Neurogenic Orthostatic Hypotension¶

VERSION: 1.1 CREATED: February 8, 2026 REVISED: February 8, 2026 STATUS: Draft

DIAGNOSIS: Neurogenic Orthostatic Hypotension

ICD-10: I95.1 (Orthostatic hypotension), G90.09 (Other idiopathic peripheral autonomic neuropathy)

CPT CODES: 85025 (CBC), 80048 (BMP), 84443 (TSH), 82607 (Vitamin B12), 82382 (Catecholamines, fractionated), 82384 (Catecholamines, 24-hour urine), 95924 (Tilt table test / autonomic testing), 95923 (QSART / sudomotor testing), 95922 (Autonomic function test — adrenergic), 95921 (Autonomic function test — cardiovagal), 93000 (12-lead ECG), 93306 (Echocardiogram), 70553 (MRI brain with and without contrast), 88305 (Skin biopsy pathology), 86235 (ANA), 83516 (Ganglionic AChR Ab), 83036 (HbA1c), 82533 (Cortisol), 78451 (Cardiac MIBG scintigraphy), 95907-95913 (Nerve conduction studies)

SYNONYMS: nOH, neurogenic orthostatic hypotension, orthostatic hypotension, postural hypotension, autonomic failure, orthostatic syncope, autonomic neuropathy with OH, sympathetic vasoconstrictor failure, neurogenic OH, autonomic orthostatic hypotension, orthostatic hypotension due to autonomic failure, adrenergic failure, autonomic insufficiency with orthostatic hypotension

SCOPE: Evaluation and management of neurogenic orthostatic hypotension (nOH) in adults. Includes differentiation from non-neurogenic OH, etiologic workup (alpha-synucleinopathies, diabetic autonomic neuropathy, amyloid neuropathy, autoimmune autonomic ganglionopathy, pure autonomic failure), autonomic testing, pharmacologic and non-pharmacologic management, supine hypertension management, and falls prevention. Covers ED, hospital, and outpatient settings. Excludes non-neurogenic causes of OH (dehydration, hemorrhage, medication-induced) unless as differential.

DEFINITIONS: - Orthostatic hypotension (OH): Sustained reduction in SBP ≥20 mmHg or DBP ≥10 mmHg within 3 minutes of standing or head-up tilt ≥60 degrees - Neurogenic OH (nOH): OH caused by failure of the autonomic nervous system to adequately increase sympathetic output on standing; characterized by attenuated heart rate response (HR increase <15 bpm despite significant BP drop) - Non-neurogenic OH: OH due to volume depletion, medications, cardiac pump failure, or other non-autonomic causes; typically with preserved or exaggerated heart rate response - Supine hypertension: SBP ≥140 mmHg and/or DBP ≥90 mmHg in the supine position; present in up to 50% of nOH patients and complicates management - Alpha-synucleinopathy: Group of neurodegenerative diseases (Parkinson disease, multiple system atrophy, Lewy body dementia, pure autonomic failure) characterized by alpha-synuclein aggregation and frequent autonomic dysfunction - Pure autonomic failure (PAF): Isolated autonomic failure without other neurological deficits; may phenoconvert to MSA, PD, or DLB - Autoimmune autonomic ganglionopathy (AAG): Antibody-mediated autonomic failure due to ganglionic acetylcholine receptor antibodies; potentially treatable

PRIORITY KEY: STAT = Immediate | URGENT = Within hours | ROUTINE = Standard | EXT = Extended/atypical cases | - = Not applicable to this setting

1. LABORATORY WORKUP¶

1A. Core Labs (All Patients)¶

Test	ED	HOSP	OPD	ICU	Rationale	Target Finding
CBC (CPT 85025)	ROUTINE	ROUTINE	ROUTINE	ROUTINE	Anemia contributing to orthostatic symptoms	Normal hemoglobin
BMP (CPT 80048) (electrolytes, creatinine, glucose)	ROUTINE	ROUTINE	ROUTINE	ROUTINE	Dehydration, renal insufficiency, electrolyte abnormalities	Normal
HbA1c (CPT 83036)	-	ROUTINE	ROUTINE	-	Diabetic autonomic neuropathy screening	<5.7% (normal); ≥6.5% suggests diabetes
TSH (CPT 84443)	-	ROUTINE	ROUTINE	-	Thyroid dysfunction contributing to autonomic symptoms	Normal
Vitamin B12 (CPT 82607)	-	ROUTINE	ROUTINE	-	B12 deficiency neuropathy with autonomic component	>300 pg/mL
AM cortisol (CPT 82533)	-	ROUTINE	ROUTINE	-	Adrenal insufficiency as cause of orthostatic hypotension	>10 mcg/dL (AM)
Orthostatic vital signs (lying → standing at 1 and 3 min)	STAT	STAT	ROUTINE	STAT	Confirm OH; assess HR response to differentiate nOH vs non-neurogenic	SBP drop <20, DBP drop <10; HR rise ≥15 bpm
12-lead ECG (CPT 93000)	STAT	STAT	ROUTINE	STAT	Exclude cardiac arrhythmia or structural cause	Normal sinus rhythm

1B. Extended Labs (Based on Clinical Suspicion)¶

Test	ED	HOSP	OPD	ICU	Rationale	Target Finding
Supine and standing plasma norepinephrine	-	ROUTINE	ROUTINE	-	Differentiates preganglionic vs postganglionic autonomic failure; PAF shows low supine NE without standing rise	Supine NE >200 pg/mL with ≥2× rise on standing
Serum protein electrophoresis (SPEP) with immunofixation	-	ROUTINE	ROUTINE	-	Screen for AL amyloidosis	No monoclonal protein
Serum free light chains	-	ROUTINE	ROUTINE	-	Amyloidosis evaluation (AL type)	Normal kappa/lambda ratio
Anti-ganglionic AChR antibodies (anti-gAChR Ab)	-	EXT	ROUTINE	-	Autoimmune autonomic ganglionopathy (AAG); treatable cause	Negative
Paraneoplastic antibody panel (anti-Hu, CRMP-5, others)	-	EXT	EXT	-	Paraneoplastic autonomic neuropathy	Negative
24-hour urine catecholamines and metanephrines	-	-	EXT	-	Exclude pheochromocytoma as rare cause	Normal

1C. Rare/Specialized Labs¶

Test	ED	HOSP	OPD	ICU	Rationale	Target Finding
Transthyretin (TTR) gene sequencing	-	-	EXT	-	Hereditary ATTR amyloidosis (hATTR)	No pathogenic variant
Fat pad or tissue biopsy with Congo red staining	-	EXT	EXT	-	Tissue confirmation of amyloidosis	No amyloid deposits
Skin punch biopsy (epidermal nerve fiber density)	-	-	ROUTINE	-	Small fiber neuropathy with autonomic involvement	Normal ENFD (age/site adjusted)
CSF analysis	-	EXT	EXT	-	If autoimmune or inflammatory etiology suspected	Normal

2. DIAGNOSTIC IMAGING & STUDIES¶

2A. Essential Autonomic Testing¶

Study	ED	HOSP	OPD	ICU	Timing	Target Finding	Contraindications
Orthostatic vital signs (bedside)	STAT	STAT	ROUTINE	STAT	Immediate; ALL patients	No sustained SBP drop ≥20 or DBP drop ≥10 within 3 min	None
Head-up tilt table test (CPT 95924)	-	ROUTINE	ROUTINE	-	Outpatient or inpatient autonomic lab	Quantify BP and HR response to passive tilt; confirm nOH	Severe aortic stenosis, critical carotid stenosis
Valsalva maneuver with beat-to-beat BP monitoring (CPT 95922)	-	ROUTINE	ROUTINE	-	Autonomic lab	Normal 4-phase response; absent phase IV overshoot in nOH	Recent eye surgery, aortic aneurysm
Cardiovagal testing — deep breathing, Valsalva ratio (CPT 95921)	-	ROUTINE	ROUTINE	-	Autonomic lab	Normal HR variability with deep breathing	Atrial fibrillation (may limit interpretation)

2B. Extended Autonomic and Diagnostic Studies¶

Study	ED	HOSP	OPD	ICU	Timing	Target Finding	Contraindications
QSART — quantitative sudomotor axon reflex test (CPT 95923)	-	-	ROUTINE	-	Autonomic lab	Normal sweat volumes at standard sites	Skin lesions at test sites
Thermoregulatory sweat test (TST)	-	-	EXT	-	Specialized center	Normal sweat distribution; identifies pattern of postganglionic vs preganglionic sudomotor failure	Acute illness, cardiovascular instability
Cardiac MIBG scintigraphy (CPT 78451)	-	-	EXT	-	Nuclear medicine	Normal myocardial uptake; decreased uptake in postganglionic lesions (PD, PAF, DLB) vs preserved in MSA	Medications affecting NE uptake (TCAs, labetalol)
Nerve conduction studies / EMG (CPT 95907-95913)	-	-	ROUTINE	-	EMG lab	Evaluate for peripheral neuropathy causing autonomic dysfunction	None
MRI brain (CPT 70553)	-	ROUTINE	ROUTINE	-	Radiology	Evaluate for MSA (hot cross bun sign, cerebellar/pontine atrophy), PD, DLB	Pacemaker, metal implants

2C. Rare/Specialized Studies¶

Study	ED	HOSP	OPD	ICU	Timing	Target Finding	Contraindications
DaTscan (Ioflupane I-123)	-	-	EXT	-	Nuclear medicine	Differentiates PD/DLB (abnormal) from MSA (often abnormal) or non-synucleinopathy	None significant
24-hour ambulatory blood pressure monitoring (ABPM)	-	-	ROUTINE	-	Outpatient	Characterize circadian BP pattern; identify nocturnal hypertension, post-prandial hypotension	None
Cardiac echo	-	ROUTINE	EXT	ROUTINE	If cardiac cause suspected	Normal structure and function	None
Echocardiogram with strain imaging	-	EXT	EXT	-	If cardiac amyloidosis suspected	Normal; or identify restrictive cardiomyopathy pattern	None

3. TREATMENT¶

3A. Acute/Emergent Management¶

Treatment	Route	Indication	Dosing	Contraindications	Monitoring	ED	HOSP	OPD	ICU
IV normal saline	IV	Acute symptomatic nOH with hemodynamic compromise	500-1000 mL :: IV :: once :: NS 500-1000 mL bolus; repeat if needed based on response	Heart failure, volume overload	Volume status, BP, HR	STAT	STAT	-	STAT
Medication review and discontinuation of offending agents	-	All patients with new or worsening OH	N/A :: - :: per protocol :: Review and reduce/discontinue diuretics, alpha-blockers, vasodilators, tricyclics, dopamine agonists	Clinical judgment	BP, symptoms	STAT	STAT	ROUTINE	STAT
Supine positioning with legs elevated	-	Acute symptomatic hypotension	N/A :: - :: continuous :: Trendelenburg or leg elevation to improve venous return	None	BP	STAT	STAT	-	STAT
Bolus water drinking	PO	Acute symptomatic OH; pressor effect within 5-15 min	480 mL (16 oz) :: PO :: once :: Rapid ingestion of 480 mL cold water; raises SBP 20-30 mmHg within 5-15 min via osmopressor reflex	Fluid restriction, NPO status	BP	STAT	STAT	ROUTINE	STAT

3B. Non-Pharmacologic Therapies¶

Treatment	Route	Indication	Dosing	Contraindications	Monitoring	ED	HOSP	OPD	ICU
Increased dietary salt intake	PO	Chronic nOH management; volume expansion	6-10 g/day :: PO :: daily :: 6-10 g sodium per day via dietary supplementation or salt tablets (1 g tabs, 2-3 TID with meals)	Heart failure, severe supine HTN, renal failure	BP, edema, renal function	EXT	ROUTINE	ROUTINE	-
Increased fluid intake	PO	Chronic nOH; volume expansion	2-3 L/day :: PO :: daily :: Target 2-3 L fluid per day; distribute throughout the day	Heart failure, fluid restriction	Volume status	EXT	ROUTINE	ROUTINE	-
Compression garments — abdominal binder	-	Chronic nOH; venous pooling reduction	N/A :: - :: continuous :: Abdominal binder (20-40 mmHg); more effective than leg stockings alone	Abdominal wound, skin breakdown	Compliance, comfort	-	ROUTINE	ROUTINE	-
Compression garments — waist-high stockings	-	Chronic nOH; venous pooling reduction	N/A :: - :: continuous :: Waist-high compression stockings 30-40 mmHg; thigh-high less effective	Peripheral vascular disease, skin ulcers	Compliance	-	ROUTINE	ROUTINE	-
Head-of-bed elevation	-	Supine hypertension mitigation; reduce nocturnal diuresis	N/A :: - :: nightly :: Elevate head of bed 10-20 degrees (6-9 inch blocks under head posts); reduces nocturnal natriuresis and expands daytime plasma volume	None	Supine BP, symptoms	-	ROUTINE	ROUTINE	ROUTINE
Counter-pressure maneuvers	-	Acute or chronic nOH; during prodromal symptoms	N/A :: - :: per episode :: Leg crossing with muscle tensing, squatting, bending forward; raises BP 20-30 mmHg acutely	Severe arthritis limiting maneuvers	Symptom relief	ROUTINE	ROUTINE	ROUTINE	ROUTINE
Small frequent meals; reduce carbohydrate load	PO	Post-prandial hypotension component	N/A :: PO :: daily :: 5-6 small meals per day; limit carbohydrate intake per meal; avoid alcohol	None	Post-prandial BP	-	ROUTINE	ROUTINE	-

3C. First-Line Pharmacotherapy¶

Treatment	Route	Indication	Dosing	Contraindications	Monitoring	ED	HOSP	OPD	ICU
Midodrine	PO	nOH with persistent symptoms despite non-pharmacologic measures	2.5 mg TID; 5 mg TID; 10 mg TID :: PO :: TID :: Start 2.5 mg TID; titrate by 2.5 mg q1-2 weeks; max 10 mg TID; last dose no later than 4 PM to avoid supine HTN; onset 30-45 min, duration 3-4 hr	Supine hypertension (SBP >180), urinary retention, pheochromocytoma, severe organic heart disease, thyrotoxicosis	Supine BP (especially at night), urinary retention, scalp tingling, piloerection	EXT	ROUTINE	ROUTINE	EXT
Droxidopa (Northera)	PO	Symptomatic nOH specifically due to autonomic failure (PD, MSA, PAF, dopamine beta-hydroxylase deficiency)	100 mg TID; 200 mg TID; 300 mg TID; 600 mg TID :: PO :: TID :: Start 100 mg TID; titrate by 100 mg TID q24-48 hr; max 600 mg TID; FDA-approved for symptomatic nOH	Supine hypertension	Supine BP (measure before next dose and at bedtime); must re-evaluate efficacy after 2 weeks per FDA requirement	EXT	ROUTINE	ROUTINE	EXT
Fludrocortisone	PO	nOH with volume depletion component; plasma volume expansion	0.05 mg daily; 0.1 mg daily; 0.2 mg daily :: PO :: daily :: Start 0.05-0.1 mg daily; titrate q1-2 weeks; max 0.2 mg daily; monitor for hypokalemia and supine HTN; off-label for nOH	Heart failure, severe supine HTN, hypokalemia	K+, Mg2+, supine BP, weight, edema	EXT	ROUTINE	ROUTINE	EXT

3D. Second-Line/Refractory Therapies¶

Treatment	Route	Indication	Dosing	Contraindications	Monitoring	ED	HOSP	OPD	ICU
Pyridostigmine	PO	Mild-moderate nOH; augments ganglionic neurotransmission; less supine HTN risk	30 mg TID; 60 mg TID :: PO :: TID :: Start 30 mg TID; may increase to 60 mg TID; best for mild OH; may combine with midodrine 5 mg for added benefit	Asthma, mechanical GI/urinary obstruction, bradycardia	Cholinergic side effects (GI cramping, diarrhea), HR	-	ROUTINE	ROUTINE	-
Atomoxetine	PO	nOH refractory to first-line; norepinephrine reuptake inhibitor; benefits central autonomic lesions (MSA)	10 mg daily; 18 mg daily :: PO :: daily-BID :: Start 10-18 mg daily or BID; enhances residual sympathetic tone; evidence from small trials	Narrow-angle glaucoma, pheochromocytoma, concurrent MAOIs	HR, BP (standing and supine), mood	-	EXT	EXT	-
Yohimbine	PO	Refractory nOH; alpha-2 adrenergic antagonist; enhances norepinephrine release	2 mg TID; 5.4 mg TID :: PO :: TID :: Start 2 mg TID; increase to 5.4 mg TID; limited evidence	Renal/hepatic disease, concurrent antidepressants	BP, anxiety, palpitations	-	-	EXT	-
Erythropoietin (EPO)	SC	nOH with concurrent anemia (Hgb <11); expands red cell mass and blood volume	25 units/kg; 50 units/kg :: SC :: 3x/week :: 25-50 units/kg SC 3x/week; target Hgb 11-12 g/dL; slow response over weeks	Uncontrolled HTN, thrombotic risk, pure red cell aplasia history	Hgb, Hct, iron studies, BP, thrombotic events	-	EXT	EXT	-
Octreotide	SC	Refractory post-prandial hypotension in nOH	12.5 mcg; 25 mcg; 50 mcg :: SC :: pre-meal :: 12.5-50 mcg SC 30 min before meals; reduces splanchnic blood pooling	Cholelithiasis	Blood glucose, GI symptoms, gallbladder US q6-12 months	-	EXT	EXT	-
Desmopressin (DDAVP)	PO/IN	Nocturnal polyuria contributing to morning OH; reduces nighttime urine output	0.1 mg; 0.2 mg :: PO :: nightly :: 0.1-0.2 mg PO at bedtime (or 10-20 mcg intranasal); must hold fluids after dosing to avoid hyponatremia	Hyponatremia, HF, polydipsia	Serum Na+ (check within 1 week of initiation and regularly), weight	-	EXT	EXT	-
Acarbose	PO	Post-prandial hypotension refractory to meal modifications	50 mg; 100 mg :: PO :: pre-meal :: 50-100 mg with first bite of carbohydrate-containing meals; reduces post-prandial glucose spike and splanchnic vasodilation	Inflammatory bowel disease, bowel obstruction, cirrhosis	GI symptoms (flatulence, diarrhea), liver function	-	-	EXT	-

3E. Supine Hypertension Management¶

Treatment	Route	Indication	Dosing	Contraindications	Monitoring	ED	HOSP	OPD	ICU
Nitroglycerin transdermal patch	Transdermal	Supine HTN (SBP ≥150 mmHg at bedtime); removed upon waking	0.1 mg/hr; 0.2 mg/hr :: Transdermal :: nightly :: Apply 0.1-0.2 mg/hr patch at bedtime; remove in the morning before rising; short-acting to minimize morning orthostatic worsening	Severe aortic stenosis, concurrent PDE-5 inhibitors, hypovolemia	Supine BP at bedtime and morning standing BP	-	ROUTINE	ROUTINE	ROUTINE
Losartan	PO	Supine HTN at bedtime; ARB with moderate duration	25 mg; 50 mg :: PO :: nightly :: 25-50 mg at bedtime only; assess morning standing BP to ensure no worsening of OH	Hyperkalemia, bilateral renal artery stenosis, pregnancy	K+, creatinine, morning standing BP	-	ROUTINE	ROUTINE	ROUTINE
Hydralazine	PO	Supine HTN at bedtime; direct vasodilator	10 mg; 25 mg :: PO :: nightly :: 10-25 mg at bedtime; short-acting; useful for isolated nocturnal HTN	Lupus, tachycardia, coronary artery disease	HR, BP, lupus-like symptoms with chronic use	-	EXT	EXT	EXT
Sildenafil	PO	Supine HTN at bedtime; vasodilatory effect	25 mg :: PO :: nightly :: 25 mg at bedtime; reduces supine BP without excessive morning orthostatic worsening; off-label use	Concurrent nitrates, severe hepatic impairment	BP, headache, visual symptoms	-	-	EXT	-

4. OTHER RECOMMENDATIONS¶

4A. Referrals & Consults¶

Recommendation	ED	HOSP	OPD	ICU	Clinical Rationale
Autonomic disorders specialist / dysautonomia clinic	-	ROUTINE	ROUTINE	-	Formal autonomic testing; tailored management; etiologic diagnosis; monitoring for phenoconversion in PAF
Neurology	URGENT	ROUTINE	ROUTINE	URGENT	Evaluation for underlying neurodegenerative cause (PD, MSA, DLB); neuropathy workup
Cardiology	URGENT	ROUTINE	EXT	ROUTINE	Exclude cardiac causes of OH; evaluate cardiac amyloidosis if suspected; assess supine HTN burden
Physical therapy / falls prevention	-	ROUTINE	ROUTINE	-	Gait training; counter-pressure maneuver education; fall risk assessment and mitigation
Endocrinology	-	-	EXT	-	If adrenal insufficiency, refractory diabetes, or endocrine cause suspected
Hematology/Oncology	-	EXT	EXT	-	If amyloidosis confirmed (AL type); paraneoplastic evaluation

4B. Patient/Family Instructions¶

Recommendation	ED	HOSP	OPD	ICU	Rationale
Rise slowly from lying to sitting to standing; sit on edge of bed 1-2 minutes before standing	ROUTINE	ROUTINE	ROUTINE	ROUTINE	Prevents rapid BP drop and reduces fall risk
Recognize warning symptoms (lightheadedness, visual graying, neck/shoulder pain "coat hanger" distribution) and sit or lie down immediately	ROUTINE	ROUTINE	ROUTINE	ROUTINE	Early symptom recognition prevents syncope and injury
Perform counter-pressure maneuvers (leg crossing, squatting, bending) when symptomatic	ROUTINE	ROUTINE	ROUTINE	ROUTINE	Raises BP 20-30 mmHg acutely; non-pharmacologic first-line
Avoid large carbohydrate-heavy meals; eat small frequent meals	ROUTINE	ROUTINE	ROUTINE	-	Reduces post-prandial hypotension
Avoid prolonged standing, hot environments, hot baths/showers, and alcohol	ROUTINE	ROUTINE	ROUTINE	-	All worsen venous pooling and orthostatic BP drop
Maintain adequate fluid intake (2-3 L/day) and salt intake (per physician guidance)	ROUTINE	ROUTINE	ROUTINE	-	Plasma volume expansion is foundation of nOH management
Drink 480 mL (16 oz) of water rapidly when symptomatic (osmopressor effect)	ROUTINE	ROUTINE	ROUTINE	-	Raises SBP 20-30 mmHg within 5-15 min
Report all falls, near-falls, and new symptoms to care team	ROUTINE	ROUTINE	ROUTINE	ROUTINE	Falls are primary source of morbidity in nOH; guides treatment titration

4C. Lifestyle & Prevention¶

Recommendation	ED	HOSP	OPD	ICU	Rationale
Avoid offending medications when possible (alpha-blockers, diuretics, long-acting antihypertensives, tricyclics, dopamine agonists at high doses)	ROUTINE	ROUTINE	ROUTINE	ROUTINE	Medication-related orthostatic worsening is the most modifiable risk factor
Elevate head of bed 10-20 degrees nightly	-	ROUTINE	ROUTINE	ROUTINE	Reduces nocturnal supine hypertension and natriuresis; improves morning orthostatic tolerance
Home fall safety assessment (remove trip hazards, install grab bars, adequate lighting)	-	-	ROUTINE	-	nOH patients have 2-3x increased fall risk; home modifications are evidence-based prevention
Wear waist-high compression garments during the day; remove at bedtime	-	ROUTINE	ROUTINE	-	Reduces venous pooling; must remove at night to avoid worsening supine HTN
Avoid straining (Valsalva); manage constipation proactively	-	ROUTINE	ROUTINE	ROUTINE	Valsalva maneuvers can precipitate syncope in autonomic failure
Driving restrictions: counsel per local regulations until symptoms stabilized	-	ROUTINE	ROUTINE	-	Syncope or near-syncope while driving is a safety risk; individualized per severity

5. DIFFERENTIAL DIAGNOSIS¶

Alternative Diagnosis	Key Distinguishing Features	Tests to Differentiate
Non-neurogenic OH (hypovolemia, medication-induced)	Preserved HR response (≥15 bpm rise); identifiable cause (dehydration, hemorrhage, diuretics); resolves with volume or medication change	Orthostatic vitals with HR assessment; clinical history; medication review
Vasovagal syncope	Triggered by emotional stress, pain, prolonged standing; prodromal nausea/warmth; bradycardia during episode; typically younger patients	Tilt table test (vasodepressor or cardioinhibitory pattern); clinical history
Cardiac syncope (arrhythmia, structural)	Sudden onset without prodrome; exertional; palpitations; abnormal ECG; structural heart disease	ECG, telemetry, echo, Holter, EP study
Postural tachycardia syndrome (POTS)	HR rise ≥30 bpm (or HR ≥120) on standing without significant BP drop; predominantly young females; lightheadedness, palpitations	Tilt table test or active stand showing HR criteria without OH
Multiple system atrophy (MSA)	Cerebellar ataxia or parkinsonism + severe autonomic failure; poor levodopa response; early prominent OH; hot cross bun sign on MRI	MRI brain, autonomic testing, cardiac MIBG (preserved uptake)
Parkinson disease with autonomic failure	Motor symptoms preceding or accompanying OH; levodopa responsive; tremor predominant	Clinical exam, DaTscan, cardiac MIBG (decreased uptake)
Lewy body dementia	Cognitive fluctuations, visual hallucinations, parkinsonism, REM sleep behavior disorder + OH	Clinical criteria, DaTscan, cardiac MIBG
Diabetic autonomic neuropathy	Known diabetes with poor glycemic control; concurrent peripheral neuropathy; gradual onset	HbA1c, NCS/EMG, autonomic testing, QSART
Amyloid neuropathy (AL or ATTR)	Length-dependent neuropathy with autonomic features; cardiac involvement; weight loss; proteinuria	SPEP/UFLC, fat pad biopsy, TTR gene testing, cardiac imaging
Autoimmune autonomic ganglionopathy (AAG)	Subacute onset; diffuse autonomic failure (OH, GI dysmotility, sicca, pupil abnormalities); may follow infection	Anti-ganglionic AChR antibodies
Adrenal insufficiency	Fatigue, weight loss, hyperpigmentation, hyponatremia, hyperkalemia	AM cortisol, ACTH stimulation test
Deconditioning / prolonged bed rest	Occurs after immobilization; preserved autonomic reflexes; improves with reconditioning	Clinical history; autonomic testing normal

6. MONITORING PARAMETERS¶

Parameter	Frequency	Target/Threshold	Action if Abnormal	ED	HOSP	OPD	ICU
Orthostatic vital signs (supine, 1 min, 3 min standing)	Per encounter; BID if inpatient	SBP drop <20, DBP drop <10	Adjust medications; increase non-pharmacologic measures	STAT	q shift	Every visit	STAT
Supine blood pressure	Daily (inpatient); every visit (outpatient)	SBP <160 mmHg supine at bedtime	Add/adjust bedtime short-acting antihypertensive	-	Daily	Every visit	q4h
Serum potassium	At baseline, 1 week after fludrocortisone initiation, then q3 months	3.5-5.0 mEq/L	Supplement K+; reduce fludrocortisone dose	-	ROUTINE	q3 months	Daily
Serum sodium	At baseline, 1 week after DDAVP initiation, then monthly	135-145 mEq/L	Hold DDAVP if Na <135; restrict fluids after DDAVP	-	ROUTINE	Monthly if on DDAVP	Daily
Renal function (BMP)	Baseline, then q6-12 months	Normal creatinine and eGFR	Adjust medications; nephrology referral	-	ROUTINE	q6-12 months	ROUTINE
Falls/near-falls	Every encounter	Zero falls	Reassess treatment; PT referral; home safety evaluation	Every visit	Daily	Every visit	Every visit
Symptom burden (OH symptom scales — OHQ or similar)	Every visit	Improving or stable	Escalate pharmacotherapy; add agents	-	ROUTINE	Every visit	-
Weight	Weekly (inpatient); monthly (outpatient)	Stable; no excessive edema	Reassess fludrocortisone dose; diuretic if HF develops	-	Weekly	Monthly	Weekly

7. DISPOSITION CRITERIA¶

Disposition	Criteria
Discharge from ED	Mild OH responsive to fluids; no syncope or injury; known nOH on stable regimen with mild exacerbation; reliable outpatient follow-up arranged
Admit to telemetry/medicine	Syncope with injury; recurrent falls; new diagnosis of severe nOH requiring inpatient workup; supine SBP >200 mmHg; significant cardiac arrhythmia on workup
ICU admission	Hemodynamic instability refractory to initial measures; syncope with significant traumatic injury; concurrent cardiac emergency
Outpatient autonomic clinic referral	Confirmed or suspected nOH requiring formal autonomic testing; etiologic workup for alpha-synucleinopathy or amyloidosis; titration of nOH medications
Neurology follow-up	New diagnosis nOH; monitoring for phenoconversion (PAF to PD/MSA/DLB); co-management of neurodegenerative disease
Primary care follow-up	Stable nOH on medication; routine lab monitoring; medication reconciliation; BP management

8. EVIDENCE & REFERENCES¶

Recommendation	Evidence Level	Source
Consensus diagnostic criteria for OH (SBP drop ≥20 or DBP drop ≥10 within 3 min)	Consensus Statement	Freeman et al. Clin Auton Res 2011
Droxidopa for symptomatic nOH in autonomic failure (FDA-approved; short-term efficacy)	Class I, RCT (Phase III)	Kaufmann et al. Neurology 2014
Midodrine for orthostatic hypotension (FDA-approved; raises standing BP)	Class I, RCTs	Low et al. JAMA 1997
Comprehensive review of nOH pathophysiology and management in alpha-synucleinopathies	Expert Review	Gibbons et al. Neurology 2017
Management of supine hypertension in autonomic failure: a balancing act	Expert Consensus	Biaggioni. Pharmacol Rev 2017
AAN Practice Parameter: treatment of nOH and supine hypertension	Practice Guideline	Rizzo et al. Neurology 2014
Autoimmune autonomic ganglionopathy: clinical features and response to immunotherapy	Case Series	Vernino et al. N Engl J Med 2000
Non-pharmacologic management of OH (compression garments, counter-pressure maneuvers, fluid/salt)	Consensus/Review	Fanciulli & Wenning. N Engl J Med 2015
Cardiac MIBG differentiates Lewy body disorders from MSA	Diagnostic Study	Goldstein et al. Ann Intern Med 2000
Pyridostigmine for nOH: augments ganglionic transmission with minimal supine HTN	RCT	Singer et al. Arch Neurol 2006
Water drinking as pressor response in autonomic failure	Mechanistic/Clinical	Jordan et al. Circulation 2000
Consensus definition of neurogenic supine hypertension in autonomic failure	Consensus Statement	Biaggioni et al. Clin Auton Res 2018
Expert consensus on nOH screening, diagnosis, and treatment algorithm	Expert Consensus	Gibbons et al. J Neurol 2017

NOTES¶

nOH is distinguished from non-neurogenic OH primarily by the attenuated heart rate response (HR rise <15 bpm) despite significant BP drop; this reflects the inability of the autonomic nervous system to compensate
"Coat hanger" distribution pain (posterior neck and shoulders) is a characteristic symptom of nOH due to ischemia of postural neck muscles
Supine hypertension is present in approximately 50% of nOH patients and creates a therapeutic dilemma: treating OH may worsen supine HTN and vice versa
Head-of-bed elevation is a critical intervention that addresses both supine HTN and morning OH by reducing nocturnal natriuresis
Droxidopa (Northera) is the only FDA-approved medication specifically for symptomatic nOH in autonomic failure; its long-term efficacy beyond 2 weeks is debated
Patients with pure autonomic failure should be monitored for phenoconversion to MSA, PD, or DLB (occurs in approximately 34% over a median of 8 years)
Cardiac MIBG scintigraphy differentiates postganglionic lesions (PD, PAF, DLB — decreased uptake) from preganglionic lesions (MSA — preserved uptake)
Post-prandial hypotension is common in nOH and may be the most debilitating symptom; manage with small meals, reduced carbohydrates, acarbose, or octreotide
Anti-ganglionic AChR antibodies should be tested in all subacute-onset autonomic failure, as autoimmune autonomic ganglionopathy is a treatable cause
Falls are the primary cause of morbidity in nOH; fall prevention must be addressed at every visit

CHANGE LOG¶

v1.1 (February 8, 2026) - Added ICU column to Section 4B (Patient Instructions) and 4C (Lifestyle) for structural consistency across all venue-tagged tables - Expanded ED coverage in Section 4B: counter-pressure maneuvers, dietary guidance, environmental avoidance, fluid/salt intake, and water bolus now tagged ROUTINE for ED discharge teaching - Added EXT coverage in ED and ICU for Section 3C first-line pharmacotherapy (midodrine, droxidopa, fludrocortisone) for patients already on these medications presenting acutely - Added ICU coverage (ROUTINE) for Section 3E supine HTN management (nitroglycerin patch, losartan) and EXT for hydralazine; ICU patients with nOH commonly require supine HTN treatment - Added HOSP EXT coverage for select Section 3D second-line agents (atomoxetine, EPO, octreotide, DDAVP) for refractory inpatient nOH - Added HOSP ROUTINE coverage for Section 6 symptom burden monitoring (OHQ scale) - Added ICU STAT coverage for bolus water drinking in Section 3A (patients able to take PO); added NPO status as contraindication - Added ICU ROUTINE coverage for head-of-bed elevation in Section 3B and Section 4C - Added ICU ROUTINE coverage for counter-pressure maneuvers in Section 3B - Added URGENT ED coverage for cardiology referral in Section 4A (exclude cardiac etiology in acute presentations) - Added ICU coverage for medication avoidance and constipation management in Section 4C - Strengthened directive language in Section 3D atomoxetine row (removed "may benefit") - Added 13th evidence reference (Gibbons et al. 2017 nOH screening/treatment algorithm) - Updated version to 1.1

v1.0 (February 8, 2026) - Initial template creation - Comprehensive laboratory and autonomic testing workup - Treatment organized by acuity: acute, non-pharmacologic, first-line, second-line/refractory, supine HTN management - 15 individual medication rows with structured dosing - Etiologic differential including alpha-synucleinopathies, amyloidosis, and AAG - Supine hypertension management section with 4 short-acting agents - 12 evidence references with PubMed links