24 Questions
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)
What is the pathological hallmark of Parkinson's Disease?
Degeneration and loss of nigrostriatal dopaminergic innervation
What are the neuronal inclusions in Parkinson's Disease?
Containing α-synuclein
What protein is abundant in dopamine-producing nerve cells?
Alpha-synuclein
What are the clumps called that form when alpha-synuclein misfolds and aggregates in Parkinson's disease?
Lewy Bodies
What is the main pathological process in Parkinson's disease related to alpha-synuclein?
Misfolding and aggregation into Lewy Bodies
What are the typical clinical symptoms of Parkinson's disease?
Gradual slowness of movement, loss of postural reflexes, poor balance, and motor coordination
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
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
What is the main pathological feature seen in Parkinson's disease (PD)?
Insoluble cytoplasmic protein aggregates (Lewy Bodies)
What is the genetic basis of Huntington Disease (HD)?
Autosomal dominant trinucleotide CAG repeat in the huntingtin gene (HTT) on chromosome 4
What is the average life expectancy after diagnosis in Huntington Disease (HD)?
15 years
What type of involuntary movements are characteristic of Huntington Disease (HD)?
Jerky movements (Chorea)
What are the clinical features of the presymptomatic phase in Huntington Disease (HD)?
Neuropsychiatric - irritability, disinhibition
What are the hyperkinetic phenotypes observed in Huntington Disease (HD)?
Prominent chorea and dystonia
What is the chronic, progressive neurologic disease characterized by degeneration of upper and lower motor neurons?
Amyotrophic lateral sclerosis (ALS)
What gene mutation is associated with ALS?
Superoxide dismutase gene (SOD1)
What is the clinical feature of ALS characterized by brisk tendon reflexes and spasticity?
Upper motor neuron specific signs and symptoms
What are the specific signs and symptoms associated with lower motor neurons in ALS?
Skeletal muscle weakness, wasting, and fasciculations
What is the neuropsychiatric condition resulting from thiamine (vitamin B1) deficiency?
Wernicke encephalopathy (WE)
What are the clinical features of Korsakoff syndrome (KS) associated with thiamine deficiency?
Profound anterograde amnesia and temporally graded retrograde amnesia with confabulation
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.
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