Multiple Sclerosis

A progressive autoimmune disease of the CNS

Authors
Affiliations

Nate Yomogida, B.S., SPT

Doctor of Physical Therapy

B.S. in Kinesiology

Chloë Kerstein, B.S., SPT

Doctor of Physical Therapy

B.A. in Neuroscience

Resources
AKA
  • Charcot’s triad

Multiple sclerosis (MS) refers to a progressive autoimmune disease where the immune system attacks oligodendrocytes resulting in demyelination of the brain, spinal cord, CNI Olfactory n., and CNII Optic n.. MS is characterized by inflammation, selective demyelination, and gliosis.

Etiology

Genetics

It appears that although individuals do not inherit the disease, they may inherit a genetic susceptibility to immune system dysfunction

Pathogenesis

Molecular mimicry

  • An autoimmune response when individuals with a genetic susceptibility are exposed to a viral agent

Major Pathologic Mechanisms that result in clinical manifestations

Theories

Theories(Olek and Mowry 2023)

Types of MS

Secondary progressiveRelapsing remittingPrimary progressiveProgressive relapsingTimeSeverity
Figure 1: MS Types

Relapsing remitting (RRMS)

  • Short duration attacks with full or partial recovery
  • May or may not leave lasting symptoms/deficits
  • Most common
Secondary progressiveRelapsing remittingPrimary progressiveProgressive relapsingTimeSeverity
Figure 2: RPMS time-severity pattern

Primary progressive (PPMS)

  • Worsens over time
  • Lack of attacks/exacerbations
  • Steady increase in disability
Secondary progressiveRelapsing remittingPrimary progressiveProgressive relapsingTimeSeverity
Figure 3: PPMS time-severity pattern

Secondary progressive (SPMS)

  • Combo of primary progressive and relapsing remitting
  • Initially relapsing remitting then turns to progressing as sx inc without remission periods
Secondary progressiveRelapsing remittingPrimary progressiveProgressive relapsingTimeSeverity
Figure 4: SPMS Time-severity pattern

Progressive relapsing (PRMS)

  • Worst type
  • Steady inc in disability with attacks
  • Eliminated from usage in 2013 (this type)
Secondary progressiveRelapsing remittingPrimary progressiveProgressive relapsingTimeSeverity
Figure 5: PRMS Time-severity pattern with diagonal connections

EDSS Scale

Score Rating Criteria
0.0 Normal neurological exam All FS Scores were grade 0
1.0 No disability Minimal signs in one FS (i.e., grade 1)
1.5 No disability Minimal signs in more than one FS* (more than 1 FS grade 1)
2.0 Minimal disability in one FS (one FS grade 2, others 0 or 1)
2.5 Minimal disability in two FS (two FS grade 2, others 0 or 1)
3.0 Moderate disability in one FS One FS grade 3, others 0 or 1
Or
mild disability in 3-4 FS (three or four FS grade 2, others 0 or 1) though fully ambulatory.
3.5 Fully ambulatory but with moderate disability in one FS (one grade 3) and one or two FS grade 2; or two FS grade 3 (others 0 or 1) or five grade 2 (others 0 or 1)
4.0 Fully ambulatory without aid, self-sufficient, up and about some 12 hours a day despite relatively severe disability consisting of one FS grade 4 (others 0 or 1), or combination of lesser grades exceeding limits of previous steps; able to walk without aid or rest greater than 500 meters.
4.5 Fully ambulatory without aid, up and about much of the day, able to work a full day, may otherwise have some limitation of full activity or require minimal assistance; characterized by relatively severe disability usually consisting of one FS grade 4 (others or 1) or combinations of lesser grades exceeding limits of previous steps; able to walk without aid or rest greater than 300 meters
5.0 Ambulatory without aid or rest for about 200 meters; disability severe enough to impair full daily activities (e.g., to work a full day without special provisions); Usual FS equivalents are one grade 5 alone, others 0 or 1; or combinations of lesser grades usually exceeding specifications for step 4.
5.5 Ambulatory without aid for about 100 meters; disability severe enough to preclude full daily activities; (Usual FS equivalents are one grade 5 alone, others 0 or 1; or combination of lesser grades usually exceeding those for step 4.0).
6.0 Intermittent or unilateral constant assistance (cane, crutch, brace) required to walk about 100 meters with or without resting; (Usual FS equivalents are combinations with more than two FS grade 3+)
6.5 Constant bilateral assistance (canes, crutches, braces) required to walk about 20 meters without resting; (Usual FS equivalents are combinations with more than two FS grade 3+).
7.0 Unable to walk beyond approximately 5 meters even with aid, essentially restricted to wheelchair; wheels self in standard wheelchair and transfers alone; up and about in wheelchair some 12 hours a day; (Usual FS equivalents are combinations with more than one FS grade 4+; very rarely pyramidal grade 5 alone)
7.5 Unable to take more than a few steps; restricted to wheelchair; may need aid in transfer; wheels self but cannot carry on in standard wheelchair a full day; May require motorized wheelchair; (Usual FS equivalents are combinations with more than one FS grade 4+)
8.0 Essentially restricted to bed or chair or perambulated in wheelchair, but may be out of bed itself much of the day; retains many self-care functions; generally has effective use of arms; (Usual FS equivalents are combinations, generally grade 4+ in several systems)
8.5 Essentially restricted to bed much of day; has some effective use of arm(s); retains some self-care functions; (Usual FS equivalents are combinations, generally 4+ in several systems)
9.0 Helpless bed patient; can communicate and eat; (Usual FS equivalents are combinations, mostly grade 4+)
9.5 Totally helpless bed patient; unable to communicate effectively or eat/swallow; (Usual FS equivalents are combinations, almost all grade 4+).
10.0 Death due to MS
Note
  • Excludes cerebral function grade 1.
  • Note 1: EDSS steps 1.0 to 4.5 refer to patients who are fully ambulatory and the precise step number is defined by the Functional System score(s). EDSS steps 5.0 to 9.5 are defined by the impairment to ambulation and usual equivalents in Functional Systems scores are provided.
  • Note 2: EDSS should not change by 1.0 step unless there is a change in the same direction of at least one step in at least one FS.

Functional systems (FS)

Functional system Abbr.
Pyramidal P
Cerebellar C11
Brainstem BS
Sensory S
Bowel and Bladder BB
Visual V
Cerebral or Mental Cb
Other O

FS Scoring

The FS are scored on a scale of 0 (low level of problems) to 5 or 6 (high level of problems) to best reflect the level of disability observed clinically. The “Other” category consists of any other neurologic findings attributed to MS and is dichotomous, with 0 as none and 1 as any present. In contrast, the total EDSS score is determined by two factors: gait and FS scores.

EDSS scores below 4.0 are determined by the FS scores alone. People with EDSS scores of 4.0 and above may have some degree of gait impairment. Scores between 4.0 and 9.5 are determined by both gait abilities and the FS scores. For simplicity, many experts gauge the EDSS scores between 4.0 and 9.5 entirely by gait, without considering the FS scores. The EDSS is widely used and accepted as a valid tool to clinically measure and evaluate MS patients’ level of functioning.

Symptoms

Motor

  • Spasticity
    • Spasticity in LE

Coordination

  • Ataxia
  • Cerebellar coordination symptoms
    • Nystagmus
    • Coordination
    • Balance
    • Ataxia
    • Intention tremor

Gait

  • Scissoring gait
  • Uneven steps
  • bladder-spastic/flaccid
  • Dysphagia
  • Dysphonia

Cognition

  • Cognition is diminished attention/concentration, fatigue

Emotion

  • Pseudobulbar affect - laughing/crying inappropriately

Vision

  • Optic neuritis
  • Abnormal Decreased pupillary light reflex (CN2)
    • Marcus gunn pupil: Light in eye → pupil will dilate (not restrict)

Sensation

  • Trigeminal neuralgia present
  • Numbness/paresthesia

Signs

  • Lhermitte’s Sign
    • Neck flexion → electric shock down the spine! Do not do this w/ exercises
  • Uhthoff’s Phenomenon
    • Excessive heat → temporary worsening of symptoms
    • Can potentially pre cool them before exercise
    • Keep under 85° in water if they go in
  • Charcot’s Triad
    • When cerebellum affected: SIN:
      • S - Scanning speech
      • I - Intention tremor
      • N - Nystagmus
  • CNII Optic n. dysfunction
    • Abnormal pupillary light reflex – dilates and does not restrict w light
    • Marcus gunn pupil, blindness

Clinical Presentation

Onset

  • 3% to 5% Occur before 16years
  • Onset of MS typically occurs between ages 20 and 50 years and is uncommon in children
  • 9% of patients experience sx onset after 50 yeras old

Gender

  • Prevalence: MS occurs more often in women than men (2-3:1) when sx onset before 50y/o
  • Severity: Men experience more progressive disease course and more rapid disability

Motor Control

  • Facilitation
    • Bilateral impairments, more stability

Interventions

Fitness prescription

Exercise guidelines for MS consistently recommendedL

Aerobic training

  • 2 to 3 days/week
  • 10-30 minutes/session at moderate-intensity

Resistance training

  • 2-3 days/week
  • 1-3 sets of 8-15 repetitions

Start w 2 days a week of each then progress to 3

The intensity should be moderate and range between 11 and 13 on the 20-point rating of perceived exertion scale, or between 40% and 60% peak oxygen consumption or peak heart rate.

References

1.
Fulk GD, Chui KK, eds. O’Sullivan and Schmitz’s Physical Rehabilitation. 8th ed. F. A. Davis Company; 2024.
2.
Goodman C, Fuller K. Goodman and Fuller’s Pathology. 5th ed. Elsevier, Inc; 2020.
3.
Kryger MH, Roth T, Goldstein CA. Kryger’s Principles and Practice of Sleep Medicine - E-book. Elsevier Health Sciences; 2021. https://books.google.com/books?id=yDVVEAAAQBAJ
4.
O’Sullivan SB, Schmitz TJ, Fulk GD, eds. Physical Rehabilitation. 7th ed. F.A. Davis Company; 2019.

Citation

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