Latest Diagnosis of Ehlers Danlos / Benign Hypermobility Syndrome

This is the latest in my journey to find out what is happening to me and to explain the symptoms i am experiencing.
I went to see my Pain managment consultant in September 2011. I explained to him that i was experiencing constant dizziness , brain fog, severe pains in my hips, ankles , neck , hands , wrists , knees etc. I told him that my head felt too heavy for my neck and that i had to keep resting my head in my hands and keep mostretching it out to give me some type of relief.
He ask me to do a series of tests called the Beighton test .Helps to determine the degree of joint hypermobility. Currently it is used as part of the Brighton Criteria. For a positive Beighton Score, 4 or more points out of nine are required (i.e. two elbows and two hypermobile knees):

1. More than 10º hyperextension of the elbows

2. Passively touch the forearm with the thumb, while flexing the wrist.

3. Passive extension of the fingers or a 90º or more extension of the fifth finger (Gorling’s sign). This is used as a “Screen Test”.

4. Knees hyperextension greater than or equal to 10º (genu-recurvatum)

5. Touching the floor with the palms of the hands when reaching down without bending the knees. This is possible as a result of the hypermobility of the hips, and not of the spine as it is commonly believed.

I scored a total of 7 points and he told me that he believed i was suffering with Benign Hypermobility Syndrome / Ehlers Danlos syndrome type III. He then made me a referal to see a Rheumatologist who specialises in these syndromes.
I attended the appointment with the Rheumatologist a couple of weeks ago where she asked me to do the Beighton test again, ( again i scored 7 points ) This time the Rheumatologist looked at scarring in various places on my body to see how they had healed ( papyraceous scarring ). She made comment of how my eye lids had drooped. She even tested how flexible my ears were.
She told me that i had BHS / EDS type III and that she would arrange for me to see a specialist Physiotherapist to help me with strengthening my muscles. She told me that it explains why i have cranial instability in my neck which explains the pains i am having in that area. I am now taking 400mg of Tramadol a day to help with the pain and 40mg baclofen before bed.
I am awaiting now to see a Cardiologist to see if he can test me for POTS as this may explain the dizziness. My Journey continues.

Major Criteria

· A Beighton score of 4/9 or greater (either currently or historically)

· Arthralgia for longer than 3 months in 4 or more joints

Minor Criteria

· A Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+)

· Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.

· Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.

· Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).

· Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactily [positive Steinberg/wrist signs]).

· Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.

· Eye signs: drooping eyelids or myopia or antimongoloid slant.

· Varicose veins or hernia or uterine/rectal prolapse.

The Joint Hypermobility Syndrome (JHS) is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.

JHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1986 and the Villefranche 1998 criteria respectively).



Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.

The journey Continues

Hi everyone, Just a quick post to let you know how things are progressing.
I had my appointment with a Neurologist at the QE earlier this month (May) and i explained that i was getting problems with my speech slurring, losing my sight in my left eye, the usual pains , headaches,fatigue and drop attacks, balance and dizziness.
He told me that it looked like i had brain damage either through surgery ( what !!!) or through a fall i had at work back in 2001 which i went to see him about when it happened ( i cant remember seeing him , in fact i cant remember seeing anyone let alone the accident ?). Anyway, he is sending me for a EEG to see if i ma experiencing Epileptic seizures . He will be writing to my Neurosurgeon with regards to my visit to him. I guess i will know more when i get to see my Neurosurgeon in June.

Friday just gone, i was pottering around in the garden,when i felt a pop in my head, i passed out with the pain it caused and when i came round, my left eye and mouth had drooped and my tongue on the left side had gone numb. I thought it may be one of my ridiculous headaches again, so i went for a lie down. All feeling came back later that afternoon and my headache had stopped with the help of copious amounts of pain killers. I went to see my GP on Monday (yesterday) and mentioned what had happened to him. He wasn't happy with the fact i left it to see him and said i should have gone to A & E as i may have had a bleed somewhere. He advised me to go and see a optician asap as he is concerned about my sight in the left eye deteriorating. ( today i have tried to get a appointment with about seven opticians, they all say the same, " sorry, we have no appointments for today) I have give up now as i really cannot be bothered anymore.). Its just a constant battle to be seen let alone be heard by anyone. I will leave it now until i see my Neurosurgeon or get another one of those attacks.

I will go to my appointment next month, knowing exactly what the outcome will be. ( "You have had the surgery, you are now cured, don't worry about that CSF leak you have , it wont cause a problem, its normal").Anyway rant over. I will post the outcome for you all to see next month.

Take care all, until next time.

Heart Problems

Has anybody had heart problems ?

Just after Christmas 2010, i had to lie on my lounge floor after i got crushing pains in my chest, heavy sweating, pains in my left arm and up into my neck. It seemed to pass after about 10 minuets. My wife returned home from work later that day and said that i didn't look very well, i told her what had happened and she called the NHS direct who then called for a ambulance. They took me to my local Hospital and after a blood test and ECG told me all was fine but suggested i could have Angina so they gave me a spray to use under my tongue.

A `couple of weeks went by and i was asked to attend the hospital to see a cardiologist who sent me for a stress test. The results were fine, or so i thought.

Again, a few weeks went by and i received a letter saying that they had looked at my results again and it showed i was getting insufficient blood supply to the heart muscle, i would be called for further tests to be made.

Last month i was called in to have a angiogram done, the results showed that i had no blocked arteries ( what a relief ).

I have now had a letter asking me to attend the hospital for another scan as they are still not happy.

I wait in anticipation.

Has anybody else had these problems following Chiari ( can it all be connected ) who knows.

pseudomeningocele

Hi everyone, just a little update to how things are with myself.
As most of you know, i am still having a lot of symptoms after decompression. Headaches, memory loss, vision problems, speech, pain, seizures, drop attacks, the list goes on.
I had a full MRI done in December 2010 and was told by my Syringomyelia nurse that my Neurosurgeon could do no more for me and that he was happy if i wanted to get a second opinion on the matter ? ( why would he say such a thing if he was so sure ). Anyway she basicaly told me that he could do no more for me surgicaly ( good news for me ).
I took the liberty of purchasing a copy of my MRI results . The reults are as follows.

Findings:

The small Pseudomeningocele at the craniovertebral junction is again demonstrated and appears unchanged since previous examination. The previously documented syrinx is no longer visible. The cord returns normal signal throughout the cervical, thoracic and lumbar region and the roots of the cauda equina appear unremarkable. A small posterior disc bulge is noted at L5/S1 level. There is however no evidence of neural compression.

That all sounded fine to me and i took it as gospel.

I recieved a letter from my NS in March , at the end it said " all i can do now is reasure Darren that he is not harbouring any serious or sinister disease process and i do not think that any further neurosurgical procedures are appropriate. If Darren wanted to meet with me and discuss matters face to face , then i would be happy to arrange a further appointment for him ".

I dont know if its me just feeling a bit touchy , but i found that bit of the letter very condecending.

I rang up his secretary and have booked a appointment for June and i will find out what can or cannot be done with this Pseudomeningocele, and could this be the cause of my problems.

Pseudomeningocele
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A pseudomeningocele is an abnormal collection of cerebrospinal fluid (CSF) that communicates with the CSF space around the brain or spinal cord. In contrast to a meningocele, in which the fluid is surrounded and confined by dura mater, in a pseudomeningocele, the fluid has no surrounding membrane but is contained in a cavity within the soft tissues.

Pseudomeningocele may result after brain surgery, spine surgery, or brachial plexus avulsion injury.

Treatment for pseudomeningocele is conservative or may involve neurosurgical repair.

What the hells wrong with you today.

What the Hells the matter with you today, Watch where your walking, Just think, What have you been doing all day.

Do these words sound familiar to any of you. These are the words some people here on the rare occasion but to me it is daily.
Some people find it hard to get to grips that maybe i don't feel well and that maybe i try my damn hardest to do what i can. I am not being lazy, i am not being clumsy , i am not ignoring you, i am not miserable, i am not being rude.

It is because maybe i am suffering and don't want to show it to others and i try to keep it to myself. I seem clumsy because i have trouble negotiating my steps and use all my efforts to keep my balance, I haven't done all the jobs you have asked because i have exhausted my self and i have needed to stop at intervals throughout the day to rest. Sorry i didn't think because my mind is not my own any longer and i feel vacant a lot throughout the day. I am not miserable, i am just trying my hardest to cope with the pain and maybe i am trying to keep it to myself as i don't want to burden you. I am not being rude, i just sometimes either feel like i don't want to socialise or sometimes i feel so drained that i just need to rest my body.

I know i am not alone with these comments. Sometimes loved ones try to ignore these facts as it helps them cope as well.
It is like a breath of fresh air when you get someone who really cares and is really interested in helping you all they can.
I had a couple that i know approach me the other day and took me to one side so others could not hear and ask me how i was as they said they could see how i had changed.
As usual, i said I am fine , don't worry about me. This is something i have become accustomed to as i don't really have anyone to listen to me and share my worries and concerns.
I felt deeply touched by this couple offering their help and a ear whenever i needed it. Whether it was genuine or not, the thought was there. So thank you ( you know who you are ).

That's where these blogs and forums help us all as we all share the same problems. That is why awareness is needed here in the UK as it people will understand the torment we go through daily.

If you was diagnosed with say Multiple Sclerosis, people would understand as it is widely recognised and there is help out there for those sufferers.

God bless you all and thanks for reading this.

Chiari Meet up

Hi everyone, just to let you know that i am organising a get together for all sufferers and partners/carers/family. I posted it on Chiari UK but this site seems to keep going down for some reason.
Anyway i have arranged for it to take place in Feb 2011 Dates either 19th Feb or 26th Feb which are both Saturdays. It will be in Birmingham in a chosen hotel just off Junction 7 of the M6 so its accessable to all. I have phoned a number of Hotels and have found some desent prices. Perhaps i could have your views on the dates so i can get it booked and the number of people who care to attend. please contact me via email daz.norton01@blueyonder.co.uk. I look forward to hearing off you. Meanwhile i will endevour to post it on the Chiari UK.

Chiari support forum down

Hi all, as some of you know the UK support forum is down at the moment and at a crucial time as well. I have contacted a few members and suggested we all use my forum Chiari care (follow the link above )for the time being so we can carry on our posts .
Please feel free to use it any time .

Darren