Neurodegenerative Diseases Comparison Quiz

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Questions and Answers

What is the role of neurons in the substantia nigra in movement control?

Communicate with neurons of the basal ganglia by liberating dopamine

What neurotransmitter is responsible for the interaction between neurons of the substantia nigra and the basal ganglia?

Dopamine (DA)

What are the characteristic symptoms of Parkinson's Disease (PD)?

Tremor, rigidity, bradykinesia, postural instability

Which neurodegenerative disease is the second most common after Alzheimer's disease?

Parkinson's Disease (PD) Signup and view all the answers

What is the pathological hallmark of Parkinson's Disease?

Degeneration and loss of nigrostriatal dopaminergic innervation Signup and view all the answers

What are the neuronal inclusions in Parkinson's Disease?

Containing α-synuclein Signup and view all the answers

What protein is abundant in dopamine-producing nerve cells?

Alpha-synuclein Signup and view all the answers

What are the clumps called that form when alpha-synuclein misfolds and aggregates in Parkinson's disease?

Lewy Bodies Signup and view all the answers

What is the main pathological process in Parkinson's disease related to alpha-synuclein?

Misfolding and aggregation into Lewy Bodies Signup and view all the answers

What are the typical clinical symptoms of Parkinson's disease?

Gradual slowness of movement, loss of postural reflexes, poor balance, and motor coordination Signup and view all the answers

How do the symptoms of Parkinson's disease usually progress over time?

Over 10 to 15 years, eventually leading to severe motor slowing and near immobility Signup and view all the answers

What are the typical gross features seen in the pathological examination of a healthy brain compared to a brain affected by Parkinson's disease?

Pigmented dopamine-producing neurons in the substantia nigra in a healthy brain, loss of these neurons in Parkinson's disease Signup and view all the answers

What is the main pathological feature seen in Parkinson's disease (PD)?

Insoluble cytoplasmic protein aggregates (Lewy Bodies) Signup and view all the answers

What is the genetic basis of Huntington Disease (HD)?

Autosomal dominant trinucleotide CAG repeat in the huntingtin gene (HTT) on chromosome 4 Signup and view all the answers

What is the average life expectancy after diagnosis in Huntington Disease (HD)?

15 years Signup and view all the answers

What type of involuntary movements are characteristic of Huntington Disease (HD)?

Jerky movements (Chorea) Signup and view all the answers

What are the clinical features of the presymptomatic phase in Huntington Disease (HD)?

Neuropsychiatric - irritability, disinhibition Signup and view all the answers

What are the hyperkinetic phenotypes observed in Huntington Disease (HD)?

Prominent chorea and dystonia Signup and view all the answers

What is the chronic, progressive neurologic disease characterized by degeneration of upper and lower motor neurons?

Amyotrophic lateral sclerosis (ALS) Signup and view all the answers

What gene mutation is associated with ALS?

Superoxide dismutase gene (SOD1) Signup and view all the answers

What is the clinical feature of ALS characterized by brisk tendon reflexes and spasticity?

Upper motor neuron specific signs and symptoms Signup and view all the answers

What are the specific signs and symptoms associated with lower motor neurons in ALS?

Skeletal muscle weakness, wasting, and fasciculations Signup and view all the answers

What is the neuropsychiatric condition resulting from thiamine (vitamin B1) deficiency?

Wernicke encephalopathy (WE) Signup and view all the answers

What are the clinical features of Korsakoff syndrome (KS) associated with thiamine deficiency?

Profound anterograde amnesia and temporally graded retrograde amnesia with confabulation Signup and view all the answers

Study Notes

Neurons in the Substantia Nigra and Movement Control

Neurons in the substantia nigra play a critical role in regulating movement and coordination.
They modulate the activity of the basal ganglia, which is essential for smooth and controlled movement.

Neurotransmitters in the Substantia Nigra

Dopamine is the primary neurotransmitter involved in the interactions between substantia nigra neurons and basal ganglia.

Parkinson's Disease Symptoms

Characteristic symptoms of Parkinson’s Disease (PD) include tremors, bradykinesia (slowness of movement), rigidity, and postural instability.
PD also presents with non-motor symptoms such as depression and cognitive changes.

Prevalence of Neurodegenerative Diseases

Parkinson's Disease is the second most common neurodegenerative disease after Alzheimer’s Disease.

Pathological Hallmarks of Parkinson's Disease

The main pathological hallmark of PD is the degeneration of dopamine-producing neurons in the substantia nigra.
Neuronal inclusions known as Lewy bodies are a defining feature of PD.

Alpha-Synuclein in Parkinson's Disease

Alpha-synuclein is a protein that is abundant in dopamine-producing neurons.
It misfolds and aggregates, forming clumps called Lewy bodies in the brains of those with Parkinson's.

Main Pathological Process in PD

The misfolding and accumulation of alpha-synuclein constitutes the main pathological process in Parkinson's Disease.

Clinical Symptoms and Progression of PD

Typical clinical symptoms of PD include resting tremors, stiffness, and balance issues.
Symptoms generally progress over time, often worsening as the disease advances.

Pathological Examination of the Brain

Gross features observed in pathological examination reveal significant loss of dopaminergic neurons in the substantia nigra in PD-affected brains compared to healthy brains.

Genetic Basis of Huntington Disease

Huntington Disease (HD) is caused by a genetic mutation in the HTT gene, leading to abnormal expansions of CAG repeats.

Life Expectancy in Huntington Disease

The average life expectancy after diagnosis of Huntington Disease is approximately 15 to 20 years.

Involuntary Movements in Huntington Disease

Characteristic involuntary movements associated with Huntington Disease include chorea, which features irregular and unpredictable movements.

Clinical Features in Presymptomatic Phase of HD

The presymptomatic phase of HD may include subtle mood changes, cognitive decline, and difficulties in tasks involving coordination.

Hyperkinetic Phenotypes in Huntington Disease

Hyperkinetic phenotypes observed in HD include chorea, dystonia, and other abnormal motor behaviors.

Amyotrophic Lateral Sclerosis (ALS)

ALS is a chronic, progressive neurologic disease marked by the degeneration of both upper and lower motor neurons.

Gene Mutation in ALS

A mutation in the SOD1 gene is commonly associated with familial forms of Amyotrophic Lateral Sclerosis.

Clinical Features of ALS

In ALS, brisk tendon reflexes and spasticity are specific clinical features representing upper motor neuron involvement.
Signs associated with lower motor neurons in ALS include muscle weakness, atrophy, and fasciculations.

Korsakoff Syndrome

Korsakoff Syndrome is a neuropsychiatric condition arising from a deficiency in thiamine (vitamin B1).
Clinical features include severe memory impairment, confabulation, and disorientation.

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Description

Test your knowledge on Parkinson's Disease and Huntington Disease, two neurodegenerative disorders characterized by the loss of specific neurons and progressive movement disorders. Understand the differences in symptoms, pathology, and progression of these conditions.