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S4- Neuro 1 > Week 7- Basal Ganglia Disorders (Parkinson's) > Flashcards

Week 7- Basal Ganglia Disorders (Parkinson's) Flashcards

1
Q

PART 1: NEUROANATOMY REVIEW

A

PART 1: NEUROANATOMY REVIEW

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2
Q

What are the (4) parts of the basal ganglia?

A
  • Caudate
  • Putamen
  • Globus Pallidus (internus and externus)
  • Substantia Nigra (compacta and reticularis)
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3
Q
  • What are the caudate and putamen typically referred to?
  • Virtually all inputs to the basal ganglia arrive via the ______ and _______.
  • Outgoing information leaves the basal ganglia via the ___________ and _________.
A
  • Striatum
  • caudate and putamen
  • globus pallidus and substantia nigra
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4
Q

The globus pallidus and substantia nigra send information out from the basal ganglia back up to the cortex via the _________, but also send projections down to important structures in our brain stem including our _________ ________.

A
  • thalamus

- reticular formation

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5
Q

The basal ganglia has ______ and ________ function.

A

motor (control) and nonmotor

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6
Q

Basal Ganglia Motor Control:

  • _______ and ________ of movement.
  • Prevention of unwanted movements through _________ control.
  • Direct and Indirect pathways that work together to help create ________ movement.
A
  • INITIATION and EXECUTION
  • inhibitory
  • desired
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7
Q

What are some additional roles of the basal ganglia?

A
  • Eye movement loop
  • Goal-directed behavior loop
  • Social behavior loop
  • Emotion loop
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8
Q

Does the basal ganglia directly equate to movement?

A

No, modulates incoming info from the cortex and then sends it back up to help regulate things like muscle contraction, muscle force, multi-joint movements and sequencing of movements.

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9
Q
  • What is initiation?

- What is execution?

A
  • “I want to move, I move.”

- How well we move, how forcefully we move.

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10
Q

Our goal-directed and social behavior loops are often combined into the __________ loop that involves the ___________ _________ cortex.

A
  • prefrontal

- dorsolateral prefrontal cortex

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11
Q

Our emotion loop is often referred to as the __________ loop which involves the anterior cingulate and orbital frontal cortex.

A

-limbic

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12
Q

Direct (GO) Pathway of Basal Ganglia Control:

  1. ) Thalamus, gone unchecked, sends constant __________ signals to cortex to elicit movement.
  2. ) _____, _____ inhibit thalamus to prevent unwanted movement.
  3. ) When a movement is needed, cortex sends information to striatum, which in turns ________ GP & SN. In turn, opens gate for thalamus to resume excitatory projections to cortex to elicit movement.
A
  • excitatory
  • Globus pallidus internus (GPi) and Substantia nigra pars reticularis (SNpr).
  • inhibits
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13
Q

What inhibitory NT is sent to the thalamus via the GP and SN to prevent unwanted movement?

A

GABA

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14
Q

What excitatory NT is sent to the striatum via the cortex to cause the striatum to send GABA to the GP and SN to turn off its inhibitory function?

A

Glutamate

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15
Q

The ____________ pathway is what happens when we want to move.

A

Direct (GO)

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16
Q
  • Normally the globus pallidus externus (GPe) acts by ___________ the subthalamic nuclei.
  • When the indirect pathway is activated, the cortex will send an excitatory message to the striatum, and instead of talking to the GPi and SNpr, it sends _________ information to the GPe.
  • This means the GPe can no longer inhibit the subthalamic nuclei, then the cortex starts directly sending excitatory information to the __________ _________.
  • The subthalmic nuclei then sends excitatory information to the ______ and ______ causing movement to stop.
A
  • inhibiting
  • inhibitory
  • subthalamic nuclei
  • GPi and SNpr
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17
Q

The ___________ pathway is what happens when we don’t want movement.

A

-Indirect (NO GO)

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18
Q
  • What structure is watching over both the Direct (GO) and Indirect (NO GO) pathway by modulating loops?
  • It has connections directly to the _________.
  • These connections modulate activity of indirect pathway through ________ release in striatum.
A
  • Substantia Nigra pars compacta (SNpc)
  • striatum
  • dopamine
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19
Q

What are the main BG neurotransmitters?

A
  • Dopamine
  • Acetylcholine (ACh)
  • GABA, Glutamate
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20
Q

Dopamine:

  • Made in _________, which modulates striatum activity through dopamine release to impact Direct and Indirect pathways.
  • Excitatory to striatum neurons in _______ pathway, inhibitory to striatum neurons in ________ pathway.
  • Dual effect = powerful ________ in suppression of thalamus by BG, which leads to further facilitation of movement.
A
  • SNpc
  • direct, indirect
  • DECREASE
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21
Q

Acetylcholine:

  • Inhibits _________ when appropriate.
  • Can quickly interrupt ongoing ________ behavior in response to salient environmental stimuli.
A
  • dopamine

- motor

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22
Q

________ and _________ are the primary NTs in direct and indirect pathways.

A

GABA and Glutamate

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23
Q

PART 2: INTRO TO PARKINSON’S DISEASE

A

PART 2: INTRO TO PARKINSON’S DISEASE

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24
Q

What are some general SxS of BG dysfunction? (3)

A
  • Difficulty initiating, continuing, or stopping movement.
  • Muscle tone abnormalities (rigidity).
  • Increased involuntary movements (hemiballismus, athetosis, chorea, tremor),
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25
Q
  • What is the most common disorder associated with BG dysfunction?
  • It is an ________, _______ progressive degenerative disease.
  • Does it have motor or non-motor symptoms?
A
  • Parkinson’s Disease
  • idiopathic, slowly progressive
  • both motor and non-motor
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26
Q

Parkinson’s Incidence and Prevalence:

  • Incidence ________ with age.
  • ________ cases in US
  • Does it affect men or women more?
  • What is the mean age of onset?
  • _______ prevalence in black and Asian populations?
A
  • increases
  • 1 million
  • Men>Women (3:2)
  • early 60s
  • decreased
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27
Q
  • Parkinson’s Disease can also be called _________ ___________.
  • What is the cause?
A
  • Primary Parkinsonism

- Unknown, hypothesis is a complex interaction of factors (age, genetics, environment)

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28
Q

What are some causes of Secondary Parkinsonism?

A
  • Infections/postencephalitic
  • atherosclerosis
  • toxic
  • drug-induced
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29
Q

Parkinson’s Disease Pathophysiology:
-Degeneration of ___________ neurons in the BG
(Loss of DA stores in substantia nigra).
-As disease progresses, numerous other regions of brains involved as well as impaired modulation of other _____________.

A
  • dopaminergic

- neurotransmitters

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30
Q

What are 3 ways we may diagnose Parkinson’s Disease?

A
  • clinical examination
  • levadopa/carbidopa trial (recent guidelines shifting away)
  • SPECT scan (DaTscan)
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31
Q

What is the only definitive way to diagnose Parkinson’s?

A

post-mortem examination of brain

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32
Q

PART 3: PARKINSON’S DISEASE MOTOR SYMPTOMS

A

PART 3: PARKINSON’S DISEASE MOTOR SYMPTOMS

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33
Q

Motor symptoms do not appear until ~___% of neurodegeneration has already occurred in the basal ganglia.

A

~60%

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34
Q

What are the 4 cardinal motor symptoms of Parkinson’s Disease?

A
  • Bradykinesia***
  • Akinesia
  • Hypokinesia
  • Rigidity
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35
Q

Bradykinesia, Akinesia, Hypokinesia:

  • __________ and ___________ movements affected.
  • Initiation, alteration in direction, stoppage all affected
  • _________ tasks > _______ commands
A
  • spontaneous and purposeful

- complex tasks > simple commands

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36
Q
  • What is bradykinesia?
  • What is akinesia?
  • What is hypokinesia?
A
  • Bradykinesia = Reduction (slowing) of movement.
  • Akinesia = Loss of spontaneous movement.
  • Hypokinesia = Decreased amplitude or range of movement.
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37
Q
  • __________ is present in all types and subtypes of Parkinson’s.
  • It is a result of insufficient recruitment of muscle ______ during movement due to dopamine depletion.
A
  • Bradykinesia

- force

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38
Q

Where will we see akinesia over bradykinesia in Parkinson’s Disease?

A
  • In the face, patients will present with masked, blunted, resting facial features.
  • Loss of arm movement during gait.
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39
Q
  • Hypokinesia, like bradykinesia, is thought to be an issue of ______ production.
  • Where will we easily find hypokinesia in Parkinson’s?
A

-force

  • When asking patient to write something down. (small and squished letters)
  • Very minimal trunk movement when walking.
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40
Q

What are often the most disabling symptoms of Parkinson’s?

A

Kinesias

41
Q

Rigidity:

  • Felt _________ in all directions. (asymmetrical early → eventual whole-body involvement)
  • Is it usually seen proximally or distal first?
  • Leads to increased ________ load, emotional stress, energy expenditure of movement. (long-term effects are decresed ROM, contractures, postural deformities).
  • Presents as _____ _____ or _________ rigidity.
A
  • uniformly
  • proximal
  • cognitive load
  • lead pipe or cogwheel rigidity
42
Q

What is the difference between lead pipe and cogwheel?

A
  • Lead pipe: sustained resistance

- Cogwheel: jerky, ratchet-like (lead pipe + tremor)

43
Q

What are 2 other cardinal motor symptoms seen with Parkinson’s?

A
  • Tremor

- Postural Instability

44
Q

Tremor:
-________ tremor early on (can see kinetic as disease progresses).
-______ and _______ most common, can see in head, neck, jaw or tongue.
Tends to be ______, _____ frequency.

A
  • resting
  • Hand and foot
  • mild, low frequency
45
Q

What is a good piece of education to give to patients regarding tremor?

A

Tremors are often exacerbated by stress, emotional strain, or excitement. Relaxation techniques can be used to help.

46
Q

Postural Instability:

  • Abnormal and inflexible postural responses.
  • Smaller functional limits of _________.
  • Difficulties with ____-________ movements.
  • Reduced ___________ postural adjustments and control.
  • Abnormal patterns of ____________.
  • _______ disorientation.
  • Additional contributing factors: rigidity, weakness, loss of ROM, freezing, medication side effects.
A
  • stability
  • self-initiated
  • anticipatory
  • coactivation
  • midline
47
Q

The most frequent presentation of midline disorientation in Parkinson’s is __________.

A

retropulsion

48
Q

The Implications of PD Parkinson’s Instability:

  • Patients with PD are __x more likely to fall.
  • Falls become increasingly prevalent as disease progresses to the ________ stages. (Disappear in late stages as patients become immobile)
  • ___% of patients with PD fall each year.
  • ___% of patients with PD report recurrent falls.
  • ___% of those that fall experience injury.
  • ___% of patients experience a hip fracture within 10 years of diagnosis.
A
  • 9x
  • middle
  • 70%
  • 50%
  • 40%
  • 25%
49
Q
  • What are the 3 biggest risk factors for falls in PD patients?
  • What are some other risk factors for falls?
A
  • Postural Instability
  • Disease Severity
  • Gait Impairments (most notable in freezing gait)

-dementia, depression, postural hypotension, involuntary movements from long-term medication use

50
Q

Do we see weakness with Parkinson’s patients? Why?

A

Yes

  • Decrease in torque production at all speeds
  • Dopamine related?
  • EMG: Delayed MU recruitment, asynchronization
  • Disuse weakness common
  • Fatigue
51
Q

Parkinson’s patients also present with a breakdown of complex motor _________. This involves sequential movements and transitioning between movements.

A

planning

52
Q

PART 4: GAIT DISTURBANCES AND NON-MOTOR IMPAIRMENTS

A

PART 4: GAIT DISTURBANCES AND NON-MOTOR IMPAIRMENTS

53
Q

Gait characteristics of Parkinson’s are broken into what 2 characteristics? What are they?

A
  • Continuous (typically seen whenever they get up and around)
  • Episodic (what happens occasionally)
54
Q

Continuous Characteristics:

  • Overall ___________ presentation (smaller steps/reduced arm swing/minimal trunk rotation, LE regidity/axial rigidity).
  • Increased __________ and ____________.
  • Poor __________ control (achieving, maintaining, and restoring balance impacted)

As Disease Progresses:

  • _________ gait pattern emerges (“festinating gait” becomes more continuous)
  • Increased tendency for _____/_______pulsion.
A
  • hypokinetic
  • variability and asymmetry
  • postural
  • shuffling
  • retropulsion/anteropulsion
55
Q

What are the 4 main episodic gait characteristics seen with PD?

A
  • Festinating Gait Pattern
  • Midline Disorientation
  • En Bloc Turning
  • Freezing of Gait
56
Q

Episodic gait characteristics of PD are seen in the _____/_______ stages of PD.

A

early/middle

57
Q
  • What is festinating gait pattern?

- How is a festinating gait pattern different from shuffling gait?

A
  • Unintentionally quick, shuffled steps that worsens as gait progresses.
  • They start to take increasingly shorter and faster steps as they are walking. (shuffle gait doesn’t change with time)
58
Q
  • Common types of midline disorientation?

- Why can midline disorientation be episodic?

A
  • retropulsion/anteropulsion
  • Tends to be mild but present, might not impact someone performing easier tasks such as flat surface but will when put on incline. (specific tasks)
59
Q

What is en bloc turning?

A

Strategy to overcome significant difficulty with turning.

60
Q

En Bloc Turning:

  • ___ rotations of head, trunk, pelvis to complete turns.
  • ___ instabilities observed during turns.
  • Reduced ______, more steps to complete turns.
  • Can be further impacted by ___ postural tone, axial rigidity, and/or loss of flexibility.
A
  • ↓
  • ↑
  • speed
  • ↑
61
Q

What is “freezing of gait”?

A

“Brief, episodic absence or marked reduction of forward progression of the feet despite the intention to walk.”

62
Q

Freezing of Gait:

  • How long does it typically last?
  • Leads to significant ____ risk.
  • What is the mechanism of FoG?
  • When is it most often seen?
  • Can be exacerbated by _______/______/_______.
  • Does it worsen with disease progression?
A
  • Typically last a few seconds but can occasionally exceed up to 30s, and even can be entirely unable to generate steps for several minutes until compensatory or manual interventions are applied.
  • fall risk
  • not well understood
  • When it involves a confrontation of competing afferent stimuli (walking through doorways, turning, variable surfaces).
  • stress/fatigue/distractions
  • Yes
63
Q

List non-motor symptoms of PD and %.

A
  • Loss of smell (90%)
  • Sleep disturbances (90%)
  • Constipation (20%–79%)
  • Pain and Paraesthesias (76%)
  • Visual impairments (75%)
  • Orthostatic hypotension (may or may not be symptomatic) (60%)
  • Fatigue (58%)
  • Urinary symptoms (25%–50%)
  • Apathy (40%)
  • Early mild cognitive impairment (25%)
  • Dementia (80% late stage)
  • Depression (22%)
64
Q

Pain:

  • Pain is common with PD and is present in __-__% of patients as an early symptom. (MSK, dystonic, neuropathic, central, akathisia)
  • What is akathisia?
  • Central pain though to be due to abnormal modulation of pain caused by _________ deficiency.
  • What are common areas of pain?
  • ___________ common.
A
  • 60-80%
  • Inner restlessness, inability to remain still.
  • dopamine
  • lower back, legs, shoulders, face
  • hypersensitivities
65
Q

PART 5: CLASSIFICATION, MEDICAL MANAGEMENT, AND PROGNOSIS

A

PART 5: CLASSIFICATION, MEDICAL MANAGEMENT, AND PROGNOSIS

66
Q

What are the 2 ways we classify PD?

A
  • Symptoms

- Where in progression disease pattern is at.

67
Q

What are the 2 main subtypes (phenotypes) of PD?

A
  • Postural Instability Gait Disorder phenotype

- Tremor-Dominant phenotype

68
Q

Patients will be identified as part of phenotypes based off of what?

A

Most early and dominant symptoms.

69
Q

Postural Instability Gait Disorder:

  • ___% of all PD cases.
  • What are the dominant symptoms?
  • More _________ disease course.
A
  • 25%
  • postural instability and gait disturbances
  • significant
70
Q

Tremor-Dominant:

  • Typically demonstrate ________ problems with bradykinesia or postural instability.
  • Lower prevalence of ____-_______ symptoms.
  • Less likely to develop _________ and other _______ deficits.
A
  • fewer
  • non-motor
  • dementia and other cognitive deficits
71
Q
  • What scale is used for staging of PD?
  • How many stages are there?
  • It primarily considers ______ impairments.
  • What 3 things does it look at?
A
  • Hoehn and Yahr Scale for Staging of PD
  • 5 Stages
  • motor
  • unilateral vs bilateral, impact balance, impact function
72
Q

Describe each stage of PD.

A

Stage 1
-Unilateral involvement only usually with minimal or no functional disability.
Stage 2
-Bilateral or midline involvement without impairment of balance.
Stage 3
-Bilateral disease: mild-to-moderate disability with impaired postural reflexes; physically independent.
Stage 4
-Severely disabling disease; still able to walk or stand unassisted.
Stage 5
-Confinement to bed or wheelchair unless aided.

73
Q

At which stage do we see the classic PD flexed position?

A

Stage 3

74
Q

At which stage do we start to see akinesias over bradykinesias?

A

Stage 4

75
Q

What is the primary mode of medical management for PD?

A

Medication

76
Q

What are some drugs used to manage PD?

A
  • Carbidopa and Levodopa (Sinamet)
  • Dopamine agonists
  • Catechol-O-methyltransferase (COMT) inhibitors
  • Monoamine oxidase-B (MOA-B) inhibitors
  • Anticholinergics
77
Q

PD symptoms can be reduced by trying to elevate depleted _________ or reducing _________ effects.

A
  • dopamine

- acetylcholine

78
Q
  • What is the primary drug (first line of defense) that will be prescribed to PD patients?
  • This drug is a _________ replacement drug.
  • __________ converts into dopamine; _________ facilitates the process and minimizes the side effects.
A
  • Sinamet
  • dopamine
  • levodopa, carbidopa
79
Q

What drugs mimic effects of dopamine by stimulating dopamine receptors? They can be used to delay the need for C/L, but often is used in combination with it.

A

Dopamine agonists

80
Q

What drugs increase the availability of dopamine by blocking disruptive enzymes? They must be used with C/L to be effective.

A

COMT inhibitors

81
Q

What drugs function mimics that of COMT inhibitors but is often used as monotherapy in early stages or in combination with C/L later on?

A

MAO-B inhibitors

82
Q

What drugs combat the reciprocal increase of ACh that is seen in response to dopamine depletion and is primarily used to treat tremors in early stages of disease?

A

Anticholinergics

83
Q

Early initiation of pharmaceuticals are shown to help with progression of disease long-term, but prolonged treatment can lead to ___________.

A

dyskinesias

84
Q

Describe ON/OFF times of PD medications.

A
  • Sinamet and other PD drugs have half lives that often lead to times in which effects of medications have worn off (“OFF times”)
  • Patients’ function and capabilities can look significantly different when comparing ON and OFF times.
  • With disease progression, see more fluctuations and increased frequencies during the day of OFF times.
85
Q

Does levadopa improve all Parkinson’s symptoms?

A

No

86
Q

Levadopa:

  • Does not improve _____ rigidity.
  • Typically worsens _________ responses to external perturbations.
  • Shown to improve hypokinetic gait in the early stages of PD but tends to be less effective at improving gait as the disease progresses.
  • Generally improves freezing of gait in the “___” state but not during the “___” state.
A
  • axial
  • postural
  • “OFF”, “ON”
87
Q

What is the common surgical option used for PD?

A

Deep Brain Stimulation

88
Q

Deep Brain Stimulation:
-Typically reserved for bradykinesia, rigidity and tremor in patients who no longer respond to __________ in a predictable manner or who suffer medication-induced dyskinesias.
Can increase ____ periods, reduce frequency of _____ periods, and lead to a reduction in many PD symptoms.

A
  • medication

- ON, OFF

89
Q

Symptoms not responsive to medication _____ responsive to surgery.

A

are not

90
Q

Prognosis:

  • Symptoms of PD vary widely and disease can progress very quickly or can go several decades with _____ limitations.
  • Typically does not lead directly to mortality. Life expectancy often just slightly less than average. What are some common secondary sequalae that can lead to death?
A
  • mild

- CHF and Pneumonia

91
Q

List some negative prognostic factors of PD.

A
  • Degree of symmetry of symptoms
  • Postural Instability Gait Disorder (PIGD) phenotype
  • Higher baseline UPDRS motor scores
  • Age at onset
  • Early cognitive decline or baseline cognitive impairment
  • Smoking history
  • Male gender
92
Q

PART 6: OUTCOME MEASURES

A

PART 6: OUTCOME MEASURES

93
Q

List some PD outcome measures.

A
  • PDQ-39
  • MDS-UPDRS
  • Montreal Cognitive Assessment (MoCA)
  • Parkinson’s Fatigue Scale
  • Freezing of Gait Questionnaire
94
Q

PDQ-39:

  • Evaluates Parkinson’s Disease-specific health-related quality of life over the last _______.
  • What are the 8 domains assessed?
  • Closely correlates with ____, better construct validity than generic measures.
A
  • month
  • Mobility, ADLs, emotional well-being, stigma, social support, cognition, communication, bodily discomfort
  • H and Y scale
95
Q

MDS-UPDRS:

  • ______________ assessment designed to monitor the burden and extend of PD across the longitudinal disease course.
  • What are the 4 parts of MDS-UPDRS?
A

-comprehensive (33 pages)

  • Part I: Non-motor experiences of daily living (Cognition, emotions, hallucinations, depression, anxiety, apathy, etc)
  • Part II: Motor experiences of daily living (Speech, saliva/drooling, chewing/swallowing, eating, dressing, hygiene, tremor, transferring, walking, etc)
  • Part III: Motor examination
  • Part IV: Motor complications (Functional impact of dyskinesias, OFF states, functional impact of dystonia, etc)
96
Q

MoCA:

  • Rapid screen of __________ abilities designed to detect dysfunction.
  • Common component of OT evaluation.
  • What is the cut-off score for normal cognitive function?
  • Is it specific to Parkinson’s disease?
A
  • cognitive
  • > /=26 points considered normal cognitive function
  • No, but is recommended.
97
Q

Parkinson’s Fatigue Scale:

  • Reflection of _________ aspects of fatigue; measures presence of fatigue and impact on daily function.
  • Excludes _________ and __________ features of fatigue.
  • Excludes _________ and ___________ of fatigue symptoms.
A

-physical
-cognitive and emotional
severity and frequency

98
Q

Freezing of Gait Questionnaire:

  • Assess FOG severity unrelated to _______ in patients with PD.
  • 6-item questionnaire with 4 questions on FOG _____________ and 2 questions on disturbances in ______.
  • Correlates well with _______ stage.
A
  • falls
  • frequency, gait
  • H and Y

S4- Neuro 1 (12 decks)

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