Neurodegenrative Movement Disorders Flashcards
A 68-year-old man presents with progressive difficulty walking and a resting tremor. Neurological examination reveals cogwheel rigidity and bradykinesia. MRI of the brain shows loss of pigmentation in the midbrain. Which of the following structures is most likely affected?
A) Caudate nucleus
B) Putamen
C) Substantia nigra pars compacta
D) Subthalamic nucleus
E) Globus pallidus interna
C
Why designs ration occur in parkinsons D ?
Depigmentation occurs due to the loss of neuromelanin, a pigment found in dopaminergic neurons.
A 65-year-old man presents with a tremor that occurs when his hands are resting on his lap but disappears when he reaches for objects. What is the most likely diagnosis?
A) Essential tremor
B) Parkinson disease
C) Cerebellar tremor
D) Wilson disease
E) Intention tremor
B
The tremor in Parkinson disease occurs at rest and improves with movement.
It is described as a “pill-rolling” tremor because patients appear to be rolling a pill between their fingers.
A 72-year-old woman presents with progressive stiffness in her limbs. On physical exam, passive movement of her arms reveals resistance that occurs in a ratchet-like fashion. What is this finding called?
A) Clasp-knife rigidity
B) Cogwheel rigidity
C) Flaccidity
D) Fasciculations
E) Myoclonus
B
Rigidity (Cogwheel or Lead-Pipe)
Line: “Rigidity (cogwheel or leadpipe).”
Explanation:
Cogwheel rigidity: Resistance to passive movement, interrupted by small, jerky movements.
Lead-pipe rigidity: Continuous resistance throughout the range of motion.
Due to increased muscle tone from lack of dopamine-mediated inhibition in the basal ganglia.
A 70-year-old man has difficulty initiating movement and a progressively smaller handwriting pattern. He also walks with a slow, shuffling gait. Which neurotransmitter is deficient in the affected brain region?
A) Acetylcholine
B) GABA
C) Dopamine
D) Glutamate
E) Serotonin
C
Bradykinesia: Slowness of movement.
Akinesia: Difficulty initiating movement.
Micrographia: Handwriting becomes progressively smaller.
Shuffling gait: Patients take small, slow steps with decreased arm swing.
A 73-year-old man with Parkinson disease is at increased risk of falls. Which of the following tests is most appropriate to assess his postural stability?
A) Romberg test
B) Dix-Hallpike maneuver
C) Pull test
D) Heel-to-toe walking test
E) Finger-nose test
C
A brain autopsy of a 75-year-old man with Parkinson disease shows eosinophilic cytoplasmic inclusions in the neurons of the substantia nigra. These inclusions are primarily composed of which protein?
A) Tau
B) β-amyloid
C) α-synuclein
D) Ubiquitin
E) TDP-43
C
A 68-year-old man presents with parkinsonian symptoms but also has difficulty looking up. What is the likely diagnosis?
A) Parkinson disease
B) Huntington disease
C) Progressive supranuclear palsy
D) Multiple system atrophy
E) Lewy body dementia
C
PSP has Parkinson-like symptoms but with vertical gaze palsy (difficulty looking up or down).
Cognitive dysfunction is more severe than in Parkinson disease.
Which type of neurons are primarily lost in Huntington disease?
A) Dopaminergic
B) Serotonergic
C) GABAergic
D) Cholinergic
E) Glutamatergic
C
A 40-year-old man develops involuntary movements and psychiatric symptoms. Genetic testing reveals a CAG repeat expansion. What is the inheritance pattern of this disease?
A) Autosomal recessive
B) Autosomal dominant
C) X-linked recessive
D) X-linked dominant
E) Mitochondrial
B
A 36-year-old man with a family history of a neurodegenerative disorder undergoes genetic testing, revealing a trinucleotide repeat expansion disorder on chromosome 4. Which of the following mechanisms is responsible for neuronal degeneration in this condition?
(A) Excitotoxicity due to NMDA receptor overstimulation
(B) Loss of function mutation leading to apoptosis
(C) Protein misfolding and prion-like spread
(D) Impaired dopamine synthesis
A
A 38-year-old man presents with mood changes, irritability, and involuntary jerky movements. His father experienced similar symptoms in his 40s. Which of the following is most likely seen on brain imaging?
(A) Atrophy of the caudate nucleus
(B) Enlargement of the substantia nigra
(C) Demyelination of the corpus callosum
(D) Cerebellar degeneration
A
A 45-year-old man with progressive involuntary movements and dementia undergoes MRI, which shows ventricular enlargement out of proportion to sulcal atrophy. What is the most likely cause of these findings?
(A) Hydrocephalus
(B) Neuronal loss in the caudate nucleus
(C) Cerebellar atrophy
(D) Hippocampal sclerosis
B
Which of the following neurotransmitter changes is expected in a patient with Huntington disease?
(A) ↑ ACh, ↓ Dopamine, ↑ GABA
(B) ↓ ACh, ↓ Dopamine, ↑ GABA
(C) ↑ Dopamine, ↓ GABA, ↓ ACh
(D) ↑ Dopamine, ↑ ACh, ↓ GABA
C
What is the primary mechanism of neuronal death in Huntington disease?
(A) β-amyloid plaque deposition
(B) Excitotoxicity due to glutamate overstimulation
(C) Impaired dopamine synthesis
(D) Autoimmune-mediated apoptosis
B
A young woman with a family history of Huntington disease undergoes genetic testing, which confirms a trinucleotide repeat expansion. What best explains her earlier disease onset compared to her father?
(A) Loss of heterozygosity
(B) Skewed X-inactivation
(C) Somatic mosaicism
(D) Anticipation
D
Which of the following symptoms most strongly differentiates PSP from Parkinson’s disease?
(A) Resting tremor
(B) Cogwheel rigidity
(C) Vertical gaze palsy
(D) Micrographia
C
A 72-year-old man presents with postural instability and difficulty moving his eyes up and down. MRI reveals midbrain atrophy with preservation of the pons, producing a “hummingbird sign.” What is the diagnosis?
(A) Parkinson disease
(B) Progressive supranuclear palsy
(C) Multiple system atrophy
(D) Alzheimer disease
B
