Sorry it's taken so long for me to follow-up on my visits to the National Institutes of Health and their reaction to my CT venogram that showed stenosis in my left internal jugular.
Just to review, for the newbies: I've had a diagnosis of atypical PPMS for the last 6 1/2 years. For most of that time, I've disputed this diagnosis.
My MRIs have always shown only two nonenhancing lesions, and only one that is of any consequence. This one lesion is at the base of my brainstem, and has not changed in size or appearance in the years that I've been having MRIs. My spinal fluid analysis has always been clear, with no O-bands, or any sign of any inflammation or immune system response.
My clinical presentation has been progressive from the start. Six and half years ago I first presented with a slight limp in my right leg, and today my right side is pretty much paralyzed, and my left side is weakening. I have bowel and bladder issues, as well as sensory dysfunction. My autonomic nervous system has also been affected by my brainstem lesion.
Through the years I've tried almost every mainstream MS treatment, and several experimental ones. None have put a dent in my disease. I've been seen by some of the best MS doctors in the world, all of whom have found my case to be puzzling, but who until recently were content to leave it in the "atypical PPMS" pile.
In July I started making visits to the NIH, in Bethesda, Maryland, to participate in a "Natural History of MS" study. They have found my case to be very intriguing, and have put me through an extensive regimen of diagnostic tests (multiple brain and spine MRIs, MRI SPECT, diffusion tensor imaging, spinal fluid analysis, motor evoked potentials, sensory evoked potentials, lymphopherisis, and multiple physical examinations).
These tests have indicated that my problematic lesion is an inactive scar that almost completely transects my upper cervical spine, that it shows signs of calcification, and that some of my nerve fibers have been routed around it. From the looks of it, I should have suffered considerable disability at the time of the "event" that caused the lesion, and most likely would have remained stable since. As I noted above, this is the opposite of how my disease has actually progressed, and there is a disconnect between my clinical presentation and what my diagnostic testing would suggest.
Spurred by an interest in CCSVI, and at the advice of Dr. Dake, in July I had a CT venogram done from my head to my sternum. The radiologist at the hospital where the scan was done found no abnormalities. When I sent the scan to Dr. Dake, he reported that he saw significant stenosis in both of my upper internal jugulars. I then had the scan read by a family friend who is the chief radiologists at one of the local hospitals here in New York. He reported stenosis in the left upper internal jugular, but said the right was normal. I took the scan with me to my next visit to the National Institutes of Health.
The radiologists at the NIH examined the scan and confirmed that I did have stenosis in my left internal jugular, but not in the right. They held onto the scan for further examination, and wanted to consult with other research physicians both inside the NIH and at outside facilities to determine its relevance to my case.
During my last visit to the NIH, in October, I learned that the NIH neuroimmunology team had added a neuroradiologist, in large part to study the CCSVI phenomenon. Although they remain skeptical, they do believe the theory warrants further investigation, and will be conducting their own studies in regards to CCSVI. I was told that several NIH researchers had attended ECTRIMS and had left interested but unconvinced by the research presented by Dr. Zamboni. Still, my showing up with an abnormal venogram intrigued them, and help kickstart their own efforts in investigating CCSVI.
I was also informed that after careful consideration by the entire neuroimmunology team, the NIH does NOT think that I have MS! They don't know what neurodegenerative disorder I do have, as there are literally dozens of conditions that can cause progressive neurodegeneration, and I've tested negative for those that can be tested for. Unfortunately, none of them have any effective treatment.
The NIH does want to further investigate whether my stenosis is relevant to my disease. They are particularly intrigued because my jugular stenosis is directly adjacent to the area of my spine that is damaged. They told me that the fact that they don't believe I have MS makes them think it more likely that the stenosis might be responsible for my condition, since they can find no other reason to explain it.
I was ordered to have a neck MRI, to see if there are any soft tissue abnormalities that might be contributing to the vascular problems. I had the MRI done last week, and I'm currently awaiting results.
The NIH team told me that my stenosis is caused by a bony structure, is very high up in my skull, at the C1 one level, and would be very difficult to stent. They have adamantly and repeatedly warned me of the dangers of placing stents in the cranium, and referred me to studies done in the late 90s of endovascular surgery into the carotid arteries that resulted in a number of brainstem hemorrhages.
As we are all too painfully aware, one of our own tragically died from just such an infarct. The NIH docs knew of of this recent terrible incident, and told me that research conducted in the late 90s revealed that these hemorrhages could be directly attributed to endovascular surgery, although admittedly that surgery was conducted on the arteries. The NIH contends that venous surgery could be even more problematic, because veins are less robust than arteries, and thus are much more likely to tear. They have urged me to not elect to undergo such surgery until they have determined that the there is a relationship between my stenosis and my neurodegeneration. Here is an abstract related to the late 90s studies:
The NIH is currently evaluating my neck MRI, and will decide on a plan of action after that evaluation. Most likely, additional imaging will be done to ascertain a more precise picture of what is happening in my cerebrospinal vascular system, and then a decision on what kind of intervention, if any, should be attempted.
On a specific topic of CCSVI, I'd say that the NIH is interested but not nearly convinced. I do find it very encouraging that they are interested enough to have decided to investigate the theory themselves. They are currently designing study protocols, and expects to have something started within the next six months.
Incidentally, my local neurologist, after consulting with the NIH, is hesitant to back off of the MS diagnosis, based strictly on my clinical presentation. He's also extremely skeptical of the CCSVI theory, although he was slightly more open-minded about it than I expected him to be. As many of us are discovering, resistance to the CCSVI idea within the mainstream MS community will be intense. Hopefully, studies currently underway at the University of Buffalo, in future studies like that being contemplated at the NIH, will produce results that will cause a seismic shift in the thinking about MS.
On a personal level, although it has validated my doubts about my diagnosis, the NIH's opinion about my disease has also been quite disquieting. I'm still trying to wrap my mind around my "new normal", and I'd really like to to reconcile the opinions of the NIH and my primary neurologist to arrive at some kind of consensus. I'm hopeful that the NIH will find a connection between my vascular issues and my neurodegenerative condition, and also undertake an intervention to correct it. If they find no such connection, I'll have some difficult decisions to make regarding the course of my treatment. My disease, whatever it is, continues to progress unabated, and time is definitely of the essence...
Sorry about the length of this post, there was just a lot of information to convey. Hope I haven't put anyone to sleep...