Download CCC Dysautonomia -- Non Audio Guide

Complex Care Curriculum: DYSAUTONOMIA
Facilitator Guide for Small Group Teaching
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Please allow 35 minutes for this session with an additional 10-15 minutes for discussion and questions
Below is a slide-by-slide guide with suggested discussion points. The slides are equipped with animations that
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SLIDE
Complex Care
Curriculum
DISCUSSION POINTS/NOTES
Welcome to this discussion on Dysautonomia.
DYSAUTONOMIA
Dysautonomia
Sonaly Rao McClymont, MD
Sarah Helen Evans, MD
Melanie Anspacher, MD
Neha H. Shah, MD, MPH
Complex Care
Curriculum
DYSAUTONOMIA
Define dysautonomia in simple terms
Identify risk factors
Summarize basic pathophysiology
Identify signs and symptoms
Make the diagnosis
Describe the consequences
Make a basic treatment plan
By the end of this learning module you should be able to:
[click] Describe dysautonomia in simple terms
[click] Identify patients at risk for developing dysautonomia
[click] Have a basic understanding of the leading theories on
pathophysiology
[click] Identify signs and symptoms of dysautonomia
[click] Make the diagnosis
[click] Explain the consequences of untreated dysautonomia
[click] Have a basic understanding of the most common
medications used to treat dysautonomia, Make a
preliminary management plan for it and be able to revise
this plan over time with the help of a PM&R consultant
Complex Care
Curriculum
DYSAUTONOMIA
Metabolic Overdrive
Dysautonomia is a syndrome characterized by episodes of
autonomic nervous system “up-regulation” resulting in
metabolic hyperdrive and increased muscle tone.
It has also been described as a syndrome of simultaneous
and paroxysmal sympathetic and muscle overactivity
In simpler terms, dysautonomia can also be thought of as
[click] “metabolic overdrive” or “brainstorming” [click]
[click again to end]
“BRAIN-STORMING”
Complex Care
Curriculum
DYSAUTONOMIA
Severe Brain Injury
Traumatic
Infectious
Anoxic
Metabolic
Dysautonomia can occur in patients with old
neurologic injury.
Autoimmune
Increased
It
should
not
be
present
in the patient with no
Intracranial
known neurologicIntracranial
injury.
Pressure
Hemorrhage
*Incidence after Traumatic Brain Injury is approximately 10%
Complex Care
Curriculum
Spinal Cord
Injury
DYSAUTONOMIA
Dysautonomia
without Brain Injury
Dysautonomia: TACHYCARDIA
Autonomic Dysreflexia: BRADYCARDIA
Spinal
Cord Injury
above T6
Autonomic
Dysreflexia
Who is at risk for dysautonomia?
[click] Any patient with severe brain injury can develop
dysautonomia. Types of neurologic injury may include:
Traumatic, Anoxic, Acute increase in ICP, Infectious,
Metabolic, Autoimmune, or Intracranial Hemorrhage.
[click] Of note, incidence is approximtely 10% after
Traumatic Brain Injury (TBI)
[click] While any patient with a new brain injury is at risk,
keep in mind that dysautonomia may also occur in patients
with old neurologic injury without prior treatment for
dysautonomia.
[click] In addition, dysautonomia should not be present in a
presumed neurologically normal patient, therefore, if sxs of
dysautonomia persist in a person who is not known to have
any neurologic deficits, then strongly consider neurologic
imaging as part of the diagnostic evaluation.
I’d like to take a quick moment to differentiate between
brain injury and spinal cord inury
[click] Patients with spinal cord injury (SCI) without brain
injury will [click] NOT have dysautonomia
[click] If the SCI is above the level of T6 a patient can have
[click] 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 [click], Autonomic Dysreflexia is
generally associated with BRADYCARDIA (due to intact
parasympathetic inhibitory baroreceptor reflexes mediated
by the vagal nerve)
Complex Care
Curriculum
Disconnection
Theory
Cerebral Damage
DYSAUTONOMIA
D
Y
S
A
U
T
O
N
O
M
I
A
Excitatory:Inhibitory
Ratio Model
Damage to Central
Inhibition
Cortex↔Hypothalamus
x
Excessive Autonomic
Reactions
Autonomic Hyperactivity
Complex Care
Curriculum
Complex Care
Curriculum
DYSAUTONOMIA
DYSAUTONOMIA
Heart Rate
Respiratory
Rate
Agitation
SYMPTOMS
Sweating
Blood
Pressure
DysautonomiaTemperature
causes hyperthermia,
NOT temperature instability
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, [click] The Disconnection Theory and [click]the
Excitatory:Inhibitory Ratio Model are the leading theories on
pathophysiology.
[click] The Disconnection theory states that [click] diffuse or
focal cerebral damage affects the [click] functional
interaction between the cortex and hypothalamus [click]
causing autonomic hyperactivity
[click] The Excitatory:Inhibitory Ratio Theory states that
[click] damage to the central inhibitory structures and
pathways which regulate afferent information cause [click]
excessive autonomic reactions to painful or even normal
stimuli
In simpler terms – in patients with dysautonomia [click] the
control mechanism or regulation of the autonomic nervous
system is [click] broken. The lack of feedback inhibition
causes the ANS to act like a “Runaway Train.”
Can you name the 6 most common symptoms associated
with a dysautonomic storm?
[click] Tachycardia, [click] Tachypnea, [click] Hypertension,
[click] Hyperthermia, [click] Diaphoresis, [click] and Marked
Agitation. Additional symptoms include posturing
(decerebrate or decorticate), spasticity and dystonia.
-When assessing blood pressure with a manual or automatic
cuff, keep in mind that if a patient has increased tone
associated with his/her episodes, it makes the blood
pressure reading difficult to interpret - the reading is most
likely partially real and partially mechanical. A reading from
an arterial line, however, should be reliable.
-Also, remember to rely only on central temperatures when
assessing for hyperthermia.
[click] Make note that dysautonomia classically does not
include temperature instability – it is usually limited to
hyperthermia.
Complex Care
Curriculum
DYSAUTONOMIA
Differential Diagnosis for
Dysautonomia
Diagnosis Infection
High
Pain
of Dehydration
Index of
Drug fever
ExclusionHeart
Suspicion
disease
Rhabdomyolysis
Narcotic withdrawal
Complex Care
Curriculum
DYSAUTONOMIA
Random
Noxious
Stimuli
Constellation
Complex Care
Curriculum
DYSAUTONOMIA
Duration
Risks
Cost$
In this section, I will discuss how to make the diagnosis of
dysautonomia.
[click] The 2 main points to remember are that
dysautonomia is a diagnosis of exclusion and it requires a
high index of suspicion. As such, you must always rule out
other causes of these symptoms.
[click] [click] The differential diagnosis should be tailored to
your patient’s individual circumstances; however the
following diagnoses could masquerade as dysautonomia in
an at risk patient: Infection, Pain, Dehydration, Drug fever,
Heart disease, Rhabdomyolysis, and Narcotic Withdrawal
When the work-up for other causes of your patients
symptoms is negative, you must have a high index of
suspicion to ultimately diagnose and treat for
dysautonomia. Here are some clues that may help point you
in the right direction:
[click] There is a random pattern or timing to the onset of
symptoms
[click] The symptoms occur in response to noxious stimuli.
Keep in mind that the stimuli that trigger autonomic storms
may seem to be normal to the care providers however to
the brain-injured patient they can be noxious (for example,
loud noises, constipation, or full bladder).
[click] Dysautonomic storms occur as a constellation of
symptoms. Keep in mind that if the patient is already on
medications that control some symptoms you may not see
the entire constellation. For example a patient on a beta
blocker may not have tachycardia associated with their
storms.
Consequences of dysautonomia are associated with
sympathetic overdrive [click] such as increased energy
expenditure and decreased feeding tolerance. If left
untreated, it can lead to [click] longer duration of
posttraumatic amnesia, mechanical ventilation, and coma.
The longer the duration of symptoms, the increase in [click]
risk for secondary brain injury, nosocomial infections,
spasticity , dislocation of joints, fractures, development of
heterotopic ossification, and overall worse outcomes.
[click] All of these things translate into prolonged
hospitalization and greater overall healthcare cost.
Unfortunately, there is often a cyclical pattern associated
with untreated dysautonomia as the consequences are
frequently perceived as noxious to the patient resulting in
an increase in dysautonomic storms.
Complex Care
Curriculum
DYSAUTONOMIA
Complex Care
Curriculum
DYSAUTONOMIA
Decrease External Stimulation
Complex Care
Curriculum
DYSAUTONOMIA
Bowel and
Bladder
Musculo-
FEN/GI
Remove
Noxious
Stimuli
Respiratory
skeletal
DERM
Because dysautonomia is a hyperadrenergic state,
associated with elevation of catecholamine levels, the goals
of treatment are to [click] decrease sympathetic outflow
and to [click] enhance the parasympathetic system with
both [click] nonpharmacologic and pharmacologic methods.
Nonpharmacologic treatment includes [click] decreasing
external stimulation for your patient. What makes a
comfortable environment will vary from patient to patient
and it may take time to recognize what calms or triggers a
patient. [click] For example if a patient is out of bed and
becomes symptomatic you may try putting him/her back in
bed, however if a patient is laying supine you may try
different positioning. Try to create a calm and quiet
environment for your patient by [click] dimming the lights,
[click] decreasing the noise level, and limiting the number of
visitors. [click] Allow your patient plenty of rest and sleep.
[click] Holding or swaddling the patient can also help.
Nonpharmacologic treatment also includes [click] avoiding
noxious internal stimuli that may trigger an autonomic
storm.
These triggers may include
[click] Constipation, full bladder, kinked foley catheter,
urinary tract infection
[click] Fracture, heterotropic ossification, splints that are too
tight or have been on too long
[click] Pressure ulcers, IV site irritation, fingernails or
toenails that are too long
[click] Mucus plugs, increased secretions, inappropriate
ventilator settings
[click] Cold feeds, Gastroesophageal reflux, or 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.
I will now move on to discuss pharmacologic treatment
options.
Complex Care
Curriculum
DYSAUTONOMIA
First-line Agents
• Benzodiazepines
• Dopamine Agonists
• Beta Antagonists
Second-line Agents
• Alpha Agonists
• GABA B Agonists
• Calcium Channel Modulators
• Opiate Agonist
Complex Care
Curriculum
DYSAUTONOMIA
Benzodiazepines:
Diazepam
ACUTE management
Spinal cord AND Brain
Not for LONG TERM
Tolerance, Resp depression
[click] There are many types of medications that have been
shown to help manage dysautonomia and we will review
these individually over the next few slides. Keep in mind that
all drug classes will cause cognitive impairment. In addition
most of the drugs will have the additional effect of helping
with tone. [click] In the following slides pay particular
attention to Diazepam, which is an excellent choice for
acute mgmt, as well as Bromocriptine and Propanolol which
are the top choices for longer term management. [click]
Second line agents can also be used in combination with first
line agents to enhance treatment effect.
[click] Of the benzodiazepines, diazepam works very well
and works quickly, making it the best choice for acute
management.
[click] It acts both in the spinal cord AND the brain.
[click] Some of the cons of Diazepam include that it is not
suitable for longterm management of dysautonomia and
there is rapid development of tolerance. Due to the way in
which it is metabolized, there is an additive effect and thus a
risk of respiratory depression.
Complex Care
Curriculum
DYSAUTONOMIA
Acute
Management of
Dysautonomia
with Diazepam
Established on
Benzodiazepine?
YES
Complex Care
Curriculum
NO
Extra PRN
dose
Use starting
dose
Increase
frequency
Can repeat x
1
Increase
dose
WARNING:
RESP DEPRESSION
DYSAUTONOMIA
Benzodiazepines:
Clonazepam
Spinal cord AND Brain
LONGTERM management
Watch for seizures when weaning…
Tolerance
Cognitive Suppression
As stated, Diazepam is the best choice for acute
management of an autonomic storm.
To determine dosage the practitioner must first
[click] determine if the patient is on a benzodiazepine at
baseline.
[click] If already on a BZD, then consider these options:
[click] Give an extra prn dose of that BZD which is the same
amount as the scheduled dose,
[click] Increase frequency of dosing, or
[click] Increase the dose itself
If your patient has not yet been treated for dysautonomia
and is already on a BZD, it is most likely for management of
a seizure disorder or a narcotic wean. If this established BZD
does not appear to be controlling the new onset of
dysautonomia, consider switching from the established BZD
to Diazepam until the Dysautonomia is under better control.
[click] If your patient is NOT already on a benzodiazepine
then
[click] Use Diazepam at the starting dose in the formulary;
[click] If no results after 30 minutes you may repeat the
dose once.
[click] Remember, If you increase the dose too rapidly, give
frequent PRN doses, or start with too high a dose the active
metabolite will eventually build up and cause [click]
respiratory depression.
Also, when you are trying to adjust doses, keep in mind that
parents, if available and involved, can be very helpful in
knowing the background of what works for the patient.
Finally, remember to take vital signs more frequently when
assessing for response to new doses.
[click] If a patient responds to acute management with
Diazepam you may transition to Clonazepam for better long
term management.
[click] Like Diazepam, it also acts in the spinal cord AND
brain.
[click] While tolerance and cognitive suppression are side
effects of clonazepam, it causes the slowest progression to
tolerance of all the benzodiazepines and it is less cognitive
suppressive compared to diazepam.
[click] KEY POINT: When weaning clonazepam monitor
closely for seizures which may occur due to unmasking of
an underlying seizure disorder OR simply weaning too
quickly
Complex Care
Curriculum
DYSAUTONOMIA
Dopamine agonists:
Bromocriptine
1st line
Acts centrally
Least cognitive suppression
[click] Bromocriptine, a dopmaine agonist, is thought to be
effective for treating dysautonomia by regulating neuronal
excitability. Dopamine is an inhibitory neurotransmitter that
is thought to help regulate the autonomic nervous system
which is in a state of chaos in patients with dysautonomia.
[click] It is considered first line treatment for longterm
management. It acts centrally and has the least cognitive
suppressive effects of all the medications used to treat
dysautonomia. [click] The main side effect to watch for is
hypotension.
Hypotension
Complex Care
Curriculum
DYSAUTONOMIA
Beta-antagonists:
Propranolol
1st choice if Hypertension
sympathetic overdrive
Penetrates
blood-brain
barrier
Albuterol
can be used
to counteract side
effects
of Propranolol acutely
Bradycardia
Secondary Hypotension
Complex Care
Curriculum
DYSAUTONOMIA
Beta antagonists:
Labetalol
Intravenous formulation
Hypotension
$$$
[click] Of the beta-antagonists, Propanolol is the [click] 1st
choice for patients with prominent HYPERTENSION. It
minimizes sympathetic overdrive by decreasing circulating
catecholamines, reducing cardiac work and reducing
catabolic drive. It also penetrates the Blood Brain Barrier
and is therefore thought to act centrally as well. [click] Side
Effects include : Bradycardia and Secondary Hypotension.
KEY POINT: If your patient is experiencing bradycardia or
hypotension secondary to an increased dose of propanolol
then consider giving albuterol [click], a beta agonist, to
counteract this beta blocker. Also, if a patient requires
Propanolol at baseline for dysautonomia and also requires
Albuterol for pulmonary disease – consider switching to
Xopenex for pulmonary disease to improve the efficacy of
propanolol on the heart.
[click] Labetolol, another beta antagonist, while less
commonly used for dysautonomia, [click] is an excellent
choice for the patient who is NPO because it is available in
an IV formulation. [click] The most notable side effect is
hypotension. Due to differences in mechanism of action,
one must monitor blood pressure more closely than you
would with propanolol. In addition, it is significantly more
expensive than propanolol.
Complex Care
Curriculum
DYSAUTONOMIA
Alpha agonists:
Clonidine
Transdermal patch available
Acts at spinal cord AND brain
Rapid onset of action - oral
Cognitive suppression
Hypotension
Complex Care
Curriculum
DYSAUTONOMIA
Calcium Channel Modulators:
Gabapentin
[click] Of the alpha agonists, Clonidine is the most
commonly used medication for treatment of dysautonomia,
often used [click] transdermally in a patch formulation. It
acts at the spinal cord and brain and has the added benefit
of rapid onset of action when given orally so can be used in
acute situations or in patients who are already on multiple
drugs. [click] Clonidine is not good for cognitive recovery in
patients with neurologic injury. In addition it can cause
hypotension and requires careful monitoring of blood
pressure. Once your patient is stabilized, wean off Clonidine
as soon as able to limit further cognitive suppression.
[click] The calcium channel modulators are less efficacious
medications for treating dysautonomia. [click] Gabapentin,
while helping to regulate dysautonomia, can also manage
tone and neuropathic pain. In addition, it has a relatively
safe profile. [click] The major side effect is sleepiness that is
dose-dependent and resolves with time.
Can also manage tone and
neuropathic pain
Sleepiness
Complex Care
Curriculum
DYSAUTONOMIA
Calcium Channel Modulators:
Dantrolene
No affect on seizure threshold
No cognitive suppression
Treats hypertonia
Severe hepatotoxicity
[click] Dantrolene is another calcium channel modulator
that can be part of a management plan for dysautonomia.
[click] Benefits include the fact that it does not affect seizure
threshold, it does not cause cognitive suppression, and it is
effective treatment for hypertonia. [click] However the
severity of the potential side effects, mainly hepatotoxicity,
are worth considering. There is a black box warning
associated with the use of dantrolene secondary to the risk
of fatal chemical hepatitis. For most patients it is dose
related. Careful monitoring of the liver enzymes may
preclude this serious side effect.
Complex Care
Curriculum
DYSAUTONOMIA
GABA B Agonists:
Baclofen
Useful in patients with spasticity
Acts in Spinal Cord
in Brain
if given
orally
Abrupt AND
withdrawal
can lead
to seizures
and
rhabdomyolysis
Sleepiness initially
Lowers seizure threshold
Complex Care
Curriculum
DYSAUTONOMIA
Opiate Agonists:
Morphine, Methadone
Used to treat pain
Suppresses sympathetic outflow
Opiate wean can unmask dysautonomia, which
can look similar to opiate
withdrawal
Sedation
Respiratory depression
Limit rehab potential
Complex Care
Curriculum
MOST
DYSAUTONOMIA
Morphine, Methadone
Clonidine
Diazepam, Clonazepam
Propranolol, Labetalol
Baclofen
LEAST
Gabapentin, Dantrolene
Bromocriptine
[click] Baclofen, a GABA B Agonist, is not used in first line
treatment for dysautonomia. [click] It is however a 1st line
treatment for spasticity and often has the helpful side effect
of decreasing sxs of dysautonomia. It acts primarily in spinal
cord, but oral formulation may act on the brain as well.
[click] Side Effects include sleepiness when initiating which
usually resolves after a few days and lowering of the seizure
threshold.
[click] KEY POINT: Very rapid withdrawal can cause seizures
and rhabdomyolysis; this is usually only seen with
intrathecal baclofen withdrawal.
[click] Similarly, Opiates like morphine and methadone, are
not used in first line treatment for dysautonomia. [click]
They are used to treat pain and have the helpful side effect
of easing the symptoms of dysautonomia. Specifically, the
opiates modify the extremes of autonomic nervous system
changes and dystonic posturing while suppressing
sympathetic outflow. [click] Unfortunately, opiates “turn
off” the whole system due to sedative effects which makes
rehabilitation therapies very difficult.
KEY POINT: Many brain injured patients will require an
opiate and/or benzodiazepine wean after prolonged
sedation (for example after prolonged intubation in the
intensive care unit). [click] Keep in mind that what may
appear to be symptoms of withdrawal could actually be the
unmasking of dysautonomia, particularly if the patient is on
a slow wean. If this is suspected, consider adding another
medication that treats dysautonomia while continuing to
wean the other medications.
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 [click] most cognitive
suppression first and continue to wean all medications as
tolerated.
Complex Care
Curriculum
DYSAUTONOMIA
TIPS:
Multiple categories
Consider sleep habits
Complex Care
Curriculum
DYSAUTONOMIA
Do you think this
is dysautonomia?
I tried bromocriptine,
what else can I try?
[click] Aggressive early management with medications from
multiple categories will aid in preventing secondary brain
injury and further consequences of uncontrolled
dysautonomia as discussed earlier. Once a patient is
controlled you can slowly wean off medications as tolerated
[click] When considering dosing schedule remember that
patients should not be storming when asleep
--If your patient is sleeping well, dosing can be during the
daytime (BID, TID, QID)
--If not sleeping well then give around the clock dosing (q12,
q8, q6)
Pediatric physical medicine and rehabilitation physicians
specialize in managing patients with dysautonomia. Call
them early for expert help when you suspect dysautonomia
and need help managing it. As a hospitalist, you can start
and titrate meds on your own based on your comfort level,
but always call PM&R for assistance when needed.
Be sure to have vital signs and patterns to report.
How soon can I titrate
the dose?
Complex Care
Curriculum
DYSAUTONOMIA
In this final section, I will summarize the key points
presented in this module.
[click] Dysautonomia occurs when the control mechanism or
regulation of the autonomic nervous system is broken
resulting in [click] metabolic overdrive. Any patient with
brain injury is at risk.
HR
RR
BP
Temp
Sweating
Agitation
[click] The most prominent symptoms are tachycardia,
tachypnea, hypertension, hyperthermia, diaphoresis,
agitation—they occur together as a constellation of
symptoms.
Complex Care
Curriculum
DYSAUTONOMIA
Diagnosis
of
Exclusion
High
Index of
Suspicion
Non-pharmacologic
Pharmacologic
• Decrease external
stimulation
• Remove internal noxious
stimuli
• Diazepam Acute
• Propranolol and
BromocriptineChronic
Complex Care
Curriculum
DYSAUTONOMIA
[click] Remember that dysautonomia is a diagnosis of
exclusion and requires a high index of suspicion.
[click] Treatment is both nonpharmacologic and
pharmacologic.
Diazepam is the best choice for acute management.
Propanolol and Bromocriptine are the best choices for long
term management.
[click] Remember, difficult cases may require medications
from multiple categories.
[click] When the patient is stabilized, always wean off the
most cognitive suppressive medications first.
[click] Finally, call your friendly neighborhood pediatric
physiatrist early! And always have vital signs and patterns
to report.
Wean meds
based on
cognitive
supression
Complex Care
Curriculum
DYSAUTONOMIA
Baguley IJ, Heriseanu RE, Cameron ID, Nott MT, SlewaYounan S: A Critical Review of the Pathophysiology of
Dysautonomia Following Traumatic Brain Injury. Neurocrit
Care 2008; 8(2):293-300.
Baguley IJ, Cameron ID, Green AM, Slewa-Younan S,
Marosszeky JE, Gurka JA: Pharmacological management of
Dysautonomia following traumatic brain injury. Brain Inj
2004 May; 18(5):409-17.
Hendricks HT, Heeren AH, Vos PE.: Dysautonomia after
severe traumatic brain injury. Eur J Neurol 2010
Sep;17(9):1172-7.
Taketomo, Carol K, Jane H Hodding, and Donna M Kraus.
Pediatric Dosing Handbook. Hudson: Lexi-Comp, 2010. Print.
To generate audience discussion consider using the
following reflective questions:
Have you ever take care of a patient with dysautonomia?
-Describe that experience.
-Describe your management of that patient.
What can you take away from this session that will change
your management of dysautonomia in the future?