NMJ Electrophys and Nerve growth Flashcards

1
Q

describe a growth cone in 2 sentences

A

expansion on the tip of axon that senses environment. dictates growth via cytoskeletal remodeling in response to trophic factors.

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2
Q

ensure survival of neuron once proper synapse has been made

A

neurotrophins

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3
Q

3 things trophic interactions are responsible for

A

1) matching neurons to available target space 2) regulating the degree of innervation of individual afferents and their postsynaptic partners 3) modulating growth and shape of axon and dendritic branches

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4
Q

explain the neural response to botox administration (once vesicle fusion is blocked resulting in denervation); use trophic and muscle in your answer

A

in response to trophic factors produced by now denervated muscles, nerves try to re-inervate the muscle therefore they sprout terminal branches

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5
Q

flexing all toes in response to …..

A

stroking of lateral aspect of plantar foot

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6
Q

babinski reflex:

A

dorsiflexion of big toe and slight fanning of the toes upon plantar stroking of foot.

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7
Q

babinski normal in ages

A

normal up to 2 yrs old

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8
Q

in adults babinski indicates (less than sentence)

A

UMN lesion

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9
Q

describe wallerian degeneration in terms of response to …., and what part of …. is affected

A

response to cut or crush, distal parts of PNS axon degenerate

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10
Q

what two cell types cause breaking down of axon and myelin

A

proliferating schwann cells, and macrophages

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11
Q

describe retrograde degeneration using terms: weeks, cell body, and schwann cells

A

within 1-2 weeks of injury, proximal to cut, cell body swells and nissl bodies disperse, dendrites may be replaced by schwann cells

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12
Q

what are two (either or) requirements for regeneration?

A

endoneurial sheath intact OR severed nerves <2mm away

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13
Q

saturday night palsy is example of … type of injury via mechanism of ….

A

neuropraxia via temporary interference with fxn but no degen (e.g transient sustained pressure)

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14
Q

axonotmesis and mechanism

A

nerve injury where axon damaged but endoneurial sheath and schwann cells are fine; crush/ischemia

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15
Q

neurotmesis

A

nerve injury where axon and sheath and myelin are severed

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16
Q

what is first sign of regeneration of neuron

A

the cell body’s nissl bodies structure recovers

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17
Q

explain role of schwann cells in neuron regeneration use words such as bridge, trophic, and surround

A

schwann cells proliferate and lose their myelin. they then surround the basal lamina of endoneurium and act as a bridge. They provide trophic support as well.

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18
Q

explain axon sprouting in context of regeneration of nerve contrast this with botox poisoning

A

axon makes numerous sprouts on proximal end of cut, that all advance. only one sprout ends up innervating the end organ botox the neuron isnt regenerating strictly, but is merely sprouting in response to the denervated muscle trophic factors

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19
Q

when more than one axon is injured, and if multiple axons enter an endoneurial tube during regeneration, what can happen?

A

the wrong neuron type can pair with the wrong end organ.

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20
Q

why is recovery better in nerves that all all sensory or all motor?

A

if things get scrambled, at least same type of neuron

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21
Q

what is a mechanism by which a neuroma can form?

A

during regeneration if some of the terminal sprouts escape the epineurial sheath they can get tangled up and form neuroma.

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22
Q

what surrounds individual nerve fascicles?

A

perineurium

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23
Q

what surrounds groups of fascicles?

A

epineurium

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24
Q

when is myelin put on the axon in terms of the regeneration process?

A

when it reaches target organ

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25
axonal loss results in ... on NCS
decreased amplitude
26
demyelination loss results in .... on NCS
longer latency and slower velocity. also temporal dispersion for acquired demyelinating
27
vasculitic neuropathy is classified as a
multiple mononeuropathy
28
diabetes and etoh neuropathy are classified as a
polyneuropathy
29
distal muscle pain and weakness are more characteristic of ....; name an exception
neuropathies more so than myopathies; exception is inclusion body myositis
30
name 4 signs and sxs consistent with myopathy
muscle weakness (esp proximal); muscle atrophy; early fatigue; myotonia
31
what is a test to order to assess muscle damage?
CK
32
clinical criteria for rhabdo
myoglobinuria or elevated CK
33
what is acute complication of rhabdo?
ARF
34
can you have muscle atrophy without a primary myopathy?
yes, in denervation atrophy
35
do steroids cause nerve damage?
no just type II muscle fiber atrophy
36
do mitochondrial myopathies cause nerve damage?
no
37
list some diseases that are neuropathic but can cause muscle atrophy
ALS, SMA, poliomyelitis, motor neuron disease
38
grouped atrophy is a result of ..... (acute or chronic) denervation?
chronic
39
fiber type grouping is a result of ..... following acute denervation
re-innervation
40
example etiologies of fiber type grouping
CMT disease compression neuropathy poliomyelitis nerve transection w/surgical repair
41
normal muscle biopsy in these conditions
botulinum toxin, myasthenia gravis, lambert eaton
42
grouped atrophy often seen in
ALS, SMA, motor neuron disease
43
larger amplitude and fewer peaks (Reduced recruitment), longer duration
neurogenic diseases
44
smaller amplitude and more peaks (early recruitment), shorter duration
myopathic disease
45
early and reduced recruitment examples of (..APs)
MUAPs
46
diminished amplitude of muscle action potential upon repeated stimulation is consistent with
myasthenia gravis
47
increased amplitude of muscle action potential upon repeated stimulation is consistent with
lambert eaton
48
explain cause of myasthenia gravis and lambert eaton
mg: abs to post synaptic ach receptor le: abs to presynaptic calcium channels
49
only two nerve fiber types that are not myelinated:
c and IV
50
ice pack test for myasthenia gravis
cold enough on eyes to stop ach esterase therefore improving ptosis after ice applied
51
get stronger after admin of ach esterase indicates this disease
myasthenia gravis
52
thymectomy, plasmapheresis, and immune suppression are all ways to treat ....
myasthenia gravis
53
what is dystrophin?
protein that holds actin cytoskeleton with the sarcolemmal membrane/ECM.
54
inheritance pattern for DMD and BMD
x linked recessive
55
explain gowers sign
pt must push on floor or on own legs to get up 2/2 proximal thigh weakness.
56
additional complications of DMD
cardiac and pulm
57
list 3 histopathological findings in DMD and less so in BMD
1. endomysial fibrosis 2. internalized nuclei 3. necrosis and fat buildup
58
duchenne age of onset and course
onset 1-5 yrs old proximal muscle weakness; delayed motor skills; 10-12 y/o wheelchair dependent; death 20-40 y/o
59
can you essentially live normal life span with becker? when is onset?
yes onset: late childhood or early adolescence
60
most common adult muscular dystrophy
myotonic muscular dystrophy
61
myotonic muscular dystrophy pathophys and muscles most affected
trinucleotide repeat in myotonin; anticipation; demonstrates myotonia; cataracts; progressive weakness and muscle degeneration; facial and sternomastoid
62
hallmark histopath of myotonic dystrophy
many internal nuclei and ring fibers
63
McCardle's disease clinical presentation (3 facts) +inheritance and enzyme deficiency +histopath
fatigue after brief intense exercise; second wind; \<15 y/o male predominant; AR inheritance; glycogen phosphorylase enzyme glycogen in subsarcolemma
64
Pompe's disease clinical presentation of two main types + histopath +inheritance + enzyme deficiency
infantile/severe: weakness hypotonia, death \<1 yr adult/child/moderate: proximal muscle weakness histopath: glycogen in membrane bound vacuole enzyme: acid maltase /lysosome trafficking
65
CPTII disease enzyme
lipid storage myopathy from carnitine palmitoyl transferase II
66
CPTII clinical presentation and inheritance + 3 more sxs
infants: often fatal adults: myopathy and myoglobinuria after exercise AR inheritance + cardiomyopathy, liver failure, seizures
67
presents in young adulthood with proximal muscle weakness and sometimes with EO muscle involvement
mitochondrial myopathies
68
parking lot inclusions and ragged red fibers are
mitochondrial myopathies
69
systemic weakness and facial muscles also ptosis and sometimes diplopia
myasthenia gravis
70
Kearns Sayre syndrome manifestations (5)
external opthalmoplegia that is progressive over years. + pigmentary retinopathy, cardiac conduction, ataxia, high protein csf (cf cidp)
71
dermatomyositis: clinical presentation age of onset responsive to steroids? histopathologic findings
proximal symmetric muscle weakness heliotrope rash gottron papules juvenile and adult yes perifascicular atrophy and sometimes perimysial inflammation (CD4)
72
polymyositis: clinical presentation age of onset responsive to steroids? histopathologic findings
Insidious onset w/ pelvic and pectoral girdle muscle weakness \> 1 month adult (45-60 y/o F\>M) yes endomysial cd8 infiltrate
73
inclusion body myositis clinical presentation age of onset responsive to steroids? histopathologic findings
distal muscle weakness; forearm atrophy common inflammatory myopathy affecting adults \>50 year old M\>\>F NO steroids. rimmed vacuoles and amyloid containing lesions; endomysial infiltrates
74
steroids cause atrophy of ....
type II muscle fiber
75
onion bulb sign found in
cidp and hereditary neuropathies (eg CMT)
76
clinical presentation of GBS
paresthesias and pain with symmetric weakness, decreased DTRs; starts distally and spreads proximally (ascending); sxs onset within 4 weeks; 1/3 require intubation; molecular mimicry esp/ w/ campy?
77
tx of GBS:
IvIG or plasma exchange
78
epi of GBS:
young adults and \>55 y/o most commonly
79
differentiate onset of CIDP from GBS and degree of sensory findings
weeks to months for CIDP with progression or relapse beyond 8 weeks; prominent sensory findings in CIDP
80
distal sensory polyneuropathy
diabetic neuropathy
81
diabetic neuropathy affects (size, myelinization status)
small unmyelinated or myelinated
82
3 histo hallmarks of diabetic neuropathy
axonal loss; axonal regeneration; thickened/hyalinated capillary walls
83
3 risk factors for vitamin b12 def
elderly, hiv, impaired intrinsic factor secretion
84
sxs of vitamin b12 neuropathy (myelin sheath destruction)
symmetric paresthesia, gait problems, LE\>UE
85
CMT1a disease gene lesion
duplication of PMP22 gene on chromosome 17 (a myelin protein)
86
clinical presentation of CMT1a + cmt Histo buzzword
progressive weakness and loss of sensation; peroneal atrophy; pes cavus; reduced DTRs; onion bulb on histo
87
differentiating factors clinically b/w MG and LEMS
MG: more ptosis and face LEMS: often legs. PRECEDES CANCER
88
cardiomyopathy in
duschennes, pompe's, CPTII, and (AV block for Kearns Sayer)