Primary progressive MS (PPMS)

What is primary progressive MS or PPMS?

Primary progressive MS is called this because, from the very first (or ‘primary’) signs of MS, someone’s symptoms ‘progress’. This means they slowly but steadily get worse. Young people can have this type of MS, but it’s usually diagnosed in people in their 40s or older. Men are just as likely as women to get it, unlike relapsing MS which affects women more.

Early symptoms are often subtle, like problems with walking, which develop over time. There will usually be few or no relapses with PPMS. Instead, disability and symptoms will gradually increase over a long time.

People with primary progressive MS can experience many of the same symptoms as those with relapsing remitting MS.

Diagnosing primary progressive MS (PPMS)

To get a diagnosis of primary progressive MS your disability or symptoms must have got worse over at least a year, but you’ve not had a relapse in that time. On top of that, you must have two of these three things:

• MRI scans must show one or more lesions (areas of nerve damage) in your brain
• or scans show two or more lesions in your spinal cord
• or a lumbar puncture shows signs of MS (antibodies) in the fluid around your spinal cord.

Read more about how MS is diagnosed

How is primary progressive MS (PPMS) different from other types of MS?

The difference between types of MS can be loosely organised by the way symptoms present and build up.

In relapsing MS there are distinct relapses. These can be mild or severe and people can recovery on their own or with treatments and therapies.

Secondary progressive MS (SPMS) develops out of relapsing MS as the body struggles to recover from the cumulative impact of relapses. Disability and symptoms worsen independently of relapses. Thanks to DMTs fewer people develop SPMS and, if that does happen, it can take longer.

In primary progressive MS there are no or very few attacks by the immune system on the myelin that covers nerves in your brain or spinal cord. This means you get no or very few relapses. Instead something else happens called ‘neurodegeneration’. This means nerves break down and can eventually stop working. We don’t fully understand why this happens.

Active primary progressive MS (PPMS)

Some people also get relapses early on in primary progressive MS, or their scans show inflammation in the shape of new lesions. This is called active primary progressive MS (it used to be called 'progressive relapsing MS').

'Active' here means the person's immune system is still attacking the myelin around nerves in their brain or spinal cord, and causing inflammation. A relapse or new lesions on MRI scans are signs of this.

What comes after primary progressive MS (PPMS)?

There’s no other type of MS that comes after primary progressive MS. It doesn’t develop into another form of MS. But your symptoms can change over time, so you might find the treatments and support that help also change.

Managing primary progressive MS (PPMS)

Managing symptoms

There are many ways to manage the symptoms of primary progressive MS.

Find out more about managing symptoms

Disease modifying therapies (DMTs)

Trials are also testing drugs to see if they can slow down how fast people with progressive MS see their disability and symptoms get worse. One drug is now available that can do this for people with early primary progressive MS. It’s called ocrelizumab (Ocrevus). You can take it if you have primary progressive MS that’s ‘active’. This means attacks from your immune system still cause inflammation. Signs of this are new or growing lesions on your MRI scans, or you get the occasional relapse.

Ocrelizumab has been recommended across the UK to treat people with early primary progressive MS since 2020.

Several drugs are now in trials to see if they can repair myelin, protect nerves, or slow down MS in people who have progressive MS that’s not active.

Wellbeing and rehabilitation

Healthy eating and appropriate exercise is a kind of ‘self management’ and is useful to many people with primary progressive MS.

When symptoms change, different people might be best placed to help.

For example:

• trained people in NHS services can offer you rehabilitation. This is help to, as much as is possible, get back the function of a part of your body that you may have lost. They’ll help you to stay as active as possible and to get back your independence
• a physiotherapist and occupational therapist can often help to minimise the effects of muscle weakness or unsteadiness on your feet
• a dietician might help you find ways to eat healthily if you're less mobile
• an occupational therapist can help make adjustments around home or work to make things easier.