some confrence notes / just NOTES /long

[Guest guest]

First ...these are just my notes ...incomplete , with most info on slides not

included like symtpoms lists , medications names ect ( I couldnt keep up with

both the speach and slides ) .. many spelling errors , and a bit of

paraphrasing .... PLEASE UNDERSTAND there are bound to be mistakes ... DON't

take em as gospel , complete or accurate !!

I hope eventually ASAP will have the ability to include written synopsis

/lecture notes with either the tapes ..or ideally when the confrence lectures

are happening ...I DON't want any of our fine and wonderful Dr's upset that

this isn't accurate or complete ...so if you print or share this , please

also include this introduction !!..corrections are welcomed from anyone there

..

I highly recomend getting a tape of any lecture you might be intrested in

....there are big gaps below .

Asap Friday lectures

Dr Milhorat

Neurological Aspects of Sm

few disorders /diseases have more complex dynamics

the extent of cavanation effects depend on extent of sm , and associated

conditions that may also be present

clasifications 3 types , first identified by pathology

1 communication with the 4th ventricle /almost always has hydro associated

,,hydro is cause most often , may have acm 2 and spinal bifida as well , not

the most common form , but important because you divert the spinal fluid

instead of decompresion

2 non communicating ... acm1 , basilar invagination , arachnoiditis ect are

some underlying conditions

this form has a different pathahology and tx is through decompresion

/lamenectomy

3 . primary parchmya of the cord dammaged through trama ...injury

or arachnoiditis may cause csf obstruction in this type

central canal dialation can have few symtpoms , even if it takes up about 60

-70 % of room , since cord has room to expand out that far

pain /temp fibers may be affected ..touch , pain , temperature sensation ect

...these areas of nerve pathway are on the outside of the cord tissue , so

more vunerable to dammage through compresion and can remain after sm has

colapsed .

if sm is big , can affect movement ect too

if it ruptures , it can go into the cord tissue and affect the nerve

connections ect , this can cause major sensory impacts , pain ... 70 % go

into the dorsal , lateral area of the cord .. patient will present with

various types of pain

moter pathways are in the lateral aspects ..rupture here causes atrophy

weaknees , motor problems , atrophy , pain , sensory can all be found with

major sm problems

dyskinesthetic pain issues

40% have burning , hypersensative to touch presure ect , 1/3 develope tropic

changes , skin color /temperature changes ..seen in lateralisation of sm into

dorsal cord ..60% find some relief , 40 % remain troubled following surgery

....aka caulsailia and reflex sympatheic dystrophy ( rsd ) .

substance p is found in the dorsal cord ...study shows normal distrubution is

changed , and it accumulates in the cord ...it may be related to the pain

problems ( its involved in pain perception )

more drug interventions being studied now , pending fda approval , ( several

seem close to approval ) they may work better than what is currently

available for fibro /sm /ms ..dysethetic pain conditions

the cord sm may cause symtpoms , but other accompaning problems can be the

etiology of neuro symptoms too ..

one example would be trama= paraplegia in spinal cord injury

acm is the most common symtpoms cause , ,,,but other congential /trama ect

may be the primary cause , a physician must sort out all the etiologys

involved before you can best plan and address interventions

factors

etiology--congential , trama , meningitis

determine the pathology of cavitation

are there associated lesions

secondary skeletal derformities

the physician can't assign sm cause /relationship to symtpoms untill the

accompaning factors are sorted out fully ....

acm 1

addressed his acm 1 redifined study

the posterior fossa is about 15cc's of csf to small in volume

old dx standards -3-5 mm herniation or more ,was the minimum thought to cause

problems ...this is changing in understanding now

2 acm types ,within acm 1 , small posterior fossa , or normal posterior

fossa but tumor , hydro , ect present

pseudotumor syndrome following chiari surgery :

1symotoms : activity related headaches , retroorbitial pain , nausea and

vommiting

2 nd when LPis done csf presure above 17-32 cm H 2 O is an indication

treatment is a vp shunt

neurological workup of idiopathic sm

1. full mris scans a. brain b. cervical spine . c thoracolumbar spine ..

all should be done , before the physician can sort through what issues may be

present .

2. CINE MRI

3, Gadolinium- enhanced MRI 's

4 Cat scan myelography

You need ALL of these during workup to be sure you've found all the pieces of

the puzle when sm is found ( with no obvious acm )

CINE MRI helps be sure ..it can demonstrate occlusion even when it appears

there is room for flow in some folks

Gadolium helps rule out tumors ..could also show inflamation by enhansing

lessions , can demonstrate leaks

The Goal is to find and correct the blockage of csf , treat the cause , not

the effect . This is really important .

Dr Arnold Menezes

Basilar Invagination / Anterior Compresion

skull base abnoralities are his strong intrest ,

volume of posterior fossa is critical ..if entrouchment , there is a

limmitation ( in room ) for the brainstem and spinal cord .

anterior compresion problems ..( from the front )

if the clivus is in the wrong position , it reduces the room available , the

brainstem may show a kink .

congential , shortening of the front part of the skull ...much less space

odontiond process has climbed up

basilar invagination ...two types

1. it is primary , not a result of something else

2. it can also be aquired through other disease processes

the goal is to eliminate the impingment , one problem is instability , and

surgery to remove the bone impinging is the only solution ...it disrupts the

flow of csf , and may cause hydrocephalus as well .

problems include blood suply to the brain as well , not just csf occlusion .

it can cause strokes ect too

it can be an associated abnormality to hindbrain herniation ( acm )

...cervical medulary buckle ( kinking ) from presure in the front , adds to

small volume problems

1 acm can be caused when the first cervical bone ( c1 ) doesn't seperate

from the skull

2. acm with cvj anomalies become symtomatic if canal diameter is below 19 mm

3. abnormal ventral bone pathology is reduceable in children with atlas

assimilation , and later is irreducible should early dorsal decompression

and fusion be done when first symptomatic

Update on research ...Dr Barth Green

Chair of neurosurgury at U. of Miami

his intrest in sm developed first through trama patients

neuroscience research is exploding now

current challenges include ethics and morals disagreements , strategic

challenges , rationing of health care , stem cell debates ect

there is currently no easy fix but its goose bump time now , stem cells

,transplants ect are opening a huge variety of options

not much acm /sm research being done yet , asap will be changing this as we

commit this week to funding projects

technological considerations include

brain and cord imaging

csf flow dynamics

posterior fossa measurements

dural substitutes ( patching )

scar tissue repression

surgical technology

surgical neuro- physiological monitoring

pain control

rehabilitation

monitor sensory and motor functions of the cord are done as as part of

surgery these days . human ?? ( couldnt keep up here )

rehab

neuroprotection strategies

neuroprotective agents

transplants on the horizon , stem cell transplantation to reverse

degenerative and genetic diseases

will also help with altzhiemers , parkinsons, als, ms , diabetes

stem cells have the potential to reverse /repair /bridge the dammaged cells

pain ..nsaids help with inflamation

celebrex - narcotics

Pain in sm ..Dr. Kula

Associate Professor of Clinal Neurology clinical neurologist ( works with Dr.

Milhorat )

pain in sm and acm

neuropathic pain ...periferial nerve pain caused by other diseases more

often discussed /studied , but the research helps us too with acm /sm

the good news is they are learning how pain is representedi n the nervous

system , the out look is good for more aggresive pain mannagment in the near

future .

Pain is an umpleaseant sensory and emotional experience associated with

actual tissue dammage ..its often described in terms of such dammage

pain is an important thing as a signal tissue is being dammaged

the nervous system must decide if it's important

chronic pain ...is trigered by persisting stimlation of nocieptors in areas

of the nervous sysstem

the nervous system is good at not responding to sensations ...it screens out

what isn't important when working right ..examples might be ignoring a

conversation in the same room while you consentrate on your work ..without

this , we would be overcome by constant messages that we routinely learn to

ignore

if a message pathway goes haywire , we may be driven into chronic pain

syndromes...neuropathic pain ...

inputs into the nervous system is through the dorsal part of the cord ...its

a microprocessing system

deep presure , superficial presure , breeze or light touch ect can trigger

neuropathic sensations that are very intense

the c fibers send severe pain signals known as slow pain ...this can cause

the jerk from hot water ...travels slowly to an emotional component of the

brain intended to drive the nerves to respond and be protective

transmitter substances help amplify the sensation .. makes us alert and focus

on what needs attention .

the pathways are on both sides of the spinal cord ...helps identify what may

be responcable ..these tracks are less sensative than the brain cells

....axons are reslilinet , but the cells are more vunerable

sensitation and activation of " healthy " endings of cells , and recruit

responce from surounding cells and the area they serve ( hense jerking back

from something hot for example )

neuropathic is dysregulated transmision of these signals

mixed of sensation helps distinguish what is troublesome

disturbances in the central cord include slow responce transimission that

leads to amplification in the central cord /dorsal horn ...can lead to severe

pain syndromes ...happens before cognitive process is involved ( jerk away

from burning ) ...rapid pathway reacts , but the remainer of the signal moves

upstream to cognitive process ...

subsance p is involved in the body learning /sorting out this ..if dammaged

it says it can't be shut down , cells are recruited to send more insistant

help messages and things intensify

decending pathways have opioid binding ( these send messages back down from

the brain )

these pathways are what says its ok .. they restrain signals continuing

...dammage to interneurons is at a local level

enkephalins / substance P modulate pain ,when dammaged, they don't get the

message its ok back home where the signal started ..when this happens where

there is no shutdown of the pain allert responce signal ...its actually

amplified and the pain increases

central sensatation ...has an emotional quaility , adversion /anxiety

develops , then shifts to a suffering state

at the margin of sm , substance p is elevated ..it is responcble for the

maladdaption of pain responce

pathways become reorganized

scope of pain :

sensory , affective , mood stress

pain to the extent that it produces suffering is always in your head ...that

doesn't say its crazy .. your brain is what interprets the signals and

sensation , so its in your head

sensation ,its affect and and cognitive processes are all involved ...what

does it mean is how do we deal with it , may include cognitive and emotional

reactions , your spirtual responce , social /enviremental issues , finacial

and litigation issues

new functional studies show that

the amegyla (?? wrong spelling - amagadalya ? ) area of brain interprets what

pain means ..flight of fight syndrome is a good example

hypocampus

thalmus , and cortex are also involved

sm /acm may involve reorginsation of info into the brain

examples include burning , tingling , electric shock sensations

fibro pts have exagerated responce to mild presure for example

c fiber is the agonising /excrutiating pain source

assessment of pain :

dx workup

imaging studies

tx rational ..promptly improve the patients life ...jump in early before

it's long term changes that can't be relieved so easily

tx depression if present , makes things worse untreated ( depression may be

chronic pain caused /related dirrectly )

therapy types / approaches

pharmacology . psych , physiatrist , surgery

tylenol, asprin , ultram , motrin , celebrex ect. dont help much

nsiads address inflamation /don't help neuropathic pain

toradol /antidepressants may well be of good help because they help modifiy

relay systems to address many problems in the brain /nervous system

elavil /tricyclics work best .. may be toxic , cause someone to be drousy ,

cognitive impacts are troublesome for some folks

neurontin /antiepilectics

up to 6-8 grams of neurontin are now being used by pain specialists

baclofen

topamax may be helpful

mexilitine

e3 ( not sure what i ment by this one , ack !)

clonadine

tizaninde /zanaflex

dextromethophan

dronabinol

calcintonin ..helping in fibro

opioids Drs . are now again finding these are very effective

percocet ect

Patients may ask for more meds because they are undertreated , not as a

result of addiction .. a common problem not well understood with Dr.s who

don't work with these conditions

look at other suportive therapys , and explore if they will also help

Peds in Acm 1 and 2

Dr Mc Comb

hydrocephas specialist

head of peds neurosurgery at UCLA

syrinx .. a fluid filled cavity outside the central canal , not lined by

ependymal ( not sure i got this name /spelling right ) cells

hydromyelia , is when it's a dialated canal , with normal lining of cells

most sm is a combo of these two conditions really .

arachnoiditis can cause high protien content in csf fluid ( my note --folks

who may have ms , or suspect arachnoiditis , heads up on this one )

I missed the rest of his speach ...sorry you'll need the tape .

Dr Ellenboggen

Chronic fatique and Fibromyalgia

U of Washington ..relationship between chronic fatique /fibro through

exploration of this with rheumatoligists in research at U of Washington

Rosner and Heffez got his intrest when the wall st journal article was

published , then the media flurry of attn followed .

fact or fiction questions arose in his mind , and others ...leading to the

research .

the claim that decompresion of posterior fossa leads to improvment in fibro

and chronic fatique was intresting , worth study

Questions ...

How do you know ??

What is the real question ?

What is the frequency of hindbrain herniation in the population of folks with

chronic fatique ?

U of Washingtom has NIH funding to study chronic fatique .. Dr E called the

Dr. studing cf /fibro ...

the cause of the fatique is unexplored , and mostly unknown .

there is an overlaping population with fatique but different disorders ...

there is a csf overlap in these patients with some csf changes that may be a

common problem

chronic fatique is not relieved by rest .. for diagnosis there MUST be NO

evidence of other diseases

onset of fatique , lasting at least a 6 month period , impariment of

cognitive processes

triggers , fever , sort throat , virus infection ect

symptoms , myalgias , morning stiffness , sleep disturbance ,

Fibro ( I skiped notes where the dx criteria of both chronic fatique and

fibro were discussed more in debth ...chronic fatique dx is available through

reumatologist web info ...fibro through the nationl center for disease

control info )

lots of overlap ..11 of 18 tenderspots

widespread discomfort /pain

tx ..symptomatic relief ...no known primary treatment at this time for

either one .

chronic fatique and fibro overlap each other , many folks meet criteria for

both

then for is . chiari is thrown into the mix

chiari is a DIFFERENT diagnosis that may have overlaping symptoms

contraversy exists because the changes of acm understanding are quite

profound in the last 10 years

a recent survay of peds nsgs showed no aggreement of what is an acm problem

with regard to both MRI herniation and symptoms

MRI allowed great changes in understanding ... CINE MRI expands on this

sm may vannish with decompresion ..there is a small group that actually have

this happen . Dr M's paper helped clarify this .

in his study 32 % had herniation less than 5 mmm but had sm

herniation of one or more tonsils below 5 mm , with sm ..= acm , so

understanding is now that csf flow is the real problem ...this is a

physiology problem ...understanding is changing now ...

at U of W they look at twins through studys

if only one has problems with a chronic fatique diagnosis

they designed a study ..the question being " is there an anatomical problem

that can be indentified " ...and " are they misdx folks " ...Dr E doesn't know

" never try to teach a pig to sing "

it wastes your time and annoys the pig

Q and A session

Dr. Kula

upjohn company is making a med to block substance P ..seems to be normal in

chiari ...( not normal in sm or ms though )

Reumatologists are the ones studing this

4 meds that now look good , that Dr Milhorat will provide info on to

at the asap office ..call and ask her since he didn't have the names

at the confrence

post polio syndrome symptoms are much the same ...nerves overworked and

wearing out faster ..rest , ergonomic aids ect are helpful ? still under

investigation ...may have the same impacts ..but for the moment go with the

assumption exercise is good for everyone ...stay within your limmitations ect

..

*question refrased I think refering to post polio syndrome study

nerve distrition process ..could this be the same in sm ?, where exerise

could cause more harm than good ? again , no clear answer at this time

scaring is genetically controled ..gortex may not be the full answer

( results have been disapointing with some patients )

Dr Green responding here ... his work shows positioning may be the most

important aspect that prevents teathering . he discussed what hes now doing

....I missed this part , I think he was refering to spinal surgery not

decompresion .

( end of notes )

I'll send the next batch when I can get to cleaning em up.