Dysautonomia Flashcards

1
Q

What is the autonomic nervous system broken down into

A
  • Sympathetic (thoracolumbar)
  • Parasympathetic (craniosacral)
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2
Q

What are the functions of the autonomic nervous system

A
  • Eyes: dilate (Sym) and constricts (Para)
  • Skin: hairs stand straight, sweating, vasoconstricts (Sym)
  • Lacrimal & salivary glands: decreased secretion/thicker/more viscous (Sym) and promotes secretion/watery (Para)
  • Heart: increase HR (Sym) and decrease HR (Para)
  • Lungs: bronchodilation (Sym) and constricts bronchi (Para)
  • Digestive tract: constricts BV to digestive tract & contracts internal anal sphincter (Sym) and contracts rectum (Para)
  • Liver & gallbladder: promotes breakdown of glycogen to glucose (Sym) and promotes building of glycogen (Para)
  • Urinary tract: vasoconstriction of renal vessels (Sym) and inhibits contraction of the internal sphincter of bladder (Para)
  • Genital system: ejaculation (Sym) and erection (Para)
  • Suprarenal medulla: release of adrenaline into blood (Sym)
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3
Q

The anatomical distinction between the sympathetic and parasympathetic divisions of the ANS is based primarily on

A
  • The location of the presynaptic cell bodies: intermediolateral cell column or nuclei of spinal cord T1-L3 (Sym) and gray matter of brainstem & the fibers exit the CNS within CN III, VII, IX, & X and gray matter of sacral segments S2-S4 (Para)
  • Which nerves conduct the presynaptic fibers from the CNS
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4
Q

What neurotransmitter does each division of the ANS liberate

A
  • Sympathetic: norepinephrine
  • Parasympathetic: acetylcholine
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5
Q

Define dysautonomia

A
  • Umbrella term used to describe various conditions that cause a malfunction of the ANS
  • The ANS controls most of the essential functions of the body that we do not consciously think about
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6
Q

Autonomic dysfunction is common in what conditions

A
  • Neurologic conditions
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7
Q

Symptoms of dysautonomia

A
  • Sweating abnormalities
  • Dizziness and fainting
  • Vision problems
  • Exercise intolerance
  • Nausea
  • Digestive difficulties
  • Urinary issues
  • Sexual problems
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8
Q

What diagnosis’s fall under the umbrella of dysautonomia

A
  • POTS: postural orthostatic tachycardia syndrome
  • IST: inappropriate sinus tachycardia
  • NCS: neurocardiogenic syncope
  • MSA: multiple system atrophy
  • AAG: autoimmune autonomic ganglionopathy
  • FD: familial dysautonomia
  • PAF: pure autonomic failure
  • DBHD: dopamine beta hydrolase deficiency
  • OI: orthostatic intolerance
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9
Q

Ddx between POTS and OI

A
  • OI: no significant BP drop and no significant HR elevation
  • POTS: no significant BP drop and sustained elevated HR >30 BPM or >120 bpm
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10
Q

Ddx between neurogenic OH and cariogenic OH

A
  • Neurogenic: orthostatic drop in BP and delta HR/delta SBP ratio <0.5
  • Cardiogenic: orthostatic drop in BP and appropriate HR response
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11
Q

Describe a normal baroreceptor response

A
  • Fluid pooling occurs with posture change
  • Decreased venous return, cardiac out put
  • Decreased parasympathetic, decreased activation of carotid sinus & aortic arch baroreceptors
  • Increased Sympathetic
  • Increased vasoconstriction, HR, & cardiac output
  • Blood [pressure maintenance
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12
Q

Primary causes of neurogenic OH

A
  • Neurocardiogenic syncope
  • Postural orthostatic tachycardia syndrome (POTS)
  • Multiple system atrophy
  • Familial dysautonomia
  • Pure autonomic failure
  • Parkinson’s disease
  • Lewy body dementia
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13
Q

Steps in the process of OH intervention

A
  • Medication review: identify meds that may cause/make OH worse
  • Nonpharmacologic measures: increased fluid & salt intake, compression stockings, abdominal binder, activity & exercise, and education about triggers
  • Pharmacologic measures to reduce OH: Midodrine & droxidopa (FDA approved); fludrocortisone & pyridostigmine (off-label)
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14
Q

Education for OH prevention with nonpharmacologic measures

A
  • Manage fluid intake/loss & venous pooling: drink 2 liters/day of water, increase salt intake, elevate head of bed while sleeping, compression garments, ankle pumps/isos, treat anemia/B12 deficiency if present
  • Avoid triggers/reduce risk: smaller meals, fewer carbs, & anticipate OH after large meals, avoid increased body temp., bathe with shower chair
  • Physical conditioning: recumbent bike, rowing, & water activities
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15
Q

Cardiovascular response during exercise

A
  • Increased HR, cardiac mass, & cardiac function
  • Increased baroreceptor reflex
  • Decreased sympathetic tone & increased vagal tone
  • Increased renal & adrenal function
  • Increased hemoglobin mass & blood/plasma volume
  • Decreased insulin resistance
  • Increased endothelial function
  • Decreased blood pressure
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16
Q

Describe paroxysmal sympathetic hyperactivity (PSH)

A
  • Most common in severe TBI patients
  • Often observed with withdrawal of sedation
  • Average duration is 18-162 days
  • Typically resolves within a year
  • Episodic presentation if increased HR/sympathetic Sx
  • Sympathetic overactivity: increased HR, BP, RR, temp., & sweating; motor activity (posturing)
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17
Q

What are the 2 hypotheses as to why PSH happens

A
  • 1) Epileptogenic cause: not well supported
  • 2) Cerebral brain stem disconnection syndromes
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18
Q

Clinical features of PSH

A
  • HR <100
  • RR <18
  • Systolic BP <140
  • Temperature <98.6
  • No sweating
  • No posturing during episodes
  • Rated on 0-3 scale, zero values listed above
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19
Q

How to diagnose PSH

A
  • Person had a brain injury
  • Sympathetic overactivity to normally non painful stimuli
  • Features persist for >3 consecutive days for >2 wks post injury despite Tx of other possible diagnoses
  • Medications used to decrease sympathetic function
  • No other explanations
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20
Q

What is the scoring ranges for PSH

A
  • Mil: 0
  • Mild: 1-6
  • Moderate: 7-13
  • Severe: ≥13
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21
Q

What to do for autonomic dysreflexia

A
  • Recognize symptoms
  • Confirm increased BP
  • Take steps to lower BP: elevate head/sit up & lower feet, look for triggers (catheter malfunction, kinked tube, full leg bag, tight clothes, binder), & investigate other triggers
  • Medical treatment to lower BP
  • Patient/family education: have AD kit available
22
Q

Describe autonomic dysreflexia

A
  • Serious condition that can occur with lesions above T6
  • Rare before one month post injury
  • Starts in first 6 mo-1 yr
  • Systolic >20 mmHg over baseline (adults)
  • > 15 mmHg over baseline (children)
  • Can result in increased BP >300 mmHg systolic = life-threatening levels
23
Q

What are the systolic values for those with autonomic dysreflexia

A
  • > 150 mmHg in adults
  • > 140 mmHg in adolescents
  • > 130 mmHg in children 6-12 yrs
  • > 120 mmHg in children ≤5 yrs
24
Q

Symptoms associated with POTS

A
  • Lightheadedness
  • Palpitation (heart racing)
  • Tremulousness
  • Atypical chest discomfort
25
Q

Symptoms associated with POTS that are not necessarily associated with particular postures

A
  • Sleep disturbances
  • Headaches
  • Chronic fatigue
  • Chronic pain
  • Exercise intolerance/deconditioning
  • Perceived cognitive impairment (brain fog)
  • Peripheral acrocyanosis (POTS feet)
  • Frequent nausea
  • Mild diarrhea/constipation/bloating/unspecific abdominal pain (irritable bowel syndrome)
26
Q

POTS hemodynamic criteria

A
  • Excessive orthostatic tachycardia: sustained increase in HR from supine to upright of 30 bpm within 10 min of standing if older than 19 yrs
  • HR above threshold should be seen on at least 2 measurements at least 1 min apart to be sustained
  • Diagnostic orthostatic tachycardia must occur in the absence of sustained OH (BP changes within 3 min of standing)
  • Transient initial OH (lasting <1 min) doe snot preclude the diagnosis of POTS
27
Q

Symptoms of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS)

A
  • Severe fatigue
  • Difficulty sleeping
  • Headaches
  • Muscle aches
  • Difficulty with concentration & memory
  • Joint pain
    Less common
  • Vision problems
  • Chills
  • Night sweats
  • Swollen lymph nodes
  • Dizziness & fainting
  • Tingling or numbness
  • Mood swings, irritability, & anxiety
  • Irritable bowel, painful bloating, gas, constipation, & diarrhea
28
Q

Diagnosis of ME/CFS

A
  • Requires all 3 symptoms occur at least half of the time with moderate, substantial, or severe intensity: profound fatigue & impairment that lasts >6 mo, boost-exertional malaise (PEM), & unrefreshing sleep
  • In addition at least one of the following symptoms must be present: impaired memory or ability to concentrate and/or orthostatic intolerance
29
Q

Causes of ME/CFS

A
  • Cause of disease is unknown but onset may follow an infectious like syndrome
30
Q

Describe myalgic encephalomyelitis (ME)/Chronic fatigue syndrome (CFS)

A
  • Disabling & complex disease diagnosed by exclusion
  • 50-80% start with flu-like symptoms & Donn’t recover
  • Cognitive, immunological, endocrinological, & autonomic dysfunction
  • Characterized by persistent post-exertional malaise
31
Q

What is mast cell activation syndrome (MCAS)

A
  • Improper functioning of mast cells: possible link to EDS and/or POTS
  • Constant histamine responses -> sympathetic overdrive -> poor response to treatment
  • Respond well to dysautonomia protocol once medically managed
32
Q

What is long COVID

A
  • Research has established a connection b/w long COVID & dysautonomia but more is needed
  • Present with GI, thermoregulatory, & OI complaints
  • Clinically presents similarly to post-concussive syndrome
  • Possible improvements with longer treatment duration
33
Q

No-pharmacologic management of dysautonomia

A
  • Minimize exacerbating factors: heat, carb rich meals and/or alcohol, & prolonged standing
  • Cooling vest
  • Sleep hygiene
  • Electrolyte replacements
  • 2-3L fluid daily
  • Compression
  • Exercise prescription: daily cardio is key, can incorporate breath work/meditation, & educate on rationale
34
Q

Pharmacologic management of dysautonomia

A
  • Fludrocortisone
  • Propranolol
  • Midodrine
  • Pyridostigmine
35
Q

What outcome measure can be used to screen for dysautomia

A
  • Compass 31
  • Patient specific functional scale (PSFS)
  • Post concussion symptom scale
  • Dizziness handicap inventory
  • Brain injury visual symptom survey (BIVSS)
  • Malmo POTS questionnaire
  • Depaul symptom questionnaire (shot form) - post-exertional malaise
36
Q

Examination for dysautonomia

A
  • Exertional testing: upright tolerance testing, Buffalo concussion treadmill test, & 5 times sit to stand
  • NASA lean test
  • Active stand test
37
Q

Importance of HR variability

A
  • Indicator of autonomic function reflecting adaptation to change over time
  • More variability is better generally
  • Reduced HRV: less ability to respond to stress of all types
  • Diurnal variation: PS input greater at night, sympathetic increases in response to daily activities & stress
38
Q

HRV metrics and why measurement duration is important

A
  • Time based domain: SDNN (standard deviation of normal-to-normal) - sympathetic & parasympathetic contributions, 24 hr monitoring to ID cardiac risk
  • Frequency domain: power spectrum analysis at least 5 min., high frequency of 0.15-0.4 Hz = respiratory effects/sinus arrhythmia, low frequency of 0.04-0.15 Hz = sympathetic/parasympathetic, barorecptor, & vasomotor activity
39
Q

Criteria for transitioning within stages for CHOP protocol for POTS

A
  • Increase HR by 5 bpm or 1/10 RPE once pt is able to complete the following for 3-5 days
  • 20 min in 1 duration
  • No symptom increase during or after exercise (goal is not symptom free)
  • Good HR control
  • W/o manipulating the workload (consistent load); normal that each day the target load may be different
40
Q

Criteria for transitioning between stages for CHOP protocol for POTS

A
  • Target 3 increases per stage
  • Re-establish resting HR in new posture: add 15-20 bpm > resting HR/start back at 2-3/10 RPE
  • Always alternate days in transition to ensure tolerance: progress to daily, then recommend HR changes
  • Exertional testing: Utah ADaPT: at least by stage 3 -> 4 transition to confirm ready for upright
41
Q

Describe the preconditioning stage of CHOP protocol for POTS

A
  • Goal: total minutes per day & reducing fear avoidance/central sensitization to exercise
  • Initiate 2x5 min., 4x5 min., 2x10 min & build minutes until 20 continuous minutes
  • If regressing -> may have to recommence in pre-conditioning stage pending severity
  • Considerations: motivational interviewing & reduction of pain catastrophizing, improving pacing strategies & symptom management, meditation/grounding/neural calming techniques, psych/counseling interventions
42
Q

Describe stages 1 & 2 in the CHOP protocol for POTS

A
  • Stage 1(Horizontal plane): wall slides, heels slides, hook lying marching, pool, total gym or reformer; typically use heel slides on table to achieve higher HR & increases core activation
  • Stage 2 (Recumbent): bike at 15-20 bpm > supine resting HR (2/10 RPE); continue sequential transition & modify once on upright bike; easier to regulate workload due to RPM/resistance recordings
43
Q

Describe stages 3 & 4 in the CHOP protocol for POTS

A
  • Stage 3 (Upright bike): 15-20 bpm > seated resting HR (2-3/10 RPE), not all bikes have speedometer but can purchase one
  • Stage 4 (Walking): treadmill is preferable; be mindful of strength & conditioning (inclines will become limiting factor for HR increases but can use interval training)
44
Q

Describe stage 5 of the CHOP protocol for POTS

A
  • Interval training stage
  • Most important to increase parasympathetic drive (vagal tone)
  • Will be easier than steady state day: work/recovery HR
  • Alternate with walking steady state day
45
Q

What are some interval modifications for POTS patients working through the CHOP protocol

A
  • Pts struggling to transition to upright: focus on building exercise tolerance on bike & add interval training on bike days/walking 2/10 RPE alternate days
  • Complete all workouts on bike (steady/intervals)
  • Add upright minutes in unstructured way: walk to mailbox then to next house down; strength training upright while cardio seated; 5 minutes walking on TM & finish workouts on bike
46
Q

What is the maintenance for POTS patients

A
  • Different for every body
  • Goal is not as high HR as possible
  • Building capacity in appropriate HR zones: 65-80% age expected HR max
  • Long term goal of at least 150 minutes per week when applicable
47
Q

How to manage regressions

A
  • Motivational interviewing
  • compassion
  • Fear avoidance & impact of disability
48
Q

Can you do orthostatic testing on beta blockers

A
  • NO
  • MD clearance for 2-4 day trial without to allow better testing
49
Q

For Post exercise symptom exacerbation (PESE) what things will decrease at peak exertion & at ventilatory anaerobic threshold

A
  • Volume of oxygen consumed
  • Workload
  • Heart rate (HR)
  • Volume of air cleared per minute
50
Q

Common autonomic FMD triggers

A
  • Fear
  • Lowered HRV
  • Stressful environment
  • Exercise or exertion
  • Change in position
  • Change in BP or HR
51
Q

Objective measures for autonomic features of FMD

A
  • COMPASS 31
  • HRV
  • Pupilometry
  • Breathing pattern
  • Response to breath
  • Active stand test
52
Q

Treatment options for autonomic FMD pathophysiology

A
  • HRV biofeedback
  • Breath work
    -Weightings
  • Water & diet
  • Safety/grounding
  • Graded exercise training