Neurodisease
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Questions and Answers

What is a hallmark symptom of Duchenne muscular dystrophy? select all that apply

  • Pseudohypertrophy of muscles, especially calves (correct)
  • Chronic respiratory insufficiency (correct)
  • Progressive weakness, clumsy gait (correct)
  • Gower's sign - using hands to help get up due to pelvic girdle weakness (correct)

    • Which of the following anesthetic considerations should be prioritized for patients with Duchenne muscular dystrophy?

  • Awareness of delayed gastric emptying and decreased motility to reduce aspiration risk (correct)
  • Pulmonary and cardiac complications are common (correct)
  • Macroglossia can lead to difficult airway management (correct)
  • Avoid succinylcholine and volatile anesthetics due to hyperkalemia and rhabdomyolysis (correct)

    • What distinguishes Becker muscular dystrophy from Duchenne muscular dystrophy?

  • Adolescent onset with a milder presentation (correct)
  • Characterized by rapid symptom progression
  • Onset during later adulthood
  • Absence of cardiac involvement

    • Which of the following is a symptom associated with myotonic muscular dystrophy?

    <p>Delayed relaxation after muscle contraction resulting in symptoms of spasticity</p> Signup and view all the answers

    What is the expected trend in serum CK levels as muscular dystrophy progresses?

    <p>They initially rise and then progressively decrease when there is no muscle left to break down</p> Signup and view all the answers

    Which statement accurately describes the action of acetylcholinesterase inhibitors?

    <p>They increase the availability of acetylcholine at nicotinic receptors by inhibiting acetylcholinesterase.</p> Signup and view all the answers

    Which type of receptor is primarily affected by anticholinergics?

    <p>Muscarinic ACh receptors</p> Signup and view all the answers

    What is the primary mechanism by which Myasthenia Gravis leads to muscle weakness?

    <p>Autoimmune destruction of nicotinic acetylcholine receptors at the neuromuscular junction</p> Signup and view all the answers

    Bulbar symptoms are associated with which condition, and what do they primarily affect?

    <p>Myasthenia Gravis; they primarily result in oropharyngeal weakness and difficulty swallowing.</p> Signup and view all the answers

    What symptom pattern is characteristic of Myasthenia Gravis?

    <p>Gradual onset of severe muscle weakness that improves with rest.</p> Signup and view all the answers

    What is the main reason for using amide-based local anesthetics in patients with myasthenia gravis?

    <p>Ester LA are inactivated by plasma cholinesterase leading to a prolonged duration of action in patients that take acetylcholinesterase inhibitors</p> Signup and view all the answers

    What dosing consideration should be taken when administering cholinesterase inhibitors intravenously compared to oral dosing?

    <p>The IV dose should be one-thirtieth of the oral dose.</p> Signup and view all the answers

    Which condition is associated with an increased risk of acute respiratory failure in myasthenia gravis patients?

    <p>Pregnancy</p> Signup and view all the answers

    Which clinical scenario is likely a trigger for exacerbation in autoimmune diseases like myasthenia gravis?

    <p>Severe infections</p> Signup and view all the answers

    What untoward effect does excess dosing of pyridostigmine have on a patient?

    <p>Cholinergic crisis due to excess acetylcholine</p> Signup and view all the answers

    Which treatment is most commonly used for mild myasthenia gravis?

    <p>Anticholinesterase drugs like pyridostigmine</p> Signup and view all the answers

    During preoperative management for thymectomy, which is a critical consideration for avoiding complications? select all that apply

    <p>Optimize medical management - take normal regimen of acetylcholinesterase inhibitor</p> Signup and view all the answers

    What is the primary cause of airway obstruction and respiratory failure in tetanus?

    <p>Sustained spasm of airway musculature</p> Signup and view all the answers

    Which symptom is characteristic of botulism? select 2

    <p>Progressive descending flaccid paralysis</p> Signup and view all the answers

    What underlying factor triggers Guillain-Barre syndrome? select 2

    <p>Acute infections e.g. flu, COVID-19, vaccinations</p> Signup and view all the answers

    Which anesthetic agent is recommended to be avoided in patients with ALS or any patient with an upregulation of fetal nAChR?

    <p>Succinylcholine</p> Signup and view all the answers

    What treatment option is known to prolong life in ALS patients?

    <p>Riluzole</p> Signup and view all the answers

    What condition primarily affects males (x-linked) due to a genetic defect in dystrophin?

    <p>Duchenne muscular dystrophy</p> Signup and view all the answers

    What anesthetic drug should be avoided in patients undergoing surgery while on MAOIs?

    <p>Meperidine</p> Signup and view all the answers

    What condition involves a genetic mutation causing defects in myelin formation and leads to reduced nerve conduction?

    <p>Charcot-Marie tooth disease</p> Signup and view all the answers

    Which of the following medications can cause dystonia when administered rapidly? select 2

    <p>Fentanyl</p> Signup and view all the answers

    What is a potential risk associated with the placement of deep brain stimulation electrodes for Parkinson's?

    <p>Seizure</p> Signup and view all the answers

    Which anesthetic consideration is crucial for patients with Parkinson’s dementia being treated with acetylcholinesterase inhibitors? select 2

    <p>Increased resistance to NDNMBs</p> Signup and view all the answers

    What does the presence of Lewy bodies in Parkinson's disease indicate?

    <p>Clumps of abnormal protein in dopamine-producing cells</p> Signup and view all the answers

    What symptom typically presents first in patients with Parkinson's disease?

    <p>Motor symptoms before cognitive impairment</p> Signup and view all the answers

    What is the primary characteristic of facioscapulohumeral muscular dystrophy?

    <p>Weakness of face and shoulder girdle</p> Signup and view all the answers

    Which of the following is NOT considered a type of periodic paralysis?

    <p>Chronic hyperkalemic paralysis</p> Signup and view all the answers

    In patients with Huntington's disease, which symptom is primarily associated with the disease? select 3

    <p>Depression and psychosis</p> Signup and view all the answers

    Which anesthesia consideration is particular to patients with Alzheimer's disease?

    <p>Risk of worsened confusion with benzodiazepines</p> Signup and view all the answers

    In the distinction between Guillain-Barré Syndrome and multiple sclerosis, which statement is correct?

    <p>GBS is usually self-limiting with treatment while MS is progressive</p> Signup and view all the answers

    Which condition involves autoimmune destruction of myelin in peripheral nerves?

    <p>Guillain-Barré Syndrome</p> Signup and view all the answers

    What is the primary treatment for managing symptoms of Huntington's disease?

    <p>Palliative interventions</p> Signup and view all the answers

    What defines vascular dementia?

    <p>Dementia resulting from disrupted blood flow in the brain</p> Signup and view all the answers

    In Parkinson's disease, what leads to the characteristic symptoms?

    <p>Loss of dopamine</p> Signup and view all the answers

    What is an effect of chronic steroid use in surgical patients?

    <p>Adrenal insufficiency due to suppression of the HPA axis</p> Signup and view all the answers

    What are the targets for anticholinesterase inhibitors?

    <p>Acetylcholinesterase and Plasma Cholinesterase</p> Signup and view all the answers

    Where are nicotinic acetylcholine receptors (AChRs) primarily found?

    <p>Autonomic ganglia and skeletal muscle</p> Signup and view all the answers

    Which drug class blocks nicotinic acetylcholine receptors (AChR)?

    <p>Neuromuscular blockers</p> Signup and view all the answers

    How do anticholinergics work?

    <p>They competitively block the binding of acetylcholine and prevent receptor activation at muscarinic receptors.</p> Signup and view all the answers

    anticholinergic = antimuscarinic

    <p>True</p> Signup and view all the answers

    Acetylcholinesterase inhibitor drugs = neostigmine, pyridostigmine, edrophonium, physostigmine

    <p>True</p> Signup and view all the answers

    Where are the antibodies produced that cause the symptoms associated with myasthenia gravis (MG)?

    <p>Thymus</p> Signup and view all the answers

    Which of the following describes the pattern of symptoms in Myasthenia Gravis (MG)?

    <p>Descending skeletal muscle weakness that worsens with activity and improves with rest</p> Signup and view all the answers

    Why do babies born to mothers with Myasthenia Gravis experience transient symptoms?

    <p>Because antibodies can cross through the placenta and attack the fetal postsynaptic nAChR.</p> Signup and view all the answers

    What is the target for antibody-mediated destruction in Myasthenia Gravis (MG)?

    <p>Postsynaptic nicotinic AChR</p> Signup and view all the answers

    What test was historically used to assess for Myasthenia Gravis (MG)?

    <p>Tensilon test - increased ACh levels resulting in symptom improvement</p> Signup and view all the answers

    What is thymus hyperplasia associated with?

    <p>Myasthenia Gravis 47% -81% of all pts</p> Signup and view all the answers

    What is a complication of regional techniques for a thymectomy, specifically the use of high regional techniques like supraclavicular block?

    <p>Worsening of respiratory symptoms in myasthenia gravis (MG)</p> Signup and view all the answers

    What are postoperative ventilation predictors for the patient with Myasthenia Gravis (MG)? (Select all that apply)

    <p>High BMI</p> Signup and view all the answers

    What is the normal range for vital capacity in adults?

    <p>60-70 ml/kg</p> Signup and view all the answers

    Which of the following drugs can cause muscle weakness in patients with Myasthenia Gravis (MG)? select 2

    <p>Steroids</p> Signup and view all the answers

    What is the preferred extubation technique for a patient with muscular weakness disorder?

    <p>Awake extubation</p> Signup and view all the answers

    What is the expected outcome when administering succinylcholine to a patient with Myasthenia Gravis (MG)?

    <p>Resistance to succinylcholine requiring greater doses due to less AChR at the postsynaptic membrane</p> Signup and view all the answers

    What is unique about the effects of organophosphates as compared to anticholinergics?

    <p>Organophosphate exposure causes irreversible cholinesterase inhibition.</p> Signup and view all the answers

    What is the baseline Train-of-Four (ToF) response in a patient with Myasthenia Gravis (MG)?

    <p>Fade</p> Signup and view all the answers

    What is the preferred neuromuscular blocking drug (NMBD) used in myasthenia gravis (MG)?

    <p>Rocuronium because you can reverse with sugammadex</p> Signup and view all the answers

    Cholinergic crisis = SLUDGE = salivation, lacrimation, urination, diaphoresis, GI sx, emesis, AND miosis

    <p>True</p> Signup and view all the answers

    What is a differentiating feature of myasthenic crisis?

    <p>Respiratory failure due to weakness</p> Signup and view all the answers

    What is Lambert-Eaton syndrome associated with?

    <p>Lung cancer</p> Signup and view all the answers

    What is the pathophysiology of Lambert-Eaton Syndrome (LES)?

    <p>Muscle weakness is caused by autoimmune-mediated destruction of presynaptic voltage-gated calcium channels, resulting in reduced ACh release at the neuromuscular junction.</p> Signup and view all the answers

    What is the pattern of skeletal muscle weakness in Lambert-Eaton Syndrome (LES)?

    <p>Ascending weakness - opposite of MG</p> Signup and view all the answers

    What is unique about muscle weakness in Lambert-Eaton Syndrome (LES)?

    <p>It improves with exercise due to increased release of ACh.</p> Signup and view all the answers

    What causes muscle weakness in Lambert-Eaton Syndrome (LES)?

    <p>Decreased availability of ACh at the NMJ due to less being released from the presynaptic membrane</p> Signup and view all the answers

    LES is characterized by ____.

    <p>increased sensitivity to NMBs(depolarizing and non-depolarizing)</p> Signup and view all the answers

    What is a treatment option for Lambert-Eaton Syndrome (LES)?

    <p>3,4-diaminopyrimidine to increase presynaptic calcium and release of acetylcholine</p> Signup and view all the answers

    Weakness often precedes the discovery of a tumor in which neurodegenerative disease?

    <p>LES</p> Signup and view all the answers

    What is polymyositis?

    <p>An inflammatory myopathy of skeletal muscles affecting primarily proximal limb muscles, thoracic muscles, and oropharyngeal muscles.</p> Signup and view all the answers

    What other complication is Stiff Person Syndrome associated with?

    <p>Cancer</p> Signup and view all the answers

    What are patients with polymyositis prone to? (Select all that apply)

    <p>Dysphagia</p> Signup and view all the answers

    What is limbic encephalitis?

    <p>A degenerative autoimmune CNS disorder associated with cancer</p> Signup and view all the answers

    What can cause acute flaccid paralysis?

    <p>Viral infection of the brainstem and lower motor neurons</p> Signup and view all the answers

    Which two neurodegenerative diseases are caused by bacteria? select 2

    <p>Tetanus</p> Signup and view all the answers

    What disease process is similar to botulism due to the inhibition of presynaptic release of acetylcholine (ACh)?

    <p>Lambert-Eaton Syndrome (LES)</p> Signup and view all the answers

    What neurodegenerative condition is considered a neuromuscular emergency?

    <p>Guillain-Barré Syndrome (GBS)</p> Signup and view all the answers

    What is Guillain-Barré Syndrome (GBS)?

    <p>An autoimmune disease affecting peripheral nerves and nerve roots, usually triggered by an acute infection.</p> Signup and view all the answers

    What is the alternate name for Guillain-Barré Syndrome (GBS)?

    <p>Acute Inflammatory Demyelinating Polyneuropathy (AIDP)</p> Signup and view all the answers

    Which is the only neurodegenerative disease that is characterized by its resistance to succinylcholine?

    <p>Myasthenia Gravis</p> Signup and view all the answers

    What is the symptom pattern in Guillain-Barré Syndrome (GBS)?

    <p>Bilateral paresthesia that starts in lower extremities and ascends bilaterally</p> Signup and view all the answers

    What differentiates the symptoms of Lambert-Eaton Syndrome (LES) from Guillain-Barré Syndrome (GBS)? select 2

    <p>LES has a gradual onset of symptoms, while GBS has an acute onset.</p> Signup and view all the answers

    What is ALS?

    <p>A degenerative motor neuron disease affecting both upper and lower motor neurons</p> Signup and view all the answers

    What commonly used neuromuscular blocking drug (NMBD) has a black box warning for use in pediatric patients?

    <p>Succinylcholine</p> Signup and view all the answers

    What is the pattern of weakness in ALS?

    <p>Spasticity in upper extremities and flaccidity in lower extremities until there is no function</p> Signup and view all the answers

    What causes adverse reactions when using volatile anesthetics (VA) on a patient with muscular dystrophy?

    <p>Calcium dysregulation due to the abnormal sarcolemma and muscle damage resulting in MH-like symptoms</p> Signup and view all the answers

    What causes non-depolarizing neuromuscular blockades (NMBDs) to have a delayed onset and prolonged duration in patients with muscular dystrophy?

    <p>Smaller muscle mass resulting in less distribution of NMBD - higher concentrations and prolonged DoA</p> Signup and view all the answers

    What type of muscular dystrophy results in symptoms similar to premature aging?

    <p>Myotonic muscular dystrophy</p> Signup and view all the answers

    Which types of muscular dystrophy usually appear during adolescence or early adulthood? select 2

    <p>Becker muscular dystrophy</p> Signup and view all the answers

    What is usually spared from weakness in Facioscapulohumeral muscular dystrophy?

    <p>Respiratory muscles</p> Signup and view all the answers

    What does CK (Creatine Kinase) measure?

    <p>Muscle damage</p> Signup and view all the answers

    What is myotonia congenita?

    <p>An incurable genetic disease causing myotonia</p> Signup and view all the answers

    No cardiac involvement in myotonia congenita

    <p>True</p> Signup and view all the answers

    What is periodic paralysis?

    <p>A disorder characterized by spontaneous episodes of weakness or paralysis due to K+,Na+, Ca++, or thyroid hormone imbalance</p> Signup and view all the answers

    What is considered a risk factor for multiple sclerosis (MS)? select 2

    <p>Epstein-Barr Virus</p> Signup and view all the answers

    What is expected during the anesthetic management of a patient with multiple sclerosis (MS)?

    <p>Reduced MAC and delayed emergence</p> Signup and view all the answers

    Which of the following factors are associated with worsening symptoms in patients with Multiple Sclerosis (MS)?

    <p>Both A and B</p> Signup and view all the answers

    What should be considered if your patient with adrenocortical suppression is hypotensive intraoperatively? select all that apply

    <p>Administer steroids</p> Signup and view all the answers

    What is Huntington's disease?

    <p>A rare progressive neurodegenerative disease that leads to degenerative changes in the brain.</p> Signup and view all the answers

    What are the expected acetylcholine levels in a patient with Alzheimer's disease?

    <p>Low levels</p> Signup and view all the answers

    What neurodegenerative disease is characterized by dystonia?

    <p>Huntington's disease</p> Signup and view all the answers

    What type of medications should be avoided in patients with Parkinson's disease?

    <p>Antidopaminergic medications (e.g., Reglan)</p> Signup and view all the answers

    What causes increased sensitivity to both succinylcholine and non-depolarizing NMBDs in certain neurodegenerative diseases? select 2

    <p>Extrajunctional receptors</p> Signup and view all the answers

    What is a seizure?

    <p>A sudden, uncontrolled electrical disturbance in the brain</p> Signup and view all the answers

    What is commonly used to treat chorea in Huntington's disease?

    <p>Tetrabenazine</p> Signup and view all the answers

    What are some common treatments for Parkinson's disease? (Select all that apply)

    <p>Dopaminergic agents</p> Signup and view all the answers

    Most patients with Parkinson's disease also have Lewy bodies

    <p>True</p> Signup and view all the answers

    How much can seizure activity increase brain metabolism?

    <p>400%</p> Signup and view all the answers

    Match the following types of seizures with their descriptions:

    <p>Tonic = Stiffening of body muscles with falling and loss of consciousness Atonic = Sudden loss of muscle tone with no loss of consciousness Myoclonic = No loss of consciousness Tonic-clonic = Grand mal seizure Hyperkinetic = Motor activity with or without preserved awareness</p> Signup and view all the answers

    What is the cause of most perioperative seizures in non-epileptic patients?

    <p>Metabolic derangements from hyponatremia</p> Signup and view all the answers

    What should be used to manage an intraoperative seizure? select 2

    <p>Benzodiazepines</p> Signup and view all the answers

    What is the effect of chronic antiepileptic drugs on anesthetic drugs?

    <p>They increase dosage requirements for anesthetics and non-depolarizing neuromuscular blocking drugs.</p> Signup and view all the answers

    What is status epilepticus? select 2

    <p>A continuous motor seizure lasting more than 20 minutes</p> Signup and view all the answers

    What are the symptoms of an intraoperative seizure? (Select all that apply)

    <p>Tachycardia</p> Signup and view all the answers

    Study Notes

    Acetylcholine

    • Activates muscarinic receptors in the SA node, bronchial smooth muscle, and salivary glands
    • Blocked by anticholinergics
    • Activates nicotinic receptors in autonomic ganglia and skeletal muscle
    • Blocked by neuromuscular blockers
    • Acetylcholinesterase inhibitors increase acetylcholine at nicotinic receptors by inhibiting acetylcholinesterase.
    • Acetylcholinesterase inhibitor drugs include neostigmine, pyridostigmine, edrophonium, and physostigmine.
    • Anticholinergics (antimuscarinics) competitively block acetylcholine binding and prevent receptor activation.
    • Bulbar symptoms are related to the corticobulbar tract, including oropharyngeal weakness and difficulty swallowing.
    • Myasthenia Gravis (MG) is characterized by a grave muscle weakness improving with rest.
    • Autoimmune destruction of postsynaptic nicotinic acetylcholine receptors at the NMJ leads to reduced functional receptors.
    • Symptoms include skeletal muscle weakness worsening with activity, improving with rest, diplopia, dysarthria, dysphagia (difficulty swallowing), and limited facial expressions.
    • Symptoms start in the eyes and move downward.
    • Associated with other autoimmune diseases such as rheumatoid arthritis, lupus, polymyositis, or autoimmune thyroiditis/hyperthyroidism.
    • Life expectancy is normal.
    • MG is characterized by remissions and exacerbations.
    • Triggers for exacerbation include stress, infection, surgery, and pregnancy.

    Treatment

    • Mild disease: anticholinesterase drugs (e.g., pyridostigmine).
    • Moderate disease: anticholinesterase drugs, corticosteroids, IVIG, or immunosuppressants.
    • Severe disease: plasmapheresis or thymectomy
    • Pyridostigmine dosing should be limited to 1/30 of oral dose for IV administration.
    • Thymectomy may resolve MG
    • Optimize medical management before surgery.
    • Aspiration risk, therefore, consider RSI and pre-treat with reglan/H2 blockers.
    • Regional anesthesia may be appropriate, but note possible worsening of respiratory complications.
    • Postoperative mechanical ventilation is a possible predictor for complications.

    Additional Notes

    • Tensilon/edrophonium test can increase acetylcholine levels, potentially resulting in symptom improvement.
    • Blood tests and electromyography are used to detect serum acetylcholine receptor antibodies or investigate specific muscle activity.
    • Thymectomy and appropriate medical management are possible treatments for MG.
    • Excess pyridostigmine dosing causes cholinergic crisis.
    • Cholinergic crisis is an adverse effect of anticholinesterase medications characterized by excessive muscarinic and nicotinic stimulation.
    • Myasthenic crisis results from a lack of acetylcholine.
    • Anticholinesterases are used in cholinergic crisis, steroids or immunotherapy can be used in myasthenic crisis.
    • Succinylcholine is contraindicated in patients with myasthenia gravis due to hyperkalemia and possibly worsened myasthenic problems.
    • Medications that can worsen muscle relaxation such as steroids or aminoglycosides, should be avoided.
    • Minimizing or avoiding muscle relaxants, if appropriate.
    • Succinylcholine may require increased dosages for patients resistant to the effects.
    • Rocuronium or other aminosteroidal neuromuscular blockers (NMBs) may be considered with predictable reversal through sugammadex.

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    Description

    This quiz covers the role of acetylcholine in activating muscarinic and nicotinic receptors, its inhibition by various blockers, and its relationship with conditions such as Myasthenia Gravis. Test your knowledge on acetylcholinesterase inhibitors and the physiological effects associated with acetylcholine signaling.

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