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Re: HNPP/CMT ?

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Becky,

Lamar here,

That is correct. The symptoms of HNPP can improve with proper treatment.

About the only thing that I can think of that CMT has similar is that

carpal tunnel. Sometimes it can be helped. At the same time the HNPP is

still there just as our CMT is still there.

----- Original Message -----

From: Maxwell

Sent: Wednesday, March 07, 2001 05:01 PM

Subject: [] HNPP/CMT ?

I am still confused over these two. And now the most confusing

thing is what I copied and placed below. I have never heard of any person

with CMT having a full recovery, have any of you? What we have with CMT,

we have it for good, when it hits it stays. In HNPP, according to this,

people with it recover and if they are careful to not cause compression of

nerves as in crossing legs, leaning on elbows, they won't have the pressure

palsies at all. You'd have to read the whole site to understand it all,

then hope your luckier then I was in understanding it! LOL~>Becky M.

http://www.geneclinics.org/profiles/hnpp/details.html

Author: D Bird, MD; University of Washington

Last revision: 15 June 2000

Summary

Disease characteristics. HNPP is a disorder of peripheral nerves in which

individuals are predisposed to repeated pressure neuropathies such as carpal

tunnel syndrome and peroneal palsy with foot drop. Recovery from acute

neuropathy is often complete; when recovery is not complete, the resulting

disability is usually mild. Some affected persons also have signs of a mild

to moderate peripheral neuropathy.

Management

No specific treatment for the underlying genetic or biochemical defect

exists and no special diet or vitamin regimen is known to alter the natural

course of HNPP. Risk factors for pressure palsies, and thus activities to

avoid, include prolonged sitting with legs crossed, occupations requiring

repetitive movements of the wrist, prolonged leaning on elbows, and rapid

weight loss [Cruz-ez et al 1997, 2000].

Many patients have slow but complete recovery from the acute neuropathy

episode. Those with incomplete recovery usually retain good function for

activities of daily living. Transient bracing, such as with a wrist splint

or ankle-foot-orthosis (AFO), may be useful. Some persons with residual foot

drop may permanently use an AFO. Controversy exists as to whether surgical

decompression of nerves is of benefit. Because spontaneous recovery is

common and because no systematic-controlled study of surgical intervention

has been done, this decision must be made on an individual basis, taking

into consideration knowledge of the natural history of the disease. There is

a developing consensus that surgical repair of carpel tunnel syndrome in

these patients is of little benefit and transposition of the ulnar nerve at

the elbow may actually produce poor results.

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Hi Becky,

You are not the only confused one here. Even some of the neuro-docs have

blinders on - then again, we are gaining knowledge of CMT disorders at an

increasing rate lately.

I have HNPP as clearly demonstrated by blood DNA test at Athena Labs. I also

have foot deformities, various atrophied muscles, and pain typical of many

CMT1A people. I even have swallowing and breathing problems which are

increasingly being appreciated as associated with CMT deterioration. Yes, I

have pressure palsies as well and they do go away, but, they are also more

susceptible than " normal " people. In addition, I have numbness to pain in

the periphial areas, balance problems, and inability to know where my feet

are unless I'm looking at them.

So, my conclusion is that the notion HNPP is limited to episodes of pressure

palsies is no longer valid and certainly not up to date. Read the Quest

article in the current issue. It does bring one up to date on what HNPP is -

which is just another genetic mutation in the same area that produces CMT or

any other generalized term that describes nerve deterioration caused by

genetic deviations primarily noticed by periphial effects on nerves and

muscle atrophy. We don't know all of the phenotype yet for CMT, but many of

us now believe that further study is indicating effects in some of the

semi-autonomus muscles such as swallowing and diaphragm. - EdM

-----Original Message-----

From: Maxwell <rmax@...>

< >

Date: Wednesday, March 07, 2001 6:01 PM

Subject: [] HNPP/CMT ?

> I am still confused over these two. And now the most confusing

thing is what I copied and placed below. I have never heard of any person

with CMT having a full recovery, have any of you? What we have with CMT,

we have it for good, when it hits it stays. In HNPP, according to this,

people with it recover and if they are careful to not cause compression of

nerves as in crossing legs, leaning on elbows, they won't have the pressure

palsies at all. You'd have to read the whole site to understand it all,

then hope your luckier then I was in understanding it! LOL~>Becky M.

>

>

>http://www.geneclinics.org/profiles/hnpp/details.html

>

> Author: D Bird, MD; University of Washington

>

>Last revision: 15 June 2000

>Summary

>Disease characteristics. HNPP is a disorder of peripheral nerves in which

individuals are predisposed to repeated pressure neuropathies such as carpal

tunnel syndrome and peroneal palsy with foot drop. Recovery from acute

neuropathy is often complete; when recovery is not complete, the resulting

disability is usually mild. Some affected persons also have signs of a mild

to moderate peripheral neuropathy.

>

>

> Management

>No specific treatment for the underlying genetic or biochemical defect

exists and no special diet or vitamin regimen is known to alter the natural

course of HNPP. Risk factors for pressure palsies, and thus activities to

avoid, include prolonged sitting with legs crossed, occupations requiring

repetitive movements of the wrist, prolonged leaning on elbows, and rapid

weight loss [Cruz-ez et al 1997, 2000].

>

>Many patients have slow but complete recovery from the acute neuropathy

episode. Those with incomplete recovery usually retain good function for

activities of daily living. Transient bracing, such as with a wrist splint

or ankle-foot-orthosis (AFO), may be useful. Some persons with residual foot

drop may permanently use an AFO. Controversy exists as to whether surgical

decompression of nerves is of benefit. Because spontaneous recovery is

common and because no systematic-controlled study of surgical intervention

has been done, this decision must be made on an individual basis, taking

into consideration knowledge of the natural history of the disease. There is

a developing consensus that surgical repair of carpel tunnel syndrome in

these patients is of little benefit and transposition of the ulnar nerve at

the elbow may actually produce poor results.

>

>

>

>

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CMT Group:

D Bird, that's one of the doctors my mom and I saw at the

University of Washington. We offered ourselves to help in any studies

of his, he gave us pamphlet/survery. That was it. Just thought you

might like to know.

-Marta

Author: D Bird, MD; University of Washington

> >

> >Last revision: 15 June 2000

> >Summary

> >Disease characteristics. HNPP is a disorder of peripheral nerves

in which

> individuals are predisposed to repeated pressure neuropathies such

as carpal

> tunnel syndrome and peroneal palsy with foot drop. Recovery from

acute

> neuropathy is often complete; when recovery is not complete, the

resulting

> disability is usually mild. Some affected persons also have signs

of a mild

> to moderate peripheral neuropathy.

> >

> >

> > Management

> >No specific treatment for the underlying genetic or biochemical

defect

> exists and no special diet or vitamin regimen is known to alter the

natural

> course of HNPP. Risk factors for pressure palsies, and thus

activities to

> avoid, include prolonged sitting with legs crossed, occupations

requiring

> repetitive movements of the wrist, prolonged leaning on elbows, and

rapid

> weight loss [Cruz-ez et al 1997, 2000].

> >

> >Many patients have slow but complete recovery from the acute

neuropathy

> episode. Those with incomplete recovery usually retain good

function for

> activities of daily living. Transient bracing, such as with a wrist

splint

> or ankle-foot-orthosis (AFO), may be useful. Some persons with

residual foot

> drop may permanently use an AFO. Controversy exists as to whether

surgical

> decompression of nerves is of benefit. Because spontaneous recovery

is

> common and because no systematic-controlled study of surgical

intervention

> has been done, this decision must be made on an individual basis,

taking

> into consideration knowledge of the natural history of the disease.

There is

> a developing consensus that surgical repair of carpel tunnel

syndrome in

> these patients is of little benefit and transposition of the ulnar

nerve at

> the elbow may actually produce poor results.

> >

> >

> >

> >

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