LEC-3 Neuropathology (1) Flashcards

1
Q

Hypoxia/Ischemia results in degeneration of what neurons?

A

Hippocampus

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2
Q

Huntington’s Disease results in degeneration of what neurons?

A

Caudate Nucleus

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3
Q

Parkinson’s Disease results in degeneration of what neurons?

A

Substantia Nigra

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4
Q

Amyotrophic lateral sclerosis (ALS) results in degernation of what neurons?

A

Motor Neurons

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5
Q

What are the signs of Acute Neuronal Injury?

A
  • Red neurons (eosinophilic neuronal necrosis)
  • Dark & smudgy chromatin
  • Hypoxic/ischemic injury
  • Eosinophilic, shunken cytoplasm
  • Cell death is by necrosis or apoptosis
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6
Q

Myelin disorders are associated with what component of the nervous system?

A

Oligodendrocytes

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7
Q

What component of the nervous system is most resistant to injury?

A

Astrocytes

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8
Q

What are the major mechanisms of cellular injury?

A
  • Free radical injury
  • Excitotoxic injury
    • Excitatory amino acids: Glutamate and aspartate
    • Excitatory amino acid receptors: NMDA receptor
    • Depolarization
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9
Q

Describe the excitotoxic injury.

A

Inappropriate activation of NMDA receptor that results in:

  • Membrane depolarization
  • Calcium influx
  • Mitochondrial dysfunction
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10
Q

What does subacute/chronic neuronal injury result in?

A
  • Degeneration (take longer than acute neuronal injury)
  • Cell loss is hard to see early on; reactive glial changes is best indication
  • Cell death is mainly due to apoptosis
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11
Q

What is the axonal reaction?

A
  • Changes that occur in the neuron cell body in response to axonal injury
  • Regenerate attempt
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12
Q

What are the features of the axonal reaction?

A
  • Central chromatolysis
  • Damage to axon
  • Margination of Nissil substance
  • Central clearing of the cytoplasm
  • Peripheral displacement of the nucleus
  • Rounding of the cell body
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13
Q

What is this image?

A

Red Neuron

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14
Q

What is this image?

A

Axonal reaction

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15
Q

What is this image?

A

Axonal spheroid

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16
Q

Axons are highlighted as black lines with what stain?

A

Silver

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17
Q

What inclusions are associated with Parkinsons?

A

Lewy body

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18
Q

What inclusion is associated with Alzheimers?

A

Neurofibrillary tangles

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19
Q

What inclusions are associated with Pick’s Disease?

A

Pick’s body

Fronto-temporal lobar degernation

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20
Q

Neuronal inclusions may occur as a manifestation of aging, when there are intracytoplasmic accumulations of complex lipids (___________).

A

Lipofuscin

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21
Q

Abnormal cytoplasmic deposition of complex lipids and other substances also occurs in ___________________________ in which substrates or intermediates accumulate.

A

genetically determined disorders of metabolism

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22
Q

Viral infection can lead to abnormal intranuclear inclusions, as seen in __________.

A

Herpetic infection

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23
Q

Viral infection can lead to abnormal cytoplasmic inclusions, as seen in _______.

A

Rabies. (Negri body)

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24
Q

Viral infection can lead to abnormal nuclear AND cytoplasmic inclusions, as seen in _______

A

Cytomegalovirus (CMV) infection

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25
Q

Abnormal vacuolization of the perikaryon and neuronal cell processes in the neuropil is seen in what disease?

A

Creutzfeldt-Jacob disease

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26
Q

What is Trans-synaptic degeneration?

A
  • Secondary degeneration of a neuron connected to a dying neuron
  • Anterograde or retrograde
27
Q

What is this image and what is it diagnostic of?

A
  • Alzheimers Glia Type II
  • Hyperammonemia
28
Q

What is this image and what is it diagnostic of?

A
  • Creutzfeldt astrocyte
  • Not diagnostic of any specific disease; just a reactive astrocyte
    • Commonly seen in demyelinating disease
29
Q

What is this image and what is it diagnostic of?

A
  • Satellitosis (satelliting)
  • Diagnostic of oligodendroglial changes
30
Q

What is this image and what is it diagnostic of?

A
  • Onion Bulb Schwaan Cell
  • Diagnostic of Charcot-Marie-Tooth Disease
31
Q

What is this image?

A

Large Group Neurogenic Atrophy

32
Q

What is the cause of Myasthenia Gravis?

A

Auto-antibodies against ACh-receptors at neuromuscular junction

33
Q

What are the symptoms of Myasthenia Gravis?

A

Progressive weakness throughout the day (feel fine in the morning, can’t physically keep eyes open by nighttime)

34
Q

What are associated disorders of Myasthenia Gravis?

A
  • Other autoimmune disorders
  • Thymoma/thymic hyperplasia
35
Q

What is the cause of Lambert-Eaton Syndrome?

A

Auto-antibodies against voltage-gated Calcium channels at axon terminal, inhibiting release of NT-filled vesicles following an action potenial

36
Q

What is this image?

A

Muscular dystrophy

37
Q

What does this image show?

A
  • Mitochondrial disease
  • Ragged red fiber
38
Q

What is this image show?

A

Polymyositis: Endomysial inflammation and atrophy

39
Q

What is the cause, symptoms, and pathology associated with Polymyositis?

A
  • Cause: CD8+ mediated attack on muscle cells
  • Symptoms: Diffuse pain
  • Pathology: Intrafascicular inflammation
40
Q

What does this image show?

A

Dermatomyositis: Extrafascicular inflammation and perifascicular atrophy

41
Q

What is the cause, symptoms, and pathology associated with Dermatomyositis?

A
  • Cause: Antibody-mediated attack on muscle cells
  • Symptoms: Diffuse pain and rash
  • Pathology: Extrafascicular inflammation; perifascicular atrophy
42
Q

What does this image show?

A

Inclusion Body Myositis: Rimmed vacuole (with faint blue dots)

43
Q

What is the cause, symptoms, and pathology associated with Inclusion Body Myositis?

A
  • Cause: Degenerative
  • Symptoms: Steroid resistance
  • Pathology: Inclusions; rimmed vacuoles
44
Q

What is the cause of Duchenne Muscular Dystrophy?

A

Dystrophin deficiency due to X-linked frameshift mutation of gene

Onset: 5-6 years, death in adolescence or yound adulthood

45
Q

What is Becker Muscular Dystrophy?

A

A milder form of Duchenne MD due to a point mutation in the dystrophin gene

46
Q

What are congenital myopathies?

A

Fixed, structural defects in muscle

Weakness at brith, nonprogresssive

Names after histological appearance:

  • Central core
  • Rod body
  • Centronuclear
47
Q

What does this image show?

A

Central core myopathy

48
Q

What does this image show?

A

Rod body myopathy

49
Q

What does this image show?

A

Centronuclear myopathy

50
Q

What is the onset of Multiple Sclerosis (MS)?

A

> 50 years

51
Q

What are the symptoms of of Multiple Sclerosis (MS)?

A
  • Seeminly random
  • Episodic losses of neural function (vision problems, weakness in arm, parasthesia in leg)
  • Increased Ig levels in CSF
52
Q

What is the cause of Multiple Sclerosis (MS)?

A

Auto-immune attack of myelin (mainly CD4+ and macrophage mediated)

53
Q

What is associated with the acute phase of Multiple Sclerosis (MS)?

A
  • Demyelination
  • Lots of immune cells near axons
54
Q

What is associated with the chronic phase of Multiple Sclerosis (MS)?

A
  • Reactive astrocytosis
  • Axon loss
55
Q

What is the cause of Devic Disease (Neuromylitis Optica)?

A

Autoantibodies against Aquaporin-4 receptors

56
Q

What are the symptoms of Devic Disease (Neuromylitis Optica)?

A

Simultaneous blindness and paraplegia

57
Q

What is the onset of Acute Disseminated Encephalomyelitis (ADEM)?

A

A few weeks post-infection/vaccination

58
Q

What is the cause of Acute Disseminated Encephalomyelitis (ADEM)?

A

Possibly a cross-reaction with or autoimmunity against myelin

59
Q

What symptoms are associated with Acute Disseminated Encephalomyelitis (ADEM)?

A

Rapid onset of headache, lethargy, coma

60
Q

What is the onset of Acute Necrotizing Hemorrhagic Encephalomyelitis (ANHE; Weston-Hurst Disease)?

A

Shortly after UTI or M. pneumoniae infection

61
Q

What is associated with Infectious Demyelination?

A
  • HIV
  • Progressive multifocal leukoencephalopathy (PML)
62
Q

What is Marchiafava-Bignami?

A

Demyelinating disorder that is associated with a lesion of the corpus callosum

Also associated with alcoholism

63
Q

What is Demyelinating pseudotumor (tumefactive demyelination)?

A

Demyelinating disorder that has a single mass lesion that mimics a neoplasm; moderate correlation between this and the patient developing MS later on

64
Q

What is Central Pontine and Extrapontine Myelinolysis (CPM; osmotic demyelination syndrome)?

A

Primary demyelinating disease with a lesion that appears at the base of pons or any location where white and gray matter closely associate (so, most of hindbrain/parts of midbrain)

  • Associated with rapid correction of electrolye imbalance