Progression and Speed of Neuropathy

[MARY ANN]

I have what they think is Hereditary Neuropathy with Pressure Palsies or HNPP. But the DNA test was negative. I seem to have lots of the problems of the numb hands that lsat for weeks and I can't hold things when my hands are numb.

You talk about these neuropathies being progressive. Can you say more? I know progressive means it will gt worse, but like how much worse? How bad does this get? The neurologist just shrugs his shoulders when I ask this.

Also, when you say soemthing is slowly progressive, what does that mean? It takes months to get worse or what? I wnet from walking ok to leg braces that go up to my knee in about two years. This seems fast, not slow.

[Vincent Bertolino]

Dear Mary Ann:

You may want to refer your physician to Athena Diagnostics (800-394-4493)for a reprint, Schenone, et al., "Molecular basis of inherited neuropathies."  Current Opinion In Neurology 1999;12(5):603-616.  This reprint discusses the genetics of HNPP as an overview, and cites three references that may be helpful in providing more information about your HNPP variant.  Additionally, your doctor can get further information from Athena about PMP22 gene sequencing (since you are deletion negative), as well as be put in contact with some HNPP experts who study cases like yours.

You might also want to contact www.hnpp.org.  This is a new non-profit organization dedicated exclusively to HNPP.

[CCF Neuro[P] MD, RPS]

thanks for the comment.

CCF Neuro MD

[goodgal31]

phyllis,

Did your Dr mention anything about Multiple Myeloma?  Someone close to me also had bone pain, started in the hip, then the arm and a bone marrow biopsy detected myeloma.  The first thought was she had Lupus.  She was 56 yrs old.  I dont understand any of the techinical stuff you mentioned, but bone pain is usually the first sign. (usually in the hip area) I dont mean to scare you, and if you had this im sure your Dr would have known, also the person I knew who had it had off white and red blood counts and her platlets were off to.  

Im here also looking for help, in misery.  I hope you and everyone else on the board here are feeling better soon.  Ask your Dr about the above as I really dont mean to scare you,but if it were the case catching it early would really help.  Dont be afraid, im sure you will get an answer. take care.

[CCF Neuro[P] MD, RPS]

Dear Phyllis G:

Sorry to hear of all your troubles.  Usually, we watch and follow the monoclonal gammaopathy.  Hopefully it will not turn into malignant plasms cell dyscrasia.  This condition will lead to a neuropathy, usually sensory. This is what your symptoms sound like.  I would suggest either a rheumatologist or neurologist.

Sincerely,

CCF Neuro MD

[PhyllisG]

I pray that someone will read this and reply.I am a 50 year old female, post THR of right knee 1997.I have had several Drs.say that I have DJD.I just had a bone marrow biospy that revealed that I have monoclonal gammopathy IgG-Lambda>4% plasma cells with M-urine protein.I started having bone pain and polyarthralgia 20 months ago,thought I may have gotten Lymes. I have severe neck pain with swollen right lymph node.I can't even turn over in bed.My hands are swollen also.My Reumatologist says that I have a bone degeneration disease, OA.
I realize that I have osteoarthritis,but I can't get any relief.
I will be having a Mylegram next week,although I don't have great expectations.I have had positive ANA tests off and on and
have beeen told I have Lupus-Like Syndrome>Fibromyalgia>and I desperately need a diagnosis> I feel that my problems have just
not be defined and no on is willing to help.
I am not over- weight and have a great husband and child.My question is who,and what kind of Doctor do I need? Thanks for any comment>DESPERATE and TOO YOUNG

[CCF Neuro[P] MD, RPS]

Dear Mary Ann:

The B is just the second category or not the chromosome 17 deletion disease(the deletion is termed A).

CCF Neuro MD

[MARY ANN]

The EMGs on my Dad, brother and myself were "consistent" with HNPP. We all have carpal tunnel syndrome. Only Dad had the biopsy and his nerves were too far gone to tell for sure.

What does the "B" stand for in HNPPB? I am in contact with others who DO have a positive DNA test. Is the progression different for them? Some seem to be on this fast track too.

Thank you for your help.

[Concerned person]

I would like to as a question that is completely off subject.  I have unexplained sensory neuropathy along with MG.  I had sinus surgery to remove cysts and inflammed tissue last Weds.  I still have not received the feeling back in my front teeth and certain areas of my teeth on the top.  The worst thing that I noticed today is that when I smile, my smile is crooked.  It is down on the left side.  The rest of my face seems normal.  Could a nerve have been damage in the surgery process, if so, will it fix itself and get back to normal?  My mouth or lips are not numb.

Thank you.

[PATTY]

I also suffer from neuropathy, although mine is just a sensory neuropathy and I don't experience any numbness, just pain.  My neurologist has told me that it is a slowly progressing disease as well.  Does that mean that if my case becomes so severe that my hands and feet become numb, that I will not be able to walk or hold things?  Is that what eventually happens to people who have a purely sensory neuropathy when it gets worse?

Patty

[CCF Neuro[P] MD, RPS]

Dear Mary Ann:

I am not sure how to answer you.  I am assuming that the entity your speaking about is the condition linked to chromosome 17p, but you do not have the deletion, so you would be labeled HNPPB.  I then assume that your EMG was abnormal showing motor conduction block and slowing of the conduction velocity at one or more entrappment sites.  Did you have a sural nerve biopsy showing focal sausagelike thickenings of myelin called tomacula as well as segmental demyelination?

If I am assuming correctly, the reason why the neurologist shrugged his shoulders, is that the progression is variable.  It sounds like yours is a more rapid progression that what we usually see.  Many times we see a progression,then a slow down, then another progression, but others are just a steady progression with variable speed.  The advice we give is to make sure you do what is needed, avoid foot ulcers, watch what your doing to avoid injury,etc.  

I am sorry that your disease is progressing more rapidly than others.  Those patients without the deletion of the 17p area, we do not fully understand and that is the problem in trying to give you some time frame of progression.

Sincerely,

CCF Neuro MD