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neurology FA > acute inflammatory demyelinating polyradiculopathy / Multiple sclerosis > Flashcards

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
Multiple sclerosis - treatment
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
acute inflammatory demyelinating polyradiculopathy - eye
papilledema
27
Adrenoleukodystrophy typically affects (why)
males | X-linked genetic disorder
28
Adrenoleukodystrophy - pathophysiology / mode of inheritance
X-linked genetic disorder --> disrupts metabolism of very-long-chain fatty acids --> excessive buildup (accumulation) in nervous system, adrenal glands and testes
29
Adrenoleukodystrophy - coarse of disease
Progressive disease that can lead to | 1. long-term coma/death and 2. adrenal crisis
30
demyelinating and dysmelinating diseases - types
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
Acute disseminated (postinfectious) encephalomyelitis?
multifocal periventricular inflammation and demyelination after infection or certain vaccination
32
Acute disseminated (postinfectious) encephalomyelitis is usually caused by (examples)
1. infection --> measles, VZV | 2. certain vaccinations --> rabies, smallpox
33
Acute disseminated (postinfectious) encephalomyelitis - presentation
rapidly progressive multifocal neurologic symptoms
34
Charcot-Marie-Tooth disease - AKA ... / mode of inheritance
hereditary motor and sensory neuropathy (HMSN) | AD
35
Charcot-Marie-Tooth disease - definition and mechanism
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
Charcot-Marie-Tooth disease - symptoms and signs
1. foot deformities (pes cavus) 2. lower extremities weakness 3 .sensory deficits
37
Krabbe disease - is a ......disease / mode of inheritance / mechansim
lysosomal storage disease / AR | deficiency of galactocerebrosidase
38
Krabbe disease - pathophysiology
deficiency of galactocerebrosidase --> buildup (accumulation) of galactocerebroside and psychosine destroys myelin sheath
39
Krabbe disease - accumulation of
1. galactocerebroside | 2. psychosine
40
Krabbe disease - characteristic cells
Globoid cells
41
Krabbe disease - findings (symptoms, signs, histology etc)
1. peripheral neuropathy 2. Developmental delay 3. Optic atrophy 4. Globoid cells
42
Metachromatic leukodystrophy - is a ......disease / mode of inheritance / mechansim
lysosomal storage disease / AR | deficiency of arysulfatase A (Most commonly)
43
Metachromatic leukodystrophy - pathophysiology
deficiency of arysulfatase A --> buildup (accumulation) of sulfatides --> impaired production and destruction of myelin sheath
44
Metachromatic leukodystrophy - findings (symptoms, signs, histology etc)
1. central and peripheral demyelination with ataxia | 2. dementia
45
Progressive multifocal leukoencephalopathy - mechanism/definition
demyelination of CNS due to destruction of oligodendrocytes
46
Progressive multifocal leukoencephalopathy - associations, risk factors etc
1. associated with JC virus 2. Seen in 2-4% of AIDS patients 2. increased risk with natalizumab, rituximab
47
Progressive multifocal leukoencephalopathy - course of the disease
rapidly progressive, usually fatal
48
Acute disseminated (postinfectious) encephalomyelitis is usually caused by (examples)
1. infection --> measles, VZV | 2. certain vaccinations --> rabies, smallpox

neurology FA (31 decks)

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