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Physiology > Neuromuscular disorders > Flashcards

Neuromuscular disorders Flashcards

1
Q

What are upper motor neurons?

A

pathways completely contained within the CNS

cerebral cortex –> ventral horn of spinal cord

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2
Q

Primary role of upper motor neurons

A

influence

  1. reflex arcs,
  2. low-level control centers &
  3. motor neurons (some sensory)
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3
Q

Corticospinal tract supplies what

A

voluntary muscles of the trunk and extremities

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4
Q

corticospinal pathway

A

precentryal gyrus
internal capsule - midbrain - pons
90% decussate in medulla –> lateral corticospinal
10% decussate low @ s/c –> ventral corticospinal

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5
Q

corticospinal synapse w/

A

spinal nerves

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6
Q

ventral corticospinal tract ?

A

posture

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7
Q

corticobulbar tract supplies what

A

voluntary muscles of head

same pathway of corticospinal UNTIL brainstem

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8
Q

corticobulbar pathway

A

precyntral gyrus
CN 3, 4, 6, 9, 10, 11 bilaterally
CN 7, 13 unilaterally (facial & hypoglyssal)

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9
Q

where are lower motor neurons located?

A

brain stem or spinal cord

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10
Q

what do lower motor neurons do?

A

direct influence on muscles! they send out axons via nerves in PNS to synapse on sk. muscles

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11
Q

lower motor neurons passing through spinal nerves control…

A

muscles of limbs/trunk

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12
Q

lower motor neurons passing through cranial nerves control….

A

sk muscles of head/neck

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13
Q

umn (pyramidal cells) motor cortex

A
  • muscle groups affected, mild weakness, minimal disuse muscle atrophy
  • no fasiculations
  • hypertonia & spasticity
  • increased stretch reflex, + babinski
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14
Q

lmn ventral horn (S/C) and motor nuclei (brainstem)

A

individual muscles affected

  • mild weakness, marked muscle atrophy
  • fasciculations
  • decreased stretch, hypotonia, flaccid
  • no babinski
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15
Q

name 5 umn diseases

A

cp, cva
ms
parkinson’s
huntington’s

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16
Q

cp patho

A

non-progressive disorder caused by injury/abnormal development in the immature brain before, during, or after birth for up to a year

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17
Q

what path is affected by cp

A

corticospinal

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18
Q

s/s cp

A

muscle weakness
no speech, drooling
exaggerated DTR, spasticity, rigidity
scoliosis, contractures

vision/hearing
swallowing , sz, slow
gerd

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19
Q

cp treatment (4)

A 
no cure - s/s treatment only
dorsal rhizotomy
antireflux
intrathecal baclofen
botulinum toxin
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20
Q

cp anesthesia

A 
hold sedatives, opioids (short acting only)
vascular access sucks
difficult airway
RSI
sux - usually ok, but 30% do have extrajunctional receptors
ndmr - if on anticonvulsants, resistant
mac decreased 20 - 30%
bleeding - antisz meds
hypothermia - no fat
dry
slow emergence
high risk for latex
no regional d/t scoliosis etc
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21
Q

m/s patho

A

autoimmune
demyelination, inflammation, axonal damage of CNS
PNS ok

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22
Q

s/s ms

A 
paresthesia
muscle fatigue
muscle spasms
visual problems
ans dysfunction
bulbar muscle dysfunction
cognitive dysfunction - advnaced
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23
Q

tx m/s

A 
diazepam
dantrolene
baclofen
steroids
immunosuppresants
cd20 monoclonal abs, interferons, glatiramir
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24
Q

s/s of m/s exacerbated by

A

stress
hyperthermia
infection
hyponatremia

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25
Q

anesthesia & m/s

A 
no sux, scopolamine, atropine
nmdr: be careful
no spinal block
epidural is ok
aspiration, dvt risk
stress dose steroids
exaggerated hypotensive effect
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26
Q

anterior cerebral artery

A

contralateral leg weakness

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27
Q

mca

A

contralateral hemiparesis and hemisensory deficit
(face & arm > leg)
contralateral visual defect

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28
Q

posterior ca

A

contralateral visual defect + hemiparesis

29
Q

penetrating arteries

A

contralateral hemiparesis & hemisensory deficits

30
Q

basilar artery

A

oculomotor deficits
ataxia
crossed sensory/motor

31
Q

vertebral artery

A

lower cranial nerve deficits
ataxia
crossed sensory

32
Q

cva tx

A

asa
tpa (ischemic)
surgery

33
Q

cva anesthesia

A 
rsi
dvt risk (use regional!)
b/s
b/p
initially, pt has htn - don't rapidly decline their bp b/c cpp
34
Q

parkinson’s patho

A

loss of dopaminergic fibers in basal ganglia

unopposed ach

35
Q

s/s parkinsons

A

tremor during rest
rigid, akinesia
diaphragmatic spasm, dementia
facial immobility

36
Q

parkinson’s tx

A 
levodopa
carbidopa
amatadine
selegiline + rasagiline (avoid meperidine, check b/s)
COMti (lfts)
antiviral amantadine prolongs QT
37
Q

anesthesia & Parkinsons

A 
levodopa continue dos
hypotension + arrhythmias or htn
RSI, airway risk (post extubation stridor)
hard sticks
if on anticholinergic, ndmr less effective
SEVOFLURANE
ketamine if on ldopa
direct acting pressors
sux?
38
Q

huntington’s patho

A

degenerative disease of CNS

marked atrophy of caudate nucleus + putamen + globus

39
Q

s/s huntington’s

A

dementia - progressive
chorea, tremors, rigid
mood swings
cant talk or swallow

40
Q

anesthesia & huntingtons (3)

A

rsi
sux + nmdr prolonged
avoid reglan, anticholinergics. atropine

41
Q

lmn (6)

A 
mg
muscular dystrophy
myotonic dystrophy
mitochondrial d/o
g/b
spinal muscular atrophy
42
Q

myasthenia gravis

A

autoimmune (igg) against the nachr

43
Q

m/g s/s

A

diplopia, ptosis
fluctuating fatigue/weakness imrpoves w/rest
face weakness

44
Q

m/g tx

A 
pyridostigmine (cholinesterase inhibiotr)
plasmapheresis
steroids
immunosuppresants
thmectomy
45
Q

m/g and anesthesia

A

aspiration risk
ndmr sensitivity
regional preferred (amides)
resistant to sux

46
Q

lems s/s (3)

A

proximal muscles are affected most
weakness worse in a.m., gets better during the day
resp/diaphragm weakness and dysfunction

47
Q

lems & anesthesia

A

sux and ndmr sensitivity
bad reversal w/acei
lots have small cell carcinoma

48
Q

duchenne s/s

A 
kyphoscoliosis
degeneration of cardiac muscles/impaired conduction
gi hypomotility/decr. airway reflex
pulm htn
cognitive impairment
49
Q

becker s/s

A

less slowness

proximal muscle weakness

50
Q

tx of musc. dystrophy

A

steroids, biphosphates

mystatin or protease inhibitors

51
Q

m/d anesthesia

A

tiva
sensitive to ndmr
local/regional yay
aspiration risk

52
Q

myotonic dystrophy

A

hereditary disease of sk. muscle resulting in dysfunctional ca+ sequestration by SR. na/chl also involved

53
Q

myotonic dystrophy s/s

A

distal limb weakness, myotonia, face/thorax/intercostal/diaphragm

cardiomyopathy, conduction defects
endocrine
central sleep apnea
ptosis
male triad (balding, cataracts, sm. testes)
54
Q

myotonic dystrophy tx

A

procainamide/phenytoin
baclofen/dantrolene
carbamezepine/cardiac pacemaker

55
Q

myotonic dystrophy & anesthesia

A

no sux
do a tiva
myotonia aggravated by neo/physo
have pacing at bedside, they vagal lots

56
Q

mitochondrial d/o

A

heterogenous group of d/o of sk. muscle energy metabolism

mitochondria produce energy required by sk. muscle cells through the oxidation-reduction reactions of etc and oxidative phopohrylation

57
Q

mitochondrial d/o s/s

A

fatigue, muscle pain
hearing loss
balance, sz, learning deficits
organ problems

58
Q

mitochondrial d/o tx

A

give metabolites & cofactors
sodium bicarb/dichlloroacetate
ketogenic diet

59
Q

mitochondrial d/o anesthesia

A 
acidosis, dehydration
lactate high
avoid sux, LR, prop gtt
nmdr&LA - be careful
no bupi
60
Q

GB

A

sensory deficits, ans dysfunction

61
Q

GB tx

A

plasmapheresis
ivig
NO STEROIDS

62
Q

gb & anesthesia

A 
no sux
sensitive to ndmr
dvt increased risk
siadh
don't use indirect sympathomimetics
63
Q

spinal muscle atrophy

A

d/t deletions or mutations in the survival motor neuron gene. this gene product is involved in the formation of rna complexes & their trafficking out of the nucleus

loss of smn function promotes apoptosis of lmn & affect anterior horn of s/c

64
Q

sma & tx

A

spinraza/zolgensma/evrydsi

give abx early

65
Q

sma. & anesthesia

A

difficult intuation
no sux
nmdr prolonged

66
Q

als s/s

A

atrophy in hands
resting tachycardia
snesation ok

67
Q

als tx

A

riluzole, edavaone
spasmolytics
analgesics

68
Q

als anesthesia

A

avoid sux
nmdr prolonged
ans dysfunction
no spinal anesthesia

Physiology (5 decks)

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