Category Archives: HNPP

New Clinical reviews – full text articles via pubmed

Standard

Just a quick note to post links for these two articles that have recently been published. Both are full text, which is good to see.

Clinical and neurophysiological features of the hereditary neuropathy with liability to pressure palsy due to the 17p11.2 deletion

This is the pubmed Abstract, to find the full article follow link in the top right of the pubmed page  or click here  ‘Full Text Links’ to go to scielo.br where the file is hosted.

The broad clinical spectrum of hereditary neuropathy with liability to pressure palsy (HNPP).

As above, follow link top right on the pubmed page or  click here to go to scielo.br for the full article

What A Year!

Standard

It’s been a long time since I last posted here. I get weary, writing isn’t one of my better skills, and typing can be a bit of a pain, and by pain I mean actual physical pain due to pressure on nerves in the hand, wrist, forearm and elbow, which then start jabbing me with electrical sparks and pins needles, sensation loss and finally some loss of strength. So why am I wasting precious time bellyaching about it… see what I mean… I get weary

Tennis Elbow.

Hmm. Is this really what is happening to my elbow and forearms. Good grief it’s painful and causes sudden loss of grip and twist strength. The physio that I was seeing noted that I had tennis elbow in both arms, at the time left was worse than right. It started in the 1st quarter of last year and got steadily worse. But I have since heard of a condition, which is related to tennis elbow, and indeed some ‘experts’ would say it’s all part and parcel of the same thing. Radial Tunnel Syndrome.

Radial Tunnel Syndrome. RTS (time for a break, because this is getting rather Ouchy)

It would seem that RTS is a repetitive strain injury to the radial nerve as it passes along and between the muscle and fascia of the forearm. The pain is felt further down the forearm than in Tennis elbow which is mostly a repetitive strain injury of the tendons which attach the forearm muscles to the elbow joint. (forgive my Noddy anatomical descriptions, I’m not that familiar with the terminology without looking it up).

There is a distinction in both signs and symptoms, and can co-exist.

A couple of links to better descriptions than I can give will help here

http://www.houstonmethodist.org/orthopedics/where-does-it-hurt/elbow/radial-tunnel-syndrome/

http://my.clevelandclinic.org/services/orthopaedics-rheumatology/diseases-conditions/hic-radial-tunnel-syndrome

http://livehealthy.chron.com/radial-nerve-entrapment-tennis-elbow-1135.html

I particularly like the last link as it gives a good description of the distinguishing signs and symptoms and the tests which help to differentiate between RTS and Tennis Elbow.

For me, I think it is at least 80% RTS and 20% Tennis elbow. It can be excruciatingly painful at night, but I’m fairly used to disturbed nights by now.

The difficulty now comes in trying to get a neurologist to discuss and understand this. If the past is anything to go by, I will fall at the ‘but HNPP only causes painless palsy’ hurdle, and hence any discussion and acknowledgement is likely to come to a swift end.

Dr. Jun Li – Research Projects

Standard

Apologies for such a long absence.

Several people on the HNPP facebook groups have been in touch with Dr. Jun Li, who is currently running a research project into PMP-22 and CMT, HNPP and other related neuropathies.

I’ll leave a link to his Research Lab pages, there are a lot of useful links from the page, including his research work, which may be of interest to some readers.

In my haste to post, I forgot the link

Dr. Li’s Lab

A new group on Facebook

Standard

A new group for HNPP in the UK has been started.

https://www.facebook.com/groups/hnppuk/

To quote from it’s tagline

To offer support and advice to suffer’s and families of HNPP and related Neuropathies

There are also several other National groups for HNPP on facebook, here’s a few that I know of

HNPP i Sverige

HNPP Finland

There are 2 well established groups for HNPP that have an international flavour,

https://www.facebook.com/groups/hnpphelp/

Hereditary Neuropathy and CMT

Group description for this last group is,

Hereditary Neuropathy with liability to Pressure Palsies.
A source for those of us with HNPP, or CMT (and family) to get in touch, discuss and compare symptoms.

 

NHS CHoices: New Information Page On HNPP

Standard

At last, thanks to the hard work and persistence from Mark of the HNPP HELP facebook group, NHS choices information page has an entry for HNPP.

This is a really good step forward. There is a comments section on the webpage which should allow people to enter their comments about the page and their own experiences of living with HNPP, hopefully this will promote further understanding of HNPP and provide the writers of the page further information from which to update the page as necessary.

So without further ado, here is the link (also in the blogroll and neuropathy links section menus to the right)

NHS Choices: HNPP Information

 

Something New – Allodynia

Standard

I haven’t written a “Day in the life” entry for a long time. But I’ve been prompted to do so due to a new development, which I assume is due to HNPP.

One of my first persistent symptoms of HNPP was due to Ulnar nerve compression, usually when doing desk or table work. It first started to be a noticeable when I was in my late teens. It is a fairly regular occurrence and is so easy to trigger by such minor pressure that often I don’t notice that it is occurring until it is too late and my little finger and half third finger plus the outer aspect of my hand and sometimes into my forearm has gone completely numb. As long as I don’t aggravate it, sensation will gradually return over several days to a week.

Over recent years I’ve noticed that if I rest on my elbows I will get a sudden electric shock like pain shoot down the outer side of my arm, it’s a good warning, so pain which serves a purpose.

Several weeks ago I had a sudden inexplicable intense burning pain right in the bony groove of my left elbow, it lasted only 15 seconds or so, but was so intense that it really got my attention. It occured again several times that day, and seemed to be triggered by the merest touch and stretching or tensing my upper arm muscles.

Over several days this seemed to gradually move up the outer side of my arm toward my tricep muscles. I thought it must be a muscular pain, a strain or pulled muscle or tendon. For a few days it seemed to be moving toward the lower outer side of my arm and into my axilla, and chest, but thankfully it has retreated back to my arm only. However, what has developed is that that small area of my upper arm has become so sensitive to touch and movement, even the slightest touch results in sudden piercing burning pain. Stretching seems to bring on an almost cramp like aspect to it, but it is the burning sensations which really grab my attention.

I cannot understand what has caused it, could it be to do with the repeated ulnar nerve compressions, but then why would the pain move upwards. It doesn’t seem to be muscular, it is unlike any muscle strain that I have had before. And the sensitivity to touch seems to suggest that it is nerve related, though it might be too early to assume that.

Anyway, it’s there, it’s not making my life a misery, though it does give me a chance to yelp every now and then.

So what is Allodynia?

Here’s a wikipedia definition (pinch of salt and all that, so don’t rely on wiki to get it right)

Allodynia    ……             is a pain due to a stimulus which does not normally provoke pain. Temperature or physical stimuli can provoke allodynia, which may feel like a burning sensation, and it often occurs after injury to a site. Allodynia is different from hyperalgesia, an extreme, exaggerated reaction to a stimulus which is normally painful.

And here’s the link to the rest of the wiki article,

Allodynia, a wikipedia definition

It will be interesting to see how it develops or improves, though at the moment I don’t feel too positive about it improving as my continuing problems with shoulder and upper arm palsies and ulnar nerve palsies, mean that it is bounded by two areas of nerve injury.  QI