Immune-mediated Disorders Of The CNS Flashcards
What are some immune mediated disorders of the CNS?
Multiple sclerosis (MS)
Clinically isolated syndrome (CIS)
Neuromyelitis optica (NMO)
Acute disseminated encephalomyelitis (ADEM)
What is MS?
Progressive autoimmune disease characterized by inflammation, selective demyleination, and gliosis of the CNS
What is the most common chronic inflammatory condition of the CNS?
MS
What areas of the CNS does MS affect?
The brain, optic nerve, and SC
What are the cardinal symptoms of MS?
Intention tremor
Scanning speech
Nystagmus
T/f: vision loss is an early characteristic of development of MS
True
What is the primary cause of non-traumatic disability in young and middle aged adults?
MS
What age is usually affected by MS? (Age of dx)
20-50 yo
Average of 32 yo
Are more men or women affected by MS?
Women (3:1)
Is the prognosis for MS worse in men or women?
Men
T/f: genetics have a significant influence on MS
False
What ethnicity has the greatest incidence of MS?
Caucasians
What is an autoimmune mediated focal demyleination accompanied by variable axon damage/destruction and reactive gliosis?
MS
T/f: MS interferes with normal conduction of neural signals
True
Does MS affect gray or white matter?
Both
How is white matter affected in MS?
Inflammatory lesions w/immune system response (infiltrates) that result in reactive loss of axons and oligodendrocytes
How is gray matter affected in MS?
Demyelination and loss of neurons w/o immunologic and inflammatory infiltrates
What is the pathology process in MS?
Neuronal damage activates astrogliosis which produces glial scarring or the sclerosis called plaques
T/f: active disease process is followed by periods of decreased acute inflammation or remission in most cases of MS
True
What determines the severity of MS?
The degree of axonal loss is associated with the severity of the inflammation
What is recovery in MS?
Axonal remyelination that occurs variably
T/f: The degree of fxnal recovery is related to the degree of axonal remyelination during remission periods of MS
True
T/f: during later stages of MS there is less neural recovery (less remyelination) in remission periods
True
Cumulative sensory, motor, and cognitive disability in MS results from the extent of ____ matter pathology
Gray
T/f: there is both acute and degenerative lesions of varying size scattered throughout the CNS in MS
True
T/f : MS may result in spasticity, hemiplegia, B/B symptoms, cognitive symptoms, or visual issues depending where it is
True
What are the 3 clinical courses/ phenotypes of MS?
Relapsing remitting MS (RRMS)
Secondary progressive MS (SPMS)
Primary progressive MS (PPMS)
Why is there so much variability even within subtypes?
Bc of differences in exercise levels, body composition, social support, age, meds, and temperature of the area
What are the risk factors for rapid progression of MS?
Older age
Male
Multifocal initial symptoms (particularly cerebellar, motor, sphincter)
Short duration by attacks 1 and 2
Frequent attacks within first 5 years
What do we need to be aware of in MS disease course?
Changing s/s and severity
What is the most common phenotype of MS?
RRMS
What percent of person with MS have RRMS?
85%
RRMS will eventually turn into what other phenotype of MS?
SPMS
What percent of person with MS have PPMS?
10-15%
What percent of cases of MS are benign MS (CIS)?
25%
What is clinically isolated syndrome (CIS)?
A first episode of inflammatory demyelination that lasts at least 24 hours and resolves completely
When CIS has no clinical relapses or new MRI activity, is it active or not active?
Not active
If CIS has clinical relapses with MRI activity is it active or not active
Active and it is now RRMS
What are possible symptoms we may see in patients with MS?
Fatigue (biggest one)
Sensory sx
Imbalance, tripping
Speech/swallowing issues
Variable pain
Visual symptoms
Affective sx
Motor symptoms
B/B sx
Sexual sx
T/f: MS is often misdiagnosed
True
A dx of MS requires a careful medical hx and ___ key features on MRI imaging
2
What are often the first reported signs of MS?
Loss/reduction of vision in one eye (painful eye movt or double vision)
Ascending sensory disturbance and or loss distal to proximal
Progressive difficulties with gait and balance
T/f: patients with MS are often over 50 yo
False they are usually younger than 50
T/f: pts with MS may have a hx of previous neuro symptoms
True
Pts with MS have symptoms that have evolved over more than _______
24 hours
T/f: most pts with MS will have symptoms that persist over several days or weeks and then improve
True
T/f: pts with MS often have a fever and infection present
False
What does an MRI of the brain and SC of a pt with MS show?
The presence of plaques as a sign of neurodamage
What is a highly specific blood test used to test for MS?
IgG test
What do CSF studies show us in pts with MS?
Specific antibodies associated with MS (oligocional bands)
Intrathecal beta lymphocyte activation (hallmark of MS)
Activation of what cells is a hallmark of MS?
Intrathecal beta lymphocytes
How can we evaluate fatigue?
Six minute walk test and RPE
What are we looking at in a 6MWT for fatigue in MS?
What the first three minutes look like compared to the last three minutes
In the initial stages of MS, what are the goals of disease modifying therapies (DMT)?
Reduce inflammation
Reduce immune system infiltrates
T/f: early medical management of MS is shown to be effective in reducing the # of attacks
True
Are DMTs more effective in the early or later stages of MS?
Early stages
T/f: DMTs act on various components of the immune system and modify the course of the disease for MS
True
T/f: all DMTs have non-serious side effects
True
When are corticosteroids used in MS?
During an acute flare up
_______ are used for the management of symptoms like spasticity, pain, and fatigue
Medications
What is the test that helps group pts for more disease specific interventions?
The expanded disability status scale (EDSS)
What are the 7 domains included in the EDSS?
Pyramidal
Cerebellar
BS
B/B
Sensory
Visual
Mental
What are the pyramidal s/s in the EDSS?
Weakness, difficulty moving limbs
What are the cerebellar s/s included in the EDSS
Ataxia, decreased coordination
What are the BS s/s included in the EDSS?
Speech, swallowing, and nystagmus issues
What scores on the EDSS are fully ambulatory?
0-4.5
What score on the EDSS indicates that a patient is starting to need assistance for walking?
6
What EDSS scores require a WC for all but a few steps?
7-8.5
An EDSS score of above what indicates that the patient is bed bound?
9
What is an exacerbation in MS?
New and recurrent MS symptoms lasting more than 24 hours
What are some causes of MS exacerbations?
Deteriorating health, infections, diseases of major organ systems, stress
What are pseudoexacerbations of MS?
Symptoms lasting less than 24 hours
What are the causes of MS pseudoexacerbations?
Heat intolerance (Uthoff’s symptom), increase in external temp (hot day), or internal increase in temp (fever)
T/f: pseudoexacerbations of MS resolve within 24 hours of the body cooling down
True
What mental fxns are affected in MS?
Cognition and memory
What sensory fxns are affected in MS?
Integrity, integration, superficial, cortical sensation integration, and visuospatial skills
What are the voice and speech fxns affected in MS?
Coordination or speech, respiration, and communication
What fxns of the CV, hemotologic, immunologic, and respiratory fxns are affected in MS?
Aerobic capacity, BP, HR, RR, O2, RPE, response to exercise
What genitourinary and reproductive fxns are affected in MS?
B/B fxns and continence
What neuromusculoskeletal and movt related fxns are affected by MS?
Balance, assistive technology, skeletal integrity, fatigue, reflex integrity, strength, power, motor control, posture, gait, and locomotion
What skin and integumentary fxns are affected by MS?
Skin integrity and pressure sensitive areas
What are the clinical manifestations of MS?
Sensory impairments
Pain
Visual dysfunction
Motor system impairments (weakness/spasticity)
Coordination and balance
Gait and mobility impairments
Speech and swallowing impairments
B/B impairments
Sexual dysfunction
Cognitive impairments
Depression
What are the ways to examine fatigue specific to MS?
The modified fatigue impact scale (MFIS)
The fatigue severity scale (FSS)
What exam is a comprehensive measure of fatigue?
Modified fatigue impact scale (MFIS)
What exam is used as a screening tool for fatigue?
Fatigue severity scale (FSS)
T/f: fatigue should be expected in neurological disease
True
Physicians who work with pts with neuro disease need to do what in terms of fatigue management for patients?
Test for presence of fatigue
Determine type of fatigue
Determine cause of the fatigue
Determine its effect upon physical fxning
Develop intervention plans to mitigate/remediate its effects
What is primary fatigue?
A physiological change resulting in fatigue or fatiguability that’s due to the disease process itself
The typical and expected sequence of the disease
Which type of fatigue is reversible only by treating the primary physiologic process?
Primary fatigue
What is secondary fatigue?
A result of an adaptation or compensation to the disease pathophysiology
Fatigue that occurs as a result of the disease not due to the disease itself
What type of fatigue is reversible if the compensations or adaptations are prevented or limited?
Secondary fatigue
What type of fatigue is fatigue caused by walking secondary to weakness?
Secondary fatigue
What are objective measures of fatigue?
Fatigue visual analog scale
Pt reported outcome measures (MFIS and FSS)
Is a higher score on the MFIS and FSS better or worse?
Worse
What does the FSS tell us?
How fatigue interferes with certain activities
What are the endurance tests for MS ?
6MWT
2 min step test
What are the signs of fatiguability?
Progressive slowing of gait
Progressive weakness of repeated contractions
Worsening of sensation/speech/vision during a repetitive task
Decreased performance on a fxnal measure following exertion
Physiologic measures (temp, EMG, cardiovascular changes)
Pt/ caregiver complaint/identification
What are the disease specific measures for MS?
MSQOL54
MSQLI
MSIS29 (physical and psychological impact of MS)
What are the acute care recommendations for evaluating MS?
12 item MS walking scale
9 hole peg test
Berg balance scale
MS impact scale (MSIS-29)
Timed 25 ft walk
TUG with cognitive and manual
What are the inpatient/outpatient recommendations for evaluation of MS?
12 item MS walking scale
6MWT
9 hole peg test
Berg balance scale
Dizziness handicap inventory (outpatient only)
MS fxnal composite
MS impact scale (MSIS-29)
MS Quality of life (MSQOL-54)
Timed 25 ft walk
TUG w/cognitive and manual
What does multidisciplinary programming for rehab in MS consist of?
Cognitive behavioral therapy for depression
Education
Strengthening
QOL
Aerobic training
Fatigue management
What is involved in rehab of MS?
Multidisciplinary programming
Restorative interventions
Preventative interventions
Compensatory interventions
Maintenance therapy
What are common impairments of MS in the early stages?
Few impairments, independence maintained
Motor symptoms present but no ADL restrictions
Sx for RRMS are variable and don’t progress to PPMS
What are some intervention strategies for early to mild stage MS?
Regular exercise to improve/maintain body systems
Community class to improve/maintain socialization and QOL
education about the disease
Determine the need for adaptations/modifications
Psych support to pt and fam
Referral to other health providers
What are common impairments and activity limitations in middle/ moderate MS?
Progressive course, increased severity and impairments
Minimal to moderate activity restrictions
ADL assistance
Difficulty with gait and balance
What are intervention strategies for middle/moderate MS?
Regular exercise to improve/maintain body systems
Community classes to improve/maintain socialization and QOL
AD to maintain fxn
Motorized WC for community
Pt/fam/caregiver education and training
Psych support
Referral to other health providers
What are the common impairments and activity limitations in late/advanced MS?
Progressive course, numerous impairments and severe
Severe activity restrictions
Assistance for all ADLs
WC or bed most of the day
Not able to live alone (typically assisted facility)
Prominent cognitive problems
What are intervention strategies for late/advanced MS?
Maximize upright posture and time out of bed
Maximize ADL participation
Prevent contractures, pressure wounds, and pneumonia
Use pressure relieving devices
Use of a hospital bed, WC, mechanical lift
Pt/fam/caregiver education and training (transfers, positioning, skincare)
Psych support
Referral to other health providers
What are unfavorable indicators for prognosis in MS?
Male sex
Onset of symptoms
Initial symptoms involve the cerebellum, mental fxn, or urinary control
Initial symptoms involve multiple regions of the body
Frequent relapses in the first year of onset or short time by the first 2 relapses
Incomplete remission
Rapid progression to disability
What are favorable indicators for prognosis of MS?
Female sex
Onset <40 yo
Initial symptoms are sensory only
Involvement of only 1 CNS system at time of onset
Full recovery bw relapses
Absence/late onset of cerebellar symptoms
What are the general goals for pts with progressive CNS disorders?
Impact of pathology is reduced
Impact of impairments is reduced
Ability to perform physical actions, tasks, or activities is improved
Disability associated with chronic illness is reduced
Health status and QOL are improved
Pt satisfaction is improved
What are some contraindications/precautions for MS?
Avoiding excessive rise in body temp
Postpone treatment and notify physician if s/s suggest attack/relapse
Monitor for DVT
What is Unthoff’s phenomenon?
Excessive heat causing an exacerbation of symptoms in MS
T/f: time of day and environment should be considered with MS treatment to prevent overheating
True
What is involved in PT interventions for MS?
Management of sensory deficits
Management of bladder control
Exercise training
Strength and conditioning
Management of spasticity
Flexibility exercises
Locomotor training
Balance and coordination training
Orthotic and AD management
Fxnal training
Speech and swallowing
Cognitive training
Psychosocial management
Pt and caregiver education
What is involved in management of sensory deficits in MS?
Awareness, compensation, promotion of safety (prevention)
Proprioception
Visual dysfunction
Sensory ataxia
Skin (keep clean and dry and pressure relief)
What is involved in management of bladder control?
Voiding schedule (compensatory)
Pelvic floor PT (restorative)
T/f: exercise is safe and won’t increase the risk for relapse in MS
True
What is involved in exercise training in MS?
Avoiding sedentary lifestyle
Careful attention to the response to exercise
Strength and conditioning
Should we exercise during an active relapse in RRMS?
NOOOO
Why should we exercise in PPMS?
To prevent deterioration
What is involved in strength and conditioning for MS?
Individualized, scheduled exercise on non-endurance days during optimal times of temp regulation
What activities fall under strength and conditioning for MS?
Circuit training
What should we do during strength and conditioning for MS?
Rest periods
Avoid overworking
Monitor fatigue
Focus on CKC activities
Should we focus on open or closed chain activities in strength and conditioning for MS?
Closed chain activities
What are the exercise recommendations for EDSS 0-4.5?
Aerobic, advanced aerobic, resistance, and neuromotor training
What is the aerobic training recommendation for EDSS 0-4.5 and 5-6?
2-3x/wk
40-60% HRmax
11-13 RPE
10-60 min
What are the advanced aerobic recommendations for EDSS 0-4.5 and 5-6?
5x/wk up to 40 minutes
80%HRmax
RPE 15/20
What are the resistance training recommendations for MS EDSS 0-4.5 and 5-6?
2-3x/wk
1-3 sets
8-15 reps
What are the recommendations for neuromotor training in MS EDSS 0-4.5 and 5-6?
3-6x/wk
20-60 minutes
Postural stability, fall prevention, and coordination
What is the purpose of adaptive exercise in MS?
To limit fall risk
When does caregiver education become a priority in MS treatment?
EDSS 7-7.5
What are the exercise parameters for EDSS 7-7.5?
20 min/day
3-7 days/wk
Can be accumulated through shorter sessions and gradual progression
What are the parameters for breathing exercises in EDSs 7-7.5?
Every other day
3x10 with a resistive breathing apparatus
What are the exercise recommendations for EDSS 7-7.5?
Breathing exercises
UE training
LE training
Core exercises
What are the parameters for UE training for EDSS 7-7.5?
6 3min intervals at 70% HRtarget
Active ROM with resistance arm cycling
3x/wk
3x10 weight and resistance band
What are the parameters for LE training in EDSS 7-7.5?
Overground walking with walker as available
3x10 STS
3-5x/wk for 30 min power assist cycling
30 min standing
2-5x/wk BWS treadmill
What are the parameters for core exercises for EDSS 7-7.5?
2x/wk, 4-5 seated isometric abdominal muscle strengthening, 10-15s holds
3-5 min/day, moving or stationary seated balance, unsupported or supported
What are the exercise recommendations for EDSS 8-8.5?
UE training
LE training
Core exercises
What are the parameters for UE training in EDSS 8-8.5?
6 3min intervals at a target HR (or 70% effort)
AROM w/resistance arm cycling
3x/wk, 3x10 weight and resistance band
What are the parameters for LE training in EDSS 8-8.5?
2-3x/wk, 1-2 min standing w/assistance; 3x/wk, 30 min standing w/assistance
What are the parameters for core training in EDSS 8-8.5?
2x/wk, 4-5 seated isometric abdominal muscle strengthening, 5-6s holds
1-2 min/day, moving or stationary seated balance, unsupported or supported
What are the exercise parameters and recommendations for EDSS 9?
Up to 10min 3-7days/wk as tolerated
Daily PROM of all jts
AROM as noted
FES for ROM to maintain muscle mass and circulation
Despite normal linear response in other vitals, ____ and ____ may be blunted if dysautonomia is present
HR, BP
What is the preferred method for improved aerobic conditioning in MS?
Upright or recumbent bicycle
T/f: most individuals with MS cannot achieve 70-85% HRmax
False, most can
What should we monitor for during exercise with patient with MS?
Temp, fatigue, overwork
What are the FITT principle parameters for aerobic training in MS?
F: 3-5 days/wk
I: 60-85% HRmax
T: 30 min/session or 3 10 min sessions
T: cycling, walking, swimming, water aerobics, circuit training
What needs to be considered with water aerobics in MS?
Temp of the environment
What can be included in locomotor training for MS?
BWS
Robotic assistance
Overground
What can be involved in balance and coordination training in MS?
Seated, standing, or dynamic
COG, BOS training Task and fxn specificity
Biofeedback for sensory deficits
Vestibular interventions
T/f: rhythmic auditory stimulation is effective in improving stride length, stride time, cadence, and gait speed in MS
True
What is involved in management of fatigue?
Aerobic training
Cognitive behavioral therapy
Energy conservation techniques
Is aerobic training restorative or compensatory?
Restorative
What is involved in energy conservation in MS?
Analysis of the causes of individual fatigue
Education about fatigue
Prioritization of activities
Activity pacing
Ergonomic analysis and recommendations
Why are KAFOs rarely used with MS?
Bc they tend to be more cumbersome and can cause fatigue
What is the overall focus of pt/caregiver education?
Maintaining hope, encouragement, and realism of disease course
Prevention of complications
Monitoring effects of fatigue, exercise, and therosensitivity
Use of AD
ongoing wellness and routine checkups
What is neuromyelitis optica (NMO)?
A relapsing and remitting autoimmune disorder w/o progression
Does NMO occur more in men or women?
Women
What ages are mostly affected by NMO?
40-50 yo
Immune cells and antibodies primarily attack what structures in NMO?
Optic nerves, SC, and sometimes the brain
What is the result of the immune cells and antibodies attacking the optic nerve in NMO?
Swelling and inflammation that cause pain and loss of vision
What is the result of the immune cells and antibodies attacking the SC in NMO?
Weakness or paralysis in the legs or arms
Loss of sensation
Problems with B/B fxn
T/f: symptoms are generally more severe after an NMO attack than an MS attack
True
Disability in NMO results from _____ _____
Repeated attacks
What makes NMO different from MS?
Symptoms are more severe in NMO attacks
Episodes of NMO are usually more severe
NMO rarely has a secondary progressive stage
Present across the world
Fatigue is typically secondary in NMO while it is usually primary in MS
NMO is often associated with other autoimmune diseases
What other autoimmune diseases is NMO usually associated with?
Sjögren’s syndrome or systemic lupus erythematosus (SLE)
What is acute disseminated encephalomyelopathy?
Multifocal inflammatory demyelination disorder of the CNS
acute/subacute onset of Multifocal neurological deficits with HA and impaired consciousness
What population is mostly effected by ADEM?
Children
What is the disease course in ADEM?
Subacute progression to a peak then plateau
T/f: if an individual survives ADEM, it spontaneously improves
True
What is transverse myelitis?
An autoimmune response to viral infection resulting in inflammation and demyelination of a segment of the SC
T/f: transverse myelitis can be associated with MS, NMO, or ADEM
true
What is the presentation of someone with transverse myelitis?
Rapid onset of weakness
Possible significant spasticity
Sensory loss is typically spinothalamic but can involve the DCML
What part of the spine is typically involved in transverse myelitis?
The thoracic spine
T/f: B/B dysfunction is associated with transverse myelitis
True
What is involved in the medical management for transverse myelitis?
Methylprednisone (high dose corticosteroids)
Plasmaphoresis
T/f: medical management of transverse myelitis is subtype dependent
True
