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Additional Basal Ganglia Pathologies Flashcards

1
Q

What is the pathophysiology involved in Vascular Parkinsonism?

A
  • Clinical features of PD that are caused by multiple small CVAs
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2
Q

What are the typical signs and symptoms of Vascular Parkinsonism?

A

Symmetrical lower-body parkinsonism
- Gait unsteadiness - +freezing, festinating
- Bradykinesia, akinesia, hypokinesia
- Absence of tremors
- Rigidity
- Pyramidal signs (weakness, spasticity, hyperreflexia, slow RAM)

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3
Q

How do we manage Vascular Parkinsonism and how is its prognosis?

A

Management
- CVA treatment – treat the cause
Prognosis
- Fair – multifocal strokes have the worst prognosis of strokes
- High morbidity

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4
Q

What is the average age of onset of Progressive Supranuclear Palsy (PSP)?

A

60s

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5
Q

Explain the pathophysiology behind PSP.

A
  • Accumulation of tau protein that leads to neuronal death and astrogliosis
    o Astrogliosis – abnormal accumulation of astrocytes (leads to neuronal death)
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6
Q

How is PSP diagnosed? What specific MRI findings are involved?

A
  • MRI
    o Frontal lobe subthalamic, and putamen atrophy
    o Morning Glory sign (flower) – abnormal concavity in basis and tegmentum of midbrain
    o Hummingbird sign – flattening of the brainstem
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7
Q

What is PSP commonly misdiagnosed as?

A
  • Depression
  • Dementia
  • PD
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8
Q

What are the signs & symptoms associated with PSP

A
  • Gait disturbances
  • Loss of righting reactions – fall backwards suddenly
  • Motor impulsivity – rocket sign
  • Severe axial rigidity – extensors > flexors
  • Supranuclear opthalmoplegia
  • Dysphagia
  • Dysarthria
  • Frontal cognitive dysfunction
  • Pseudobulbar affect
  • Sleep disturbances
  • Fixed Mona Lisa Stare (surprised expression)
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9
Q

Which direction do PSP patients typically fall? Why?

A
  • fall backwards suddenly due to loss of righting reaction
  • due to rocket sign (shoot right up) and axial rigidity that typically affects the extensors > flexors
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10
Q

What type of eye movement dysfunction do PSP patients have?

A

supranuclear opthalmopegia
- limited vertical eye movement
- loss of convergence
- impaired vertical saccades
- loss of visual acuity
- loss of eyelid control

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11
Q

How is PSP managed?

A
  • No effective treatment
  • Anti-PD meds – minimal and short lasting
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12
Q

How is the prognosis for PSP?

A
  • Severe disability in 3-5 years of onset
  • Mortality commonly seen 5-8 years
  • Serious complications are common – Pneumonia, falls with injuries
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13
Q

What is the hallmark for multiple system atrophy? Why?

A

marked autonomic dysfunction
- thoracic and sacral spine involved which leads to autonomic dysfunction

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14
Q

What is the average age of onset of Multiple Systems Atrophy (MSA)?

A
  • Early 50s
  • Rarely diagnosed past 70 years
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15
Q

Explain the pathophysiology behind MSA.

A
  • Unknown cause
  • Proteins in glial cells that affect oligodendrocytes who create and maintain myelin in CNS
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16
Q

What are the two subtypes of MSA and how do they differ?

A
  • MSA-P – resembles typical PD
  • MSA-C – resembles cerebellar issues
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17
Q

How is MSA diagnosed? What specific MRI findings are involved?

A
  • Clinical exam
  • PET scan
  • MRI – hot cross buns sign (atrophy in pons)
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18
Q

What is MSA commonly misdiagnosed as?

A
  • PD but often corrected once patients do not respond to dopamine therapy
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19
Q

What are the signs & symptoms associated with MSA?

A
  • Autonomic dysfunction
  • Bradykinesia, rigidity, tremors
  • Gait and limb ataxia
  • Head/oral dyskinesias and dystonias – coat hanger pain
  • Antecollis – looking down posture
  • Pisa syndrome
  • Oculomotor dusturbances
  • Speech deficits
  • Sleep disorders
  • Cognitive deficits are RARE
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20
Q

How is MSA managed?

A
  • No effective treatment
  • Anti-PD meds effective for 1/3 of patients – can worsen OH
  • Medications for symptoms
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21
Q

How is the prognosis for MSA?

A
  • 50% need AD after 3 years
  • 60% become wheelchair dependent in 5 years
  • Mortality ranges from 5-10 years after onset
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22
Q

What predicts slower disease progression of MSA?

A

cerebellar phenotype and later onset of autonomic symptoms

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23
Q

What is the average age of onset of Corticobulbar Degeneration (CBD)?

A
  • 60-80 years
24
Q

Do women or men get CBD more commonly?

A

women > men

25
Explain the pathophysiology behind CBD.
- Unknown cause - Glial lesions on cortical and substantia nigra - Neurons look ballooned/give appearance of over-pronounced sulci
26
What MRI findings will be found with CBD?
- Brain shrinkage – white matter is shrinking away - Over-pronounced sulci
27
What are the signs & symptoms associated with CBD?
- PD symptoms - Cognitive impairments - Visuospatial impairments - Apraxia - Myoclonus - Dysphagia - Progressive aphasia
28
How is CBD managed?
treat symptoms
29
How is the prognosis for CBD?
- Variable prognosis - Mean survival 6-8 years from symptoms
30
What is the typical age of onset for dystonia?
childhood and early adulthood
31
When is the onset for dystonia if it typically begins with limb movement?
early onset
32
When is the onset for dystonia if it typically begins with neck and/or facial involvement?
adult-onset
33
How do we classify dystonia? (5)
- Focal – 1 segment affected - Segmental – 2 or more continuous areas affected (hand and forearm) - Multifocal – 2 non-continuous areas affected (hand and foot) - Generalized – typically involves trunk and 2 other areas of extremities - Hemi – ½ body
34
Explain the pathophysiology behind dystonia.
- Cause – idiopathic or genetic - No identifiable damage can be found on imaging - Think basal ganglia disruption
35
What are the typical signs and symptoms associated with dystonia?
- Patterned, twisting, may be tremulous - Common initial symptoms o Sporadic foot cramping and/or toe dragging o Excessive inversion during swing phase of gait - General symptoms o Eyelid dystonias causing excessive and rapid blinking or inability to open eyes entirely o Neck and trunk can be impaired = postural abnormalities o Limb involvement frequently leads to severe contractures
36
What are some additional characteristics associated with dystonic movements?
- Intermittent in early stages and more constant during later stages - Worsened with activity - Can be circumstantial – ex: musician has dystonia while playing an instrument but can write easily
37
How is dystonia managed?
- Meds o Botox for focal o Anticholinergic agents, GABAergic agents, and Dopaminergic agents - Deep brain stimulation
38
How is the prognosis for dystonia and why is it considered progressive?
- Progressive disease though high variability - Childhood onset more likely to spread to other body regions
39
What causes tardive dyskinesia (TD)?
- Drug-induced disorder characterized by involuntary and uncontrolled stiff, jerky movements of face and body
40
What are some known risk factors for TD?
- Age > 55 y/o - Older women - Amount of drug ingested - Long-term use of drug - Alcohol or drug abuse - Black and Asian populations
41
How is TD diagnosed?
- Review of med history - Clinical exam
42
What are the typical signs and symptoms associated with TD?
- Slow, gradual onset of symptoms after ingestion of medication - Dyskinesias of face most common o Grimacing, sticking out tongue, sucking or fish-like facial movements common - Choreoathetoid (slow writhing movements) or dystonic movements in limbs - Abnormal postural tone, abnormal postural adjustments o Increased extension of trunk, lordosis, neck flexion and rotation
43
Where are dyskinesias common in tardive dyskinesias?
face
44
How is TD managed?
- Cessation or dosage adjustment of medication - Deutetrabenazine and Valbenazine
45
How is the prognosis for TD?
- Typically irreversible with long-term presence of symptoms - Some patients see symptoms reversed with cessation of medication
46
What is the typical age of onset for Huntington’s Disease (HD)?
- 30-50s
47
What types of populations has HD been found to be more common in?
- Caucasian populations of western European descent
48
Explain the pathophysiology behind HD.
- Dominant autosomal trait - Disruption to protein (Huntington protein) that becomes toxic and leads to disruption of normal neuronal function, atrophy, neuronal death
49
What occurs during the early stages of HD?
loss of striatum in indirect (NO GO) pathway, causing disinhibition of thalamus - NO GO = suppressed unwanted movements
50
What occurs during the late stages of HD?
loss of striatum neurons in direct (GO) pathway - start and increase amplitude movements
51
How is HD diagnosed?
- Review of family hx - Genetic testing - Clinical examination
52
What are the typical signs and symptoms associated with HD?
- Choreiform movement - Motor impersistance – fly-catchers tongue (can’t keep tongue in their mouth) - Dystonia - Ataxia - EOM and visuospatial impairments - Speech and swallowing deficits - Impaired memory, attention, executive function - Mood changes, depression, anxiety, uncharacteristic anger and irritability
53
What is the prodromal phase of HD?
- + genetic test without S&S
54
During the early stages of HD, what occurs?
loss of striatum neurons in the indirect (NO-GO) pathway, causing disinhibition of thalamus
55
During the later stages of HD, what occurs?
loss of striatum neurons in direct (GO) pathway - also see loss of widespread cortical neurons

Neuro 2 (19 decks)

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