Neuropathology Part IV- Demylinating (10) Flashcards

1
Q

what do demylenating diseases affect (2)

A
  • Olgodendroglia cells
  • Mylein sheath cells

(result in a net loss of myelin)

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2
Q

What are the 2 forms of demylating disoders

A

Primary (leukodystrophies

Acuired (ms)

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3
Q

What is Ms and what does it affect

A

m/c demylenating disorder of the CNS

characterized by recurrent episodes of demylenation in the brain (optic nerve first) and spinal cord

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4
Q

epidemiology of MS

A

1/1000 w higher prevelence in northern countries
women double risk
-usual onset in 3/4th decade

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5
Q

what genetic factor is associated w MS

A

HLA DR2

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6
Q

what immune factors are associated w MS

A

Cd4 and CD8 are inflitrated in lesions associated w MS

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7
Q

3 clinical forms of MS

A

devic disease
acute ms (marburg)
Balo disease

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8
Q

Devic disease

A

classical ms pres

-bilat optic neuritis and spinal cord involvement, rapidly progressive

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9
Q

Acute MS

A

widespread myelin injury, affects younger pts and has rapid death

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10
Q

Balo disease

A

Rare MS, progressive varient associated w the development of lesions containing rings of myelinated and demylinated white matter

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11
Q

Morphology of MS and where does it like to affect

A

Grey/white translucent plaques in the white matter

loc- corner of lat ventricles, optic nerves and spinal cord

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12
Q

What are the types of plaques in MS (4)

A

Acute- soft/slightly pink

Remote- more firm, pearly grey and relatively circumscribed

Active plaque- sharply defined collections of foamy macrophages, associated with relative preservation of neurons

Shadow- axons w thin layer of myelin at edge (partial demylenation)

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13
Q

What are the common S/s of MS

A
  • unilateral optic neuritis and transversemyelitis

- diplopia, ataxia, spatisity, weakness

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14
Q

What lab results will you see with MS

A

CSF

  • myelin in csf
  • Basic pro (due to active myelin breakdown)
  • Oligoclonal bands
  • elevated CSF igG index
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15
Q

What is acute disseminated encephalomyelitis

A

Immune mediated demylinating disease that follows a viral illness

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16
Q

clinical course of acute disseminated encephalomyelitis + s/s

A
  • monophasic (no remissions) with abrubt onset
  • seizures, coma, focal neurological defits

-collection of foamy macrophages similar to MS

17
Q

What causes acute necrotizing hemorrhagic encephalomyelitis

A

immune mediated demyelinating disease that follows an upper respiratory inf (mycoplasma pneumonia)

18
Q

Acute necrotizing hemorrhagic encephalomyelitis- clinical course/ morphology

A

acute onset, fulminant course

-has white and grey matter necrosis, neutrophilic infiltrate and fibrin deposision

19
Q

What causes progressive multifocal leukoencephalopathy

A

Demyelination as result of viral inf involving oligodendroglial cells

cause- JC virus in association w immunosupression

20
Q

What is central pontine myelinolysis- what does it affect and cause

A

Focal demylinisation of central basal pons

-pts who have low lvls of sodium- Severly malnourished, alcoholics, liver disease

21
Q

What is an axonal neeuropathy

A

associated w the formation of fragmented axonal processes
-wallerian degen)

-mc type of neuropathies

22
Q

what does demylinating neuropathy affect

A

Primary damage to schwann cells (characterized by selective myelin injury and relative sparing of axons)

23
Q

What is the course of guillain barre syndrome

A

-usually begins with weakness in the distal extremities and progresses to involve the prox mm groups

24
Q

what predisposes guillain barre syndrome

A

2/3 follow influenza like illness (CMV, epsien barr etc)

25
Q

what is the mc metabolic neuropathy

A

Diabetes mellitis- distal symmetric sensory or sensorimotor/autonomic

26
Q

Pathology of diabetic neuropathy (3)

A

increased glu leads to:
vasa nervorum- capillary basement thickening (leading to improper profession)

Nerve glycoxidation- impaired axonal transport, slow

Imflammation

27
Q

What are the causes of infectious neuropathy (3)

A

leprosy
diptheria
varicella zoster

28
Q

What causes paraneoplastic neuropathy (5)

A

POEMS

  • polyneuropathy
  • organomegaly
  • endocrinopathy
  • monoclonal gammopathy
  • skin changes