Patho Exam #3: Neurodegenerative Diseases
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Questions and Answers

Huntington's disease is classified as which type of disease?

  • Endocrine
  • Neurodegenerative (correct)
  • Infectious
  • Cardiovascular

    • What is a primary characteristic associated with Huntington's disease?

  • Vision impairment
  • Cognitive Decline (correct)
  • Digestive Issues
  • Hearing Loss

    • Which of the following is most closely associated with Huntington's Disease?

  • Sudden cardiac arrest
  • Progressive neuron degeneration (correct)
  • Progressive joint inflammation
  • Rapid decrease in blood sugar

    • What is the typical effect of Huntington's disease on cognitive functions?

    <p>Progressive decline in cognitive abilities (C)</p> Signup and view all the answers

    Which category does Huntington's disease fall into based on its effects on the nervous system?

    <p>Neurodegenerative diseases (D)</p> Signup and view all the answers

    What is Edaravone known to be?

    <p>A fantastic free-radical scavenger (A)</p> Signup and view all the answers

    What does Edaravone reduce?

    <p>Oxidative damage to neurons (C)</p> Signup and view all the answers

    With which therapy is Edaravone considered an excellent adjunctive?

    <p>Viluzole (A)</p> Signup and view all the answers

    What is the function of the Substantia Nigra?

    <p>Releases glutamate (C)</p> Signup and view all the answers

    What is the relationship between Edaravone and its mechanism of action?

    <p>MOA is not fully understood (D)</p> Signup and view all the answers

    What is the thalamus associated with?

    <p>Motor nuclei (C)</p> Signup and view all the answers

    What type of radicals are affected by Edaravone?

    <p>Fantastic free-radicals (B)</p> Signup and view all the answers

    What does ROS stand for?

    <p>Reactive Oxygen Species (C)</p> Signup and view all the answers

    Which neurotransmitter is associated with the direct pathway as shown?

    <p>Glutamate (D)</p> Signup and view all the answers

    What is the function of GABA?

    <p>Inhibitory neurotransmitter (C)</p> Signup and view all the answers

    Which basal ganglia structure provides dopaminergic input?

    <p>Substantia Nigra pars compacta (D)</p> Signup and view all the answers

    Which of the following promotes neuronal survival?

    <p>Pharmacologic interventions (D)</p> Signup and view all the answers

    Where does glutamate get released in the basal ganglia circuitry?

    <p>Cortex (B)</p> Signup and view all the answers

    Which condition is Amyotrophic Lateral Sclerosis (ALS)?

    <p>Mitochondrial damage (B)</p> Signup and view all the answers

    What is located in the structure of the Striatium?

    <p>Caudate and putamen (D)</p> Signup and view all the answers

    What is the primary goal of pharmacological interventions in ALS?

    <p>Promote neuronal survival (C)</p> Signup and view all the answers

    What does the Substantia Nigra release?

    <p>Dopamine (D)</p> Signup and view all the answers

    What is the ONLY proven survival benefit for ALS?

    <p>Pharmacologic intervention (D)</p> Signup and view all the answers

    Which neurotransmitter is reduced in Parkinson's Disease?

    <p>Dopamine (D)</p> Signup and view all the answers

    The striatum is composed primarily of what?

    <p>Caudate and Putamen (C)</p> Signup and view all the answers

    Which of the following can cause seizures?

    <p>Alcohol Withdrawal (A)</p> Signup and view all the answers

    Isoniazid-induced pyridoxine deficiency leads to what?

    <p>Increased Glutamate Accumulation (D)</p> Signup and view all the answers

    Clozapine is typically used in the management of what condition?

    <p>Schizophrenia (A)</p> Signup and view all the answers

    What is a potential hematological side effect of Clozapine?

    <p>Agranulocytosis (A)</p> Signup and view all the answers

    Which of the following neurotransmitters is associated with the GPe (Globus Pallidus externa)?

    <p>GABA (D)</p> Signup and view all the answers

    What is the result of glutamate toxicity in neurons?

    <p>Hyperexcitation (C)</p> Signup and view all the answers

    Which part of the brain sends signals via GABA to the thalamus?

    <p>GPi and Substantia pars reticulata (C)</p> Signup and view all the answers

    What effect does decreased GABA typically have?

    <p>Increased excitation (D)</p> Signup and view all the answers

    Flashcards

    Huntington's Disease

    A genetic disorder causing progressive neurodegeneration, affecting movement and cognition.

    Neurodegenerative Disease

    Disorders characterized by the gradual degeneration of the nervous system.

    Clinical findings

    observable signs and symptoms associated with a condition.

    Progressive symptoms

    Symptoms that worsen over time in neurodegenerative diseases.

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    Cognitive decline

    Deterioration of cognitive functions, including memory and reasoning.

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    Thalamus

    A brain structure that relays sensory information to the cortex.

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    Gab

    Short for gamma-aminobutyric acid, an inhibitory neurotransmitter.

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    Edaravone

    A medication with an unknown mechanism, known for scavenging free radicals.

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    Oxidative stress

    An imbalance between free radicals and antioxidants in the body.

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    Free radicals

    Unstable atoms that can cause cellular damage.

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    Substantia Nigra

    A brain region involved in movement and reward.

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    Indirect pathway

    A neural pathway that helps inhibit movement to balance motor control.

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    Glutamate

    A major excitatory neurotransmitter in the brain.

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    Amyotrophic Lateral Sclerosis (ALS)

    A progressive neurodegenerative disease affecting motor neurons.

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    Pharmacologic management in ALS

    Use of medications to manage symptoms and slow disease progression.

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    Dopaminergic pathways

    Neural pathways that use dopamine to transmit signals, affecting movement.

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    Mitochondrial damage

    Cellular damage impacting energy production, linked to neurodegeneration.

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    Neuronal survival

    The ability of neurons to live and function despite injury or disease.

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    Survival benefit in ALS

    Interventions shown to improve survival rates in ALS patients.

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    Pharmacologic management in Parkinson's

    Medication strategies used to treat symptoms of Parkinson's disease.

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    Seizures

    Sudden and uncontrolled electrical disturbances in the brain.

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    Status Epilepticus

    A medical emergency characterized by prolonged seizures or repeated seizures.

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    Isoniazid

    An antibiotic that can induce seizures by lowering GABA levels.

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    Pyridoxine deficiency

    A lack of vitamin B6, can lead to seizures and neurological issues.

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    Glutamate toxicity

    Excessive glutamate can cause neuronal hyperexcitation and seizures.

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    Antipsychotics and seizures

    Certain antipsychotic medications can lower seizure thresholds.

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    Clozapine

    An atypical antipsychotic associated with decreased white blood cells and seizure risk.

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    Subthalamic Nuclei

    A component of the basal ganglia involved in movement regulation, affected in Parkinson's.

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    Study Notes

    Patho Exam #3: Neurodegenerative Diseases

    • Neurodegenerative disorders involve a loss of functional neurons, often multifactorial.
    • Basal ganglia are subcortical areas responsible for integrating and fine-tuning nerve fibers for movement. They don't directly cause movement.

    Basal Ganglia Pathways

    • Indirect Pathway (inhibition): Dopamine binding to D2 receptors inhibits the striatum; this decreases GABA release onto the Globus Pallidus External (GPe). Less inhibited GPe releases more GABA onto the Subthalamic Nuclei, decreasing glutamate release onto the Globus Pallidus Internal (GPi). Reduced glutamate to GPi means less GABA release onto the thalamus, increasing thalamic activity and resulting in decreased motor coordination.
    • Indirect Pathway (without dopamine): Striatum releases GABA to the GPe, decreasing GPe-mediated GABA release onto subthalamic nuclei. Less GABA means increased glutamate release from the Subthalamic Nuclei to the GPi, increasing GABA release onto the thalamus, leading to decreased motor coordination.
    • Direct Pathway (with dopamine): Dopamine binding to D1 receptors in the striatum increases GABA release onto the GPi. This decreases GABA release from the GPi to the thalamus, resulting in increased thalamic activity and fluidity of movement.

    Parkinson's Disease

    • Characterized by Bradykinesia (slow movements), tremors (initially with voluntary movement, later at rest), gait abnormalities (shuffling gait), and late-stage cognitive difficulties.
    • Lesions are in pars compacta (most highly affected).
    • Management:
      • Levodopa + Carbidopa (dopamine precursor, peripheral inhibitor).
      • Dopaminergic agonists (pramipexole, ropinirole, bromocriptine).
      • MAO-B inhibitors (selegiline, rasagiline).

    Huntington's Disease

    • Mutation in CAG trinucleotide repeats on chromosome 4, leading to Huntington protein accumulation.
    • Early stages: Hyperkinetic phenotypes (chorea, athetosis).
    • Late stages: Bradykinesia, rigidity, tremors, similar to Parkinson's. Non-motor symptoms:
      • Visuospatial deficits/behavioral issues due to specific striatal neuron projections.
      • Neuropsychiatric deficits/dementia.
    • Subcortical and striatal degeneration leads to disease progression(mainly in striatum).

    Amyotrophic Lateral Sclerosis (ALS)

    • Glutamate toxicity damages mitochondria, leading to neuronal death.
    • Symptoms:
      • Upper motor neuron symptoms: Hyperreflexia, spasticity, uncontrollable laughing/crying.
      • Lower motor neuron symptoms: Muscle atrophy, muscle twitches, weakness, loss of reflexes, speech issues, facial/tongue atrophy.
    • Management:
      • Riluzole (blocks glutamate transmission).
      • Edaravone (free radical scavenger, adjunct).

    Seizures

    • Focal seizures: Abnormal hyperexcitability in one area, may or may not spread to other areas, may or may not have loss of awareness.
    • Generalized seizures: Involve both hemispheres, loss of consciousness, post-ictal state.
    • Infectious causes: Meningitis (Streptococcus pneumoniae, Neisseria meningitidis, Cryptococcal Meningitis), encephalitis (Borrelia burgdorferi, herpesviruses, mosquito-borne infections), demyelination.
    • Pathophysiology: Excessive glutamate activation, decreased GABA inhibition. Abnormal neuronal firing synchronization.
    • Management:
      • IV benzodiazepines (Ativan, Midazolam, Diazepam)
      • Long-acting anticonvulsants (Phenytoin, Valproic Acid, Levetiracetam).
      • Phenobarbital or similar barbiturates (if needed,Phenytoin not available).
      • General anesthesia (propofol)

    Seizure-Promoting Medications

    • Bupropion (sodium blockade, increased excitability).
    • Flumazenil (benzodiazepine reversal agent, GABA antagonist).
    • Isoniazid (pyridoxine deficiency, decreased GABA production, increased glutamate toxicity).
    • Clozapine (increased glutamatergic transmission, possible granulocytopenia).
    • Illicit drugs (increased glutamate release).

    Withdrawal Syndromes (Seizures)

    • Benzodiazepine withdrawal: CNS hyper-excitability.
    • Alcohol withdrawal: Decreased GABA transmission, increased glutamatergic activity. Delirium Tremens (severe) symptoms include agitation, hallucinations, autonomic instability.
    • Management: IV Lorazepam, long-acting benzodiazepines, general anesthesia (severe cases).

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    Patho Exam #3 PDF

    Description

    This quiz covers essential concepts related to neurodegenerative diseases, focusing on the pathways of the basal ganglia. Understand the roles of various neurotransmitters and how they affect motor coordination and neuronal function. Prepare to test your knowledge on the implications of these pathways and their significance in neurodegeneration.

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