Although PPMS is relatively uncommon it remains a significant clinical problem both diagnostically and therapeutically. PPMS is almost certainly part of the MS spectrum and there is no clinicopathological evidence to support PPMS as being a separate disease. Unfortunately, there are no licensed DMTs that have been shown to modify the course of PPMS. Despite this there is some emerging evidence that PPMS may respond to immunomodulatory therapies. Two large phase 3 trials are currently underway to test this hypothesis.
“This is a short review on PPMS; you may be interested in the section on diagnostic criteria.”
CoI: This mini-review was written by myself and we are a trial site for both the fingolimod and ocrelizumab PPMS trials, sponsored by Novartis and Roche, respectively.
Primary progressive MS
Giovannoni. Primary progressive MS. ACNR 2012;12(3):9-12.
Did he really write it?
PPMS and leprously are the same thing? Pls explain.
Leprosy is a disease caused by a bacteria. Mycobacterium leprae The disease can be a mixture of problems with the bacteria or how the person responds to the bacteria.It is a spectrum of disease featuresIn some people the bacteria is recognised and rapidly destroyed by the cell-mediated immune response and the bacteria is encapsulated by macrophage and is called tuberculoid leprosy.In other people they do not make a good cellular response to the bacteria and you gets lots of live bacteria and this is called lepromatous leprosy.Now if you make the lepromatous person respond to the bacteria they get a "reversal reaction" or lepromatous nodosum.There is also borderline leprosy which is between tuberculous and lepromatousThe point is the same bacteria can cause quite distinct clinical features.Prof G used this analogy to RRMS and PPMS. He thinks they are both the same disease but differnt parts of a spectrum So there are aspects that look different but have the same cause
In the paper you say childhood PPMS is very rare, but it can happen. What is the youngest ever case of PPMS? Can you learn anything more about the mechanisms of PPMS in younger sufferers?
Re: "In the paper you say childhood PPMS is very rare, but it can happen. What is the youngest ever case of PPMS? Can you learn anything more about the mechanisms of PPMS in younger sufferers?"The youngest case I have been involved in is a girl of 13; she is now in her mid 20's and is very disabled.
Re: "The youngest case I have been involved in is a girl of 13; she is now in her mid 20's and is very disabled."I can't get this statement out of my head. The image of a 13-year-old girl developing PPMS and losing her whole adolescence and young adult life to this disease, whilst professionals simply monitored her degeneration without being able to do anything to better it, is atrocious.I'll hazard a guess and assume this girl never went to university or met someone special to share her life with. She just got worse and neurologists have been absolutely impotent to do anything about it.PPMS is a horrific form of the disease. It is cruel and merciless. It's so badly understood and even worse in terms of medical practitioners taking it seriously.You have to suffer a lifetime with PPMS. Shame on the neurology profession and pharmaceutical industry. Shame on the NHS for not prioritising it and shame on NICE for not greenlighting treatments that may ease suffering.
Re: "…whilst professionals simply monitored her degeneration without being able to do anything to better it, is atrocious."Not true; two neurologists who I would classify as being aggressive treaters, hit her hard with different immunotherapies. Unfortunately, the treatments didn't stop her disease from progressing. They may however have slowed it down. For confidential reasons I am unable to comment on her personal details, but it is not all bad news.