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neuro 2 > Additional Basal Ganglia Pathologies > Flashcards

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
Q

Explain the pathophysiology behind CBD.

A
  • Unknown cause
  • Glial lesions on cortical and substantia nigra
  • Neurons look ballooned/give appearance of over-pronounced sulci
26
Q

What MRI findings will be found with CBD?

A
  • Brain shrinkage – white matter is shrinking away
  • Over-pronounced sulci
27
Q

What are the signs & symptoms associated with CBD?

A
  • PD symptoms
  • Cognitive impairments
  • Visuospatial impairments
  • Apraxia
  • Myoclonus
  • Dysphagia
  • Progressive aphasia
28
Q

How is CBD managed?

A

treat symptoms

29
Q

How is the prognosis for CBD?

A
  • Variable prognosis
  • Mean survival 6-8 years from symptoms
30
Q

What is the typical age of onset for dystonia?

A

childhood and early adulthood

31
Q

When is the onset for dystonia if it typically begins with limb movement?

A

early onset

32
Q

When is the onset for dystonia if it typically begins with neck and/or facial involvement?

A

adult-onset

33
Q

How do we classify dystonia? (5)

A
  • 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
Q

Explain the pathophysiology behind dystonia.

A
  • Cause – idiopathic or genetic
  • No identifiable damage can be found on imaging
  • Think basal ganglia disruption
35
Q

What are the typical signs and symptoms associated with dystonia?

A
  • 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
Q

What are some additional characteristics associated with dystonic movements?

A
  • 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
Q

How is dystonia managed?

A
  • Meds
    o Botox for focal
    o Anticholinergic agents, GABAergic agents, and Dopaminergic agents
  • Deep brain stimulation
38
Q

How is the prognosis for dystonia and why is it considered progressive?

A
  • Progressive disease though high variability
  • Childhood onset more likely to spread to other body regions
39
Q

What causes tardive dyskinesia (TD)?

A
  • Drug-induced disorder characterized by involuntary and uncontrolled stiff, jerky movements of face and body
40
Q

What are some known risk factors for TD?

A
  • Age > 55 y/o
  • Older women
  • Amount of drug ingested
  • Long-term use of drug
  • Alcohol or drug abuse
  • Black and Asian populations
41
Q

How is TD diagnosed?

A
  • Review of med history
  • Clinical exam
42
Q

What are the typical signs and symptoms associated with TD?

A
  • 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
Q

Where are dyskinesias common in tardive dyskinesias?

A

face

44
Q

How is TD managed?

A
  • Cessation or dosage adjustment of medication
  • Deutetrabenazine and Valbenazine
45
Q

How is the prognosis for TD?

A
  • Typically irreversible with long-term presence of symptoms
  • Some patients see symptoms reversed with cessation of medication
46
Q

What is the typical age of onset for Huntington’s Disease (HD)?

A
  • 30-50s
47
Q

What types of populations has HD been found to be more common in?

A
  • Caucasian populations of western European descent
48
Q

Explain the pathophysiology behind HD.

A
  • Dominant autosomal trait
  • Disruption to protein (Huntington protein) that becomes toxic and leads to disruption of normal neuronal function, atrophy, neuronal death
49
Q

What occurs during the early stages of HD?

A

loss of striatum in indirect (NO GO) pathway, causing disinhibition of thalamus
- NO GO = suppressed unwanted movements

50
Q

What occurs during the late stages of HD?

A

loss of striatum neurons in direct (GO) pathway
- start and increase amplitude movements

51
Q

How is HD diagnosed?

A
  • Review of family hx
  • Genetic testing
  • Clinical examination
52
Q

What are the typical signs and symptoms associated with HD?

A
  • 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
Q

What is the prodromal phase of HD?

A
    • genetic test without S&S
54
Q

During the early stages of HD, what occurs?

A

loss of striatum neurons in the indirect (NO-GO) pathway, causing disinhibition of thalamus

55
Q

During the later stages of HD, what occurs?

A

loss of striatum neurons in direct (GO) pathway
- also see loss of widespread cortical neurons

neuro 2 (19 decks)

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