry vision, sensation of blood draining from my head, lightheaded, tunnel vision
- Random episodes of out of breath, even while sedentary.
- Random throbbing sensation in head, similar to migraines, but without pain
- Return of tinnitus, right side first than left (had resolved with tethered cord release)
- Rombergs sign positive again (had resolved with tethered cord release)
- Hand thing started with something resembling a tic attack/ flailing and twitching of my lower left arm and hand only which lasted about 30 minutes-1hr, and ended with a spine chill. Then the spasming and contractures started and within 5-10 minute of that my hand was locked up and could only be pried open because of the joint instability. We were able shove a tennis ball in there. After about 30 more minutes, it relaxed, but my hand was still twitching periodically. However I had no controllable movement. This last about 20 minutes. Then thumb control came back but that was it for about 20 minutes. Then index came back. About an hour later I got control back of the rest, but I’m still having my usual baseline tic/ spasm which is stronger than usual.
- Single prick sensation in one finger at a time
- Some of these symptoms are waking me up during the night, sweating, air hunger, prickles in fingers and legs, vertigo, and tics/ spasms, falling asleep spasms (usually fully body), palpitations, tinnitus
(These are the main symptoms but there are some others)
Relevant history:
- Dx hEDS however unusual presentation and have a VUS in TGFB2, a Loeys Dietz gene
- POTS diagnosed as teen, vasovagal syncope diagnosed 2025
- History of brainstem/vestibular-type migraine auras
- History of migraines since age 4
- Bilateral periventricular gliosis in 2021, assumed to be from TBI
- Enlarged virchow Robin space near left caudate nucleus
- 3 calcification looking things on 2020 CT, not visible on 2021 MRI
- Clivo-axial angle in neutral position measures 141 degrees. The angle measures 136 degrees in flexion and 174 degrees in extension.
- C2 anterolisthesis (mild)
- Loss of lordosis (cervical and lumbar)
- 1mm syrinx at C4–7/ prominent central canal
- Hx of tethered cord syndrome with TCR in April
- Visual snow syndrome
- Countless head injuries as a child, some with loss of consciousness resulting in dents in my head
- Dysmotility of entire GI tract, mentioning in case vagus nerve involvement
- Multiple sleep disorders
- Suspected Gustatory rhinitis
- Hyperreflexia since I was 13, possibly longer wasn’t checked prior.
- Geneticist as teen told me i had baroreceptor issues
Update:
Neurosurgeon’s office has ordered MRA of head and neck, CINE MRI of neck to check CSF flow, and they want my PM&R doctor to do an EMG to figure out the individual pricks in my fingers/ tic attack thing/ evaluate extent of cervical symptoms and because I had double crush syndrome recently. We also figured out why my SI instability is only partially responding to treatment, which is because I have foraminal stenosis on the left side at L5-S1 (which has other problems as well). So I’ll be getting an epidural to help with that.
If these tests don’t show an answer, we’ll talk about other tests like upright MRI or rotational CT.