Movement Disorders Flashcards

(50 cards)

1
Q

Most common neurodegenerative diseases

A

1 - Alzheimer’s
2 - Parkinson’s

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2
Q

Is Parkinson’s more common in women or men?

A

It’s 2x more common in men than women

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3
Q

Typical age of onset of Parkinson’s

A

After 50 y/o

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4
Q

How does someone get Parkinson’s?

A

By having a genetic predisposition and being exposed to different environmental triggers

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5
Q

Genetics and Parkinson’s

A

A family history of Parkinson’s increases risk

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6
Q

What is ⍺-synuclein?

A

⍺-Synuclein is a protein normally found in neurons.
In PD, ⍺-synuclein misfolds and clumps together, forming abnormal aggregates (Lewy bodies and Lewy neurites)

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7
Q

Lewy bodies vs Lewy neurites

A

Both are abnormal aggregates of ⍺-synuclein which disrupt normal neuron function.
-Lewy bodies: inside neuron cell bodies
-Lewy neurites: in the axons/dendrites

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8
Q

Parkinson’s progression in the brain

A
  1. Toxic ⍺-synuclein aggregations form Lewy bodies and Lewy neurites
  2. Lewy bodies/neurites damage neurons, especially in the substantia nigra,
  3. This causes dopamine deficiency and movement symptoms
  4. Over time, other brain regions are affected, leading to broader non-motor symptoms.
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9
Q

Overall function of the basal ganglia

A

It refines and regulates motor activity through feedback loops involving the cerebral cortex and thalamus

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10
Q

Main components of the BG

A

Striatum: consists of the caudate nucleus and the putamen, receives input from the cortex
Globus pallidus (internal + external): receives info from the striatum, major output structure regulating thalamic activity
Substantia nigra: (the pars compacta specifically) produces dopamine

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11
Q

How does the BG regulate movement?

A

Through two opposing circuits that act on the motor cortex via the thalamus:
-Direct pathway: activates the motor cortex –> facilitates movement
-Indirect pathway: inhibits the motor cortex –> suppresses movement

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12
Q

How does dopamine increase movement?

A

-Stimulates the direct pathway via D1 receptors (promotes movement)
-Inhibits the indirect pathway via D2 receptors (removes the inhibition on movement)

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13
Q

Effects of dopamine loss on the BG pathways

A

-Decreased D1 stimulation –> weak direct pathway –> less movement promotion
-Decreased D2 inhibition –> stronger indirect pathway –> more movement suppression
Overall: over-inhibition of the thalamus –> decreased excitation of the motor cortex

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14
Q

Dopamine deficiency causes what?

A

Parkinsonism - TRAP
-Tremor (resting)
-Rigidity
-Akinesia / bradykinesia
-Posture instability + gait changes (late-stage)

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15
Q

Parkinson’s disease

A

A neurodegenerative disorder caused by loss of dopamine-producing neurons in the substantia nigra pars compacta

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16
Q

Atypical parkinsonism

A

Disorders that mimic Parkinson’s but have additional neurological features
-Poor response to levodopa
-Progress faster
-More severe

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17
Q

Examples of atypical parkinsonisms

A

-Dementia with Lewy bodies
-Multiple system atrophy
-Progressive supranuclear palsy
-Cortical basal degeneration

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18
Q

Multiple system atrophy - key features

A

-Autonomic failure (hypotension, incotinence, etc.)
-Parkinsonism
-Stooped neck

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19
Q

Progressive supranuclear palsy

A

-Vertical gaze palsy
-Wide open eyes
-Early falls
-Hummingbird sign in brainstem on MRI

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20
Q

Cortical basal degeneration

A

-Asymmetric parkinsonism
-Apraxia
-Alien hand

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21
Q

Secondary parkinsonism

A

Parkinsonian symptoms caused by an identifiable external or secondary cause, not primary neurodegeneration
Ex: typical antipsychotics

22
Q

Criteria for Parkinson’s diagnosis

A

-Bradykinesia (#1 sign)
-Rest tremor OR rigidity

23
Q

Is Parkinson’s typically symmetric or asymmetric?

A

Asymmetric - usually starts on one side and remains asymmetric for the course of the disease

24
Q

When, if at all, does dementia present in Parkinson’s?

A

Late stage (one of the last symptoms)

25
Levodopa
Levodopa is the metabolic precursor of dopamine --> enters the brain and gets converted to dopamine *Used to replace lost dopamine in Parkinson’s disease
26
Why is levodopa used for PD treatment over just giving dopamine?
Levodopa can cross the BBB, dopamine cannot
27
Carbidopa
Often given with levodopa, it inhibits the enzyme that converts levodopa --> dopamine outside the brain to reduce side effects and concentrate dopamine in the brain
28
Management of PD fluctuations (3)
-Levodopa-carbidopa stretegies: higher dose, increased frequency of doses, controlled release preparation -Adjunctive treatments (enzyme inhibitors to prevent levodopa breakdown, dopamine agonist) -Advanced treatments
29
Advanced treatments for PD (4)
-Patch -Subcut / sublingual apomorphine -Levodopa-carbidopa intestinal gel -Deep brain stimulation
30
Resting tremor vs action tremor
**Resting tremor:** tremor occurs when the muscle is at rest **Action tremor:** occurs during voluntary movement
31
Essential tremor
A primary tremor (not caused by another neuro disorder) -Action tremor -Strong intentional component (gets worse during goal-directed movement, especially as the hand approaches the target) -Usually bilateral upper limbs but asymmetric
32
Supportive features of an essential tremor (2)
-Family history -Improves with alcohol
33
Tremor vs chorea
**Tremor:** rhythmic, oscillatory (back-and-forth) movements of a body part **Chorea:** irregular, unpredictable, dance-like movements
34
Is Parkinsonism present in Huntington's?
Yes, but not till later in the course
35
Things that could cause chorea (5)
-Huntington's -Immune mediated (paraneoplastic, autoimmune encephalitis, SLE) -Infectious (viral encephalitis) -Metabolic/endocrine (hyperthyroid, hyper or hypoglycemia, electrolyte imbalance) -Drug-induced
36
What is hemiballismus?
A sudden, violent, flinging, involuntary movement of one side of the body (usually proximal limbs) - a type of hyperkinetic movement disorder
37
What causes hemiballismus?
Usually a lesion in the subthalamic nucleus (STN) - ischemic stroke, hemorrhage, tumors or metabolic lesions **Mechanism:** decreases the indirect pathway of the BG, resulting in less inhibition of the thalamus and excessive cortical motor activity
38
Dystonia
Sustained or intermittent muscle contractions causing abnormal, often repetitive movements or postures (usually patterned twisting) -Exacerbated when performing voluntary movements -Can treat with botox to block Ach action at the muscle
39
Wilson disease
An autosomal recessive disorder of copper metabolism - causes copper to accumulate in many systems and so it has multisystemic effects (neurological, psychiatric, hepatic, ophthalmologic, renal)
40
Diagnosis + treatment of Wilson's disease
**Diagnosis:** liver biopsy, genetic testing **Treatment:** copper chalation
41
Myoclonus
Sudden, brief, shock-like involuntary movements
42
Causes of myoclonus (3)
-Epileptic -Toxic -Metabolic
43
Tics
Repetitive, stereotyped, involunatry movements or vocalizations -Usually start in childhood -Voluntarily suppressible (but cause an unpleasant sensation/urge if not acted out)
44
Treatment for tics
*Only treat if causing impairment -CBT -Alpha-agonists like clonidine -Antipsychotics
45
Ataxia
Impaired coordination of voluntary muscle movement *This is a finding, not a disease in and of itself - usually causes by cerebellar pathology
46
Signs of cerebellar ataxia (7)
-Limb ataxia (poorly coordinated movement of arms or legs) -Dysdiadochokinesia (can't do rapid alternating movements) -Intention tremor -Dysmetria (can't judge distance/range of movement - overshoot or undershoot) -Nystagmus -Saccades -Dysarthria / scanning speech (slow, irregular, broken-up speech)
47
Normal gait parameters
-Step height: no more than 1-2 inches from the floor -Step length: at least the length of a foot between heel and toe -Stance: feet should almost touch as one passes the other
48
How can gait disorders be classified?
By where the abnormal sensorimotor level is -**Low:** peripheral sensory or motor dysfunction -**Middle:** postural / locomotor impairment -**High:** cognitive + white matter disorders
49
Gait abnormalities in proprioceptive deficit / sensory neuropathy (3)
-Wide based gait -Steppage gait (foot drop) -Foot slap
50
Gait abnormalities with vestibular disorder (3)
-Weaving, falling to one side -Wide based -Arms held out to improve balance