LEC-3 Neuropathology (1) Flashcards Preview

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Flashcards in LEC-3 Neuropathology (1) Deck (64):
1

Hypoxia/Ischemia results in degeneration of what neurons?

Hippocampus

2

Huntington's Disease results in degeneration of what neurons?

Caudate Nucleus

3

Parkinson's Disease results in degeneration of what neurons?

Substantia Nigra

4

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

Motor Neurons

5

What are the signs of Acute Neuronal Injury?

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

6

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

Oligodendrocytes

7

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

Astrocytes

8

What are the major mechanisms of cellular injury?

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

9

Describe the excitotoxic injury.

Inappropriate activation of NMDA receptor that results in:

  • Membrane depolarization
  • Calcium influx
  • Mitochondrial dysfunction

10

What does subacute/chronic neuronal injury result in?

  • 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

11

What is the axonal reaction?

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

12

What are the features of the axonal reaction?

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

13

What is this image?

Q image thumb

Red Neuron

14

What is this image?

Q image thumb

Axonal reaction

15

What is this image?

Q image thumb

Axonal spheroid

16

Axons are highlighted as black lines with what stain?

Silver

17

What inclusions are associated with Parkinsons?

Lewy body

A image thumb
18

What inclusion is associated with Alzheimers?

Neurofibrillary tangles

A image thumb
19

What inclusions are associated with Pick's Disease?

Pick's body

Fronto-temporal lobar degernation

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20

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

Lipofuscin

21

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

genetically determined disorders of metabolism

22

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

Herpetic infection

23

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

Rabies. (Negri body)

24

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

Cytomegalovirus (CMV) infection

25

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

Creutzfeldt-Jacob disease

26

What is Trans-synaptic degeneration?

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

27

What is this image and what is it diagnostic of?

Q image thumb

  • Alzheimers Glia Type II
  • Hyperammonemia

28

What is this image and what is it diagnostic of?

Q image thumb

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

29

What is this image and what is it diagnostic of?

Q image thumb

  • Satellitosis (satelliting)
  • Diagnostic of oligodendroglial changes

30

What is this image and what is it diagnostic of?

Q image thumb

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

31

What is this image?

Q image thumb

Large Group Neurogenic Atrophy

32

What is the cause of Myasthenia Gravis?

Auto-antibodies against ACh-receptors at neuromuscular junction

33

What are the symptoms of Myasthenia Gravis?

 

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

34

What are associated disorders of Myasthenia Gravis?

  • Other autoimmune disorders
  • Thymoma/thymic hyperplasia

35

What is the cause of Lambert-Eaton Syndrome?

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

36

What is this image?

Q image thumb

Muscular dystrophy

37

What does this image show?

Q image thumb

  • Mitochondrial disease
  • Ragged red fiber

38

What is this image show?

Q image thumb

Polymyositis: Endomysial inflammation and atrophy

39

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

 

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

40

What does this image show?

Q image thumb

Dermatomyositis: Extrafascicular inflammation and perifascicular atrophy

41

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

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

42

What does this image show?

Q image thumb

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

43

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

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

 

44

What is the cause of Duchenne Muscular Dystrophy?

Dystrophin deficiency due to X-linked frameshift mutation of gene

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

45

What is Becker Muscular Dystrophy?

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

46

What are congenital myopathies?

Fixed, structural defects in muscle

Weakness at brith, nonprogresssive

Names after histological appearance:

  • Central core
  • Rod body
  • Centronuclear

47

What does this image show?

Q image thumb

Central core myopathy

48

What does this image show?

Q image thumb

Rod body myopathy

49

What does this image show?

Q image thumb

Centronuclear myopathy

50

What is the onset of Multiple Sclerosis (MS)?

 

> 50 years

51

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

 

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

52

What is the cause of Multiple Sclerosis (MS)?

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

53

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

  • Demyelination
  • Lots of immune cells near axons

54

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

  • Reactive astrocytosis
  • Axon loss

55

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

Autoantibodies against Aquaporin-4 receptors

56

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

Simultaneous blindness and paraplegia

57

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

A few weeks post-infection/vaccination

58

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

Possibly a cross-reaction with or autoimmunity against myelin

59

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

Rapid onset of headache, lethargy, coma

60

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

Shortly after UTI or M. pneumoniae infection

61

What is associated with Infectious Demyelination?

 

  • HIV
  • Progressive multifocal leukoencephalopathy (PML)

62

What is Marchiafava-Bignami?

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

Also associated with alcoholism

63

What is Demyelinating pseudotumor (tumefactive demyelination)?

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

64

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

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