Guillain-Barre Syndrome, MS, ALS Flashcards

1
Q

What is Guillain-Barre Syndrome

A
  • Autoimmune disorder of the peripheral nervous system causing progressive weakness with diminished/absent tendon reflexes
  • Acute, inflammatory, demyelinating, polyradiculoneuropathy presenting as rapid loss of myelin.
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2
Q

Etiology of GBS

A

Unknown
Associated with an autoimmune attack - occurs after recovery from an infectious illness.
can recover but can also kill you

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3
Q

What is pathophys of GBS

A

Inflammatory process affects Schwann cells

Macrophages attach Schwann cells — primary demyelination of the axon but leaves axon intact usually — affects sensory, motor, and autonomic systems.

remyelination occurs rapidly

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4
Q

Patients might experience secondary …

A

Axon al damage due to lymphocytic infiltration and cyto-destructive events in the axon causing wallerian denervation of the distal axon.

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5
Q

Signs and symptoms of GBS

A
  1. Acute demyelination of both cranial and peripheral nerves/nerve roots
  2. Sensory loss, paresthesias (tingling, burning), pain. Sensory loss usually not as significant as motor loss.
  3. Motor paresis or paralysis relative symmetrical distribution of weakness, may produce full quadriplegia with respiratory failure if not treated which is how most people die. motor loss is ascending in nature
  4. Dysarthria, dysphasia, diplopia, and facial weakness may develop in more severe cases.
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6
Q

Describe GBS progression

A
  • Progression of symptoms usually over a few days or weeks, usually ascending symptoms.
  • Recovery is slow — months to 2 years but most regain function
  • 10-20% have severe disability.
  • 5% die
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7
Q

Describe management for GBS

A
  1. Medical management - plasmapheresis, IVG
  2. Interventions in the acute phase include: respiratory care, passive movements, positioning, splinting, gentle progressive strengthening exercises.
    *in acute phase, more intensive strengthening and functional activities.
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8
Q

What are some features associated with poorer outcome?

A
  1. Requirement for respiratory support
  2. Abnormal peripheral nerve function - before or months after GBS
  3. No plasmapheresis or intravenous immune globulin performed
  4. Subgroup of GBS with primary atonal degeneration
  5. Patients with rapid onset
  6. Progression to quadriplegia
  7. Respiratory dependence
  8. Severe disease at presentation
  9. Patients showing no improvement at 3 weeks of plateau of disease.
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9
Q

What is one big thing we need to be aware of with treatment of GBS

A
  1. Fatigue like really severe fatigue. (Defined as a score of 5+ on the fatigue severity scale.
  2. Central fatigue in patients with GBS related to the decreased number of remaining motor units.
  3. Management of excessive fatigue includes use of energy conservation techniques, lifestyle changes, pacing, regular periods of naps during the day and improvement of sleep
  4. Exercise programs to reduce fatigue.
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10
Q

How would we want to schedule GBS in inpatient rehab

A

1.5 hours 2x/day — less fatigue this way getting up and going.

Once they improve and don’t have as much fatigue then you can do 3 30 minutes.

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11
Q

Treatment for sensory loss

A

Education of the patient and preventative program — positioning and moving off pressure points.

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12
Q

Treatment for autonomic dysfunction and pain

A

Symptoms include decreased sweating, OH, gastro paresis, constipation, vomiting diarrhea, impotence, and flaccid urinary bladder.

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13
Q

Treatment for neurogenic bladder and bowel

A

Pelvic floor training.

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14
Q

Treatment for physical deconditioning

A

Avoidance of overwork
because of transmission problems — fewer numbers of successfully recruited muscle fibers available to produce significant forces for function — increase force production in a muscle.

  • There are newly re-myelinated nerves and less motor units so really don’t want to overwork them!!
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15
Q

How might excess muscle activity happen in people with GBS

A
  1. Spotty/patchy demyelination
  2. Abnormal remyelination
  3. Partial denervation
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16
Q

What are overworking symptoms

A
  1. DOMS — peaking between day 1 and 5 post activity
  2. Reduction in maximum force production that gradually recovers.
    all caused by an inflammatory process resulting in a secondary muscle injury
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17
Q

What else should you avoid with treatment of muscles in GBS

A
  • Avoidance of eccentric contractions
    — probability of injury is greatest for exercise with eccentric contractions compared with isometric and concentric contractions
    — Eccentric contractions are necessary for daily activities. Eccentrically train to resist muscle damage.
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18
Q

When can you progress to antigravity movement?

A

Wait until patients can move the weight of their limb against gravity, then external stress can be applied.

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19
Q

When can you start to practice gait with GBS patients

A

Until patient has at least a fair grade in the lower extremity musculature — have to be able to move the weight of their own leg against gravity.

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20
Q

Assessment of overall weakness

A
  • Rest intervals, objective testing of strengths and evaluating complaints of muscle soreness are required.
  • PT should at minimum assess the patient’s strength and muscle soreness complaints from one day to the next or evaluate the patient after a weekend of no therapy.
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21
Q

Submax strength training

A

Minimal number of repetitions should be performed.

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22
Q

Aerobic training

A

Carefully monitor for appropriate physiological responses.
- Patients may experience autonomic involvement
- Work the patient to a moderate exercise intensity level using the borg scale of perceived exertion
- Frequent rest periods with gradual increases in continuous exercise time.

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23
Q

Fast twitch fibers

A

Recruit/train fast twitch muscle fibers
EX: push for speed or rapid rises and falls in muscle force production such as fast walking, jumping, and quick changes in direction during walking and lunges.
not doing this week 2 — more of an outpatient thing

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24
Q

Multi-joint work

A

PT should encourage patients to move in a multi joint pattern from the beginning of the disease process.
- By linking multiple joints together for a movement, the patient reinforces muscle synergies where prime movers and stabilizers are active and coordination between joints with the patient’s new neuro system will be encouraged to develop from the onset.

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25
Q

Functional activity practice

A

Practice eccentric contractions with functional activities

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26
Q

Respiratory function in GBS

A

Concern for aspiration, weakness of muscles of inspiration, weakness of muscles expiration, pulmonary complications (embolism and pneumonia)

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27
Q

What is ALS

A

Rapidly progressive neuro degenerative disease of middle life that is rarely seen prior to age 40.

Disease of UMN and LMNs — loss of anterior horn cells in SC and the motor cranial nuclei in brainstem (LMN). Demyelination and gliosis of CST and CBT in motor cortex (UMN)

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28
Q

Major signs of ALS

A

LMN - muscle weakness, atrophy, fasciculations, hyporeflexia, muscle cramps
UMN - spasticity, hyperreflexia, pathological reflexes
Bulbar signs - nocturnal respiratory difficulty, exertional dyspnea, accessory muscle use, paradoxical breathing (usually what kills them)
Others - fatigue, weight loss, cachexia, tendon shortening, contractures, frozen shoulder

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29
Q

What is the most frequent initial symptom

A

Focal weakness beginning in the leg, arm, or bulbar muscles

No sensory symptoms initially

Cognition, extraocular eye movements, and autonomic, bowel, bladder, and sexual functions usually remain intact initially.

30
Q

What is prognosis for ALS

A

Death usually results from respiratory failure with 50% of patients surviving 3-4 years after the onset of symptoms unless mechanical ventilation is used — most die within 5 years.

31
Q

Medications for ALS

A

Riluzole - expensive and adds 2-3 months to life. Affects breathing function

Radicava - unknown mechanism, maybe anti-oxidant. Slowed decline in function

32
Q

Exam/eval for ALS

A
  1. MMT/dynamometry — be sensitive to the patient and understand very focal muscle loss.
  2. Coordination
  3. Tone
  4. Balance — nothing specifically validated. TUG, Tinetti - may be determinant by advancement of disease
  5. Fatigue
  6. Psychosocial
  7. ALSFRS — self grading ADLs
  8. ALSAG-40 and ALSAQ-5 — well being
33
Q

What should you consider during the exam

A

Multi-system nature of the disease
— Autonomic (BP, sweating, HR)
— Sensory function (general sensory at first but then more focal involvement)
— Screen for cognitive impairments (represents poorer prognosis)
— Screen for extrapyramidal signs

34
Q

Symptoms that ALS patients report

A
  • Fatigue
  • Muscle stiffness
  • Muscle cramps
  • SOB
  • Sleep, pain, anxiety, depression, increased saliva, constipation
  • Falls increasingly common with advancing disease.
35
Q

How do you treat the symptoms

A
  1. Fatigue — teach energy conservation
  2. Muscle stiffness and cramps — stretching
  3. Pain — depending on source of pain: cramps = massage, stretch, skin pressure = position, MSK = P/AAROM
  4. Falls — AD, orthotics, balance training but this one is guided by stage of disease
36
Q

Weakness in ALS and strengthening

A
  1. Pattern of denervation and reinnervation in ALS — so strengthening good in early disease to restore some function.
  2. Highly repetitive or heavy resistance exercise can cause prolonged loss of strength in weakened or enervated muscles.
  3. Excessive exercise may also impair recovery of degenerated muscle fibers.
37
Q

Overal considerations for Exercise for people with ALS

A
  1. ROM and stretching are accepted forms of exerics
  2. Resistance of unaffected muscles and maybe muscles >3/5 using low to moderate load and intensity and aerobic exercise (swimming, walking, stationary bike) AT SUBMAX LEVELS (50-65% HRR) may be safe and effective
  3. Aerobic and resistance exercise more appropriate for early-middle stages and in those with slowly progressing disease.
  4. Monitoring exercise load and tolerance is critical
    — need to avoid excessive fatigue and overwork damage.
    — educate patients to not carry out activities to extreme fatigue. Not a no pain no gain situation.
38
Q

Pulmonary muscle training

A

Inspiratory muscle training was shown to prolong respiratory muscle strength with strong effect size.
Mean length of survival increased by 12 months.

Lung volume recruitment training strongly enhanced immediate cough efficacy with improved FVC

39
Q

What are safety considerations for overuse in ALS

A
  • Inability to perform daily activities following exercise due to exhaustion or pain
  • Reduction in maximal muscle force that gradually recovers.
  • Increased or excessive cramping, soreness, fatigue, or fasciculations.
  • Keep an exercise log (to log how they feel after exercise and stuff)
  • Stop if overuse signs occur — PT needs to re-assess
40
Q

Stage 1 of ALS

A

(Early)
- Independent in ADLs
- Ambulatory but clumsy (esp. with focal LE involvement)
- Mild focal weakness, asymmetrical distribution, hand cramping
what does PT do
— mostly doing education, energy conservation, home/workplace modification, and psych support, health promo
— Continue normal activities.
— Resistive strengthening for unaffected muslces — submax levels of activity otherwise.

41
Q

Stage 2 of ALS

A
  • Moderate weakness, still selective.
  • Slightly decreased independence maybe help with climbing stairs, raising arms, buttoning shirt
  • May start to see muscle wasting.
    what can PT do
    — Time to assess need for adaptive equipment, ADs, watch for falling;
    — Stretching and AROM exercise, strengthening of unaffected muscles
    — Aerobic activities and PROM by caregivers/family of affected joints to minimize contracture
42
Q

Exercise considerations for stage 2

A
  • Prevention of overuse fatigue and disuse atrophy
  • Brief exercise several times throughout day with good time for rest
    (30-45 minutes of total exercise divided into 2-3 sessions
  • Resistance, aerobic, active exercise
    active/resistance exercise may = repetitive performance of functional activities
43
Q

Stage 3 ALS

A

(Middle)
- Ambulatory but with severe weakness in certain muscle groups — distal ankles and hand.
- Mild-moderate limitation in function
- GOAL IS TO KEEP THEM PHYSICALLY INDEPENDENT.
what can PT do
— adaptive equipment, WC for longer distances, possible head control difficulty with soft colar, lightweight canes or RW only
— Begin respiratory therapy and deep breathing exercises

44
Q

Stage 4 ALS

A

(Middle)
- Severe weakness overall but with less weakness in UE.
- WC use, may be able to perform some ADLs but likely seeing lots of muscle wasting in LEs
- what can PT do
— PROM and AAROM exercises to prevent contractures
— Strengthening of any uninvolved muscles
— Education for skin inspection as a result of loss of functional mobility
— Sleep systems for pressure relief are indicated.

45
Q

Stage 5 ALS

A

(Late)
- Increasing progressive weakness throughout
- Deteriorating mobility and endurance
- Constant WC use. Transfers require significant physical assist.
- Decreased bed mobility, lots of help needed to prevent ulcers
- might have pain in immobilized joints. Pain may also be due to spasticity and cramping
- Significant head control issues — head support needed in order to eat, see, breathe.

46
Q

What can PT do for stage 6 ALS

A
  • Patients are confined to bed and require Max A with ADLs so proper posture support is needed in bed plus constant repositioning.
  • Pain management
  • Patients have progressive respiratory distress so CardioPulm PT may be required to help with body positioning, postural drainage, coughing techniques, airway clearance to mobilize secretions.
    GOAL OF PT
    preserve vital functions through compensations
    prevent pressure sores and respiratory problems
47
Q

What is Multiple Sclerosis

A

Most common primary demyelination disease of CNS characterized by lesions in the white matter
Centrally - oligodendrocytes

demyelinating lesions of MS can occur almost anywhere in the brain and SC so TON of signs and symptoms making it hard to DX

48
Q

What are common sites and associated neurological disturbances in MS

A
  1. Optic nerve (optic neuritis, progressive loss of acuity, diplopia)
  2. Brainstem (diplopia = 3,4,6 ; Trigeminal neuralgia =5 ; hemi facial spasm = 7 ; vertigo, vomitting, nystagmus= 8)
  3. Cerebellum and spinocerebellar path (dysarthria, ataxia, abnormal stance and gait)
  4. Spinal Cord (UMN weakness, spasticity, clonus, babinski, sensory loss, Bowery and bladder dysfunction)
  5. Cerebral cortex (intellectual impairment, memory loss, emotional changes, depression)
49
Q

What are the temporal patters of MS

A
  1. Relapsing/remitting — most common
  2. Primary progressive
  3. Progressive-relapsing — least common
50
Q

Relapsing remitting MS

A

Most frequent exacerbations followed by less complete recovery than in benign form.
Stable for long periods of time but have attacks
Also have a secondary progressive feature where there are fewer remissions with disease progression so a more cumulative disability.

51
Q

Primary progressive MS

A

Insidious relatively late onset and streaky progression of symptoms and disability. More lesions in SC than brain

52
Q

Progressive-relapsing MS

A

Often diagnosed as primary first until there is a relapse.

53
Q

What are the major clinical subtypes of MS

A
  1. Relapsing-Remitting
  2. Primary progressive
  3. Secondary progressive
  4. Progressive relapsing
  5. Clinically isolated syndrome
54
Q

Relapsing Remitting is characterized by

A

specific attack of deficits in the relapse stage with either full or partial recover in the remitting stage. Periods between relapses are characterized by lack of disease progression.

55
Q

Primary progressive is characterized by

A

disease progression and a deterioration of function from onset.

56
Q

Secondary progressive is characterized by

A

an initial relapsing remitting stage followed by a change to a progressive course with steady decline in function and impairments increase with or without specific attacks

57
Q

Progressive Relapsing is characterized by

A

Steady deterioration in disease from onset with occasional attacks but time between attacks has continuing progression

58
Q

Clinically isolated syndrome is characterized by

A

First episode of inflammatory demyelination in the CNS that could become MS if additional activity occurs/progression to RRMS

59
Q

Risk factors for MS

A

Females 2-3x more likely than males for RRMS
Age - 20-30 for RRMS : 40-50 for PPMS

60
Q

Prognosis for MS (more favorable outcome)

A

More favorable outlook = acute onset, female, young age, high degree of remission, initially limited to optic neuritis, other sensory symptoms, initial involvement of only one region of nervous system

61
Q

Prognosis of MS (Less favorable outlook)

A

Less favorable outlook = >35 years at onset, early cerebellar, or lower brainstem symptoms, chronically progressive (remitting)

62
Q

Falls in MS

A
  • Progressive MS classification is a significant risk factor for falls. More likely than RRMS
  • Balance measures alone may not be enough to predict fall risk — use of mobility aid is a significant risk factor
  • Also consider spasticity, gait disturbances, continence, and fear.
63
Q

Fatigue in MS

A
  • Very common complaint in patients with MS
  • Separate entity as it relates to MS - different from normal fatigue.
  • No correlation between severity of fatigue and neurological involvement or severity of fatigue and depression
64
Q

Heat sensitivity in MS

A
  • 80% have heat sensitivity — changes in core body temp.
  • can come from internal sources like vigorous exercise or external sources like environmental temperature.
  • Uhthoff symptom/phenomenon — increase or presence of neurological symptoms in response to a heating condition.
  • Range of body temp could bring on these neuro symptoms
65
Q

QOL in MS

A

MSQOL-54 is a structured, self report questionnaire that the patient can generally complete with little to no assistance.

66
Q

What guides our interventions

A

Understanding of type of MS, location of the plaques, general stages of the disease process, impairments, and functional limitations present.

67
Q

Today’s view of exercise for MS

A

Regular exercise is essential to preservation of function in this population by minimizing effects of immobility. Exercise therapy can be prescribed in people with MS without harm.

Exercise may reduce self reported fatigue and does not seem to be associated with a significant risk of MS relapse.

Just have to monitor them like crazy.

68
Q

Aerobic training in people with MS

A

Issues - autonomic regulation problems usually more in advancing disease and cardiorespiratory response

It is likely that maximal aerobic capacity in persons with MS is partially influenced by the level of neuro impairment — those with greater impairment sustain exercise for a shorter period of time, achieve a lower maximal exercise intensity and a lower oxygen uptake.

69
Q

Precautions to take into consideration with people with MS

A
  1. Strengthening and aerobic training can be safely performed in MS population — those with minimal impairments have best exercise tolerance and get best benefits
  2. Probably submax exercise for stable persons with MS
  3. To prevent overheating consider water therapy in a cool pool, cooling suits, core body temps naturally change during the day, complaints of exercise.
70
Q

Medical management for MS

A

Immunosuppressant drugs — treat acute attacks and shorten episodes
Steroids
Interferon Drugs - slow disease progression
Symptomatic management — tone reducing and helps with bladder control

71
Q

PT goals for MS

A
  1. Monitor changes in disease progression, attacks/change in status
  2. Focused on restoring function — as disease progresses may need bouts of therapy to address new problems in function, maintain current level of function, address equipment needs
  3. Energy conservation — need to have an idea on level of fatigue and impact of fatigue throughout the day, pacing, optimal scheduling, consideration of weather, psychological support.