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USMLE Neurology > Demyelinating disorders > Flashcards

Demyelinating disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

What are the symptoms of MS?

A
  • Acute optic neuritis (painful unilateral vision loss associated w. Marcus Gunn pupil)
  • Brain stem/cerebellar symptoms (diplopia, ataxia, scanning speech, intention tremor, nystagmus/INO [bilateral.unilateral]
  • Pyramidal tract demyelination (weakness, spasticity)
  • Spinal cord syndromes (electric shock like sensation along cervical spine and neck flexion, neurogenic bladder, parapesis, sensory manifestations affecting the trunk or more than one extremity)
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2
Q

In what temperatures may MS be exacerbated?

A

Increased (hot bath, exercise)

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3
Q

What is the most common clinical course of MS?

A

Relapsing remitting

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4
Q

Who is most often afected by MS?

A
  • Females 20-30
  • Low sunlight populations (low vit D exposure)
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5
Q

What will be seen on MRi with MS?

A
  • Oligoclonal bands
  • Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis)
  • Multiple white matter lesions disseminated in space and time
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6
Q

What is the treatement of MS (in terms of disease modifying)?

A
  • Beta-interferon
  • Glatiramer
  • Natalizumab
  • IV steroids for falre ups
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7
Q

What drugs are given for neurogenic bladder MS?

A
  • Muscarinic antagonists
  • Also catheterisation
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8
Q

What drugs can be given for spasticity in MS?

A
  • Baclofen
  • GABAB receptor agonists
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9
Q

What can be given for pain in MS?

A
  • TCAs
  • Anticonvulsants
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10
Q

What can osmotic demyelination syndrome also be known as?

A

Central pontine myelinosis
- Axonal dymelination in pontine white matter

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11
Q

What is osmotic dymelination due to?

A
  • Rapid osmotic changes (correction of hyponatremia most commonly)
  • Could be shifts in other osmolytes (e.g. glucose)
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12
Q

What are the symptoms of osmotic dymelination syndrome?

A
  • Acute paralysis
  • Dysarthria
  • Dysphagia
  • Diplopia
  • Loss of consciousness
  • May cause “locked in syndrome”
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13
Q

Correcting Hyponatremia (serum Na+) too fast will cause what?

A

Osmotic dymelination syndrome (pons white matter)

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14
Q

Correcting Hypernatremia too quickly can cause what?

A

Cerebral oedema / herniation

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15
Q

What is the most common subtype of GBS?

A

Acute inflammatory Demyelinating Polyneuropathy

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16
Q

What cells are mainly destroyed in GBS / Acute inflammatory Demyelinating Polyneuropathy?

A

Schwann cells

17
Q

What is the mechanism behind GBS which induces autoimmunity?

A

Molecular mimicry

18
Q

What is the main symptom of GBS / Acute inflammatory Demyelinating Polyneuropathy?

A
  • Symmetric ascending paralysis / muscle weakness (depressed/absent DTRs in lower extremities)
  • Facial paralysis
  • Resp failure
  • Autonomic dysregulation (cardiac, hyper/hypotension) + sensory abnormalities may also be seen
19
Q

What is the treatment of acute inflammatory dymelinating polyneuropathy?
What class of drugs should NOT be given?

A
  • Plasma exchange
  • IV immunoglobulins
  • Resp support

NO STEROIDS

20
Q

What does Acute disseminated (postinfectious) encephalomyelitis present with?

A

Rapidly progressive multifocal neurologic symptoms, altered mental status

21
Q

What is Acute disseminated (postinfectious) encephalomyelitis caused by?

A

Infection or vaccine

22
Q

What gene deformaity causes Charcot-Marie-Tooth [subtype CMT1A]?
How is it inherited?

A

Aut dominant
- PMP22 gene duplication

23
Q

What is the pathology behind CMT disease?

A

Hereditary nerve disease due to defective production of proteins involved in the structure and function of peripheral nerves of the myelin sheath

24
Q

What are the symptoms of CMT disease?

A
  • Foot deformaties (eg pevus cavus, hammer toe)
  • Lower extremity weakness (eg foot drop)
  • Sensory deficits (can’t move toes)
25
Q

What is Progressive multifocal leukoencephalopathy caused by?
Describe the pathophysiology

A

Reactivation of JC virus
- Destroys oligodendrocytes -> Dymelination of CNS

26
Q

What is Progressive multifocal leukoencephalopathy associated with?

A
  • Severe immunosuppression (AIDS, organ transplant, lymphoma leukemia)
  • Natalizumab and rituximab
27
Q

What areas of the brain are primarily affected in Progressive multifocal leukoencephalopathy?

A
  • Parietal and occipital areas, visual symptoms common
28
Q

What are the outcomes like for Progressive multifocal leukoencephalopathy patients?

A

Rapidly progressive, usually fatal

USMLE Neurology (19 decks)

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