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AP II > Neuromuscular Diseases > Flashcards

Neuromuscular Diseases Flashcards

1
Q

What are upper motor neurons?

A

Motor neurons that originate in the cerebral cortex and carry motor information to a lower motor neuron

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2
Q

What is a lower motor neuron?

A

A motor neuron that connects upper motor neuron’s to the end muscle

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3
Q

How are upper motor neuron lesions characterized?

A

Spasticity
Paralysis
Weakness
Hyperactive reflexes

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4
Q

How are low work motor neuron lesions characterized?

A 
Fasciculation
Paralysis
Weakness
Atrophy
Flaccid reflexes
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5
Q

What is the main difference between upper motor neuron lesions and lower motor neuron lesions in terms of paralysis

A

Upper motor neuron lesions cause spastic paralysis

Lower motor neuron lesions cause flaccid paralysis

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6
Q

What is the purpose of Myelin?

A

Speeds neuronal signal conduction

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7
Q

What is myelin?

A

And electrically insulating phospholipid layer that surrounds axons of neuron’s

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8
Q

What type(s) of cells supply the myelin for peripheral and central nerves?

A

Schwann cells = peripheral nerves

Oligodendrocytes = CNS

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9
Q

An auto immune disease with antibodies against the acetylcholine receptors of the motor end plate is known as…

A

Myasthenia gravis

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10
Q

What is the clinical hallmark of myasthenia gravis?

A

Skeletal muscle weakness worsens with activity and improves with rest

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11
Q

What does the severity of myasthenia gravis depend on (in terms of the antibodies)?

A

The ability of the antibodies to decrease the number of available acetylcholine receptors

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12
Q

The bulbar region has an effect on what parts/functions of the human anatomy?

A

Facial control
Airway control
Swallowing

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13
Q

Myasthenia gravis has a correlation with which gland?

A

Thymus glands are often hyperplastic in Myasthenia gravis patients

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14
Q

What are some of the exacerbating factors of myasthenia gravis?

A 
Vaccinations
Infection
Stress
Pregnancy
Increased temperature
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15
Q

Are newborns of myasthenia gravis mothers at risk for respiratory failure? if so, why?

A

Yes. Acetylcholine receptor antibodies cross the placenta.

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16
Q

What are some of the anesthetic considerations for patients with myasthenia gravis?

A

-Bulbar involvement (Aspiration risk)
-Respiratory weakness
-Decreased response to succinylcholine
-Prolonged effects of nondepolarizing muscle relaxants
(Worse with inhaled anesthetics, Magnesium and Aminoglycosides)

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17
Q

List the four post op risk factors for mechanical ventilation in myasthenia gravis patients?

A

Having the disease > 6 years
Chronic respiratory disease
Pyridostigmine dose > 750 mg/day
Preop vital capacity < 2.9 L

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18
Q

What is a myasthenic crisis?

A

An exacerbation of myasthenia gravis characterized by muscle weakness and respiratory insufficiency

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19
Q

What type of test can differentiate between a cholinergic crisis and a myasthenic crisis and how does it work?

A

Tensilon test: Give a little anti-cholinesterase
Pt gets better = myasthenic
Pt gets worse = cholinergic

20
Q

What is myasthenic syndrome and what is another name for it?

A 
Myasthenic syndrome (Lambert Eaton syndrome)
-Antibodies attack calcium gated channels at presynaptic nerve terminals to decrease the release of acetylcholine
21
Q

Differentiate between the physical symptoms of myasthenia gravis and myasthenic syndrome

A

MG: Worse with activity

  • Symptoms move from the top down
  • Very sensitive to non-depolarizer’s
  • Treated with anti-cholinesterase drugs

MS: Improves with activity

  • Symptoms start in the legs and move up
  • Sensitive to both depolarizer’s and non-depolarizer’s
  • Anti-cholinesterase drugs have no benefit
22
Q

Lou Gehrig’s disease is also known as

A

Amyotrophic lateral sclerosis (ALS)

23
Q

Death in ALS usually results from what?

A

Pulmonary infections

24
Q

What is ALS?

A

The loss of nerve cells in the anterior horn of the spinal cord and lower brainstem

(Effects MOTOR not SENSORY)

25
Q

ALS is characterized by

A

Atrophy
Weakness
Exaggerated reflexes
Late stages–Pharyngeal, laryngeal, and respiratory muscles affected

26
Q

What are some of the anesthetic considerations for an ALS patient?

A 
High aspiration risk
Abnormal PFTs
No succ
Sensitive to nondepolarizing agents
Regional if possible
27
Q

What is multiple sclerosis?

A

An autoimmune disease that results in the demyelination of neuron’s in the brain and spinal cord

28
Q

What are some factors that increase the risk for multiple sclerosis?

A

Two times as common in females
20 to 40 years of age
Familial tendency
Increasing latitude

29
Q

What are some of the anesthetic considerations for patients with multiple sclerosis?

A 
Sensitive to local anesthetics
No succ
Don't overheat
Autonomic lability
Resistant to non-depolarizer's
No spinal's
30
Q

What is muscular dystrophy?

A

A hereditary disease caused by abnormality in dystrophin

-Muscle wasting without denervation

31
Q

What is the most common form of muscular dystrophy and how is it contracted?

A

Duchenne’s muscular dystrophy is an X-linked recessive disease characterized by elevated creatinine kinase levels

32
Q

Lab results will show an increase in what for Duchenne’s muscular dystrophy patients?

A

Creatinine kinase levels

33
Q

What are the primary characteristics of Duchenne’s muscular dystrophy?

A 
Presents 3 to 5 years of age
Proximal muscle weakness 
pseudo-hypertrophy of calf muscles
Abnormal gait
Intellectual impairment
Relentless progression
Severe kyphoscoliosis 
Severe bulbar symptoms
Pulmonary HTN due to OSA
Death by 20 to 30 years of age
34
Q

What are some important in aesthetic considerations for muscular dystrophy patients?

A
  • Increased incidence of MH (no sux or inhalational agents)

- Respiratory issues (Bulbar symptoms and restrictive pulmonary disease)

35
Q

List three other types of muscular dystrophy and one drug that should be avoided in all cases

A

Beckers
Facioscapulohumeral dystrophy
Limb-girdle dystrophy

NO SUCCINYLCHOLINE

36
Q

Why is succinylcholine contraindicated in patients with neuromuscular diseases or those who have been immobile for extended periods of time?

A

These patients developed extra junctional receptors along the muscles
-More receptors for sux to bind to and cause hyperkalemia

37
Q

Guillain-Barre syndrome is also known as?

A

ACUTE demyelinating polyneuropathy

38
Q

How is Guillain-Barre syndrome characterized?

A

Sudden onset of ascending motor paralysis
Immune mediated against myelin of peripheral nerves
Severe autonomic dysfunction

39
Q

What are some anesthetic considerations for Guillain-Barre?

A

Bulbar muscle involvement-Aspiration
Autonomic lability
No sux
Regional controversial

40
Q

What is the recommended dose for succinylcholine in myasthenia gravis patients?

A

2x normal or 2.6x normal for intubation

41
Q

What is the recommended dose for nondepolarizing agents in myasthenia gravis patients?

A

Not recommended (extreme sensitivity)

42
Q

Discuss the role of temperature on nerve conduction in multiple sclerosis patients?

A

Increased temperature

decreases conduction

43
Q

What is the abnormal protein in muscular dystrophy?

A

Dystrophin

44
Q

What other disease has a similar defect to muscular dystrophy?

A

Malignant hyperthermia

45
Q

Guillan-Barre Syndrome is usually seen after what type of illness?

A

Viral

-Also has an association with Hodgkin’s and HIV

AP II (32 decks)

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