Parkinson disease, Huntington disease, and tremor disorders with their pathophysiology and distinguishing features tested on USMLE Step 1.
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- What brain structure degenerates in Parkinson disease?
- The substantia nigra pars compacta (dopaminergic neurons)
- What are the four cardinal (TRAP) features of Parkinson disease?
- Tremor (resting), Rigidity, Akinesia/bradykinesia, Postural instability
- Describe the classic tremor of Parkinson disease.
- A 4-6 Hz resting tremor, often described as pill-rolling, that improves with voluntary movement
- What type of rigidity is classically seen in Parkinson disease?
- Cogwheel rigidity
- What is bradykinesia?
- Slowness of voluntary movement initiation and execution
- What protein aggregates form Lewy bodies in Parkinson disease?
- Alpha-synuclein
- Describe the typical gait of a patient with Parkinson disease.
- A shuffling, festinating gait with stooped posture and reduced arm swing
- What term describes the reduced facial expression seen in Parkinson disease?
- Masked facies (hypomimia)
- What handwriting change is characteristic of Parkinson disease?
- Micrographia, progressively smaller handwriting
- What is the most effective first-line pharmacotherapy for Parkinson disease symptoms?
- Levodopa combined with carbidopa
- Why is carbidopa given together with levodopa?
- It inhibits peripheral DOPA decarboxylase, reducing peripheral dopamine conversion and side effects while increasing levodopa delivery to the brain
- What neurotoxin is used to model Parkinson disease and how does it cause damage?
- MPTP, which is converted by MAO-B into MPP+, a toxin that destroys dopaminergic neurons
- Name two dopamine agonists used to treat Parkinson disease.
- Pramipexole and ropinirole
- Name a MAO-B inhibitor used in Parkinson disease treatment.
- Selegiline
- What is the mechanism of amantadine in Parkinson disease?
- It increases dopamine release and blocks NMDA glutamate receptors