Misc for test 1 Flashcards
1
Q
what is the key manifestation of spinal muscular atrophy
A
progressive degeneration of anterior horn cells
genetic disease
2
Q
what is the infantile form of spinal muscular atrophy (SMA)
A
SMA type 1 (Werdnig -Hoffmann disease)
3
Q
what is the late infantile and more slowly progressive form of spinal muscular atrophy (SMA)
A
SMA type 2 (Kugelberg-Welander syndrome)
4
Q
what is the more chronic, juvenile form of spinal muscular atrophy (SMA)
A
SMA type 3
5
Q
what type of disease is spinal muscular atrophy (SMA)
A
genetic
one of the most frequent autosomal recessive diseases
6
Q
with spinal muscular atrophy (SMA), the earlier it presents the _____ the progression
A
more severe
7
Q
what are fasciculations
A
quivering muscle movements that typically best seen on the lateral aspect of the tongue
seen easiest when child is asleep
talked about in the Spinal muscular atrophy (SMA) section
8
Q
prognosis of SMA type 1
A
most infants with this genetic disease die within the first 2 years
9
Q
Prognosis of SMA type 2
A
may survive to adulthood
10
Q
Prognosis of SMA type 3
A
can initially appear normal with slower progression of weakness and a normal life expectancy
11
Q
clinical manifestations of SMA type 1
A
severe hypotonia
generalized weakness
facial involvement
fasciculations
absent deep tendon reflexes
normal cognitive, social and language skills and sensation
with disease progression -> breathing becomes rapid, shallow, and predominantly abdominal
resp compromise leads to atelectasis, pulmonary infection and death
12
Q
labs and diagnostic studies for spinal muscular atrophy (SMA)
A
genetic testing
creatine phosphokinase (CK) may be normal or mildly elevated
Electromyelogram (EMG) shows fasciculations, fibrillations and other signs of denervation
muscle biopsy specimens show grouped atrophy
13
Q
treatment for Spinal muscular atrophy (SMA)
A
no specific treatment delays progression
nusinersen (antisense oligonucleotide) is being studied
symptomatic therapy is directed toward minimizing contractures, preventing scoliosis, optimizing nutrition, avoiding infections
manage resp infections aggressively - pulmonary hygiene, oxygen and abx
14
Q
in SMA (spinal muscular atrophy) what gene is missing
A
SMN1 gene and do not produce a protein necessary for the survival of the motor cells
15
Q
what drug has recently been approved for SMA and how does it work
A
Nusinersen - injected intrathecally and modifies mRNA from a nearly identical gene (SMN2) to increase SMN protein production.
16
Q
an acute post-infectious demyelinating disorder of the spinal cord
A
transverse myelitis
17
Q
how to treat transverse myelitis
A
high dose steroids
18
Q
symptoms of an epidural abscess
A
fever
spinal pain
neurological deficits
19
Q
lesions of the spinal cord are best seen in what imaging
A
MRI
20
Q
flaccid, areflexic paralysis
think…
A
acute spinal cord lesions
may mimic lower motor neuron disease
21
Q
timeline for transverse myelitis symptom progression
A
1-21 days
22
Q
on spinal fluid - WBC normal; no OCB
neuro paralysis like symptoms
A
not inflammatory causes
Think infarct dural AVF spondylosis tumor check B12 level
23
Q
On spinal fluid - increased WBC, +/- OCBs
neuro paralysis like symptoms
A
think inflammatory causes
MS
NMOSD (neuromyelitis optica spectrum disorder)
MOGAD (myelin oligodendrocyte glycoprotein antibody disorders)
infectious
sarcoid
24
Q
on spinal fluid - markedly increased CSF protein, normal cell count
neuro paralysis like symptoms
A
Think
Spinal block (tumor/spondylosis)
Guillain - Barre
25
on spinal fluid - decreased glucose
neuro paralysis like symptoms
think meningomyelitis
26
what can transverse myelitis cause
aggressive damage to the spinal cord without warning leading to varying degrees of weakness, paralysis, loss of sensation and bladder dysfunction
27
causes of transverse myelitis
usually postinfectious or idiopathic
secondary transverse myelitis is caused by other autoimmune diseases (ie multiple sclerosis), infection, paraneoplastic disorders
28
clinical features of transverse myelitis
motor: rapidly progressing weakness of the legs (+ arms if cervical cord affected), initially flaccid
sensory: pain, dysesthesia, and paresthesia below the level of the lesion
autonomic: urinary urgency or retention, constipation, bladder or bowel incontinence
29
diagnosis for transverse myelitis
MRI total spine with and without contrast
-will also r/o compressive myelopathy
Lumbar Puncture
-cell count and diff, protein, glucose, VDRL, oligoclonal bands, IgG index, and cytology (looking for cancer)
-Normal CSF, or elevated CSF lymphocytosis (<100) or elevated protein level
30
what deficiencies can cause symptoms similar to transverse myelitis
Vit B12
Vit E
serum copper
Ceruloplasm
31
what infections can cause a similar picture to transverse myelitis
HIV
enterovirus
Syphilis
32
what autoimmune disease can cause transverse myelitis
MS
33
what disorder triggered by cancer can attack the cells in the nervous system - transverse myelitis
Paraneoplastic syndromes - abnormal immune system response to a neoplasm.
T cells mistakenly attack normal cells in the nervous system
(you can look at Anti-Hu and anti-CRMP5 labs)
34
a group of genetic muscle diseases characterized by progressive myofiber degeneration and the gradual replacement of muscle by fibrotic tissue
muscular dystrophies
35
what is the most common muscular dystrophy and one of the most common genetic disorders of childhood
duchenne muscular dystrophy
36
what gene for duchenne muscular dystrophy
X-linked disorder (Xp21) that arises from a mutation in the dystrophin gene
37
autosomal recessive disorder of the anterior horn cells in the brainstem and spinal cord
Spinal muscle atrophy (SMA)
38
Degenerative disease characterized by progressive denervation of the muscle, leading to progressive hypotonia and muscle weakness.
Spinal muscle atrophy (SMA)
39
what gender are more commonly affected in spinal muscle atrophy (SMA)
Males
40
SMA type one presents by what age in most cases
6 mos
41
Floppy infant. Unable to achieve milestones of rolling or sitting independently. Weakness is greater in proximal muscle groups vs. distal and lower extremities are more severely affected. Infants have poor head control. Demonstrate a weak cry or cough and have difficulty with feeding, swallowing, or handling oral secretions due to bulbar dysfunction. Paradoxical breathing due to intercostal muscle weakness with sparing of the diaphragm. Often have characteristic bell-shaped trunk and abdominal protrusion. When supine, assume the frog-legged position Areflexia, fine tremor (most notably in the hands), tongue fasciculations, facial weakness, and normal sensation are reported.
clinical manifestations of SMA type 1
42
normal development for the first 6 mos
may sit independently and demonstrate head control but never able to stand or walk independently
fine motor tremor of hands
tongue fasciculations
diaphragmatic breathing
difficulty swallowing
prone to scoliosis and contractures
SMA type 2
43
weakness is greater in proximal muscle groups vs distal
lower extremities more severely affected than the upper
pt typically achieve ability to walk independently but many are wheelchair bound by the 4th decade of life
SMA type 3
44
what does EMG show in SMA
acute denervation due to malfunction of motor neurons
motor and sensory conduction velocities are normal
45
serum CK in SMA vs Muscular dystrophy
CK in SMA may be normal or slightly elevated
Muscular dystrophy CK is classically elevated
46
what teams would be involved in an SMA pt
```
neurology
pulmonology
orthopedics
physical and occupational therapy
genetic counseling
```
47
labs in SMA
chemistry studies - can provide info on metabolic disease, electrolyte imbalances, liver and kidney functions and drug levels
if they are on antiepileptic meds - watch liver and kidney functions
lactate - may identify resulting acidosis after prolonged seizures or in presence of septic shock in meningitis
ammonia - determine presence of metabolic disease with seizures
CBC with diff - infectious process or looking for anemia while managing increased ICP
newborn screening test - inborn errors of metabolism
infectious disease titers or levels
Looking for lyme, herpes, rubella, EBV, toxoplasmosis and other viral orgins
48
what are the anti-epileptic drugs that require drug level monitoring
Fosphenytoin
Phenobarbital
valproic acid
carbamazepine
49
when would you need to check levels for anti-epileptic drugs
initiation of drug to establish therapeutic level or range
breakthrough seizures in a previously stable pt
monitor adherence to meds
measure changes in levels with addition of second med with known interactions
50
when should you draw trough levels for antiepilepic meds
30 min prior to dose administration
51
goal level for phenytonin
total (protein and non protein bound phenytoin)
10 - 20 ug/ml
free (non protein bound)
1-2.5 ug/mL
52
calculation for correction of phenytoin level with low albumin
Fosphenytoin is protein bound
corrected level = measured phenytoin level/ {(albumin x 0.1) + 0.1}
53
goal level for phenobarbital
15-40 ug/ml
54
goal level for valproic acid
50-100 ug/Ml
55
goal level for carbamazepine
4-12 ug/mL
56
when would you order a EEG
evaluation of events suspected to be seizures
seizure localization
assessment of subclinical seizures
evaluation of altered LOC
Adjunct diagnostic test in the determination of brain death
57
contraindications to EEG
scalp infection
scalp skin breakdown
58
Hz for brain waves
1-3 Hz - Delta
4-7 HZ - Theta
8-12 Hz - Alpha
13-20 Hz - Beta
59
Brain wave Frequency ____ from birth to adulthood
increases
60
EEG - single or repetitive spikes that can be focal, multifocal or generalized
Epileptiform waves
61
Brain or head CT images are used in the clinical evaluation of
Bony abnormalities
hydrocephalus
ICH
Cerebral edema
Space occupying lesions
intracranial calcifications
62
Brain MRI is used in the clinical evaluation of
Ischemia or infarct
Degenerative diseases
Congenital anomalies
Arteriovenous malformations
Lesions in the posterior fossa and spinal cord
63
type of MRI that uses pulses of radio wave energy to provide images of blood vessels inside the body
MRA
64
MRA images are useful in the clinical evaluation of
reduced blood flow
aneurysm
arteriovenous malformations
65
Uses xenon-133, a radioactive dye which is injected prior to scan
cerebral perfusion scan
66
which imaging is more sensitive in detecting blood which makes it ideal for trauma eval
CT scan
67
what is the advantage of MRI
does not use ionizing radiation - eliminates exposure risk
higher level or gray/white differentiation
provides higher resolution detail at skull base and orbits
68
disadvantage of CT
radiation exposure
69
disadvantages of MRI
pt with metallic devices are unable to enter
longer imaging time, usually sedation required
70
cerebral perfusion scan may be considered in the clinical evaluation of
brain death
acute stroke
71
what does a lumbar puncture evaluate
can be therapeutic also
CNS infection
malignancy
autoimmune disease
inflammatory process
72
when is LP indicated
suspected infectious, immunologic or inflammatory process involving CNS
measure opening pressure
administration of intrathecal meds (anesthesia or chemo)
Therapeutic CSF removal to relieve increased ICP due to pseudotumor cerebri
73
contraindications for LP
increased ICP
platelet dysfunction
bleeding disorder
resp or hemodynamic instability
skin infection near site of entry
spinal cord trauma or compression
posterior spinal cord fusion in lumbar region
focal neuro deficit
74
Potential complications of LP
bleeding
infection
back pain
headache
CSF leak
Epidural or subdural spinal hematoma
Herniation (rare)
75
lab studies for LP
White and red blood cell counts
culture
gram stain and sensitivities
PCR and certain titers
76
what should you get prior to an LP on any pt with suspected increased ICP or suspected space occupying lesion
Head CT
negative CT does not completely exclude possibility of increased ICP
77
preferred device for monitoring ICP and can also be used for draining CSF
Ventriculostomy
78
for a ventriculostomy what is it connected to for monitoring
an external pressure transducer and monitor
79
what does a waveform indicate during ventriculostomy monitoring
resp variations and pulse pressure as well as ICP
80
Goals of ICP monitoring (ventriculostomy)
ICP and CPP numbers
Maintain ICP <20 -25 mmHg in older children
<15-20 in children <1 yr
Maintain CPP by maintaining adequate MAP; goal CPP >60mmHg in adolescents, >50mmHg in children and >40 mmHg in infants
81
factors to avoid that aggravate or precipitate elevated ICP
agitation
pain
obstruction of venous return (keep head in neutral position)
rep insufficiency such as airway obstruction, hypoxia or hypercapnia
```
fever
HTN
hyponatremia
anemia
seizures
```
82
what is anisocoria
unequal size of eyes pupils
83
what is the most appropriate imaging for a 3 week old to look for brain hemorrhage - unstable pt
Ultrasound is the most appropriate imaging modality for an unstable neonate with an open fontanel
84
6 yr old with no med history comes to ED with generalized tonic clonic seizures. what is the initial management
give lorazepam (ativan) to control seizure activity
85
what can you do for a intubated child to help decrease the blood flow to the brain which decreases blood volume in the cranial vault -> lowers the ICP
mild hyperventilation (PaCO2 32-35mmHg)
86
what diagnostic imaging is the most important technique to show an exact depiction of the AVM, its feeder vessels, nidus and draining veins
cerebral angiography
87
in a symptomatic, unstable pt, what diagnostic study is best to identify the presence of an AVM
CT - immediate viewing of the general location and will show if there is hemorrhage and edema
88
infant with a progressive, neuromuscular disease which typically presents with poor feeding, weakness, constipation, weak cry and other neurologic symptoms
infant botulism
89
what practice is most important to minimize infection rate in a child with a ventriculoperitoneal (VP) shunt in place
antibiotic prophylaxis with individually tailored specifications following a shunt placement
90
what is the most common cause of shunt failure in pediatrics
obstruction
91
how is a bacterial infection in CSF identified
Elevated WBC count (>1,000)
Elevated protein (>100mg/dL)
decreased glucose (<60% of serum glucose)
```
Normal WBC (0-5 cells/UL)
Normal protein (15-60 mg/dL)
Normal glucose (50-80mg/dL)
```
92
Disseminated encephalomyelitis often occurs following what?
a viral illness
93
clinical presentation for acute disseminated encephalomyelitis
```
behavioral changes
AMS
fatigue
fever
headache
nausea
vomiting
seizures
```
94
Asymmetric smile on exam post ischemic stroke located in pons. What CN is affected
Facial nerve (CN VII)
95
what can happen if you perform an LP on a pt with increased ICP
can result in transtentorial herniation or herniation of the cerebellar tonsils
96
gold standard study for a child suspected of arteriovenous malformation
Cerebral angiography
97
at what age do most boys become symptomatic for for Duchenne muscular dystrophy, what is their clinical presentation at this age
2-3 years old
develop an awkward gait and inability to run properly
some may have antecedent history of mild delay in attaining motor milestones or of poor head control during infancy
98
Gower sign
Duchenne muscular dystrophy sign
The child arises from a lying position on the floor by using his arms to climb up his legs and body (pushing off of thighs)
99
exam finding of
firm calf hypertrophy
mild to moderate proximal leg weakness with a hyperlordotic, waddling gait
Duchenne muscular dystrophy
other manifestations - cardiomyopathy, scoliosis, resp decline, cognitive and behavioral dysfunction
100
in Duchenne muscular dystrophy arm weakness is evident by what age
6 years old
101
Duchenne muscular dystrophy most boys are wheelchair dependent by what age
12 years old
102
Prognosis of Duchenne muscular dystrophy
many live into adulthood
| most die in their 20s or early 30s usually as a result of progressive resp decline or cardiac dysfunction
103
labs for Duchenne muscular dystrophy
serum CK - always markedly elevated (10,000-50,000 international units)
Aldolase is elevated
diagnosis - genetic testing (looking for dystrophin gene mutation)
muscle biopsy shows muscle fiber degeneration and regeneration accompanied by increased interfascicular connective tissue
104
treatment for Duchenne muscular dystrophy
chronic oral steroid therapy to slow pace of disease, delay motor disability and improve longevity (prednisone)
Supportive care - physical therapy, bracing, proper wheelchairs, treatment for cardiac dysfunction or pulmonary infections
105
what does the gene for dystrophin do
connects the contractile protein actin to the cell membrane
| Duchenne muscular dystrophy
106
females and Duchenne muscular dystrophy
usually are asymptomatic carriers or exhibit only mild symptoms - there are rare cases of severely affected females
107
muscular dystrophies commonly present with what (gait)
gait disturbance due to a hip girdle weakness
108
what are some things a caregiver might report for Duchenne muscular dystrophy (early signs)
a child who is mildly late to learn to walk (13-18 mths)
moves more slowly than other toddlers
displays toe walking
rolling gait or waddle
large calves (pseudohypertrophy)
Gower sign (pushing off thigh when arising from the ground
109
weakness in Duchenne muscular dystrophy is progressive and starts with _____ muscles and those of the ___ and ____ ____
volunteer muscles
| hips and pelvic girdle
110
pattern of progressive weakness in Duchenne muscular dystrophy
proximal leg involvement -> loss of ambulation -> arms and hands -> spinal support -> resp function
111
for Duchenne muscular dystrophy with exon 51 skipping what treatment options
weekly IV infusions of eteplirsen
results in small amount of dystrophin which slows progression of disease
112
Duchenne muscular dystrophy with stop codons (nonsense mutations) can be treated with
Ataluren - oral drug which also restores a small amount of dystrophin - results in slower weakness progression
this is only approved in European Union and still experimental in US
113
for Duchenne muscular dystrophy when should they see cardiology and obtain first echo
6-7 years
generally asymptomatic during first decade
may also get a cardiac MRI for greater detail
114
What cardiac problems are commonly seen developed in Duchenne muscular dystrophy
arrhythmias
cardiomyopathy from myocyte hypertrophy
atrophy
fibrosis
115
what cardiac meds may be considered with Duchenne muscular dystrophy
by age 10
ACE inhibitors or ARBS to reduce cardiac afterload and help with adding more years of normal ejection fraction before overt cardiac decline
Corticosteroids for similar and additive effect to ACE/ARB
B blockers, digoxin, furosemide and others may be required
116
As the child moves out of the plateau phase with Duchenne muscular dystrophy, what service should be consulted
pulmonology
progressive chest wall and paraspinal muscle weakness lead to impaired ventilator excursion, restrictive lung disease perhaps spine and chest deformities
ability to cough and clear secretions is impaired
117
common reasons for ED visits for a pt with Duchenne muscular dystrophy
resp insufficiency triggered by upper or lower resp infection
fractures secondary to osteopenia
Arrhythmias
heart failure
118
treating pain in a pt with Duchenne muscular dystrophy
be very careful in using narcotics due to resp depression in a child with a fragile ventilatory system
119
The incidence of _____ _____ is higher in those with Duchenne muscular dystrophy then with other muscle disorders
what anesthetic can cause this
malignant hyperthermia
succinylcholine
120
type of severe reaction that occurs in response to particular medications used during general anesthesia, among those who are susceptible
Malignant hyperthermia
121
symptoms of malignant hyperthermia
```
high fever
muscle rigidity
rhabdomyolysis
tachycardia
tachypnea
acidosis
increased Carbon dioxide production
```
122
drug to treat malignant hyperthermia
Dantrolene
123
dystrophin is present in what type of muscle
smooth
124
late complications for Duchenne muscular dystrophy
constipation
gastroesophageal reflex
gastroparesis
chewing becomes weak - gastrostomy feeding often required
Pressure ulcers
Low bone density (occurs with diseases of weakness and made worse by steroids)
125
what should be monitored for a pt with Duchenne muscular dystrophy related to bone density and what type of meds should be considered to help with this
monitor Calcium and vit D
Bisphosphonates
126
Xp21.2-p21.3 deletion
Duchenne muscular dystrophy
127
which muscular dystrophy is worse
Duchenne muscular dystrophy
or Beckers muscular Dystrophy
Duchenne muscular dystrophy
| Duchenne is Devestating
128
Pogressive myofiber damage ->loss of dystrophin -> muscle necrosis
Duchenne muscular dystrophy
129
in Duchenne muscular dystrophy there is progressive ______ muscle weakness
proximal
130
how often should a Duchenne muscular dystrophy receive an echo
every 1-2 years
131
autoimmune condition where antibodies block the acetylcholine receptors (AChR) at the neuromuscular junction which decreases the number of effective receptors
Myasthenia Gravis
132
3 childhood varieties of Myasthenia Gravis
juvenile myasthenia gravis (late infancy and childhood)
Transient neonatal myasthenia
Congenital myasthenia
133
variable ptosis (drooping eye)
diplopia (double vision)
ophthalmoplegia (paralysis or weakness of the eye muscles)
facial weakness
are the presenting symptoms for what?
Juvenile Myasthenia
(Myasthenia Gravis)
dysphagia, poor head control and extremity weakness may occur
134
what distinguishes Myasthenia Gravis from other neuromuscular disorders with progressive worsening over the day or with repetitive activity
rapid fatigue of muscles
135
prognosis for Juvenile Myasthenia
for some it never progresses past eyes (ophthalmoplegia and ptosis - ocular myasthenia)
Others have a progressive and potentially life threatening illness that involves all musculature to include resp and wallowing
136
treatment for Juvenile Myasthenia
pyridostigmine (acetylcholinesterase inhibitor) and depending on severity - various forms of immunosuppression
137
what type of Myasthenia Gravis develops in the first hours to days after birth to almost all newborns whose mother has myasthenia gravis
Transient neonatal Myasthenia Gravis
they have maternal anti-AChR antibodies (they all have them but only 10-20% will become symptomatic)
138
signs of transient neonatal myasthenia gravis
```
ptosis
ophthalmoplegia
weak facial movements
poor feeding
hypotonia
resp difficulty
variable extremity weakness
```
139
treatment for transient neonatal myasthenia gravis
cholinesterase inhibitors and supportive care for a few days to weeks until the weakness remits
140
Myasthenia Gravis that is due to gene mutations in the components of the neuromuscular junction
Congenital myasthenia gravis
141
typically present in infancy with hypotonia, ophthalmoparesis, facial diplegia and extremity weakness - can present throughout childhood.
Congenital myasthenia gravis
142
Prognosis for Congenital myasthenia gravis
lifelong disability
some might respond to pyridostigmine or other drugs that improve neuromuscular junction function
most children with MG will lead normal lives with treatment, with temporary or permanent remission
143
in autoimmune Myasthenia Gravis, the majority of individuals have antibodies to
AChR (Acetylcholine receptors)
144
for autoimmune Myasthenia Gravis, what med can be used for diagnostic verification?
administration of a cholinesterase inhibitor, edrophonium chloride can result in transient improvement in strength, particularly of ptosis, and can be used for diagnostic verification
(Tensilon test)
Edrophonium is a rapid onset (30-90 seconds) and short acting (5 min)
145
what is the bottom line problem in myasthenia gravis
production in of ACh is normal and the neural function is intact but there are not enough receptors on the muscle to allow for an adequate contraction
146
Myasthenic crisis is a rapid deterioration of neuromuscular and respiratory function and can occur due to
infection
stress
changes in medications (anticholinesterase inhibitors)
147
Diagnosis for myasthenia gravis
physical exam
pulmonary function tests
serological testing for AChR antibodies
EMG with repeated nerve stimulation (causing the pt to become weak)
148
Treatment for myasthenia gravis
mechanical ventilation
anticholinesterase meds (to inhibit breakdown of ACH) so it remains bound to the muscle and produces a sustained contraction (Pyridostigmine, neostigmine)
Immunosuppressants such as corticosteroids, azathioprine,
cyclophosphamide , cyclosporine, methotrexate, mycophenolate, and tacrolimus
IVIG
other therapies
plasmapheresis
y globulin
thymectomy (if have a thymoma, may relieve symptoms in some pt without thymoma)
149
```
diaphoresis
emesis
fecal incontinence
urinary frequency
tearing
drooling
bronchorrhea
miosis
hypotension
bradycardia
weakness
tremors
paralysis
hypoventilation
dysrhythmias
```
Organophosphate poisoning (insecticide)
prevents metabolism of ACH -> leads to accumulation at muscarinic and nicotinic sites -> produces cholinergic toxicity
150
Organophosphate poisoning management
decontamination
supportive care
reversal of nicotinic and muscarinic effects
treat with atropine (competitive inhibitor of Ach) and pralidoxime which breaks up the organophasphate-Achase bond and restores normal Achase activity
151
chronic neuromuscular disease characterized by varying degrees of weakness and fatigability of the skeletal muscles
myasthenia gravis
152
myasthenia gravis that is AChR negative
Congenital myasthenia gravis (not autoimmune)
153
should you use immunosuppressants in congenital myasthenia gravis
no, it is not autoimmune so will not help
154
is pupillary response effected in ocular myasthenia gravis
no
155
acute inflammatory demyelinating polyradiculoneuropathy
Guillain-Barre Syndrome
156
post-infectious autoimmune peripheral neuropathy that can occur
Guillain-Barre Syndrome
157
How many days post infection does Guillain-Barre Syndrome usually present
(what type of infections)
about 10 days after a resp or gastrointestinal infection
158
what bacterial culprits are classic for Guillain-Barre Syndrome
Mycoplasma pneumonia
or
Camylobacter jejuni
159
what ages does Guillain-Barre Syndrome occur
all ages
160
most common cause of acute flaccid paralysis in children
Guillain-Barre Syndrome
161
characteristic symptoms of Guillain-Barre Syndrome
areflexia
flaccidity
symmetrical ascending weakness
162
areflexia
flaccidity
symmetrical ascending weakness
Guillain-Barre Syndrome
163
how fast does progression of Guillain-Barre Syndrome occur
can occur rapidly in hours or more indolently over weeks
164
typically symptoms for Guillain-Barre Syndrome start where and progress to where
numbness or paresthesia in hands and feet
then heavy, weak feeling in legs.
Weakness ascends to involve the arms, trunk and bulbar muscles (Tongue, pharynx, larynx)
165
deep tendon reflexes for Guillain-Barre Syndrome
absent even when strength is relatively preserved
166
what associated autonomic problems r/t Guillain-Barre Syndrome
```
blood pressure changes
tachycardia
other arrhythmias
urinary retention or incontinence
stool retention
```
167
what key factors can help point you toward Guillain-Barre Syndrome when trying to distinguish from acute spinal cord syndrome
Preservation of bowel and bladder function
loss of arm reflexes
absence of a sensory level
lack of spinal tenderness
168
CSF in Guillain-Barre Syndrome
sometimes normal early in the illness
classically shows
elevated protein levels without significant pleocytosis
169
What will MRI show in Guillain-Barre Syndrome
MRI with gadolinium - enhancement of the spinal nerve roots
170
Guillain-Barre Syndrome treated with
initially IVIG
plasma exchange and immunosuppressive drugs are alternatives with IVIG is unsuccessful or in rapidly progressing disease
171
is Guillain-Barre Syndrome unilateral or symmetrical
symmetrical
172
why does Guillain-Barre Syndrome occur
the pt has an infection such as Campylobacter jejuni or mycoplasma pneumoniae
These organisms contain protein moieties that are similar to proteins located on host myelin sheaths.
Antibodies produced to eradicate these organisms then recognize the hosts myelin and attack it like that foreign protein
173
suggestive parameters for mechanical ventilation in pt with Guillain-Barre Syndrome
vital capacity less than 20mL/kg and a negative inspiratory force greater than -20 cm H2O
174
max weakness in Guillain-Barre Syndrome usually occurs how long after onset of symptoms
1-2 weeks
175
symptoms of Guillain-Barre Syndrome
```
Areflexia
numbness
CN palsies
hypoventilation due to weak resp muscles
with or without altered LOC
With/without ataxia
autonomic instability can occur - fluctuations in BP, diaphoresis, vasoconstriction, pupil dilation and constriction, cardiac arrhythmias
```
176
How does IVIG work for pt with Guillain-Barre Syndrome
IVIG may work via several mechanisms, including blocking macrophage receptors, inhibiting antibody production, inhibiting complement binding, and neutralizing pathologic antibodies.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 586). Wolters Kluwer Health. Kindle Edition.
177
How does Plasmapheresis work with Guillain-Barre syndrome
Removal of circulating humoral factors (e.g., immunoglobulins and antibodies) responsible for demyelination.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 586). Wolters Kluwer Health. Kindle Edition.
178
global brain dysfunction leading to an altered mental state
Encephalopathy
179
brain requires a delicate balance of what
```
neurotransmitters
substrates
water
electrolytes
adequate perfusion
acid base balance
temperature
```
180
encephalopathy etiologies
```
electrolyte imbalance
medication
environment
organ dysfunction
inborn errors of metabolism
```
181
what type of encephalopathy occurs after an event such as cardiac arrest and why
hypoxic-ischemic encephalopathy
| -brain is deprived of adequate perfusion or oxygenation resulting in cerebral ischemia, hypoxia and cell death
182
Acute encephalopathies
Hypoxic-ischemic
infectious
postinfectious
vascular disorders
183
what type of encephalopathy has cerebral dysfunction that is permanent and nonprogressive
Static encephalopathy
184
what type of encephalopathy is most commonly associated with neurogenerative disorders and autoimmune disorders
progressive encephalopathies
185
a condition of acute global cerebral dysfunction, resulting in altered consciousness, behavior changes and/or seizures not because of brain disease, TBIs or infection...This is a result of cytotoxic injury and/or neurotransmitter disruption
Acute toxic/metabolic encephalopathy
186
what type of events can lead to hypoxic-ischemic encephalopathy
before, during or after birth
trauma
asphyxiation
congenital abnormality
stroke
medications
prematurity
in older pediatric pt
asphyxiation
airway obstruction with foreign body or airway edema
submersion injury
severe asthma
```
sepsis
low cardiac output states
chronic hypoxia from pulmonary disease
resp and cardiac arrest
systemic hemorrhage
carbon monoxide poisoning
cardiac arrhythmias
```
187
infections that can cause encephalopathies
```
bacterial meningitis
encephalitis
fungal meningitis
tuberculosis meningitis
parasitic infection
AIDS
HIV
ADEM (acute disseminated encephalomyelitis - lymphocytes attack myelin sheath
```
188
immune mediated encephalopathy as a result of autoimmune most often caused by
anti-N-methyl-D-aspartate (Anti-NMDA) receptor encephalitis - antibodies target the subunit of the receptor
189
clinical presentation in infant for encephalopathy
irritability
lethargy
poor feeding
190
clinical presentation in child for encephalopathy
personality or behavioral changes
Cognitive decline
concentration problems
other symptoms: seizures, nystagmus, tremor, myoclonus, abnormal movements
191
labs to order for encephalopathy workup
CBC with diff
CMP
LFT
Ammonia
Coagulation panel
Blood gas
blood cultures
urinalysis and urine cultures
CSF cultures
Metabolic: serum amino acids, urine organic acids, lactate, thyroid function tests
Tox screen
med drug levels
lead level
Inflammatory: CSF or oligoclonal bands, serum for antibodies (thyroid, NMDA)
look at what meds they take, could they be contributing?
192
Imaging for encephalopathy workup
CT or MRI
EEG to look for sizures
193
when should you consider steroids in enephalopathy
for postinfecious or autoimmune encephalopathy
194
what disorders may have encephalopathies
Tay Sachs disease
Mucopolysaccharidosis types I and II
adrenoleukodysrophy
mitochondrial encephalopathies such as MELAS and spinocerebellar ataxia
195
CBC is useful in evaluating
inflammation
infection
anemia
immune status
196
Metabolic panel (CMP) will identify
electrolyte imbalance
kidney function
hydration status
serum glucose levels
197
Liver function tests will show
enzyme activity and accumulation of toxic metabolites such as bilirubin or ammonia
198
Coagulation panels will show
coagulopathy or hypercoagulable states
199
ABG will show
acid-base
| oxygenation/ventilation
200
what can you measure on the ABG if you suspect carbon monoxide poisonings
Carboxyhemoglobin
201
what is the first imaging study that should be ordered with encephaolopathy
CT
202
what improves survival and neurodevelopment in newborns with moderate to severe HIE
hypothermia
203
result of a sudden interruption of arterial or venous blood flow to a focal region of the brain
Cerebrovascular accidents (stroke)
204
a decrease or disruption of blood flow, leading to dysfunction of brain tissue, caused by systemic hypoperfusion, embolism, thrombus, and sinus venous thrombosis
Ischemic stroke
205
a vessel or aneurysm ruptures and leaks into the surrounding tissue and cells in the brain
Hemorrhagic stroke
206
most common risk factors for arterial ischemic stroke in children
congenital and acquired heart disease
Arteriopathies (arterial dissection, moyamoya disease, vasculitis)
Sickle cell disease
Hypercoagulable conditions
207
clinical presentation of neonates for ischemic stroke
seizures, decreased responsiveness, focal weakness
208
clinical presentation of children for ischemic strokes
focal neurologic deficits such as hemiparesis
aphasia
visual disturbances
headhache
209
clinical presentation for hemorrhagic stroke
signs of increased ICP such as headache and vomiting
irritability
seizures
hemiparesis
210
CT for stroke
initial (hours after infarct) could be negative - serial imaging may be necessary
211
what imaging should you order if concern for dissection
CTA (CT angiography)
212
What imaging should you order if concerned for venous thrombosis
CT Venography
213
supportive care for strokes
prevent fever
maintain normoglycemia
normovolemia to maintain adequate substrate delivery
214
management of ischemic strokes
Heparin (unfractionated or low molecular weight) may be administered for as long as 1 week
ASA 3-5mg/kg used for ischemic stroke prophylaxis
215
Management of hemorrhagic stroke
Neurosurgical evaluation for ICP monitoring
216
when a patient presents with what symptoms should stroke be high on the differential
headache
seizure
focal weakness
217
what is the preferred method to identify an acute stroke
MRI
218
lab studies for stroke workup
coagulation panel
platelets
electrolytes
fasting cholesterol
triglycerides
```
if an autoimmune disorder is suspected
Protein C
Protein S
antithrombin III deficiency
anticardiolipin antibodies
antiphospholipid antibodies
```
219
what genetic testing for stroke pt
factor V leiden mutation
prothrombin gene mutation (G20210A)
methylenetetrahydrofolate reductase TT677 mutation
220
strokes in children with sickle cell can be due to
```
arterial ischemia
subarachnoid or intracerebral hemorrhage
aneurysm rupture
dissection
moyamoya syndrome
SoVT
```
221
if your imaging studies do not identify stroke in a pt who has stroke like symptoms. What should you order?
EEG to evaluate for seizures
222
what are the AHA class I recs for management of neonatal strokes
supplement coagulation factors in coag disorders
replace platelets in those with intracranial hemorrhage
ventricular drainage for those who develop hydrocephalus
treat dehydration, anemia
if they have a MTHFR mutation give folate or vit B
evacuating a hematoma that elevates ICP
223
for a stroke how should head of bed be placed
flat to promote cerebral perfusion unless there is a concern for increased ICP or other contraindications
224
for arteriopathy, idiopathic stroke or Moyamoya what drug is the drug of choice for stroke prevention. also include dosing
3-5mg/kg/day ASA
risk of Reye syndrome in children following viral or varicella
225
for extracranial dissection or cardioembolic stroke what to use for anticoagulation
heparin or low molecular weight heparin (LMWH) for acute....and switch to anticoagulants or ASA for long term
226
is tPA approved in children in strokes?
no , only clinical trials
227
infections that can increase risk of arterial ischemic stroke
meningitis
varicella
tuberculosis
228
what cardiac problems increase risk for stroke
```
Congenital heart disease
arrhythmias
cardiomyopathy
cardiac surgery
Kawasaki disease (KD)
Endocarditis
```
229
a disturbance of brain function caused by paroxysmal discharges within the neuronal system
seizure
230
Prognosis for children with a first time unprovoked seizure in about 25% of kids
will demonstrate a normal neuro exam and have few or no recurrent seizures
231
what can cause seizures
genetic or abnormal cortical development
metabolic (hyponatremia, hypoglycemia)
infection (meningitis)
fever
stroke
traumatic brain injury
toxic ingestion/exposure
subtherapeutic anticonvulsant med levels for someone with h/o epilepsy
232
patho for seizures
hypersynchronous discharges within the cortex combine with high frequency bursts of the action potentials to generate a seizure
233
sustained extension or flexion of head, trunk, and extremeties
tonic seizure
234
potential complications of status epilepticus
rhabdomyolysis
hyperthermia
cerebral edema
resp failure
235
labs to look at in first time seizures based on individual circumstances - why would you order
```
CBC
serum electrolytes
glucose
calcium
magnesium
```
vomiting, diarrhea, dehydration, failure to return to baseline promptly
236
in majority of febrile and non-febrile seizures, lab studies often show what?
unremarkable
237
what meds can you give for persistent seizures
fosphenytoin or phenobarbital
238
motor onset generalized seizures include:
```
tonic
clonic
tonic-clonic
myoclonic
atonic
epileptic spasm
```
239
non-motor onset generalized seizures include:
typical absence
atypical absence
myoclonic absence
absence with eyelid myoclonia
240
the example given of throwing a remote across the room or dropping a plate are examples of what type of seizure
myoclonic
241
seizure where ictal discharge occurs in a limited area of the cortex....presentation is dependent on the locus in the brain - intact recall of the event assists with diagnosing this type of seizure bc consciousness is not lost
focal aware seizures
242
seizure - impaired consciousness and imply a larger region of spread within the cerebral cortex....usually present with automatisms (lip smacking, repetitive swallowing and clumsy motor movements)
focal seizures with impaired awareness
243
focal or generalized onset characterized by either flexion of neck, trunk and extremities and/or abrupt extension of arms, legs, neck and trunk.....frequently occur in clusters, during sleep or just upon waking
epileptic spasms
244
what age are epileptic spasms seen
typically present between 4-6 mos....most prior to 1 yr old
245
epileptic spasms are associated with what
epileptic encephalopathy
246
febrile seizures can be considered when associated with temp greater than or equal to ______ within ____ hrs of seizure without CNS infection, history of afebrile seizures, underlying epilepsy or underlying neurologic condition that predisposes the child to seizure
38C (100.4F)
| 24 hours
247
what type of seizures occur at the time of systemic insult or in close temporal association with a documented brain insult
acute symptomatic seizures
248
if there is clinical concern for meningitis in a pt under 12 mos of age what test needs to be done
LP
| big risk factor is unvaccinated
249
what age should you get labs for a seizure workup (first time, no underlying)
6 mos
- electrolytes
250
what age should the pt go home with a rescue med for first time unprovoked seizure
2 yrs and older
251
meds to treat seizure
lorazepam (ativan) or diazepam
if lasts after 5 min -? fosphenytoin, phenobarbital, levetiracetam or valproic acid
252
what is SUDEP
sudden unexplained death in epilepsy
rare but less likely to occur in well controlled epilepsy
253
risk factors for SUDEP
uncontrolled generalized tonic clonic seizures
antiseizure medication polytherapy
no use of anti-seizure meds
intellectual disability
254
for a ventriculostomy. where does the 0 need to line up with
tragus of the ear
255
what happens during a drowning
involuntary laryngospasm as the water comes into contact with the airway after submersion - causing a conscious person to cough and inhale more water. Aspiration of water and vomitus causes further laryngospasm leading to hypoxemia and LOC.
Hypoxemia stimulates a shift in the acid base balance resulting in arrhythmias, myocardial and cardiac arrest due to metabolic or resp acidosis
256
when water enters the airway, tell me about the inflammatory cascade
the inflammatory cascade -> causes pulmonary vasoconstriction and pulmonary edema -> surfactant is denatured and lungs become noncompliant -> difficult to ventilate with increased atelectasis -> intrapulmonary shunting with ventilation perfusion mismatching occur
257
what happens in the brain in a submersion injury
cerebral ischemia results from inadequate blood flow to brain and if deprived of oxygen for extended time -> brain tissue begins to die (cerebral infarcts) - > injured brain begins to swell (cerebral edema) -> raises ICP -> further injury to nervous system
258
submersion injury in r/t cardiac injury
indirect -> it is the resultant hypoxia and pulmonary injury that affect the myocardium
hypothermia and electrolyte disturbances result in arrhythmias
cardiovascular system can recover if oxygenation and acid base balance are restored
pulmonary vasoconstriction if severe enough can result in decreased R ventricular stroke volume, decreased l ventricular preload and worsened cardiac output
259
asymptomatic presentation of drowning to ED - be careful...why?
even mild hypoxia can increase permeability of pulmonary capillaries with alveolar fluid leak and surfactant damage
resp dysfunction may take hours to manifest
children need to be observed for a prolonged time frame after submersion incident
260
what should you order in a submersion injury
ABG values
chest x ray (looking for atelectasis, pulmonary edema, aspiration)
vital signs
261
symptoms tied to a submersion injury
```
anxiety
vomiting
cough
wheezing
hypothermia
AMS
metabolic acidosis
resp failure
cardiac arrest
```
262
Indications for intubation post submersion
PaCO2
Pa02
unconscious child
peripheral arterial carbon dioxide (PaCo2) >50
inability to maintain peripheral arterial oxygen (PaO2) > 90% with supplemental Oxygen
263
how to use PEEP in intubated pt for a submersion injury
prevent atelectasis and overcome intrapulmonary shunting
264
noninvasive ventilation options in submersion injury
continuous positive airway pressure or
bilevel positive airway pressure
265
gastric decompression in submersion injury treatment
via orogastric or nasogastric tubes should to minimize aspiration risk in patients with altered LOC
266
common findings in submersion injury
hypothermia
hypoglycemia
electrolyte abnormalities
267
how do you protect the pt from secondary cerebral injury post submersion injury
the initial cerebral ischemic injury is during the submersion
secondary occurs later from prolonged hypoxemia, cerebral edema, acidosis, hypovolemia, seizures, electrolyte imbalances
resp treatment and intervention guided by ABG values
Avoid hypercapnia (can increase cerebral HTN ->worsens cerebral edema)
Give ABX to prevent aspiration pneumonitis
normalize BP - prevent hypotension
avoid hypervolemia (may need diuretics and fluid restriction)
treat hypermetabolic states such as seizure and fever to avoid secondary brain insults
EEG should be used to detect subclinical seizures, esp if pt requires neuromuscular blocking agents
268
why laryngospasm during submersion?
bodies way to protect you from water going into your lungs. This is to prevent that...but if your awake your coughing and choking more
269
aspiration specific to fresh water
surfactant denaturing with subsequent alveolar collapse
intrapulmonary shunting
hypoxia
270
aspiration specific to saltwater aspiration
large osmotic gradient with transudation of fluid from pulmonary vasculature to alveoli -> results in surfactant dilution and washout with alveolar collapse
shunting
hypoxia
271
how is drowning defined?
resp impairment from submersion/immersion in a liquid.
The outcome can be death, morbidity, or no morbidity
avoid terms - "wet drowning, dry drowning"
instead - fatal or non-fatal drowning should be used
272
drowning is the ____ leading cause of accidental death among children in the US
2nd leading cause
273
what age group is the second peak in age distribution for drowning and why
adolescence
often associated with alcohol, drug use and risk-taking behavior
274
Neuro classifications for submersion injuries
Category A - PT awake/alert, minimal brain injury
Category B - more serious asphyxial injury. Blunted to stuporous, normal pupillary response and purposeful responses to pain
Cat C - more severe injury
comatose, abnormal resp patterns (Cheyne Stokes), abnormal motor responses to pain
C1 - decorticate movements
C2 - decerebrate movements
C3 - Flaccid, apneic
275
most submersion victims aspirate an average of ___ mL/kg
3
276
for blood volume alterations to occur, what do you need to aspirate during a submersion ____mL/kg
11
277
for electrolyte alterations to occur what do you need to aspirate during a submersion? ____mL/kg
22
278
after submersion, pt often require supplemental oxygen or positive pressure ventilation for how long?
48-72 hours which is how long it takes for surfactant to reconsititute
279
children who progress to ARDS after submersion injury should undergo mechanical ventilation aimed at minimizing
barotrauma and volutrauma
280
Indications for ECMO after a submersion
refractory shock
refractory hypoxemia
controlled rewarming
281
when should you give submersion victim abx
if they become febrile
increasing leukocytosis
worsening infiltrates on chest x ray
develop hemodynamic instability
282
empiric steroids and submersion?
do not use....outcome not improved and shown to increase rates of infection
283
continuous hypotension post submersion
inotropic support with dopamine, dobutamine, or epinephrine may be needed
284
Poor neurologic outcome from a submersion injury include submersion duration greater than
what else?
4-5 min
warm water temp
CPR in field
CPR in field with intubation and epi administration
Continued apnea, asystole and coma after arrival to ED
285
what lab values post submersion are associated with worse neurologic outcomes
pH < 7.1
elevated serum glucose
286
what is the difference for children who drown in ice cold water
warrant additional consideration because they may survive prolonged cardiopulmonary arrest with a favorable neurologic outcome
287
nearly all children who demonstrate significant problems in gas exchange will do so by ___ to ___ hours after submersion
4-6 hours
288
discharge requirements post submersion from ED
If at the 6 hour mark they are asymptomatic and they are neurologic cat A they can be discharged from ED. No labs or x ray required
289
symptomatic submersion injury patients will need what?
chest x ray
ABG
consider -
CBC
coags
electrolyte panel
must be admitted for obs until symptoms completely resolve
290
leading cause of injury related deaths and disability for age groups newborn to 4 years old and ages 15-19 years old
Traumatic brain injury
291
what gender is at higher risk for a TBI
males
292
an acquired brain injury from a blow to the head or a penetrating head injury that disrupts the normal function of the brain
TBI
293
secondary brain injury occurs as the result of
a cascade of biochemical, cellular and metabolic responses influenced by associated hypoxia and hypotension which affect long term outcomes
294
what type of injury occurs when rapid acceleration, deceleration or rotational forces result in axonal shearing at the interface of gray and white matter?
Diffuse axonal injury -> leads to cell edema, damage or death - no surgical intervention available
295
TBI clinical presentation
Altered loc
resp distress/failure
hemodynamic instability with shock
seizures
vomiting
visual disturbances
motor/sensory impairments
296
diagnostic eval for TBI
Head CT
Head MRI
diffusion tensor imaging (if available)
297
management of TBI
manage cerebral edema and increased ICP via external ventricular drainage
Keep ICP <20 mmHg and optimal perfusion pressure for age
mild hyperventilation
hyperosmolar therapy
hypothermia
surgical intervention if indicated
maintain hemodynamic stability with opimal oxygenation/ventilation
control seizures
pain control
sedation
neuromuscular blockade as indicated
optimize nutrition with parenteral nutrition and introduction of enteral feedings when appropriate
DVT prophylaxis
298
scale to assess cognitive function and rate of disability in ABI patients
Rancho los Amigos scale of cognitive levels - based on cognitive and behavioral presentations as patients emerge from a coma
299
anti epileptics and kids with TBI
young kids higher risk for seizures
use only when clinically necessary - should only be used when clinically required and at the lowest clinically effective dosage to facilitate max cognitive recovery
AEDs can interfere with a developing brain
300
signs/symptoms of a concussion
somatic (headache)
cognitive (slowed reaction times, poor attention)
emotional (lability, irritability)
sleep disturbances (drowsiness)
physical signs (loss of consciousness, amnesia)
301
what would warrant imaging in a concussion
increase in somnolence
headache
vomiting
302
recovery time for concussion
majority resolve within 10 days
recovery may be slower in children
303
repeated concussions, esp within a short time frame (days or weeks), carry a significant risk of permanent brain injury called
second impact syndrome
304
symptom progression of a epidural hemorrhage
lucid interval following the brain injury -> decreased LOC -> then returns to normal for several hours -> then developing rapidly progressive neurological symptoms
305
lens shaped extracerebral hemorrhage compressing brain
epidural
306
crescentic extracranial hemorrhage compressing brain
Acute subdural
307
Crescentic, low density mass on CT
chronic subdural
308
on CTmultifocal low density areas with punctate hemorrhages
contusion
309
Battle sign
bleeding over mastoid process
skull fracture
310
racoon eyes
bleeding around orbit
skull fracture
311
hemotympanum
bleeding behind tympanic membrane
skull fracture
312
when is surgical intervention needed for depressed skull fracture
more than 0.5-1cm
surgical elevation
repair of associated dural tears
313
Monroe-kellie Doctrine equasion
V of the intracranial vault (constant) = V of the brain +V of the CSF + V or the blood + V of any mass lesion
314
components inside the skull and %
Brain 80%
CSF 10%
Blood 10%
315
ICP normal values
- in older children
- in children < 1 year
<20-25cm H2O older children
<15-20 mmHg in children <1 yr
316
ICP generally peaks ___ to ___ hours after a traumatic injury
24-72
317
equation for cerebral perfusion pressure (CPP)
Mean Arterial pressure (MAP) - ICP = CPP
318
CPP goal for infant
children
adolescents
infant > 40
children > 50
adolescence >60 mmHg
319
Net pressure gradient supplying blood flow to brain depends on ___ and ___-
MAP and ICP
320
Map is equal to
1/3 systolic BP and 2/3 diastolic BP
321
CPP can be decreased from an increase in _____
ICP
or decrease in systemic BP
322
low CPP has been associated with ___ outcomes in traumas
poor outcomes
323
what causes increased ICP without evidence of space-occupying lesion or vascular abnormality
idiopathic intracranial hypertension AKA psudotumor cerebri
324
Persistent increase in ICP can lead to ____ and ___
brain herniation and death
325
Cerebral edema may be the result of
intracellular swelling
capillary endothelial cell dysfunction
interstitial edema
326
what is the most common problem leading to increased ICP
traumatic brain injury
327
symptoms of increased ICP
```
LOC
Altered LOC
vomiting
increased head circumference in infant
tense fontanel in infant
seizures
status epilepticus
coma
```
cushings triad
328
what is cushings triad
sign of increased ICP
irregular resp
widening pulse pressure
bradycardia
(indicates impending herniation)
329
elevations in ICP typically take ____ to ____ to peak following an injury
24-72 hours
330
medical management of increased ICP
sedation
drainage of CSF
osmotic diuresis with either mannitol or hypertonic saline
Head of bed elevated and midline
mild hyperventilation is helpful in decreasing ICP with goal CO2 30-35 mmHg
if evidence of cerebral ischemia and no contraindications, consider hypothermia
331
for intracranial hypertension refractory to initial medical management
barbiturate coma
hypothermia
decompressive craniectomy should be considered
332
most common method of ICP monitoring use either
an intraventricular catheter
or an
intraparenchymal catheter
333
contraindications to contrast
pregnancy
allergy to shellfish or iodinated dye
unstable vitals
impaired kidney function
before given - BUN, creatinine levels and occasionally a creatinine clearance to check kidney function
334
pt may experience what with contrast
flushing /feeling of warmth
transient headache
salty or metallic taste in mouth
nausea
vomiting
335
what is important to facilitate after contrast
oral or IV hydration
336
what diagnostic imaging for movement disorders?
MRI
| PET
337
what uses nuclear medicine technology to primarily evaluate the nervous system through cerebral metabolism and cerebral blood flow
PET - Positive emission tomography
SPECT - Single-photon emission computerized tomography
338
currently PET scans are most commonly used to detect and provide understanding of
brain disorders (tumors, memory disorders, and seizures)
Cerebrovascular disease
cerebral trauma
other CNS conditions
339
in relation to glucose the PET scan reveals
which areas of the body are burning the most sugar
340
to prep for a PET scan
no caffeine, alcohol, nicotine, or glucose consumed for a period up to 24 hours prior
no enteral intake for 6-12 hours prior
must be able to lay flat for 2-3 hours
341
postprocedure care for PET scan
copious fluids to clear isotope
342
SPECT scan measures
cerebral blood flow and perfusion
343
SPECT is useful to demonstrate hypoperfusion in focal and diffuse cerebral disorders like
dementia
seizures
stroke
cerebral taruma
344
preferred test for brain death
SPECT
345
SPECT is helpful during acute events for
```
stroke
seizures
dementia
amnesia
trauma
neoplasms
brain death
persistent vegetative state
Psychiatric disorders such as schizophrenia or depression
```
346
major disadvantages for SPECT
high cost
IV access for isotope
must be done within 1 hr (isotope degredation)
347
special prep for SPECT
none
348
disadvantages for PET
high cost
limited availability for diagnostic purposes
necessity to produce short-lived isotopes which limits patient access to this imaging modality
349
suicide is the ___ leading cause of death in children and adolescents in the US with higher incidence of suicide completion among ____
3rd
| males
350
more than half of male suicide occurrences involve a _____; most female suicides are caused by ___
males - firearm
females - poisoning
351
what is a chief catalyst for suicide and suicidal thoughts
depression
352
Risk factors for suicide pneumonic
IS PATH WARM
```
I - ideation
S- substance abuse
P - purposelessness
A- anxiety
T-trapped
H - hopelessness
W - withdrawal
A - anger
R - recklessness
M - mood changes
```
353
what suicide risk level?
history of serious or nearly lethal attempts or planning
recently institutionalized for a psych disorder or recent psych disorder
persistent suicidal ideation, psychosis, history of aggression and impulsive acts
High risk
354
what suicide risk level?
under medical care of a psychiatric specialist
suicidal ideation without plans or attempts
no other identified signs
moderate risk
355
what suicide risk level
mild suicidal ideation, but without attempt
social support
no previous attemps
low risk
356
refusal to maintain normal body weight for age and height: generally body weight <85% expected.
anorexia nervosa
357
primary or secondary amenorrhea in postmenarchal females (eating disorder)
anorexia nervosa
358
recurrent behaviors in an effort to avoid weight gain, including induced vomiting, abuse of laxatives, diuretics, fasting, excessive exercise
Bulimia nervosa
359
eating disorder associated lab abnormalities
electrolyte abnormalities
chloride responsive metabolic alkalosis
significant hypophosphatemia
intravascular volume depletion or shock
360
heart and eating disorders
risk for cardiac arrhythmias due to electrolyte abnormalities - depletion of total body potassium, hypomagnesemia, serum hypophosphatemia and altered acid base balance
361
eating disorders and bone marrow
bone marrow failure may develop
362
clinical presentations of anorexia nervosa and bulimia nervosa
emaciated body habitus
cachetic appearance
decreased body temp
bradycardia
hypotension
acrocyanosis
edema
dry skin with discoloration
cold extremeties
scalp hair loss
increased lanugo
decreased deep tendon reflexes
arrhythmias
evidence of dehydration and/or shock
363
Patients with bulimia nervosa may be normal weight and body habitus with enlarged
parotid glands or tonsils
erosion of tooth enamel
superficial ulceration, calluses or scarring on dorsum of fingers
decreased or absent gag reflex
swelling of hands and feet r/t hypoalbuminemia
364
eating disorder pt complaints
fatigue
muscle weakness
cramps
abd pain
constipation
easy bruising
sore throat
sleep disturbances
difficulty concentrating
increased physical activity
dizziness
fainting
365
eating disorder labs and other orders
```
CMP
carotene levels
zinc
copper
prealbumin
amylase
lipase
cholesterol
LFT
thyroid function test
estradiol levels
Urinalysis: urine electrolytes
ECG
```
366
in diagnostic eval for eating disorders, what other problems to evaluate for
malignancy
inflammatory bowel disease
malabsorption syndromes such as celiac disease
Diabetes mellitus
hyperthyroidism
hypopituitarism
367
management of eating disorders
stabilize -
correct intravascular volume depletion and normalize electrolytes
reverse hypotension, tachycardia
refer to psych
get history of anxiety, difficulty with separation from parents, peer relationships
368
components to presenting a case
1) be organized
2) tell a story
3) one liner and impression statement
4) Present a pt, not just a disease
5) logical sequence
6) pertinent positives and pertinent negatives
7) summarize the case
8) include a differential diagnosis
9) include your management plans
369
muscle weakness and fatigue are associated with
Hyperkalemia
Hypokalemia
Hypokalemia
370
what symptoms are associated with hypokalemia and which ones are for hyperkalemia
```
ventricular arrhythmias
Peaked t waves
flattened t waves
decreased diastolic function
muscle weakness and fatigue
```
Hypokalemia
- muscle weakness and fatigue
- ventricular arrhythmias
- flattened T waves
- decreased diastolic function
Hyperkalemia
-peaked T waves
371
critically low phosphorous levels (<1mg/dL) can result in symptoms that include
muscle weakness including resp muscle function
372
what to do with phosphorous levels can cause laryngospasm and tetany
hyperphospatemia
373
seizures, irritability and lethargy can be r/t hyponatremia or hypernatremia
both
374
to restore sodium levels using hypertonic saline...how is this calculated?
(0.6 x (weight in kg) x (target sodium - measured sodium)) to achieve a target sodium level of 125 mEq/L
375
what hormone is primarily responsible for Calcium regulation
PTH (regulates calcium absorption from bone and renal excretion of calcium)
376
assessment of intravascular volume depletion in a 14 year old with eating disorder
orthostatic vital signs
377
what low electrolyte can cause seizures
low sodium
normal is 135-145
give 3% saline solution to correct
low potassium will not cause seizures
378
what serial evaluation for guillain barre will be most important for assessing resp status
Negative inspiratory force (NIF)
379
left subdural hematoma in the frontoparietal region what impaired motor function would be anticipated
R hemiparesis
380
after a traumatic injury, that required antiepileptic meds as prophylaxis. For how long after the injury should you keep the pt on the meds?
7 days after injury - switch to keppra if you still need an anticonvulsant
381
rancho los amigos level?
no response to stimuli
Level I
382
rancho los amigos level?
generalized response with inconsistent and nonpurposeful reaction to stimuli in a nonspecific manner. responses may be delayed and include physiologic changes, gross body movement and/or vocalizations
LevelI
383
rancho los amigos level?
response to stimuli is more localized, such as withdrawal from painful stimuli or inconsistent following of simple, one step commands
Level III
384
rancho los amigos level?
exhibits automatic but appropriate responses although "robotic-like and demonstrates decreased safety awareness and impaired judgement
Level VII
385
what area of brain is responsible for production of speech
Broca area
386
where is the Broca area located
left frontotemporal
387
wernicke area is located in the
Parietal-temporal area
388
Wernicke area is responsible for
language comprehension and receptive speech
389
injury to Broca area may result in
aphasia or
| dysarthria
390
injury to Wernicke area may result in
fluent aphasia - pt may express thoughts fluently with a nonsensical speech pattern
391
detailed pertinent history is collected during which portion of survey
secondary
392
Sacral sparing is an example of an _____ spinal cord injury (SCI)
incomplete - some degree of sensory and/or motor function is preserved
393
the most common cause of spinal cord injury in children is
MVC
preventative measure - use a seat belt with shoulder harness in vehicles
394
what is the most sensitive test to evaluate for suspected spinal cord injury
MRI
395
if x ray and CT are neg for fracture what would most likely be cause of paralysiss
SCIWORA - spinal processes remain partially cartilaginous and may not demonstrate fractures but the impact can cause partial or complete spinal cord transection
396
most common chest injury in children resulting from a high energy impact injury such as a auto-ped.
pulmonary contusion
often has subtle symptoms and no other obvious injury to chest or chest wall
symptoms of resp distress can occur within 24-48 hours after injury
397
What types of hormones do the adrenal glands produce
glucocorticoids
mineralocorticoids
sex hormones
398
where are the adrenal glands located
on the kidneys
399
what type of hormones are the following:
Cortisol
Corticosterone
Glucocorticoids
400
what type of hormones maintain glucose control by affecting protein and carbohydrate metabolism
Glucocorticoid Hormones
| produced by the adrenal glands
401
what type of hormones inhibit glucose uptake in the muscle and adipose tissue
Glucocorticoid Hormones
402
What type of hormones stimulate gluconeogenesis (particularly in the liver)
Glucocorticoid Hormones
403
what type of hormones respond to stress by upregulating the expression of anti-inflammatory mediators and downregulating the expression of proinflammatory mediators
Glucocorticoid Hormones
404
If you have prolonged exposure to glucocorticoids, what syndrome will develop?
Cushing Syndrome
405
Obesity, muscle wasting/weakness, decreased glucose tolerance/hyperglycemia, buffalo hump.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 654). Wolters Kluwer Health. Kindle Edition.
Cushing Syndrome
406
what type of hormones are the following:
aldosterone
dehydroepiandrosterone
Mineralocorticoid hormones
407
what type of hormones maintain the balance of sodium and potassium in the body
Mineralocorticoid hormones
408
When aldosterone is increased, ______ develops
aldosteronism
409
Hypernatremia and hyperkalemia, resulting in hypertension.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 654-655). Wolters Kluwer Health. Kindle Edition.
aldosteronism
410
aldosteronism
Hypernatremia and hyperkalemia, resulting in hypertension.
411
If androgens are increased, _______ will be affected
sex characteristics
412
What type of hormones are
Androgens
progestins
estrogens
sex hormones
413
Disorders affecting the adrenal cortex lead to inadequate or absent production of
hormones
414
The most common causes of acute adrenal insufficiency are
Waterhouse–Friderichsen syndrome, sudden withdrawal of long-term corticosteroid therapy, and stress states in patients with chronic adrenal insufficiency.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 655). Wolters Kluwer Health. Kindle Edition.
415
sudden withdrawal of long-term corticosteroid therapy, and stress states in patients with chronic adrenal insufficiency
Waterhouse–Friderichsen syndrome
