Neuromuscular disorders

Question 1

Myasthenia Gravis:

1. these pts are sensitive to nondepolarizing or depolarizing NMBs?
2. pts with this and resp muscle or bulbar involvement have an increased risk of what? (PA)
3. this disease is an autoimmune destruction or inactivation of what at the NMJ? (PAR)

Answer 1

1. nondepolarizing
2. pulmonary aspiration
3. postsynaptic Ach receptors

Question 2

myasthenia gravis risk for postop ventilation required after a thymectomy:

1. disease duration greater than how many years?
2. peak inspiratory pressure less than how many cm H2O?
3. if disease where is present?
4. vital capacity less than how much mL/kg?
5. if pyridostigmine dose is greater than how many mg/day?

Answer 2

1. > 6 years
2. < -25 cm H2O
3. lungs
4. < 4 mL/kg
5. > 750 mg/day

Question 3

MG prevalence:

1. highest incidence in women during what decade?
2. men experience 2 peaks during what 2 decades?
3. what is the range of ppl per million?

Answer 3

1. 3rd
2. 3rd and 6th
3. 50-200

Question 4

In MG is there complement mediated damage to postsynaptic motor end plate?

Answer 4

yes

Question 5

myasthenia gravis:

1. what % range of pts develop thymoma?
2. what % range of pts have ocular myasthenia?
3. what % of pts have thymic lymphoid follicular hyperplasia?
4. IgG antibodies are present against Ach receptors in what % range of pts with generalized MG?
5. MG crisis is an exacerbation that requires what? (MV)
6. an MG crisis should be suspected in any pts with resp failure of unclear what? (E)

Answer 5

1. 10-15%
2. 50-70%
3. 70%
4. 85-90%
5. mechanical ventilation
6. etiology

Question 6

myasthenia gravis:

1. what 4 things can lead to an exacerbation? (I, S, S, P)
2. does muscle strength increase or decrease with rest?
3. does muscle strength increase or decrease with exertion?
4. may this disease be asymmetric, confined to one muscle group or be generalized?
5. what muscles are most frequently affected? (O)
6. when there is bulbar involvement there are problems clearing what and with what else? (S, PA)
7. because of this most frequent muscle group involvment, what two things are fluctuating? (P, D)

Answer 6

1. infection, stress, surgery, pregnancy
2. increases
3. decreases
4. yes
5. ocular
6. secretions, pulmonary aspiration
7. ptosis, diplopia

Question 7

myasthenia gravis treatment:

1. what type of meds are used?
2. what is the name of med often prescribed? (P/M)
3. what is a non-medicine treatment for this disease? (P)
4. what is the surgery for this disease? (T)
5. what is the DOA hour range of pyridostigmine?
6. what is the medication used to test for a cholinergic crisis? (E/T)
7. what other 2 med types can be used to treat this disease? (S, I)

Answer 7

1. anticholinesterase
2. pyridostigmine (mestinon)
3. plasmapheresis
4. thymectomy
5. 2-4 hours
6. edrophonium (tensilon)
7. steroids, immunosuppressants

Question 8

Cholinergic crisis:

1. this can occur if too much of what med is taken? (A)
2. what are 4 muscarinic symptoms of this? (S, D, M, B)
3. is strength or weakness increased in a cholinergic crisis after tensilon test?
4. is strength or weakness increased in a myasthenic crisis after tensilon test?
5. what 2 things are seen with this crisis? (IW, EME)

Answer 8

1. anticholinesterase
2. salivation, diarrhea, miosis, bradycardia
3. increased weakness
4. increased strength
5. increased weakness, excessive muscarinic effects

Question 9

up to what % of pts < 55 years old improved after a thymectomy?

Answer 9

85%

Question 10

myasthenia gravis:

1. it pts have preop resp/oropharyngeal weakness, what 2 procedures can be done to decrease the risk of postop resp depression? (I,P)
2. in advance generalized disease, these pts may deteriorate significantly when what agents are held? (A)
3. anticholinesterases can be given parenterally at what fraction of the PO dose?
4. pts can be sensitive to the resp depressant effects of what 2 med types? (O, B)
5. propofol and opioids have a marked depression effect on what system?
6. pts are sensitive or resistant to nondepolarizing NMBs?
7. pt are sensitive or resistant to succs?

Answer 10

1. IVIG, plasmapheresis
2. anticholinesterases
3. 1/30
4. opioids, benzos
5. respiratory depression
6. sensitive
7. resistant

Question 11

myasthenia gravis:

1. Succs dose is increased to what mg/kg to overcome resistance?
2. DOA will increase by what range of minutes at higher doses?
3. parturients have increased weakness during what trimester?
4. what is the preferred anesthesia over GA for parturients even tho a high motor block can lead to hypoventilation? (E)
5. infants of myasthenic mothers may show transient myasthenia for what week range after birth?

Answer 11

1. 2 mg/kg
2. 5-10 minutes
3. 3rd
4. epidural
5. 1-3 weeks

Question 12

Lambert-eaton myasthenic syndrome:

1. is it a distal or proximal muscle weakness that begins in the upper or lower extremities?
2. it may spread to and involve what other parts of the body? (UL, B, RM)
3. often associated with what type of cancer? (SCLC)
4. characterized by a presynaptic defect of neuromuscular transmission where antibodies are made against what voltage gated channels reducing the release of what?

Answer 12

1. proximal, lower
2. upper limb, bulbar, respiratory muscles
3. small cell lung cancer
4. Ca, Ach

Question 13

Lambert-eaton myasthenic syndrome:

1. small cell lung ca cells express identical VG Ca channel blockers resulting in what type of response? (A)
2. does muscle weakness improve or get worse with repeated effort?
3. what 2 meds can be given to increase presynaptic Ach release? (G, D)
4. what other one med and other intervention can be done to help? (C, P)

Answer 13

1. autoimmune
2. improve
3. guanidine, 3, 4 diaminopyridine (DAP
4. corticosteroids, plasmapheresis

Question 14

Lambert-eaton myasthenic syndrome:

1. proximal or distal limb weakness?
2. greater effect on arms or legs?
3. does exercise improve strength or cause weakness?
4. is muscle pain common or uncommon?
5. are reflexes absent/decreased or normal?
6. are more males or females affected?
7. what is the common coexisting disease?
8. sensitive or resistant to succs?
9. sensitive or resistant to nondepolarizing MRs?
10. good or poor response to anticholinesterases?

Answer 14

1. proximal
2. arms
3. improve strength
4. common
5. absent/decreased
6. males
7. small cell lung cancer
8. sensitive
9. sensitive
10. poor response

Question 15

myasthenia gravis:

1. muscle weakness in what 3 ares? (E, B, F)
2. good or poor response to anticholinesterases?
3. does exercise improve strength or cause weakness?
4. is muscle pain common or uncommon?
5. are reflexes absent/decreased or normal?
6. are more males or females affected?
7. what is the common coexisting disease?
8. sensitive or resistant to succs?
9. sensitive or resistant to nondepolarizing MRs?

Answer 15

1. extraocular, bulbar, facial
2. poor
3. weakness
4. uncommon
5. normal
6. females
7. thymoma
8. resistant
9. sensitive

Question 16

which disease is it: limbic encephalitis, neuromyotonia, stiff person syndrome, or polymyositis?

1. inflammatory myopathy of skeletal muscles in proximal limbs with weakness and fatigability
2. peripheral nerve hyperexcitability with myokymia (continuous muscle movements), stiffness and impaired muscle relaxation
3. degenerative CNS disorder with hallucinations, dementia, and personality changes
4. progressive disorder with axial stiffness/rigidity and paraspinal rigidity that may cause spinal deformities

Answer 16

1. polymyositis
2. neuromyotonia
3. limbic encephalitis
4. stiff person syndrome

Question 17

which muscular dystrophy is the most common and severe form?

Answer 17

Duchenne’s

Question 18

Duchenne’s muscular dystrophy:

1. more in males or females?
2. what age range for presentation?
3. symmetric or asymmetric, proximal or distal muscle weakness?
4. progressive weakness and contractures that lead to what? (K)
5. is pulmonary HTN common with disease progression?
6. marked kyphoscoliosis and muscle wasting leads to obstructive or restrictive ventilatory defects?
7. CK level is how many times increased? (range)
8. X-linked dominant or recessive?
9. what kind of infiltrates cause muscles pseudohypertrophy?

Answer 18

1. males
2. 3-5 years
3. symmetric proximal muscle weakness
4. kyphoscoliosis
5. yes
6. restrictive
7. 10-100 times
8. recessive
9. fatty infiltration

Question 19

Duchenne’s muscular dystrophy:

1. what meds can delay progression for 2-3 years? (G)
2. biopsy where to diagnose?
3. is there cardiac muscle degeneration?
4. what % of pts have cardiomyopathy?
5. mitral regurgitation secondary to papillary muscle dysfunction in what % of pts?
6. death at young age d/t what 3 causes? (RPI, RF, CF)
7. most pts are wheelchair bound by what age?
8. is intellectual impairment common?

Answer 19

1. glucocorticoids
2. muscle
3. yes
4. 10%
5. 25%
6. recurrent pulmonary infections, respiratory failure, cardiac failure
7. 12
8. yes

Question 20

Duchenne’s muscular dystrophy EKG changes:

1. what interval is prolonged?
2. arrythmias are common in what part of the heart?
3. what waves are prominent over the right precordium?
4. what waves are deep over the left precordium?
5. what 2 parts of the EKG have agnormalities?

Answer 20

1. PR
2. atrial
3. R waves
4. Q waves
5. QRS, ST segment

Question 21

Becker’s muscular dystrophy:

1. X-linked dominant or recessive?
2. manifestations similar to Duchenne’s but disease occurs when in life? (L)
3. at what point in life does this disease manifest? (A)
4. is progression faster or slower than Duchenne’s?
5. is mental retardation less common than Duchenne’s?
6. pts often reach what decades?
7. death is usually from complications of what system?

Answer 21

1. recessive
2. later
3. adolescence
4. slower
5. yes
6. 4th or 5th
7. respiratory

Question 22

myotonic dystrophy:

1. slowing of relaxation after muscle contraction r/t what stimuli? (E)
2. can be paraneoplastic disorder associated with tumor? (T)
3. muscle weakness and atrophy of what muscles? (CM)
4. what device is needed in these pts with significant conduction defect? (CP)
5. what are 5 antimyotonic drugs? (B,C,D,M,P)
6. do cold or warm temperature worsen stiffness?
7. can stiffness be lessened by continued activity?
8. what is this phenomenon called that occurs when stiffness is lessened by continued activity? (WP)

Answer 22

1. electrical
2. thymoma
3. cranial muscles
4. cardiac pacemaker
5. baclofen, carbamazepine, dantrolene, mexiletine, phenytoin
6. cold
7. yes
8. warm-up phenomenon

Question 23

facioscapulohumeral dystrophy:

1. autosomal recessive or dominant?
2. presents in what 2 decades of life?
3. weakness is confined to muscles in what 2 areas? (F, SG)

Answer 23

1. dominant
2. 2nd/3rd
3. face, shoulder girdle

Question 24

limb-girdle dystrophy:

1. slow progressive muscle weakness that presents in childhood to what 2 decades?
2. what lab value is elevated?
3. respiratory complications d/t what? (H, RRI)

Answer 24

1. 2nd/3rd
2. CK
3. hypoventilation, recurrent respiratory infections

Question 25

Duchenne's/Becker's and anesthesia: 1. manifestations in what 2 systems along with what weakness? (C, P, M) 2. may be associated with what anesthesia emergency? 3. should preop sedatives/opioids be avoided? 4. preop sedatives/opioids should be avoided d/t increased risk of aspiration from what 2 reasons? (RMW, GH) 5. intraoperative positioned can be complicated d/t what 2 things? (K, N/E FC) 6, avoid what med in these pts? (S) 7. are these pts very sensitive or resistant to nondepolarizing MRs? 8. what 2 systems are markedly depressed from VAs in advanced disease? 9. may regional/local be preferred? 10. postop mortality is noted to be d/t complications with what system? 11. pts with VC less than what % of their predicted VC appear to be at greatest risk and often require temporary postoperative mechanical ventilation?

Answer 25

1. cardiac, pulmonary, muscle weakness 2. malignant hyperthermia 3. yes 4. respiratory muscle weakness, gastric hypomotility 5. kyphoscoliosis, neck/extremity flexion contractures 6. succs 7. sensitive 8. circulatory and respiratory 9. yes 10. respiratory 11. <30%

Question 26

myotonic dystrophy: 1. very sensitive to small doses of what 2 types of drugs as well as what 2 ways to give anesthetic drugs? (O, S; I,I) 2. should preop meds be avoided? 3. relative CI to what med d/t chance to precipitate intense myotonic contractions that could complicate intubation? (S) 4. what 3 other drugs that act on motor endplate can aggravate myotonia? (D, N, P) 5. what is the preferred method of anesthesia even though it doesn't prevent myotonic contractions? 6. myotonic contraction of what 2 things make ventilation difficult/impossible? (CW, LM)

Answer 26

1. opioids, sedatives; inhalation and IV 2. yes 3. succs 4. decamethonium, neostigmine, physostigmine 5. regional 6. chest wall, laryngeal muscles

Question 27

myotonic dystrophy: 1. is response to nondepolarizing MRs normal? 2. does the use of nondepolarizing MRs prevent or relieve myotonic contractions? 3. Reversal of NDMRs can induce what? (MC) 4. the use of what type of NDMRs is recommended? (SA) 5. what thing that can happen postoperatively can induce myotonic contractions? (S) 6. if myotonic contractions are caused by shivering, what med should be given to treat them? (M) 7. is induction with propofol or VAs okay? 8. this disease may be associated with what anesthesia emergency? 9. maintenance is recommended with what 2 anesthetic agents? 10. avoid MR reversal with what type of drug? 11. what are 2 names of drugs that should be avoided with myotonic dystrophy? (N, P)

Answer 27

1. yes 2. no 3. myotonic contractions 4. short acting 5. shivering 6. meperidine (demerol) 7. yes 8. MH 9. N2O, VAs 10. anticholinesterases 11. neostigmine physostigmine

Question 28

Myotonic dystrophy postop complications: 1. what are 4 postop complications? (PH, A, A, P) 2. pts need close monitoring and aggressive pulmonary therapy with what 2 things? 3. pt should receive what type of prophylaxis? (A) 4. pts undergoing what surgery or with what type of weakness are more likely to have pulmonary complications? (UAS, SPW)

Answer 28

1. prolonged hypoventilation, atelectasis, aspiration, pneumonia 2. physical therapy, incentive spirometry 3. aspiration 4. upper abd surgery, severe proximal weakness

Question 29

myotonia congenita and paramyotonia congenita: 1. confined to what muscles? 2. is weakness a lot or minimal? 3. abnormal response to what med? (S) 4. what can happen intraoperatively? (MC) 5. what needs to be avoided? (H) 6. infiltration of muscles with what solution may alleviate refractory myotonic contractions? (DLA)

Answer 29

1. skeletal muscles 2. minimal 3. succs 4. myotonic contractions 5. hypothermia 6. diluted LA

Question 30

paramyotonia congenita: 1. does stiffness worsen with activity? 2. characterized by transient stiffness and weakness after exposure to what? (C)

Answer 30

1. yes | 2. cold

Question 31

periodic paralysis: 1. what exacerbates the frequency and severity of muscles weakness/paralysis episodes? (H) 2. genetic types d/t inherited mutations of what 3 ion voltage gated channels? 3. muscle membrane inexcitability to direct and indirect stimulation d/t decreased what ion conductance or increased what ion conductance? 4. what toxicity is associated with secondary form hypokalemic periodic paralysis? (T) 5. secondary hypokalemic paralysis can develop if there are marked losses of K via what 2 routes? (K, GIT) 6. what can be given for acute attacks of secondary form hypokalemic periodic paralysis associated with thyrotoxicosis? 7. Potassium levels greater than what episodes of weakness are related to secondary form of hyperkalemic periodic paralysis? 8. For thyrotoxicosis associated hypokalemic periodic paralysis, what should be manage and what 2 things (one hi and one low) should be avoided with meals?

Answer 31

1. hypothermia 2. Na, K, Ca 3. decreased K, increased Na 4. thyrotoxicosis 5. kidneys, GI tract 6. KCl 7. > 7 8. hyperthyroidism, hi CHO, low K meals

Question 32

anesthesia and periodic paralysis 1. prevent introp what? (A) 2. check what frequently? 3. monitor for what? (A) 4. don't use solutions containing what in pts with hypokalemic paralysis d/t lower K concentration? 5. is response to NDMRs predictable or unpredictable? 6. is succs CI'd in hyperkalemic paralysis or hypokalemic paralysis? 7. important to maintain what since shivering and hypothermia may trigger episodes of periodic paralysis? (CT)

Answer 32

1. attacks 2. K 3. arrhythmia 4. glucose 5. unpredictable 6. hyperkalemic 7. core temp

Question 33

multiple sclerosis: 1. virus may activate T cells that penetrate what? 2. inflammation and demyelination of what to things? (B, SC) 3. ability of neural tissues to repair itself during early phases of disease explains what? (R) 4. what are 5 characteristics of brainstem involvement? (N, D, TN, AD, AV) 5. what are 2 characteristics of SC lesions? (W, P) 6. are legs or arms affected more?

Answer 33

1. BBB 2. brain, spinal cord 3. relapses 4. nystamus, diplopia, trigeminal neuralgia, autonomic dysfunction, altered ventilation 5. weakness, paresthesias 6. legs

Question 34

multiple sclerosis: 1. what % of pts have secondary MS with relapsing/remitting? 2. onset is what age range? 3. S&S of CNS is what color matter? 4. to diagnose need 2+ attacks separated by at least how many months? 5. increased IgG in what fluid?

Answer 34

1. 85% 2. 10-50 3. white 4. 1 month 5. cerebral spinal fluid

Question 35

multiple sclerosis drugs: 1. interferon decreases transit of what cells across BBB? 2. glatiramer mimics what and acts as a decoy for autoantibodies? (M) 3. mitoxantrone is toxic to what organ? 4. what med type can be given for acute relapses? 5. do MS pts have chronic pain? 6. what med can be given for painful dysesthesias, tonic seizures and ataxia? (C) 7. these pts need to avoid what 3 things? (EF, ES, H) 8. demyelinated fibers sensitive to heat can block what? (IC)

Answer 35

1. T-cells 2. myelin 3. heart 4. corticosteroids 5. yes 6. carbamazepine 7. excessive fatigue, emotional stress, heat 8. impulse conduction

Question 36

anesthesia and multiple sclerosis: 1. may regional and GA exacerbate MS? 2. what 3 things can contribute to exacerbation? (I, H, ES) 3. despite a well managed anesthetic, can an MS exacerbation still occur? 4. are demyelinated areas of the SC more sensitive to LAs? 5. can epidural analgesia be provided for MS pts in labor? 6. since demyelinated areas of the SC are more sensitive to LAs, what can be caused? (RN) 7. VAs and autonomic dysfunction can cause a decrease in what? 8. baclofen increases sensitivity to what drugs?

Answer 36

1. yes 2. infection, hyperpyrexia, emotional stress 3. yes 4. yes 5. yes 6. relative neurotoxicity 7. BP 8. NDMRs

Question 37

Guillain-Barre: 1. paresthesia precedes what? (W) 2. paralysis progresses in what direction and includes muscles in what 2 areas? (C; T, A) 3. max weakness what range of weeks after onset? 4. what is the most serious problem? (RI) 5. what % of pts require mechanical ventilation? 6. what % of pts achieve good recovery? 7. what % range of pts have chronic neuropathy? 8. s/s is skeletal muscle weakness/paralysis of what? (L)

Answer 37

1. weakness 2. cephalad; trunk, arms 3. 2-4 weeks 4. respiratory insufficiency 5. 25% 6. 85% 7. 3-5% 8. legs

Question 38

anesthesia and Guillain-Barre: 1. ANS dysfunction can lead to BP drop caused by what 3 things? (PC, BL, PPV) 2. laryngoscopy and ventilation may cause an increase in what 2 things? 3. avoid what med? 4. what 2 short acting NDMR are useful because they have minimal CV effects? 5. sensitivity to NDMR varies from extreme sensitivity to resistant depending on phase of what? (D) 6. likely that pts will need what postop? (MV) 7. if pronounced sensory disturbances, pts may benefit from what? (NO)

Answer 38

1. postural changes, blood loss, positive pressure ventilation 2. HR, BP 3. succs 4. rocuronium, cisatracurium 5. disease 6. mechanical ventilation 7. neuraxial opioids

Question 39

key points: 1. cytoskeletal muscle membrane in pts with muscular dystrophy is susceptible to what? (D) 2. massive release of intracellular contents including potassium with exposure to what 2 things? (S, HIA) 3. myotonic dystrophy produces cardiac conduction delays that manifest as what? (HGAVB) 4. MG pts need what type of MRs and what type of monitoring? (SA, C) 5. should MS pts be advised that an exacerbation of their symptoms may occur during periop? 6. many types of cancer including small cell ca of lung can produce what syndrome? (M)

Answer 39

1. damage 2. succs, halogenated inhalation agents 3. high grade AV block 4. short acting, close 5. yes 6. myasthenic

Question 40

edrophonium (tensilon) 1. what class of med is it? 2. what NT does it increase?

Answer 40

1. anticholinesterase | 2. Ach

Question 41

what is the only disease that affects females more?

Answer 41

myasthenia gravis

Question 42

what are 5 antimyotonic drugs for myotonic dystrophy? (B,C,D,M,P)

Answer 42

1. baclofen 2. carbamazepine 3. dantrolene 4. mexiletine 5. phenytoin