Multiple sclerosis (MS) is a disorder of the central nervous system that affects about 400,000 people in the United States. Most people with MS have relapsing symptoms and experience episodes lasting days or weeks of symptoms, such as:
Numbness or tingling
Weakness of a leg or arm
Unsteadiness
Bladder difficulty
Visual symptoms
About one in ten people with MS do not have a relapsing course of the disease. These patients tend to have symptoms that gradually progress without typical relapses. Patients with the gradually worsening disease are considered to have primary progressive multiple sclerosis or PPMS.
What causes primary progressive multiple sclerosis?
Just as with multiple sclerosis in general, the cause of PPMS is not known. It is thought that the disease occurs in people who are susceptible to it due to some kind of genetic tendency. It is believed by some to be caused by a trigger, perhaps a virus. However, no specific cause for PPMS is known. PPMS, however, is not infectious.
What other diseases can mimic primary progressive multiple sclerosis?
There are other diseases that can look like PPMS and need to be considered. For example, some people inherit a tendency for stiffness and weakness in the legs; a family history may be helpful. Some people with low vitamin B12 levels may develop numbness and stiffness in the legs and will show changes in their MRI scans. Occasionally, Lyme disease may look like MS. Some people with viral infections such as HTLV-1 may have a spinal cord syndrome. Sometimes discs or spinal arthritis may compress the spinal cord, or a tumor may be pushing on the spinal cord. Other diagnoses should be considered when the diagnosis of PPMS is being made.
How is primary progressive multiple sclerosis diagnosed?
First steps in diagnosing PPMS may combine a neurological history and exam with results from testing, primarily MRI imaging of the brain and spinal cord, as well as studies such as evoked potentials and spinal fluid testing.
The story of a gradually progressive neurological problem--such as weakness on one side, unsteadiness, or numbness in the legs--is characteristic. The condition must have been present for one or more years to make this diagnosis. The examination should show changes suggesting central nervous system disease. The MRI should show lesions that are characteristic of multiple sclerosis. The spinal fluid often, but not always, shows oligoclonal banding. This is a sign that the immune system is active around the brain and spinal cord. The evoked potentials may show slowing in nerve conduction in the central nervous system. There should be no other diagnosis to cause the symptoms.
Is there any treatment for primary progressive multiple sclerosis?
Standard FDA-approved medications for MS (for example interferons, glatiramer acetate, fingolimod) have not been proven useful in slowing the progression of PPMS. Ocrelizumab is the first FDA-approved medicine for primary progressive MS. It appears to slow the progression of disability.
In terms of making physical function as good as it can be, people with PPMS will be helped by exercise, stretching, physical, and occupational therapy. Maintaining mobility and fitness are as important in people with PPMS as with anyone else. In addition, there are medications which may be used to treat symptoms such as bladder and bowel urgency, erectile problems, spasticity, and pain, if such treatments are needed.
What is the prognosis for primary progressive multiple sclerosis?
As with multiple sclerosis in general, the prognosis varies in PPMS. Most people start to have symptoms at about age 50 or older, or about 10 years later than is typical of relapsing forms of MS. A recent Canadian study showed that many people with PPMS were able to walk years after a diagnosis, but usually patients tended to get worse over time. This worsening usually affected the ability to walk and was less likely to cause visual symptoms or tremor. Generally, thinking, memory, and intellect are relatively spared with PPMS. People who have PPMS may also have bowel, bladder, and/or sexual problems.
Last reviewed by a Cleveland Clinic medical professional on 01/28/2019.
I hope this helps! jimeka 🤣🤗💕🌠 And of course everyone else! 🤗🤗💕💕🌠🌠
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Jesmcd2
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Its not mentioned because no one really brings it up. And most ppl are dxnd with RRMS. Im really sorry about the PPMS, what symptoms do you have the most trouble with? 🤗💕🌠
I could, including stairs (though with some difficulties), until I ended up in Intensive Care Unit (ICU)after being found unconscious on the floor, so how much is the MS and how much is the after-effects of intensive care I don't know. No idea how I got to be on the floor in the first place as I have no memory of it, that's probably the effect of ICU sedation, maybe I fell and couldn't get up, which would be the MS, maybe I collapsed for some other reason, but no idea what that might have been.
After-effects of ICU are very similar to MS - muscle weakness, fatigue, brain fog, amongst others. Plus trauma, PTSD and other delights of the like kind.
I have more or less got over the brain fog. I do not have pain.
Im so sorry you had to go thru all that Kit10 🤗 and are still going thru it! I know how scary waking up in the ICU can be. 🙂 N not having a clue about what, why, or how it happened... My Neuro still asks me🤔 IDK? Just next time.. dont yell at me! 😡
Im just glad that lm here, and lm glad your here too! 🤗💕🌠
I have posted many times in the ICUSteps community, I won't repeat it all here.
Someone said here that nobody dies of MS. If I fell and couldn't get up, and if the ambulance had been an hour later, then I would have died of MS. Hmmmm... Not a pleasant thought.
I’m glad you’re okay! I battle with the same, my left knee just doesn’t want to bend to enable the walking. It’s a constant battle. I will never give up researching to heal and make my body better. I understand I have primary progressive MS, but that doesn’t mean I can’t be hopeful for a more positive day! I’m looking into hyperbaric to see if that will help down the line.
thank you for the funny ...hahahhaha...it is still all just MS..ahahahhah..thank you for it...hahhaahhh we all know more about it that them and i think we could write about it better with everyone telling them about the different stages of it for someone in this group has the different stages of it and how it has affected them...good read but still funny....hahahhaah
I would agree it is all very similar but not the same. I believe I have PPMS but have not pushed to have it changed in my chart. I was diagnosed at age 45 and have several lesions in my cord. I had bands on lumbar puncture and abnormal evoked potential but those aren't specific to PPMS.The main difference to me is my symptoms never remitted. For most with RRMS new symptoms occur but after a round of steroids and/or some time they get better but may not return to baseline. Mine are the same as when they first occurred.
Similarities would be the flare of symptoms with change in body temperature or stressor on the body such as infection (Covid really messed me up).
I developed new lesions on both Gilenya and Rebif. Now, even though I have had no new lesions since starting Ocrevus symptoms are slowly progressing.
I did start up an exercise program at a gym a month ago and hoping to see improvement in strength and walking. Glad this says exercise helps, although, I have known this I had not gotten started. Another reminder to stick with it and give it time to see results!
I have PPMS plus more. Dysautonomia,POTS, small nerve fiber neuropathy and 11 other autoimmune markers found by blood test not because of MS. I walked until I fell and broke my hip in December 2020. My first MS doctor said I wouldn't be walking in 5 years from diagnosed. I walked for 9.5 years. Severe spasticity along with being 6'6" tall makes my life a virtual living hell. My first MS doctor said being tall with PPMS is the worst. Doing transfers with long spastic legs it terrible. I'm now in a fancy,super expensive class3 power wheelchair. In tons of nerve pain too. It all started with Lyme for me....
The only thing I hate about the ppms or the spms, it that you don't get all better.. like I had with rrms.. it stays with no Major setbacks, but small setbacks..... they just get a little bit worse.. with no bright spots on the MRI.. to indicate what has been a little bit re-activated...... the old spots come back.. it's frustrating to say the least.. but we will all work through it.. and keep our heads up.. and our ch ins up.. and fight on!!!!😊😊😊😊
My mom converted from RRMS to PPMS. At the time where she went to be with GOD, she went legally blind ( she only saw shadows.)It's true what they say "she's in a better place now " 🙏❤️Lisa💕
Thanks, Jes for talking about PPMS. I didn't have RRMS or SPMS when I was diagnosed three years ago. my neuro went straight to PPMS and I believe him. My symptoms don't relapse the Numbness, tingling, Weakness in the legs and arms, and balance problem is always there. Nonstop pain with PPMS.
I have PPMS. I’ve been told by my specialists that everyone’s ms is different ( as you know ) and some progress and some stay the same and some get better for unknown reasons. I finally got a diagnosis Christmas eve 2020. Im taking Ocrevus, getting Botox for my calves, wear a Bioness for foot drop and other meds. Before my diagnosis I felt like my orthopedic surgeon should have given me a punch card 🤣 bc I broke so many bones due to falls. ANYWAY I have been in pt 3 times a week so far for a year and will continue. My strength and balance have improved. I haven’t seen much if any progression since symptoms started in 2017. It is frustrating that there is little attention given to ppms and most articles are outdated and somewhat negative. One really needs to use caution when reading some of the info . 😉
This is pretty accurate for my husband. He had first symptoms of foot drop at age 52 and was diagnosed a couple of years later. He has progressed through the EDSS scale at about .5 change every 2 years (sometimes 1.5 years, sometimes 2.5 years). Right now, at age 70, he is a level 8. He cannot stand unassisted and even when assisted can only balance shakily for 30 seconds to a minute, he cannot take a single step, he cannot sit up straight, he cannot hold his head up straight, he has absolutely no use of his left arm and gets Botox injections for spasticity in that arm/shoulder, he has partial use of his right arm (he must bend his head down a bit to get food from a fork), he is barely able to sign his name, he is incontinent, sexually dysfunctional, has severe heartburn (at the moment), he has proprioception problems, he has severe edema in his feet (in spite of very tight compression socks) along with skin shedding, he has tingling and nerve pain (for which he takes gabapentin and Aleve). The worse thing about the incontinence is that he cannot urinate while sitting in his scooter. I have to raise him in the lift so he can go. He can luckily go on the toilet, too, but never makes it before the first urination.
I'm still not sure what the difference is between Level 8 and level 8.5. He cannot sit up by himself, but is able to tolerate being bent over in his scooter so not sure if he would be considered mostly bedridden or not. The scale is so imprecise. He has been at level 8 for 2 years and this year the incontinence began so not sure if that bumps him to 8.5.
He is on Ocrevus, but I'm not sure it is doing much for him. Maybe, he would be worse without it - hard to say.
In spite of all these severe symptoms, life is worth living, He is amazing! He has engineered a stand for his trumpet and practices with one hand every day and plays in a community band. Until recently we attended the local symphony, but the urination problems may make that uncomfortable now. His years-long poker group now congregates for poker at our house once a month instead of rotating - isn't that kind of them? We can't easily go out to dinner, not to mention the dangers of picking up a virus, so we do periodic take-out for fun. Last year we entertained quite a bit and the year before we had people over twice a week for dinner. He listens to his high quality stereo nearly every day and reads lots of books on his Kindle. He corresponds with his friends through phone and email, using voice to text features. He has a very kind hairdresser who makes house calls and a concierge GP who also makes house calls. Televisits with MS doctor. The only trips out of the house are for band, symphony, dentist, eye doctor, Ocrevus and Botox. Sometimes, his fatigue makes him morose which is a bummer for me, but I would not call him depressed.
I just recently hired care 8 hours a day Mon-Fri. I am retired and I could handle everything myself since I can leave him home alone between diaper changes, but this gives me a mental and physical break so I can run long errands or do time consuming house projects and hike with my friends and play pickleball and other forms of exercise. We are lucky to have the resources to be able to do this. I did retire about a year earlier than planned because he needed more help with transfers and we did not want caregivers in the house during Covid. I made it about a year into Covid working from home without the caregivers before I just couldn't do it anymore and retired.
I am posting because there is so little out there about the folks at level 8. Most women with RRMS do not get to this level. Public material on MS is noticeably silent about anything past level 6. Maybe this will help other PPMS sufferers or caregivers understand what is coming, even though, of course, each person's course is slightly different.
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