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Neurodegenerative Diseases Overview

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24 Questions

What specific group of neurons in the central nervous system is responsible for fine-tuning an organism's movements?

Neurons in the substantia nigra

Which neurotransmitter is primarily involved in the interaction between neurons of the substantia nigra and the basal ganglia?

Dopamine

What are the cardinal motor symptoms associated with Parkinson's disease?

Tremor, rigidity, and bradykinesia

In the context of neurodegenerative diseases, what is one of the pathological features of Parkinson's disease?

Neuronal inclusions containing α-synuclein

After Alzheimer's disease, which disorder is considered the second most common neurodegenerative disease?

Parkinson's disease

Which area of the brain is affected by degeneration and loss of nigrostriatal dopaminergic innervation in Parkinson's disease?

Substantia nigra

Which neurodegenerative disease is characterized by the degeneration of striatal neurons and the presence of involuntary jerky movements called chorea?

Huntington Disease

What is the autosomal dominant trinucleotide repeat found in the gene responsible for Huntington Disease?

CTG

Which phase of Huntington Disease progression is characterized by prominent chorea and dystonia as clinical features?

Hyperkinetic phenotype

What type of protein aggregates are potentially injurious in Huntington Disease?

Mutant huntingtin protein aggregates

Which area of the brain is affected by degeneration in Huntington Disease?

Striatum (caudate and putamen)

What is a common cognitive dysfunction seen in individuals with Huntington Disease?

Poor executive function

What is the main reason for the mis-folding and aggregation of alpha-synuclein into Lewy Bodies in Parkinson's disease?

Defects in autophagy and lysosomal degradation

What is the typical clinical presentation seen in patients with Parkinson's disease?

Tremor, rigidity, and bradykinesia

What is the usual cause of death in patients with Parkinson's disease?

Aspiration pneumonia or trauma from falls

What microscopic feature can be observed in the substantia nigra area of a healthy brain?

Melanin-pigmented substantia nigra neurons

What is the consequence of the loss of substantia nigra neurons in Parkinson's disease?

Gradual slowness of spontaneous movement

What is the main consequence of alpha-synuclein aggregation on neurotransmission in Parkinson's disease?

Reduced availability of dopamine for neurotransmission

Which gene mutation is associated with ALS pathogenesis?

SOD1

What is the main cause of death in ALS patients?

Respiratory failure

Which cells are affected in ALS that lead to muscle weakness and wasting?

Lower motor neurons

What specific symptom is characteristic of Wernicke encephalopathy?

Confabulation

Which region of the spinal cord is primarily affected in subacute combined degeneration?

Posterior and lateral columns

What is the main mechanism causing Wernicke encephalopathy?

Thiamine (vitamin B1) deficiency

Explore the characteristics and pathogenesis of neurodegenerative diseases like Parkinson's Disease and Huntington's Disease. Understand the loss of neurons, presence of protein aggregates, and progressive movement disorders associated with these conditions.

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