What are the other Names for this Condition? (Also known as/Synonyms)

• SPMS (Secondary-Progressive Multiple Sclerosis)

What is Secondary-Progressive Multiple Sclerosis? (Definition/Background Information)

• Multiple sclerosis (MS) is a common and chronic autoimmune disorder affecting the central nervous system (brain and spinal cord). In this disorder, the myelin sheath, a protective layer surrounding the nerve cells is progressively destroyed (called demyelination), resulting in associated neurological signs and symptoms
• The following four main courses of multiple sclerosis is reported, but not all courses may be observed in each individual:
  • Clinically isolated syndrome (CIS)
  • Relapsing-remitting multiple sclerosis (RRMS)
  • Secondary-Progressive Multiple Sclerosis (SPMS)
  • Primary-progressive multiple sclerosis (PPMS)
• Secondary-Progressive Multiple Sclerosis may be described as an advanced stage of multiple sclerosis that develops 10-20 years after relapsing-remitting multiple sclerosis. It is generally observed in middle-aged to slightly older adults
• In individuals with Secondary-Progressive Multiple Sclerosis, the neurologic function keeps deteriorating and the cycles of relapses and remissions, observed with RRMS, gets lesser and lesser, since the nerve sheath inflammation and destruction has now progressed to irreversible nerve damage. The destruction results in a neurodegenerative process, which makes the demyelination disorder potentially more severe
• The condition is termed secondary-progressive because individuals show increased disability only after the relapsing and remitting period has ended, unlike primary-progressive, wherein individuals start showing a progressive worsening of the neurologic symptoms right from the beginning of multiple sclerosis itself
• The common signs and symptoms associated with Secondary-Progressive Multiple Sclerosis include memory problems, chronic fatigue, dizziness, muscle stiffness causing walking difficulties, and vision problems
• It is important to note that not all individuals with relapsing-remitting multiple sclerosis develop Secondary-Progressive Multiple Sclerosis; it is also difficult to predict who develops the condition. Current standards of medication therapy are also observed to delay progression of RRMS to SPMS

Who gets Secondary-Progressive Multiple Sclerosis? (Age and Sex Distribution)

• Secondary-Progressive Multiple Sclerosis is most commonly observed in middle-aged and older adults; although most cases are noted between 30 and 40 years
• Both males and females are affected, and no gender preference is noted; some studies however show a slight male or female preference
• The condition is observed worldwide, and individuals of all racial and ethnic groups may be affected. Some studies indicate that Caucasians have a higher risk for SPMS
• Some reports indicate that approximately 85% of the individuals with relapsing-remitting multiple sclerosis develop SPMS

What are the Risk Factors for Secondary-Progressive Multiple Sclerosis? (Predisposing Factors)

The primary risk factor associated with Secondary-Progressive Multiple Sclerosis include individuals with MS who develop the relapsing-remitting type of multiple sclerosis. The following risk factors are described:

• Individuals, who have undergone inadequate treatment of RRMS, may have an increased risk for SPMS
• Individuals who are older when MS is initially diagnosed in them
• Those who have frequent and severe flare-ups and present a greater number of lesions on the brain and spinal cord on an MRI scan on initial diagnosis
• Those who do not recover completely following phases of flaring-up or exacerbations in RRMS
• Individuals in whom an involvement of the spinal cord is more prominently observed than the brain (during RRMS)

In general, the risk factors associated with the development of multiple sclerosis include:

• Young adult women are known to have a higher risk
• Family history of multiple sclerosis
• People of Northern European descent
• Environmental triggers: People living in certain countries with temperate climate, such as Southern Canada, Northern United States, New Zealand, and Europe, report a greater number of MS cases; hence, certain environmental factors may be involved
• Presence of autoimmune diseases such as type 1 diabetes mellitus and thyroid disease
• Smoking
• Studies have also shown that a lack of vitamin D may lead to MS
• Epstein-Barr virus (EBV) infection
• Individuals with certain HLA gene types have an increased risk for MS

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Secondary-Progressive Multiple Sclerosis? (Etiology)

The exact cause of multiple sclerosis (MS) is unknown, but it is considered an autoimmune disorder. In MS, the body’s own immune system attacks the central nervous system, resulting in a set of neurological symptoms.

• Secondary-Progressive Multiple Sclerosis develops from relapsing-remitting multiple sclerosis (RRMS) over a few decades (median period 19 years), although the exact reason behind this progression is not well-established
• Even though it is not presently possible to predict the course of RRMS, studies indicate that this may be influenced by several factors that include:
  • Ineffective treatment of RRMS
  • Individuals who have severe flare-ups quite regularly, from which they do not recover entirely (or take much longer to do so)
  • When more spinal cord associated symptoms are observed in the RRMS stage
  • Individuals who are older at first diagnosis of MS
• In relapsing-remitting multiple sclerosis, there are regular inflammatory attacks on the nerve fiber sheath that cause local nerve damage and signal disruption. The condition is characterized by a series of relapses and remissions that tend to progressively worsen over several years
• Secondary-Progressive Multiple Sclerosis reportedly develops when inflammatory attacks on the central nervous system result in increased nerve damage and nerve loss to the point that recovery is not possible anymore, meaning it becomes permanent. Research scientists explain this by the decrease in numbers of relapses and remissions (with milder nerve inflammations), though symptom severity and disability progressively increase

What are the Signs and Symptoms of Secondary-Progressive Multiple Sclerosis?

Secondary-Progressive Multiple Sclerosis is characterized by reduced number of relapses and remissions that tend to slowly stop. This course of MS is also characterized by marked worsening of neurologic function and increased disability in the affected individuals, since there is only a partial recovery from the relapses (or incomplete remissions).

Based on how the condition progresses over time, it is described as:

• Active and progressive, showing new activity on MRI scans and disease worsening
• Active and non-progressive, showing new activity on MRI scans but without any worsening of the disease
• Inactive and progressive, which is marked by the absence of new activity on MRI scans, but showing worsening of the condition
• Inactive and non-progressive that is marked by the absence of new activity on MRI scans and no progression of the condition

Evaluation of the above immensely aids the healthcare provider in understanding the course of SPMS and in bringing about any treatment modifications and rehabilitation strategies that may be necessary.

The speed of progression of SPMS and the signs and symptoms may be significantly different from one individual to another. The commonly observed signs and symptoms include:

• Severe and longstanding fatigue
• Cognitive issues including poor memory
• Poor bowel and bladder function
• Poor eyesight
• Poor hand-eye-body coordination
• Sexual-related problems
• Numbness and tingling sensation
• Dizziness
• Unable to stand or walk due to weakened and/or stiffened leg muscles; muscle spasticity
• Depression

How is Secondary-Progressive Multiple Sclerosis Diagnosed?

A diagnosis of Secondary-Progressive Multiple Sclerosis (SPMS) is challenging since the condition gradually develops from relapsing-remitting multiple sclerosis (RRMS), over several years and decades. The diagnosis may involve the following tests and exams:

• Complete evaluation of medical history and family history, along with a thorough physical exam
• Evaluation of the presenting signs and symptoms and their progression over time following the onset of RRMS is important. The healthcare provider may use the expanded disability status scale (EDSS) to measure progression of multiple sclerosis
• Neurological examination that involves the central nervous system (brain and spinal cord)
• Magnetic resonance imaging (MRI) scan of the brain and spinal cord; a vast majority of the individuals show abnormal MRI scan results
• Spinal tap: Cerebrospinal fluid is examined for the presence of oligoclonal bands (these are antibodies present in the spinal fluid of individuals with multiple sclerosis)
• Visual evoked potential (VEP) test: The test measures the electrical activity in the scalp region in response to a light stimulus. Individuals with MS may have an abnormal VEP test
• Nerve function test
• Specialized blood tests to rule out other disorders or conditions with signs and symptoms similar to multiple sclerosis
• A diagnosis generally involves ruling out other conditions of the central nervous system that may cause similar neurological symptoms

Even though it is difficult to establish a criterion for the diagnosis of Secondary-Progressive Multiple Sclerosis, the neurologist may assess progression of the RRMS condition observing the patient for a prolonged period (at least 6 months), including noting the frequency and numbers of relapses and remissions, prior to arriving at a diagnosis of SPMS.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Secondary-Progressive Multiple Sclerosis?

The complications associated with Secondary-Progressive Multiple Sclerosis may include:

• Arms and legs shake due to seizure-like muscle spasms
• Loss of balance
• Increased use of assistive devices, such as walkers or walking sticks, including motorized wheelchairs for mobility
• Trigeminal neuralgia - a chronic condition involving the trigeminal nerve causing severe pain and burning sensation on the face
• Migraine headaches
• Severe emotional distress
• Abnormal sense of touch, strange sensation to heat and cold
• Sudden sensation of electric shocks that are ‘felt’ down the spinal cord
• Difficulty eating, swallowing, and performing simple daily tasks due to poor coordination
• Vision loss
• Adverse effects from the long-term use of medication

How is Secondary-Progressive Multiple Sclerosis Treated?

Following a diagnosis of multiple sclerosis, the healthcare provider in consultation with the patient decides on the treatment plan and the use of prescription medications. However, since medications that help control and manage multiple sclerosis tend to change the course of the condition, such therapies are termed disease-modifying therapies (immunotherapies).

Any combination of the following medications may be administered after careful consideration by the healthcare expert, in treating Secondary-Progressive Multiple Sclerosis:

• Beta interferons
• Use of monoclonal antibodies
• Glatiramar acetate (immunomodulator drugs)
• Oral sphingosine 1-phosphate (S1P) receptor modulator medication
• Use of immunosuppressive medications, when other medications prove to be ineffective in controlling the symptoms
• Anti-inflammatory medications
• Other medications are administered to reduce inflammation, reduce nerve damage, and prevent destruction of the myelin sheath

Apart from the above, certain other treatment measures may be needed, depending on the severity and duration of the condition. These include:

• Acute attacks of the disorder are treated with corticosteroids and plasma exchange therapy (plasmapheresis)
• Pain medications, when required
• Use of other drugs such as baclofen (to reduce muscle spasm) or benzodiazepine
• Physical therapy is also helpful in treating the symptoms of the disorder
• Cholinergic drugs can be used to decrease urinary-related signs and symptoms
• Stem cell treatment (HSCT): It is an investigative therapy for consideration in clinical trials for patients with progressive MS
• Anti-depressants may be used for mood or behavior associated conditions
• Medications for sexual dysfunction (including erectile dysfunction)
• Physical therapy including occupational therapy and rehabilitation
• Healthy living including a well-balanced diet and exercising, including adequate sleep

How can Secondary-Progressive Multiple Sclerosis be Prevented?

• Currently, there are no effective preventative methods available for multiple sclerosis including Secondary-Progressive Multiple Sclerosis
• Adequate and effective treatment of relapsing-remitting multiple sclerosis is known to delay progression of the condition to SPMS

What is the Prognosis of Secondary-Progressive Multiple Sclerosis? (Outcomes/Resolutions)

Presently, multiple sclerosis is an incurable condition and its prognosis is difficult to predict. Nevertheless, in many, the life expectancy can be normal or near-normal. Most individuals with MS are able to continue with their life and work with minimal disabilities.

• Secondary-Progressive Multiple Sclerosis may be described as an advanced stage of MS; the prognosis depends on its severity and long-term course
• Regular medical evaluation is essential to assess how the disease is progressing and if any changes are necessary to be brought into the treatment plans
• According to study reports, nearly two-thirds of the affected individuals may need walking aids, but generally remain mobile

Additional and Relevant Useful Information for Secondary-Progressive Multiple Sclerosis:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/health-topics/neurological-institute/