Natural Highs
Think about them one at a time before going on to the next one.  It Does Make
You Feel Good, especially #45.

1. Falling in love.

   2. Laughing so hard your face hurts.

   3. A hot shower.

   4. No lines at the supermarket.

   5. A special glance.

   6. Getting mail.

   7. Taking a drive on a pretty road.

   8. Hearing your favorite song on the radio.

   9. Lying in bed listening to the rain outside.

   . Hot towels fresh out of the dryer.
11. Chocolate milkshake (vanilla or strawberry).
12. A bubble bath.
13. Giggling.
14. A good conversation.
15 The beach
16. Finding a 20 dollar bill in your coat from last winter.
17. Laughing at yourself.
18. Looking into their eyes and knowing they Love you
19 Midnight phone calls that last for hours.
20. Running through sprinklers.
21. Laughing for absolutely no reason at all.
22. Having someone tell you that you're beautiful.
23. Laughing at an inside joke with FRIENDS
25. Accidentally overhearing someone say something nice about you.
26. Waking up and realizing you still have a few hours left to sleep.
27. Your first kiss (either the very first or with a new partner).
28. Making new friends or spending time with old ones.
29. Playing with a new puppy.
30. Having someone play with your hair.
31. Sweet dreams.
32. Hot chocolate.
33. Road trips with friends.
34. Swinging on swings.
35. Making eye contact with a cute stranger.
36. Making chocolate chip cookies.
37. Having your friends send you homemade cookies.
38 Holding hands with someone you care about.
39 Running into an old friend and realizing that some things (good or bad) never
change.
40. Watching the _expression on someone's face as they open a much desired
present from you.
41. Watching the sunrise.
42. Getting out of bed every morning and being grateful for another beautiful
day.
43. Knowing that somebody misses you.
44. Getting a hug from someone you care about deeply.
45. Knowing you've done the right thing, no matter what other people think.


Lord, keep Your arm around my shoulder and Your hand over my mouth

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It's not the destination...It is the Journey!

---------------------------------
  Check out  the hottest 2008 models today at Yahoo! Autos.

[Non-text portions of this message have been removed]

Melody...same here meds and me are like red and orange...:-( seems we
are darned if we do and darned if we don't...take meds.

Hope Hanger does a good job of cushioning the arm crutches...and do
relate to the pressure palsy...as well.

On occasion I do take soma...to prevent takin the stronger pain
meds...that makes me sooo *ditzy* n very nausious as well. merely
a no win situation...huh!

The best to you,
Dawn ;-)




>
> >  Or it's pressure palsy from the crutch.  I think drugs and
> > me just don't mix well at all anyway.
> >
>
> > I'm going to get cushioning on my crutch handles to  protect my
hands
> > better from the pressure...Hanger will do that for me.
> >
> > Melody



>JB wrote >
>
> On the advise of the OT at the local mda clinic I have cushioned my
> cane handle by surrounding it with a thin-ish piece of foam
encircled
> by black duct tape/electrical tape.  It is very cushy and I think
> helps my hand(s) a lot.  Hanger will probably do a prettier job
> though, just make sure they pad it enough.
>
> JB
>

JB...lol...I never told  anyone I was a time traveler. :-)

Thank you for the lovely complement, & a good laugh is always
welcomed...:-) It is so amazing the progress that has been made
just over the past few years.

Now if the research and studies continue at this pace, we may have
some positivie, outcomes.

Guess it is like taking one step at a time...and with so much to
cover or discover...:-) Now a medication for fatigue...so some
progress is coming with CMT! n Thankfully! ;-)

Dawn








"archivejbb" .> wrote:
>
> --- In CMT-Support@yahoogroups.com, "spiritfree_dawn"
> <spiritfree_dawn@> wrote:
> >
> > Please forgive the type -O- the report was done in 2007.
>
>
> Aaahh, and I thought you'd time traveled 990 years in the future to
> get the article (lol).  I was impressed by your diligence and hard
> work, but was disappointed that CMT knowledge had not progressed as
> much as hoped in that time frame.
>
> Actually thanks for posting a good article. Just had to tease a
little.
>
> JB
>

>  Or it's pressure palsy from the crutch.  I think drugs and
> me just don't mix well at all anyway.
>

> I'm going to get cushioning on my crutch handles to  protect my hands
> better from the pressure...Hanger will do that for me.
>
> Melody
>

On the advise of the OT at the local mda clinic I have cushioned my
cane handle by surrounding it with a thin-ish piece of foam encircled
by black duct tape/electrical tape.  It is very cushy and I think
helps my hand(s) a lot.  Hanger will probably do a prettier job
though, just make sure they pad it enough.

JB

http://www.ncbi.nlm.nih.gov/sites/entrez?term=17060310&cmd=search&db=pubmed

   This has also been used in people with MS...and documented at this site.


    [input] 1: Am J Hosp Palliat Care. 2006 Oct-Nov;23(5):412-6.     Links

      Modafinil reduces fatigue in Charcot-Marie-Tooth disease type 1A: a case
series.  Carter GT, Han JJ, Mayadev A, Weiss MD.
   Department of Rehabilitation Medicine, University of Washington School of
Medicine, Seattle, Washington, USA. gtcarter@...
   Charcot-Marie-Tooth disease, the most common hereditary motor and sensory
neuropathy, is a slowly progressive disorder characterized by diffuse muscle
weakness and prominent distal atrophy that predominantly involves the intrinsic
muscles of the feet and the peroneal muscles. It results in marked reduction in
functional aerobic capacity during exercise and fatigue is commonly reported. To
date, no pharmacologic treatment has been shown to be effective for treating
fatigue in Charcot-Marie-Tooth. Modafinil is used to treat the symptoms of
fatigue and excessive daytime sleepiness in narcolepsy. However, fatigue and
subsequent excessive daytime sleepiness secondary to fatigue are common symptoms
in many neurologic disorders. Prior reports on patients with myotonic muscular
dystrophy, multiple sclerosis, Parkinson's disease, and amyotrophic lateral
sclerosis, have shown beneficial effects of modafinil in treating fatigue. We
report 4 patients with genetically confirmed
  Charcot-Marie-Tooth disease who had significant fatigue that was almost
completely relieved by modafinil.
   PMID: 17060310 [PubMed - indexed for MEDLINE
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-->


It's not the destination...It is the Journey!

---------------------------------
Catch up on fall's hot new shows on Yahoo! TV.  Watch previews, get listings,
and more!

[Non-text portions of this message have been removed]

--- In CMT-Support@yahoogroups.com, "spiritfree_dawn"
<spiritfree_dawn@...> wrote:
>
> Please forgive the type -O- the report was done in 2007.


Aaahh, and I thought you'd time traveled 990 years in the future to
get the article (lol).  I was impressed by your diligence and hard
work, but was disappointed that CMT knowledge had not progressed as
much as hoped in that time frame.

Actually thanks for posting a good article. Just had to tease a little.

JB

http://www.mdausa.org/publications/breathe/


         Contents:
    Dear Friends

    Introduction
    Neuromuscular Disease and Breathing

    Respiratory Evaluation

       Pulmonary Function Tests


    Prevention

       Symptoms of Chronic Underventilation

    Respiratory Treatments
    Assisted Ventilation

       Comparison: Noninvasive & Invasive Assisted Ventilation
       Respiratory Care in Duchenne Muscular Dystrophy
       Respiratory Care in ALS

    Quality of Life
    Advice from a Veteran Vent User
    For More Information
   Additional information about the diseases in MDA's program and locating an MDA
clinic is provided elsewhere on this site.









It's not the destination...It is the Journey!

---------------------------------
Don't let your dream ride pass you by.    Make it a reality with Yahoo! Autos.

[Non-text portions of this message have been removed]

This article is from the Italian CMT Association

   http://www.aicmt.org/gallosti.htm


           REHABILITATION MANAGEMENT OF PERIPHERAL NEUROPATHIES.

   Vinci P.

In: Gallosti L, Rossi G, editors. Practice opinions in neurorehabilitation.
Venice, 2002. CD Rom: chapter 13.


   Neuropathies are pathological processes of nerves, interfering with the normal
transportation of the nervous impulse from the central nervous system to the
muscle fibers (through somatic and visceral motor fiber), and from peripheral
receptors to the center (through sensitive fiber).
   The main consequence of the involvement of the motor nerve fiber is muscle
weakness, while the alteration of sensory fibres causes deficit of sensation
and, sometimes, pain.
   Neuropathies may involve a single nerve or root or plexus (mononeuropathies)
or may be symmetrically widespread to all nerves (polyneuropathies). In this
latter case, with the exception of Guillain-Barrè syndrome (GBS) and few other
disorders, the process is length-dependent, that is, the fibers reaching the
distal parts of the limbs are involved more severely than the ones for the
proximal districts.
   Because  the main task of the rehabilitator is to preserve or restore the
functions necessary to guarantee each person the widest independence, it is very
important to assess the severity and extension of the muscular and sensitive
involvement, and to understand the rule of each weakened muscular group in the
movements which are necessary to carry out each function.
   In fact, the weakening of a muscle (or muscle group) alters a movement
according to its role (agonist, antagonist, neutralizer, stabilizer) in that
specific movement.
   The specification of the function is also very important: for example, the
plantarflexor muscles, in the first stage of Charcot-Marie-Tooth disease (CMT),
must be considered as "strong" for ambulation and "weak" for running.
   With the exception of GBS and few other disorders in which the patients'
functional deficits are due only to the muscle weakness, in the majority of
polyneuropathies the functions are altered not only by the muscle weakness
caused by the neuropathy but also, and in some cases especially, by the muscle
and joint alterations following the muscle weakening and by compensations.
   The sensory deficit that affects the most important daily living functions,
such as normal stance and ambulation, is the one involving proprioception.
However, in some prevalent sensory neuropathies, it is hypoesthesia, since it
may cause trofic changes in the feet, with consequent difficulty to wear shoes
and to walk.
   In order to plan an effective rehabilitation program, it is very important to
know whether the neuropathic process, responsible for the motor and/or sensitive
deficit, is reversible, spontaneously or as a result of a therapy, or if it is
not reversible and not progressive (stabilized) or, worst case, progressive.
   In reversible neuropathies, it is necessary to minimize the impact of muscle
weakening and sensitive deficit on functionality until sprouting (in case of
axonotmesis or neurotmesis) or conduction (in neuroapraxia) take place, and, at
the same time, to prevent muscle and articular damage caused by inactivity and
by use of pathological motor engrams, which would make it difficult or
impossible the restore normal functions even in case of complete nerve healing.
   Therefore, compensations should not be encouraged until any possibility of
recovery is lost.
   An example of mononeuropathy is the one affecting the peroneal nerve, that's a
very common event in traumatology.
   The weakening of the foot dorsiflexor muscles, as a result of it, causes
footdrop, with alteration of the swing phase of gait and risk of tripping, as
well as unbalance of strength between them and their preserved antagonists
(plantarflexors) which undergo a contracture.
   The first problem is solved by an orthotic for footdrop, such as a Codivilla's
spring, allowing the function ambulation to be performed safely while awaiting a
reinnervation and therefore a power recovery in the dorsiflexor muscles.
   The use of a dorsiflexion assisting device also avoids the adoption of
compensations which might cause pain to the spine and persist even after distal
recovery.
   The plantarflexor contracture must be addressed by passive kinesitherapy
(stretching), to avoid it to become permanent (equinus deformity), which would
cause a deficit of dorsiflexion, even in case of complete strength recovery in
the dorsiflexor muscles.
   When reinnervation starts, the dorsiflexor muscles will be stimulated to
contract, in the beginning in a favorable contest (in water), then in the gym
both as an exercise and in the context of a functional re-education to walk
normally.  Only if the dorsiflexor strength recovery will not be sufficient,
permanent compensatory solutions will be adopted, such as shoes with higher
heels or orthotics for foot drop or compensatory motory patterns.
   An example of polyneuropathy susceptible of improvement is GBS.
   In this disease, there is generally a first phase of fast ascending paralysis,
followed, after a short stationary time, by a phase of recovery, either fast or
slow, that leads to complete healing in 60% of cases, and to permanent severe
deficits in 10% of patients.
   In the first phase, it is important to monitor the respiratory function, in
order to ventilate the patient promptly in case of respiratory failure due to
severe diaphragm weakening; besides, communication must be facilitated, a
psychological support should be provided and the consequences of prolonged bed
rest, such as joint rigidity, venous stasis and pressure sores, must be
prevented.
   As muscle strength starts to be recovered, rehabilitators will have to
facilitate the recovery of functions which, in the axonal forms, can require a
very long time and cannot be complete.
   The remaining deficits will have to be addressed either by facilitating the
most suitable compensatory patterns of movement, or, when compensations cause
muscular or articular overload, or are not sufficient, by orthotics.
   In progressive polyneuropaties, such as genetic ones, rehabilitation is aimed
at improving the main functional activities and quality of life, through a
complex intervention involving numerous professionals (neurologist, orthopedic
surgeon, pedorthotist, orthotist, physiotherapist, occupational therapist,
geneticist, psychologist) coordinated by a physiatrist experienced in
neuromuscular rehabilitation.
   Although rehabilitation has no possibility to stop the progression of the
muscular weakening in a distoproximal direction, it is very important to know in
which order the ability to move joints is lost, both to facilitate muscle
contractures that will be useful in the future and to prevent the consequences
of the evolution to the next stage.
   For instance, in the majority of CMT cases, dorsiflexion fails before
plantarflexion, so a triceps surae muscle contracture develops. If this
contracture is severe, a poor balance will result; on the contrary if it is
slight and not associated to a severe rotation, it is useful, as it allows the
ankle stabilization on the sagittal plane and the use of shoes with a small
heel, easier to find on the market as compared to flat ones, even when the
plantaflexors will become completely atrophic.
   It is up to the rehabilitator to monitor the progression of the plantarflexor
weakening, in order to stop the stretching program at the right moment allowing
the optimal contracture to develop, so patients, whose triceps surae has
weakened beyond a certain degree, can continue to wear shoes provided with a
small heel and do not overload the quadriceps muscles.
   On the contrary, in Hereditary Motor Neuropathy (HMN or DSMA) plantarflexion
generally weakens earlier than dorsiflexion. This does not allow the use of
heeled shoes since the first stages and, in many cases, also causes a rotation
of the legs forwards during stance, with consequent need to keep the knees
slightly bent, thanks to the isometric contraction of the quadriceps that will
undergo an overloading.
   In case the evolution of the muscle progression is known, the rehabilitator
will encourage a triceps surae muscle shortening before its complete
degeneration, so allowing a more stable stance and the use of shoes with a small
heel.
   The goals of rehabilitation management in CMT are: improvement of muscle
strength and balance, prevention of fatigability, prevention and treatment of
joint deformities, promotion of mobility, prevention of falls, improvement of
hand function, solution of psychological problems, prevention and treatment of
pain.
   In CMT there is great variability in the extension and severity of  muscle
weakening, with cases in which the weakness is restricted to the foot muscles
and cases with progressive involvement of the pelvic girdle ones.
   Also in upper limbs, there are cases in which all the intrinsic hand muscles
are strong even against a moderate resistance and cases in which they are
completely atrophic and, in addition, also the forearm muscles are weak.
   In order to help rehabilitation professionals to evaluate easily the severity
of rehabilitative problems and provide the most appropriate rehabilitation, we
suggest categorization of patients in levels of increasing severity (seven for
the lower limbs, and four for the upper limbs).
   The functional classification of the lower limb impairment takes into account
the problems caused by the progressive weakening of muscles in the lower limbs
and by its biomechanical consequences on gait.
   In every stage, there is a new problem, typical of the present stage, which is
added to the ones of the previous stages, modifying the pattern of gait and
requiring a different rehabilitative intervention.
   In each stage, rehabilitation management, which consists of lifestyle changes,
orthotics (proper shoes, foot orthoses, ankle-foot-orthoses,
knee-ankle-foot-orthoses), stretching, postural kinesitherapy and orthopedic
surgery, is different and specific.
   It is very important to stress the importance of aesthetics in choosing
orthotics, since many patients, especially women and young people, refuse to
wear orthopedic shoes and traditional ankle-foot-orthoses: in these cases, the
"aesthetic in-shoe device for footdrop" inserted in fashionable above-the-ankle
shoes or boots is recommended.
   Functional classification of the upper limb impairment is based on the
possible type of handgrip. At stage 1, the pinch between the pulps of thumb and
forefinger is preserved, although it is weaker and the thumb is less rotated
than in normal individuals. At stage 2, a weak but functional pinch between
thumb and second or third finger is still possible, thanks to compensations or
tricks. At stage 3, the thumb lies on the same plane as the other fingers and
only a lateral pinch between thumb and forefinger is possible. At stage 4, the
muscles of the hands are wasted, deformities are often present and only a rough
grasp by the lateral fingers is possible. For each stage there is a different
rehabilitation management, which includes recommendations to avoid overwork
weakness and cold, stretching to prevent or treat permanent contractures and
special tools to retain the ability to perform daily living activities.
   About the possibility of strengthening the muscles weakened by CMT disease
through exercise against resistance, there are still doubts, both about efficacy
(since studies have been carried out on proximal muscles) and harmlessness
(since overwork weakness has been found also in this disease).
   References

      Vinci P. Rehabilitation management of Charcot-Marie-Tooth disease. Rome,
It: Spazio Immagine Editore, 2001.

      Vinci P. La riabilitazione nelle neuropatie periferiche. Neurol Sci 2001;
22: S443-6.

      Vinci P, Perelli SL. Aesthetic in-shoe device for footdrop: a multicentric
study on 58 patients. Eur Medicophys 2001; 37: S551-553.

      Vinci P. Strengthening of the proximal muscles in Charcot-Marie-Tooth
disease. Arch Phys Med Rehabil 2001; 4: 563

      Vinci P, Perelli SL. Footdrop, foot rotation and plantarflexor failure in
Charcot-Marie-Tooth disease. Arch Phys Med Rehabil 2002 (in press)

   Aknowledgements
   The Author thanks the Italian Charcot-Marie-Tooth Association (AICMT) for
active collaboration in rehabilitative research.
   AICMT (Associazione Italiana malattia di Charcot-Marie-Tooth) - via Pisacane
10 - 00152 Roma -  Tel/fax 06 3038338
   AICMT website containing information about rehabilitation (both in English and
Italian language):  www.aicmt.org
   AICMT e-mail: aicmt@...
   Author's e-mail:  paolovinci@...
   The book "Rehabilitation Management of Charcot-Marie-Tooth Disease" is
available from the publisher on the website www.stradeservizi.it/cmt.htm




It's not the destination...It is the Journey!

---------------------------------
Moody friends. Drama queens. Your life? Nope! - their life, your story.
  Play Sims Stories at Yahoo! Games.

[Non-text portions of this message have been removed]

This involves the Spinal root abnormalties in the lumbar region, and what an
former MRI had show with me.

   http://www.ajnr.org/cgi/content/full/21/10/1793



   MR Imaging of the Cauda Equina in Hereditary Motor Sensory Neuropathies:
Correlations with Sural Nerve Biopsy Martino Cellerinia,b, Stefania Saltia,
Veronica Desideria and Gianpiero Marconia   a From the Neuroradiology Unit
(M.C.), Azienda Ospedaliera Careggi, Florence; and the Radiodiagnostic Section,
Department of Clinical Physiopathology (S.S., V.D.) and Division of Neurology,
Department of Neurological and Mental Sciences (G.M.), University of Florence,
Florence, Italy.
b Address reprint request to M. Cellerini M.D., U.O. Neuroradiologia, Centro
Traumatologico (CTO), Azienda Ospedaliera Careggi, Largo Palagi 1, 50134
Firenze, Italy.
              Abstract

BACKGROUND AND PURPOSE: Although spinal root abnormalities are known to occur,
spinal MR examination is seldom performed in hereditary motor and sensory
neuropathies (HMSN). The following work was undertaken to assess the MR imaging
spectrum of lumbosacral spinal nerve root abnormalities and determine whether
intradural nerve root involvement could be related to any biopsy feature.  
METHODS: Ten consecutive patients (eight male, two female; age range, 28–65 yrs)
with Charcot-Marie-Tooth (CMT) (type I = 5, type II = 2) and Déjèrine-Sottas
disease (DSD) (n = 3) underwent a contrast-enhanced lumbosacral MR examination.
Sural nerve biopsy was perfomed in all patients. Atypical clinical features were
present in two patients. The MR scans of each patient were reviewed for possible
causes of myeloradiculopathy, spinal nerve root and ganglia dimensions, signal
change, and abnormal enhancement.   RESULTS: In the seven patients with CMT,
abnormal MR findings were intradural nerve
  root hypertrophy (n = 2), signal abnormalities (n = 2), and enhancement (n =
3). Two of three patients with DSD had the abnormal MR finding of intradural
nerve root enhancement. In both patients with atypical clinical features, MR
imaging showed nerve root hypertrophy and enhancement. Both findings were
related to an increased number of onion bulbs at sural nerve biopsy.
Inflammatory infiltrates were not observed in any patients.   CONCLUSION: In
patients with HMSN enhancement of intradural spinal nerve roots, whether or not
associated with marked thickening, may be found on lumbosacral MR examinations.
Spinal nerve root thickening may be responsible for atypical symptoms, and its
visibility on MR images represents a useful adjunct to diagnosis. Lumbosacral
spinal nerve root abnormalities were related to an extremely high number of
onion bulbs (indicating active demyelination) at sural nerve biopsy. Nerve root
enhancement does not seem to be related to inflammatory
  infiltrates.
              Introduction

Hereditary motor and sensory neuropathies (HMSN) are a heterogeneous group of
genetically determined peripheral neuropathies characterized by symmetrical and
predominately distal motor and sensory disturbances and a slowly progressive
course. Charcot-Marie-Tooth (CMT) type I and Déjèrine-Sottas disease (DSD) are
the disorders most characteristically associated with marked thickening of
peripheral nerves (hypertrophic neuropathies). Diagnosis is founded on familial
history, clinical-laboratory data, electromyography and nerve conduction
studies, sural nerve biopsy, and molecular genetic studies. Spinal nerve root
abnormalities have been described in patients with HMSN. MR imaging is a
noninvasive tool for in vivo study of the cauda equina nerve roots, and although
occasional reports on the utility of spinal MR examination have been published
(1–4), spinal root involvement in these disorders has not been widely discussed.
We performed lumbosacral MR examinations in a series
  of patients with HMSN to assess the spectrum of intradural nerve root
abnormalities. Correlations between MR findings and sural nerve biopsies were
obtained to determine whether intradural nerve root involvement could be related
to biopsy features.
              Methods

From January 1998 to March 1999, 10 consecutive patients (eight male, two
female; age range, 28–65 yrs) with HMSN (type I = 5, type II = 2, and type III =
3; mean follow-up period, 6.3 yrs) underwent plain and contrast-enhanced MR
imaging of the lumbosacral spine. All clinical data were obtained from
examination of the clinical charts. In all patients, both light (LM) and
electron microscopy (EM) were performed on sural nerve specimens. In patients
with type I-II HMSN, diagnosis was confirmed with molecular genetic
investigations. Two patients with CMT I suffered from progressive urinary
bladder dysfunction and severe low back pain. These clinical features are not
usually present in HMSN and were considered atypical. Palpable peripheral nerve
enlargement was not present in any patient. All patients with clinical and/or
laboratory suspicion of a concomitant inflammatory or infectious
polyneuroradiculopathy were excluded. Two patients with type III HMSN were in
the
  "classical" or "juvenile" form, and one had the "congenital hypomyelinating"
form.   MR examination of the lumbosacral spine was performed on a
superconducting 1.5-T unit with a linear surface coil. All patients underwent
the same study protocol: sagittal spin-echo (SE) T1-weighted 500/15/2
(TR/TE/excitations), then sagittal and axial fast spin-echo (FSE) T2-weighted
4000/120/4 acquisitions with an echo train length of 16, followed by
postcontrast sagittal, axial, and coronal SE T1-weighted 500/15/2–4
acquisitions. IV injection of paramagnetic contrast medium was performed at a
0.1 mM/kg dose. Section thickness was 3–4 mm for sagittal and coronal scans and
5–6 mm for axial scans. Field of view was 30–35 cm for sagittal and coronal
acquisitions and 20–25 cm for axial scans. The acquisition matrix was 256 x 192
pixels. A fat saturation technique (spectral presaturation with inversion
recovery [SPIR]) was performed during at least one of the postcontrast SE
T1-weighted
  imaging sessions.   Hard copies of MR examinations were reviewed in a
non-blinded fashion by two observers for the following findings: possible causes
of myeloradiculopathy, intra- and extradural spinal root and ganglia dimensions,
and signal change and enhancement. MR imaging findings of the lumbosacral spine
in five healthy subjects were available for comparison. Histologic specimens
from sural nerve biopsy were evaluated by a neurophatologist (GM) for the
following findings: reduction of myelinated fibers, amount of onion bulbs,
collagen tissue, and cellular infiltrates. Each abnormality was classified as
slight (+), moderate (++), severe (+++), and marked (++++).
              Results

An overall schematic representation of microscopic and MR findings is reported
in the Table.
                   View this table:
[in this window]
[in a new window]
     Correlations between pathologic features from sural nerve biopsy and
lumbosacral MR examination

   MR Imaging FindingsType I HMSN In two patients (case 1 and 5), plain and
contrast-enhanced MR examinations were negative. In two patients (case 3 and 4),
the plain sagittal scans revealed a large soft-tissue mass with polylobulated
margins, showing low-to-medium signal intensity on SE T1-weighted and
medium-to-high signal intensity on FSE T2-weighted images in the region of the
cauda equina (Fig 1A). Thickened intradural nerve roots showing medium-to-high
signal intensity were confirmed on the axial FSE T2-weighted images. The roots
of the cauda equina were packed together, mimicking a subarachnoid block at the
L2–L3 level (Fig 1B). Nerve root enlargement extended to the spinal ganglia and
the lumbosacral plexus. Marked enhancement of intra- and extradural nerve roots
occurred in both patients on postcontrast SE T1-weighted images (Fig 1C–E).
Intradural nerve root enhancement was also seen in one patient (case 2) in the
absence of hypertrophic changes.

View larger version (148K):
[in this window]
[in a new window]
     FIG 1. A–E, MR images of the lumbosacral spine in a patient with CMT I and
atypical clinical features (case 3).   Marked thickening of spinal nerve roots,
completely filling the spinal canal, is seen on the sagittal (A) and axial (B)
FSE T2-weighted (4000/120 [TR/TE]) images. Enhancement of hypertrophic spinal
nerve roots and ganglia (arrows) is depicted in the postcontrast SE T1-weighted
(TR/TE) sagittal (C) and axial (D) images. A coronal SPIR (E) postcontrast SE
T1-weighted scan better depicts spinal ganglia hypertrophy and enhancement by
supppressing signal from the paravertebral and foraminal fat.

Type II HMSN In both patients (case 6 and 7), the MR examination was negative.  
Type III HMSN In one patient with the classical form of HMSN (case 9), the
contrast-enhanced MR examination was negative. In the other two patients (one
with the classical form, one with the congenital hypomyelinating form), a slight
but definite and homogeneous enhancement of intradural nerve roots was seen on
postcontrast SE T1-weighted images (Fig 2A–D), in the absence of hypertrophic
changes or signal abnormalities.

View larger version (108K):
[in this window]
[in a new window]
     FIG 2. A–D, MR images of the lumbosacral spine in a patient with the
congenital hypomyelinating form of DSD (case 8).   Marked diffuse enhancement of
the cauda equina nerve roots in the absence of root enlargement is seen on pre-
(A) and postcontrast (B) SE T1-weighted (500/15) sagittal images. Fat-suppressed
(SPIR) coronal postcontrast T1-weighted image (C) enables better contrast
between enhanced spinal ganglia (arrow) and surrounding fat-suppressed fat
tissue signal compared with corresponding non-SPIR image (D).

   Biopsy FindingsType I HMSN On LM, the extent of fibrotic abnormalities and
number and size of onion bulbs varied considerably among patients. Reduction in
the number of myelinated fibers correlated with disease duration and severity
and was more marked in cases 3 and 4 (<2303/mm2), in which almost all fibers
showed onion bulbs. Few residual fibers showed a thin myelin sheath, indicating
remyelination. Axons appeared either normal or hypotrophic. Nerve fascicles
showed variable hypertrophic changes, depending on the amount of onion bulbs,
increase in endoneural collagen tissue, and amorphous protein precipitate
resembling mucus on specific histochemical stains. An increase of fibroblasts
and mastocytes was seen in the endonevrium in patients 2, 3, and 4 (Fig 3). On
EM, onion bulbs showed the typical two-to-five concentric layered structure of
overlapping, intertwined Schwann cells encircling thinly myelinated and
demyelinated axons. In some onion bulbs, the axon was not
  visible (denervated onion bulb).

View larger version (147K):
[in this window]
[in a new window]
     FIG 3. Light microscopy of the sural nerve specimen (Toluidin blue stain,
semi-thin section) in a patient with CMT I and atypical clinical symptoms (case
3).   Almost all fibers show "onion bulbs" (black arrowhead), with an absence of
myelinated fibers, indicating active demyelination. Clusters of regenerating
fibers are indicated by thin black arrow and collagen hypertrophy by large black
arrow.

Type II HMSN Abnormalities were confined to a small proportion of fibers. On LM,
only a slight reduction in the number of myelinated fibers was seen. Onion bulbs
were rare. Several fibers showed a thin myelin sheath. EM demonstrated a certain
amount of nonspecific axonal degeneration in both myelinated and unmyelinated
fibers. Well-formed onion bulbs were absent.   Type III HMSN In both patients
with the "classical" form of type III HMSN, LM showed a significant reduction of
myelinated fibers, irrespective of their diameter. The major part of the fibers
showed either complete demyelination or thinning of the myelin sheath. Onion
bulbs were numerous, and in patient 10, almost every myelinated fiber was
surrounded by an onion bulb. On EM, typical onion bulbs were seen. Fibroblasts
and mastocytes were present in the endoneural collagen in both patients. In the
patient with the congenital hypomyelinating form of HMSN (case 8), an almost
complete disappearance of myelinated
  fibers, with rare basal lamina onion bulbs, was observed on both LM (Fig 4A)
and EM (Fig 4B), in the absence of cellular infiltrates.

View larger version (74K):
[in this window]
[in a new window]
     FIG 4. A and B, LM and EM of the sural nerve specimen (Toluidin blue stain,
semi-thin section) in a patient with the congenital hypomyelinating form of DSD
(case 8).   On LM (A), a decreased number of myelinated fibers with peri- and
epineural connective tissue hypertrophy is noted (arrow). Very few residual
thinly myelinated fibers (open black arrow) are observed. Note the absence of
onion bulbs and connective tissue hypertrophy (thick black arrows) with
fibroblasts (black arrowhead). On EM (B), complete absence of myelin sheaths
around axons is seen. A Schwann cell nucleus is indicated by the arrow.


              Discussion         TOP
Abstract
Introduction
Methods
Results
Discussion
Conclusion
References

Few reports describe the MR appearance of intradural nerve roots in HMSN,
because MR imaging is considered to have no diagnostic role in these disorders.
However, spinal nerve root abnormalities on MR images have been occasionally
reported, and spinal cord impingement from enlarged intradural roots has been
described in patients with CMT I and DSD (2, 4–5). In our series, we have
encountered two situations in which MR imaging was deemed valuable. The first
was characterized by patients with atypical symptoms in which lumbosacral MR
imaging showed marked enlargement, signal change, and enhancement of intradural
nerve roots. Because lumbosacral MR imaging showed neither disk nor degenerative
disease and diabetes mellitus, chronic inflammatory demyelinating
polyneuropathy, amyloidosis, acromegaly, and Refsum's disease were excluded, we
suggested that spinal root abnormalities or cord compression secondary to marked
nerve root enlargement may be responsible for the low back
  pain and bladder dysfunction. In these patients, MR imaging was a useful
diagnostic test for revealing the possible cause of atypical symptoms and
excluding other causes of nerve root or spinal cord compression.   The second
situation was characterized by patients with the classical clinical syndrome and
normal or diffusely enhancing intradural spinal nerve roots on MR images. In
these patients, lumbosacral MR examination was performed to see if intradural
nerve root abnormalities could also occur in the absence of atypical symptoms.
Further studies of a larger group of patients are needed to determine whether a
substantial proportion of patients with CMT and DSD have nerve root thickening
and impingement or if this is just an unusual association.   HMSN have been
classified by Dyck (6) into seven types (I–VII). Type I and II correspond
clinically to CMT and type III to DSD disease. Peripheral nerve hypertrophy
(noticeable sometimes by palpation) and "onion-bulb" changes
  in nerve fibers on microscopic examination are typical findings of type I HMSN.
Differentiation between type I and type II HMSN is based on reduction of mean
conduction velocity in the median or ulnar nerve: very marked (less than 38
mm/s) in type I and normal or slightly reduced in type II. The clinical
manifestations in type III HMSN (recently classified also as "congenital
dysmyelinating neuropathies") begin usually earlier than do types I–II, and its
course is more severe. Moreover, type III HMSN is usually associated with
extremely high protein content in the CSF. Based on morphologic characteristics
of the peripheral nerve, three subtypes of HMSN III are recognized: HMSN type
III with amyelination ("congenital hypomyelinating neuropathy"), with basal
lamina onion bulbs ("infantile form"), and with classical onion bulbs (the
"classical" or "juvenile" form of DSD). HMSN type IV (Refsum's desease) is an
autonomal recessive syndrome currently recognized to be caused by a
  defect in phytanic acid alpha oxidation. HMSN types V–VII are distinguished by
the presence of spastic paraplegia, optic atrophy, and retinitis pigmentosa, in
addition to HMSN features.   Although an exact correlation between MR imaging of
the cauda equina and sural nerve biopsy specimens is difficult, it may be
supposed that the pathologic process in these disorders extends diffusely to all
peripheral nerves, including the lumbosacral intradural nerve roots. MR imaging
may be considered an in vivo method for studying gross pathologic abnormalities.
In patients with a history of hereditary polyneuropathy, macroscopic enlargement
of peripheral nerves is present only in one of four patients with CMT I, which
usually represents a late clinical finding. Interestingly, palpable peripheral
nerves were not observed in any of our patients, and intradural nerve root
thickening seen on MR examinations correlated with the amount of onion bulbs,
increase in endoneural collagen tissue,
  and amorphous protein precipitate, causing a marked enlargement of nerve
fascicles.   In our series, an abnormal enhancement of intradural nerve roots
was also depicted on MR images of patients without atypical symptoms.
Immunofluorescent studies of nerves from patients with hereditary and acquired
peripheral neuropathies have shown plasma protein leakage, suggesting blood
nerve–barrier damage (7, 8). This is also confirmed by the usually high CSF
protein content, especially in patients with type III HMSN. Recently MR imaging
has been employed to study nerve roots and peripheral nerves in the extremities
(1, 2, 9). In particular, it has been reported that plain MR imaging may provide
a useful method for detection of demyelinating-remyelinating processes in the
sciatic nerve trunk of patients with type III HMSN (3). Experimental studies on
rabbit sciatic nerves and rat peripheral nerves after crush injury, neurotomy,
and/or nerve grafting seem to support this hypothesis
  (10). Moreover, it is well known that intradural nerve roots do not usually
show any enhancement in healthy subjects because of an intact blood-nerve
barrier, unless high doses of contrast medium (0.3 mM/Kg) are injected (11). In
our series, diffuse enhancement of lumbosacral spinal roots on MR scans, even in
the absence of hypertrophic or signal changes, correlated with the amount of
onion bulbs, indicating active myelin breakdown and demyelination. Therefore, it
is possible that at least in three patients with CMT I (case 2, 3, and 4) and in
one patient with the classic form of DSD (case 10), abnormal intradural nerve
root enhancement may be related to blood nerve–barrier damage secondary to
active demyelination. Because no inflammatory infiltrates were observed in our
series, it is unlikely that nerve root enhancement is caused by inflammatory
blood nerve–barrier breakdown. Blood nerve–barrier breakdown is also described
in amyloidosis, where it seems to be sustained by
  the perivascular deposit of amyloid in the absence of inflammatory infiltrates
(12). Diffuse enhancement of the cauda equina was also depicted in the patient
with the congenital hypomyelinating form of DSD (case 8). In this disease, by
definition, a primary defect in myelin formation has been suggested, and
demyelination is absent (3), as was supported by a lack of onion bulbs and
preservation of axons. It is possible that in our patient a congenital defect of
the blood-nerve barrier may also be present, as supported by the usually high
CSF protein content in this disorder.   Differential diagnosis of diffuse
intradural nerve root enlargement and enhancement include chronic inflammatory
demyelinating polyneuropathy, meningeal carcinomatosis or lymphoma, amyloid
neuropathy, leprosy, sarcoidosis, and neurofibromatosis. Simultaneous occurrence
of neurofibromatosis and CMT disease has been reported in one patient, possibly
representing a genetic linkage between these diseases
  (13). In fact, the gene for neurofibromatosis 1 has been linked to the
pericentrometric region of chromosome 17, and the HMSN type I locus is linked to
chromosomes 1 and 17. In our patients, however, the cutaneous stigmata of
neurofibromatosis were not present, and family history was negative for such
disease. Diffuse intradural nerve root enhancement can be also noted in acute
and chronic inflammatory demyelinating polyneuropathies, infective
polyradiculopathies such as cytomegaloviral AIDS-related polyradiculonevritis
(14, 15), postsurgical arachnoiditis (16), and even after radiation therapy.
Nerve root compression (eg, spinal stenosis and disk herniation) can disrupt the
blood-nerve root barrier, also leading to abnormal enhancement. The latter,
however, usually involves only a few nerve roots and in some cases it seems to
be reversible, generally resolving in approximately 6 months (17).
              Conclusion         TOP
Abstract
Introduction
Methods
Results
Discussion
Conclusion
References

Lumbosacral MR imaging of patients with HMSN show either: 1) marked thickening
and enhancement of intradural nerve roots, possibly related to atypical clinical
features, or 2) diffuse enhancement of the cauda equina nerve roots in the
absence of any abnormalities on precontrast MR images. The former lumbosacral MR
findings are useful for excluding other causes of nerve root or spinal cord
compression. An extremely high number of onion bulbs, indicating active
demyelination, correlate with spinal nerve root abnormalities. Nerve root
enhancement is not related to inflammatory infiltrates, but rather may be
related to blood nerve–barrier disruption from congenital and/or active
demyelinating processes.
              References         TOP
Abstract
Introduction
Methods
Results
Discussion
Conclusion
References


    Masuda N, Hayashi H, Tanabe H. Nerve root and sciatic trunk enlargement in
Dejerine-Sottas disease: MRI appearance. Neuroradiology 1992;35:36-37[Medline]

    Murata K, Morishita S, Nakamuro T, Kanda T, Takayanagi T. A case report of
the compression syndrome due to hypertrophic neuropathy. Clin Neurol
1991;31:213-215

    Tachi N, Kozuka N, Ohya K, Chiba S, Naganuma M. MRI of peripheral nerves and
pathology of sural nerves in hereditary motor and sensory neuropathy type III.
Neuroradiology 1995;37:496-499[Medline]

    Friedman DP, Flanders AE, Tartaglino LM. Hypertrophic Charcot-Marie-Tooth
disease: MR imaging findings. AJR Am J Roentgenol 1994;163:749-750[Medline]

    Rosen SA, Wang H, Cornblath DR, Uematsu E, Hurko O. Compression syndromes due
to hypertrophic nerve roots in hereditary motor sensory neuropathy type I.
Neurology 1989;39:1173-1177[Abstract/Free Full Text]

    Dyck PJ. Inherited neuronal degeneration and atrophy affecting peripheral
motor, sensory, and autonomic neurons. In: Dyck PJ, Thomas PK, Lambert EH, Bunge
R, eds. Peripheral Neuropathy, 2nd ed. Philadelphia: Saunders 1984;1600-1642

    Neuen E, Seitz RJ, Langenbach M, Wechsler W. The leakage of serum proteins
across the blood-nerve barrier in hereditary and inflammatory neuropathies. An
immunohistochemical and morphometric study. Acta Neuropathol
1987;73:53-61[Medline]

    Graham AR, Johnson PC. Direct immunofluorescent findings in peripheral nerve
from patients with diabetic neuropathy. Ann Neurol 1985;17:450-454[Medline]

    Naganuma M, Doi S, Shima K, Matsumoto A, Tashiro K. Chronic inflammatory
demyelinating polyradiculoneuropathy associated with multifocal nerve
hypertrophy. Report of a case with MRI study. Clin Neurol 1991;31:1186-1191

    Teitelbaum DS, Frazier JL, Grossman RI, et al. Wallerian degeneration and
inflammation in rat peripheral nerve detected by in vivo MR imaging. AJNR Am J
Neuroradiol 1989;10:741-746[Abstract]

    Jinkins JR, Rauch RA, Gee GT, et al. Lumbosacral spine: early and delayed MR
imaging after administration of an expanded dose of gadopentetate dimeglumine in
healthy, asymptomatic subjects. Radiology 1995;197:247-251[Abstract]

    Adams D, Said G. Ultrastructural immunolabelling of amyloid fibrils in
acquired and hereditary amyloid neuropathies. J Neurol 1996;243:63-67[Medline]

    Roos KL, Pascuzzi RM, Dunn DW. Neurofibromatosis, Charcot-Marie-Tooth
disease, or both? Neurofibromatosis 1989;2:238-243[Medline]

    Crino PB, Zimmerman R, Laskowitz D, Raps EC, Rostami AM. Magnetic resonance
imaging of the cauda equina in Guillain-Barré syndrome. Neurology
1994;44:1334-1336[Abstract/Free Full Text]

    Talpos D, Tien RD, Hesselink JR. Magnetic resonance imaging of AIDS-related
polyradiculopathy. Neurology 1991;41:1996-1997

    Jinkins JR, Osborn AG, Garret D Jr, Hunt S, Story JL. Spinal nerve
enhancement with Gd-DTPA: MR correlation with the postoperative lumbosacral
spine. AJNR Am J Neuroradiol 1993;14:383-394[Abstract]

    Van Goethem JW, Van de Kelft E, Biltjes IG, et al. MRI after successful
lumbar discectomy. Neuroradiology 1996;38:S90-S96

Received November 1, 1999; accepted after revision May 10, 2000.




It's not the destination...It is the Journey!

---------------------------------
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[Non-text portions of this message have been removed]

Hi Erin,

I don't know if you mentioned why the doctor wants you to have a
bi-pap or a c-pap.  Is it due to a weakend diaphragm or other reasons?
  Just curious.  Thanks.

Sal
> >
> > Hi Erin,
> >
> > I know of a person here in my area who uses a c-pap or a bi-pap.
> > Can't remember which.  And it's not due to CMT.  So there are many
> > reasons to use one or the other of these as well.
> >
> > Sal
> >
> >
> > --- In CMT-Support@yahoogroups.com, "Erin Kelly" <ekelly4@> wrote:
> > >
> > > Well Dawn... I think, due to your last 2 articles... on top of
> > other's shared experiences... I think I understand enough to rest
> > assured that my doctors know what they're doing ;-)
> > > No matter the details of a breathing problem, c-pap is used as
> first
> > choice, but maybe harder to get used to.
> > >
> > > bi-pap still delivers a low flow of air, but deceases during
> > exhalation & increases during inhalation (keeping the airway open
> > *only* when needed) = more comfortable & easier to get used to.
> > >
> > > so the c-pap is probably the least expensive way to solve the
> > problem, and not a bad choice as long as it's tolerated well...
> > knowing that other alternatives are available
> > >
> > > I'll certainly try my best! Thanks so much everyone!
> > >
> > > My eyes are also getting a bit 'ucky' & Shaw's waiting for a
> walk :))
> > >
> > >     ~Erin~
> > >
> > >
> > >   ----- Original Message -----
> > >   From: Dawn
> > >   To: cmt-support@yahoogroups.com
> > >   Sent: Thursday, October 04, 2007 3:31 PM
> > >   Subject: [CMT-Support] Addresses both C-Pap and bi-pap usage
> > >
> > >
> > >   http://www.medicinenet.com/sleep_apnea/page6.htm
> > >
> > >   It's not the destination...It is the Journey!
> > >
> > >
> > >
> > > [Non-text portions of this message have been removed]
> > >
> >
>

Hello Sal, yes there are many without CMT that use the bi-pap,
as a matter of fact several here, have the oxygen bottles on a
stand with wheels,

and some have the small portiable packs, which attaches to either
their walker or power chair. Amazing how technology has progressed,

and allows the person in need more independance and not merely

confined indoors due to their need of oxygen. :-) Thinking this will
be my next endeavor, after the other 2 have been addressed.

A good evening to you!
Dawn





Sal wrote:
>
> Hi Erin,
>
> I know of a person here in my area who uses a c-pap or a bi-pap.
> Can't remember which.  And it's not due to CMT.  So there are many
> reasons to use one or the other of these as well.
>
> Sal
>
>
> --- In CMT-Support@yahoogroups.com, "Erin Kelly" <ekelly4@> wrote:
> >
> > Well Dawn... I think, due to your last 2 articles... on top of
> other's shared experiences... I think I understand enough to rest
> assured that my doctors know what they're doing ;-)
> > No matter the details of a breathing problem, c-pap is used as
first
> choice, but maybe harder to get used to.
> >
> > bi-pap still delivers a low flow of air, but deceases during
> exhalation & increases during inhalation (keeping the airway open
> *only* when needed) = more comfortable & easier to get used to.
> >
> > so the c-pap is probably the least expensive way to solve the
> problem, and not a bad choice as long as it's tolerated well...
> knowing that other alternatives are available
> >
> > I'll certainly try my best! Thanks so much everyone!
> >
> > My eyes are also getting a bit 'ucky' & Shaw's waiting for a
walk :))
> >
> >     ~Erin~
> >
> >
> >   ----- Original Message -----
> >   From: Dawn
> >   To: cmt-support@yahoogroups.com
> >   Sent: Thursday, October 04, 2007 3:31 PM
> >   Subject: [CMT-Support] Addresses both C-Pap and bi-pap usage
> >
> >
> >   http://www.medicinenet.com/sleep_apnea/page6.htm
> >
> >   It's not the destination...It is the Journey!
> >
> >
> >
> > [Non-text portions of this message have been removed]
> >
>

Hi Erin,

I know of a person here in my area who uses a c-pap or a bi-pap.
Can't remember which.  And it's not due to CMT.  So there are many
reasons to use one or the other of these as well.

Sal


--- In CMT-Support@yahoogroups.com, "Erin Kelly" <ekelly4@...> wrote:
>
> Well Dawn... I think, due to your last 2 articles... on top of
other's shared experiences... I think I understand enough to rest
assured that my doctors know what they're doing ;-)
> No matter the details of a breathing problem, c-pap is used as first
choice, but maybe harder to get used to.
>
> bi-pap still delivers a low flow of air, but deceases during
exhalation & increases during inhalation (keeping the airway open
*only* when needed) = more comfortable & easier to get used to.
>
> so the c-pap is probably the least expensive way to solve the
problem, and not a bad choice as long as it's tolerated well...
knowing that other alternatives are available
>
> I'll certainly try my best! Thanks so much everyone!
>
> My eyes are also getting a bit 'ucky' & Shaw's waiting for a walk :))
>
>     ~Erin~
>
>
>   ----- Original Message -----
>   From: Dawn
>   To: cmt-support@yahoogroups.com
>   Sent: Thursday, October 04, 2007 3:31 PM
>   Subject: [CMT-Support] Addresses both C-Pap and bi-pap usage
>
>
>   http://www.medicinenet.com/sleep_apnea/page6.htm
>
>   It's not the destination...It is the Journey!
>
>
>
> [Non-text portions of this message have been removed]
>

Well Dawn... I think, due to your last 2 articles... on top of other's shared
experiences... I think I understand enough to rest assured that my doctors know
what they're doing ;-)
No matter the details of a breathing problem, c-pap is used as first choice, but
maybe harder to get used to.

bi-pap still delivers a low flow of air, but deceases during exhalation &
increases during inhalation (keeping the airway open *only* when needed) = more
comfortable & easier to get used to.

so the c-pap is probably the least expensive way to solve the problem, and not a
bad choice as long as it's tolerated well... knowing that other alternatives are
available

I'll certainly try my best! Thanks so much everyone!

My eyes are also getting a bit 'ucky' & Shaw's waiting for a walk :))

     ~Erin~


   ----- Original Message -----
   From: Dawn
   To: cmt-support@yahoogroups.com
   Sent: Thursday, October 04, 2007 3:31 PM
   Subject: [CMT-Support] Addresses both C-Pap and bi-pap usage


   http://www.medicinenet.com/sleep_apnea/page6.htm

   It's not the destination...It is the Journey!



[Non-text portions of this message have been removed]

Hi Erin,

You know I don't really understand what "they" mean when they say it
is rare.  We always think of rare as rare.  But, in the grand scheme
of things, there are so many types of CMT that they know of now and
how many people (CMTers) are involved in that bunch.  So maybe it is
rare even though I know someone personally with it and you and Cathy's
daughter, etc.  So, perhaps a lot of us can think of people who have
this weakness. I'm still thinking it can be more prevalent in certain
types of CMT.  Of course, non-CMTers can have it as well.  Just rambling.

Sal


--- In CMT-Support@yahoogroups.com, "Erin Kelly" <ekelly4@...> wrote:
>
> Thanks for sharing your knowledge Sal :) I've read about the areas
of involvement you mentioned, as well as swallowing (laryngeal) problems.
>
> As a Speech Therapist I focused on various therapies to improve
function. But I'll bet a good proportion of my patients could have
been better served by a c-pap or bi-pap..... that was almost 20 years ago.
>
> Whether *part* of CMT or a *concomitant* feature. I want it if it
will help me!
>
> Thanks again'
>
>     `Erin~!
>   ----- Original Message -----
>   From: sal2491
>   To: CMT-Support@yahoogroups.com
>   Sent: Wednesday, October 03, 2007 9:02 PM
>   Subject: [CMT-Support] Re: CPAP??
>
>
>   Hi Erin,
>
>   From what I have read it is rare.  The involvement of the nerves to
>   the diaphragm (phrenic nerves) can cause weakness of the diaphragm.
>   I'm not sure if this is more common in a certain type of CMT or not.
>   I know the involvement of weak vocal cords and such is more likely in
>   CMT type 2 I believe.  This is just what I've read.
>
>   Sal
>
>
>   --- In CMT-Support@yahoogroups.com, "Erin Kelly" <ekelly4@> wrote:
>   >
>   > Hello all :)
>   >
>   > I had a sleep apnea study last night.  The procedure went very well.
>   But  the tech told me I had apneas & hypotneac events, among other
>   breathing problems.... She, the tech, explained the difference. And
>   said the doctor would explain more at a follow-up appt; but would
>   probably want another sleep test with a CPAP on (to regulate settings)
>   as soon as he reads the strips & such. I was shown a number of
>   different masks to choose from.
>   >
>   > Well...... it looks to me like I need a CPAP. I know basically what
>   a CPAP regulates, but not many details or specifics. And why do so
>   many of us CMTers need them or Bi-PAPs?
>   >
>   > Now it's night & reality may be 'sinking in'; So I'm getting a bit
>   concerned. ..I'm obviously getting worse? And what about PFTs?
>   Different difficulties?
>   >
>   > Any comments will be much appreciated :))
>   >
>   >     ~Erin~
>   >
>   >
>   > "This is the last freedom - to choose one's attitude in any given
>   set of circumstances, to choose one's own way."
>   > ~ Victor Frankl
>   >
>   >
>   > [Non-text portions of this message have been removed]
>   >
>
>
>
>
>   Group Owners:   CMT-Support-owner@yahoogroups.com
>
>
>
>
>   Yahoo! Groups Links
>
>
>
>
>
> [Non-text portions of this message have been removed]
>

For parent who may have an interest, MDA offers a booklet: Teacher Guide which
lists the number of neuromuscular conditions,

   as well as a list of questions to assist the teacher/teachers.

   The question section is hyper-linked to click on for the answers given...thus
I did not copy paste for a full review.

   Dawn





   http://www.mdausa.org/publications/tchrdmd/




It's not the destination...It is the Journey!

---------------------------------
Looking for a deal? Find great prices on flights and hotels with Yahoo!
FareChase.

[Non-text portions of this message have been removed]

August 09, 2007  Study data from Texas Scottish Rite Hospital for Children
update understanding of Charcot-Marie-Tooth disease in children  By
NewsRx.com  Researchers detail in "Scoliosis in patients with
Charcot-Marie-Tooth disease," new data in Charcot-Marie-Tooth disease. According
to a study from the United States, "Scoliosis appears to occur in approximately
one-third of patients with Charcot-Marie-Tooth disease. Little is known about
the response of these curves to treatment."
   "The purpose of this study was to establish the prevalence of scoliosis in a
large population of children and adolescents with Charcot-Marie-Tooth disease,
to evaluate factors linked with curve progression, and to assess the response to
orthotic and surgical treatment. The medical records of 298 patients were
retrospectively reviewed. Radiographs were reviewed for patients identified as
having spinal deformity. The type, size, and progression of the scoliotic curve
were measured, and the effectiveness of bracing and surgical treatment was
assessed. Forty-five patients with scoliosis associated with Charcot-Marie-Tooth
disease were identified. The average age at the diagnosis of the spinal
deformity was 12.9 years, and the average curve magnitude at the time of
diagnosis was 27.6 degrees. One-third of the curves were left thoracic, and 49%
were associated with increased thoracic kyphosis. Twenty-four of the thirty-four
curves that were followed for more than one year
  progressed. Brace treatment was successful in only three of sixteen patients.
Surgery was performed in fourteen of the forty-five patients. Long posterior
spinal fusions were performed most often, with an average of 13.1 spinal
segments fused. Instrumentation was used in all posterior fusions.
Intraoperative neurologic monitoring was possible for only three of the twelve
patients for whom it was attempted during surgery, but there were no
intraoperative neurologic complications. Scoliosis in patients with
Charcot-Marie-Tooth disease differs from that in patients with idiopathic
scoliosis. Thoracic hyperkyphosis is common, and bracing is usually
unsuccessful," wrote L.A. Karol and colleagues, Texas Scottish Rite Hospital for
Children.
   The researchers concluded: "Surgical fusion does not appear to be associated
with a high rate of complications, although it is often impossible to perform
intraoperative neurologic monitoring."
   Karol and colleagues published the results of their research in the Journal of
Bone and Joint Surgery (Scoliosis in patients with Charcot-Marie-Tooth disease.
Journal of Bone and Joint Surgery, 2007;89(7):1504-10).
   For additional information, contact L.A. Karol, Texas Scottish Rite Hospital
for Children, 2222 Welborn Street, Dallas, TX 75219 USA..
   The publisher of the Journal of Bone and Joint Surgery can be contacted at:
Journal Bone Joint Surgery Inc., 20 Pickering St., Needham, MA 02192, USA.
   Keywords: United States, Dallas, Bone, Charcot-Marie Tooth Disease,
Charcot-Marie-Tooth Disease





  
http://www.merckmedicus.com/pp/us/hcp/hcp_newsarticle.jsp?newsid=862079&newsgrou\
p=2





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Shape Yahoo! in your own image.  Join our Network Research Panel today!

[Non-text portions of this message have been removed]

http://www.medicinenet.com/sleep_apnea/page6.htm

It's not the destination...It is the Journey!

---------------------------------
Building a website is a piece of cake.
Yahoo! Small Business gives you all the tools to get online.

[Non-text portions of this message have been removed]

Erin...thought this may be a light on the subject,
   for the moment.
   Dawn


   http://www.dukemednews.duke.edu/news/article.php?id=7969


It's not the destination...It is the Journey!

---------------------------------
Got a little couch potato?
Check out fun summer activities for kids.

[Non-text portions of this message have been removed]

Erin...I am only assuming if I were a clinitian...each individual
would require, the sleep apnea test, then make a determination from
the results.

Then noticing the *bi-pap* after I had sent the post and reread it.
The the wheels began to turn on, what the variances are between the C-
pap and bipap.

Hummmm! :-) now what a time for my eyes to full of uck...but will
look into it for you and see what is found. :-)

Hugs,
Dawn






"Erin Kelly.> wrote:
>
> Thanks for a really good article, Dawn! It sounds to me like a
knowledge of the origin of the raw data needs to be there in order to
make a decision for treatment.
>
> I also wonder if the recommendation of a bi-PAP pertains only to
those with "restrictive pulmonary involvement"??
>
> This lady was treated with intermittent positive airway pressure -
bi-pap - Is that correct?
>
> I'm getting myself confused.... c-pap is continual & bi-pap is
intermittent?
>
> Thanks - a good research article generates more questions than it
answers :)
>
> Hugs,
>
>     ~Erin~
>   ----- Original Message -----
>   From: Dawn
>   To: cmt-support@yahoogroups.com ;
cmt_family_support@yahoogroups.com
>   Sent: Wednesday, October 03, 2007 9:33 PM
>   Subject: [CMT-Support] Re: CPAP involvement in CMT
>
>
>   Erin this is the latest article done in 2997 on CPAP in CMT
>
>     http://www.ncbi.nlm.nih.gov/sites/entrez?
Db=pubmed&Cmd=ShowDetailView&TermToSearch=17294338&ordinalpos=1&itool=
EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
>
>
>      [input] 1: Lung. 2007 Jan-Feb;185(1):1-7. Epub 2007 Feb 9.
Links
>
>        Disorders of pulmonary function, sleep, and the upper airway
in Charcot-Marie-Tooth disease.  Aboussouan LS, Lewis RA, Shy ME.
>
>
>
> [Non-text portions of this message have been removed]
>

Thanks for a really good article, Dawn! It sounds to me like a knowledge of the
origin of the raw data needs to be there in order to make a decision for
treatment.

I also wonder if the recommendation of a bi-PAP pertains only to those with
"restrictive pulmonary involvement"??

This lady was treated with intermittent positive airway pressure - bi-pap - Is
that correct?

I'm getting myself confused.... c-pap is continual & bi-pap is intermittent?

Thanks - a good research article generates more questions than it answers :)

Hugs,

     ~Erin~
   ----- Original Message -----
   From: Dawn
   To: cmt-support@yahoogroups.com ; cmt_family_support@yahoogroups.com
   Sent: Wednesday, October 03, 2007 9:33 PM
   Subject: [CMT-Support] Re: CPAP involvement in CMT


   Erin this is the latest article done in 2997 on CPAP in CMT

    
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSear\
ch=17294338&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.\
Pubmed_RVDocSum


      [input] 1: Lung. 2007 Jan-Feb;185(1):1-7. Epub 2007 Feb 9.     Links

        Disorders of pulmonary function, sleep, and the upper airway in
Charcot-Marie-Tooth disease.  Aboussouan LS, Lewis RA, Shy ME.



[Non-text portions of this message have been removed]

Thanks Dawn... I read the article 3-4 times & printed it out to take along to my
next appt. :)

It did generate some questions I hadn't thought of before  - basically when
would a c-pap vs. a bi-pap be used.

And would a sleep test be enough to give sufficient data to the experts, in
order to decide?

Maybe I'm *too* obsessed with learning enough of what the doctors do/don't know.
But then that's just part of what us CMTers need to do along our journey...

Thanks & Hugs,

~Erin~
   ----- Original Message -----
   From: spiritfree_dawn
   To: CMT-Support@yahoogroups.com
   Sent: Wednesday, October 03, 2007 9:34 PM
   Subject: [CMT-Support] Re: CPAP involvement in CMT


   Please forgive the type -O- the report was done in 2007.










   In CMT-Support@yahoogroups.com, Dawn <spiritfree_dawn@...> wrote:
   >
   > Erin this is the latest article done in 2997 on CPAP in CMT
   >
   >   http://www.ncbi.nlm.nih.gov/sites/entrez?
   Db=pubmed&Cmd=ShowDetailView&TermToSearch=17294338&ordinalpos=1&itool=
   EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum


[Non-text portions of this message have been removed]

Thanks for sharing your knowledge Sal :) I've read about the areas of
involvement you mentioned, as well as swallowing (laryngeal) problems.

As a Speech Therapist I focused on various therapies to improve function. But
I'll bet a good proportion of my patients could have been better served by a
c-pap or bi-pap..... that was almost 20 years ago.

Whether *part* of CMT or a *concomitant* feature. I want it if it will help me!

Thanks again'

     `Erin~!
   ----- Original Message -----
   From: sal2491
   To: CMT-Support@yahoogroups.com
   Sent: Wednesday, October 03, 2007 9:02 PM
   Subject: [CMT-Support] Re: CPAP??


   Hi Erin,

   From what I have read it is rare.  The involvement of the nerves to
   the diaphragm (phrenic nerves) can cause weakness of the diaphragm.
   I'm not sure if this is more common in a certain type of CMT or not.
   I know the involvement of weak vocal cords and such is more likely in
   CMT type 2 I believe.  This is just what I've read.

   Sal


   --- In CMT-Support@yahoogroups.com, "Erin Kelly" <ekelly4@...> wrote:
   >
   > Hello all :)
   >
   > I had a sleep apnea study last night.  The procedure went very well.
   But  the tech told me I had apneas & hypotneac events, among other
   breathing problems.... She, the tech, explained the difference. And
   said the doctor would explain more at a follow-up appt; but would
   probably want another sleep test with a CPAP on (to regulate settings)
   as soon as he reads the strips & such. I was shown a number of
   different masks to choose from.
   >
   > Well...... it looks to me like I need a CPAP. I know basically what
   a CPAP regulates, but not many details or specifics. And why do so
   many of us CMTers need them or Bi-PAPs?
   >
   > Now it's night & reality may be 'sinking in'; So I'm getting a bit
   concerned. ..I'm obviously getting worse? And what about PFTs?
   Different difficulties?
   >
   > Any comments will be much appreciated :))
   >
   >     ~Erin~
   >
   >
   > "This is the last freedom - to choose one's attitude in any given
   set of circumstances, to choose one's own way."
   > ~ Victor Frankl
   >
   >
   > [Non-text portions of this message have been removed]
   >




   Group Owners:   CMT-Support-owner@yahoogroups.com




   Yahoo! Groups Links





[Non-text portions of this message have been removed]

I saw my physiatrist for my annual yesterday. He helped me with the lower
back issues earlier this yr. I had read on a list that some CMTers use
muscle
relaxers for tight muscles and it works for them. I asked him about
whether he thought I should use such for my hip muscles when they're
tensed up...he said no...they can increase muscle weakness. I took
Piroxicam (NSAID) last yr and the muscles on top of my index finger
got weaker.
He said today the weakness is not nerve related, meaning progression,
but didn't say it was
drug related either. I think it could be...it was listed as a possible
side effect.  Or it's pressure palsy from the crutch.  I think drugs and
me just don't mix well at all anyway.

I also spoke to him about using my little scooter in the house...I
could tell, he is worried about that....that I'll stop being upright
and get weaker.  I see his point and only want to use it if I have to.
I'm going to get cushioning on my crutch handles to  protect my hands
better from the pressure...Hanger will do that for me.

Melody

Thanks so much Kathy! I knew Cheryl used a bi-pap, but wasn't sure why other
than breathing problems. I really admire your courage in sharing Cheryl's, and
your own, struggles with CMT. In all the years I've known you, I've seen you
'come out' of your grief to share fun memories; as well as those that may help
the rest of us! You are a great inspiration! I'm glad to have you as a member
here and at Family_Support :)

I checked my fingernails & lips - looks pinked-up to me. So maybe a c-pap *is*
the appropriate treatment. I'd think with air output & pulse-ox being measured
during the sleep test, CO2 retention would be measured.

When I mentioned the symptoms I was having to my neuro & said I knew CMTers
often have sleep problems, he said "Oh, right. Thanks for reminding me. We'll
schedule a sleep-test" What that means is up to interpretation.

Sounds like Cheryl was able to continue functioning at a higher level after
using the c-pap - what a blessing!

I'll make sure I pay particular attention to the fit of the seal on the mask. At
least I'll get to try some of them before the next test, and go through a night
using it. My tech said there are "hundreds" to choose from. And the machines are
small enough to sit on a bedside table :)

Thanks for your help & confidence in me, Kathy :)

     ~Erin~


----- Original Message -----
   From: AquaKath@...
   To: CMT-Support@yahoogroups.com
   Sent: Wednesday, October 03, 2007 9:41 PM
   Subject: Re: [CMT-Support] Re: CPAP??


   Erin, Cheryl used a bi-pap because she retained Co2, which was making her
   very spacey...
   She used it for 10 years and her health improved immensely....She had much
   more energy and was never sick once in those 10 years....

   The c-pap means you don't need help getting rid of oxygen and thus retaining
   Co2....I think my breathing is becoming involved as well....I don't wake up
   with headaches, but there are times when I feel a little short of breath...I
   have, on occasion, woke up gasping for air, although not often, thankfully...

   Look at your lips and fingernails...If they're pink that's good....See how
   they look in the morning when you get up...

   It's my own personal opinion, but I think we CMTers have more breathing
   problems than doctors know....I don't believe it's rare at all....Cheryl had
   pneumonia 6 times and when I asked her neuro if it was because of her CMT, he
acted
   like I was an idiot for asking such a stupid question...."No way," he
   said...".It's just one of those things."....I knew in my heart that there was
a
   connection and I was right...A child doesn't have pneumonia 6 times by age 6
for no
   reason...

   Cheryl had no trouble getting use to the bi-pap, but the mask did cut her on
   the nose......Just make sure in the process of getting a tight seal, it isn't
   so tight that it hurts, or digs in...I think in the last few years they have
   improved the masks dramatically....

   I'm sure others can give you more detailed advice, but I wanted to share what
   I know with you...
   I admire your courage and determination...Please keep us posted....Hugs,
   Kathy





[Non-text portions of this message have been removed]

By reading this article, it's no wonder why there are so many CMTers that
have fibromyalgia on top of the CMT, IMO.

Sharon






Web address:
<http://www.sciencedaily.com/releases/2000/10/001030082926.htm>
http://www.sciencedaily.com/releases/2000/10/001030082926.htm













Source:

  <http://www.rheumatology.org> American College Of Rheumatology


Date:

November 1, 2000

More on:

  <http://www.sciencedaily.com/news/health_medicine/fibromyalgia/>
Fibromyalgia,
<http://www.sciencedaily.com/news/health_medicine/pain_control/> Pain
Control,  <http://www.sciencedaily.com/news/health_medicine/joint_pain/>
Joint Pain,  <http://www.sciencedaily.com/news/health_medicine/arthritis/>
Arthritis,
<http://www.sciencedaily.com/news/health_medicine/today's_healthcare/>
Today's Healthcare,
<http://www.sciencedaily.com/news/health_medicine/diseases_and_conditions/>
Diseases and Conditions


Abnormal Pain Memory Helps To Explain Fibromyalgia


  <http://www.sciencedaily.com> Science Daily - The symptoms of fibromyalgia
may be the result of a central nervous system that "remembers" pain
sensations for an abnormally long time, according to research presented at
the American College of Rheumatology Annual Scientific Meeting Oct. 29 --
Nov. 2 in Philadelphia, Pennsylvania.

Fibromyalgia, sometimes called fibrositis, is associated with widespread
pain, stiffness and fatigue. People with fibromyalgia are found to have
multiple tender points in specific body areas. The painful disorder affects
about two percent of the U.S. population.

Researchers at the University of Florida applied heat stimuli to the hands
of healthy controls and fibromyalgia patients. In contrast to normal
controls, fibromyalgia patients experienced a great amount of cumulative
pain from these stimulations, indicating abnormalities in spinal cord pain
processing. Furthermore, the fibromyalgia patients experienced residual pain
when the stimuli were applied at intervals at which the healthy controls
were not affected. Normally, pain sensations quickly subside after a single
heat stimulus, but will accumulate with repetitions if they occur frequently
enough. This "pain memory" appears to linger for an abnormally long period
of time in fibromyalgia patients.

The researchers also found that the residual pain experienced by
fibromyalgia patients was widespread and not limited to a single area of the
body.

"Because the effect of the first experimental stimulus does not rapidly
decay in fibromyalgia patients, the effect of subsequent stimuli adds to the
first, and so on, resulting in ever increasing pain sensations," said lead
investigator Roland Staud, MD. "Our findings provide evidence for abnormal
central nervous system mechanism of pain in fibromyalgia patients and have
significant implications for future therapies, which need to target these
abnormal central pain mechanisms."

The American College of Rheumatology is the professional organization for
rheumatologists and health professionals who share a dedication to healing,
preventing disability and curing arthritis and related rheumatic and
musculoskeletal diseases. For more information on the ACR's annual meeting,
see http://www.rheumatology.org

Note: This story has been adapted from material provided by American College
Of Rheumatology.





[Non-text portions of this message have been removed]

Hi Ed,
My son, Zack 13 yrs old, talks all the time. Even to himself and he
will mutter anything and everything on the TV. I just thought that it
was connected to his ADD, however he has done this his whole life. Now
sometimes I wish I never wished for him to talk. He doesn't shut up.
But he is doing well with it. He knows he does it that is when he
started to mutter more. He'll also just move his lips like he is
talking when nothing comes out. Also when he started to take Diazapam
in the evenings for his pain. I believe its okay to talk to yourself.
And sometimes it might be the only intellegent conversation you have. I
do it often but mine is stress related. So Ed continue to be yourself.
If you talk to yourself often and it is bothering people then you might
want to just talk under your breath so they can't hear you and your
family will deal with it because they are your family. Yea sure it gets
on my nerves when Zack does it but I have learned to deal with it.
Sometimes I don't think he is actually knows that he is doing it.
Hugs,
Terri

Hello group, In seeking an answer for the inflamation
   I have been experiencing, and wanting to post this
   for some insight into such.

   Please note the article stating that the involvement with the celluar immune
system...which has been a question here in the past....and it's possible
involvement with CMT.

   Dawn


  
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSear\
ch=12062260&ordinalpos=2&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.\
Pubmed_RVDocSum


1: Neuromuscul Disord. 2002 May;12(4):405-14.     Links
      Immune mechanisms in acquired demyelinating neuropathies: lessons from
animal models.  Mäurer M, Toyka KV, Gold R.
   Department of Neurology, Section of Developmental Neurobiology,
Julius-Maximilians-Universität, Würzburg, Germany.
   The peripheral nervous system (PNS) is the target for a heterogenous immune
attack mediated by T-cells, B-cells, and macrophages.

       The interaction of the humoral and cellular immune system with the
structural components in the peripheral nervous system may determine the extent
of inflammation and possibly repair mechanisms. The animal model experimental
autoimmune neuritis (EAN) allows detailed study of the various effector pathways
and tests novel therapeutic strategies in vivo.

       Unexpectedly, involvement of the immune system is also found in animal
models for inherited neuropathies and in its human counterpart
Charcot-Marie-Tooth (CMT) disease, suggesting an autoimmune reaction triggered
by the genetically determined demyelinating disorder. A better understanding of
immune regulation and its failure in the peripheral nervous system may help to
develop more specific and more effective immunotherapies.
   PMID: 12062260 [PubMed - indexed for MEDLINE]


      Related Links
    Mechanisms of immune regulation in the peripheral nervous system. [Brain
Pathol. 1999]
    Role of immune cells in animal models for inherited peripheral neuropathies.
[Neuromolecular Med. 2006]
    Experimental allergic neuritis (EAN) as a model for the immune-mediated
demyelinating neuropathies. [Rev Neurol (Paris). 1996]
    [The role of the immune system in hereditary demyelinating neuropathies]
[Nervenarzt. 2005]
    Mechanisms of disease: inherited demyelinating neuropathies--from basic to
clinical research. [Nat Clin Pract Neurol. 2007]
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-->

It's not the destination...It is the Journey!

---------------------------------
Boardwalk for $500? In 2007? Ha!
Play Monopoly Here and Now (it's updated for today's economy) at Yahoo! Games.

[Non-text portions of this message have been removed]

Sal....my pleasure and you are quite welcomed.  This was the latest
at PUB MED...on the topic
and a very indepth article...which may assist Erin or others with the
same situation.

Dawn :-)





Sal wrote:
>
> Good article.  Thanks Dawn.
>
> Sal
>
>
> --- In CMT-Support@yahoogroups.com, Dawn <spiritfree_dawn@> wrote:
> >
> > Erin this is the latest article done in 2997 on CPAP in CMT
> >
> >
> http://www.ncbi.nlm.nih.gov/sites/entrez?
Db=pubmed&Cmd=ShowDetailView&TermToSearch=17294338&ordinalpos=1&itool=
EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
> >
> >
> >    [input] 1: Lung. 2007 Jan-Feb;185(1):1-7. Epub 2007 Feb 9.
Links
> >
> >      Disorders of pulmonary function, sleep, and the upper airway
in
> Charcot-Marie-Tooth disease.  Aboussouan LS, Lewis RA, Shy ME.
> >   Department of Pulmonary & Critical Care Medicine, Cleveland
Clinic
> Foundation, 26900 Cedar Road, Suite 325-S, Beachwood, OH 44122, USA.
> aboussl@
> >   Charcot-Marie Tooth disease (CMT) encompasses several inherited
> peripheral motor-sensory neuropathies and is one of the most common
> inherited neuromuscular diseases. Charcot-Marie-Tooth disease can be
> associated with several disorders that may be encountered by the
> pulmonary physician, including restrictive pulmonary impairment,
sleep
> apnea, restless legs, and vocal cord dysfunction. Restrictive
> pulmonary impairment has been described in association with phrenic
> nerve dysfunction, diaphragm dysfunction, or thoracic cage
> abnormalities. Central sleep apnea may be associated with diaphragm
> dysfunction and hypercapnia, whereas obstructive sleep apnea has
been
> reported as possibly due to a pharyngeal neuropathy. Restless legs
and
> periodic limb movement during sleep are found in a large proportion
of
> patients with CMT2, a type of CMT associated with prominent axonal
> atrophy. Vocal cord dysfunction, possibly due to laryngeal nerve
> involvement, is found in association with
> >  several CMT types and can often mimic asthma. There may be
special
> therapeutic considerations for the treatment of those conditions in
> individuals with CMT. For instance, bi-level positive airway
pressure
> may be more appropriate than continuous positive airway pressure
> (CPAP) for the treatment of sleep apnea in the individual with
> concomitant restrictive pulmonary impairment. The prominence of
> peripheral neuropathy as a cause of the restless legs syndrome in
CMT
> may justify treatment with neuropathic medications as opposed to the
> more commonly recommended dopaminergic agents. The risk of
progression
> to bilateral vocal cord dysfunction in CMT and the risk of
aspiration
> with laryngeal neuropathy may limit the therapeutic options
available
> for vocal cord paralysis.
> >   PMID: 17294338 [PubMed - indexed for MEDLINE]
> >
> >
> >      Related Links
> >    Diaphragmatic dysfunction in siblings with hereditary motor and
> sensory neuropathy (Charcot-Marie-Tooth disease). [Chest. 1987]
> >    Intermittent positive airway pressure by nasal mask as a
> treatment for respiratory insufficiency in a patient with
> Charcot-Marie-Tooth disease. [Acta Clin Belg. 2006]
> >    [A case of Charcot-Marie-Tooth disease (CMT) type 1 complicated
> by diabetes mellitus (DM) showing bilateral phrenic nerve palsy]
> [Rinsho Shinkeigaku. 2002]
> >    Charcot-Marie-Tooth disease with diaphragmatic weakness.
[Intern
> Med. 1992]
> >    Charcot-Marie-Tooth disease and sleep apnoea syndrome: a family
> study. [Lancet. 2001]
> > See all Related Articles...  = 4) {
> if(navigator.appName=="Netscape") {
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> window.innerHeight) { H=window.innerHeight-50;}       }else{
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+',height='+String(H)).focus();
>     }    -->                 Display
> SummaryBriefAbstractAbstractPlusCitationMEDLINEXMLUI
> ListLinkOutASN.1Related ArticlesCited in BooksCancerChrom
LinksDomain
> Links3D Domain LinksGEO DataSet LinksGene LinksGene (GeneRIF)
> LinksGenome LinksProject LinksGENSAT LinksGEO Profile
LinksHomoloGene
> LinksCoreNucleotide
> >  LinksCoreNucleotide (RefSeq) LinksEST LinksEST (RefSeq) LinksGSS
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Good article.  Thanks Dawn.

Sal


--- In CMT-Support@yahoogroups.com, Dawn <spiritfree_dawn@...> wrote:
>
> Erin this is the latest article done in 2997 on CPAP in CMT
>
>
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSear\
ch=17294338&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.\
Pubmed_RVDocSum
>
>
>    [input] 1: Lung. 2007 Jan-Feb;185(1):1-7. Epub 2007 Feb 9.     Links
>
>      Disorders of pulmonary function, sleep, and the upper airway in
Charcot-Marie-Tooth disease.  Aboussouan LS, Lewis RA, Shy ME.
>   Department of Pulmonary & Critical Care Medicine, Cleveland Clinic
Foundation, 26900 Cedar Road, Suite 325-S, Beachwood, OH 44122, USA.
aboussl@...
>   Charcot-Marie Tooth disease (CMT) encompasses several inherited
peripheral motor-sensory neuropathies and is one of the most common
inherited neuromuscular diseases. Charcot-Marie-Tooth disease can be
associated with several disorders that may be encountered by the
pulmonary physician, including restrictive pulmonary impairment, sleep
apnea, restless legs, and vocal cord dysfunction. Restrictive
pulmonary impairment has been described in association with phrenic
nerve dysfunction, diaphragm dysfunction, or thoracic cage
abnormalities. Central sleep apnea may be associated with diaphragm
dysfunction and hypercapnia, whereas obstructive sleep apnea has been
reported as possibly due to a pharyngeal neuropathy. Restless legs and
periodic limb movement during sleep are found in a large proportion of
patients with CMT2, a type of CMT associated with prominent axonal
atrophy. Vocal cord dysfunction, possibly due to laryngeal nerve
involvement, is found in association with
>  several CMT types and can often mimic asthma. There may be special
therapeutic considerations for the treatment of those conditions in
individuals with CMT. For instance, bi-level positive airway pressure
may be more appropriate than continuous positive airway pressure
(CPAP) for the treatment of sleep apnea in the individual with
concomitant restrictive pulmonary impairment. The prominence of
peripheral neuropathy as a cause of the restless legs syndrome in CMT
may justify treatment with neuropathic medications as opposed to the
more commonly recommended dopaminergic agents. The risk of progression
to bilateral vocal cord dysfunction in CMT and the risk of aspiration
with laryngeal neuropathy may limit the therapeutic options available
for vocal cord paralysis.
>   PMID: 17294338 [PubMed - indexed for MEDLINE]
>
>
>      Related Links
>    Diaphragmatic dysfunction in siblings with hereditary motor and
sensory neuropathy (Charcot-Marie-Tooth disease). [Chest. 1987]
>    Intermittent positive airway pressure by nasal mask as a
treatment for respiratory insufficiency in a patient with
Charcot-Marie-Tooth disease. [Acta Clin Belg. 2006]
>    [A case of Charcot-Marie-Tooth disease (CMT) type 1 complicated
by diabetes mellitus (DM) showing bilateral phrenic nerve palsy]
[Rinsho Shinkeigaku. 2002]
>    Charcot-Marie-Tooth disease with diaphragmatic weakness. [Intern
Med. 1992]
>    Charcot-Marie-Tooth disease and sleep apnoea syndrome: a family
study. [Lancet. 2001]
> See all Related Articles...  = 4) {
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>  LinksCoreNucleotide (RefSeq) LinksEST LinksEST (RefSeq) LinksGSS
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5102050100200500Sort ByPub DateFirst AuthorLast AuthorJournalSend
toTextFilePrinterClipboardE-mailOrder
>
> It's not the destination...It is the Journey!
>
> ---------------------------------
> Tonight's top picks. What will you watch tonight? Preview the
hottest shows on Yahoo! TV.
>
> [Non-text portions of this message have been removed]
>

>           Erin....perhaps this may shed some light on the topic.
>   Dawn
>
>
>    [input] 1: Acta Clin Belg. 2006 Jul-Aug;61(4):176-81.     Links
>
>      Intermittent positive airway pressure by nasal mask as a
treatment for respiratory insufficiency in a patient with Charcot-
Marie-Tooth disease.  Darquennes K, De Jonghe P, Daems D, De Backer
W, Verbraecken J.
>   Department of Pulmonary Medicine, University Hospital Antwerp,
Wilrijkstraat 10, 2650 Edegem, Belgium. karendarquennes@...
>   Charcot-Marie-Tooth disease (CMT) is a slowly progressive
hereditary neuropathy characterised by degeneration of motor and
sensory peripheral nerves resulting in distal muscle weakness with
atrophy and sensory impairment. We report a 35-year-old woman with
CMT presenting with respiratory failure due to a pneumonia, sputum
impaction and insufficient cough reflex. After recovery, we diagnosed
a very severe restrictive lung function disturbance caused by muscle
weakness and a possible coexistent unilateral diaphragm paralysis. A
very severe REM (Rapid Eye Movement Sleep) related sleep hypopnea
syndrome was successfully treated with Nasal Intermittent Positive
Pressure Ventilation (NIPPV).
>   PMID: 17091914 [PubMed - indexed for MEDLINE]
>
>
>      Related Links
>    [A patient of Charcot-Marie-tooth disease with rigid spine and
respiratory failure] [Rinsho Shinkeigaku. 2000]
>    Aortic root diameter and nasal intermittent positive airway
pressure treatment in Marfan's syndrome. [Clin Genet. 2003]
>    Disorders of pulmonary function, sleep, and the upper airway in
Charcot-Marie-Tooth disease. [Lung. 2007]
>    Charcot-Marie-Tooth disease with diaphragmatic weakness. [Intern
Med. 1992]
>    Diaphragmatic dysfunction in siblings with hereditary motor and
sensory neuropathy (Charcot-Marie-Tooth disease). [Chest. 1987]
> See all Related Articles...
>
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