Lecture 7: Movement Disorders Flashcards

1
Q

What are the characteristics of hypokinetic movement disorders?

A

decreased or slow movement

loss of direct pathway in basal ganglia

loss of dopamine

Parkinsonism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the characteristics of hyperkinetic movement?

A

excessive movement

loss of direct pathway

loss of inhibition of thalamus

chorea, dystonia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the direct pathway of movement in the basal ganglia?

A

facilitates movement

fueled by dopamine (Parkinson’s)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the indirect pathway of movement in the basal ganglia?

A

inhibits movement

slows down movement (Huntington’s)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What other systems is the basal ganglia connected to through the caudate?

A

limbic
visual
oculomotor
motor cortex
frontal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are some characteristics of Parkinsonism?

A

bradykinesia

rigidity

rest tremor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the causes of Parkinsonism?

A

degenerative (PD)
drugs (neuroleptics)
vascular
infectious (HIV)
toxins (CO)
tumors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What injury in the basal ganglia causes Parkinson’s?

A

decreased action of the direct pathway

indirect system is active so it inhibits the thalamus so motor cortex can’t be active

no gas pedal, all break

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the process of diagnosis of Parkinson’s disease?

A

remains a clinical diagnosis (stiffness, rest tremor)

investigations (such as CT, MRI) not proven to be useful for diagnosis on evidence based review but rule out other causes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the stats of the incidence of Parkinson’s disease?

A

5-24/10,000 worldwide

incidence of PD rising slowly with aging population

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the stats of the prevalence of Parkinson’s disease?

A

57-371/10,000 worldwide (USA/Canada, 300/10,000)

40% of cases undiagnosed at any time

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the stats of the prevalence of Parkinson’s disease?

A

mean 62 years

rare before age 30

10% cases before age 50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the risk factors of Parkinson’s disease?

A

genetic factors: more in younger people

environmental toxins: greater risk in rural area (exposure to pesticides, MPTP)

other factors: aging, older we get more dopamine we loose

biggest risk factor is aging

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the environmental risk factors of Parkinson’s disease?

A

manganese (or CO2)

MPTP

epidemiologic studies suggest that agricultural chemicals may play a role

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What risk factors cause Parkinson’s disease in younger people?

A

more likely to be caused by genetic factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What risk factors cause Parkinson’s disease in older people?

A

combination to genetic predisposure and environmental or other factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are neuroprotective factors against Parkinson’s disease?

A

exercise, coffee, nicotine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the pathology of Parkinson’s disease?

A

deposition of alpha-synuclein in Lewy bodies (becomes misfolded, clumps up, and get deposited in Lewy body)

degeneration of dopaminergic neurons in pars compacta of substantia nigra

degeneration in brainstem of pigmented nuclei, spinal cord, cortex, gut

Parkinson’s is not just limited to dopamine in substantia nigra, gets deposited in other NS areas as well

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What does the substantia nigra look like with Parkinson’s disease and without?

A

without: makes lots of dopamine so lots of melanin

with: no dopamine so no melanin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the Braak system of Parkinson’s disease?

A

Parkinson’s starts in the gut

Stage 1 of PD: dorsal motor, olfactory, start in the nose or gut then moves to the brain to cause depression, anxiety, and sleep problems (dorsal motor, olfactory, then marches UP brainstem)

Stage 2: coeruleus, sub-coeruleus complex (function in sleep, mood)

Stage 3: motor symptoms

Stages 4-6: cortical involvement, dementia, etc.

so, other signs before motor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is the pharmacology of treating Parkinson’s disease?

A

dopamine can’t cross blood-brain barrier, so can’t ingest it

have to give supplements to make sure it doesn’t turn into dopamine entry

LD is a neutral amino acid that gets into the brain, brain makes it into dopamine

22
Q

What are the drugs involved in the replacement of dopamine?

A

MAO B inhibitors (rasagiline, selegiline)

anti-cholinergics

amantadine

dopamine agonists (pramipexole, ropinirole, rotigotine)

levodopa (short-acting and long-acting)

23
Q

What are the non-motor problems associated with Parkinson’s disease?

A

fatigue, apathy

psychiatric (anxiety and depression)

sleep disturbances

autonomic nervous system (bladder, bowel, sexual dysfunction and hypotension)

cognitive dysfunction

24
Q

What are other treatments of Parkinson’s disease?

A

counseling: referral to support society groups

alternative treatments: exercise, calcium, Vitamin D

neuroprotection: none proven to date, studies ongoing

25
Q

What is the management of moderate disease?

A

multiple medications needed in combination

gait problems (physio, gait assessment, risk of falls)

dysphagia, speech abnormalities

26
Q

What are the surgeries of Parkinson’s disease?

A

advanced disease

GPi vs. STN, DBS vs. lesion

helps all motor symptoms of PD

meds can be decreased with STN DBS

27
Q

What is the treatment of advanced disease?

A

treatment of motor symptoms (adjustment of meds often limited by side effects)

non-motor symptoms (dementia, depression)

planning for care (placement, caregiver burden)

28
Q

What is Parkinson’s disease dementia?

A

up to 70% of PD patients affected

onset heralded by visual hallucinations

loss of insight and multi-tasking, visuospatial abnormalities

Montreal Cognitive Assessment vs. MMSE

29
Q

What are the three types of choreaform disorders?

A

ballism (more proximal, violent)

athetosis (more sinuous)

chorea (distal, irregular, non-stereotypical movement)

30
Q

What are the acute causes of chorea?

A

medications
vascular
immune-mediated
hormonal
infectious
metabolic disorders
toxins/substance abuse

31
Q

What are the chronic causes of chorea?

A

genetic
Huntington disease
SCA 2, 3, 17
DRPLA
Wilson disease
mitochondrial
dopaminergic blockers
infectious
nutritional
endocrine

32
Q

What is Huntington disease?

A

autosomal dominant CAG repeat

age of onset (and progression) dependent on size of triplet repeat

50% of AOO dependent on other genes

slow progression (15-30 years)

33
Q

What is juvenile-onset HD (JHD)?

A

symptom onset before the age of 21 years

childhood onset (<10 years)
adolescent onset (10-20 years)

34
Q

What is pediatric HD?

A

patients with manifest disease who are still below the age of 18 years

35
Q

What are the motor features of early stage Huntington’s disease?

A

restlessness
motor
impersistence
slowing of eye movement

36
Q

What are the motor features of mild-moderate stage Huntington’s disease?

A

chorea gradually becomes more apparent and disabling

37
Q

What are the motor features of late stage Huntington’s disease?

A

chorea lessens

significant rigidity and bradykinesia, gait and balance problems

38
Q

What are the clinical features of Huntington’s disease?

A

motor (chorea, dystonia, rigidity/bradykinesia, myoclonus, gait disorder, dysphagia/dysarthria, eye movement abnormalities)

psychiatric (personality changes, behavioral disorders, depression, irritability/anxiety, obsessive compulsive, psychosis (rare), suicidal ideation)

cognitive (executive dysfunction, dementia)

weight loss

apathy

39
Q

What is the treatment of Huntington’s disease?

A

interdisciplinary management

ongoing psychosocial support

genetic counseling

research

40
Q

What is the definition of dystonia?

A

abnormal sustained muscle contraction and postures

may be associated with tremor

may be alleviated by sensory tricks

41
Q

What is the classification of dystonia?

A

age of onset (early vs. late)

distribution (focal, segmental, generalized)

42
Q

What is the pathophysiology and etiology of dystonia?

A

pathophysiology poorly understood

multiple causes: brain injury, toxic, genetic (particularly in children)

some forms may be related to decreased dopamine (usually children)

43
Q

What is primary generalized dystonia?

A

childhood onset, progressive

legs –> trunk –> arms

DYT 1 mutation most common (autosomal dominant with variable penetrance and expressivity)

44
Q

What is focal dystonia?

A

adult onset, non-progressive

blepharospasm (eyes)
oromandibular (mouth and jaw)
occupational (writing, playing instruments)
cervical (neck)

45
Q

What is the treatment of focal dystonia?

A

botulinum toxin injections (botox was created to treat dystonia, most effective treatment)

medications

surgery (DBS of GPi)

46
Q

What are tics?

A

brief, non-rhythmic semi-suppressible movements

47
Q

What are types of tics?

A

simple motor
clonic
tonic
dystonic
simple vocal
sensory
complex motor
complex vocal

48
Q

What are the primary diagnoses of tics?

A

transient tics of childhood

chronic tics (motor or vocal)

Tourette syndrome

49
Q

What are the secondary diagnoses of tics?

A

infections
post-infectious
neurodegenerative
chromosomal disorders
drug induced
toxic - metabolic
head injury
stroke
neuropsychiatric

50
Q

What is needed for a diagnosis of Tourette’s syndrome?

A

multiple motor + > 1 vocal tics

duration greater than 1 year

51
Q

What are associated features of Tourette’s syndrome?

A

obsessive-compulsive symptoms
ADHD
behavior problems

52
Q

What are treatments of Tourette’s syndrome?

A

education and counselling

modification of home/school/work environment

hyperactivity/ADHD (clonidine, CNS stimulants)

obsessive-compulsive behavior (SSRIs, clomipramine, venlafaxine, atypical antipsychotics)