"Pain Management in Connective Tissue Disorders" - Pradeep Chopra, MD

[Music] for those of you who don't know me my name is Pradeep Chopra I'm a crazy pain specialist but I'm crazy because I like taking care of complex patients it just makes my day I'm sorry but yeah the psychiatrist gave up on me a while back anyway so my topic is to talk about pain and EDS and I'm gonna really go through it as much as it this is these are take-home messages so there's no blah blah data on this one of the things about EDS is that they have basically two kinds of trauma is a macro trauma and there's a micro trauma all right macro trauma is easy to identify their broken bones even my car mechanic can decide what's wrong there but it's a micro trauma that these patients go through every single day multiple times that we can't identify not only that these people don't heal well so now they're enduring their tissue their connective tissue and they're not healing well and this is happening on a not just daily basis on an hourly basis the minute they stand to walk the tissue this micro trauma the connective tissue is fragile they're there these people are very fragile and their tissue is so just it just breaks easily and it doesn't feel that's the problem just one thing what ideas don't talks about this there are some of the most wonderful people they're amazing and extremely smart I have ideas No so so neuropathic surface of them this is a pretty old study and you know they looked at a neuropathic pain in 80s and they said like okay what's the what's the reason why these people have pain you know be honest he almost joint use of boxing and joint so you have pain but they found out that about 68 percent of them had neuropathic pain and they of course at that time looked at classical and hypermobile type so they came to this conclusion that they said all right so it's neuropathic pain is much higher than nociceptive pain so no sees if there is structural pain and they didn't find any difference between classical and hypermobile which are not surprised the question is is there something genetically going on with these people that's causing the neuropathic pain we don't know though we do know that in the it has an X deficient type there is a sensory external sensory motor polyneuropathy that's what we don't notice so far that this concept of central sensitization is is limited to not just EDS but in general and chronic pain but obviously applies a lot in EDS so really what happens is that when there's a barrage of pain signals to the central nervous system the central nervous system gets sensitized and then any other pain signal however small it is is now dialed up it gets magnified so normal sensations normal sensations become abnormal have abnormal responses so we know this you know that patients have central sensitization there are most chronic pain conditions are like that hence drugs don't work as well now so so what is they tell you what happens to the central nervous system in central sensitization to come back to some biology over here there's the neuron okay and then the neuron is literally closely packed I mean the physical contact with the neuron our the glial cells and glial cells we know that the brain is is made up of 70% of them are glial cells I mean 20% of our actually nerves and so these grid cells their job is to protect this this neuron over here they are the scavenger cells part of the auto immune function they they they protect the central nervous system so in what happens in central sensitization is that these grille cells are activated and once they get activated they release all kinds of dirty stuff cytokines inflammatory mediators and these then in turn cause neuro inflammation so this is the one place where we have real cells are an important link between the nervous system and the immune system and that's where we get the whole concept of inflammation and pain in these patients for those who are present this is from my friend Jared younger who's done a lot of research and Grille cells these are the activated real cells these are inactive glial cells and they shine up anyway so your motion from fuel cells is evidence that aromatic conditions like complex regional pain syndrome almost cellular than neuronal which is why nerve blocks are really not the greatest in CRPS they don't really make a big difference is because we are treating the wrong thing is more from the cells the glial cells in the central nervous system so going on to cranial cervical instability what happens so you have a spine your spinal cord which is protected well into the spine and then there's this amazing architectural design that protects the spine as the spinal cord but at the same time makes it also gives you the flexibility and that comes with a price so the roller injury results from stretching or deformity stresses that happen now these patients with EDS have joint laxity they have and you know the spine has tons of ligaments around them we saw that this morning so when these neurons they don't get damaged but when they get stretched with an influx of calcium and then there's altered gene expression and then they finally die that's that's the fancy name apoptosis is a fancy name for dying so they die so the deformation of the brain stem and this upper spinal cord results in a bunch of things pyramidal and sensory changes I didn't put in the parable of changes but I put in the sensory changes these are the para seizures they have immense headaches neck pain weakness hyperreflexia this near on and on and on they were multitude of these conditions and it's from this now I we did talk about these angles this morning the clio axial angle and then we got the Harris measurement and the grab Maps or Oak angle the reason I put this in there is because but you wouldn't when as physicians we send our patients to be evaluated by a radiologist for pinning a cervical suspected cranium circle and stability pass the radiologists very nicely if they can measure it because measuring this angley's angles is not easy and it's not covered by insurance so they are not too excited about it so you have to ask them really nicely but these are the angles any angle lists in the private zero angle less than hundred thirty five degrees the highest measurement greater than twelve or the grabbed maps to Nokes angle more than nine millimeters is passive pathologic so dr. strick had a really nice to open spontaneous CSF leak and then not a Roth or now in interacting with hypertension I'm not going to dwell on this a lot but what I do want to mention is let's imagine the patient so EDF have low their lows and say there's quantity of CSF leak now they have spontaneously SLV and the leak keeps happening do you think it could be continued that's the question we need to answer so he they have stenosis a trap near Venus stenosis by the precious differentiate a lot now the other thing is what because may not be a normal entertainer pressure for CDSs so this they don't fall into that algorithm so again when you have a spontaneous CSF leak think of intracranial hypertension everybody thinks of hypo but it could be hyper also segmental if stability is meaning that a part of the spine usually the set harassing spine we know that the cervical spine is and the lumbar spine a very hyper mobile but the the thoracic spine can also do this so these patients develop this copper T and early degenerative changes the reason they develop only degenerative changes is because these patients don't heal as fast as other people and so what happens is the destruction continues but the reparative process is much slower it doesn't keep up with it and of course so when during flexion what happens is the lateral and the ventral column to the spinal cord get compressed to get d-formed and so during extension their spinal cord this buckling of this ligamentum flavum and then these patients and then I'm sorry the lateral major columns is during flexion and extension is buckling of the ligamentum flavum so then extend the mcgovern flower literally buckles up it bunches up and pushes on the spinal cord and of course this is much more important in the cervical and thoracic especially the thoracic spine and if they present with all the usual procedures the high procedures anything that that relates to nerve irritation and of course in late cases they start to develop more muscular symptoms like spasticity and neuronal death this is not really for Barry Goudreau Vani is that is this is the same urologist from the gioberti syndrome and we all know about you are very syndrome in this I think this is much more common than we think it is what happens is we know that the sympathetic chain the gang here are literally plastered to the cervical spine and clearly and if you do if there's any sense of flexion like in a whiplash injury for example this this column gets affected this this sympathetic chain gets affected so these patients present with symptoms of basically sympathetic overdrive and that's difficulty swallowing blurred vision tinnitus dizziness neck pains headaches and that I I think that this is one of the pathologies behind whiplash injuries a lot of these patients present with this fortunately it recovers or for some time and they're fine but in the in the ABS population we do see this a lot and they it's amazing how some of this like for example dizziness or tinnitus will will get better if they get their cervical fusion ready so moving on to temporal mandibular joint dysfunction this so so by now I have about a database of 500 patients with EDS and HSD now next they're all mixed and I we haven't analyzed the data yet but I think almost I would say about 80 to 90 percent of them have to improve mental or joint dysfunction I get surprised when they say they don't we and of course this is the area of pain that you can see is this the face the neck the head but what was interesting was that the incidence of cervical spine disorders in this patient is very high it's 70% that's amazingly high for a patient with temporal mandrel joint dysfunction something to think worked now this looks like a real nice picture of somebody with cervical radiculopathy it is the discarnation there's something going on in the spine cervical spine and now they have radicular symptoms down their arm you do an MRI of the neck this shows up or may not show up and you assume that this is cervical radiculopathy radiculitis these patients present with thoracic outlet syndrome and it's a really very high incidence of thoracic outlet syndrome the difference over here is that in the non EDS population the thoracic outlet syndrome can be from a spasm with a PEC minor muscle okay which is which is like this but it causes toxic effects and repeated it loves us neurovascular bundle going right through this area so the veins are these and the nerves get blocked by the PEC minor but in ETS earth it is more from the subluxation of this bond the the caracal and because people have this and they also need to have so these patients have pain in their extremities visceral pain is huge its massive and it's I can't even count the number of reasons they can have it but median the ones I put here are these are take-home messages median output ligament syndrome is pretty common in this is a debate about whether it's sympathetically mediated or whether it's actually actual compression of the median artery we do we don't know there's vesser up test as they I haven't seen this too many times but I think what happens is they have Lacs intestines it it drops down they have this motility which could be from this or anomia which is again very common in this group they can have it from mast cell activation syndrome it's the freaky sofa Derek is good because they're just producing boatloads of histamine so they're producing they get this good small ball understand which overgrowth SIBO is it's fairly common in this group it's just because of the descender dysmotility they have so basically what you have you have about a thousand different types of bacteria that live in a small understanding of ten thousand different types of bacteria that live in a larger user now if you're not if you're moving well these guys go over to the smaller design and cover populated and that's called Simo pelvic pain it's you trying prolapse rectal prolapse hemorrhoids dysmenorrhea vulvodynia we you know we've we've had this debate in fact i had this afternoon whatever santos is that mast cell is a tethered cord she didn't think it was tethered cord but i don't know they present with well were Delia industry for cystitis i formerly think that these patients have a mass or activation syndrome but then I understood poor pinned on mister up toises oftentimes these patients have a large colon cancers following their kids loaded and it drops a prime into the pelvis we've seen x-rays where the the travel squad is sitting in the house and when it sits there is compression compressing every known structure there so what happens is these patients I'm going to put down to the lower extremities so if they have instability other three turn angles we have flat feet they have which then throws their knees off and here what we have is to live and enjoy which then throws the hip joints off which then goes and really pulls down the spine and then you have to be where they're having back pain so almost all my back patients that come in and if I was so you know stabilizing this will make a reference to the knee joint which is all the way up this is another commonly missed condition is called the is it's approximately your favorite joints of the physicians over here and from you know right right next to it so position of that causes nerve to get and that would happen if they enter the joint versa boxing it could be less articulate the fibula from the tibia and these patients then and they present with pain going down their side of their leg so the diagnostic conundrum here is I mean but that's what it means small fiber peripheral neuropathy up there's a very close like almost more than fifty percent actually with hyper mobile type and disor anomia that's hot stance of postural orthostatic tachycardia syndrome which almost all idiot says have a very close length about 50 percent and present with small fiber neuropathy is there a genetic defect along with the idss doing it we don't know mast cell activation syndrome is definitely not a Miam condition we know that SIBO can do it they might have an empty HFR variant this is something that I've been seeing a lot in my patients with areas is 70h ever variant I'll get to that if you have some time additional regions they do I I think a lot of the sicker ones I think have a secondary mitochondrial dysfunction they are not primary but there's no point in doing a genetic testing in there but I think they do have secondary mitochondrial dysfunction this worth trying a mitochondrial cocktail but before you do that find out what their MTHFR variant is they do have dis Aramaic and cause stability issues causes fatigue or immune dysfunction poor proprioception they have no idea about their place in this world they don't know where their joints are they don't know where their feet and ankles are and they trip and get fall and a pop we don't always do one chair in the room they'll hit that chair yeah all the idiots are laughing rip subluxation all right so these missions present with chessmen just volcanoes we have some fun writers and this is not I feel it's just other bands to move up and down right and they have to be all coordinated ten reps on each side you go up and down all coordinated with the lack of moving right now I think that because of the poor proprioception these reps on coordinating itself which leads to the muscles between the ribs caregivers there is one where injections so it probably does too but that's my theory again I do a second so MTHFR mutation so this I can't even read that name is so huge but this is this is so this is responsible this this particular gene is responsible for from using this way limiting enzyme that converts folic acid to its actual form and so and if you look carefully I said yoga whenever all sorts of conditions so and they've also found out that women who have two of these see 617 area have a child neural tube defect so the question is should we be doing an MTHFR test and all these patients it's a really easy it has to do its work doing that again my two cents mass cell activation syndrome this is a vicious cycle microglia respond to pro inflammatory signals released from mast cells so mast cells release these inflammatory signal site cytokines and then the micro player interns relief release more inflammatory molecules so the party goes on each one triggering the other one again patients of massive is very easy to figure out they have fluid symptoms penny now eNOS LT hurts this is a blanket over I'll be done in two seconds thank you [Applause]

Channel: Bobby Jones Chiari & Syringomyelia Foundation

Views: 12,434

Rating: 4.9256506 out of 5

Keywords: pain management, Ehlers-Danlos syndrome, EDS, connective tissue disorders, Chiari malformation, chronic pain, syringomyelia, neuropathic pain

Id: Pz9EFG6-ZC8

Length: 21min 9sec (1269 seconds)

Published: Tue Aug 07 2018