Movement Disorders/Cerebellar Disorders and Ataxia Flashcards by Shirley Yancey

T/F: Parkinson’s Disease is a hypokinetic but also has action tremors and dyskinesia

False. Parkinson’s Disease is a hypokinetic but also has RESTING tremors and dyskinesia

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Most common movement disorder

Tremor

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Name the type of tremor:

tremor produced during voluntary contraction of muscle

Action

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Name the type of tremor:

tremor present while voluntarily maintaining a position against gravity

Postural

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Name the type of tremor:

tremor occurring during any voluntary movement

Kinetic

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Name the type of tremor:

tremor occurring during target directed movement

Intention. Subtype of kinetic

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Name the type of tremor:

tremor that occurs in body part that is not voluntarily activated and completely supported against gravity

Resting tremor

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Name the type of tremor:

tremor easily visible, mainly postural with NO underlying neurological disease

Enhanced physiologic

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Most common type of tremor

Essential Tremors (ET)

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T/F: Essential tremor is characterized by unilateral, mainly postural or kinetic tremor involving feet.

False

BIlateral, mainly SYMMETRIC postural or kinetic tremor involving HANDS and FOREARMS

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Pathophysiology of Essential Tremor

Impaired GABA-ergic inhibitory cerebellar output

Increased activity in cerebellothalamocortical circuit

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Medication used to treat essential tremor include ALL except:
A. Beta blocker
B. Anti seizure drugs
C. Muscle relaxants
D. Benzodiazepines

Beta blocker: propranolol
Anti-seizure drugs: primidone, gabapentin, topiramate
Benzodiazepines: clonazepam

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4 cardinal features of Parkinson’s Disease

Tremor at rest (asymmetric)
Bradykinesia
Rigidity
Postural instability

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BRAAK staging of Parkinson’s

Preclinical presents with ____ in ____

Lewy bodies
Olfactory bulb –> anosmia
Medulla, pons

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BRAAK staging of Parkinson’s

Clinical presents with ____ in ____

Lewy bodies
Midbrain (substantia nigra)
Basal forebrain

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BRAAK staging of Parkinson’s

Late disease presents with ____ in ____

Lew bodies

Neocortex

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T/F: Chorea is characteristic of involuntary, irregular, purposeless, asymmetric, non-rhythmic movements that gets better with activity and usually involves distal lower extremities

False. WORSEN with activity

distal UPPER extremities

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Name the type of Chorea:
• Involuntary writhing, dance like movements
• Onset after infection (Group A streptococcus)
• Problems concentration
• Impulsivity
• Resolves over time

Sydenham Chorea

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Which of the following is TRUE about the pathophysiology of Huntington Disease?
A. Increased GABA-ergic input to medial globus pallidus
B. Decreased inhibition of subthalamic nucleus
C. Increased tonic activity on lateral globus pallidus and substantia nigra pars reticularis
D. Increased glutaminergic stimulation of cerebral cortex

A. Decreased, lateral globus pallidus
B. Increased
C. Reduced, medial globus pallidus

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Psychiatric symptoms of Huntington’s Disease possibly due to pathology in ____

medial anterior caudate

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Treatment of Huntington's Disease include all of the following EXCEPT:
A. Haloperidol
B. Tetrabenazine
C. Propranolol
D. Sertraline
E. Buspirone
F. Levodopa

F. Levodopa used to treat Parkinsonism features of juvenile onset HD

A. dopamine-blocking drug
B. Dopamine depleting agent
C, D, E: for irritability/aggression

Dystonia results in disorder of ____

basal ganglia function

Dystonia is treated with:

Botox for focal or segmental
Anticholinergic: trihexiphenidyl, benztropine
Muscle relaxant
Deep brain stimulation of globus pallidus interna

Head or truncal oscillation (axial tremor) =

Titubation

Difficulty with target directed movement (finger to nose) (Overshoot or undershoot target) =

Dysmetria

Inability to coordinate single joint movements=

Dysrhythmia

Inability to coordinate multiple joint movements =

Dysdiadochokinesia

Decreased resistance to passive muscle stretch

Hypotonia

Pendular reflexes =

multiple oscillation

Inability to perform the Romberg test with eyes open suggests ___ dysfunction

cerebellar

Inability to perform the Romberg test with eyes closed suggests ___ dysfunction

dorsal column or peripheral nerve

Which of the following is FALSE about sensory ataxia? A. Impaired feedback of position sense to coordinate movements B. Romberg: unable to perform when eyes open C. Dorsal column or peripheral nerve dysfunction

B. when eyes CLOSED

Which of the following is FALSE about vestibular ataxia? A. Impaired sense of head movement B. Lack of orientation w/ respect to gravity C. Gait and balance difficulty D. Incoordination of limbs or speech

D. NO incoordination of limbs or speech

Name the type of cerebellar ataxia: o Impaired equilibrium = postural instability/titubation o Difficulty with eye movements o Romberg: unable to perform with eyes open

Vestibulocerellum | Flocculonodular lobe

Name the type of cerebellar ataxia: | Truncal ataxia: "drunken sailor gait"

Spinocerebellum | Vermis/midline structures

Name the type of cerebellar ataxia: Appendicular ataxia: Disruption of fine motor movements and limbs Dysarthria

Cerebrocerebellum | Lateral hemisphere

Name the disease: | Mutation of FXN gene → decreased frataxin protein production

Friedrich's Ataxia

Which of the following is FALSE about pathophysiology of Friedrich's Ataxia? A. PNS degneration of sensory fibers B. CNS degeneration of spinal cord and parietal cortex C. Ataxia due to proprioceptive loss

B. spinal cord and spinocerebellar tract

``` Which of the following is NOT a symptom of Friedrich's Ataxia? A. Scoliosis B. Lose of ability to walk C. Abnormal ocular movement D. Diabetes E. Cardiomyopathy F. Dysarthria G. Loss of proprioception in feet H. None of the above ```

Multiple System Atrophy shows ___ on MRI

atrophy of cerebellum and brainstem

Multiple System Atrophy is caused by degeneration of (3)

Cerebellum Pons Inferior olives

Which of the following is FALSE about Multiple System Atrophy? A. Weakness B. Autonomic dysfunctions (incontinence, orthostasis) C. Parkinsonism D. Dementia E. Cerebellar ataxia F. Visual impairment

``` Drug induced cerebellar syndrome includes all of the following EXCEPT: A. Transient gait ataxia B. Sensory ataxia C. Dysarthria D. Nystagus ```

Vitamin B12 deficiency causes what kind of ataxia?

o Dorsal column degeneration → Sensory ataxia | o Spinocerebellar tract degeneration → cerebellar ataxia

Acute vitamin B1 deficiency causes what kind of ataxia?

Gait ataxia | Wernicke-Korsakoff

Chronic vitamin B1 deficiency causes what kind of ataxia?

Truncal ataxia | Cerebellar cortical degeneration

Vitamin E deficiency causes ____ degeneration

spinocerebellar

anti-Yo =

Anti-Purkinge cell antibodies → loss of Purkinje cells and cerebellar atrophy --> Multiple System Atrophy In Ovarian/breast cancer

anti-Hu =

Anti-Purkinge cell antibodies → loss of Purkinje cells and cerebellar atrophy --> Multiple System Atrophy In small cell lung cancer

T/F: Gluten ataxia causes reversible cerebellum damage

False. Irreversible

T/F: Unilateral stroke causes contralateral symptoms

False. Ipsilateral