Multiple sclerosis





Abstract:


This chapter describes the rehabilitation management of people with multiple sclerosis (MS). The chapter reviews the incidence, prevalence, pathology, prognosis, and medical management of the disease. The chapter then focuses on the multidisciplinary management of disease sequelae resulting in impairments that limit the ability to perform activities and participate fully in life.




Keywords:

Multiple sclerosis, rehabilitation, multidisciplinary management, assessment, intervention, impairments, activities, participation, quality of life

 




Objectives


After reading this chapter the student or therapist will be able to:



  • 1.

    Describe the pathological processes, prevalence, and clinical presentation of people with multiple sclerosis.


  • 2.

    Compare and contrast the subtypes of multiple sclerosis and the common disease progression in each.


  • 3.

    Discuss the medical management of the disease and the disease symptoms, including diagnosis and treatment.


  • 4.

    Describe the concept of variability and how it applies to people with multiple sclerosis, pathology, medical management, and rehabilitation.


  • 5.

    Describe how the International Classification of Functioning, Disability and Health provides a common language for describing the impact of disease on people with multiple sclerosis and how it provides a framework for rehabilitation management.


  • 6.

    Describe the outcome measures that can be used to examine people with multiple sclerosis that cover body system problems (impairments), functional skill and activity limitations, participation restrictions, and quality of life.


  • 7.

    Develop a rehabilitation plan of care using evidence-based interventions to maximize patient function and quality of life.







Overview of multiple sclerosis


Multiple sclerosis (MS) is a progressive disease of the central nervous system that can impact the brain, spinal cord, and optic nerve. Because there is no known cure for the disease, rehabilitation is fundamental to management of the various symptoms and dysfunctions that occur. One key aspect of this disease for rehabilitation clinicians to understand is variability. This applies to how the disease initially manifests, how it progresses, and the medical and rehabilitation interventions used to slow disease progression and enhance function. The clinical presentation and disease progression of each person with MS will be unique. This requires clinicians to assess each patient/client individually and collaborate with them to determine the optimal plan of care.


Incidence and prevalence


MS is one of the most common causes of nontraumatic disability in young people and adults and is the most common inflammatory condition of the central nervous system (CNS). It is reported that approximately 400,000 people in the United States and over 2.5 million people worldwide have the disease. People are most commonly diagnosed between 20 to 50 years of age, with the average age around 31. However, MS can be diagnosed in people of almost any age. Approximately 3% to 5% of all people with MS are diagnosed before their 18th birthday, with an estimated 10,000 children and adolescents with MS in the United States. Another 15,000 people aged 18 years or less have symptoms consistent with MS.


MS is found in people who reside above the northern or below the southern latitude of 40 degrees with greater frequency than in those who live closer to the equator ( Fig. 17.1 ). Given the increased sun exposure of people living closer to the equator, lack of vitamin D is being investigated as a potential factor contributing to disease development ; there is a reduced relapse rate noted with higher blood levels. Other environmental risk factors include smoking, obesity, and exposure to infectious agents: Epstein-Barr virus and infectious mononucleosis are currently considered the most likely candidates. , Additionally, genetics may play a role in the development of MS. An identical twin with MS means that the other twin will have a 25% chance of diagnosis, suggesting something beyond genetics. Having a first-degree relative with MS will increase the risk of disease from 1 in 750 to 1 in 40. Currently, the most likely genetic factor linked to MS is a form of the HLA-DRB1 gene.




Fig. 17.1


World Distribution of Multiple Sclerosis.

(From Multiple Sclerosis Resource Center, www.msrc.co.uk .)


Women are affected 1.5 to 2.5 times more frequently than men. Men are, however, more likely to have a more aggressive disease progression and a worse prognosis. , It has been generally accepted that Caucasians with Northern European ancestry have the greatest incidence of MS, whereas people of Asian, African, or Hispanic ethnicity are at lower risk. However, these findings may represent a significant bias as few studies have looked at the incidence and prevalence of MS in many parts of the world, including Africa, Asia, and the Middle East, which may put the longitudinal hypothesis into question. A recent investigation in the US found that African Americans have a greater incidence than whites, with black females having the highest incidence. African Americans develop MS at an earlier age, have more frequent relapses and become disabled earlier than Caucasians, and have a greater risk of developing progressive disease. These factors suggest that tissue destruction occurs earlier and progresses more rapidly in this population. Additionally, African Americans with MS have poorer responses to disease modifying medications. In contrast, Inuits, Yakutes, Hutterites, Hungarian Romani, Norwegian Lapps, Australian Aborigines, and New Zealand Maoris do not appear to develop MS. Overall, being diagnosed with MS may be related to age, sex, genetics, geography, or ethnic background.


Subtypes of multiple sclerosis


Several MS disease phenotypes or subtypes have been identified ( Fig. 17.2 ). Importantly, the course of the disease is highly variable regardless of the subtype of MS.




Fig. 17.2


Types of Multiple Sclerosis.


The initial neurological episode or attack is typically identified as the clinical isolated syndrome (CIS). Symptoms must last for at least 24 hours and can be monofocal or multifocal. If lesions are present on magnetic resonance imaging (MRI), there is a high risk of developing MS. In one group of people with CIS followed for 20 years, 63% were eventually diagnosed with definite MS. The newly revised McDonald criteria for diagnosing MS22 encourages the identification of lesions on MRI that indicate prior disease activity, thus allowing earlier diagnosis (see Fig. 17.2 A).


Relapsing remitting MS (RRMS) (see Fig. 17.2 B) represents about 85% of people with MS, characterized by exacerbations (attacks, relapses) that can last days to months and are typically followed by periods of less active disease or remissions. During remissions, function can return to prerelapse levels, but most frequently recovery is not complete. Attacks normally occur with a frequency of one or two per year. Approximately 90% of people with RRMS transition to secondary progressive MS (SPMS) 10 to 15 years after diagnosis ( Fig. 17.2 C). ,


With SP MS, relapses may occur early but gradually lessen over time and convert to a steady progression of increasing disability and disease severity. It is thought that the clinical disability associated with SPMS results from the neurodegeneration that occurs as a result of tissue injury that accumulates from early in the disease process. In addition to less inflammation, there is a greater amount of brain atrophy in people with SPMS compared with RRMS. Fig. 17.3 shows the natural history of RMS and SPMS, comparing the change in brain volume with increasing clinical disability and disease burden.




Fig. 17.3


Natural history of relapsing remitting multiple sclerosis (RRMS) —conversion to secondary progressive multiple sclerosis (SPMS) . Figure shows the typical clinical course of RRMS with conversion to SPMS. Magnetic resonance imaging (MRI) activity (gray line and boxes) indicates the inflammatory lesions; they occur more frequently early in the disease and occur with greater frequency than in clinical disability (solid black line). Brain volume indicated by the stippled line shows brain atrophy increasing as the inflammatory component of the disease slows and is replaced by neurodegeneration.


Primary progressive MS (PPMS) is less common, affecting only 10% to 15% of people with MS. From disease onset, progression results in a gradual worsening of symptoms without relapses. People tend to be older when diagnosed (late 30s or early 40s), have fewer abnormalities on brain MRI, and respond less favorably to standard MS drug therapies. Progressive myelopathy is commonly associated with PPMS (see Fig. 17.2 D).


The term radiologically isolated syndrome (RIS) is used to describe a group of individuals whose imaging studies (MRI) strongly suggest the presence of MS; however, they have no clinical manifestations of the disease (see Fig. 17.2 E). These individuals are typically identified after MRI has been prescribed for an unrelated concern, such as headaches. Small studies indicate that about a third of these people develop MS within 5 years; there is an increased risk of developing MS in individuals with asymptomatic lesions in the spinal cord.


There is a push to categorize people with MS as either relapsing (RMS) or progressive (PMS), with primary or secondary as two types of progressive disease.


Pathology


Multiple sclerosis is an incurable autoimmune disease resulting from dysregulation of the immune system, both T-cells and B-cells. Initiation of immune system dysregulation may occur in response to genetic and environmental factors discussed above. The disease is characterized by inflammation, demyelination, axonal injury, and death that are present in lesions throughout the CNS. , Initially, MS was thought to be a disease of the white matter (WM) or myelinated parts of the neurons; however, the gray matter (GM), primarily neuron cell bodies, is significantly involved from early in the disease. Areas of demyelination and axonal damage interfere with normal conduction of neural signals, leading to a disruption of function.


Early in the course of the disease, lesions are composed of proinflammatory immune system components that produce demyelination, axonal injury, and loss of oligodendrocytes, referred to as active disease. Astrogliosis activated by the damaged neurons produces gliotic scarring called plaques. Plaques are visualized as sclerosis in postmortem brain tissue, hence the name multiple sclerosis. Active disease is followed by periods of remission in which acute inflammation is reduced ( inactive disease) . Axonal remyelination occurs but is highly variable and in RMS is related to recovery of function during periods of remission. Treatment in the initial stages of the RMS disease is aimed at reducing inflammation and immune system dysfunction with disease-modifying therapies (DMTs).


Later in the course of the disease inflammation becomes more diffuse while demyelination and axonal loss continue, suggesting a greater neurodegenerative process. In most individuals, disease progression becomes more constant with a lack of exacerbation. Owing to the lack of inflammation, DMTs are less effective in progressive forms of the disease.


Brief description of the common clinical manifestations


MS can affect the optic nerve and any tissue within the brain or spinal cord, so almost any neurological symptom can result. Individual assessments are required to identify the problems present. The following list, however, constitutes the most common problems encountered by people with MS.


Due to the heterogeneous nature of the disease, symptoms are widespread according to the part or parts of the CNS that are affected. Common symptoms include fatigue, weakness, spasticity, sensory impairments (vision, somatosensation, vestibular, and hearing), pain, bladder or bowel dysfunction, tremor, incoordination or ataxia, sexual dysfunction, disorders of emotion, dysarthria, and dysphagia. Dysfunctions related to the disease include mobility and balance impairments and cognitive deficits. The most commonly reported problems in RMS are paresthesia, fatigue, and weakness, while PMS includes balance dysfunction and fatigue. The symptoms most related to a reduced health-related quality of life for people with relapsing disease are balance dysfunction, spasticity, and depression while spasticity, paralysis, weakness, and pain have the greatest impacts in PMS.




Medical management


Diagnosis


Historically, people with MS would wait for a diagnosis for a year or more—sometimes even longer. Although there are no definitive tests that diagnose MS, the addition of MRI has accelerated diagnosis. In 2001 the International Panel on the Diagnosis of Multiple Sclerosis updated criteria to include MRI, visual evoked potentials, and cerebrospinal fluid (CSF) analysis published as the McDonald criteria. Revisions to these criteria occurred in 2005 and 2010, with the latest iteration in 2017.


Key requirements for MS diagnosis in the 2017 McDonald criteria include dissemination of lesions in the CNS in space and time. Demonstration of dissemination in space on clinical exam or MRI plus the presence of oligoclonal bands found in CSF enable the diagnosis of MS. Additionally, lesions on MRI with clinical sequelae can be used to demonstrate the presence of disease in space or time in patients with supratentorial, infratentorial, or spinal cord lesions. Cortical lesions can be used to indicate dissemination in space.


Due, in part, to the increased use of paraclinical assessments (MRI, CSF, and evoked potentials) the diagnosis of MS is occurring earlier, and treatment is begun more quickly with the hope that this will result in a slower accumulation of clinical disability or even reduction in the total disability experienced by individuals with MS. Even with the improved technological measures used to facilitate diagnosis, an accurate clinical history is critical. Historically, diagnosis of MS often required many years with the onset of more than one clinical episode of MS. Often patients recall episodes of transient symptoms that did not last long enough to require attention by a primary care provider.


MRI studies have shortened the time taken to diagnose MS. Although T2-weighted MRI images show MS lesions as hyperintense and identify new or active inflammatory lesions, MRI has been shown ( Fig. 17.4 ) to overestimate clinical relapses. Conventional MRI with T1 weighting identifies older lesions (with less inflammation) as hypointense (black holes) and can identify brain atrophy. T1 imaging demonstrates a stronger correlation with clinical status and disease severity than the lesion load found with T2 weighting. Gadolinium-enhanced T1-weighted MRI images also show active inflammatory MS lesions as hyperintense (white). Lesions identified on MRI that are characteristic of MS include periventricular, cortical or juxtacortical, and infratentorial lesions, as well as and lesions that span 1 to 2 segments in the spinal cord. Therefore the presence of both old and new lesions on MRI fulfills the criterion of dissemination in time.




Fig. 17.4


T2-weighted magnetic resonance imaging scan of plaques associated with multiple sclerosis. Plaques are indicated by arrows .

(From Frey H, Lahtinen A, Heinonen T, et al. Clinical application of MRI image processing in neurology. Int J Bioelectromagnet. 1999;1[1].)


Two additional paraclinical tests can be used to aid in the diagnosis of MS and differentiate it from other diseases and conditions. The first is analysis of CSF. This requires a lumbar puncture in which CSF is gathered and analyzed to identify oligoclonal bands indicating the presence of immune system proteins (immunoglobulins) or active inflammation. The majority of people with MS have oligoclonal bands; however, because people with other diseases or conditions also have oligoclonal bands, the test is not specific for MS. The lack of oligoclonal bands at diagnosis has been related to a slower progression of the disease and increased time to reach markers of disability such as walking with an assistive device.


Evoked potentials record the nervous system’s response to stimulation of a specific sensory pathway (visual, auditory, vestibular, or general somatosensory). Evoked potentials use an externally evoked sensory stimulus to activate the specific sensory system while the timing of the response to the stimulus is monitored. Demyelination and axonal degeneration cause a slowing of signal transmission along stimulated neurons and therefore will increase the time it takes to respond to the presented stimulus. Damage to the optic system is a common first symptom in MS, and therefore visual evoked potentials are often helpful in diagnosis.


Quantifying disease severity


Evidence supporting many interventions for individuals with MS has been researched according to how severely an individual is impacted by the disease. Severity of disease and progression can be monitored by ongoing clinical assessments, MRI imaging, and the use of several outcome measures. The Kurtzke Disease Severity Scale, an outcome measure, was initially developed to allow primary care providers a way to measure clinical disability and monitor disease progression. It has been replaced by the Expanded Disability Status Scale (EDSS) ( Table 17.1 ). The EDSS is a 11-point ordinal scale completed by a physician or physician extender, with 0 indicating no disability and 10 indicating death caused by MS. The scale addresses 8 functional systems (pyramidal, cerebellar, brain stem, sensory, bladder/bowel, visual, cerebral [mental], and ambulatory status). Mild disease severity is related to EDSS scores of 0 to 3, moderate disease 4 to 6.5, and severe disease indicated by scores greater than 6.5. Scores greater than 4 are primarily determined by ambulatory status. Using a unilateral assistive device such as a cane or crutch equals an EDSS score of 6.0, while a 6.5 indicates the need for bilateral assistance. Levels beyond 6.5 indicate a progressive nonambulatory status. The National MS Society (NMSS) Task Force on Clinical Outcomes Assessment also recommends the Multiple Sclerosis Functional Composite (MSFC) as a measure of disease severity and progression. This set of outcome measures is used to chart change in physical and cognitive function. It includes three tests that measure upper-extremity function (Nine-Hole Peg Test [NHPT]), lower-extremity function and mobility (25-Foot Timed Walk [25FTW]), and cognitive function (Paced Auditory Serial Addition Test [PASAT]). In the past, the EDSS and MSFC outcome measures have been used extensively in research evaluating the impact of various pharmaceuticals or rehabilitation interventions on people with MS. Disease severity can also be measured by therapist observation of ambulation (Disease Steps) or via patient self-report (Patient Determined Disease Steps) with both of these measures having high correlations with the EDSS.



TABLE 17.1

Expanded Disability Status Scale




































































Score Description
0 Neurologically normal
1.0 No disability, minimal signs in one FS
1.5 No disability, minimal signs in more than one FS
2.0 Minimal disability in one FS
2.5 Mild disability in one FS or minimal disability in two FS
3.0 Moderate disability in one FS, or mild disability in 3 or 4 FS. No walking impairments
3.5 Moderate disability in one FS and more than minimal disability in several others. No walking impairment
4.0 Significant disability and self-sufficient and able to be up and about 12 h a day. Able to walk without aid or rest 500 m
4.5 Significant disability and up and around much of the day, able to work a full day, may have some limitation of full activity or require minimal assistance. Able to walk without aid or rest 300 m
5.0 Disability severe enough to limit full day activity and ability to work a full day without special requirements. Able to walk without aid or rest 200 m
5.5 Disability severe enough to limit full day activity. Able to walk without aid or rest 100 m
6.0 Requires assistance to walk—one cane or crutch, etc. Able to walk 100 m with or without rest
6.5 Requires two walking aids—canes, crutches, walker, etc. Able to walk about 20 m without rest
7.0 Unable to walk more than 5 m with or without aid. Essentially restricted to wheelchair (WC) for mobility. Can independently propel self in WC, perform transfers. Able to be up and about for about 12 h a day
7.5 Unable to take more than a few steps. Restricted to WC and may need aid in transferring. Can wheel self and cannot carry on in a standard WC for a full day, may require a motorized WC
8.0 Essentially restricted to bed, chair, or dependent in WC. May be out of bed for much of the day. Retains many self-care abilities. Generally, has effective use of arms
8.5 Essentially restricted to bed much of the day. Has some arm function and retains some self-care abilities
9.0 Confined to bed. Can still communicate and eat
9.5 Confined to bed and totally dependent. Unable to communicate effectively or eat/swallow
10.0 Death due to MS

FS, Functional systems (FS include pyramidal, cerebellar, brain stem, sensory, bladder/bowel, visual, cerebral, and ambulatory status); MS, multiple sclerosis; WC, wheelchair.


Medical management of disease


Disease-modifying therapeutics


Disease-modifying therapeutics (DMTs) are aimed at reducing immune system dysfunction, thereby reducing damage to neural tissue and long-term disability for people with RMS. There are several different medications that act on various components of the immune system with the intention of modifying the course of the disease ( Table 17.2 ). These drugs can be injected, taken orally, or infused. In general, most of the drugs are approved for use with RMS and have been shown to reduce the number of attacks experienced. These drugs are often used off-label for other forms of MS. Measurement of therapeutic effectiveness includes relapse rate, progression of disability (EDSS), and quantitative evidence of lesions on MRI. All DMTs have side effects (see Table 17.2 ), and some of them are very serious. Medications with more serious side effects are generally used if drugs with fewer side effects have failed or produced breakthrough relapses. It is common that people will try several DMTs before finding the one they best tolerate.



TABLE 17.2

Disease Modifying Therapeutics for Multiple Sclerosis


























































Disease Modifying Agent Indication Common Side Effects
Injectable Medications (All Can Have Infusion Site Reactions)



  • IFN β-1a (Avonex, Rebif)



  • IFN β-1b (Betaseron, Extavia)




  • RMS




  • Flulike symptoms



  • Injection-site reactions



  • Depression



  • Elevated liver enzymes




  • Glatiramer acetate (Copaxone, Glatopa)




  • RMS




  • Systemic reactions/immediate postinjection



  • Elevated liver enzymes




  • Peginterferon β-1a (Plegridy)




  • RMS




  • Hepatotoxicity



  • Depression or suicidal thoughts




  • Daclizumab (Zinbryta)




  • RMS




  • Withdrawn from worldwide market 2018

Oral Medications



  • Dimethyl fumarate (Tecfidera)




  • RMS




  • Flushing



  • Diarrhea



  • Nausea



  • Pain



  • Elevated liver enzymes




  • Fingolimod (Gilenya)




  • RMS




  • Flulike symptoms



  • Increased liver enzymes



  • Headache



  • Diarrhea



  • Pain



  • Cough




  • Teriflunomide (Aubagio)




  • RMS




  • Liver damage



  • Diarrhea



  • Nausea



  • Influenza



  • Alopecia



  • Peripheral neuropathy

Infused Medications (All Can Result in Infusion Reactions)



  • Alemtuzumab (Lemtrada)




  • RMS




  • Infections (urinary, respiratory, viral, and fungal)



  • Itching



  • Pain and paresthesia



  • Dizziness



  • Diarrhea



  • Vomiting




  • Mitoxantrone (Novantrone)




  • RMS



  • SPMS




  • Cardiotoxicity



  • Treatment related leukemia



  • Infections



  • Alopecia



  • Amenorrhea



  • Nausea




  • Natalizumab (Tysabri)




  • RMS




  • Hepatotoxicity



  • Muscle weakness



  • Headache



  • Fatigue



  • Pain



  • Infections



  • Depression




  • Ocrelizumab (Ocrevus)




  • RMS



  • PMS




  • Itching and hives



  • Rash



  • Fatigue



  • Dizziness



  • Shortness of breath and coughing



  • Tachycardia



  • Headache



  • Pain


PMS , Progressive multiple sclerosis; RMS, remitting multiple sclerosis; SPMS , secondary progressive multiple sclerosis.


Ocrelizumab is a new drug that targets B-cells and is the first to be approved by the FDA for use with PMS. Several of the DMTs are typically used with RMS and PMS when there is evidence of inflammation in the form of relapses or active disease on MRI, but they appear to be less effective if inflammation is no longer present.


Management of acute relapses (antiinflammatory medications)


High-dose corticosteroids (such as prednisone or methylprednisolone) are used to reduce the inflammatory response during exacerbations for people with RMS. These drugs are generally given over 3 to 5 days via IV (Solu-Medrol) or orally (Deltasone). An additional medication is injection of HP Acthar Gel, a purified form of adrenocorticotropic hormone. Lastly, plasmapheresis can be used for severe relapses that do not respond to the other forms of treatment. Although no medications have demonstrated effectiveness in people with PMS, anecdotal evidence suggests that intermittent pulses of intravenous methylprednisolone can help slow progression of clinical disability in some patients.


Prognosis


More aggressive forms of MS are related to greater number of relapses in the first 2 years postdiagnosis, short intervals between first and second relapses, incomplete recovery from first attack and poor recovery from relapses, male sex, African American descent, older age at disease onset, initial symptoms involving the cerebellum or motor systems, or multifocal involvement at onset. , Paraclinical factors related to more destructive disease include greater disease burden on MRI, evidence of brain atrophy, and low serum levels of vitamin D. Previous research had reported that people with MS have a suicide rate that is twice that of the general population, with recently diagnosed younger males at greatest risk (Feinstein 2017).36a A systematic review found that suicide in MS was only slightly higher than an age-matched cohort. In general, people with MS live about 6 to 7 years less than healthy cohorts. ,




Multidisciplinary management of clinical manifestations


Overview


Physical rehabilitation has changed over the past 20 years for people with MS. Increasing evidence indicates that patients respond well to exercise that was previously thought to be too fatiguing or led to increased relapses in this population. Such evidence provides guidance for those who can apply exercise appropriately. Appropriate application, however, varies from patient to patient. The myriad locations and combinations of CNS lesions in MS, along with the differences in individual lifestyles and genetic makeup, result in variable clinical presentations. No one approach represents the gold standard for rehabilitation management. Thus clinicians must assess the preferences and needs of each individual before negotiating with the patient/client the best interventions to prescribe.


Rehabilitation for people with MS occurs in every setting: inpatient hospital, outpatient clinics, skilled nursing facilities, home care, and community-based support or exercise groups. With the current health care climate of decreasing access to and reducing coverage for rehabilitation, therapists and the interprofessional team must be able to make evidence-based decisions for intervention effectiveness to insurers as well as patients. Effective interventions across settings and disciplines achieve the goals of optimal physical and cognitive functioning, safety, and quality of life for the individual with MS.


Throughout the episode of care, the patient/client’s preferences and goals should guide rehabilitation—from assessment through intervention. An important framework for patient/client assessment is the International Classification of Functioning and Health: ICFH model (2001). See Chapter 1 for a complete description of this model. Fig. 17.5 describes the common problems that occur in people with MS.




Fig. 17.5


Common Problems That Occur in People With Multiple Sclerosis. CP , Cardiopulmonary; MS , musculoskeletal; NM , neuromuscular.


Understanding the patient or patient’s perspective is critical to achieving best results from rehabilitation. Several tools exist for documenting patients’ preferences and have been used with MS; the Goal Attainment Scale (GAS), the Canadian Occupation Performance Measure (COPM), and the Patient-Specific Functional Scale (PSFS). A more broad-based assessment of movement ability is the Movement Ability Measure (MAM) developed and validated by Allen (2007a and 2007b). , This measure includes the six dimensions of movement—flexibility, strength, endurance, speed, accuracy, and adaptability—based on the Movement Continuum theory. This self-report instrument records a patient’s current and preferred movement ability in the performance of daily activities during home, work, and leisure. See Fig. 17.6 for a sample of the questions for flexibility. Examining patient-identified gaps between current and preferred abilities should guide therapists’ assessment and interventions. In people with MS, these gaps correlate strongly with measures of physical performance, with the dimension of speed showing the greatest overall gap. Fig. 17.7 illustrates the Movement Ability levels and gaps from an individual with MS.




Fig. 17.6


Sample Questions From the Movement Ability Measure.



Fig. 17.7


Example of Patient Determined Gaps on the Movement Ability Measure.


To be most effective, rehabilitation must challenge each individual with sufficient intensit y and repetition , be specific to the goals of the patient/therapist, and be salient to the patient. Application of these five concepts are critical to produce a relatively permanent change in the capacity to perform physical activities and skilled movement via physiological adaptation. To determine the correct dosage of intervention, assessment must be finely tuned to the individual’s abilities and needs. Whenever possible, the goal should include restoration of impaired function rather than simply compensation for lost abilities as was common in the past. This section reviews and describes a multidisciplinary approach to addressing the common dysfunctions that occur with MS. It includes assessment tools for examining the patient’s and patient’s status, frameworks for applying rehabilitative techniques and interventions, and special considerations for rehabilitation in this population.


Assessment


The initial interview should include a quick screen or questioning about the body systems and areas that are commonly impaired in people with MS and the problems commonly encountered: motor strength, coordination, spasticity, sensory disruption (vestibular, visual, and somatosensory), bladder control, depression, and cognition. If impairments are present, there is a strong likelihood of negative impact on the patient’s ability to perform activities of daily living (ADLs) or participate in meaningful life roles related to work, home, and leisure.


Special dysfunctions associated with multiple sclerosis


All patients with MS must be asked if they have fallen in the last 6 months because of the high fall rate in this population. Results of the patient/client interview and chart review will help develop hypotheses about which potential impairments might be contributing to the patient’s or patient’s presentation and difficulty participating in meaningful life roles. These hypotheses can help guide the therapist’s examination plan. The examination needs to be designed to observe the problematic tasks identified by the individual and test the therapist’s hypotheses about the causes of these activity limitations or participation restrictions.


During the assessment, examiners must determine if the problems identified by the patient (or those found by the assessor) fit within their scope of practice or whether the patient requires a referral to an appropriate health care professional. The Patient Health Questionnaire-2 is a two-question depression screening tool found accurate for adolescents, adults, and older adults in the general population. See Fig. 17.8 . A score of 3/6 indicates that major depression is likely, particularly in groups at higher risk for depression (such as people with MS), and should trigger a referral to the patient’s primary care provider for follow-up. See Table 17.3 for signs and symptoms that may indicate a referral to another health care professional.




Fig. 17.8


Patient Health Questionnaire-2.

(Adapted from Patient Health Questionnaire [PHQ] Screeners. www.phqscreeners.com . Accessed October, 2018.)


TABLE 17.3

Common Referrals for People With Multiple Sclerosis

























Signs/Symptoms Appropriate Health Professional Referral
Changes in vision Neuroophthalmologist/optometrist
Bladder dysfunction (nocturia, urgency, frequency, retention) Neurologist, urologist, neurourologist, and pelvic physical therapist
Increase/change in spasticity/muscle tone Neurologist
New onset of potential exacerbation Neurologist
Depression/anxiety/other mental health manifestations Psychologist
Balance deficits, falls, weakness Physical therapist


The physical examination should start with testing the patient’s ability to perform functional activities that the patient or his or her caregivers have identified as problematic. Activities may include performance of transfers, gait, ADLs, or cognitive tasks as well as the specific activities that the person states are impaired in his or her work, home, and recreational life. Examining the movement analysis of patient-identified key functional tasks will likely give the therapist insights into which body, structure, and function impairments are influencing the patient’s ability to complete the task in an optimal manner.


To gain a greater understanding of a patient’s prognosis and current presentation and track changes throughout the rehabilitation program, therapists may consider choosing a couple of outcome measures. Outcome measure selection is unique for each individual and should be dependent on the patient’s chief complaints and therapist-identified problematic areas. It is recommended that both subjective and objective outcome measures are included to gain a greater understanding of patient perspective and presentation. Comprehensive lists of standardized tests and measures for impairment, activity limitations, and participation restrictions and QOL are provided in Chapter 7 . The next section of this chapter will focus on the tests and outcome measures found to be valid and reliable in the examination of individuals with MS. In 2011, the Academy of Neurologic Physical Therapy, a component of the American Physical Therapy Association, charged a group of experts in MS to recommend outcome measures for use in clinical practice, education, and research. The recommendations of this group were published in 2014 and are included in Fig. 17.9 . Through a modified Delphi process, this group chose outcome measures based on an extensive review of the literature concerning psychometric properties and clinical utility of outcome measures in people with MS. The critical review and recommendations for each outcome measure were entered into the Evaluation Database to Guide Effectiveness (EDGE) form and are available at: www.google.com/search?q=APTA+MS+EDGE&ie=utf-8&oe=utf-8&patient= firefox-b-1 . Measures were chosen for use in people across the span of EDSS levels; across the ICF constructs of (1) body structure and function, (2) activity, and (3) participation; in settings in which patients with MS are managed—inpatient, outpatient, and at home; and use of the outcome measures in clinical practice, education, and research. Ratings of the outcome measures ranged from 1 to 4 with 4 representing the highest recommendation and 1 indicating not recommended due to low clinical utility, poor psychometric properties, and or the measure was not tested in MS.




Fig. 17.9.


APTA MSEDGE Recommended Outcome Measures.


Assessing impairments and activity


In general, standardized methods of examining muscle strength and endurance, somatosensation, vision, coordination, cardiovascular status and endurance, posture, muscle tone, reflexes, fatigue pain, and cognition are useful in examining individuals with MS. As with many neurological conditions, abnormal posturing or pain may necessitate using nonstandardized test positions or methods that must be noted in the patient documentation. If a patient is unable to attain the normal test position while performing a muscle strength test, the assessed strength is documented along with the position in which the muscle or muscle group was tested. Functional movement analysis is used to assess patients’ and patient’s ability, strategies, and the quality of movements used to perform ADLs such as transfers, bed mobility, and gait. Outcome measures standardize the assessment of activity or task performance and are an excellent way to document change in ability over time.


Spasticity.


Therapists can use several tools to assess spasticity in people with MS. Clinicians typically measure spasticity using the Modified Ashworth Scale. The numerical rating scale (NRS)—spasticity is good scale for assessing change over a single session or intervention. These two scales have been shown to have a moderate correlation in people with MS (Anwar, 2009).55 Clinicians should be aware that because spasticity is typically measured while the patient is at rest in the MAS protocol, the scores may not reflect the degree to which spasticity may be interfering with movement. Careful observation of the patient’s movements may also inform the clinician about how spasticity is affecting the patient’s physical function. Several spasticity self-report measures are recommended by the MS EDGE Taskforce and might be more valuable to assess change in the perception of the impact of spasticity on function over time, such as across an episode of care (see Fig. 17.9 ).


Ataxia and incoordination.


Few standardized tests have been developed to specifically measure ataxia. The Scale for the Assessment and Rating of Ataxia (SARA) , and the International Cooperative Ataxia Rating Scale (ICARS) are examples of standardized tests that clinicians may use to assess an individual’s ataxia. Both the SARA and ICARS have good psychometric properties and have been tested in people with MS. The ICARS includes four subsection assessments of posture and gait, limb ataxia, dysarthria, and the oculomotor system. The maximum score is 100 with higher scores indicating greater disability. While the ICARS has shown high correlation with EDSS, the clinical utility of the SARA may be greater due to its brevity. The SARA does not include an ocular motor examination; therefore clinicians should be sure to include these tests in other portions of their evaluation. Due to cerebellar damage or loss of somatosensation and proprioception, people with MS can also experience uncoordinated movement.


Tests of nonequilibrium coordination are designed to measure the presence of dysmetria or dysdiadochokinesia, both of which occur in patients with MS. However, these tests (including finger to nose and heel to shin) are somewhat subjective and are therefore difficult to use to demonstrate improvement pre- and post-intervention. However, using a stopwatch during these tests can increase the objectivity of the measurements. Counting the number of repetitions of a given activity performed in a set amount of time (e.g., how many alternating forearm supinations and pronations can be performed in 30 seconds), or recording the time it takes to complete a set number of repetitions of a given activity (e.g., how long it takes to complete five alternating supination-pronation movements), can quantify otherwise subjective impressions. Refer to Chapter 7 for additional assessment tools.


Vestibular dysfunction.


The vestibular system can be affected by MS both centrally (lesions in the vestibular nucleus, cerebellum, or sensory pathways) or peripherally with conditions such as benign paroxysmal positional vertigo. The techniques used to assess vestibular disorders are similar to those discussed in Chapter 21 . Computerized platform posturography (CPP) assessment is reliable and valid for people with MS and can identify vestibular dysfunction. It is important to keep in mind that people with MS will often have additional problems that might require modification of the vestibular intervention—for example, heat intolerance, additional visual or somatosensory deficits, spasticity, and motor weakness.


Fatigue.


Identifying if and when fatigue occurs in individuals with MS is important to assessment and the structuring of intervention. Questions should address the type of fatigue, whether mental or physical; when during the day it occurs; whether it is related to physical or mental exertion; and what the person with MS does, if anything, to relieve it. In addition, fatigue-related self-report scales can help the rehabilitation professional gain an understanding of the perceived impact that fatigue may be having on a patient with MS. Two of the commonly used self-report scales recommended by the MS EDGE Taskforce are the Modified Fatigue Impact Scale (Fisk 1994) and the Fatigue Scale for Motor and Cognitive Function. These measures may also aid the therapist in determining if the intervention had any impact on the patient’s perceived level of fatigue.


Cognition.


Cognitive deficits common to people with MS include deficits in learning and memory, information processing speed, and executive functions such as planning, organization, and initiation. In MS, delays in processing speed are connected to learning impairments. The Paced Auditory Serial Addition Test (PASAT) is recommended by an expert panel of the National MS Society as a test for disease-related cognitive impairments in people with MS. Components of the PASAT specifically assess working memory and processing speed.


Balance.


Balance is foundational to upright movement and is produced by a complex interaction among sensory inputs, central processing, and motor responses. It can be discussed under both body structure and function or activity. In either case balance dysfunction has been identified in people with MS with minimal as well as more advanced disability. , Cameron and Lord report the three most common problems with balance to be delayed response to postural perturbations, increased body sway while standing quietly, and an inability to move outside the base of support.


Some balance tests focus on stationary or static tasks that allow observation of body sway in standing, including single-leg stance test, Romberg test with eyes open or eyes closed, tandem stance, and CPP; others add movement and challenge dynamic balance. Other tests challenge anticipatory balance (reactions to perturbations related to self-generated movement) or reactive balance. The MS EDGE Taskforce recommends Berg Balance Scale and Functional Reach Test to assess balance. See Fig. 17.9 . Frzovic and co-workers found that single-leg stance, tandem stance, response to external perturbations, and the Functional Reach Test were able to distinguish people with MS from healthy controls.


Developed by Horak and colleagues, the Balance Evaluation Systems Test (BESTest) is an instrument examining complex balance disorders that includes the six domains that underlie orientation and postural stability: biomechanical constraints, stability limits and verticality, transitions and anticipatory postural reactions, reactive postural responses, sensory orientation, and stability in gait. The BESTest has been shown valid and reliable for people with MS. The authors suggest that total test scores should be used because several of the subsections of the test showed ceilings effects. There is an abbreviated version of the BESTest, the mini-BESTest, that covers four of the six systems, focusing on dynamic balance. These promising tests may offer the clinician a better way of identifying which components of orientation and postural control are dysfunctional and which may allow more targeted interventions. A systematic review of the use of the miniBESTest demonstrated it to be reliable, valid, and responsive in community-dwelling elders, people with Parkinson disease, and people post-stroke.


CPP provides an objective assessment of sensory contributions to balance dysfunction in people with MS, which is reliable in people with MS. , The Sensory Organization Test (SOT) is useful in identifying the relative sensory contributions (visual, vestibular, and proprioceptive) to stationary balance and response to perturbation. Understanding the sensory conditions under which the patient loses balance and falls assists the therapist in providing exercises that will challenge those conditions in a safe and controlled manner. For example, the patient who relies heavily on visual input to maintain balance (conditions with eyes closed in the SOT) would be prescribed exercises and activities that challenge the vestibular and proprioceptive systems, such as standing on foam while the eyes are closed. An additional measure that may be more assessable for clinical use is the Nintendo Wii Balance Board (WBB). Keune and co-workers found excellent internal consistency and reliability when testing individuals with MS for 4 minutes on the WBB, 4 months apart. They found that WBB had consistent results with both the Timed Up and Go test and the Berg Balance Scale (BBS) and showed increased body sway in people with EDSS scores greater than 3.


Two self-report measures of balance ability and confidence are Activity-specific Balance Confidence scale (ABC) and the Dizziness Handicap Inventory (DHI). Both have been used extensively in MS. Cattaneo and colleagues found that both the ABC and DHI tools discriminated between fallers and nonfallers and were therefore good predictors of fall status in people with MS. Refer to Chapter 20 for additional information on balance.


Gait.


Gait can be measured in myriad ways depending on the goal of the assessment. Speed, distance, and quality may all be important to the patient and therapist. Observational gait analysis is the gold standard for clinical measurement of gait quality. Although motion-analysis laboratories are able to provide detailed kinetic and kinematic assessment of joint angles and gait cycle, it is costly and typically not available in most clinical settings. Instrumented mats can provide clinicians with temporal and spatial gait parameters such as step length, step width, cadence, and single-leg support and double-leg support times. Although this is less costly than motion analysis, it may still be out of reach for many clinics. Gait speed and velocity can also be measured by having the patient walk a given distance while being timed. These walks can occur at a self-selected pace or as fast as the person can walk safely. See Fig. 17.9 for recommended gait assessment measures. Gijbels and co-workers report that the 6-Minute Walk Test (6MWT) was better at predicting habitual walking in people with mild to moderate MS than the 25-Foot Timed Walk (25FTW). However, the 25FTW may be more sensitive to change when compared with the EDSS. The 6MWT distance was reduced in people with MS compared with healthy controls and was inversely related to disability.


The Multiple Sclerosis Walking Scale–12 (MSWS-12) , is a 12-item patient-rated questionnaire that measures the perception of the impact of MS on walking ability and is recommended by the MS EDGE Taskforce.


Upper-extremity tests of function.


Movement impairments of the upper extremities can result in decreased ability to perform ADLs and other functional activities. Standardized tests such as the Box and Block Test (BBT) or the Nine-Hole Peg Test (NHPT) provide objective data about unilateral manual dexterity or the ability to manipulate objects. Both tests are inexpensive but do require some equipment and a stopwatch. The NHPT is part of the MSFC and therefore has been used extensively in evaluating people with MS.


Assessing quality of life and participation.


For people with MS, participation restrictions are associated most strongly with cognitive deficits and to a lesser amount with walking, balance, and upper extremity dysfunctions. The following are participation and quality of life (QOL) measures commonly used in people with MS. See Fig. 17.9 for a list of the measures recommended by the MS EDGE Taskforce. An individual’s perceived ability to participate may also be included in some QOL outcome measures that are included in the following sections.


QOL measures are patient-report tools that evaluate the value a person places on his or her abilities and limitations and how these affect the individual’s social, emotional, and physical well-being. Many of these tools include questions that address an individual’s perception of how well he or she is able to fulfill life roles and how the disease affects this participation. In a meta-analysis of exercise training on QOL in people with MS, Motl and Gosney found that disease-specific measures of QOL detected larger changes than generic QOL measures. The two highly recommended outcome measures are the Multiple Sclerosis Quality of Life–54 (MSQOL-54) and the MS Impact Scale–29. While both have strong psychometrics, the MS Impact Scale is easier to score and is a shorter questionnaire for patients/clients to take.


The MSQLI was developed by the Consortium of Multiple Sclerosis Centers Health Research Subcommittee in 1997. It is composed of 10 components covering issues important in MS. It includes the Health Status Questionnaire, Modified Fatigue Impact Scale, MOS Pain Effects Scale, Sexual Satisfaction Survey, Bladder Control Scale, Bowel Control Scale, Impact of Visual Impairment Scale, Perceived Deficits Questionnaire, Mental Health Inventory, and MOS Modified Social Support Survey. It takes about 45 minutes to administer the complete set of questionnaires and does not provide a sum score for all tests. There is good test-retest reliability for the MSQLI even in people with MS and cognitive dysfunction. A shortened version of the tool exists, but the psychometric properties have not been thoroughly tested. The MS EDGE task force document recommends that the MSQLI be used only in the outpatient MS population.


Interventions


Benefits of exercise in multiple sclerosis


Exercise is an important intervention given that people with MS are less physically active than their healthy peers. Exercise is one of the interventions that have shown consistent benefits for people with MS, similar to healthy adults and people with other neurological dysfunctions. The literature examining the impact of exercise on impairments, activity, and participation/quality of life for people with MS has exploded in the past 10 years. A recent review reiterated that exercise is safe and well tolerated in this population.


The benefits of exercise have been determined by both systematic review and meta-analysis. Meta-analysis has confirmed the benefits of exercise on the impairments of aerobic capacity, lower extremity muscle strength, fatigue, and depression ; reduced activity limitations such as walking performance and balance ; and confirms that it positively impacts quality of life. , Systematic reviews have also revealed that exercise reduced fatigue, improved cognition, and improved health-related quality of life.


In addition to the benefits outlined above, Motl and Pilutti reviewed the growing evidence that exercise may play an additional role as a disease-modifying treatment. They reported that potential slowing of disease progression and reducing relapse rate, , a reduced progression of walking disability, , and decreased lesion volume on MRI. , However, according to a recent systematic review, the impact of aerobic or resistance training on markers of inflammation have shown equivocal results. The authors hypothesize that the reason for a lack of results may be due to two main factors: the low level of fitness in people with MS compared with a healthy cohort and training periods of less than 8 weeks duration in most of the studies.


While evidence supporting the benefits of increased physical activity and exercise is clear, in order for people with MS to achieve the best result, exercise prescription must be tailored to the individual’s needs. Appropriate assessments must be completed to link deficits in underlying capacity and movement with the necessary exercise intervention. Table 17.4 presents a summary of the exercise guidelines for aerobic, resistance, and flexibility training for people with MS based on guidelines from White and Dressendorfer, Dalgas and colleagues Latimer-Cheung and colleagues and the American College of Sports Medicine. When beginning an exercise program, the initial prescription should be based on the goals of the individual, the initial level of fitness, familiarity with the type of exercise, and the individual’s preferences for type of exercise. As with every exercise intervention, the intensity, duration, and frequency should be gradually increased with the individual’s tolerance. For people with MS that have heat intolerance, intermittent exercise and shorter exercise bouts, interspersed with periods of rest that allow heat to dissipate, will allow a greater volume of exercise to be performed. , Cooling before exercise has improved physical performance in people with MS.


Apr 22, 2020 | Posted by in NEUROLOGY | Comments Off on Multiple sclerosis
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