Special Populations Exam 1-S5 Flashcards
Which type of muscle is not under conscious control and is found within walls of organs and structures such as?
- esophagus
- stomach
-intestines
- bronchi
- uterus
- blood vessels
Smooth muscle
Which type of muscle contain sarcomeres and packed into highly regular repeating arrangements of bundles?
- Striated
Cardiac and Skeletal muscle
Cardiac muscles connects at branching, irregular angles called ___ ___?
Intercalated discs
In the neuromuscular junction which area contains acetylcholinesterase (AChAse)?
Synaptic cleft
Nerve stimulation is caused by depolarization and voltage gated ____ channels open?
Calcium
ACh diffuses across the synaptic cleft and binds to the _____ receptor postsynaptically?
nicotinic cholinergic
Only 2 ____ subunits are capable of binding ACh
Alpha
With depolarization Action potential, ____ and ____ move IN ?
Sodium and Calcium
With depolarization Action potential, ____ moves OUT?
Potassium
The amount of ACh released is influenced by the amount of ___?
Calcium that enters the nerve terminal
Small concentrations of what 2 cations can compete with Calcium influx and can decrease the ACh release and impair neuromuscular transmission?
Magnesium
Aminoglycoside antibiotics
When the frequency of stimulation of NMJ decreases over days to severe burns, immobilization, infection, sepsis and prolonged use of NMBA can cause what?
UPregulation of receptors
Chronic neostigmine use can lead to what?
- Too much ACh
DOWNregulation of receptors
This condition is characterized by inflammation, demyelination, immune dysfunction and failure of cell repair in the CNS?
- muscle weakness
- memory loss
- optic neuritits
- personality disorders
- 8x higher in females
- chronic relapses and remissions
- peripheral nerves not affected
Multiple sclerosis
This condition is an autoimmune disease characterized by T-cell mediated autoantibodies against myelin?
Multiple sclerosis
This condition symptoms begin
20-40 years of age and have 2 or more attacks separated by a month, involve 2 or more areas and elevated IgG and albumin in the CSF?
Multiple sclerosis
How is multiple sclerosis affected by pregnancy?
Reduced risk of exacerbations while pregnant, but increased risk of relapse in postpartum
Treatment for Multiple Sclerosis includes which medications?
Interferon beta- disease modifying agent
Azathioprine, corticosteroids and IV immunoglobins for immunosuppression
Gabapentin for neuropathic pain
Lhermitte sign on assessment is associated with what disease?
Multiple sclerosis
Neck flexion induced electrical sensation
Unthoff sign on assessment is associated with what disease?
Multple sclerosis
Increased body temp/hot flashes
Anesthetic implications of Multiple sclerosis include what?
symptoms of hyperthermia
- Avoid succinycholine due to hyperkalemia
- spinal anesthesia may exacerbate symptoms
Which condition is caused by Amyloid beta protein that creates amyloid plaques, neurofibrillary tangles, and neuronal apoptosis?
- Loss of cholinergic activity
Alzheimer disease
Treatment of Alzheimer disease include the use of which medications?
Acetycholinesterase inhibitors
- Glycopyrrolate due to quat
Which condition is caused by loss of dopaminergic cells in the basal ganglia (lewy bodies)?
Parkinson Disease
Which condition is associated with resting tremor, bradykinesia, shuffling gait, facial immobility and diminished inhibition of extrapyramidal motor system?
Parkinson Disease
Treatment for Parkinson disease?
Levodopa
- do not stop medication for more than 6 hours (muscle rigidity, ventilation issues)
Which 2 medications should you not given to patients with parkinsons?
Reglan and Phenergan (dopamine antagonists)
Increased risk of neuroleptic syndrome
Deep brain stimulator would be most beneficial for which type of patients?
Parkinsons patients due to treatment of severe tremors
Which cerebral cortex motor neurons are affected with ALS?
Upper, Lower, or Mixed
Mixed
Which cerebral cortex motor neurons are affected with Kennedy’s disease?
Upper, Lower, or Mixed
Spinobulbar muscular atrophy
Which cerebral cortex motor neurons are affected with Friedreich’s ataxia?
Upper, Lower, or Mixed
Mixed
Which cerebral cortex motor neurons are affected with Spinal muscular atrophy?
Upper, Lower, or Mixed
Lower
Which condition is this?
- males affected more
- onset 40-60 years
- selective and progressive motor neuronal death
- no treatment
- should avoid general anesthesia and avoid succinycholine
- avoid opioids and benzos due to resp depression
ALS
amytrophic lateral sclerosis
This condition is caused by a sodium channel defect causing prolonged depolarization and flaccid paralysis?
- muscle weakness to tongue and eyelids
- precipitated by metabolic acidosis and cold exposure
- avoid succinycholine
Hyperkalemic periodic paralysis
K >5.5
This condition is caused by calcium or sodium channel defect
- chronic myopathy with aging
- precipitated by stress, hypothermia, high glucose meal
- sensitive to nondepolarizing NMBA
Hypokalemic periodic paralysis
K < 3
This condition is an autoimmune disorder characterized by onset of ascending skeletal muscle weakness/paralysis of the legs due to viral or bacterial infection?
- autonomic dysfunction fluctuations in HR and BP
- cardiac dysrhythmias
- weak respiratory muscles
Guillain-Barre syndrome
Treatment for Guillain-Barre syndrome includes?
Plasma exchange, immunoglobulin
Anesthetic considerations of Guillain-Barre syndrome include?
Avoid rapid movement of patient
Maintain temperature
Monitor for respiratory failure
Avoid succinycholine
Which condition is caused by a decrease in the number of functional postsynaptic, acetylcholine receptors in the neuromuscular junction available for acetylcholine binding?
Myasthenia Gravis
Fatigability/muscle weakness that improves with rest is the hallmark sign of what?
Myasthenia Gravis
What is the protein that allows AChR to cluster at the NMJ?
Muscle Specific Kinase (MuSK)
What is the major antigen in Myasthenia Gravis?
Acetylcholine receptor (AChR)
What is the first notable sign of Myasthenia Gravis?
Weakness of extraocular muscles, diplopia
In Myasthenia Gravis, patients with thymomas can develop what?
Myocarditis
The Tensilon test in diagnosing Myasthenia Gravis is done using which medication?
Edrophonium
Improvement in strength = positive
What represents the most common anterior mediastinal mass shift in patients with Myasthenia Gravis?
Thymus hyperplasia
This condition is characterized by progression to severe muscle weakness, respiratory failure, bundle branch blocks and AFIB?
Myasthenia Crisis
Pregnancy exacerbates the symptoms of ____ by 33%?
- most often in 1st trimester or 6 weeks postpartum
Myasthenia Gravis
The newborn of a mother with Myasthenia Gravis can suffer from ____ which presents as difficulty feeding, ptosis, facial weakness, and respiratory distress 12-48hrs after birth?
Transient Neonatal Myasthenia
lasts for weeks
Difficulty swallowing
Slurring of speech
Difficulty breathing
Neck muscle weakness
are all known as what?
Bulbar symptoms
What is the treatment of Myasthenia Gravis?
Oral Pyridostigmine
- quaternary, does not cross BBB
- improves muscle strength for several hours
- increases conc of ACh at postsynaptic membrane
Diarrhea, urination, miosis, bradycardia, emesis, lacrimation, lethargy and salivation are symptoms of what condition?
Cholinergic Crisis
A thymectomy can be beneficial for treatment of which disease?
Myasthenia Gravis
Discussion for possibility of postoperative ventilation is needed for which type of patients?
Myasthenia Gravis
Forced vital capacity < 2.7L
Vital capacity < 4ml/kg
History of myasthenia crisis
Disease duration > 6 years
Daily Pyridostigmine 750mg or more
COPD
Bulbar symptoms
all what???
Factors that anticipate postoperative mechanical ventilation
Which condition does exercise improve the muscle weakness-related symptoms?
Lambert-Eaton Myasthenic syndrome
What condition is an autoimmune disorder associated with paraneoplastic small cell lung cancer, IgG antibodies attack pre-synpatic calcium channels?
- Decreased release of ACh from presynaptic terminals
- No ocular involvement
- No improvement with ancticholinesterase
Lambert-Eaton Myasthenic Syndrome
What condition is an X-linked recessive disease in which the muscles are replaced with fat/tissue?
- Presents in childhood (3-5)
- Caused by the loss of functional Dystrophin
- Proximal muscle weakness and gait issues (Gower’s Sign)
- elevated CK levels
Duschenne’s Muscular Dystrophy
Which protein plays a major role in stabilization of the muscle membrane and signaling cytoskeleton and extracellular matrix?
- Loss of this protein causes DMD
Dystrophin
Patients with Duchenne’s Muscular dystrophy are especially sensitive to what medications?
Cardiac depressants
Avoid inhalational, sedatives and narcotics
Which medication is an absolute contraindication to Duchenne muscular dystrophy?
Succinycholine
Hyperkalemia
Rhabdomyolysis
Which condition is caused by decreased in normal amounts of dystrophin. Milder than DMD and onset is around 12 years old?
Becker Muscular Dystrophy
Which condition is caused by mutations in 2 proteins that presents with contractures of the ankles, elbows and neck?
- progressive weakness of humeral and peroneal muscles
- Cardiomyopathy and conduction abnormalities
Emergy-Dreifuss Muscular Dystrophy
These symptoms are common to all what?
- Inability of skeletal muscles to relax after stimulation
- Dysfunction of ion channels
- Reduced conductance of chloride ions
- Progressive muscle wasting with weakness
Myotonia
Which condition is an autosomal dominant disorder that has a slow, progressive deterioration of skeletal, cardiac, and smooth muscle wasting?
- Characterized by hypoplastic, dystrophic and weak skeletal muscles and prone to persistent contraction
2 types
Myotonic Dystrophy
Which type of Myotonic Dystrophy?
- Congenital, childhood onset, adult onset
- Most common type
- Myotonic-protein kinase gene
- Defect in Na and Cl channel function
- Conduction defects, heart blocks
- muscle weakness begins distally
- MR, frontal baldness, and cataracts
- Diabetes
DM-1 Steinerts
Which type of Myotonic Dystrophy?
- Proximal myotonic myopathy and myopathy
- Less likely to have Diabetes
- Normal life expectancy
DM-2
Which condition is associated with Sudden cardiac death related to 3rd degree block, respiratory failure or pneumonia?
DM-1
Myotonic Dystrophy
Anesthetic considerations for Myotonic Dystrophy I and II patients?
Have pacer avaliable due to conduction defects
CAN use inhalational agents
NO greater risk of MH
Which condition is an autosomal dominant disorder linked to RYR1 ryanodine receptor on the Sarcoplasmic Reticulum mutation resulting in a massive release of intracellular calcium within skeletal muscle?
Malignant Hyperthermia
Triggering agents for Malignant Hyperthermia include?
Volatile agents
Succinycholine