Dysautonomia Flashcards
What is the autonomic nervous system broken down into
- Sympathetic (thoracolumbar)
- Parasympathetic (craniosacral)
What are the functions of the autonomic nervous system
- 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)
The anatomical distinction between the sympathetic and parasympathetic divisions of the ANS is based primarily on
- 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
What neurotransmitter does each division of the ANS liberate
- Sympathetic: norepinephrine
- Parasympathetic: acetylcholine
Define dysautonomia
- 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
Autonomic dysfunction is common in what conditions
- Neurologic conditions
Symptoms of dysautonomia
- Sweating abnormalities
- Dizziness and fainting
- Vision problems
- Exercise intolerance
- Nausea
- Digestive difficulties
- Urinary issues
- Sexual problems
What diagnosis’s fall under the umbrella of dysautonomia
- 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
Ddx between POTS and OI
- OI: no significant BP drop and no significant HR elevation
- POTS: no significant BP drop and sustained elevated HR >30 BPM or >120 bpm
Ddx between neurogenic OH and cariogenic OH
- Neurogenic: orthostatic drop in BP and delta HR/delta SBP ratio <0.5
- Cardiogenic: orthostatic drop in BP and appropriate HR response
Describe a normal baroreceptor response
- 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
Primary causes of neurogenic OH
- Neurocardiogenic syncope
- Postural orthostatic tachycardia syndrome (POTS)
- Multiple system atrophy
- Familial dysautonomia
- Pure autonomic failure
- Parkinson’s disease
- Lewy body dementia
Steps in the process of OH intervention
- 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)
Education for OH prevention with nonpharmacologic measures
- 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
Cardiovascular response during exercise
- 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
Describe paroxysmal sympathetic hyperactivity (PSH)
- 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)
What are the 2 hypotheses as to why PSH happens
- 1) Epileptogenic cause: not well supported
- 2) Cerebral brain stem disconnection syndromes
Clinical features of PSH
- 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
How to diagnose PSH
- 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
What is the scoring ranges for PSH
- Mil: 0
- Mild: 1-6
- Moderate: 7-13
- Severe: ≥13
What to do for autonomic dysreflexia
- 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
Describe autonomic dysreflexia
- 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
What are the systolic values for those with autonomic dysreflexia
- > 150 mmHg in adults
- > 140 mmHg in adolescents
- > 130 mmHg in children 6-12 yrs
- > 120 mmHg in children ≤5 yrs
Symptoms associated with POTS
- Lightheadedness
- Palpitation (heart racing)
- Tremulousness
- Atypical chest discomfort
Symptoms associated with POTS that are not necessarily associated with particular postures
- 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)
POTS hemodynamic criteria
- 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
Symptoms of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS)
- 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
Diagnosis of ME/CFS
- 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
Causes of ME/CFS
- Cause of disease is unknown but onset may follow an infectious like syndrome
Describe myalgic encephalomyelitis (ME)/Chronic fatigue syndrome (CFS)
- 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
What is mast cell activation syndrome (MCAS)
- 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
What is long COVID
- 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
No-pharmacologic management of dysautonomia
- 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
Pharmacologic management of dysautonomia
- Fludrocortisone
- Propranolol
- Midodrine
- Pyridostigmine
What outcome measure can be used to screen for dysautomia
- 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
Examination for dysautonomia
- Exertional testing: upright tolerance testing, Buffalo concussion treadmill test, & 5 times sit to stand
- NASA lean test
- Active stand test
Importance of HR variability
- 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
HRV metrics and why measurement duration is important
- 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
Criteria for transitioning within stages for CHOP protocol for POTS
- 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
Criteria for transitioning between stages for CHOP protocol for POTS
- 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
Describe the preconditioning stage of CHOP protocol for POTS
- 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
Describe stages 1 & 2 in the CHOP protocol for POTS
- 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
Describe stages 3 & 4 in the CHOP protocol for POTS
- 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)
Describe stage 5 of the CHOP protocol for POTS
- 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
What are some interval modifications for POTS patients working through the CHOP protocol
- 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
What is the maintenance for POTS patients
- 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
How to manage regressions
- Motivational interviewing
- compassion
- Fear avoidance & impact of disability
Can you do orthostatic testing on beta blockers
- NO
- MD clearance for 2-4 day trial without to allow better testing
For Post exercise symptom exacerbation (PESE) what things will decrease at peak exertion & at ventilatory anaerobic threshold
- Volume of oxygen consumed
- Workload
- Heart rate (HR)
- Volume of air cleared per minute
Common autonomic FMD triggers
- Fear
- Lowered HRV
- Stressful environment
- Exercise or exertion
- Change in position
- Change in BP or HR
Objective measures for autonomic features of FMD
- COMPASS 31
- HRV
- Pupilometry
- Breathing pattern
- Response to breath
- Active stand test
Treatment options for autonomic FMD pathophysiology
- HRV biofeedback
- Breath work
-Weightings - Water & diet
- Safety/grounding
- Graded exercise training