Note: These Answers From Doctors Cannot Be Reprinted In Any Way.
- I have heard some people talk about having Autonomic Failure.....from what I have read/heard about it has a lot to do with POTS. I also thought POTS was involved with Chiari and Instability issues.....First, I was also told that the fusion usually helps the POTS. But then I've heard from people on the boards that say that POTS and Autonomic Failure are related to EDS and have nothing to do with ACM/CCI. Do you know the answer to this?
- Is it safe to have a lumbar epidural injection at the L5/S1 level with a 2mm C6/7 syrinx?
- The issue of thyroid/adrenal and basic pituitary function has come up on the list.
What specific tests we should/could be doing in this regard. Also, could someone have flattening of the pituitary gland if they had a systemic disease? Would it appear the same? And could someone have 2 problems causing the flattening?
- A question in regards to especially EDS by a member of the support group:
"My 14 year old son with CM (decompressed at age 5 with TCI doctors)
also has EDS-hypermobility type. In the past I had noticed blotches
resembling a rash and he currently (for the past week or more) has
these blotches on his stomach and the top of one foot. The blotches
don't seem to change (or move), vary in size and are pale pink/reddish
color.I wondered if there was any chance this might be related to EDS.
Also, certain areas of his body (like his legs) will get very red in
the shower (even if the water is only warm)."
- Has anyone ever read anything about Vitamin C being good for those with EDS?
- I had duraplasty with a bovine graft and my neurosurgeon said there can be possibilities of scar tissue build up at that sight. Can anyone make an educated guess as to a timeframe on how SOON post op scar tissue can begin to build up?
For example, I am 19 months post op and experiencing a reoccurrence of some of my Chiari symptoms and wonder if scar tissue could be the issue.
- After decompression and fusion surgery, what changes happen to the nerves in the brain during recovery.
For example, the nerve sensations a lot of us get makes our body temperature seem so cold. Is this normal and what can be done?
- After decompression surgery what would cause a patient to still suffer from balance issues.
For instance after sitting for long periods of time, once going from one position to another, it is difficult to maintain balance, not necessarily falling but almost like a drunk sort of stagger into a wall or object.
- Could you discus the finding of arachnoid cyst, in regard to CM, syrinx, etc.?
- The very important "waiting for Chiari surgery" topic
- How are "severe symptoms" defined?
I have heard some people talk about having Autonomic Failure.....from what I have read/heard about it has a lot to do with POTS. I also thought POTS was involved with Chiari and Instability issues.....First, I was also told that the fusion usually helps the POTS. But then I've heard from people on the boards that say that POTS and Autonomic Failure are related to EDS and have nothing to do with ACM/CCI. Do you know the answer to this?
September 2009
Dr. Bolognese:
"POTS and autonomic failure (AF) can occur along with a number of disorders
including EDS, CMI, and craniocervical instability.Different disorders can cause POTS and AF
by different mechanisms. Some disorders like EDS can cause POTS and AF with more than one mechanism.
Poor sympathetic tone, suboptimal vascular constriction, impaired neurovegetative reflexes are
treated conservatively.
Other mechanisms can be treated surgically; the best example is when the tip
of the odontoid compresses the part of the brainstem called medulla (where
an important nucleus devoted to the regulation of the overall vascular tone
is located, along with circuitry controlling the heart rate); if cervical
extraction can reverse such compression, then a craniocervical fusion can
help.
About these special cases, TCI has treated over the years a number of
patients who could not get up from their own beds for more than a few
minutes at the time, and now can stand and go around for up to 6 hours at a
time. "
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Is it safe to have a lumbar epidural injection at the L5/S1 level with a 2mm C6/7 syrinx?
Dr. Michael Rosner:
"Doubt there is any danger in the lumbar epidural with a small syrinx. Most likely adverse result is back pain does not c
hange or worsens. Should consider the possibility of the back pain being amplified by brainstem or cervical cord
compression which may alter pain thresholds-as in the Chiari/stenosis patient misdiagnosed as "Firbromyalgia". Back pain
one year after Chiari decompression often improves substantially."
"Rosner, Michael J., Flechas, J and Bailey, R.K. Neurosurgical Subsets of Fibromyalgia: Part III: Functional and symptomatic
outcome at 12 months post-operatively. J Musculoskeletal Pain, 9 (S5): 32, 2001.
Flechas, J and Rosner, Michael J. Neurosurgical Subsets of Fibromyalgia: Part IV: Long term outcome as measured by dolorimetry.
J Musculoskeletal Pain, 9 (S5): 29, 2001."
Dr. Bolognese:
"A Pituitary function test and a battery of stimulation tests will do;
For more inblems in CMI" on the TCI website."
Dr. David Frim:
"Several of our patients with syrinx at various spinal levels have had complication free epidural injections. However,
there is the usual success rate of the injections which can be low."
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The issue of thyroid/adrenal and basic pituitary function has come up on the list.
What specific tests we should/could be doing in this regard. Also, could someone have flattening of the pituitary gland if they had a systemic disease? Would it appear the same? And could someone have 2 problems causing the flattening?
September 2009
Dr. Michael Rosner:
"Large number of Chiari patients have endocrine dysfunction, though systematically collected data does not exist.
Hypothyroidism of many different causes, diabetes, dysmenorrhea, endometriosis, fertility problems, multiple miscarriages,
and a large percentage of children with Chiari who are pan hypopituitary all suggest dysfunction of the hypothalamic-pituitary
axis. Many patients are orthostatic intolerant, have Positional Orthostatic Tachycardia Syndrome (POTS) or Neurally Mediated
Hypotension (NMH) and Raynaud's phenomena which in most tend to improve after decompression. Often menstrual abnormalities improve.
We have seen a number of younger women develop larger breasts, acne and improved libido. Diabetes and reactive hypoglycemia may
become more easily managed.
The empty sella is often missed in the evaluation of Chiari, has traditionally been dismissed as being of little to no consequence,
but is indicative of high intracranial pressure--which is typical of Chiari. I find the empty sella or partially empty sella
association too coincidental and feel that it is a direct link with at least some of the endocrinological dysfunction seen
in this group. Additionally, a part of the problem may lie in the direct input from the brainstem (potentially impaired) to the
hypothalamus which then controls the pituitary rather directly. Still, most of this is conjecture."
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A question in regards to especially EDS by a member of the support group:
"My 14 year old son with CM (decompressed at age 5 with TCI doctors)
also has EDS-hypermobility type. In the past I had noticed blotches
resembling a rash and he currently (for the past week or more) has
these blotches on his stomach and the top of one foot. The blotches
don't seem to change (or move), vary in size and are pale pink/reddish
color.I wondered if there was any chance this might be related to EDS.
Also, certain areas of his body (like his legs) will get very red in
the shower (even if the water is only warm)."
September 2009
Misao Nishikawa, MD, Ph. D.:
"I think that its blotches seem to be inflammatory or immunological reaction in dermis from your mentions, so that might not
be related to EDS. I recommend you have consultation to Dermatologist."
Dr. Bolognese:
"It helps a bit but is not a panacea"
Dr. Michael Rosner:
"Likely reason is autonomic instability or dysfunction of the sympathetic vasoconstrictor system. It may over-react to heat-cold
stimuli or spontaneously and lead to blotchy, reticulated or diffusely reddened skin changes. Pressure on the red areas may cause
them to pale with return of the changes with release of pressure. If this happens, the color changes are due to blood flow changes
in the skin. Not related to EDS in my opinion. Often associated with palpitations, panic attacks, orthostatic intolerance, NMH,
POTS, etc."
Rosner, Michael J., Flechas, J and Bailey, R.K. Neurosurgical Subsets of Fibromyalgia: Part III: Functional and symptomatic outcome at
12 months post-operatively. J Musculoskeletal Pain, 9 (S5): 32, 2001.
Michael J. Rosner, Jorge Flechas, Royce K. Bailey. Neurally Mediated Hypotension (NMH) and Positional Orthostatic
Tachycardia Syndrome (POTS): Part III: Symptomatic and functional outcome one-year after surgery. (Abstract, AANS
Annual meeting 2002)
Michael J. Rosner, Jorge Flechas, Royce K. Bailey. Treatment of Neurally Mediated Hypotension with Surgical Decompression
of the Hypoplastic Posterior Fossa: Twelve month outcome with repeat tilt table results (Congress of Neurological Surgery, 2003 Meeting)"
Dr. Clair Francomano
"I have seen several patients with EDS who have had unexplained rashes/blotches on their skin. I do not have an explanation for
why they occur."
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Has anyone ever read anything about Vitamin C being good for those with EDS?
September 2009
Dr. Clair Francomano:
"The question of vitamin C in EDS has been raised several times. As far as I know, there has never been a controlled study done to
see if it has a positive effect. However, Lines Pauling has written about the crucial role that vitamin C plays in collagen synthesis.
I am attaching two documents from the website of the Vitamin C Foundation that cite Lines Pauling and Roger Williams' writings about
the relationship of vitamin C to collagen metabolism. It seems very reasonable to be sure a person with EDS is taking adequate amounts
of vitamin C. Current recommendations for daily consumption range from 75 mg (US government) to 3000 mg (Vitamin C Foundation). I think
500 - 1000 mg is a reasonable dose to start. Some people experience gastrointestinal symptoms (abdominal discomfort, diarrhea) with too
much vitamin C, but it is unlikely to occur at this level."
Misao Nishikawa, MD, Ph. D.
"Although Vitamin C is co-enzyme for producing collagen, because EDS has the essential problems in which collagen is basically
produced, vitamin C is unlikely to be effective for EDS directory. Vitamin C is certain of some support to make collagen, however
excessive taking vitamin C is meaningless."
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I had duraplasty with a bovine graft and my neurosurgeon said there can be possibilities of scar tissue build up at that sight. Can anyone make an educated guess as to a timeframe on how SOON post op scar tissue can begin to build up?
For example, I am 19 months post op and experiencing a reoccurrence of some of my Chiari symptoms and wonder if scar tissue could be the issue.
September 2009
Dr. Michael Rosner
"All wounds heal with scar tissue. The bovine graft, in my opinion, incites little in the way of additional scar.
Scar formation starts early, traditionally is maximal at 6-8 weeks and then the scar matures over the next year or so.
My experience: recurrent symptoms, if consistent and progressive, are usually due to hydrocephalus, concomitant cervical
disease or inadequate surgery"
Dr. Bolognese:
"Post op scarring start on the Post op day 1, and evolves over time, ending in its definitive form in about 12-15 months"
Dr. David Frim:
"Scar tissue can build up around a foreign body patch graft as early as a few months and cause symptoms up to several years
later. We have seen bovine patches that we have needed to remove due to scar build-up and fluid obstruction anywhere from a
few months to 10 years or more."
Misao Nishikawa, MD, Ph. D.
"Scar tissue formation can be divided into three parts according immuno- responses.
1. hyper-acute response; the immunological response for invasion and/or foreign body (bovine graft)(reversible). (a couple of days)
2. acute response; the repairing response of tissue (reversible). (a couple of weeks)
3. chronic response; persistent reaction through antibody in immunological system (irreversible). (a couple of months ~ )
Excessive scar is caused by excessive above 2 and 3 responses."
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After decompression and fusion surgery, what changes happen to the nerves in the brain during recovery.
For example, the nerve sensations a lot of us get makes our body temperature seem so cold. Is this normal and what can be done?
September 2009
Dr. Michael Rosner:
"Continued complaints of a 'cold' sensation can represent continued sensory abnormality or abnormal processing of
sensory information. They can also represent a Raynaud-like effect with abnormal vasomotor control and response.
Continued abnormality may represent permanent neurological change/damage or incomplete treatment."
Dr. Bolognese:
"Check your thyroid hormones"
Misao Nishikawa, MD, Ph. D.
"The brain stem, cranial nerves and upper cervical spinal cord are released from compression by decompression surgery and
fusion surgery. Those neural strictures include sensory nerves, parasympathetic and sympathetic pathways (autonomic nervous system)
which control perspiration, heart beats and respiration etc.). The changing (improvement) of balance in those nerves could cause
that's sensation.
I guess: You might have the improvement of parasympathetic nervous system by decompression for medulla oblongata which
includes parasympathetic pathway. By this improvement of parasympathetic nervous system caused the increasing of sweat in
the upper trunk, so you feel cold."
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After decompression surgery what would cause a patient to still suffer from balance issues. For instance
after sitting for long periods of time, once going from one position to another, it is difficult to maintain balance, not necessarily falling
but almost like a drunk sort of stagger into a wall or object.
September 2009
Dr. Bolognesse:
"Semicircular canals and raised CSF pressure and pressure of a small Posturer Fossa on the rest of the cerebellum are two of the reasons."
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Could you discus the finding of arachnoid cyst, in regard to CM, syrinx, etc.?
September 2009
Dr. Michael Rosner:
"Theoretically, arachnoid cyst is a developmental anomaly of the nervous system. However, we have seen a number of dilated subarachnoid spaces,
usually in the posterior fossa and occasionally a 'mega-supra-cerebellar cistern' which are not true cysts, but dilated spaces due to the high
intracranial pressure and abnormal spinal fluid circulation of the Chiari patient. Some have even been shunted (with poor clinical results) as
arachnoid cysts. Effective treatment of the Chiari generally takes care of the problem."
Dr. Bolognese :
"Arachnoid Cysts can cause tonsillar herniation"
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The very important "waiting for Chiari surgery" topic
September 2009
Dr. Bolognese:
"Data from literature indicated that the best outcomes are obtained
when surgery takes place within two years from the onset of severe
symptoms"
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How are "severe symptoms" defined?
September 2009
Dr. Bolognese:
"Severity is defined by the impact of the symptoms on the quality of life"
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