sioux falls 2016 chronic - avera health · cerebral venous sinus thrombosis (cvst) posted by...
TRANSCRIPT
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When the Main Vein from the Brain Won’t Drain, Rooke thinks
it Might Cause Pain …
… Is he Insane?
Thom Rooke Krehbiel Professor of Vascular Medicine
Mayo Clinic
Sioux Falls 2016
30 min
Acute
Chronic
Renal vein (“nutcracker”) with gonadal
vein reflux
Venous obstruction / reflux has predictable effects …
http://radiologyinthai.blogspot.com/2011/06/cerebral-venous-sinus-thrombosis-cvst.html
Cerebral Venous Sinus Thrombosis (CVST) Posted by Rathachai Kaewlai, M.D
… including intracranial … Cerebral Sinus Thrombosis
The head and neck can be affected, too …
Volume 4, Issue 7, July 2005, Pages 437–444
Transient global amnesia (TGA) is one of the most striking syndromes in clinical neurology … Studies with … venous duplex … confirmed the importance of jugular-vein-valve insufficiency.
… headache probably results from stretching
of the … veins that drain into the sinuses, although elevated ICP may also play a role. In
addition to headache, clouding of consciousness and seizures are common …
https://www.dartmouth.edu/~dons/part_2/chapter_18.html
The head and neck can be affected, too …
Other effects
J Neurol Neurosurg Psychiatry 2009;80:392-399
Chronic cerebrospinal venous insufficiency in patients
with multiple sclerosis P Zamboni, et. al.
First in 2008
http://www.nature.com/nri/journal/v3/n6/fig_tab/nri1108_F1.html
Why not still more effects?
MS
http://missinglink.ucsf.edu/lm/ids_104_demyelination/didactic/msmicropath.htm
MS Plaques
Veins
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Alamelu V. Is chronic venous ulcer curable? A sample survey of a plastic surgeon. Indian J Plast Surg 2011;44:104-9
Venous Ulcer Multiple Sclerosis
Journal of Cerebral Blood Flow & Metabolism (2009) 29, 1867–1878; 2 September 2009 Anomalous venous blood flow and iron deposition in multiple sclerosis Ajay Vikram Singh1 and Paolo Zamboni2
Venous Ultrasound … venous outflow anomalies were dramatically associated (with MS) (OR 43, p<0.0001).
“… MS is strongly associated with
CCSVI, a scenario that has not previously been described …”
J Neurol Neurosurg Psychiatry 2009;80:392-399
Chronic Cerebro-Spinal Venous Insufficiency (CCSVI)
The “Liberation Procedure”
“The results of this pilot study (65 pts) warrant a subsequent
randomized control study.”
Zamboni, J Vasc Surg. 2009 Dec;50(6):1348-58.
“… CCSVI endovascular treatment significantly improved MS clinical outcome measures … the rate of relapse-free patients changed from 27% to 50% postoperatively …
Focus on Obstruction Meta-Analysis of the Correlation Between Chronic Cerebrospinal Venous Insufficiency and Multiple Sclerosis Vascular and Endovascular Surgery 47(8) 620-624
2013
Odds Ratio = 2.6
Looks very promising!
This triggered an effusive world-wide response. For example …
… it stirred rare hope among the … 75,000 Canadians suffering from the incurable, degenerative condition.”
“By June 2010, many were travelling out of country, paying upwards of $10,000 for CCSVI … The issue had become a flashpoint. … CCSVI was up for debate in the House of Commons.” “… the Ministry of Health … was under pressure to act.”
The silent treatment: How Canada has failed MS sufferers: Internal documents show why Canada has not kept its promise to accelerate
contentious clinical trials for MS Anne Kingston June 25, 2012
“Canada has one of the highest per capita MS populations: three people are diagnosed every day.
“… there is increasing worldwide acceptance of CCSVI … even though there is no supporting scientific evidence. … most of the information we have comes from one source only. The treatment is called “liberation treatment,” and the results of the treatment can be watched on YouTube. The current forum for the reporting of success … seems to be the Internet. There are … testimonies by MS patients who have gained improvement … (but not) …from patients who underwent unsuccessful treatments …”
Cardiovascular and Interventional Radiological Society of Europe Commentary on the Treatment of Chronic Cerebrospinal Venous Insufficiency Feb 2011, Volume
34, Issue 1, pp 1-2
CardioVascular and Interventional
Radiology
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PLoS One. 2013;8(2) Italy
Chronic cerebrospinal venous insufficiency is not associated with MS and its severity: a blind-verified study.
… CCSVI was not associated with MS … nor its severity.
Neurology. 2011 Aug 30;77(9):844-50. Italy
Progressive MS is not associated with chronic cerebrospinal venous insufficiency.
… CCSVI is not … associated with disability.
BMC Med. 2013 Jul 18;11:167 Buffalo, NY
Chronic cerebrospinal venous insufficiency is not associated with cognitive impairment in MS.
… We find no evidence of an association between the presence and severity of CCSVI … in patients with MS
Mult Scler October 2013 vol. 19 no. 11 1508-1517 multicenter Italy
Observational case-control study of the prevalence of chronic cerebrospinal venous insufficiency in multiple
sclerosis: results from the CoSMo study
1874 subjects aged 18–55.
Color … duplex sonography
The primary (goal) is to compare the prevalence of CCSVI in patients with MS versus patients affected by other neurodegenerative diseases (OND) and healthy volunteers.
Conclusions: CCSVI is not associated with MS.
Lancet. 2014 Jan 11;383(9912):138-45. Canada
Prevalence of extracranial venous narrowing on catheter venography in people with multiple sclerosis,
their siblings, and unrelated healthy controls: a blinded, case-control study.
Extracranial venous narrowing > 50% is a
frequent finding in patients with MS …
(and) controls.
The significance of venous
narrowing to MS symptomatology remains unknown …
Neurology Volume 83, Issue 5, 29 July 2014, Pages 441-449 USA
Prospective randomized trial of
venous angioplasty in MS (PREMiSe)
… sham-controlled, randomized, and double-blind (10 sham procedure, 9 treated). … endpoints included changes in disability, brain volume, cognitive tests, and quality of life.
Conclusion: Venous angioplasty is not an effective treatment for MS … and may exacerbate underlying
disease activity.
FDA NEWS RELEASE
For Immediate Release: May 10, 2012
FDA issues alert on potential dangers of unproven treatment for multiple sclerosis
The U.S. Food and Drug Administration is alerting
health care professionals and patients about injuries and death associated with the use of an
experimental procedure sometimes called “liberation therapy” or the “liberation procedure” to treat
chronic cerebrospinal venous insufficiency (CCSVI).
Mult Scler October 2013 vol. 19 no. 11 1508-1517
Observational case-control study of the prevalence of chronic cerebrospinal
venous insufficiency in multiple sclerosis: results from the CoSMo study
Conclusions: CCSVI is not
associated with MS.
Nobody wants to be associated with
CCSVI anymore – it’s embarrassing
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But … could venous insufficiency (obstruction) produce other (non-MS)
neurological/head symptoms?
What about?: • Headache • Visual changes • Speech difficulties • Imbalance • Cognitive issues • Elevated IC pressure
• Others
# of migraine sufferers … in the US = 12 million
Lipton RB, Stewart WF, Diamond S, et al. Prevalence and burden of migraine in the United States: data from the American Migraine Study II. Headache. 2001;41(7):646-657.
Prevalence of tension-type headache = 78%.
Rasmussen BK, et. al. Epidemiology of headache in a general population — a prevalence study. J Clin Epidemiol 1991;44:1147-1157.
These are Very Common Problems
How often does venous impairment play a role?
Non-Compliant
Mechanism of “Venous” HA ?
Intracranial blood flow increases
Vasodilators
If the venous blockage is not
“severe” …
#1 Problem -- How do we determine if a patient has CCSVI?
Very Complicated
Simpler?
Reflux
"Perhaps the most striking finding in our study was the range of venous outflow anomalies detected that did not reflect pathology, but
instead demonstrated the large natural variance in intracranial and extra-cranial venous anatomy …”
120 patients with multiple sclerosis and 60 healthy controls.
“… We detected no differences in the proportion of
venous outflow abnormalities between patients with multiple sclerosis and healthy controls.”
Validity of the diagnostic criteria for chronic cerebrospinal venous insufficiency and association with multiple sclerosis
CMAJ June 2, 2014
Variants of “normal” causing venous compression (often asymptomatic) are common
Popliteal vein entrapment Thoracic outlet syndrome
Vasc Med. 2006 Nov;11(4):278-9.
Normal?
Normal?
Normal?
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Movement of the neck is much more complicated
Pinched
Open
Normal?
FINDINGS: “MR venography the cervical and intracranial vasculature is negative. The dural venous sinuses are widely patent. No evidence of obstruction of the internal jugular veins in the neck.”
Collateral vein? Or
congenital variant?
• Stenosis? • Compression? • Normal variant?
Is this congenitally “small” transverse sinus “too small?” Is flow obstructed?
“Pathology?” Or just a “Variant?”
Decisions based entirely on anatomical
appearance +/- catheter-assessed pressure gradient
Dilemma
Case #1 78 year-old woman with chronic, severe headache
Dilatation of jugular stenosis X 3 sessions. Each helped … for 3-6 weeks. Then symptoms – and stenosis – returned.
Finally treated with stent. Complete resolution of symptoms. No recurrence.
Gradient 2-3 mmHg
“No Dx” “Nothing to do”
Case #2
Stenosis
Collaterals
Nothing!
No stenosis
No gradient
38 year old male. Chronic visual “fatigue,” headache, “brain fog.”
He’s worried he may have MS MRV findings may have
been positional? Or “overcall?” Did we miss something?
Case #3 68 year-old man. DVT (Factor V positive). Subdural hematoma on Coumadin.
Hematoma evacuated X 2. Still unstable.
Bleeding Shift
Clot
Small transverse sinus. Possible obstruction?
Case #4
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Dominant jugular is occluded? Collateral
It gets worse … Do you attempt to open the jugular?
MRV X 2 – findings consistent
What we need is a “functional test …”
Ultrasound seems like a good place to start. It’s the basis for
many other hemodynamic / functional vascular tests …
… But can we see the area of interest with Duplex?
“Normal”
“Abormal?”
• Increase inflow (vasodilator)
• Outflow obstructed (Limits overall flow)
This produces:
• Symptoms?
• Increased venous velocity
• Increased ICP
• Brain “stiffness”?
• EEG / PET scan?
• Other changes?
How to Test?
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78 year old female Worsening “myoclonus” (Most often, myoclonus occurs as a result of a nervous system (neurological) disorder, such as epilepsy, or of a metabolic condition, or as a reaction to a medication. The cause of essential myoclonus is often unexplained (idiopathic) or, in some cases, hereditary.) http://www.mayoclinic.org/diseases-
conditions/myoclonus/basics/definition/con-20027364
Case Example? Work-up = negative
Attempted dobutamine echo (to assess possible CAD) – stopped because of severe exacerbation
of myoclonus.
MRV – Venous obstruction?
Does she have physiologically
significant venous obstruction?
Is it contributing to her myoclonus?
Chronic (Cervical) Venous Occlusive Disease:
• Probably exists and is under-recognized
• Does not appear to cause MS?
• May cause HA and other CNS symptoms
However …
• Diagnostic criteria are lacking (And thus all the controversy)
• There is a need for better anatomic / hemodynamic / functional testing