Table of ContentsView AllTable of ContentsWhat It IsSymptomsCausesDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
What It Is
Symptoms
Causes
Diagnosis
Treatment
Primary progressive multiple sclerosis (PPMS) is a type ofmultiple sclerosis (MS)that gets progressively worse over time. Of thethree typesof MS, PPMS is considered the rarest, affecting 10% of people living with MS.
As with other forms of MS, the course of PPMS is highly variable. Some people may become disabled within a few years of diagnosis, while others may remain stable for decades.
This article discusses primary progressive multiple sclerosis and how it is treated. It also goes over symptoms and causes.
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What Is Primary Progressive MS?
Primary progressive MS is a form of the disease that is progressive from the time of onset. It differs fromsecondary progressive MS (SPMS), which is diagnosed when someone with relapsing-remitting MS (RRMS), the most common form of the disease, begins to show signs of disease progression.
PPMSProgressive disability from the startMay include occasional acute relapses or plateausAverage age at onset is 40 yearsMakes up around 10% of MS cases at onsetOccurs in males and females equally
Progressive disability from the start
May include occasional acute relapses or plateaus
Average age at onset is 40 years
Makes up around 10% of MS cases at onset
Occurs in males and females equally
May become progressive overtime
Average age at onset is 30 years
Accounts for 85 to 90% of MS cases at onset
Occurs two to three times more often in females than males
PPMS can be characterized even further at different stages into these categories:
Symptoms of Primary Progressive MS
MS symptoms are different in every individual, and in PPMS, they tend to be associated with movement.
Spinal Cord Symptoms
People diagnosed with PPMS often have walking problems due to the progressive atrophy (wasting and degeneration) of the spinal cord.
Also known asprogressive myelopathy, these symptoms may include:
Cerebellar Symptoms
While the spinal cord is the main target of injury in PPMS, your brain may also be affected, primarily the part known as thecerebellum, which regulates balance and coordination.
This condition, known asprogressive cerebellar syndrome (PCS), is seen less often than progressive myelopathy but can manifest with:
How MS Affects Your Muscles
Uncommon Symptoms
While far less common, PPMS can affect other parts of the central nervous system such as thebrainstem, which is situated between the brain and spinal cord, and the cerebrum, the main body of the brain.
These symptoms are rare in PPMS but may include:
Symptoms of Multiple Sclerosis
What Causes Primary Progressive MS?
It’s not clear exactly what causes any type of MS, though there seems to be a genetic component involved that raises your susceptibility to develop it when you’re exposed to the right environmental factors such as:
What Causes Multiple Sclerosis?
Diagnosing PPMS has special challenges since people with it have a slow gradual loss of function over months to years. Because the imaging tests can be similar between PPMS and RRMS, your healthcare provider will use your symptom history to help make this diagnosis. It may take several years or more to definitively diagnose PPMS, especially if your symptoms have just started.
To diagnose any form of MS, your practitioner will do a thorough medical and symptom history, a physical examination, and an MRI of your brain and spinal cord. If your MRI doesn’t show enough evidence to confirm a diagnosis of MS, your healthcare provider may do a lumbar puncture and/or visual evoked potentials for additional evidence.
MRI
In order to diagnose PPMS, your symptoms must have worsened for at least one year and you should have typical MS lesions in your brain and/or spine.
However, using MRI to diagnose PPMS presents a bit of a challenge since the results of brain MRIs of people with PPMS may be more subtle than those of people with RRMS, with far fewer gadolinium-enhancing (active) lesions.
What Are Gadolinium Enhancing Lesions?
Lumbar Puncture
Also referred to as a spinal tap,lumbar puncturescan be very helpful in making the diagnosis of PPMS and ruling out other conditions.
Having one of two findings from a spinal tap is important in confirming a diagnosis of PPMS, including:
Visual Evoked Potentials
Visual evoked potentials (VEPs)testing involves wearingelectroencephalogram(EEG)sensors on your scalp while watching a black-and-white checkered pattern on a screen. The EEG measures slowed responses to visual events, which indicates neurological dysfunction.
VEPs have also been helpful in solidifying a diagnosis of PPMS, especially when other criteria are not met definitively.
Diagnostic CriteriaDefinite PPMS can be diagnosed when you have at least one year of documented clinical progression, which means that your MS symptoms have steadily gotten worse,plustwo of the following:A brain lesion that’s typical of MSTwo or more MS lesions in the spinal cordA positive lumbar puncture, meaning that there’s evidence of either oligoclonal bands or an elevated IgG antibody level
Diagnostic Criteria
Definite PPMS can be diagnosed when you have at least one year of documented clinical progression, which means that your MS symptoms have steadily gotten worse,plustwo of the following:A brain lesion that’s typical of MSTwo or more MS lesions in the spinal cordA positive lumbar puncture, meaning that there’s evidence of either oligoclonal bands or an elevated IgG antibody level
Definite PPMS can be diagnosed when you have at least one year of documented clinical progression, which means that your MS symptoms have steadily gotten worse,plustwo of the following:
Differential Diagnoses
Many neurological diseases can mimic MS, so much of the burden of diagnosing any type of MS is eliminating the possibility that it could be something else. Some of these include:
How Primary Progressive MS Is Treated
Treatment for PPMS may include medications and/or rehabilitation therapies. Note, however, that it is more difficult than for RRMS.
Medications
Ocrevus (ocrelizumab) was approved to treat PPMS in 2017. The first dose is given intravenously in two 300-milligram (mg) doses two weeks apart. After that, it’s given in 600-milligram (mg) doses once every six months.
Ocrevus Zunovo (ocrelizumab and hyaluronidase) was approved in 2024 to treat PPMS. The recommended dose is 920 mg/23,000 units (920 mg of ocrelizumab and 23,000 units of hyaluronidase), given as a single subcutaneous injection every 6 months.
Ocrevus for MS
Other DMTs haven’t been found to be effective for treating PPMS, so most doctors don’t use them. There’s more research being done on effective treatments for PPMS though, so there’s hope that new medications will emerge in the future.
Beyond Ocrevus, your doctor can give you prescription medications to help you manage your MS symptoms, such as:
Rehabilitation Therapies
Rehabilitation specialists can also help you deal with MS symptoms like fatigue, mobility difficulties, muscle pain and spasticity, swallowing difficulties, aspiration, and cognitive impairment. These rehabilitation therapies include:
Summary
The speed of progression for PPMS is different for everyone. Some people progress quickly while others may progress slowly over many years. Treatment for PPMS may involve medications and rehabilitation therapies.
7 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Multiple Sclerosis Society.Types of MS.Johns Hopkins Medicine.Primary progressive multiple sclerosis.National Multiple Sclerosis Society.What causes MS?National Multiple Sclerosis Society.Primary progressive multiple sclerosis (PPMS).National Multiple Sclerosis Society.Primary progressive multiple sclerosis (PPMS).Food and Drug Administration.Ocrevus Zunovo label.National Multiple Sclerosis Society.Rehabilitation.Additional ReadingAbdelhak A, Weber MS, Tumani H.Primary Progressive Multiple Sclerosis: Putting Together the Puzzle.Frontiers in Neurology. 2017;8:234. doi:10.3389/fneur.2017.00234.Lublin FD, Reingold SC, Cohen JA, et al.Defining the Clinical Course of Multiple Sclerosis: The 2013 Revisions.Neurology. 2014;83(3):278–286. doi:10.1212/WNL.0000000000000560.Olek MJ, Howard J.Clinical Presentation, Course, and Prognosis of Multiple Sclerosis in Adults. UpToDate.Olek MJ, Howard J.Evaluation and Diagnosis of Multiple Sclerosis in Adults. UpToDate.Olek MJ, Mowry E.Treatment of Progressive Multiple Sclerosis in Adults. UpToDate.
7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Multiple Sclerosis Society.Types of MS.Johns Hopkins Medicine.Primary progressive multiple sclerosis.National Multiple Sclerosis Society.What causes MS?National Multiple Sclerosis Society.Primary progressive multiple sclerosis (PPMS).National Multiple Sclerosis Society.Primary progressive multiple sclerosis (PPMS).Food and Drug Administration.Ocrevus Zunovo label.National Multiple Sclerosis Society.Rehabilitation.Additional ReadingAbdelhak A, Weber MS, Tumani H.Primary Progressive Multiple Sclerosis: Putting Together the Puzzle.Frontiers in Neurology. 2017;8:234. doi:10.3389/fneur.2017.00234.Lublin FD, Reingold SC, Cohen JA, et al.Defining the Clinical Course of Multiple Sclerosis: The 2013 Revisions.Neurology. 2014;83(3):278–286. doi:10.1212/WNL.0000000000000560.Olek MJ, Howard J.Clinical Presentation, Course, and Prognosis of Multiple Sclerosis in Adults. UpToDate.Olek MJ, Howard J.Evaluation and Diagnosis of Multiple Sclerosis in Adults. UpToDate.Olek MJ, Mowry E.Treatment of Progressive Multiple Sclerosis in Adults. UpToDate.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
National Multiple Sclerosis Society.Types of MS.Johns Hopkins Medicine.Primary progressive multiple sclerosis.National Multiple Sclerosis Society.What causes MS?National Multiple Sclerosis Society.Primary progressive multiple sclerosis (PPMS).National Multiple Sclerosis Society.Primary progressive multiple sclerosis (PPMS).Food and Drug Administration.Ocrevus Zunovo label.National Multiple Sclerosis Society.Rehabilitation.
National Multiple Sclerosis Society.Types of MS.
Johns Hopkins Medicine.Primary progressive multiple sclerosis.
National Multiple Sclerosis Society.What causes MS?
National Multiple Sclerosis Society.Primary progressive multiple sclerosis (PPMS).
Food and Drug Administration.Ocrevus Zunovo label.
National Multiple Sclerosis Society.Rehabilitation.
Abdelhak A, Weber MS, Tumani H.Primary Progressive Multiple Sclerosis: Putting Together the Puzzle.Frontiers in Neurology. 2017;8:234. doi:10.3389/fneur.2017.00234.Lublin FD, Reingold SC, Cohen JA, et al.Defining the Clinical Course of Multiple Sclerosis: The 2013 Revisions.Neurology. 2014;83(3):278–286. doi:10.1212/WNL.0000000000000560.Olek MJ, Howard J.Clinical Presentation, Course, and Prognosis of Multiple Sclerosis in Adults. UpToDate.Olek MJ, Howard J.Evaluation and Diagnosis of Multiple Sclerosis in Adults. UpToDate.Olek MJ, Mowry E.Treatment of Progressive Multiple Sclerosis in Adults. UpToDate.
Abdelhak A, Weber MS, Tumani H.Primary Progressive Multiple Sclerosis: Putting Together the Puzzle.Frontiers in Neurology. 2017;8:234. doi:10.3389/fneur.2017.00234.
Lublin FD, Reingold SC, Cohen JA, et al.Defining the Clinical Course of Multiple Sclerosis: The 2013 Revisions.Neurology. 2014;83(3):278–286. doi:10.1212/WNL.0000000000000560.
Olek MJ, Howard J.Clinical Presentation, Course, and Prognosis of Multiple Sclerosis in Adults. UpToDate.
Olek MJ, Howard J.Evaluation and Diagnosis of Multiple Sclerosis in Adults. UpToDate.
Olek MJ, Mowry E.Treatment of Progressive Multiple Sclerosis in Adults. UpToDate.
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