Secondary progressive MS symptoms

What is disability?

People who are diagnosed with secondary progressive MS may worry about becoming disabled. Disability means different things to different people. What one person considers disabling may not count as disability to someone else. Much of disability is not about the person but the environment in which we all live. For example, if a shop has steps, then it will not be accessible to all. If products in the supermarket have labels with tiny writing, then not everyone will be able to read them.

There are various definitions of disability. A dictionary would typically say that disability is any result of having a condition that limits what you can do. For example, if you have a bit of numbness in your fingertips, it could make it hard to put in your earrings. Some people might class this as disability but many wouldn’t.

The Equality Act covers people with MS from the point of diagnosis even though they may not see themselves as disabled at all.

The public’s idea of disability can be different again. People often look for outward symbols of disability like walking sticks or wheelchairs. This is not surprising, given that the symbol of a wheelchair is often used to represent disability.

Will I become disabled?

You will have your own way of thinking about disability and it will be up to you how you define yourself. MS may affect what you can do but only you can decide if this is disabling. As the symptoms of MS vary, you may have different views of yourself at different times.

It can be worrying to be told that disability is likely to increase from the beginning of your MS. However, this is not always a fast or a continuous increase as there can be times of improvement and times when symptoms remain the same. As with all types of MS, symptoms tend to be up and down from day to day and each person’s MS is different so no one can say how much your MS might, or might not, affect you.

You will have support from your MS team and many symptoms can be improved by trying different treatments. For some activities in daily life, it can be a question of doing the same thing but in a slightly different way. For example, to help manage fatigue, you might find it helpful to sit on a stool or chair to do the ironing or prepare food.

Getting support

Although the word ‘disability’ may be uncomfortable or feel inappropriate, it can help you access support. It may be particularly helpful at work or when considering whether any government assistance or financial benefits apply to you. There is no countrywide scheme to register as disabled so each benefit has its own definition of disability.

At work, the Equality Act covers everyone with MS and requires employers to make reasonable adjustments so that you are not at a disadvantage because of any difficulty caused by your MS. Examples of reasonable adjustments may include allowing you to return to work by gradually increasing your hours or, if heat makes your symptoms worse, allowing you to sit in a cooler part of the office. You may not need any adjustments or protection but it can be good to know where the law stands.

Similarly, you may find that you are eligible for some government benefits especially if you have difficulties with walking or the activities of daily life. Possible benefits include Personal Independence Payments and the council-run Blue Badge scheme which gives access to reserved parking places.

Secondary progressive multiple sclerosis (SPMS) is a stage of MS that follows relapsing-remitting multiple sclerosis (RRMS).

With this type of MS, a person’s disability gets steadily worse. The individual is no longer likely to have relapses, when the symptoms get worse but then get better.

Most people with RRMS will eventually develop SPMS. However, because of advancements in disease-modifying treatments, fewer people today develop SPMS than before, and the transition to SPMS occurs later.

SPMS can be further classified as:

  • active (with relapses or evidence of new brain lesions) or not active
  • with progression (which means that the disease worsens over time) or without progression

Active and inactive forms can both occur with or without progression.

Causes of SPMS

It is not well-understood what factors influence the progression from RRMS to SPMS, and progression is difficult to predict.

The median time from RRMS onset to SPMS is about 19 years. The older the patient at the onset, the shorter the time from onset to secondary progression.

Incomplete recovery from an initial exacerbation also is associated with a shorter time to secondary progression. In some studies, male sex was associated with a shorter time to secondary progression than female sex, but data are inconsistent.

Several studies reported that visual or sensory, and sometimes brainstem-related symptoms are associated with an increased time to secondary progression. In contrast, spinal cord-related symptoms are associated with shorter time to secondary progression.

In some studies, the number of relapses during the first two-to-five years of the disease was inversely related to the time to secondary progression, but the data are inconsistent.

The role of inflammation in SPMS also remains unclear. It seems to be smaller in SPMS than in relapsing forms of MS, and disease progression appears to be due to increased nerve damage.

Symptoms of SPMS

While patients in relapsing disease stages experience relapses and remissions of symptoms, remissions in patients with SPMS are less pronounced with only a mild reduction of symptoms. Instead, symptoms gradually worsen over time.

There is a wide range of SPMS symptoms, and they differ in severity from patient to patient.

Common symptoms of SPMS include:

  • numbness or tingling
  • chronic fatigue
  • cognitive problems
  • depression
  • coordination problems
  • sexual difficulties
  • bladder and bowel dysfunction
  • weak and stiff legs
  • vision problems

Patients with SPMS also may experience less common symptoms. These symptoms are often paroxysmal, which means that they are felt suddenly and disappear quickly.

Such symptoms include the following:

  • trigeminal neuralgia, a condition that affects the trigeminal nerve, characterized by a sudden burning sensation in the face
  • pseudobulbar affect, which is caused by lesions in the amygdala, a brain region that controls emotions, and is characterized by unprovoked crying or laughing
  • the feeling of electric shocks along the spine, which are caused by inappropriate communication between damaged nerves in the neck and the brain
  • unusual sensory symptoms, such as a strange sensation of cold or wetness along the limbs
  • dysesthesia, which is an abnormal and unpleasant sense of touch, such as the feeling of a tight hug and itchiness
  • blurred vision
  • optical illusions
  • seizure-like spasms in the arms and legs
  • dizziness, vertigo, and migraines

How SPMS differs from other disease courses

In RRMS, symptoms are caused by inflammation, and come and go in waves. In between flareups, patients experience much milder symptoms or may even be symptom-free. Relapses refer to a temporary worsening of symptoms. These come and go, but their severity is stable, which means that they do not worsen over time.

In contrast, SPMS is driven by neurodegeneration, which means that nerve damage worsens over time. At the SPMS stage, patients experience progression or a continuous worsening of symptoms. Symptom flareups can still occur, but symptom changes are much less drastic than in the RRMS stage. In SPMS, symptoms do not disappear even in the remission phases.

Diagnosis of SPMS

Establishing a diagnosis of SPMS can be challenging, as the changes between RRMS and SPMS occur gradually. The date of disease progression is difficult to define and is usually determined retrospectively once a constant rate of change from a remitting to a progressive course is observed. Typically, at least six months of progression must be noted before RRMS is considered as SPMS.

SPMS is characterized by a reduction in the number and severity of relapses, while at the same time the damage progresses. The diagnosis of SPMS, therefore, involves a careful analysis of the symptoms. Disease progression can be measured with the expanded disability status scale (EDSS). The scale ranges from 0 to 10, with 0.5-unit increments. The higher the number on the scale, the more severe the symptoms.

EDSS assesses:

  • muscle weakness or difficulty moving limbs
  • difficulties with speech and swallowing
  • numbness or reduced sensation
  • impaired visual function
  • loss of balance and coordination
  • ataxia (lack of muscle control) and tremor
  • bowel and bladder problems
  • cognitive impairment

A neurologic examination and magnetic resonance imaging (MRI) also may detect disease progression.

Treatment of SPMS

Treatment of MS is always a balance between effectiveness and side effects, and it can take time to find a satisfying solution. Once current medications no longer sufficiently control the symptoms, different therapies may be tried.

There are a several disease-modifying medications for the treatment of SPMS:

IFN beta

Interferons are proteins that control inflammation. Interferon (INF) beta has been shown to reduce inflammation in MS. Different INF beta medications exist for the treatment of MS, but their use for SPMS is controversial.

Immune suppressive medications

Novantrone inhibits the proliferation of specific immune cells. It is a U.S. Food and Drug Administration (FDA)-approved medication for the treatment of SPMS, progressive-relapsing MS (PRMS), and worsening RRMS.

Cyclophosphamide works by inhibiting the division of immune cells. The medication can slow progression in SPMS patients who show an inadequate response to other disease-modifying treatments.

Monoclonal antibodies

Tysabri is an antibody that blocks the entry of immune cells to the brain. It is an FDA-approved medication for relapsing forms of MS. Clinical data suggest that the medication might reduce the progression of upper limb disability in SPMS patients.

Stem cell therapies

Mesenchymal stem cells (MSCs) are adult stem cells found in different parts of the body, such as adipose (fat) tissue, bone marrow, and skin. MSCs can potentially develop into myelin-producing cells and reverse the damage to the myelin sheath — the protecting coat surrounding nerve fibers — that is characteristic of MS.

MSCs therapy for multiple sclerosis is currently being investigated in clinical trials. Some data suggest they could be of clinical benefit for SPMS patients.

Autologous hematopoietic stem cells (aHSCTs) are extracted from a person’s blood or bone marrow. After ablation of the patient’s immune system, a process called immunoablation, the aHSCTs are used to reconstitute the individual’s immune system. The procedure is supposed to halt the body’s autoimmune attack on myelin.

aHSCTs are currently under investigation in clinical trials, and a meta-analysis suggests that it may slow the progression of SPMS.

Other therapies

Siponimod decreases the number of lymphocytes in the blood, thereby reducing the inflammation that is involved in MS. The medication has been found to reduce the risk of progression in SPMS patients. It was approved by the FDA in March 2019 for the treatment of active SPMS and relapsing forms of MS.

Another therapy, Simvastatin, is a cholesterol-lowering medication that also has anti-inflammatory and potentially neuroprotective properties. Data from clinical trials suggest that the medication can lower the rate of brain atrophy.

Vumerity regulates the immune response and lowers oxidative stress, and is thereby supposed to prevent the degeneration of myelin. The medication is approved by the FDA for the treatment of SPMS and relapsing forms of MS.

A tyrosine kinase inhibitor, Masitinib targets immune cells called mast cells and macrophages. It thereby reduces inflammation and neuronal damage. Data from a pilot study suggest that the medication may be of benefit for people with relapse-free SPMS.

Management

Apart from disease-modifying therapies, medications that help control symptoms are used for the management of SPMS and other forms of MS. Certain medications also can be used to decrease the side effects of disease-modifying therapies.

These include medications to:

  • reduce muscle spasms and tremors
  • improve gait
  • relieve fatigue
  • reduce dizziness
  • improve bowel function
  • treat sexual problems
  • reduce bladder issues
  • treat depression and emotional changes
  • manage pain

A healthy diet also can improve the overall health and general well-being of MS patients. Several different diets have been suggested to be beneficial for people with MS, but there is no robust scientific evidence that supports one particular diet. Most recommended diets have in common that they restrict highly processed foods, and foods with a high glycemic index, and focus instead on an increased intake of fruits and vegetables. A glycemic index is a relative ranking of carbohydrate in foods according to how they affect blood glucose levels.

Exercise can help improve heart, bladder, and bowel function, increase strength, and reduce fatigue and depression. Exercise programs should always be individualized and adapted to the patient’s capabilities and limitations. Yoga, tai chi, and water exercises are examples of physical activities that may benefit MS patients.

Last updated: Nov. 12. 2019

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Multiple Sclerosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

Secondary-Progressive MS

The main difference between secondary-progressive MS (SPMS) and primary-progressive MS (PPMS) centers on the presence or absence of relapses. SPMS develops in patients who initially had relapsing-remitting MS (RRMS) as a secondary phase of that disease course. By contrast, PPMS follows a progressive disease course from the beginning in patients who never experience relapses.

Who develops SPMS?

SPMS is a form of MS which follows RRMS, the most common form of MS affecting 85% of patients diagnosed with MS. Most patients with RRMS will eventually develop SPMS. Their MS will become more steadily progressive, with increasing disability and loss of neurologic function, while they experience fewer and fewer relapses.

How is SPMS different from RRMS?

When RRMS transitions into SPMS, the disease becomes more progressive with a gradual worsening of symptoms or loss of neurologic function. With RRMS, flare-ups or exacerbations are usually followed by periods of remission, during which time symptoms improve somewhat or completely.

In contrast to RRMS, where symptoms during attacks are primarily caused by inflammation, in SPMS, symptoms are caused by the permanent damage or loss of nerves and/or brain atrophy. With SPMS, the patient may continue to have relapses (exacerbations, flare-ups, or attacks) with slight remissions and plateaus, while experiencing a general worsening of symptoms and neurologic function.

When does the transition to SPMS happen?

Before disease-modifying treatments became available, research found that about half of patients who were initially diagnosed with RRMS would develop SPMS within 10 years and 90% would develop SPMS within 25 years.

It is believed that MS medications may be able to slow the progression of MS, however we do not currently know how effective disease-modifying treatments are in delaying the transition of RRMS to SPMS.

How is SPMS diagnosed?

It is difficult to know exactly when RRMS has transitioned to SPMS because this transformation can happen very gradually. However, there are clues that the transition to SPMS has taken place.

If MS symptoms continue to worsen after an exacerbation has ended, this may mean that the patient has transitioned from RRMS to SPMS. However, if increased symptoms persist but stabilize following an exacerbation, then the patient may still be considered to have RRMS.

Tools to help determine if a patient has transitioned from RRMS to SPMS include a careful medical history and neurologic exam, during which the doctor notes changes in a patient’s symptoms. Laboratory tests including repeat magnetic resonance imaging (MRI) may also be useful in detecting disease progression.

Prognosis

Multiple Sclerosis is not considered a fatal condition however it is progressive and degenerative, particularly so if untreated or managed. Many people live long and relatively fulfilling with MS if managed well with changes to lifestyle and if available, treatment. Life expectancy for people with MS continues to increase which may be due to advances in treatment, diagnosis, and a better understanding of the impact of a health lifestyle. On average, a person with MS can expect to live 7-10 years fewer than those without MS. Most people find the condition to be very unpredictable with the type, frequency and severity of symptoms varying significantly from one person to another.

NZ MS Incidence Study

According to the 2012-14 NZ MS Incidence Study 88% of those are initially diagnosed with relapsing remitting MS and 12% with progressive MS. Up to 80% of people will eventually convert from relapsing remitting to secondary progressive MS during the course of their condition. Of the 277 participants who were originally recruited as clinically isolated syndrome (CIS – following their first attack or symptom presentation) 35% were subsequently diagnosed as having clinically definite MS during the study period.

Prognosis and Relapsing MS

For those with relapsing forms of MS there is no predictor as to the frequency or timing of relapses. In some cases people have been known to go for many years, or decades without new relapses. A relapse does not mean that your disability will progress as in most cases following a relapse and a period of recovery there will be no lasting damage. In some cases, depending on the severity and localisation of new brain lesions disability progression may occur. You should keep a record of any relapses and ensure that you discuss these with your health professional. Disease modifying treatments available in New Zealand, in most cases, effectively help to minimise the relapse rate and disability progression.
Currently in New Zealand disease modifying treatments (DMTs) are only available for those with relapsing remitting forms of MS. Where treatments aren’t available not wanted it is important to work with your health professionals and community supports and address lifestyle changes such as diet, sunlight, exercise, and meditation. Read more about available DMTs here.

Prognosis and Progressive MS

The nature of progressive MS means that disability will occur much faster however there is no definitive research to predict how this will occur and over what time period. According to a 2005 study a quarter participants with primary progressive MS required a walking cane within 7.5 years however a quarter still did not require one after 25 years. Research also shows that men tend to progress faster than women by 38%. Other studies have shown that from diagnosis to an EDSS score of 6, the median time for secondary progressive groups was 10 years, while it was just three years for the primary progressive group.

Factors Influencing Prognosis

Research suggests there are factors that may influence how MS may develop. These have been observed in long-term studies and reflect the trend associated with certain symptoms. They do not represent a guide as to how an individual’s MS will develop as the course of a person’s MS is unique.
Factors that are suggestive of a relatively better disease course are:

  • diagnosed at a younger age, eg. in the 20’s or 30’s
  • few relapses in the first few years after diagnosis
  • complete recovery from relapses with no long-term damage
  • long intervals between relapses
  • symptoms that are sensory in nature (such as numbness or tingling).

It is generally recommended that the longer that you can maintain a healthy brain and lifestyle the better your prognosis.

Further information and acknowledgements

Prognosis – MS Trust
How does MS Progress – Overcoming MS
Multiple Sclerosis Prognosis and Life Expectancy – Multiple Sclerosis News Today
Is MS Fatal – National MS Society

Research into MS Prognosis

  • Bronnum-Hansen H, Koch-Henriksen N, Stenager E. Trends in survival and cause of death in Danish patients with multiple sclerosis. Brain 2004; 11:11Vukusic S, Confavreux C. Natural history of multiple sclerosis: risk factors and prognostic indicators. Current Opinion in Neurology 2007;20(3):269-274.
  • Confavreux C, Vukusic S. Natural history of multiple sclerosis: a unifying concept. Brain 2006
  • Confavreux C, Vukusic S. Age at disability milestones in multiple sclerosis. Brain 2006
  • Confavreux C, et al. Course and prognosis of multiple sclerosis assessed by the computerized data processing of 349 patients.Brain 1980;103(2):281-300. Kremenchutzky M, Rice GP, Baskerville J, et al. The natural history of multiple sclerosis: a geographically based study 9: Observations on the progressive phase of the disease. Brain 2006
  • Hammond SR, McLeod JG, Macaskill P, et al. Multiple sclerosis in Australia: prognostic factors. J Clin Neurosci 2000; 7:16-19
  • Pittock SJ, Mayr WT, McClelland RL, et al. Disability profile of MS did not change over 10 years in a population-based prevalence cohort. Neurology 2004; 62:601-606
  • Tremlett H, Paty D, Devonshire V. The natural history of primary progressive MS in British Columbia, Canada. Neurology 2005; 65:1919-1923
  • Tremlett H, Paty D, Devonshire V. Disability progression in multiple sclerosis is slower than previously reported. Neurology 2006; 66:172-177
  • Neurologic Impairment 10 Years After Optic Neuritis. Arch Neurol 2004; 61:1386-1389

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