Primary progressive MS (PPMS): symptoms, diagnosis, treatment and living with it

The NHS describes multiple sclerosis (MS) as a condition that affects the brain and spinal cord. It cannot currently be cured, but treatment can often help manage it. There are 3 main types: relapsing remitting, secondary progressive and primary progressive. In relapsing remitting MS there are flare-ups followed by remission; in secondary progressive MS symptoms are there all the time and get slowly worse, usually after a relapsing phase; and in primary progressive MS symptoms slowly worsen over time with no periods of remission.

The MS Trust explains that in PPMS progression occurs from the outset, which is why it is called primary, and that in contrast with secondary progressive MS there is no initial relapsing remitting phase. It says it is rare to have any relapses, and that the rate at which symptoms become more severe varies. Typically the changes are slow and gradual over a number of years, though for some people they can be more rapid, and there can be periods of minor improvement or times when symptoms stay the same.

The MS Trust estimates that 10,000 to 15,000 people have PPMS in the UK. Relapsing remitting MS is usually diagnosed under the age of 40 and is more common in women, whereas PPMS is typically diagnosed in the 40s or 50s and affects men and women about equally.

The NHS lists common MS symptoms as fatigue, problems with eyes or vision, numbness or tingling, feeling off balance, dizzy or clumsy, muscle cramps, spasms and stiffness, bladder problems, problems with memory or concentration and sexual problems. In PPMS the pattern is shaped by where damage has occurred in the brain and spinal cord.

According to the MS Trust, difficulty walking is the first symptom in more than 8 out of 10 people with PPMS, perhaps as stiffness, weakness or a heavy feeling in the legs that gradually worsens and can lead to tripping on kerbs or steps. Unsteadiness, balance and coordination problems can contribute. Bladder and bowel problems are quite common and can be linked to sexual problems such as difficulty maintaining an erection, loss of desire or reduced genital sensation, because they are all caused by lesions in the spinal cord.

It also lists fatigue, which it describes as exhaustion out of proportion to what you have been doing, spasticity and spasms, cognitive problems such as concentration, memory or planning, emotional difficulties including anxiety or altered mood, and altered sensations such as numbness, tingling or burning, including nerve pain. Some of these are invisible symptoms that are not always obvious to other people.

The NHS says to see a GP if you think you have symptoms of MS, and that symptoms are common and can be caused by many different conditions, so having them does not definitely mean you have MS. It says to call 999 or go to A&E if you have sudden weakness or numbness in one arm, loss or blurring of vision, or problems with balance and coordination, because these could be signs of a stroke.

If you already have MS and notice a substantial change in symptoms, the MS Trust suggests contacting your MS team or GP rather than waiting for the next review. It recommends an annual MS review and suggests keeping a simple diary of new or worsening symptoms, when they happened and any treatment changes, to share at appointments.

The NHS says that if your GP thinks you could have MS they will refer you to a neurologist, and that there is no single test to diagnose MS. Tests may include checks on movement, coordination, vision, balance and reflexes, blood tests, an MRI scan to see whether there is damage to the nerves in the brain or spinal cord, a lumbar puncture to take a small sample of spinal fluid, and tests with small sensors on the skin to measure how quickly messages travel from the eyes or ears to the brain. The neurologist may need to monitor your symptoms and test results for some time to work out which type you have.

The MS Trust says a diagnosis of PPMS is often a clinical diagnosis based on your medical history and the symptoms you are experiencing, rather than on diagnostic tests. The neurologist is looking for a pattern of worsening symptoms due to slow, progressive loss of nerve cells. It adds that increasingly MS is described as active or not active, with or without progression, depending on whether there are relapses or new lesions on MRI.

The MS Trust notes that people with PPMS are often under the impression that there are no treatments for them, which is not true. Symptomatic treatments relieve symptoms without changing the course of the condition. Disease modifying drugs treat the underlying condition.

On a page last updated in April 2022, the MS Trust said that there was currently only one disease modifying drug available for PPMS, ocrelizumab (Ocrevus). It said that if your PPMS is active, you have had it for 15 years or less (early) and you can walk at least 20 metres (with or without a walking aid) you may be eligible, whereas if you have had it for more than 15 years, your MS is not active and you cannot walk a short distance it is unlikely to be offered. It noted that more drugs were in development.

Because that guidance dates from 2022 and eligibility rules can change, ask your neurology or MS team what is currently available to you. The NHS says that you will be supported by different healthcare professionals, which may include a specialist MS nurse, a neurologist, a physiotherapist and an occupational therapist, and that you will probably need different treatments over time.

The MS Trust says living with PPMS can mean living with a degree of uncertainty, since symptoms can get more or less intense or fluctuate over time, and that a change in symptoms does not always mean MS is getting worse. It says MS is not a terminal condition and is rarely a direct cause of death, that most people with MS live into old age, and that for most people the changes are very gradual over a number of years.

It stresses looking after general health: a balanced diet, keeping the brain and body as active as possible, drinking sensibly, not smoking, having health checks such as cancer screening and taking vaccinations that are offered. Practical adaptations, such as using a stool for food preparation or a walking aid some of the time to conserve energy, can allow you to carry on doing what you enjoy.

The MS Trust notes that the Equality Act covers you as soon as you are diagnosed with MS, which can help at work with adjustments such as gradually increasing hours, and that benefits, grants and social care assessments may be available if you have extra care or mobility needs. The NHS lists the MS Society, the MS Trust and MS-UK as national charities that offer information and support.

On the NHS, MS is managed by a neurologist with a specialist MS nurse and allied health professionals, and access to disease modifying drugs follows NHS commissioning rules. Private neurology consultations and MRI scans are available and can shorten waiting times for diagnosis, but access to NHS-funded disease modifying drugs generally depends on NHS criteria, so it is worth asking early how any private opinion would link to NHS care, particularly for infusions and monitoring.