acute inflammatory demyelinating polyradiculopathy / Multiple sclerosis Flashcards

1
Q

Most common subtype of Guillain-Barre syndrome

A

acute inflammatory demyelinating polyradiculopathy

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2
Q

acute inflammatory demyelinating polyradiculopathy - pathophysiology

A

Autoimmune condition that destroys Schwann cells –> inflammation and demyelination of peripheral nerves and motor fibers

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3
Q

acute inflammatory demyelinating polyradiculopathy - result in (symptoms)

A
  1. symmetric/paralysis (beginning in lower extremities)
  2. facial paralysis (in 50%)
  3. autonomic regulation (eg. cardiac irregulation, hypertension, hypertension)
  4. sensory abnormalities
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4
Q

acute inflammatory demyelinating polyradiculopathy - symetric paralysis course / face??

A
  • beginning in lower extremities

- facial paralysis in 50%

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5
Q

acute inflammatory demyelinating polyradiculopathy - except paralysis, it can cause

A
  1. autonomic regulation (eg. cardiac irregulation, hypertension, hypertension)
  2. sensory abnormalities
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6
Q

acute inflammatory demyelinating polyradiculopathy - course of disease

A

almost all patients survive

the majority recover completely after weeks or months

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7
Q

acute inflammatory demyelinating polyradiculopathy - lab

A

increased CSF protein with normal cell count (albuminocytologic dissociation)

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8
Q

acute inflammatory demyelinating polyradiculopathy - increased protein in CSF can cause

A

papilledema

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9
Q

acute inflammatory demyelinating polyradiculopathy - treatment

A
  1. Respiratory support (critical until recovery)
  2. plasmapheresis
  3. IV immunoglobins
    NO ROLE FOR STEROIDS
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10
Q

acute inflammatory demyelinating polyradiculopathy - associated with (it can be caused by …)

A

infection:
1. Campylobacter jejuni
2. viral

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11
Q

acute inflammatory demyelinating polyradiculopathy - caused by infection (mechanims)

A

autoimmune attack of peripheral myelin due to molecular mimicry, inoculations, and stress, but not definitive link to pathogens

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12
Q

Multiple sclerosis - definition

A

Autoimmune inflammation and demyelination of CNS (brain and spinal cord)

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13
Q

Multiple sclerosis epidemiology - MC in

A

women, 20-30 years old, whites living further from equator

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14
Q

Multiple sclerosis - patients can present with

A
  1. optic neuritis (sudden onset of vision resulting in Marcus Gunn pupils)
  2. intrenuclear ophthalmoplegia
  3. hemiparesis
  4. hemisensory symptoms
  5. bladder/bowel incontinence
  6. intention tremor
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15
Q

Multiple sclerosis - Coarse of symptoms

A

relapsing and remitting

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16
Q

Multiple sclerosis - Charcot classic triad

A

MNEMONIC –> SIN

  1. Scanning speech (dysarthria)
  2. Intention tremor (also Internuclear opthalmoplegia and Incontinence)
  3. Nystagmus
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17
Q

Multiple sclerosis - gold standard

A

MRI

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18
Q

Multiple sclerosis - findings in CSF

A

increased IgG + myelin basic protein

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19
Q

Multiple sclerosis - … are diagnostic

A

oligoclonal bands

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20
Q

Multiple sclerosis - image (and mechanims)

A

periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with destruction of axons (multiple white matter lesions separated in space and time)

21
Q

Multiple sclerosis - treatment is divided to

A
  1. slow progression
  2. treat acute flares
  3. symptomatic treatment
22
Q

Multiple sclerosis - slow progression treatment

A

disease modifying therapies (eg. β-interferon, natalizumab, glatiramer)

23
Q

Multiple sclerosis - treat acute flares

A

IV steroids

24
Q

Multiple sclerosis - symptomatic treatment

A
  1. neurogenic bladder (cathetirazation, muscarinic antagonists)
  2. spasticity (baclofen, GABA b receptor agonists)
  3. pain (opioids)
25
Q

Multiple sclerosis - treatment

A
  1. slow progression treatment –> disease modifying therapies (eg. β-interferon, natalizumab)
  2. treat acute flares –> IV steroids
  3. symptomatic treatment –> cathetirazation, muscarinic antagonists, baclofen, GABA b receptor agonists, opioids
26
Q

acute inflammatory demyelinating polyradiculopathy - eye

A

papilledema

27
Q

Adrenoleukodystrophy typically affects (why)

A

males

X-linked genetic disorder

28
Q

Adrenoleukodystrophy - pathophysiology / mode of inheritance

A

X-linked genetic disorder –> disrupts metabolism of very-long-chain fatty acids –> excessive buildup (accumulation) in nervous system, adrenal glands and testes

29
Q

Adrenoleukodystrophy - coarse of disease

A

Progressive disease that can lead to

1. long-term coma/death and 2. adrenal crisis

30
Q

demyelinating and dysmelinating diseases - types

A
  1. Multiple sclerosis
  2. Acute inflammatory demyelinating polyradiculopathy
  3. Acute disseminated (postinfectious) encephalomyelitis
  4. Charcot-Marie-Tooth disease
  5. Krabbe disease
  6. Metachromatic leukodystrophy
  7. Progressive multifocal leukoencephalopathy
31
Q

Acute disseminated (postinfectious) encephalomyelitis?

A

multifocal periventricular inflammation and demyelination after infection or certain vaccination

32
Q

Acute disseminated (postinfectious) encephalomyelitis is usually caused by (examples)

A
  1. infection –> measles, VZV

2. certain vaccinations –> rabies, smallpox

33
Q

Acute disseminated (postinfectious) encephalomyelitis - presentation

A

rapidly progressive multifocal neurologic symptoms

34
Q

Charcot-Marie-Tooth disease - AKA … / mode of inheritance

A

hereditary motor and sensory neuropathy (HMSN)

AD

35
Q

Charcot-Marie-Tooth disease - definition and mechanism

A

Group of progressive hereditary nerve disorder related to the defective production of proteins involved in the structure and function of peripheral nerves or the meylin sheath

36
Q

Charcot-Marie-Tooth disease - symptoms and signs

A
  1. foot deformities (pes cavus)
  2. lower extremities weakness
    3 .sensory deficits
37
Q

Krabbe disease - is a ……disease / mode of inheritance / mechansim

A

lysosomal storage disease / AR

deficiency of galactocerebrosidase

38
Q

Krabbe disease - pathophysiology

A

deficiency of galactocerebrosidase –> buildup (accumulation) of galactocerebroside and psychosine destroys myelin sheath

39
Q

Krabbe disease - accumulation of

A
  1. galactocerebroside

2. psychosine

40
Q

Krabbe disease - characteristic cells

A

Globoid cells

41
Q

Krabbe disease - findings (symptoms, signs, histology etc)

A
  1. peripheral neuropathy
  2. Developmental delay
  3. Optic atrophy
  4. Globoid cells
42
Q

Metachromatic leukodystrophy - is a ……disease / mode of inheritance / mechansim

A

lysosomal storage disease / AR

deficiency of arysulfatase A (Most commonly)

43
Q

Metachromatic leukodystrophy - pathophysiology

A

deficiency of arysulfatase A –> buildup (accumulation) of sulfatides –> impaired production and destruction of myelin sheath

44
Q

Metachromatic leukodystrophy - findings (symptoms, signs, histology etc)

A
  1. central and peripheral demyelination with ataxia

2. dementia

45
Q

Progressive multifocal leukoencephalopathy - mechanism/definition

A

demyelination of CNS due to destruction of oligodendrocytes

46
Q

Progressive multifocal leukoencephalopathy - associations, risk factors etc

A
  1. associated with JC virus
  2. Seen in 2-4% of AIDS patients
  3. increased risk with natalizumab, rituximab
47
Q

Progressive multifocal leukoencephalopathy - course of the disease

A

rapidly progressive, usually fatal

48
Q

Acute disseminated (postinfectious) encephalomyelitis is usually caused by (examples)

A
  1. infection –> measles, VZV

2. certain vaccinations –> rabies, smallpox