Movement Disorders

Question 1

1. What are movement disorders caused by? 2

2. What modulates motor cortical activity but also the activity of the association cortex, in the frontal lobes?

Answer 1

1. • extrapyramidal (basal ganglia)
   • cerebellar dysfunction.
2. Basal ganglia

Question 2

Huntington’s Disease

1. How is it acquired?
2. What does it affect? 3
3. Mean onset is at what age?
4. What is the mean duration of illness?

Answer 2

1. Progressive autosomal dominant
2. Affects
   - motor function
   - cognition
   - behavior
3. Mean onset is age 40
4. Mean duration of illness is 20 years

Question 3

1. Huntington’s Disease Pathophysiology?

2. Where is the disease at? 2

Answer 3

1. • brain cells are wasting away/localized cell death
   • Decrease of GABA and GABA receptors in the basal ganglia
2. cauda nucleus/putemen of basal ganglia

Question 4

What is Huntingtons disease characterized by?

3

Answer 4

1. Chronic progressive chorea
2. Psychological changes
3. Dementia

Question 5

Decrease of GABA and GABA receptors in the basal ganglia is part of the pathophysiology of Huntington’s. What does this cause and why?

Answer 5

GABA is an inhibitory neurotransmitter. So without the GABA theres nothing to stop movement = chorea.

Question 6

Huntington’s adult onset clinical presentations? 6

Juvenile onset? 4

Answer 6

1. Chorea affects the limbs and trunk
2. Dystonia- twisting and loss of movement
3. Rigidity
4. Postural instability
5. Myoclonus
6. Nystagmus
7. Very rare
8. Bradykinesia
9. Rigidity
10. Quicker progression

Question 7

Early psychological manifestations of Huntington’s Disease?

10

Answer 7

1. Depression
Personality changes
2. Memory loss
3. Impulsive behavior- too much 4. dopamine
5. Moodiness
6. Antisocial behavior
7. Emotional outbursts
8. Lack of initiative
9. Loss of spontaneity
10. Inability to concentrate

Question 8

Early physical signs of Huntingtons? 2

Lateral physical signs of Huntingtons? 5

Answer 8

Early physical signs:

1. Fidgeting
2. Restlessness

Later physical signs:

1. Chorea
2. Dystonic posturing
3. Progressive rigidity
4. Akinesia
5. Dementia

Question 9

Diagnostic Studies for Huntingtons?

Answer 9

1. MRI
2. PET
3. Genetic testing

Question 10

Huntington’s Disease
What would the following show?
1. MRI?
2. PET?

Answer 10

MRI
1. Caudate atrophy

PET
2. Abnormal metabolic changes in the caudate

Question 11

Huntington’s Treatment?

3

Answer 11

Treatment of symptoms only, no medication will change the course of Huntington’s

1. Tetrabenazine (VAMT Inhibitor)
2. Antidopaminergic agents (Phenothiazine, Haloperidol)
3. Antipsychotics (Resperidone, Olanzapine)

Question 12

What is tetrabenazine used to treat in parkinson’s?

Answer 12

dyskinesia

Question 13

1. In Huntingtons what are we mostly treating?

2. What other things are we treating?

Answer 13

1. Chorea
2. • Postural instability
   • Rigidity worsen
   • Treatment of depression
   • Psychosis

Question 14

1. What are the anticonvulsants used for Huntington’s? 2
2. What are the antipsychotics used for Huntington’s? 2
3. What are the antidepressants used for Huntington’s? 3

Answer 14

1. • Clonazepam (Klonopin)
   • Valproic Acid (Depakote)
2. • Resperidone (Resperdal)
   • Olanzapine (Zyprexa)
3. • Fluoxetine (Prozac)
   • Sertraline (Zoloft)
   • Nortriptyline (Aventyl, Pamelor)- Tricyclic antidepressant

Question 15

What is the risk of TCAs?

2

Answer 15

suicide risk and also really dangerous in a overdose.

Question 16

Side effects from many of the drugs used to treat the symptoms of Huntington’s disease may include what?
3

Answer 16

1. hyper-excitability,
2. fatigue
3. restlessness.

Question 17

Antipsychotic drugs may cause side effects that mimic the signs of what disease?

Answer 17

Parkinson’s disease, including involuntary twitching in the face and body (tardive dyskinesia).

Question 18

What is the most common cause of tremors?

Answer 18

Essential tremors

Question 19

Essential Tremors

1. How is it acquired?
2. What are the ranges of presentation?
3. Unilateral or Bilateral?
4. More prominent with action or with rest?
5. Describe the frequency of the tremor?

Answer 19

1. Inherited
2. Ranges from cosmetic to disabling
3. Affect both sides of the body symmetrically**
4. More prominent with action than rest**
5. Frequency of tremor is constant***

Question 20

1. Essential Tremor affects what parts of the body? 3
2. Tremor (increased amplitude) aggravated by? 3
3. What relieves essential tumor?
4. Describe the onset compared to parkinson’s?

Answer 20

1. • Neck and head muscles
   • Muscles of the voice
   • Muscles of the arms and hands
2. • Stress
   • Sleep deprivation
   • Stimulants
3. ETOH
4. Usually starts earlier than parkinson’s

Question 21

Treatment for essential tumor? 3

Treatment for essential tumor for pts with asthma or bronchospasm? 1

Answer 21

1. Propranolol (Inderol) – Beta Blocker
2. Mysoline (Primidone) – Anticonvulsant
3. Gabapentin (Neurontin) - Anticonvulsant
4. Atenolol (Tenormin) – Beta Blocker of choice for those with asthma or bronchospasm

Question 22

Parkinson’s Disease
A progressive neurodegenerative disorder characterized by? 4

TRAP

Answer 22

1. Resting and postural Tremor – T
2. Rigidity – R
3. Akinesia (Bradykinesia) – A
4. Postural instability – P

Question 23

Describe the Pathophysiology of Parkinson’s disease?

Answer 23

1. Marked loss of dopaminergic nerve terminals in the substantia nigra***
   - -Relative increase in cholinergic interneuron activity occurs due to degeneration of dopamine pathways, which contributes especially to tremor.

Question 24

Possible causes and risk factors?

7

Answer 24

1. Primary cause is unknown
2. Genetics – 2004- Parkin gene found with early-onset Parkinson’s.
3. Defective alpha-syneclein and Lewy bodies
4. Complex l and oxygen free radicals**
5. Immune factors
6. Viral infections/Environmental exposures
7. Aging

Question 25

Cardinal Manifestations of Parkinson's?

Answer 25

Tremor | Rigidity

Question 26

Parkinson's Tremor? 1. Affects what part of the limbs? 2. What sign do you see often? 3. Unilateral or Bilateral? 4. Appears at rest or with action? 5. Disappears with what? 2

Answer 26

1. Affects distal segment of the limbs 2. “Pill rolling” 3. Usually unilateral 4. Appears at rest 5. Disappears with movement and sleep

Question 27

Cardinal Manifestations: Rigidity. How does this manifest? 4

Answer 27

1. Resistance to movement 2. Cog-wheel movements 3. Ratchet-like movements 4. Starts unilateral, progresses to involve both sides of the body

Question 28

Cardinal manifestation: Akinesia. How does this manifest? 5

Answer 28

1. Most debilitating symptom 2. Slowness in initiating and performing movements 3. Difficulty in sudden, unexpected stopping of voluntary muscles 4. Difficulty turning 5. Feel frozen in place, especially when moving through a doorway or preparing to turn

Question 29

Cardinal Manifestations: Postural Instability How does this manifest? 3

Answer 29

1. Lean forward to maintain center of gravity 2. Take small “shuffling” steps without swinging the arms 3. Prone to fall, especially backwards

Question 30

Parkinson’s Disease Other symptoms? 15

Answer 30

1. Emotional and voluntary facial movements become limited and slow leading to a “mask like” facies. 2. Loss of blinking reflex 4. Tongue, palate and throat muscles become rigid l/t drooling 5. Uncontrolled sweating 6. Sebaceous gland secretion 7. Seborrhea 8. Micrographia 9. Hypophonia 10. Depression 11. Orthostatic hypotension 12. Constipation 13. Impotence 14. Urinary incontinence 15. Dementia

Question 31

Parkinsons Diagnosis: 1. The practical diagnosis of PD during life is based on what? 2. What things will not show signs of parkinsons disease? 3. What is the gold standard of diagnosis? 4. What is the generally accepted way to diagnosis PD? 3

Answer 31

1. clinical impression 2. No physiologic tests or blood tests will confirm Dx Neurodiagnostic testing with computerized imaging is almost always unrevealing. 3. The true "gold standard" for diagnosis is neuropathologic examination. 4. It is generally accepted that two out of the three cardinal manifestations 1. tremor, 2. bradykinesia, 3. rigidity must be present in order to make the diagnosis of idiopathic PD.

Question 32

Rating scale: UPDRS (Unified Parkinson’s Disease Rating Scale) Rating scale: UPDRS (Unified Parkinson’s Disease Rating Scale). Describe each step on the scale? I-IV

Answer 32

I. Mentation/Behavior/Mood ll. Activities of Daily Living (Subjective) lll. Motor Examination (Objective) lV. Complications of Therapy

Question 33

Parkinson’s Disease Treatment? 6

Answer 33

1. Levadopa/Carbidopa 2. MAO-B Inhibitors 3. Dopamine Agonists 4. COMT inhibitors 5. Amantadine 6. Acetylcholine-blocking agents

Question 34

Treating complications: 1. Depression? 1 2. Hallucinations? 2 3. Orthostatic hypotension? 2

Answer 34

1. Depression - SSRI’s 2. Hallucinations - Decrease levadopa/dopamine agonists - Zyprexa (Olanzapine) 3. Orthostatic hypotension - TED hose - Slow rising

Question 35

Treating complications: 1. Sexual Disturbances? 1 2. Constipation? 3 3. Freezing/Difficulty Initiating? 2

Answer 35

1. Viagra/Dopamine agonist 2. - Stop offending medication - Bulking agents - NO REGLAN (why?)- dopamine resceptor antagonists. will make symtpoms worst 2. - Tape through a doorway - Mark on floor at turning point

Question 36

After we've maxed out medical therapy what is our treatment for PD?

Answer 36

Deep Brain Stimulation

Question 37

Deep Brain Stimulation 1. What does it do? 2. How often does this need to be replaced? 3. Who are our candidates for this? 4 4. What are the complications in this? 2

Answer 37

1. Uses electric pulse generators to control symptoms attached to STN or thalamus 2. Generators must be replaced every 5 years 3. Candidates are generally - under 70, - who have motor fluctuations, - drug induced dyskinesias, and - no other serious medical or psychiatric conditions. 4. Complications occur in 2-4% Bleeding in the brain and infection

Question 38

When, despite optimal pharmacotherapy, PD patient experiences troubling motor symptoms, such as: 4

Answer 38

1. Frequent motor fluctuations 2. Wearing off periods with severe bradykinesia, rigidity, tremor and/or freezing gait 3. Severe on-dyskinesia 4. Severe, treatment-resistant tremor

Question 39

A practice parameter from the American Academy of Neurology (AAN) issued in 2006 concluded that various physical therapy modalities are probably effective in improving functional outcome for patients with PD. These modalities include: 5

Answer 39

1. Multidisciplinary rehabilitation with standard physical and occupational therapy components 2. Treadmill training with body weight support 3. Balance training and high-intensity resistance training 4. Cued exercises with visual (mirror), auditory (metronome), and tactile feedback 5. Active music therapy

Question 40

``` 1. Restless Leg Syndrome (RLS) is also known as? 2. What is RLS? 3. What brings relief? 4. What is it brought on by? 5. When are symtpoms often worse? ```

Answer 40

1. (Wittmaack-Ekbom's Syndrome) 2. Uncontrollable urges to move limbs in order to stop uncomfortable, painful or odd sensation in the body, most commonly the legs 3. Movement brings brief relief 4. Brought on by inactivity involving sitting or lying 5. Sometimes symptoms are worse in the evening and early in the night

Question 41

Difficult for patient to describe unusual sensations, but some ways are the following? 7

Answer 41

1. Uncomfortable 2. Antsy 3. Electrical 4. Creeping 5. Painful 6. Itching 7. Pins and needles

Question 42

1. Describe the onset of RLS? | 2. Where is the cause probably located in the brain?

Answer 42

1. Onset is usually slow | 2. Something in the substania nigra

Question 43

What has RLS been associated with? | 9

Answer 43

1. pregnancy 2. obesity 3. smoking 4. iron deficiency 5. anemia 6. nerve disease 7. polyneuropathy (which can be associated with hypothyroidism, heavy metal toxicity, toxins, and many other conditions) 8. Diabetes 9. Kidney failure (which can be associated with vitamin and mineral deficiency).

Question 44

What medications might cause RLS? | 6

Answer 44

1. Anti-nausea 2. H2 blockers (Tagamet) 3. Antihistamines 4. TCA’s (Elavil) 5. SSRI’s 6. Antipsychotics

Question 45

What foods might cause RLS? | 2

Answer 45

1. Diet soda - -Aspartame 2. ETOH

Question 46

Tretament for RLS? | 10 (nonpharm)

Answer 46

1. Correct underlying cause i. e. Iron deficiency 2. Take pain relievers – OTC ibuprofen 3. Try baths and massages 4. Apply warm or cool packs, or alternating the two 5. Try relaxation techniques, such as meditation or yoga 6. Establish good sleep hygiene 7. Exercise 8. Avoid caffeine 9. Cut back on alcohol and tobacco. 10. Stay mentally alert in the evening.

Question 47

Pharm Treatment of RLS | Which kind drugs are often used?

Answer 47

Medications for Parkinson's disease

Question 48

Pharm Treatment of RLS drugs | 5

Answer 48

1. Pramipexole (Mirapex) 2. Ropinirole (Requip) 3. Carbidopa/levodopa (Sinemet) 4. Lyrica 5. Gabapentin (Neurontin)