Multiple sclerosis (MS) is a chronic disease affecting the brain and spinal cord. It involves damage to myelin, the protective coating around nerve fibers, disrupting signal transmission throughout the body. Progressive multiple sclerosis refers to a steady, gradual worsening of neurological function and disability over time. This progression occurs independently of the sudden flare-ups or attacks seen in other MS forms. It is distinct from the more common relapsing-remitting form, where symptoms appear and then improve before another attack.
The Two Forms of Progressive MS
Progressive multiple sclerosis manifests in two forms: Primary Progressive MS (PPMS) and Secondary Progressive MS (SPMS). These classifications characterize the disease’s trajectory and influence management.
Primary Progressive MS (PPMS)
Primary Progressive MS (PPMS) is characterized by a gradual accumulation of disability from the onset of symptoms, without initial relapses or periods of remission. This means a steady decline in neurological function from the disease’s beginning. PPMS accounts for 10% to 15% of MS cases. The worsening of symptoms in PPMS is primarily attributed to nerve damage or loss rather than inflammation.
Secondary Progressive MS (SPMS)
Secondary Progressive MS (SPMS) follows an initial period of relapsing-remitting MS (RRMS). During the RRMS phase, individuals experience distinct attacks followed by partial or complete recovery. Over time, many with RRMS transition to SPMS, where the disease shifts to a steady, gradual progression of disability. This progression can occur with or without occasional relapses.
Common Symptoms and Signs
Individuals with progressive MS often experience a range of symptoms that gradually worsen. These symptoms arise from ongoing damage to nerve fibers in the brain and spinal cord.
Mobility and Balance
Challenges with mobility and balance are common. Many individuals experience difficulty walking due to muscle weakness, stiffness (spasticity), or poor coordination. Spasticity causes muscles to feel tight and rigid, making movement cumbersome. Weakness, particularly in the legs, can also contribute to gait problems and an increased risk of falls.
Cognitive Function
Cognitive issues are a frequent symptom, affecting mental processes like memory, attention, and information processing speed. Individuals may find it harder to recall recent events, maintain focus, or process new information quickly. This “brain fog” can make work or complex tasks challenging.
Fatigue
Fatigue in progressive MS is a profound and debilitating symptom, distinct from normal tiredness. It can be overwhelming, appearing suddenly and severely, often interfering with daily routines regardless of rest. This fatigue results directly from the disease’s impact on the central nervous system.
Other Symptoms
Progressive MS can also lead to bladder and bowel dysfunction. Common bladder problems include urgency or difficulty emptying. Chronic pain, often neuropathic, can also occur, causing burning, tingling, or aching sensations. Vision problems, such as double vision or blurred vision, may also be experienced.
How Progressive MS Is Diagnosed
Diagnosing progressive MS involves a comprehensive evaluation. The diagnosis is typically reached by observing a pattern of symptoms and using various diagnostic tools to confirm MS and its progressive course.
Clinical Evaluation
A thorough medical history and neurological examination are initial steps in diagnosis. Doctors assess symptoms, looking for evidence of gradual, steady worsening of neurological function over at least one year. The neurological exam checks for changes in reflexes, coordination, balance, vision, and sensation.
Magnetic Resonance Imaging (MRI)
Magnetic Resonance Imaging (MRI) of the brain and spinal cord is a standard diagnostic tool for identifying lesions characteristic of MS. These lesions, also known as plaques, represent areas of myelin damage. While MRI confirms MS, differentiating progressive from relapsing forms can be challenging. Progressive MS often shows more spinal cord or brainstem lesions and fewer new enhancing brain lesions over time.
Cerebrospinal Fluid Analysis
A spinal tap (lumbar puncture) may be performed to collect cerebrospinal fluid (CSF) for analysis. This fluid is examined for markers of inflammation. The presence of oligoclonal bands or an elevated IgG index in the CSF can support an MS diagnosis. These findings suggest an immune response, a hallmark of MS.
Treatment and Management Approaches
Managing progressive MS involves a multi-faceted approach aimed at slowing disease progression and alleviating symptoms. While fewer disease-modifying therapies exist for progressive forms compared to relapsing-remitting MS, significant strides have been made.
Disease-Modifying Therapies (DMTs)
Disease-modifying therapies (DMTs) are medications that slow the accumulation of disability. For Primary Progressive MS (PPMS), ocrelizumab is an approved treatment. For Secondary Progressive MS (SPMS), several DMTs approved for relapsing-remitting MS may also be used, particularly if there is evidence of ongoing inflammatory activity, such as new relapses or new lesions on MRI. Examples include siponimod and cladribine, which are approved for SPMS. These therapies modulate the immune system to damage myelin and nerve fibers.
Symptom Management
Symptom management is a significant component of treatment, improving daily function and comfort. This often involves a multidisciplinary team, including neurologists, physical therapists, occupational therapists, and pain specialists. Medications address specific symptoms like spasticity, chronic pain, bladder dysfunction, or fatigue.
Rehabilitation and Wellness
Rehabilitation helps individuals maintain function and adapt to changes caused by progressive MS. Physical therapy improves strength, balance, and walking ability through exercises and assistive devices. Occupational therapy assists with adapting daily tasks and recommending tools for independence. Speech and language pathologists can also help with speech clarity and cognitive issues.
Mental health support, including counseling, helps manage emotional challenges. Lifestyle strategies, such as exercise and a balanced diet, support overall well-being and may mitigate symptom severity.