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Multiple sclerosis (MS) is a neurodegenerative disease that interferes with the brain’s ability to control the body. It develops when our immune system attacks the insulation on the nerve cells called myelin, and this can damage the nerves in the brain and spinal cord. Primary Progressive Multiple Sclerosis (PPMS) is a form of disease that is characterized from the beginning of the disease as a progressively worsening condition. About ten percent of all multiple sclerosis patients have reported being diagnosed with PPMS, so let’s get a better understanding of how this works.

Understanding Primary Progressive Multiple Sclerosis

Doctors believe that any kind of MS happens when the human body attacks itself, like in the case of any autoimmune disease. In MS, the immune system damages the protective coating around the nerves in your brain and spinal cord, which causes inflammation. However, there’s little inflammation in PPMS, meaning nerve damage is the primary problem here. Areas of scar tissue form along the damaged nerves in the brain and spinal cord, thereby preventing them from sending and receiving signals the way they should. 

For a better understanding, there are four main types of MS:

  • Relapsing-remitting MS (RRMS)
  • Primary-progressive MS (PPMS)
  • Secondary-progressive MS (SPMS)
  • Progressive-relapsing MS

Each of these types could be mild, moderate, or severe since MS affects different people differently.

Primary Progressive Multiple Sclerosis Symptoms

Since this disease primarily affects the nerves in the spinal cord, the main PPMS symptoms usually include:

  • Walking problems
  • Weak, stiff legs
  • Headaches, pain in the legs, feet, or back 
  • Muscle spasms
  • Trouble with balance
  • Speech or swallowing issues
  • Vision problems
  • Fatigue and pain
  • Bladder and bowel trouble

Most people usually go to see a doctor when their legs are weak or if they are having trouble walking. Some of the PPMS early symptoms include tingling sensations and numbness usually in the face, arms, legs, and fingers. They are normally one of the most common warning signs of MS. 

Primary Progressive Multiple Sclerosis Diagnosis

In case of PPMS, neurologic functions steadily start worsening on the onset. There are no symptom flare-ups right away, or any recovery (remission). The progression of the disease may vary and sometimes things could even be stable, with periods of short-term, minor improvements that are temporary. But on the whole, the decline in neurologic progression remains constant.

PPMS is known to get worse over time, although how fast or how much tends to vary, and can be hard to predict. For this reason, it can be hard for doctors to diagnose PPMS. One could have symptoms for a few years, but no major flares, before doctors can tell that they are getting worse. Notably, about 10% to 15% of people with multiple sclerosis have this form. Those who do, are usually diagnosed later in life than people with other types.

Here are the methods that your healthcare provider might use to diagnose MS:

  • Discussion: The healthcare provider will talk to the patient about their symptoms.
  • Physical exam: This is conducted to see how the patient’s muscles and nerves are functioning.
  • MRI scans of your brain and spinal cord: These images will help the healthcare provider to look for signs of damage that might suggest MS.
  • Optical coherence tomography (OCT): This test measures nerve fibers in the retina.
  • Spinal tap (lumbar puncture): The healthcare provider takes a sample of spinal fluid to check for signs of MS.
  • Visual evoked potentials (VEP): This test is done to see how well the patient’s optic nerves are working.

After the general diagnosis of MS, the diagnosis of PPMS is based almost exclusively on the patient’s symptom history. Therefore, it may take time for the PPMS diagnosis to be made.

It is also found that relapsing forms of MS (including Relapsing-Remitting Multiple Sclerosis, and secondary progressive in those individuals who continue to experience relapses) are defined by inflammatory attacks on myelin. In PPMS, much lesser inflammation of the type is seen in relapsing MS. As a result, people with PPMS tend to have fewer brain lesions than people with relapsing MS, and the lesions tend to contain fewer inflammatory cells. Furthermore, people with PPMS also tend to have more lesions in the spinal cord than in the brain. Together, all these differences make PPMS more difficult to diagnose and treat than relapsing forms of MS.

Primary Progressive Multiple Sclerosis Treatments

Physical and occupational therapy works wonders for Multiple Sclerosis Rehabilitation, although Stem Cell Therapy, specifically the administration of mesenchymal stem cells (MSCs) has demonstrated great potential to help improve symptoms. The immunomodulatory (ability to regulate the immune system), tissue-protective and repair-promoting properties of MSCs demonstrated in multiple models make them a lucrative therapy for MS and other similar conditions. Patients can expect an increase in energy, flexibility, strength, mobility, and control of basic function. Recent data also shows that mesenchymal stem cells administered intravenously may have the ability to halt disease progression for an extended period.

While traditional therapy often includes medications to prevent relapse and disease progression, regenerative therapy is an effective solution for many. It rejuvenates the body and bypasses adverse side effects associated with medications. Regenerative therapy allows the body to repair itself and may replace damaged nerve cells with new ones. On the whole, healthcare providers are likely to provide treatments that can relieve one’s symptoms and improve the quality of life. These may address problems such as depression, sexual problems, and extreme tiredness (fatigue). Exercise is also good for all types of MS. It can help one stay active and mobile, control one’s weight and give more energy while also boosting one’s mood. Brisk walking, swimming, stretching and strengthening will improve the range of motion and can get the heart pumping. The patient will also need to meet the healthcare provider on a regular basis to monitor the disease and its symptoms. 

FAQs

How long can a person live with primary progressive MS?

People with advanced forms of MS are usually at a greater risk for life-threatening complications, and this can surely lead to a shorter life expectancy. A study published in 2017 reported that the average life expectancy for people with PPMS was 71.4 years.

Who gets primary progressive multiple sclerosis?

Primary progressive MS is typically diagnosed when someone is in their 40s or 50s. Men and women are evenly affected. In fact, about 10% of people diagnosed with MS have PPMS. The earliest symptoms usually begin by ages 35-39. People with relapsing-remitting MS typically move into the secondary progressive phase about the same time as others are diagnosed with primary progressive MS.

Is there any treatment for primary progressive MS?

PPMS is not known to have any definitive cures, but it is not considered fatal either. All current treatments for PPMS are focused on managing the symptoms and increasing daily functioning. This said, stem cell therapy and regenerative rehab are known to be extremely beneficial to patients with PPMS.

Is primary progressive MS the worst kind?

Secondary progressive MS (SPMS), the stage of MS after relapsing-remitting MS, is considered the worst for many people. With this type of MS, your disability gets steadily worse. Some might also argue that “Fulminate MS” is a rapidly progressive disease course with severe relapses within five years after diagnosis, and this form may need to be treated more aggressively than other forms.

Can progressive MS be fatal?

MS, by itself, is rarely fatal. However, complications due to the disease can impact a person’s life expectancy.

References:

https://www.cedars-sinai.org/health-library/diseases-and-conditions/p/–primary-progressive-multiple-sclerosis-ppms.html

https://www.nationalmssociety.org/What-is-MS/Types-of-MS/Primary-progressive-MS

https://www.hopkinsmedicine.org/health/conditions-and-diseases/multiple-sclerosis-ms/primary-progressive-multiple-sclerosis

https://www.webmd.com/multiple-sclerosis/primary-progressive-multiple-sclerosis

Dr Na'eem Sadiq is a respected stem cell specialist at Plexus, and a prominent neurologist in Bangalore. He studied neurology and clinical neurophysiology in London, and worked with some of the most prestigious medical institutions in England, and the Middle East. He completed his MBBS at Bellary Government Medical College, and a postgraduate degree in psychiatry from NIMHANS in Bangalore.

Dr Na'eem has perfected his knowledge and expertise in Continuing medical education (CME), and training in tissue culture, Stem Cell Therapy, and neurology. Dr Na'eem Sadiq possesses an undying passion to improve people’s lives. This led to the creation of Plexus, a neuro and Stem Cell Research centre in Bangalore in neurosurgery, and neurorehabilitation.