Primary progressive multiple sclerosis is the rarest and most disruptive type of MS for patients. It causes both diagnostic and therapeutic difficulties. Treatment options for Primary Progressive Multiple Sclerosis are limited - only one drug at the moment. Other methods, proposed to patients, do not have any proven effectiveness.

Primary progressive multiple sclerosis(abbreviatedPPMS ) appears, according to statistics, in 10 up to 15% of MS patients. Women and men suffer from this disease with a similar frequency.

The first thing that distinguishes PPMS from other types of MS is that its symptoms appear slightly later in patients - typically they occur in people aged 35 to 39 (and in most cases, the first manifestations of MS start around 10 years earlier).

Primary progressive multiple sclerosis: causes

As with all other types of MS, the exact causes of MS are unknown.

It is known that the course of multiple sclerosis causes damage to the myelin sheaths that worsen over time, which, however, leads to them - this has not been clearly stated so far.

Hypotheses about the pathogenesis of MS can be really different, but two are the most popular.

According to one of them, the disease belongs to the group of autoimmune diseases, according to another, it occurs after a viral infection that leads to an overreaction of the immune system.

It is also noticeable that in the course of multiple sclerosis, inflammatory reactions occur around the myelin sheaths.

Primary progressive multiple sclerosis is special, however, because the inflammatory processes are far less pronounced in it - making it by far the most enigmatic type of MS and at the same time making it even harder to know exactly what the causes of PPMS are.

Primary progressive multiple sclerosis:symptoms

The symptoms that patients with PPMS experience are similar to those experienced by patients struggling with other forms of MS. The symptoms of primary progressive multiple sclerosis include:

  • sensory disturbance (e.g. in the form of a feeling of stiffness)
  • difficulty maintaining balance
  • memory impairment
  • unusual sensation (e.g. prickling, itching or burning sensation in various parts of the body)
  • pain ailments
  • muscle weakness
  • visual disturbance
  • sphincter dysfunction (resulting in difficulty urinating or stools)
  • constant feeling of fatigue
  • sexual dysfunction

It is impossible to list here all the symptoms that may appear in a person suffering from PPMS - because different patients have different compilations of ailments.

So far, the most characteristic feature of primary progressive multiple sclerosis has not been mentioned here - it is that when patients develop symptoms, they bother them all the time.

In the course of PPMS, we cannot talk about relapses or remissions that are typical for most MS cases - patients constantly struggle with symptoms of the disease, which gradually, over time, unfortunately become more and more intense.

Primary Progressive Multiple Sclerosis: Diagnosis

Difficulties with the diagnosis of multiple sclerosis arise quite often, but PPMS may be considered the most difficult form of disease to diagnose.

This is one of the reasons why patients experience a smaller number of changes in the brain, typical of MS, in the course of this unit - they are much more numerous in the spinal cord.

A slightly different clinical picture leads to the fact that sometimes patients are diagnosed with a certain delay. The following tests are used to diagnose primary progressive multiple sclerosis:

  • imaging tests (the most important is magnetic resonance imaging)
  • lumbar puncture (followed by examination of the cerebrospinal fluid)
  • study of visual evoked potentials

However, before the patient is ordered any tests, first a medical interview (during which the patient may be diagnosed with symptoms typical of MS) ​​and a neurological examination are conducted.

It's worth italso mention here that before diagnosing a patient with multiple sclerosis, it is necessary to rule out possible causes of symptoms other than this disease. Among the units that should be distinguished primarily from the primary progressive form of multiple sclerosis, the following can be mentioned:

  • vitamin B12 deficiency
  • Lyme disease
  • viral infections (e.g. HTLV-1 virus infection)
  • tumors of the central nervous system
  • inflammatory joint diseases

Primary progressive multiple sclerosis: treatment

The greatest difficulties in treating MS concern the primary progressive form of multiple sclerosis. As there are commonly available agents for the treatment of other types of this disease, unfortunately, it is a bit different in the case of PPMS.

As previously mentioned, the pathophysiological processes of this form of MS are slightly different, so pharmaceuticals that help people with other forms of MS are not necessarily effective in patients with primary progressive multiple sclerosis.

Scientists don't leave the sick on their own and they are still looking for PPMS treatments. Hope for patients appeared in 2016, when the US FDA approved a preparation that can be used in the treatment of primary progressive multiple sclerosis - this drug was ocrelizumab.

As in patients with other forms of MS, it is also important to manage their symptoms in patients with PPMS. In this case, the therapeutic effects depend on what exactly the problems are most severe in the patient.

For example, in patients with significant spasticity, baclofen may be used, and in patients struggling with severe pain, agents recommended for neuropathic pain (e.g. carbamazepine) may be used.

And all patients with primary progressive multiple sclerosis - no matter what the symptoms of the disease are the most severe - can be helped to stay fit for as long as possible by regular physical therapy.

Rehabilitation in multiple sclerosis

Physiotherapy exercises for MS patients

Multiple Sclerosis (MS) Spasticity

PPMS: alternative therapies

Since the treatment options for Primary Progressive Multiple Sclerosis are simply limited, it is not surprising that patients are looking for alternative treatments, thanks towhich their condition could improve.

Quite often, unfortunately, they are offered interventions that not only will not help them at all, but may also have a negative impact on their he alth.

Just as the Zamboni method is controversial, but at the same time effective for some people, other methods used by patients can hardly be considered reliable in any way and bringing the expected results.

Among such dubious methods we can mention the use of vitamin supplements by patients (containing, among others, vitamin C, vitamin E, omega-3 acids, selenium and zinc) or various herbal mixtures (e.g. containing lemon balm, pumpkin seeds) and aloe).

Work is also underway on collecting patients' own T lymphocytes, "taming" them - outside the patient's body - with myelin antigens and then reintroducing them into the patient's bloodstream.

It is difficult to deny the legitimacy of using unconventional methods of treating multiple sclerosis, but one thing can be stated with certainty.

If a PPSM patient gives up the care of doctors, putting himself entirely in the hands of people dealing with alternative MS therapies, it is not safe and in a short period of time may even result in complete loss of he alth.

Primary progressive multiple sclerosis: prognosis

It is simply impossible to define the prognosis of patients with primary progressive multiple sclerosis - the course of the disease in different patients can be very different.

As some patients experience a significant impairment of their fitness only many years after they become ill, others become unable to move without supporting devices within just a few years of the first symptoms of the disease.

About the authorBow. Tomasz NęckiA graduate of the medical faculty at the Medical University of Poznań. An admirer of the Polish sea (most willingly strolling along its shores with headphones in his ears), cats and books. In working with patients, he focuses on always listening to them and spending as much time as they need.

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Category:

Neurology