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Neuro 3 > Vascular and Mov't Disorder Path > Flashcards

Vascular and Mov't Disorder Path Flashcards

1
Q

Describe the cerebral vascular territories

-describe the territories in coronal section

A

Red = anterior cerebral artery (also middle on coronal section)

Purple = middle cerebral artery (lateral on coronal section)

Green = posterior cerebral artery (inferior on coronal section)

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2
Q

Give an explanation for why cerebral blood vessels may be more susceptible to aneurysm?

A

B/c they don’t have an external elastic lamina

  • peripheral BV have both internal and external elastic lamina
  • cerebral BV have just internal => less wall support => weaker walls that are more prone to aneursym
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3
Q

What are considered ‘MCA signs’?

A

Loss of gray-white differentiation and a decrease in density on the MRI

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4
Q
A

Aspergillosis induced multifocal hemorrhage

-multifocal intracerebellar hemorrhage due to mechanical destruction of blood vessel walls by aspergillosis

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5
Q
A

= Arterio-venous malformation

-possible cause of intracerebral hemorrhage

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6
Q

Prototypical diagnosis for movement disorder due to cerebellar lesion

A

Friedreich’s ataxia = impaired coordination

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7
Q

Two disease states resulting from neurodegenerative processes

A

Dementia and movement disorders

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8
Q

What is the mechanism behind the signs/symptoms of Parkinsons?

A

Manifestations (hypokinesai) due to reduced dopaminergic input to the striatum

-loss of DA pathways in the basal ganglia

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9
Q

Differentiate how to see lewy bodies in the substantia nigra vs. the cortex

A
  • substantia nigra lewy bodies (lewy bodies that accumulate in midbrain neurons) have a halo of fibrillary radiating spicules
  • cortical lewy bodies lack this obvious halo => difficult to visualize on H&E alone, but very obvious upon anti-alpha-synuclein or anti-ubiquitin staining
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10
Q

Describe the gross findings below

A

Hypopigmentation in the substantia nigra of Parkinson’s patients due to loss of neuromelanin-containing dopaminergic neurons

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11
Q

What are two proteins that make up the Lewy body structure?

A

alpha-synuclein and ubiquitin

  • also includes alphaB-crystallin and phosphorylated neurofilament proteins
  • then other proteins (tubulin, MAP1, MAP2, amyloid precursor protein etc) gets trapped in the lewy bodies
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12
Q

What is the normal function of alpha-synuclein protein?

What happens when mutant?

A

Synaptic maintenance/chaperone protein at axon terminals

-mutant proteins aggregate more easily and are fibrillogenic => mitochondrial dysfunction

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13
Q

What two disorders is a mutation in alpha-synuclein associated with?

A

Familial (early onset) Parkinsons and lewy body dementia

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14
Q

What disease ensues when lewy bodies/neurities primarily affect the brainstem?

A

Parkinson’s disease

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15
Q

What disease ensues when lewy bodies/neurities affect the neocortix and brainstem?

A

Lewy body dementia

-so basically Parkinson’s vs. lewy body dementia is based on where the lewy bodies are depositing. When lewy bodies deposite in the neocortex (an not just thebrainstem) you get the dementia properties

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16
Q

Glial cytoplasmi bodies are a feature of what disease?

A

Multiple system atrophy (synucleinopathy)

-differentiate this synucleinopathy from PD and LBD b/c the inclusions primarily affect the glia as opposed to the neuron

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17
Q

Where are glial cytoplasmic bodies deposited in multiple system atrophy?

A

Basal ganglia, brainstem, and neocortex (hence multisystem, ratehr spread out)

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18
Q

Where does pathology of synucleinopathies originate?

A

Pathology usually doesn’t start in the CNS, starts peripherally (ex: enteric NS, symp and parasymp ganglia) and then spreads up

-pts often initially complain of GI or cardiovascular complaints (synuclein deposition in Auerbach’s plexus or vagus nerve, then travels up spinal cord to brainstem/cortex)

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19
Q

What type synucleinopathy is associated w/ prominent visual hallucinations?

A

Lewy body dementia

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20
Q

Clincal diagnosis of LBD

(a) fluctuating or constant symptoms?
(b) memory loss at what stage
(c) movement probelsm

A

LBD clinically

(a) fluctuating symptoms- fluctuating cognition and marked variation in attention and alertness
(b) progressive cognitive decline but usually memory loss is not prominent early (while it is in AD)
(c) Often frequent falls, syncope, delusions

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21
Q

In what neurons do cortical lewy bodies deposit?

A

Non-pyramidal neurons of the lower cortical layers

ex: insular cortex, amygdala, parahippocampal gyrus, cingulate gyrus

22
Q

What step preceeds neuropathological stages of LBD?

A

Before LBD even reaches the NS you see lewy body deposition in the gut

-so pts can present initially w GI symptoms

23
Q

What percent of Parkinson’s cases are genetic?

A

About 5-10% familial

  • alpha-synuclein mutation
  • tons of other mutations associated
24
Q

Describe the common pathway of pathogenesis in PD

A

Common end point = neuronal loss

-mechanisms: mitochondrial dysfunction, oxidative stress, protein aggregation

25
Q

Why would an inability to deal w/ oxidative stress specifically lead to PD?

A

B/c dopamine metabolism inherintly generates a lot of free radicals => an inability to adequately detox the free radicals causes destruction of these neurons

-normal dopamine metabolism generates a lot of free radicals => DA pathways are very sensitive to any dysfunction in oxidative detox

26
Q

List other causes of Parkinsonian symptoms

A

Vascular

Infectious

Toxic/Environmental

Degenerative

Inherited metabolic disorders (Wilson’s)

27
Q

What kind of vascular disease could cause Parkinsonism?

A

Multiple infarctions (lacunar strokes) in the basal ganglia

-so loss of DA neurons not b/c of alpha-synuclein deposition, but because of anoxic necrosis

28
Q

Typical symptoms of vascular Parkinsonism

A

Typically prominent bradykinesia and less tremor than idiopathic Parkinsons

-lower body Parkinsons: symptoms in lower extremities- difficulty walking, balance problems, falls

29
Q

What kind of meds can cause Parkinsonism?

A

Anti-psychotic medications (duh they’re anti-dopamine)

30
Q

Name 3 types of neurodegenerative diseases that present with Parkinsonian symptoms

A
  • lewy body dementia
  • multiple system atrophy
  • progressive supranuclear palsy
31
Q

What inherited metabolic disorder can present w/ Parkinsonian symptoms?

A

Wilson’s disease (genetic dysfunction in copper metabolism)

32
Q

What is this gross finding indicating?

A

Showing degeneration of the basal ganglia, specifically the putamen

-classic finding of multiple system atrophy

33
Q

What is the radiologic finding indicative of?

A

Putamen hypointensity in striatonigral degeneration

34
Q

What cells are primarily affected in MSA?

A

Multiple system atrophy = alpha-syncuelin deposition in the glia cells and oligodendrocytes (not as much the neurons as seen in Parkinsons/LBD)

= glial cytoplasmic inclusions

35
Q

What is progressive supranuclear palsy?

A

PSP (progressive supranuclear palsy) = Parkinson-plus syndrome

-tauopathy: affected neurons contain neurofibrillary tangles of clumped tau protein

36
Q

Characteristic sign of PSP?

A

Progressive supranuclear palsy characterized by supranuclear palsy (supranuclear opthalmoplegia)

-due to neurofibrillary tangle deposition in the oculomotor nucleus

37
Q

Pathological mechanism of PSP

A

Progressive supranuclear palsy due to neurofibrillary tangles of clumped tau proteins in specific locations of thebrain: specifically the basal ganglia and brainstem

38
Q

What is this gross finding indicating?

A

Atrophy of the head of the caudate

-indicative of Huntington’s chorea

39
Q

Describe the pathological mechanism of how CAG repeats causes Huntingtons

A

CAG repeats cause the mutant protein to aggregate in the nucleus of the neurons

  • see ubiquinated intranuclear neuronal inclusions
  • leads to atrophy of caudate and other structures
40
Q

What is Friedreich’s ataxia?

A

Autosomal recessive inherited spinocerebellar ataxia

-genetics: caused by expansion of repeats in Frataxin gene

41
Q

Most common presenting symptom of Friedreich’s ataxia

A

Poor coordination => gait disturbance

  • can also cause scoliosis, heart disease, and diabetes
  • does not affect cognitive function
42
Q

Comparing normal vs. diseased

-what is this finding indicative of?

A

Showing marked dorsal and lateral column degeneration of the spinal cord

-indicative of Friedreich’s ataxia where lack of wild type frataxin leads to ROS accumulation and cell damage/death

43
Q

Describe the pathological mechansim of Friedreich’s ataxia

A

Auto recessive inherited disease of expanding repeats of the frataxin gene.

  • when have not enough wild-type frataxin the energy in the mitochondria fails and excess iron builds up => extra ROS generation => cell damage/death
  • overall = neuronal degeneration in dorsal and lateral spinal cord => ataxia
44
Q

What mutation is associated w/ genetic ALS?

A

ALS = amyotrophic lateral sclerosis

  • TDP-43 mutation => TDP-43 accumulation in motor neurons and anterior horn cells
  • about 10% of ALS pts are familial/genetic, otherwise cause is unknown
45
Q

What is a pathologic hallmark of ALS?

A

Bunina bodies = small eosinophilic intraneuronal inclusions in remaining lower motor neurons of ALS pts

= pathologic hallmark of ALS

46
Q

Distinguish the appearance of the motor and sensory root in ALS pts

A

Significant neuronal loss in motor root, sensory root remains unaffected

47
Q

Describe the gross pathological finding

A

Note the shrinking of the precentral gyrus (primary motor cortex)

-indicative of ALS (amyotrophic lateral sclerosis)

48
Q

Neurons of what specific tract are lost in ALS pts?

A

Marked loss of neurons of the corticospinal tracts

49
Q

Describe how Riluzole acts to delay ventilator onset in ALS pts

A

Riluzole prolongs motor neuron disease survivial

  • works by reducing glutamate signaling
  • adds evidence to the case linking excitotoxicity and motor neuron disease
50
Q

Describe the common pathway by which neurofilament mutations, axonal injury, SOD1 mutations, and excessive glutamate receptor activation cause motor neuron death

A

All lead to common pathway of protein aggregation involving TDP-43 or SOD => apoptosis of motor neurons

51
Q

What gets accumulated in the following diseases?

(a) PD
(b) AD
(c) Prion disease
(d) ALS
(e) Huntingtons

A

Showing that a common feature of these neurodegenerative disorders is abnormal protein folding that eventually leads to accumulation of oligomeric proteins

(a) PD = lewy bodies (alpha-synuclein)
(d) AD = neurofibrillary tangles of tau protein
(c) Prion disease = plaque
(d) ALS = bunina bodies
(e) Huntingtons = neuronal inclusions of truncated, ubiquinated Huntington protein

Neuro 3 (7 decks)

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