Chronic cerebrospinal venous insufficiency

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Classification according to ICD-10
I87.2 Venous insufficiency (chronic) (peripheral)
ICD-10 online (WHO version 2019)
The location of the main neck veins
MRI image: Significant narrowing of the left jugular vein
Azygos vein in CCSVI: Before (left) and after (right) placement of a stent

Chronic cerebrospinal venous insufficiency (CCSVI) is a syndrome in which the cervical and thoracic veins are unable to efficiently drain blood from the central nervous system (CNS). It is believed that this is caused by stenoses (narrowing) of the internal jugular vein and / or the azygous vein . Such a "system" was described by Paolo Zamboni in 2008. He also advocated a possible link between CCSVI and multiple sclerosis(MS), a hypothesis that was discussed as early as the 1930s. A causal connection with multiple sclerosis could not be proven. In a relatively small study by Zambonis (300 participants), the syndrome was found in all MS patients, but not in any control person. CCSVI was classified as a vascular malformation in 2009 .

diagnosis

The most reliable examination method to determine CCSVI is a catheter venography . However, this is associated with a relatively high expenditure of resources and brings with it a not inconsiderable radiation exposure for the patient. Therefore, a preliminary examination using magnetic resonance venography and extra- and transcranial Doppler sonography is usually carried out beforehand . Only if these preliminary examinations give a reasonable suspicion of pathological changes in the veins is catheter venography carried out subsequently.

Link to multiple sclerosis

Hypothesis of "venous multiple sclerosis"

As early as the 1930s to 1940s, hypotheses arose about a possible stenosis-related development of multiple sclerosis , but these could not be confirmed.

This hypothesis was discussed again in the mid to late 1980s.

From 2008 the hypothesis was revived by the Italian vascular surgeon Paolo Zamboni. According to his investigations, the CCSVI correlates highly significantly with multiple sclerosis and can only be found in patients with this disease. His study included progressive variants of MS, but not special forms such as Baló or Schilder MS. The results led to a theory that at least some cases of MS disease could be caused by CCSVI. A large (1,500 subjects) double-blind study is currently being carried out at the Buffalo Neuroimaging Analysis Center .

The theory of a connection between CCSVI and multiple sclerosis assumes that, due to different malformations of the draining blood vessels, there is a backflow of blood into the CNS and this leads to increased deposits of neurotoxic iron in the brain. These deposits and the cell death they cause trigger the autoimmune reactions typical of MS . Based on this theory, 65 MS patients were experimentally treated with balloon dilatation in a study .

To investigate the possible connection between multiple sclerosis and CCSVI in more detail, the Multiple Sclerosis Society of Canada and the National MS Society (USA) have jointly commissioned seven studies, each lasting around two years.

A German study from 2010, after examining 56 MS patients, denied a causal relationship between CCSV and multiple sclerosis.

An examination of 499 patients and healthy controls showed only moderate sensitivity and specificity of the finding.

The research status was summarized in a review article in 2011 as follows:

"A critical analysis of the scientific methods used in the original studies of chronic cerebrospinal venous insufficiency in multiple sclerosis reveals several methodological problems with regard to potential bias and confounding. The current evidence calls into question whether chronic cerebrospinal venous insufficiency in multiple sclerosis exists at all. "

These assessments were underpinned in 2013 by a study published in the Lancet which, in addition to the previously used ultrasound examinations of the veins, also used catheter venography , which is regarded as the gold standard for imaging diagnostics of venous stenoses . In this study, a CCSVI was consistently found in 2–3% of the test subjects examined, with no differences between MS patients, their siblings and a healthy control group. According to a review published in 2013, the studies carried out in Germany also did not reveal any evidence of a venous cause for MS, which is why it is still not advised to use vein dilatations as a therapeutic attempt for MS outside of controlled clinical studies.

Criticism of the CCSVI hypothesis

The German Multiple Sclerosis Society (DMSG) issued a general warning calling for a scientific review of the CCSVI theory. Your overall assessment : “According to our scientific judgment, the Zamboni et al. presented study results of a solid scientific method and are therefore worthless and even ethically questionable. "
On the occasion of the Neurowoche 2010, the DGN (German Society for Neurology) warns " ... against senseless and dangerous vascular interventions in multiple sclerosis patients, which are currently also in Germany against private Fee will be offered. The interventions are based on a scientifically untenable theory of the Italian doctor Paolo Zamboni about the origin of the disease, criticize leading neurologists. ”
The US American regulatory authority FDA issued an alert on May 10, 2012 with the title“ FDA issues alert on potential dangers of unproven treatment for multiple sclerosis "before the so-called" liberation treatment ", which is based on the CCSVI hypothesis.

On the occasion of the European MS conference ECTRIMS in October 2010, people with MS were even warned by the Italian doctor Paolo Zamboni, who initiated the controversial CCSVI hypothesis in MS, not to seek treatment too quickly and to undertake further clinical studies before an operation to be seen.

treatment

There is no independent scientific evidence of a positive effect of the therapy options currently practiced at CCSVI. Attempts are currently being made to use balloon dilatation to remove the narrowing of the veins. However, the study also showed that the success of the treatment, depending on the cause and location of the stenoses, was sometimes not permanent. In problematic cases, implantation of a stent is considered at some operation centers in order to be able to keep the vein permanently open. The risk of a renewed narrowing of the veins is significantly higher in the jugular veins than in the azygous vein .

See also

Web links

Commons : Chronic Cerebrospinal Venous Insufficiency  - Album with pictures, videos and audio files

Individual evidence

  1. ↑ a b c P. Zamboni et al. : Chronic cerebrospinal venous insufficiency in patients with multiple sclerosis . In: J. Neurol. Neurosurg. Psychiatr. . 80, No. 4, April 2009, pp. 392-399. doi : 10.1136 / jnnp.2008.157164 . PMID 19060024 . PMC 2647682 (free full text).
  2. ↑ BB Lee, J. Bergan, P. Gloviczki, J. Laredo, DA Loose, R. Mattassi, K. Parsi, JL Villavicencio, P. Zamboni: Diagnosis and treatment of venous malformations consensus document of the International Union of Phlebology (IUP ) -2009 . In: Int Angiol . 28, No. 6, December 2009, pp. 434-51. PMID 20087280 .
  3. ↑ T. Putnam: "Encephalitis" and sclerotic plaques produced by venular obstruction. In: Arch Neurol Psychiatry . Volume 33, No. 5, 1935, pp. 929-940.
  4. ^ T. Putnam: Evidences of vascular occlusion in multiple sclerosis and encephalomyelitis. In: Arch Neurol Psychiatry , Volume 37, No. 6, 1937, pp. 1298-1321.
  5. ↑ T. Putnam, A. Adler: Vascular architecture of the lesions of multiple sclerosis. In: Arch Neurol Psychiat , Volume 38, 1937, p. 1.
  6. ↑ RS Dow, G. Berglund: Vascular pattern of lesions of multiple sclerosis. In: Arch Neurol Psychiatry. Volume 47, No. 1, 1942, pp. 1-18
  7. ^ JC Walton, JC Kaufmann: Iron deposits and multiple sclerosis. In: Arch Pathol Lab Med , Vol. 108, No. 9, September 1984, pp. 755-756. PMID 6547829 .
  8. ↑ CW Adams, YH Abdulla, EM Torres, RN Poston: Periventricular lesions in multiple sclerosis: their perivenous origin and relationship to granular ependymitis. In: Neuropathol Appl Neurobiol . Volume 13, No. 2, 1987, pp. 141-152. PMID 3614542 .
  9. ↑ F. Schelling: Damaging venous reflux into the skull or spine: relevance to multiple sclerosis. In: Med Hypotheses. Volume 21, No. 2, October 1986, pp. 141-148. PMID 3641027 .
  10. ↑ Buffalo Neuroimaging Analysis Center Trial ( Memento of the original from January 8, 2010 in the Internet Archive ) Info: The archive link was automatically inserted and not yet checked. Please check the original and archive link according to the instructions and then remove this notice.  @1@ 2Template: Webachiv / IABot / www.bnac.net
  11. ↑ AV Singh, P. Zamboni: Anomalous venous blood flow and iron deposition in multiple sclerosis . In: J. Cereb. Blood flow metab. . 29, No. 12, December 2009, pp. 1867-1878. doi : 10.1038 / jcbfm.2009.180 . PMID 19724286 .
  12. ↑ Over $ 2.4 million committed to support seven operating grants to explore the relationship of CCSVI to multiple sclerosis ( Memento of the original from June 14, 2010 in the Internet Archive ) Info: The archive link was automatically inserted and not yet checked. Please check the original and archive link according to the instructions and then remove this notice.  @1@ 2Template: Webachiv / IABot / mssociety.ca
  13. ↑ F. Doepp, F. Paul, JM Valdueza, K. Schmierer, SJ Schreiber: No cerebro-cervical venous congestion in patients with multiple sclerosis. In: Annals of Neurology . 2010. doi: 10.1002 / ana.22085 .
  14. ↑ R. Zivadinov, K. Marr, G. Cutter, M. Ramanathan, RH Benedict, C. Kennedy, M. Elfadil, AE Yeh, J. Reuther, C. Brooks, K. Hunt, M. Andrews, E. Carl , MG Dwyer, D. Hojnacki, B. Weinstock-Guttman: Prevalence, sensitivity, and specificity of chronic cerebrospinal venous insufficiency in MS. In: Neurology . Volume 77, Number 2, July 2011, pp. 138-144, doi: 10.1212 / WNL.0b013e318212a901 .
  15. ^ BA Bagert, E. Marder, O. Stüve: Chronic cerebrospinal venous insufficiency and multiple sclerosis. In: Archives of Neurology . Volume 68, Number 11, November 2011, pp. 1379-1384. doi: 10.1001 / archneurol.2011.179 (review).
  16. ↑ AL Traboulsee et al .: Prevalence of extracranial venous narrowing on catheter venography in people with multiple sclerosis, Their siblings, and unrelated healthy controls: a blinded, case-control study. In: The Lancet. October 8, 2013. doi: 10.1016 / S0140-6736 (13) 61747-X
  17. ^ R. Gold et al .: Overview and analysis of international case-control studies on "Chronic cerebrospinal venous insufficiency" (CCSVI) and multiple sclerosis. In: Current Neurology. Volume 40, No. 08, 2013, pp. 445-451. doi: 10.1055 / s-0033-1351271 .
  18. ↑ DMSG: New Vascular Hypothesis of Multiple Sclerosis? DMSG opinion on the CCSVI hypothesis . December 8, 2009. Retrieved January 8, 2010. 
  19. ↑ Neurologists warn against dangerous therapy for multiple sclerosis (press release of the DGN) (PDF), DGN. September 17, 2010. Retrieved September 22, 2010. 
  20. ↑ fda.gov
  21. ↑ DGN: DMSG report on CCSVI from ECTRIMS 2010: Zamboni warns against CCSVI treatment. . November 23, 2010. Archived from the original on December 27, 2011 Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. . Retrieved November 24, 2010. @1@ 2Template: Webachiv / IABot / www.dmsg.de 
  22. ↑ a b P. Zamboni et al .: A prospective open-label study of endovascular treatment of chronic cerebrospinal venous insufficiency . In: J. Vasc. Surg. . 50, No. 6, December 2009, pp. 1348-1358.e1-3. doi : 10.1016 / j.jvs.2009.07.096 . PMID 19958985 .