Download CCC Dysautonomia

Complex Care Curriculum: DYSAUTONOMIA
Pre- and Post-Module Questions – ANWER KEY
Question #1
Circle the MOST appropriate of the 2 bolded word choices to complete each statement
related to dysautonomia:
A. Dysautonomia is a syndrome characterized by episodes of central/autonomic
nervous system dysfunction.
B. Dysautonomia causes up-regulation/down-regulation of the above-mentioned
nervous system resulting in metabolic over-activity/under-activity and
increased/decreased muscle tone.
C. Based on statements A and B, the goals of treatment are to increase/decrease
sympathetic outflow and mute/enhance the parasympathetic system.
ANSWER #1:
A. Dysautonomia is a syndrome characterized by episodes of autonomic nervous
system dysfunction.
B. Dysautonomia causes up-regulation of the autonomic nervous system resulting
in metabolic over-activity and increased muscle tone.
C. Based on statements A and B, the goals of treatment are to decrease
sympathetic outflow and to enhance the parasympathetic system.
Question #2
Which of the following patients is LEAST likely to be at risk for dysautonomia?
A. 8 year-old previously healthy female status-post a near-drowning event with
resultant hypoxic ischemic encephalopathy, who is now tracheostomy/ventilator
and gastrostomy tube dependent.
B. 1 month-old full term healthy male who presented with seizures due to GBS
meningitis and subsequently developed cerebral palsy and severe neurologic
impairment.
C. 13 year-old previously healthy male who was playing tackle football and
sustained a C5-C6 spinal cord injury resulting in quadriplegia.
D. 2 year-old ex-26 wk premature infant who is tracheostomy/ventilator dependent
and globally developmentally delayed with recent accidental decannulation
event causing prolonged oxygen deprivation and new anoxic brain injury.
E. 10 year-old male with no significant past medical history who was an
unrestrained passenger in a motor vehicle accident which resulted in severe
traumatic brain injury.
ANSWER #2: C. 13 year old football player s/p spinal cord injury.
Any patient with severe brain injury can develop dysautonomia. Types of neurologic
injury may include: traumatic, anoxic, acute increase in intracranial pressure, infectious,
metabolic, autoimmune, or intracranial hemorrhage. Patients with spinal cord injury
(SCI) without brain injury will NOT have dysautonomia. If the SCI is above the level of T6
a patient can have Autonomic Dysreflexia in which there is excessive sympathetic
outflow from the splanchnic plexus in response to noxious stimuli. Autonomic
Dysreflexia will manifest clinically with similar symptoms and signs as dysautonomia
(such as increased blood pressure, facial flushing, and sweating), however, Autonomic
Dysreflexia is generally associated with BRADYCARDIA (due to intact parasympathetic
inhibitory baroreceptor reflexes mediated by the vagal nerve).
Question #3
Which of the following is the leading theory on the pathophysiology of dysautonomia?
A. The Epileptogenic Theory – damage to the cerebral cortex causes abnormal
paroxysmal neuronal hyperactivity that can be halted with some but not all
antiepileptic drugs.
B. The Disconnection Theory – cerebral damage affects the interaction between the
cortex and hypothalamus causing nervous system dysregulation.
C. The Excitatory:Inhibitory Ratio Theory – damage to central inhibitory
structures/pathways cause abnormal reactions to painful or even normal stimuli.
D. Both A and B are leading theories at this time
E. Both B and C are leading theories at this time
ANSWER #3: E. Both B and C are leading theories at this time
There is a limited understanding of the pathophysiology of dysautonomia. It was
originally thought to have an epileptogenic cause however EEGs did not consistently
demonstrate epileptiform activity and anticonvulsant drugs failed to treat the
symptoms. Currently, The Disconnection Theory and the Excitatory:Inhibitory Ratio
Model are the leading theories on pathophysiology.
Question #4
Which of the following is NOT classically associated with an autonomic storm?
A. Tachycardia
B. Increased Tone
C. Temperature Instability
D. Agitation
E. Sweating
ANSWER #4: C. Temperature instability.
Because dysautonomia is a hyperadrenergic state (associated with elevation in
catecholamine levels) it classically results in hyperthermia and not temperature
instability.
For the remaining questions use this case scenario as the background
for making your clinical decisions:
Alice is a 5 year-old previously healthy female who choked on a grape
and sustained a severe anoxic brain injury. She has subsequently
developed severe neurologic impairment with seizures and respiratory
failure.
Question #5
Alice’s anoxic injury occurred just 1 week ago and she is currently in the ICU. She is
sedated and intubated on a ventilator. She has a central line for parenteral nutrition.
She is on multiple anti-epileptic drugs for seizure control. She is hemodynamically
stable. Today on rounds, as the team begins discussion of her management plan by the
bedside she suddenly starts to exhibit tachycardia, tachypnea, hyperthermia, sweating
and agitation.
Other than dysautonomia, name at least three other possible etiologies that must be
evaluated prior to moving forward with a diagnosis of dysautonomia.
1.
2.
3.
Others (optional):
ANSWER #5:
Dysautonomia is a diagnosis of exclusion and it requires a high index of suspicion
therefore you must always rule out other causes of these symptoms. The following
diagnoses could masquerade as dysautonomia in an at risk patient such as Alice:
Infection, Pain, Dehydration, Drug fever, Heart disease, Rhabdomyolysis, and Narcotic
Withdrawal. Further elaboration of the differential diagnosis should be tailored to your
patient’s individual circumstances. In this specific case you could also consider
intractable seizures.
Question #6
Alice continues to have these episodes that are consistent with dysautonomia. You
have been off-service for the weekend and the colleague who’s been covering for you
has been continuing the work-up you started but has not been able to control these
episodes.
If Alice ultimately does have dysautonomia and continues to go untreated name at least
4 possible consequences that could develop due to uncontrolled dysautonomia?
1.
2.
3.
4.
Others (optional):
ANSWER #6:
The consequences of dysautonomia are extensive and tend to be associated with
sympathetic overdrive such as increased energy expenditure and decreased feeding
tolerance. If left untreated, it can lead to longer duration of posttraumatic amnesia,
mechanical ventilation, and coma. The longer the duration of symptoms, the increase
in risk for secondary brain injury, nosocomial infections, spasticity, dislocation of
joints, fractures, development of heterotopic ossification, and overall worse outcomes.
All of these things translate into prolonged hospitalization and greater overall
healthcare cost.
Question #7
You are the on-call physician overnight when Alice starts having dysautonomic episodes
again. Which of the following is the best pharmacologic option for acute intervention to
stop her symptoms?
A.
B.
C.
D.
E.
Bromocriptine
Baclofen
Morphine
Propanolol
Diazepam
ANSWER #7: E. Diazepam
Diazepam works very well and works very quickly, making it the best choice for acute
management of a dysautonomic episode. The main risk of acute use of diazepam is
respiratory depression which can be treated with varying levels of respiratory support
including intubation if needed. Propanolol may also work quickly in an acute setting,
however in regard to potential overdose the risk is higher and the consequences
(bronchospasm and bradycardia) are more difficult to control compared to diazepam.
Bromocriptine is best for long term management and will not act quickly in an acute
situation. Baclofen and Morphine are not first-line agents for dysautonomia.
Question #8
The medication that you chose worked well and you have been able to stop acute
episodes when you notice them. You would like to start Alice on a scheduled
medication regimen and have decided to consult with your local pediatric physiatrist on
further options for acute management.
While you are awaiting recommendations for medical management, name 5 potential
triggers of autonomic storms that you may be able to relieve with nonpharmacologic
measures?
1.
2.
3.
4.
5.
Others (optional):
ANSWER #8:
Nonpharmacologic treatment includes avoiding noxious internal stimuli that may trigger
an episode. There are a wide variety of triggers that may include:
Bowel and Bladder issues: Constipation, full bladder, kinked foley catheter, urinary tract
infection
Musculoskeletal issues: Fracture, heterotropic ossification, splints that are too tight or
have been on too long
Dermatologic issues: Pressure ulcers, IV site irritation, fingernails/toenails that are too
long
Respiratory issues: Mucus plugs, increased secretions, inappropriate ventilator settings
GI issues: Cold feeds, Gastroesophageal reflux, dehydration
It is important to educate the entire care team about potential triggers for
dysautonomia so that action can be taken as soon as possible to relieve the noxious
stimulus.
Nonpharmacologic treatment also includes decreasing external stimulation for your
patient. What makes a comfortable environment will vary from patient to patient.
Options may include re-positioning OR creating a calm and quiet environment for your
patient by dimming the lights, decreasing the noise level, and limiting the number of
visitors. You should also allow your patient plenty of rest and sleep. Holding or
swaddling the patient can also help.
Question #9
Your friendly pediatric physiatrist evaluated Alice and gave recommendations for
multiple medications for both acute and long-term management of Alice’s
dysautonomia.
Which of the following do you think he/she recommended as the MOST appropriate
choice for long term management of dysautonomia?
A.
B.
C.
D.
E.
Bromocriptine
Baclofen
Morphine
Propanolol
Diazepam
ANSWER #9: A. Bromocriptine
Bromocriptine, a dopamine agonist, is thought to be effective for treating dysautonomia
by regulating the autonomic nervous system which is in a state of chaos in patients with
dysautonomia. Bromocriptine acts centrally and has the least cognitive suppressive
effects and is considered the MOST appropriate for long term management of
dysautonomia. Propanolol is a first line treatment in patients with dysautonomia
however due to its local cardiovascular effects, potential side effect of bronchospasm,
and cognitive suppressive effects it is not the most preferred for long term management.
Diazepam is best for acute management. Baclofen and Morphine are not first-line
agents for dysautonomia.
Question #10
Alice is now 4 months out from her initial neurologic insult and is undergoing inpatient
rehabilitation in a subacute care facility and has had no episodes of dysautonomia in the
last several weeks. You are taking care of her during this time and would like to try to
wean her off the medications she is receiving for dysautonomia to simplify her
medication regimen for her family and maximize her rehabilitation potential.
In which order would you attempt to wean these medications?
A.
B.
C.
D.
E.
Bromocriptine, Diazepam, Propanolol
Propanolol, Bromocriptine, Diazepam
Propanolol, Diazepam, Bromocriptine
Diazepam, Propanolol, Bromocriptine
Diazepam, Bromocriptine, Propanolol
ANSWER #10: D. Diazepam, Propanolol, Bromocriptine
Because most drugs used to treat dysautonomia have cognitive suppressive side effects
and because dysautonomia should improve over time with recovery from the brain
injury, careful attention should be paid to the potential for weaning these medications.
Wean the medications that cause the most cognitive suppression first and continue to
wean all medications as tolerated. You can use the following guide to prioritize base on
cognitive suppression:
MOST Cognitive Suppressive
Morphine, Methadone
Clonidine
Diazepam, Clonazepam
Propranolol, Labetalol
Baclofen
Gabapentin, Dantrolene
Bromocriptine
LEAST Cognitive Suppressive