Movement Disorders

Question 1

How would Parkison’s Disease be defined

Answer 1

A chronic, idiopathic, progressive neurodegenerative movement disorder where symptoms continue to worsen over time

Question 2

Describe the etiology of Parkinson’s

Answer 2

• loss of dopaminergic neurons in the substantia nigra (excessive inhibition of the thalamus leads to reduced cortical motor activation)
• loss of melanin from the pars compacta
  +/- the presence of lewy bodies

Question 3

What are the cardinal signs of Parkinson’s

Answer 3

- resting tremor
- cogwheel rigidity
- bradykinesia
- postural instability

other syx include: intrinsic muscle issues, stiffness, voice fluctuations, instability, micrographia, stooped/flexed posture, propulsion/retropulsion

Question 4

How do symptoms of Parkinson’s typically start

Answer 4

unilateral tremor

Question 5

What are some characteristics of Parkinson’s that may be seen on physical exam

Answer 5

• pill rolling, resting tremor
• bradykinesia, gait impairment
• postural reflexes impaired
• ask about onset of symptoms and progression

Question 6

What is the difference between Parkinsonism and Parkinson’s Disease

Answer 6

Parkinsonism = 2 or 3/4 cardinal signs

Parkinson’s Disease = 4/4 cardinal signs

Question 7

What diagnostic testing may be done in Parkinson’s disease

Answer 7

• no specific biomarker
• Lewy bodies may be seen on MRI
• olfactory testing

Question 8

Describe the treatment of Parkinson’s Disease

Answer 8

• provide symptom relief
• levodopa-carbidopa
• Entacapone (COMT Inhibitors)
• MAO-B inhibitors
• Anticholinergics

Question 9

What are some hyperkinetic disorders

Answer 9

• essential tremor
• dystonia
• Huntington’s Disease
• Wilson’s Disease
• Tardive Dyskinesia
• Restless leg syndrome

Question 10

Etiology of essential tremor

Answer 10

• sporadic or familial (autosomal dominant)
• most common movement disorder
• onset childhood to elderly (usually 45)

Question 11

What is the clinical presentation of essential tremor

Answer 11

-postural tremor with/without kinetic component
- tremor only
- bilateral and symmetric, usually upper extremity
- normal muscle tone

Question 12

What may improve essential tremor symptoms

Answer 12

alcohol, only for 30-60 mins

Question 13

What is the major differential for essential tremor

Answer 13

Wilson’s disease

Question 14

What is the first line treatment for essential tremor

Answer 14

beta blockers: propranolol

refer to neuro if contraindicated for benzos

Question 15

Define tremor

Answer 15

involuntary rhythmic oscillating movement of a body part secondary to intermittent muscle contraction

Question 16

Describe the handwriting effects of essential tremor

Answer 16

gets bigger, shaky, no resting tremor

Question 17

Describe the pathophys of dystonia

Answer 17

primarily unknown
- basal ganglion dysfunction
- excitability of motor cortex

Question 18

Describe the 3 types of dystonia

Answer 18

• primary
• focal (MC)
• secondary (drugs, neuro disorders)

Question 19

Describe the major types of focal dystonia

Answer 19

• blepharospasm
• oromandibular dystonia
• spasmodic dysphonia
• cervical dystonia
• limb dystonias

Question 20

describe the clinical presentation of dystonia

Answer 20

worse with emotional/physical stress or fatigue, decreases with sleep and rest

Question 21

Describe the diagnostic process for dystonia

Answer 21

• rule out parkinson’s and Wilson’s

Question 22

Describe the management of dystonia

Answer 22

• levodopa first line
• muscle relaxers, pumps, botox, baclofen, DBS

Question 23

Describe the etiology of Huntington’s

Answer 23

progressive neurodegenerative disorder of the CNS - atrophy of the caudate nucleus head, putamen, cerebral cortex
- autosomal dominant

Question 24

Is Parkinson’s direct or indirect pathway?

Answer 24

direct

Question 25

Huntington's - direct or indirect pathway?

Answer 25

indirect

Question 26

Tourette Syndrome - direct or indirect?

Answer 26

Indirect pathway

Question 27

Wilson's Disease - direct or indirect pathway

Answer 27

Direct pathway

Question 28

Describe the common symptoms of Huntington's

Answer 28

**- chorea - rigidity - psychiatric - progressive dementia - seizures**

Question 29

Diagnostics for Huntington's

Answer 29

- clinical - MRI, genetic testing

Question 30

treatment of huntingtons

Answer 30

symptom releif - anti-choreic (tetrabenazine - psychiatric treatment

Question 31

survival of huntingtons

Answer 31

10-30 years - death commonly due to pulmonary issues, sepsis

Question 32

Etiology of Wilson's disease

Answer 32

- **autosomal recessive** - dysfunction of **copper metabolism**

Question 33

Symptoms of Wilsons

Answer 33

- **copper rings in cornea** - speech disorder, extrapyramidal syx

Question 34

Diagnosis of wilson's

Answer 34

slit lamp (copper rings in cornea), ceruloplasmin levels

Question 35

treatment of wilson's

Answer 35

chelating agents to bind and remove copper

Question 36

Tardive dyskinesia etiology

Answer 36

commonly severe and secondary to antipsychotic drugs months to years

Question 37

symptoms of tardive dyskinesia

Answer 37

- extrapyramidal symptoms: restlessness, dystonia, parkinsonism, chorea

Question 38

etiology of restless leg syndrome

Answer 38

- limbs associated with sleep complaint - W>M

Question 39

symptoms of restless leg

Answer 39

involuntary forceful dorsiflexion of foot, sleep disturbance, fatigue, repetitive periodic jerking **pins and needles, internal itch, creeping crawling**

Question 40

diagnostics for restless leg

Answer 40

**- CBC with diff - iron panel** - BUN - Blood gluc - TSH - Vitamin B12 - folate

Question 41

treatment of restless leg

Answer 41

First line: dopaminergic agents (ropinirole) - clonidine - iron supplementation - education and sleep hygeine

Question 42

What condition is this

Answer 42

huntington's

Question 43

what might this disease be characterized by

Answer 43

tardive dyskinesia

Question 44

Which diseases of the basal ganglia affect the **direct pathway**

Answer 44

- parkinson's disease - Wilson's disease - Essential tremor - bradykinesia/akinesia

Question 45

Which diseases of the basal ganglia affect the **indirect pathway**

Answer 45

- Huntington's disease - Hemiballism - Tics - Dystonia - Myoclonus - Tardive dyskinesia