Neurologists Exam Neurologists Exam Neurodegenerative & Movement Disorders 3

Question 1

Which genetic mutation is most commonly associated with familial ALS in the United States?

Accepted Answer

C9orf72 hexanucleotide repeat expansion

Answer

SOD1 mutation

Answer

FUS mutation

Answer

TARDBP mutation

Question 2

Riluzole's mechanism of action in ALS involves:

Accepted Answer

Inhibition of glutamate release and blockade of sodium channels

Answer

Dopamine receptor agonism

Answer

Acetylcholinesterase inhibition

Answer

GABA-A potentiation

Question 3

Wilson's disease presents with neurological symptoms and hepatic disease due to accumulation of which substance?

Accepted Answer

Copper

Answer

Iron

Answer

Manganese

Answer

Lead

Question 4

Which dopamine agonist side effect is particularly important to screen for in Parkinson's disease patients?

Accepted Answer

Impulse control disorders (gambling, hypersexuality)

Answer

Tardive dyskinesia

Answer

Neuroleptic malignant syndrome

Answer

Serotonin syndrome

Question 5

The 'sunflower cataract' is a characteristic finding in which metabolic movement disorder?

Accepted Answer

Wilson's disease

Answer

Niemann-Pick disease

Answer

Gaucher disease

Answer

Krabbe disease

Question 6

Deep brain stimulation (DBS) of which target is most effective for tremor-predominant Parkinson's disease and essential tremor?

Accepted Answer

Ventral intermediate nucleus (VIM) of the thalamus

Answer

Subthalamic nucleus (STN)

Answer

Globus pallidus interna (GPi)

Answer

Pedunculopontine nucleus (PPN)