PRIMARY PROGRESSIVE MS

PPMS is progressive from the time of start(onset), and typically it is not associated with relapses and remissions, even though there now a lot of evidence that some patients with clear PPMS may show acute changes consistent with the definition of relapse and such patients need much more attention. I had two of them, and both responded very well to steroids, but their MRI images did not show disease activity, and there was no gadolinium enhancement. On average, people with the primary progressive form of MS start having symptoms between ages 35 and 39 years.

In the medical literature, it is mentioned that PPMS is progressive from the beginning, and has no relapses. I have to give this statement another thought, and would probably challenge it.

MS is an inflammatory and degenerative disease, and any inflammation is an act of exacerbation of a stable MS. That in a way is a definition of a relapse in MS, but not of a relapsing and remitting phenomenon !!. I do strongly believe in the fact that degeneration in MS always follows a stage of inflammation, and the PPMS is not an exception in this case.

Why PPMS develops quicker and more aggressive than RRMS following the first relapse, and why it doesn’t remit following the first relapse is an area needs a lot of attention and research work?. Is it because the first relapse was so extensive that left a lot of axonal damage, or is it because of other immunological/pathological factors which are very much unique to PPMS is another area of interest we need to pay a lot of attention to if we really are interested in knowing the pathogenesis of MS as a disease?.  PPMS represents between 10 – 15% of all MS.

MANAGEMENT OF PPMS

Primary progressive MS is a degenerative disease characterised by an axonal damage. Currently, damaged axons are not reversible and do not regenerate or recover. Hence, the disease is steadily progressive, and complications are cumulative(building up). Most of the complications are motor and cognitive in nature, and they are clearly disabling to the sufferer.

Managing Complications

Please refer to the management of MS  as an illness, but memory and physical disabilities will be discussed in more detail here, especially with the connection of progressive MS.

Long Term Management

Here I am talking about Disease Modifying Therapy(DMT) in progressive MS.

Ocrelizumab‎

Ocrelizumab is a humanized anti-CD20 monoclonal antibody. It targets CD20 marker on B lymphocytes and hence is an immunosuppressive drug candidate. It has proven in previous phase III trials that it can slow down the progression of MS. It has been licenced in USA, and expected to gain licence in some European countries soon.

It has been tried in RRMS and PPMS. It has not been tried in SPMS.

It is given by intravenous infusion as 600 mg every 6 months(given in two 300mg infusions two weeks apart).

MD1003