Disorders of the Neurological Junction (MG) Flashcards

1
Q

Precautions during MG rehab:

A
  1. No exercise during exarcebations
  2. Stop exercise if symptoms worsen or SOB
  3. Fatigie should not last for more than 2 h post exercise
  4. Resudual muscle soreness should not be severe and resolve within a few days
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2
Q

Lambert-Eaton Syndrome AKA:

A

Lambert-Eaton Myasthenic Syndrome (LEMS)

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3
Q

acute care rehabilitation considerations of MG:

A
  • Acute care: supportive care
    • PT stablishes accurate neurologic and respiratory baselines
    • Be aware of myesthenic crisis:
      • Increased wekaness
      • respiratory distress
      • dificulty talking, chewing, swallowing
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4
Q

Treatment of Botulism:

A
  • Immediate
  • Antitoxin injection
  • Removal of unabsorbed toxin from GI
  • ICU: intubation and mechanical ventalitaion may be needed
  • Supportive care
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5
Q

In MG, an autoimmune response mediated by specific anti-ACh receptor antibodies that may:

A
  • Block the site that normally binds ACh
  • Damage postsynaptic muscle membrane
  • Result in degeneration of the ACh receptors
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6
Q

Recently discovered types of Myesthenia Gravis:

A

Antibodies against MuSK protein

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7
Q

Prognosis of MG:

A
  • Normal life with tretament
  • Variable course: remission and exarcerbations
  • Slowly progressive course
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8
Q

4 Older categories of MG:

A
  1. Ocular
  2. Mild generalized
  3. Acute fulminating
  4. Late severe
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9
Q

Rehabilitation Considerations of botulism:

A

Post-acute care:

  • Graded, progressive rehab program to address:
    • Muscle wasting
    • Deconditioning
    • Orthostatic hypotension
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10
Q

Generalized weakness in MG:

A
  • Occurs in 85% of persons with MG-Affects limb musculature
  • Usually more mild involvement
  • Proximal muscles more affected than distal
  • May see unstable, waddling gait-In more severe forms, can have respiratory muscle weakness
  • No sensory loss
  • Normal reflexes: because is a short contraction
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11
Q

Peak incidence of MG in females ages:

A

20’s – 30’s

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12
Q

Diagnosis of Lambert Eaton Syndrome:

A
  • Decreased DTR
  • Muscle atrophy
  • Weakness that improves with activity
  • Detection of voltage gated calcium channel
    antibodies in serum
  • Positive Tensilon test
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13
Q

Treatment of Lambert Eaton Syndrome:

A
  • Symptomatic: lung cancer treatment
  • Other treatment similar to MG
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14
Q

Clinical features of Lambert-Eaton Syndrome:

A
  1. Weakness and fatigue of muscles
  2. Muscle Atrophy
  3. Little involvement of ocular muscles
  4. Depressed reflexes
  5. ¾ have autonomic involvement:
    • Dry mouth
    • Constipation
    • Blurred vision
    • Impaired sweating
    • Orthostatic hypotension
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15
Q

True or false: reumatoid arthritis and lupus are risk factors of MG

A

True

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16
Q

Signs & symptoms of LEMS often precede what other serius condition?

A

Dx of cancer by 2 to 4 years

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17
Q

Cardinal feautures of MG:

A
  1. Skeletal muscle fluctuating weakness
  2. Skeletal Muscle fatigability
    • Extraocular muscles 1st to be affected
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18
Q

Autoimmune disorder associated mainly with carcinoma of lung and may also be associated wuth DM I, Vitiligo, hypothyroidism?

A

Lambert-Eaton Syndrome

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19
Q

Transient Neonatal Myasthenia Gravis

A
  • AChE antibodies can cross placenta
  • Occurs in 10% of infants born to mothers
    with MG
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20
Q

Most common autoummune disorder of the neuromuscular junction characterized by fluctuating weakness and fatigability of skeletal muscle:

A

Myasthenia Gravis

21
Q

What are the neuromuscular symptoms of botulism?

A
  • Flaccid paralysis of extremities
    • Symetrical
    • Proximal to distal
  • Cranial nerve palsies:
    • Blurred vision, diplopia, dysphagia, dysarthria
  • Respiratory muscle paralysis
  • Progression of symptoms depends on how much toxin
  • No sensory deficits
  • Normal mentation
22
Q

Categories of Botulism:

A
  1. Food – borne (15%): classic form. Canned foods-
  2. Wound (20%): chronic drug abusers, after cesarean section-
  3. Infant (65%): most common from honey
  4. Adult intestinal toxemia (rare)
  5. Iatrogenic (don’t know the cause) botulism (rare)
23
Q

Clostridium botulinum is an anaerobic bacterium that produces a potent neurotoxin and is found mainly where?

A

in improperly preserved or canned food

24
Q

Botulism prognosis:

A
  • Severe cases:
    • Fatal if untreated
    • Mortality rate 20%
  • Mild to moderate cases
    • Gradual recover of muscle strenght may take up to 12 months
25
Q

Rare, often fatal condition caused by a potent neurotoxin produced by Clostridium botulinum causing failure of the neuromuscular transmission:

A

Botulism

26
Q

Diagnosis of botulism:

A
  • History
  • Serum or stool analysis
  • Clinical signs: sudden paralysis
  • EMG
27
Q

Peak incidence of MG in males ages:

A

50’s – 60’s

28
Q

Treatment of MG:

A
  1. Symptomatic Tx
  2. AChE inhibitor medication
  3. Thymectomy: long term
  4. Immunosuppresants drugs: corticosteroids; SEs
  5. Plasmapheresis
  6. IVIg: short term benefits
29
Q

Risk Factors of Myasthenia Gravis:

A
  • Thymic disorders: enlarged TG
  • Thyroid related disorders: hyperthyroidism
  • Diabetes
  • Immune diseases: RA, lupus
  • Exacerbation with: emotional stress, surgery, shortly after pregnancy, exposure to bright sunlight
  • Familial association: 5% to 7%
30
Q

More recent research identified other
forms of MG:

A
  • Antibodies against MuSK protein (muscle specific kinase)
  • Lack of this protein results in improper formation
    of NM junction
  • Found in 30% - 40% of persons with Myasthenia
    gravis
31
Q

Circulating ingested neurotoxin from Clostridium botulinum binds to what structure in the body?

A

presynaptic nerve terminals inhibiting the release of ACh at motor end plate

32
Q

Rehab considerations once MG is stable:

A
  • Plan therapy with periods of max energy
  • Use maximal isometric contractions
  • Teach energy conservation principles:
    • Frequent rest
    • Home modifications
    • Avoid streneous exercise andexposure to sun
  • Minimal to moderate exer. intesities
    • Aerobic: walking, stat bike, UBE
    • Breathing Exercises
  • Cooling vetsm cold packs, cold showers may decrease muscle fatigue
33
Q

Causes of wound botulism:

A
  • Occurs with contamination ofwounds by soil
  • Chronic drug abusers
  • After cesarean section
  • Traumatic wounds
34
Q

Rehabilitation considerations of LEMS?

A
  • Exercise
  • Similar precautions as MG
  • Avoid high temperatures
35
Q

What is the incidence in children and adults of botulism?

A
  • 10 adult cases in US each year
  • 100 infants
36
Q

Which is the most common category of botulism? Why?

A
  • Infant botulism
  • Ingestion of honey
37
Q

Incidence of Myasthenia Gravis :

A
  • 1 : 200,000
  • >100,000 cases in US
  • Ratio female to male is 3:2
38
Q

true or false:
Absence of anti-ACh receptor antibodies in the serum rules out MG:

A

false

39
Q

Myasthenic Crisis:

A
  • Medical emergency
  • Often cause by infection
  • weakning of respiratory muscles
  • mechanical ventilation
40
Q

Which muscles are the first to be affected in MG?

A

Cranial muscles, especially extraocular mm

41
Q

General clinical picture of MG:

A

ALL bilateral:

  1. Ocular muscle involvement: diplopia, ptosis (dropping eyelids)
  2. Facial weakness: loss of facial expression, lips cannot close
  3. Oropharyngeal weakness: Impaired chewing, swallowing and speech
42
Q

How do patients with Lambert Eaton Syndrome differ from myastethenia gravis?

A

LEMS patients may gain strenght with repeated muscle activity.

ACh can build up in large enough amounts in
neuromuscular junction for strength to improve

43
Q

What are the initial symptoms of botulism?

When do they appear?

A

Initial symptoms of food poisoning

  • Malaise
  • Nausea
  • Vomiting
  • Diarrhea
  • Abdominal pain
  • Dryness of mouth

Symptoms usually appear within 12 to 36
hours after ingestion of contaminated food

  • Range: 6 hours – 10 days
44
Q

Autoimmune disorder in which a defect of neuromuscular transmission associated with blocked voltage gated calcium channel resulting in decreased release of ACh from the presynaptic nerve terminal:

A

Lambert-Eaton Syndrome

45
Q

Newer classification system of MGFA:

A

5 main classes of MG with several subclasses

46
Q

Medical diagnosis of MG:

A
  1. History
  2. Clinical observation of symptoms:
    • intact DTRs,
    • weakness improves with rest
    • No sensory loss
  3. Clinical test: Patient looks up for 2 – 3 minutes;
  4. Ice test: x 5min
  5. Immunologic testing:
    • Presence of anti-ACh receptor antibodies in the serum.
    • Absence of antibodies does not rule out MG. Presence of anti-MuSK protein antibodies.
  6. Pharmacologic testing: tensilon.
  7. EMG test
47
Q

Pathology of Myasthenia Gravis

A
  • Autoimmune response: anti ACh recpetor antibodies block or damage receptors.
  • Number of Ach receptors on the muscle cell is decreased
  • The remaining receptors are flattened
  • Without ACh, the nerve impulses fail to pass across the neuromuscular junction to stimulate muscle contraction
48
Q

Infant signs of botulism:

A
  • Constipation
  • Lethargy
  • Poor feeding
  • Weak cry
  • Failure to thrive
49
Q

Generalized version of MG:

A
  1. Occurs in 85% of pt with MG
  2. Affects limbs
  3. Proximal muscles more affected than distal
  4. Unastable, waddling gait
  5. No sensory loss
  6. Normal reflexes