Movement Disorders (Thomsen)

Question 1

Name that tremor:

• bilateral, mainly symmetric postural or kinetic tremor involving hands and forearms
• strongly familial
• most common movement disorder

Answer 1

essential tremor

Question 2

Name that tremor:

caused by dysregulation of GABA-ergic inhibitory cerebellar output ► increased cerebellothalamocortical circuit

Answer 2

essential tremor

Question 3

Which of the following is NOT a medication/strategy approved to treat essential tremor?

A. Deep brain stimulation

B. Beta blockers: propanolol

C. Anti-seizure drugs: primidone

D. Anticholinergics: alprazolam

E. Benzodiazepines: clonazepam

Answer 3

D

Question 4

name that disease:

• resting asymmetric tremor with gradual onset
• progressive slowness and stiffness
• shuffling gait with occasional imbalance and falling
• soft speech and facial hypotonia

Answer 4

parkinson’s disease

Question 5

preclinical sign of parkinson’s

Answer 5

anosmia

Question 6

Which of the following statements about the pathophysiology of Parknson’s is FALSE?
A. Due to the progressive loss of dopaminergic neurons in the substantia nigra

B. Doopaminergic dysfunction leads to decreased activity in cerebellothalamocortical circuit

C. Lewy bodies composed of alpha-synuclein in neurons is required for pathologic confirmation of idiopathic PD

D. Underlying cause is likely a combo of genetics, environment and aging

Answer 6

B. Leads to increased activity.

Question 7

Which of the following statements about treatment of PD is false?

A. Treatment early may help alter the progression of PD

B. Medications aim to replace dopamine function

C. Levodopa can cross the BBB

D. MAO-B inhibitors (selegilline, rasagilline) prevent the breakdown of dopamine

E. Ropinirole, pramipexole, and rotigotine are all dopamine receptor agonists

Answer 7

A. Treatment is symptomatic only - cannot alter the progression

Question 8

anxiolytic medication used to treat REM sleep behavior disorder in patients with PD

Answer 8

clonazepam (benzo)

Question 9

medications that are used to treat dementia both in PD and AD patients

Answer 9

cholinesterase inhibitors: donepezil, rivastigmine, galntamine

and

NMDA receptor antagonist: memantine

Question 10

Which of the following is not descriptive of chorea:

A. involuntary, irregular, asymmetric movements

B. jerky and explosive in character

C. improves with activity

D. involves distal upper extremities

E. non-rhythmic contractions produce alternating “milkmaid” grip

Answer 10

C. Chorea worsens with activity.

Question 11

Which of the following statements about the epidemiology of PD is TRUE?

A. Typical age of onset is early 50s

B. Onset below 21 is most likely familial parkinson’s disease

C. people who lived in rural areas and drank well water are at higher risk of developing PD

D. Onset prior to age 40 is a red flag for Huntington’s disease.

Answer 11

C.

A - typical onset is early 60s

B- onset below 45 has stronger genetic component but below 21 is considered juvenile parkinsonism not PD

D - onset prior to 21 is red flag for HD

Question 12

12 year old boy develops invluntary writhing, dance like movements 3 weeks after onset of strep throat (Gropu A strep). He also has problems concentrating on schoolwork and some impulsivity. Diagnosis?

Answer 12

Sydenham’s chorea

Question 13

34 year old male presents with onset of writhing involuntary movements 7 years ago. He has difficulty with coordination and balance and has exhibited some choking and weight loss. He has history of impulsivity and poor decision making, and has a strong family history of similar symptoms. Diagnosis?

Answer 13

Huntington’s disease

Question 14

patients with <___ CAG repeats are typically asymptomatic for HD

Answer 14

35

Question 15

what major gross finding would you expect to find in a brain specimen of a person with HD?

Answer 15

atrophy of caudate and putamen

Question 16

Which of the following is NOT part of the pathophysiology of HD:

A. Decreased GABA-ergic input to lateral globus pallidus

B. Decreased inhibition of the subthalamic nucleus

C. Reudced tonic activity on the medial globus pallidus and substantia nigra pars reticularis

D. Reduced inhibitory output on the thalamic nuclei

E. Increased glutaminergic stimulation of the cerebral cortex

Answer 16

B. The subthalamic nuclei in its normal functioning state is an inhibitor of impulse control. In HD, You get increased inhibition of the subthalamic nucleus due to the activity of the globus pallidus upon it. Essentially, we’ve “inhibited the inhibitor” of impulse control, and now impulses can project unchecked to the cerbral cortex. See this diagram for a visual.

Question 17

dopamine-blocking drugs for treating HD

Answer 17

haloperidol, tetrabenazine

*L dopa for parkinsonian features of juvenile onset HD

Question 18

35 yo male with onset of twisting posture, painful cramping of neck and shaking of head. Family history of similar symptoms (writer’s cramp). Diagnosis?

Answer 18

dystonia

*may be focal (ie cervical dystonia, writer’s cramp, blepharospasm) or generalized

Question 19

treatment of dystonia?

Answer 19

muscle relaxants, botox, anticholinergics, deep brain stimulation of globus pallidus interna

*none are very effective