Hereditary Sensory Motor Neuropathy Questions

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Posted by ccfNeuro MD on June 04, 1997 at 14:51:25:

In Reply to: Hereditary Sensory Motor Neuropathy Questions posted by Maureen on May 31, 1997 at 09:49:35:

: I have an HSMN called HNPP (Hereditary neuropathy with liability to pressure palsies). It is a relatively rare demeliniatng neuropathy and the nerves are described as tomaculous. Entrapment is typical (carpal and cubital tunnel, thoracic outlet and the evquivalent syndromses in the legs). A distinguishing feature is numbess/weakness which lasts fro minutes to months caused byu situations which would not cause problems in a "normal" person. One needs to learn to avoid situations that cause symptoms. There is no treatment.
  I have had symptoms for more than 15 years and have led a fairly normal life up until a couple of years ago. There had been periods where symptoms werre better and worse. Therre was a very slow progression where it took less to cause symptoms.
  A few years ago, I had a traumatic injury to one nerve, causing mostly pain, and that seemed to set the neuopathy off into a fasirly rapid decline. I have pain, numbness and motor problems (foot drop, difficulty writing, dropping things, etc) , in all 4 limbs, which seems to vary in intensity day to day. It's getting harder to walk and climb stairs. And while having sensations of numbness, I also have an exaggerated response to  pin pricks, etc.
  Neurontin helps the pain. I need to pace activities throughtout my day to control both pain and fatigue. And I've done the psych counseling to help with the grieving anc acceptance issues.
  After that lengthy explanation, my questions:
  1. What causes those sharp stabbibg pains?
  2. I have periods where my hans and feet are cold and it takes a warm bath or electiric blanket to warm them up. Also ther may be mild swellling or hand redness. Is this part of the neuropathy?
  3. Is there an explanation for the sudden faster progression?
  4. Are autonomic symptoms expected in a HSMN? and if so could you give more details? I've read an earlier explanation about blood pressure changes. The doctors ask about bowel and bladder problems...
  Thanks for your help.
  Maureen
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HNPP (Hereditary Neuropathy with liability for Pressure Palsies) is  as you described.  There is weakness and numbness in the distribution of the affected nerve.  These palsies are almost always painless, however.  Most patients with HNPP also have a more generalized  peripheral neuropathy, which can vary widely in severity from person to person.  It affects the longest peripheral nerves first, which are usually the nerves supplying the feet.  Typical symptoms are numbness and tingling in the toes and feet, which can be painful.  Feet can also feel cold, which you describe.  As the neuropathy progresses over time,  it affects shorter nerves and the symptoms “travel” up the body. Some of the symptoms you are describing may be the result of this. Neuropathic pain (ie pain due to dysfunction of a nerve) can definitely be “sharp” and “stabbing”.  Its possible that the painful symptoms you are experiencing are due to peripheral neuropathy, although, usually with the peripheral neuropathy associated with HNNP pain is NOT a big component.  With regards to your last two questions; sudden worsening HAS been reported  to occur in some patients with Hereditary Sensory Motor Neuropathies,  such as after a viral illness or during pregnancy.  Autonomic symptoms are usually NOT seen in patients with HNNP.  The Cleveland Clinic has several neuromuscular specialists, if you are interested in a second opinion.  (For an appointment, call 216-444-2200)  The Charcot-Marie-Tooth Association may also be helpful. Their address is:  Crozer Mills Enterprise Center, 601 Upland Ave. Upland, PA 19015. Email: ***@****, Toll Free phone: (800) 606-CMTA.