Unit 7 - Neuro - Musculoskeletal Flashcards
1
Q
patho of myasthenia gravis
A
Autoimmune destruction of post-junctional, nicotinic ACh receptors at NMJ via IgG
2
Q
how does myasthenia gravis manifest
A
skeletal muscle weakness
Key: skeletal muscle weakness becomes worse later in the day
3
Q
surgery that can relieve myasthenia gravis symptoms
A
thymectomy
↓ circulating Anti-AChR IgG in most patients
4
Q
earliest signs of myasthenia gravis
A
diploplia, ptosis
5
Q
how does bulbar muscle weakness manifest in myasthenia gravis
A
dysphagia, dysarthria, difficulty handling saliva
6
Q
5 things that exacerbate myasthenia gravis symptoms
A
- pregnancy
- infection
- electrolyte abnormalities
- surgical/psychological stress
- aminoglycoside antibiotics
7
Q
why might neonates of moms with myasthenia gravis require airway management
A
Anti-AChR IgG antibodies cross placenta & cause weakness in 15-20% of neonates for 2-4 weeks
8
Q
1st line treatment of myasthenia gravis
A
PO pyridostigmine
9
Q
what is the Tensilon test
A
To differentiate pyridostigmine overdose vs. myasthenic crisis, admin. edrophonium 1-2 mg IV
If muscle strength improves, pt had myasthenic crisis
10
Q
immunosuppressants used in treatment of myasthenia gravis
A
- corticosteroids
- cyclosporine
- azathioprine
- mycophenolate
11
Q
use of plasmapheresis in myasthenia gravis
A
temporary relief during myasthenic crisis or before thymectomy
12
Q
how do pts with myasthenia gravis react to NMBs
A
Pts have fewer Nm receptors at NMJ
* Increased sensitivity to NDNMBs (reduce dose by 1/3 to 2/3)
* Decreased sensitivity to succs (also d/t impaired efficacy of pseudocholinesterase on pyridostigmine - 1.5-2 mg/kg for RSI)
12
Q
how do pts with myasthenia gravis react to NMBs
A
- Increased sensitivity to NDNMBs (reduce dose by 1/3 to 2/3)
- Decreased sensitivity to succs d/t impaired efficacy of pseudocholinesterase on pyridostigmine (1.5-2 mg/kg for RSI)
Pts have fewer Nm receptors at NMJ
13
Q
Increased risk for postop mechanical ventilation in myasthenia gravis pts
A
- Disease duration > 6 years
- Daily pyridostigmine > 750 mg/day
- Vital capacity < 2.9 L
- COPD
- Surgical approach: median sternotomy > transcervical thymectomy
14
Q
how do volatiles cause skeletal muscle relaxation
A
via action on ventral horn of spinal cord
15
Q
patho of Eaton-Lambert syndrome
A
IgG-mediated destruction of presynaptic voltage-gated Ca2+ channel at presynaptic nerve terminal
reduces amount of ACh released into synaptic cleft
15
Q
patho of Eaton-Lambert syndrome
A
IgG-mediated destruction of presynaptic voltage-gated Ca2+ channel at presynaptic nerve terminal
reduces amount of ACh released into synaptic cleft
16
Q
how are postsynaptic nicotinic receptors affected in eaton lambert syndrome
A
present in normal quantity & function
17
Q
presentation of eaton lambert syndrome
A
- Proximal muscles most affected
- Weakness generally worse in the morning and improves throughout the day
- ANS dysfunction
18
Q
s/s ANS dysfunction in eaton lambert syndrome
A
- orthostatic hypotension
- slowed gastric motility
- urinary retention
19
Q
treatment of eaton lambert syndrome
A
3,4-diaminopyridine (DAP)
increases ACh release from presynaptic nerve terminal & improves strengt
20
Q
how do pts with eaton lambert syndrome react to NMBs
A
Sensitive to succs + NDNMBs
volatiles typically provide enough relaxation
21
Q
diagnosis to consider when patient has suspected lung cancer undergoing mediastinoscopy, bronch, or thorascopy
A
up to 60% of pts with eaton lambert syndrome have small oat cell carcinoma
22
Q
region affected by myasthenia gravis vs. eaton lambert sydrome
A
MG: postsynaptic Nm receptor
ELS: voltage gated Ca channel
23
ACh in myasthenia gravis vs. eaton lambert sydrome
MG = decreased response to ACh
ELS = decreased ACh release
24
NMJ region affected by myasthenia gravis vs. eaton lambert sydrome
MG: postsynaptic at motor end plate
ELS: presynaptic neuron
25
effectiveness of AChE inhibitors in region affected by myasthenia gravis vs. eaton lambert sydrome
MG: adequate
ELS: poor
26
patho of guillain barre syndrome
* Peripheral polyneuropathy characterized by immunologic destruction of myelin in peripheral nerves
* AP can’t be conducted
27
presentation of Guillain-Barre Syndrome
* Flu-like illness usually precedes paralysis by 1-3 weeks
* Typically persists ~2 weeks, full recovery in ~4 weeks
28
common etiologies of Guillain-Barre Syndrome
* Common: Campylobacter jejuni bacteria, EBV, CMV
* also: vaccinations, surgery, and lymphomatous disease
29
treatment of guillain barre syndrome
plasmapheresis and IVIG
| steroids and interferon do NOT improve
30
s/s guillain barre syndrome
* flaccid paralysis that begins in distal extremities and ascends bilaterally towards proximal extremities, face, & trunk
* ANS dysfunction common
31
sensory deficits in guillain barre syndrome
paresthesia, numbness, pain
32
major anesthesia concerns with guillain barre syndrome
skeletal muscle denervation, impaired ventilation, ANS dysfunction
33
NMBs and guillain barre
* Avoid succs (risk hyperkalemia from proliferation of EJRs)
* Sensitivity to NDNMBs
34
why are guillain barre pts at high risk for HD instability under GA, position changes, PPV, and blood loss
ANS dysfunction
35
guillain barre pt response to indirect acting sympathomimetics
* Exaggerated response d/t upregulation of postjunctional adrenergic receptors
36
patho of Familial Periodic Paralysis
Characterized by acute episodes of skeletal muscle weakness accompanied by changes in serum K+ concentration
| Disorder of skeletal muscle membrane (↓ excitability), not NMJ
37
type of Familial Periodic Paralysis that's a calcium channelopathy
hypokalemic periodic paralysis
38
type of Familial Periodic Paralysis that's a sodium channelopathy
hyperkalemic periodic paralysis
39
how is Hypokalemic Periodic Paralysis diagnosed
if skeletal muscle weakness follows a glucose-insulin infusion (weakness with ↓ serum K+)
40
AVOID in Hypokalemic Periodic Paralysis
* glucose containing solutions
* K+ wasting diuretics
* beta 2 agonists
* succs
41
why should D5W be avoided in Hypokalemic Periodic Paralysis
D5W = insulin spike = K+ shifted into cells = hypokalemia
42
how is Hyperkalemic Periodic Paralysis diagnosed
if skeletal muscle weakness follows oral potassium admin (weakness after serum K+ increases)
43
AVOID in Hyperkalemic Periodic Paralysis
* succs
* K+ containing solutions like LR
44
treatment of Familial Periodic Paralysis
acetazolamide for both forms
45
why is acetazolamide used in treatment of familial periodic paralysis
creates non-anion gap acidosis
protects against hypokalemia & facilitates renal K+ excretion (protects against hyperkalemia)
46
AIs of familial periodic paralysis
* Avoid hypothermia at all costs (even when on CPB)
* Serum K+ monitoring indicated
47
how is MH characterized
disordered calcium homeostasis
48
how does Ca2+ enter myocyte when T-tubule is depolarized in MH
via dihydropyridine receptor
49
receptor activated by MH that instructs SR to release too much calcium
RyR1
50
why is there a substantial amount of ATP consumed in MH
More Ca2+ to engage with contractile elements + cell attempts to return excess to SR via SERCA2 pump
51
what causes hyperkalemia and myoglobinemia in MH
Sarcolemma breakdown = K+ and myoglobin to enter systemic circulation
52
effects of increased intracellular calcium in myocyte
* Rigidity from sustained contraction
* Accelerated metabolic rate, rapid ATP depletion
* Increased O2 consumption
* Increased CO2 and heat production
* Mixed respiratory and lactic acidosis
* Sarcolemma breaks down
* K+ and myoglobin leak into systemic circulation
53
only 3 conditions definitively assoc with MH
1. King-Denborough syndrome
2. Central core disease
3. Multiminicore disease
54
why should volatiles and succs be avoided in DMD
can have MH-like syndrome from rhabdo (not true MH)
| absence of dystrophin destabilizes sarcolemma - more calcium can enter c
54
why should volatiles and succs be avoided in DMD
can have MH-like syndrome from rhabdo (not true MH)
55
patho of MH type reaction in DMD patients
* Absence of dystrophin destabilizes sarcolemma during muscle contraction, ↑ membrane permeability
* Extracellular Ca2+ free to enter cell (can ↑ rate of metabolism)
* Intracellular K+ is free to exit cell (can result in hyperkalemic cardiac arrest)
* Myoglobin free to exit cell
56
MH risk factors
* Geography: families from Wisconsin, Nebraska, West Virginia, & Michigan at ↑ risk
* Male sex
* Youth
57
early s/s MH
* tachycardia
* tachypnea
* masseter spasm
* warm soda lime
* irregular HR
58
late signs of MH
* muscle rigidity
* cola-colored urine
* coagulopathy
* irregular HR
* hyperthermia
59
most sensitive indicator of MH reaction
EtCO2 that ↑ out of proportion to Vm
60
what is trimus
tight jaw that can still be opened (normal response to succs)
61
what is masseter rigidity
tight jaw that can’t be opened
62
what causes masseter spasm
↑ Ca2+ in myoplasm
site of action distal to NMJ so NMB will not relax jaw
63
gold standard for diagnosing MH
Caffeine-Halothane Contracture Test
64
who should have a Caffeine-Halothane Contracture Test
Anyone who has experienced MH or masseter spasm should be tested
65
who should have a Caffeine-Halothane Contracture Test
Anyone who has experienced MH or masseter spasm should be tested
66
MH differential
* thyroid storm
* neuroleptic malignant syndrome
* sepsis
* pheochromocytoma
* serotonin syndrome
* heat stroke
* metastatic carcinoid
* cocaine intoxication
67
how long should the machine be flushed for MH risk patient
20-100 min high flow (time depends on model)
68
charcoal filter prep for MH risk patient
Place Vapor-Clean charcoal filter on inspiratory and expiratory port of machine & flush with high FGF (>10 L/min) for 90 seconds prior to using machine on patient
69
external parts of machine that should be removed & replaced for MH risk patient
CO2 absorbent, circuit, bag
70
1st thing you should do with suspected MH reaction
Discontinue triggering agent (volatile or succs)
convert to TIVA
71
why should you hyperventilate when MH reaction suspected
facilitates CO2 elimination, enhances O2 delivery, drives K+ into cells
| 100% O2 at minimum FGF 10 L/min
72
why should you hyperventilate when MH reaction suspected
facilitates CO2 elimination, enhances O2 delivery, drives K+ into cells
| 100% O2 at minimum FGF 10 L/min
73
purpose of charcoal filters
Maintains halogenated anesthetic concentration < 5 ppm for up to 12 hours with FGF of 3 L/min
74
how often should Vapor-clean be changed during MH crisis
every hour
75
MOA of dantrolene
1) Reduces Ca2+ release from RyR1 receptor in skeletal myocyte
2) Prevents Ca2+ entry into myocyte, which ↓ stimulus for Ca2+ -induced- Ca2+ release
76
dantrolene dosing
* 2.5 mg/kg IV repeated q5-10 minutes
* Continued in ICU: 1 mg/kg q6H or 0.1-0.3 mg/kg/hr for 48-72 hours
77
common side effect of dantrolene admin
venous irritation
78
concentration & admin of Dantrium
20 mg/vial
reconstitute with 60 mL water
79
concentration & admin of Ryanodex
250 mg/vial
dilute in 5mL sterile water
80
when should you stop cooling pt with MH reaction
stop at 38 degrees C
81
treatment for lactic acidosis in MH
Bicarb 1-2 mEq/kg titrated to ABG and base deficit
82
treating hyperkalemia in MH
5-10 mg/kg CaCl
0.15 units/kg insulin + 1 mL/kg D50
83
how to prevent against dysrhythmias in MH
Class 1 antiarrhythmics
15 mg/kg procainamide, 2 mg/kg lidocaine
| do NOT give CCBs
84
how to prevent against dysrhythmias in MH
Class 1 antiarrhythmics
15 mg/kg procainamide, 2 mg/kg lidocaine
| do NOT give CCBs
85
desired UOP in MH
**>2** mL/kg/hr
Protects against renal injury from free myoglobin
| IV hydration, 0.25 mg/kg mannitol, 1 mg/kg Lasix
86
late, often fatal complication of MH
DIC
87
etiology of DMD
X-linked, recessive disease that result from absence of dystrophin protein
88
critical structural component of cytoskeleton of skeletal & cardiac muscle cells
dystrophin
89
function of dystrophin
helps anchor actin & myosin to cell membrane
90
consequence of absence of dystrophin in DMD
* destabilizes sarcolemma during muscle contraction, ↑ membrane permeability
* CK & myoglobin can enter systemic circulation
* calcium enters cell and can cause inflammation, fibrosis, cell death
91
why are pts with DMD predisposed to hyperkalemia with succs
destabilized sarcolemma allows EJRs to populate
92
best practice for anesthesia in DMD pts
TIVA, avoid succs
93
how does DMD present
atrophy and painless muscle degeneration
94
respiratory effects of DMD
* restrictive lung disease
* ↑ secretions
* risk PNA
* Respiratory muscle weakness
95
CV effects of DMD
Cardiac muscle degeneration = ↓ contractility, papillary muscle dysfunction, mitral regurg, cardiomyopathy, CHF
96
s/s cardiomyopathy with DMD
* resting tachycardia
* JVD
* S3/S4 gallop
* displacement of PMI
97
EKG changes in DMD
impaired conduction = **sinus tach, short PR**
Scarring of posterobasal aspect of ventricle = **increased R wave in lead I, deep Q waves in limb leads**
98
why are DMD pts at increased risk aspiration
Impaired airway reflexes, GI hypomotility
99
what should be included in preop workup of DMD pts
EKG, echo, cardiac MRI
100
what is kyphoscoliosis
posterior curvature of spinal column
101
T/F - patients with myasthenia gravis have decreased ACh
false - ACh is present in sufficient quantity but there aren't enough receptors to translate extracellular signal to intracellualr response
102
why are pts with myasthenia gravis at increased risk for aspiration
bulbar muscle weakness
103
why are pts with myasthenia gravis at increased risk for aspiration
bulbar muscle weakness
104
how should you ventilate a patient with suspected MH
hyperventilate with 100% FiO2 at minimum FGF 10 L/min
105
med class that is contraindicated in MH reaction
calcium channel blockers
106
if succs is indicated in a pt with myasthenia gravis, how should dose be adjusted
increase to 1.5-2 mg/kg
| increased resistance to succs
107
if succs is indicated in a pt with myasthenia gravis, how should dose be adjusted
increase to 1.5-2 mg/kg
| increased resistance to succs
108
another name for guillain barre
acute idiopathic polyneuritis
109
another name for guillain barre
acute idiopathic polyneuritis
110
how does paralysis normally present in GBS
Flaccid paralysis begins in the distal extremities and ascends bilaterally towards the proximal extremities, trunk, and face.
111
a
112
What changes should you make to the anesthesia machine if you suspect MH?
1. Turn off the volatile anesthetic.
2. Hyperventilate with 100% O2 at a minimum FGF of 10 L/min.
3. Connect a charcoal filter to the inspiratory and expiratory limb of the circuit.
113
respiratory changes that occur with DMD
* Kyphoscoliosis decreases pulmonary reserve
* Increased secretions and risk of pneumonia
* Respiratory muscle weakness
114
why are pts with DMD at risk for life-threatening hyperkalemia with succs admin
EJRs populate sacrolemma
115
most common etiologies of scoliosis
80% idiopathic
congenital, myopathic, neuropathic, traumatic
116
what does the Cobb angle describe
the magnitude of spinal curvature
117
significance of Cobb angle 40-50 degrees
indication for surgery
118
significance of Cobb angle 60 degrees
decreased pulmonary reserve
119
significance of Cobb angle of 70 degrees
pulmonary symptoms present
120
significance of Cobb angle of 100 degrees
gas exchange significantly impaired
higher risk of postop pulmonary complications
121
Cobb angle assoc with higher risk of postop pulmonary complications
100 degrees
122
early resp changes with scoliosis
* restrictive ventilatory defect (decreased FEV1 and FRC)
* decreased lung volumes and capacities (VC, TLC, FRC, RV)
123
late resp changes with scoliosis
V/Q mismatch
hypoxemia
hypercarbia
pHTN
cardiorespiratory failure/cor pulmonale
124
significance of hypercarbia in pts with scoliosis
impending respiratory failure
| late respiratory change
125
CV changes with scoliosis
* increased PVR, RVH
* MVP, mitral regurg, coarctation of aorta
126
most common CV comorbidity with scoliosis
MVP
127
nerve injuries assoc with prone positioning for scoliosis surgery
brachial plexus & ulnar
lateral femoral cutaneous (pressure on iliac crest)
peroneal (pressure lateral to fibula)
128
complications of scoliosis surgery assoc with hip flexion
femoral vein occlusion, DVT
129
which is better for pulmonary compliance in prone surgeries - Jackson or Wilson frame
Jackson
130
is decreased chest wall compliance an early or late complication of scoliosis
early
131
What are 6 potential risks of a wake-up test?
1. Inadvertant IV removal
2. Extubation
3. Awareness
4. Pain
5. Air embolism
6. Damage to spinal instrumentation
132
What region of the spinal cord do somatosensory evoked potentials monitor?
Posterior cord (dorsal column pathway)
133
next action if pt can move hands but not feet during wake up test for scoliosis surgery
surgeon should decrease distraction on spinal rods
134
AIs for scoliosis surgery
* avoid N2O (increases PVR)
* prepare for significant blood loss
* monitor serial ABGs and UOP to assess end organ perfusion
135
what do SSEPs assess
posterior spinal cord
**sensory** function
136
pathway assessed by SSEPs
dorsal column pathway
137
evoked potentials that monitor anterior spinal cord
MEPs (motor)
138
3 anatomic airway structures impacted by RA
1. TMJ
2. cricoarytenoid joint
3. C spine
139
Patho of RA
autoimmune disease that targets synovial joints
140
what causes systemic consequences of RA
infiltration of immune complexes into small and medium arteries
(leads to vasculitis)
141
hallmark of RA
morning stiffness that generally improves with activity
142
lymph node involvement in RA
enlargement of cervical, axillary, and epitrochlear lymph nodes
143
joints affected by RA vs OA
**RA**: PIP and MCP in hands and feet
**OA**: weight-bearing joints
144
4 airway risk factors assoc with RA
1. limited mouth opening (TMJ synovitis)
2. decreased diameter of glottic opening
3. atlanto-occipital subluxation
4. limited neck extension
145
how does cricoarytenoid arthritis present
hoarseness
stridor
dyspnea
146
DL findings that suggest cricoarytenoid arteritis
edema or erythema of vocal cords
147
why should a smaller ETT be used in pts with RA
smaller glottic opening - prevent laryngeal trauma
148
most common airway complication of RA
atlantoaxial subluxation & separation of atlanto-odontoid articulation
| due to weakening of transverse axial ligament
149
complication of neck movement in RA pts
risk for quadriparesis or paralysis
| neck flexion worse than extension
150
how is atlantoaxial subluxation & atlanto-odontoid articulation separation diagnosed in RA
distance between anterior arch of atlas and odontoid process is > 3 mm
151
how to best assess for atlantoaxial subluxation & atlanto-odontoid articulation separation in RA pts
lateral c spine xray
152
surgical correction of atlantoaxial subluxation & atlanto-odontoid articulation separation
odontoid decompression
posterior cervical fusion
153
where does subaxial subluxation most commonly occur in RA pts
C5-C6
154
what is the dens
superior bony projection of axis (C2)
155
function of dens
provide pivot to facilitate head rotation
156
borders of dens
anterior arch of atlas (C1) and transverse axial ligament
157
most common hematologic complication of RA
anemia
## Footnote
other heme complications - plt dysfunction assoc with NSAIDs
158
most common pulmonary complication of RA
pleural effusion
## Footnote
other pulm complications - interstitial fibrosis, limited chest wall expansion
159
ventilatory defect assoc with RA
restrictive
(diffuse interstitial fibrosis)
160
why are RA pts at risk of renal insufficiency
vasculitis
NSAIDs
161
why are pts with RA at risk of peripheral neuropathies
nerve entrapment
162
why can RA pts have limited chest wall expansion
costochondral involvement
163
what causes aortic regurg in RA pts
dilation of aortic root
164
CV complications of RA
pericardial effusion or tamponade
restrictive pericarditis
aortic regurg
valvular fibrosis
coronary artery arteritis
165
labs assoc with RA
* rheumatoid factor increased in 90% of pts
* increased CRP, ESR
166
MOA of antirheumetic drugs
inhibit TNF, interleukin-1 and 6, T cells, B lymphocytes
167
AEs of methotrexate
liver dysfunction
bone marrow suppression
168
med used in treatment of RA that prolongs duration of succs
cyclosporine
169
meds used in treatment of RA
* NSAIDs
* steroids
* immunosuppressants (methotrexate, cyclosporin, etanercept)
170
why are RA pts at increased risk of cancer and infection
antirheumatic drugs suppress immune system
171
most consequences of lupus are due to what?
antibody-induced vasculitis and tissue destruction
172
most common problems assoc with lupus
polyarthritis
dermatitis
## Footnote
only 30-50% develop "classic" butterfly rash
173
joints affected by lupus arthritis
can affect any joint but typically doesn't involve spine
174
airway complications assoc with lupus
**cricoarytenoiditis**
s/s: hoarseness, stridor, airway obstruction
175
renal complications of lupus
nephritis with proteinuria
176
pulmonary complications of lupus
restrictive pulmonary defect
pulmonary HTN
interstitial lung disease
pleural effusion
recurrent PEs
177
CV complications of lupus
pericarditis (tamponade uncomon)
Raynaud's
HTN
conduction defects
endocarditis
178
heme complications of lupus
hypercoagulability
anemia
thrombocytopenia
leukopenia
179
how does drug-induced lupus typically present and how long does it typically last
presents with mild s/s arthralgia, anemia, leukopenia, fever
typically lasts weeks to months
180
most common offenders of induced lupus
"PISSED CHIMP"
Pregnancy
Infection
Surgery
Stress
Enalapril
D-penicillamine
Captopril
Hydralazine
Isoniazid
Methyldopa
Procainamide
181
what causes hypercoagulability in lupus patients
development of antiphospholipid antibodies
## Footnote
risk DVT, stroke, PE
182
treatment of lupus
**aims to suppress immune system**
NSAIDs, corticosteroids, immunosuppressants, antimalarials
183
lab value if lupus pt develops antiphospholipid antibodies
prolonged aPTT
184
what med may be taken by lupus patients and increase duration of succs
cyclophosphamide
| inhibits plasma cholinesterase
185
what is Marfan syndrome
autosomal dominant connective tissue disorder
186
defining characteristic of Marfan syndrome
aortic insufficiency
| aortic regurg, aortic dissection
187
defining characteristic of Marfan syndrome
aortic insufficiency
| aortic regurg, aortic dissection
188
what does a pt with Marfan typically look like
very tall
pectus excavatum
kyphoscoliosis
189
genetic musculoskeletal disorder assoc with high risk of spontaneous PTX
Marfan syndrome
190
med given to Marfan syndrome pts to minimize wall stress
beta blockers
191
what is Ehlers-Danlos syndrome
inherited disorder of collagen and procollogen
192
common problems assoc with Ehlers-Danlos syndrome
arterial aneurysm
increased bleeding tendency
## Footnote
concerns: AAA, bleeding into joints
193
what causes bleeding in Ehlers-Danlos syndrome pts
poor vessel integrity (not coagulopathy)
194
anesthetic techniques to avoid in Ehlers-Danlos syndrome
regional, IM injection
| d/t bleeding risk
195
what is osteogenesis imperfecta
an autosomal dominant connective tissue disorder that causes brittle bones
196
risks for pt with OI needing surgery/anesthesia
* risk c spine fracture with airway management
* risk fractures with positioning
* risk fractures with NIBP cuff inflation or fasciculations with succs
197
pulm complications assoc with OI
kyphoscoliosis and pectus excavatum
(decreased chest wall compliance = V/Q mismatch, arterial hypoxemia)
198
lab increase in 50% of OI patients
serum thyroxine
199
disease assoc with blue sclera
osteogenesis imperfecta
200
what is multiple sclerosis
demyelinating disease of CNS
201
why are MS pts aspiration risks
cranial nerve involvement can cause bulbar muscle weakness
202
how is MS treated
steroids
interferon
azathioprine
203
things that exacerbate MS
stress
body temp increase as little as 1 degree C
204
is succs safe in MS
no - avoid (assoc with life threatening hyper K)
205
what is myotonic dystrophy
prolonged contracture after voluntary contraction
## Footnote
result of dysfunctional calcium sequestration by SR
206
things that increase risk of contractures in myotonic dystrophy
succs
NMB reversal with anticholinesterase (theoretical)
hypothermia
207
myotonic dystrophy increases risk of:
* aspiration
* respiratory weakness
* cardiomyopathy
* dysrhythmias
* sensitivity to anesthetics
208
what is scleroderma
excessive fibrosis in skin and organs (particularly microvasculature)
209
why is succs avoided in myotonic dystrophy
risk of sustained contractures - not MH
210
what is CREST syndrome
Calcinosis
Raynaud's
Esophageal hypomotility
Sclerodactyly
Telangiectasia
| a type of scleroderma
211
what is telangiectasia
spinder veins
increase mucosal bleeding
212
why are scleroderma pts at risk for difficult airway
limited mouth opening and mandibular hypomotility assoc with skin fibrosis
213
pulm complications of scleroderma
pulmonary fibrosis
pHTN
214
why are scleroderma pts often hypertensive
decreased vessel compliance, renal artery stenosis
215
why are scleroderma pts predisposed to corneal abrasions
eye dryness
216
what is Paget's disease
excess osteoblast and osteoclast activity
causes abnormally thick but weak bone deposits
217
most common problem in Paget's disease
fractures
pain
218
pulmonary function measurements decreased with kyphoscoliosis
* lung volumes
* PaO2
