Demyelination Flashcards

(102 cards)

1
Q

AIDP sx

A

ascending paralysis, minor sensory sx

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2
Q

AMAN

A

flaccid paralysis often follows C. jejuni infection

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3
Q

AMSAN

A

acute (less than 1 wk) quadriparesis, ventilation often required

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4
Q

miler fisher syndrome sx

A

ataxia, areflexia, ophthalmoplegia

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5
Q

what is most common form of Guillain-Barre?

A

AIDP

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6
Q

cause of AIDP GBS

A

auto immune response directed against schwann cell membranes

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7
Q

cause of AMAN GBS

A

attacks motor nodes of Ranvier

theory due to attack against axoplasm of peripheral nerves

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8
Q

what type of GBS can be seasonal and have rapid recovery?

A

AMAN

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9
Q

what is responsible for salutatory conduction?

A

nodes of Ranvier

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10
Q

Miller Fisher syndrome first attacks ___

A

eyes first

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11
Q

AMSAN due to ___

A

autoimmune response directed against the axoplasm of peripheral nerves

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12
Q

AMSAN recovery is___

A

slow and often incomplete

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13
Q

BBE is ___

A
a variant of GBS 
Bickerstaff's brainstem encephalitis 
will have + Babinski's sign 
Large irregular lesions in the brainstem 
Yet pretty good prognosis 
Extremely rare
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14
Q

Acute pan autonomic neuropathy

A

the most rare variant of GBS

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15
Q

____ is associated with high mortality rate

A

Acute panautonomic neuropathy

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16
Q

Sx of Acute panautonomic neuropathy

A

autonomic symptoms: orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dry eyes

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17
Q

GBS risk increases by ___ with every 10 year increase in age beyond ____

A

20% beyond first decade of life

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18
Q

main theory of GBS cause

A

infection evokes immune response which cross reacts with peripheral nerve components

causes acute polyneuropathy

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19
Q

what bacterias are associated with GBS?

A

C. jejuni - top one
CMV, EBV,
less common: HSV, Hep A, Hep B, Hep C, influenza, ZIkea, Dengue

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20
Q

GBS linked to ___ and ___

A

Hodgkins lymphoma, SLE

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21
Q

Main progression GBS

A

symmetrical weakness
**lower limbs first then upward..only 10% in arms or facial muscles

Occurs from hours to days

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22
Q

____of GBS pts require ventiliation to assist with respiration

A

30%

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23
Q

eye mvmts are not common in _____ but more common in ____ variant of GBS

A

ascending GBS

MIller Fisher variant

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24
Q

sensory loss associated with GBS

A

Propioception and areflexia
loss of pain and temp usually mild
Pain - sore/aching
loss of autonomic function

(wide BP fluctuations, orthostatic hypotension, cardiac arrhythmias)

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25
cardinal features of GBS
progressive, symmetric muscle weakness and afrelexia | weakness from difficulty walking to paralysis
26
Diagnostics for GBS
CSF analysis from LP Electro diagnostic tests of nerves and muscles are common tests ordered in diagnosis of GBS (nerve conduction abnormalities NCS and EMG)
27
what level do a LP at?
L3-L4
28
what would CSF show for GBS?
elevated protein 45-200 as high as 1000 mg/dL | normal WBC count of less than 5 cells/ mL
29
antibodies of AIDP
nonspecific
30
antibodies of AMAN
IgG anti GM and IgG anti GD
31
antibodies of AMSAN
IgG anti GM
32
antibodies of Miller Fisher Syndrome
IgG anti-GQ
33
what 4 polyneuropathies also mimic GBS?
Heavy metal poisoning (lead/arsenic) N-hexane - glue sniffing Vasculitis Lyme Disease
34
spinal cord disorders confused with GBS
compression, myelitis, lesions ruled out with MRI
35
do you automatically do a MRI for GBS?
no - do history | usually spinal cord disorder will have additional sx rather than specific ascending paralysis like GBS
36
how to treat GBS
``` Plasmapheresis IV immunoglobulin (IVIg) Supportive Care - resp. monitor/ intubation Autonomic failure - monitor cardiac etc Rehab ``` majority pts will recover completely
37
complication of GBS
neuropathic pain - gabapentin NSAIDS Narcotics - careful they already have depressed respiratory system
38
____ is most common disorder of neuromuscular transmission
Myasthenia Gravis
39
characteristics of MG
weakness and fatigue of skeletal muscle | worsening of contractile force no sensation of tiredness
40
2 forms of MG
Ocular weakness: eyelids and EOM | Generalized: ocular, bulbar, limb, and respiratory
41
MG caused by___
block of NT ACTH | Immune mediated decrease in number of AChR and NMJ
42
women with MG affected around ages ____ while men ____
women: 20-30s men: 50-60s yet overall incidence much higher later in life overall sx appear post 50
43
10% of MG ppl also have___
thyroid disorder
44
__ ab linked ot MG
muscle specific kinase: MuSK
45
seronegative MG means
no AchR or MuSK Abs
46
MG is characterized by___
fluctuating, fatigable weakness of commonly used muscles...
47
most common MG sx
diplopia
48
what are bulbar sx?
brainstem sx
49
___ can exacerbate MG sx
pregnancy
50
MG Diagnostic test options (4)
Tensilon (edrophonium) test Labs: - AChR- binding Ab, AChR modulating Ab - test for MuSK if they do not have AChR Electrophysiological tests Imaging studies: CT or MRI for thymoma
51
thymus tumor found in ___ of pts with MG
15%
52
what makes MG antibodies?
thymocyte (thymus) T cell mediated
53
4 MG Tx
1. Treat sx: anticholinesterase agents 2. Chronic immunomodulating agents: - glucocorticoids - nonsteroidal immunosuppression 3. Rapid immunomodulating treatments - plasmapheresis and IV IgG 4. Surgical treatment - thymectomy
54
examples of nonsteroidal treatments
cyclosporine, azathioprine, rituximab
55
MS only affects the ___
CNS
56
____ is a heterogenous disease with variable clinical and pathological features
MS
57
___ is the leading cause of neurologic disability in young adults
MS
58
MS affects ___ more than ____
women 2-3 times more than men
59
prevalence of MS increases with ___
increasing distance from the equator - may be related to vitamin d low levels - infectious agents
60
average age of MS onset
30 yrs
61
relapsing remitting MS onset is
29yrs
62
progressive onset occurs around
40 yrs
63
MS is caused by multiple factors including
environmental factors Genome allelic variants Epigenetic, postgenomic, and regulatory events
64
4 major aspects to MS pathogenesis
1. inflammation 2. blood/brain barrier disruption 3. demyelination 4. axon degeneration
65
MS leads to a loss of ____
oligodendrocytes and myelin | - leads to axonal degeneration etc.
66
what is a MS plaque
multiple pink or grey areas seen in the white matter upon autopsy
67
what regions of the brain are most affected by MS?
exposed regions near ventricles corpus callosum optic nerves
68
MS destroys the __ matter of the brain
white
69
what is lhermittes sign
an electric shock radiating down the spine or into limbs with neck flexion seen with MS
70
___ occurs in up to 50% of MS
vertigo | - CN 8 involvement
71
unilateral optic neuritis MS sx
eye pain, vision loss, horizontal nystagumus, opthalmoplegia
72
opthalmoplegia
oscillating eyes of equal velocity around a center point
73
sensory sx of MS
numbness, tingling, tightness, coldness, swelling, intense itching in cervical dermatomes
74
Physical exam findings of MS
nystagmus, impaired joint position sense, spasticity, impaired pain or temp sense etc
75
4 major MS types
relapsing remitting - most common progressive relapsing secondary progressive primary progressive
76
relapsing remitting
upredictable attacks that may or may not leave permanent deficits followd by remission periods - overall trend is still + progression
77
progressive relapsing
steady decline since onset with super imposed attacks
78
secondary progressive MS
initial relapsing remitting MS that suddenly begins to have decline without periods of remission
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primary progressive
steady increase in disability without attacks
80
Diagnostic MS
MRI - lesions | clinicial - evolves over space and time etc
81
3 ways tx MS
``` treat acute exacerbations - high dose corticosteroids: methylprednisolone - plasmapheresis - may be helpful disease modifying drugs - lots options! symptomatic therapies ```
82
disease modifying drug options for MS
``` B interfereons Glatiramer acetate Methotrexate Cladribine Mitoxantrone Cyclophosphamide ```
83
__ is only fda approved therapy for secondary MS
Mitoxantrone (Novantrone)
84
other immune modulating treatment options for MS
stem cell transplantation Intravenous IgG Lymphoid irradiation
85
ALS
amyotrophic lateral sclerosis
86
Diagnosis ALS
from combo of clinical examination finding of amyotrophy or muscle wasting with the finding of lateral sclerosis
87
ALS aka
Lou Gehrigs disease | Motor neuron disease MND
88
ALS has no___ or ___
cause or cure
89
typical lifespan of ALS
3-5 yrs
90
incidence of ALS may be lower in what 3 racial groups
Asian, African and Hispanic
91
what geographic area has ALS higher incidence
gaum
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Estabilished risk factors ALS
age fH | smoking, heavy metal, repetitive muscle use
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6 genes related to ALS
``` SOD1 TARDBP ANG FUS OPTN SETX ```
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theory behind SOD1 mutation and ALS
superoxide dismutase mutations | - leads to elevated free radicals in body
95
ALS characterized by ___
upper motor neuron degeneration and death Glial cells replace the neurons leads to retrograde axonal loss and gliosis
96
what tract does ALS affect?
corticospinal tract which is our largest descending motor pathway
97
sx of ALS
upper and lower motor neuron signs and sx - asymmetric early on in dzz - Upper findings: weakness with slow, hyperreflexia, spasticity - Lower findings: weakness, atrophy, amyotrophy fasciculation's (twitch)
98
what 3 things are spared in ALS
EOM, cognitive, and sensory functions are spared
99
definite dx of ALS
upper and lower motor neuron signs in 3+ spinal regions (cervical, thoracic, lumbrosacral)
100
what do you have to consider if you are thinking ALS?
Rule out structural lesions or tumors affecting the brainstem or spinal cord - MRI can exclude these possibilities
101
pharm for ALS
Riluzole (Rilutek, Teglutik) - only FDA approved treatment for ALS
102
___ treatment for ALS should be proactive
swallowing treatment - may require PEG or semi liquid diet