Neuromuscular disorders Flashcards
1
Q
Myasthenia Gravis:
- these pts are sensitive to nondepolarizing or depolarizing NMBs?
- pts with this and resp muscle or bulbar involvement have an increased risk of what? (PA)
- this disease is an autoimmune destruction or inactivation of what at the NMJ? (PAR)
A
- nondepolarizing
- pulmonary aspiration
- postsynaptic Ach receptors
2
Q
myasthenia gravis risk for postop ventilation required after a thymectomy:
- disease duration greater than how many years?
- peak inspiratory pressure less than how many cm H2O?
- if disease where is present?
- vital capacity less than how much mL/kg?
- if pyridostigmine dose is greater than how many mg/day?
A
- > 6 years
- < -25 cm H2O
- lungs
- < 4 mL/kg
- > 750 mg/day
3
Q
MG prevalence:
- highest incidence in women during what decade?
- men experience 2 peaks during what 2 decades?
- what is the range of ppl per million?
A
- 3rd
- 3rd and 6th
- 50-200
4
Q
In MG is there complement mediated damage to postsynaptic motor end plate?
A
yes
5
Q
myasthenia gravis:
- what % range of pts develop thymoma?
- what % range of pts have ocular myasthenia?
- what % of pts have thymic lymphoid follicular hyperplasia?
- IgG antibodies are present against Ach receptors in what % range of pts with generalized MG?
- MG crisis is an exacerbation that requires what? (MV)
- an MG crisis should be suspected in any pts with resp failure of unclear what? (E)
A
- 10-15%
- 50-70%
- 70%
- 85-90%
- mechanical ventilation
- etiology
6
Q
myasthenia gravis:
- what 4 things can lead to an exacerbation? (I, S, S, P)
- does muscle strength increase or decrease with rest?
- does muscle strength increase or decrease with exertion?
- may this disease be asymmetric, confined to one muscle group or be generalized?
- what muscles are most frequently affected? (O)
- when there is bulbar involvement there are problems clearing what and with what else? (S, PA)
- because of this most frequent muscle group involvment, what two things are fluctuating? (P, D)
A
- infection, stress, surgery, pregnancy
- increases
- decreases
- yes
- ocular
- secretions, pulmonary aspiration
- ptosis, diplopia
7
Q
myasthenia gravis treatment:
- what type of meds are used?
- what is the name of med often prescribed? (P/M)
- what is a non-medicine treatment for this disease? (P)
- what is the surgery for this disease? (T)
- what is the DOA hour range of pyridostigmine?
- what is the medication used to test for a cholinergic crisis? (E/T)
- what other 2 med types can be used to treat this disease? (S, I)
A
- anticholinesterase
- pyridostigmine (mestinon)
- plasmapheresis
- thymectomy
- 2-4 hours
- edrophonium (tensilon)
- steroids, immunosuppressants
8
Q
Cholinergic crisis:
- this can occur if too much of what med is taken? (A)
- what are 4 muscarinic symptoms of this? (S, D, M, B)
- is strength or weakness increased in a cholinergic crisis after tensilon test?
- is strength or weakness increased in a myasthenic crisis after tensilon test?
- what 2 things are seen with this crisis? (IW, EME)
A
- anticholinesterase
- salivation, diarrhea, miosis, bradycardia
- increased weakness
- increased strength
- increased weakness, excessive muscarinic effects
9
Q
up to what % of pts < 55 years old improved after a thymectomy?
A
85%
10
Q
myasthenia gravis:
- it pts have preop resp/oropharyngeal weakness, what 2 procedures can be done to decrease the risk of postop resp depression? (I,P)
- in advance generalized disease, these pts may deteriorate significantly when what agents are held? (A)
- anticholinesterases can be given parenterally at what fraction of the PO dose?
- pts can be sensitive to the resp depressant effects of what 2 med types? (O, B)
- propofol and opioids have a marked depression effect on what system?
- pts are sensitive or resistant to nondepolarizing NMBs?
- pt are sensitive or resistant to succs?
A
- IVIG, plasmapheresis
- anticholinesterases
- 1/30
- opioids, benzos
- respiratory depression
- sensitive
- resistant
11
Q
myasthenia gravis:
- Succs dose is increased to what mg/kg to overcome resistance?
- DOA will increase by what range of minutes at higher doses?
- parturients have increased weakness during what trimester?
- what is the preferred anesthesia over GA for parturients even tho a high motor block can lead to hypoventilation? (E)
- infants of myasthenic mothers may show transient myasthenia for what week range after birth?
A
- 2 mg/kg
- 5-10 minutes
- 3rd
- epidural
- 1-3 weeks
12
Q
Lambert-eaton myasthenic syndrome:
- is it a distal or proximal muscle weakness that begins in the upper or lower extremities?
- it may spread to and involve what other parts of the body? (UL, B, RM)
- often associated with what type of cancer? (SCLC)
- characterized by a presynaptic defect of neuromuscular transmission where antibodies are made against what voltage gated channels reducing the release of what?
A
- proximal, lower
- upper limb, bulbar, respiratory muscles
- small cell lung cancer
- Ca, Ach
13
Q
Lambert-eaton myasthenic syndrome:
- small cell lung ca cells express identical VG Ca channel blockers resulting in what type of response? (A)
- does muscle weakness improve or get worse with repeated effort?
- what 2 meds can be given to increase presynaptic Ach release? (G, D)
- what other one med and other intervention can be done to help? (C, P)
A
- autoimmune
- improve
- guanidine, 3, 4 diaminopyridine (DAP
- corticosteroids, plasmapheresis
14
Q
Lambert-eaton myasthenic syndrome:
- proximal or distal limb weakness?
- greater effect on arms or legs?
- does exercise improve strength or cause weakness?
- is muscle pain common or uncommon?
- are reflexes absent/decreased or normal?
- are more males or females affected?
- what is the common coexisting disease?
- sensitive or resistant to succs?
- sensitive or resistant to nondepolarizing MRs?
- good or poor response to anticholinesterases?
A
- proximal
- arms
- improve strength
- common
- absent/decreased
- males
- small cell lung cancer
- sensitive
- sensitive
- poor response
15
Q
myasthenia gravis:
- muscle weakness in what 3 ares? (E, B, F)
- good or poor response to anticholinesterases?
- does exercise improve strength or cause weakness?
- is muscle pain common or uncommon?
- are reflexes absent/decreased or normal?
- are more males or females affected?
- what is the common coexisting disease?
- sensitive or resistant to succs?
- sensitive or resistant to nondepolarizing MRs?
A
- extraocular, bulbar, facial
- poor
- weakness
- uncommon
- normal
- females
- thymoma
- resistant
- sensitive
16
Q
which disease is it: limbic encephalitis, neuromyotonia, stiff person syndrome, or polymyositis?
- inflammatory myopathy of skeletal muscles in proximal limbs with weakness and fatigability
- peripheral nerve hyperexcitability with myokymia (continuous muscle movements), stiffness and impaired muscle relaxation
- degenerative CNS disorder with hallucinations, dementia, and personality changes
- progressive disorder with axial stiffness/rigidity and paraspinal rigidity that may cause spinal deformities
A
- polymyositis
- neuromyotonia
- limbic encephalitis
- stiff person syndrome