Case Report: Marburg Variant Multiple Sclerosis

Epub ahead of printTalab R. Marburg Variant Multiple Sclerosis – a case report. Neuro Endocrinol Lett. 2011 Aug 30;32(4). 

In this case the investigators describe a 24 year-old female with a fulminant demyelinating disease of white matter. Disease progression was consistent with the Marburg variant (malignant form) of MS with rapid deterioration of her clinical condition and epileptic seizures; ending with persistent coma and tetraparesis (weakness in all 4 limbs), over the course of 6 months from first symptoms. Repeated MRI showed progression of multiple demyelinating lesions culminating in a contiguous focal disorder of the white matter extending through out the brain. The serial MRI changes closely mapped the deterioration in the patients clinical status. Unfortunately the patient did not respond to repeated pulses of steroids, administration of intravenous immunoglobulins, serial plasmapheresis and high-dose immunosuppression (including mitoxantrone).

“Why have I highlighted another case of malignant MS on this blog? No, it is not to depress you. Firstly, fulminant MS is rare, hence individual case reports. Secondly, most clinicians feel compelled to write these cases up in the hope that other neurologists can learn from them. Thirdly, the real value of publishing individual case studies exists in the future when someone will almost certainly pull all these cases together in a meta-analysis. This is how a lot of diseases become recognised and established.”

“I am great supporter of the case study, provided it has something to teach the reader.”

“What does this case study teach us? That not all MS is necessarily amenable to treatment. 

“If I was looking after this case I would have considered using Rituximab (anti-CD20), which targets B cells, and Natalizumab, a selective adhesion molecule inhibitor that blocks trafficking of lymphocytes in the brain and spinal cord.”

Related posts of interest on this blog: 

26 Aug 2011
Fulminant MS can be accompanied by optic neuritis (ON); however a long interval between ON and the fulminant phase has not been reported. This is a case report of 30-year-old woman with a history of ON that occurred 1 
27 Aug 2011
The term fulminant is a carry-over from the pre disease-modifying therapy era. Most cases presenting with Marburg’s variant of MS who treated promptly with appropriate therapies, respond to treatment, and can do very well. 

11 thoughts on “Case Report: Marburg Variant Multiple Sclerosis”

  1. Prof G,You have succeeded in depressing me – dreadful waste of a young life. The scam is that we are trianign evry bright people to become neuros and when faced with a terrible case they are found to be useless (as with NMD).Grateful for an MS case study which had an excellent outcome thanks to the intervention of a neuro. This blod has all got a bit depressing – fulminant MS, more lesions seen with higher power MRI…

  2. Re: "Grateful for an MS case study which had an excellent outcome thanks to the intervention of a neuro."I believe the prognosis of this case would have been very different if she had been started on Natalizumab. I have personal experience with a patient with very very active disease, who has done very well on Natalizumab. If this case had done well, she would not be published as a case study. So buried in this story is a positive one!

  3. a horror movie!G, who is your readership? "professionals" will find very little value in here, as the info has been digested and regurgitated to the benefit of the layman."consumers" are not interested at all in this. I have not done a double blinded case study to back such a motion, but take it from me as a single case study.As you are exerting tremendous and much appreciated effort into publishing (best neuro on earth in my opinion), please stick to a predefined publishing strategy (target readership) to maximise the impact of this G we trust!

  4. Personally, I don't mind reading "depressing" things on this blog, as long as there is a balance with some optimism too (which I do think there is). That's what keeps it realistic!

  5. Jesus, I had never even heard of Marburg Variant Multiple Sclerosis nor malignant MS. Reading about these ailment is making me feel even iller.MS seems so hopeless, especially for those of us with PPMS and SPMS. I'm seeing my neuro next week for another one of our yearly pointless appointments. I think I ought to punch him in the face just to get his attention better and for him to start taking notice.Then again maybe it's not his fault. I'm sure that even if Prof G was my consultant there's little he could either.Oh well. I think I've just depressed myself even more.

  6. Re: "Rituximab being off-label, would you be able to use it?"Possibly; however, you would have to make a strong case for using it. For example, we treated one of our patients with highly-active MS who developed neutralising antibodies to Natalizumab with Rituximab; as this patient had also previously failed interferon-beta and glatiramer acetate was an exceptional case. Another patient had an overlap syndrome; MS in association with an inflammatory seronegative arthritis. And finally a young patient with progressive-relapsing MS with a large number of Gd-enhancing lesions. The funders realise the difficulties we face with exceptional cases and are prepared to look at the evidence; the data in in relation to Rituximab is quite compelling and and it costs substantially less than Natalizumab (and now fingolimod).

  7. Re "consumers" are not interested in this;All readers are not from the UK and all neuros are not so well-informed. Somebody could use such info to put the treating doctor on the right track. I'm speaking from personal experience here (thank goodness not for Marburg)

  8. It is very important to illuminate the weight of evidence in medical cases, and the word "depressing" is a consequence that must be lived with in many instances. It is not an excuse to bury needed information.

  9. My wife has been rapidly deteriorating from something, we can’t get a diagnosis fast enough. All evidence is pointing to MS so far, but it been about 1.5-2 months since symptoms first appeared. My concern is the rapid nature to which these symptoms have presented themselves. Can you tell me what how Marburg MS presents itself? How rare is it? I can’t find anything online and am very concerned for my wife. Thank you

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