I wonder if anyone can advise? I have very complex neurological issues which don't seem to affect anyone else, even within the disorders I've already had diagnosed (autonomic dysfunction secondary to Ehlers Danlos Syndrome).

As a child I had a history of migraines, and lost my hearing in one ear during my teens. In my 20s I developed severe nerve pain in my ear canal/drum made worse by cold. Several MRI/CT scans failed to find the cause. The ear pain got better over about 10 years, but I was plagued by lots of odd symptoms.

4 years ago, I had a lot of dental work. A few days later my salivary glands packed up completely, and I couldn't swallow on one side of my throat. I also developed severe ulceration in my mouth/tongue. I had a lot of problems getting anyone to take my problems seriously. I eventually saw a rheumatologist for Sjogren's testing (negative). She prescribed pilocarpine for the dryness, but this triggered severe burning in my mouth and tongue swelling.

The symptoms did gradually improve for several months, but the following year I developed severe pain in my eyes, and periorbital oedema. None of the usual ophthalmological treatments worked, the eye hospital had no idea. Things gradually got worse, my muscles started hurting badly, then one day I woke up and found my face bright red and burning like Hell. That was nearly 3 years ago, and the burning hasn't remitted or responded to treatment to this day.

To cut a long story short, I eventually saw the leading autonomic neurologist in the UK who thought the skin condition was erythromelalgia, and that the autonomic symptoms were due to Ehlers Danlos Syndrome. Since then I've had autonomic testing that has confirmed autonomic dysfunction, including vagus nerve damage and I started medications to raise my BP. Midodrine I had to discontinue due to intense vasospasm (causing pain and numbness in my mouth/throat, as well as Raynaud's in the feet). I've just started pyridostigmine, which has had beneficial effects, but also seems to be worsening the oral paresthesias (the right side of my throat and larynx appeared to be paralysed for a while.

This week, I saw a head pain neurologist. He says the facial flushing/burning is definitely erythromelalgia. He also thinks I might have some additional genetic fault in the sensory nervous system causing these problems. He was very blunt, and said the only other case he'd treated had responded to nothing other than prolonged lidocaine infusion and oral mexiletine. Based on that, and the fact that none of the many, many drugs I've tried has made the least impact on it, he thinks it's a sodium channel problem. He said ideally he'd admit me to hospital and put me on lidocaine for 5 days, but can't do that for 2 years due to his long waiting list, and the fact I don't have private health insurance. In the interim he's put me on lamotrigine, which quite honestly scares me enough in it's own right.

I really don't know what to do. I have very poor tolerance for medication, I did try very high dose pregabalin but had to stop because of severe reflux unresponsive to medication, double vision and generalised muscle weakness. A the moment, I can't feel the inside of my mouth and throat, swallow properly, can only eat about 10 bland foods (as anything acidic or high in amines worsens the mouth burning/numbness). I don't know whether the pyridostigmine is worsening these problems, they do more or less correspond to the side effect you get with electrical vagus nerve stimulation. I should really talk to my autonomic neurologist, but I know all he will say is he doesn't know. No one else on the dysautonomia forum appears to have experienced these side effects from this drug.

I'm really terrified by this condition, no one actually knows what's going on. A clue might be that my family suffers hereditary tonic pupil, which has links with hereditary sensory neuropathies, although I don't suffer Adie's pupil myself, and no one else in the family has severe neurological issues like mine.

y-gwair,

It is great to have you come and be with us. You will fine a great number of dear friends to listen when you are in need of ears. Please, just let us know how we can help you out. You will find out we are supportive and relaxing place.

Click on the following forum, there will be someone to help you.

Peripheral Neuropathy:
http://neurotalk.psychcentral.com/forum20.html

Please keep us up to date on your situation. Again welcome, looking forward to seeing you around. My thoughts and prayers are with you.

Darlene

Hi the people here are great but no one seemed to have any information about Ehlers Danlos Syndrome. If you check out the Inspire Ehlers Danlos Syndrome Foundation website I think you might get some answers or advice where to get
answers .
Best of luck,
Redcat