AUTONOMIC NERVOUS SYSTEM
- a general visceral afferent motor system that
innervates the entire neuraxis and influences all
organ systems
-regulates blood pressure, heart rate, sleep, and
bladder and bowel function
-operates automatically
-rapid action
-its full importance becomes recognized
only when it is compromised, resulting in
dysautonomia
Relevant Anatomy
-activity is regulated by central neurons’
response to diverse afferent inputs
-connections between cerebral cortex and
brainstem autonamic neurons coordinate
autonomic outflow with mental functions
-Output is controlled by/activated by the
Hypothalamus
Spinal cord
Brainstem
-Major part of it is located outside the CNS, and
in proximity to the visceral structures it
innervates
-Somatic neuromuscular system: single motor
neuron bridges the gap between CNS and the
effector organ
-IN CONTRAST, the ANS: two efferent neurons
Preganglionic -arising from it nucleus in the
brainstem or spinal cord
(intermediolateral horn)
Postganglionic -arising from specialized
nerve cells in peripheral ganglia
-ANS from an anatomic point of view, is divided
into 2 parts:
Craniosacral or parasympathetic
Thoracolumbar or sympathetic
Parasympathetic
-parasympathetic ganglia are distributed in
proximety to the structures they innervate
-Acetylcholine -main neurotransmitter
-2 divisions: cranial and sacral
-Lateral-posterior hypothalamus is part of the
supranuclear mechanism for the regulation of
parasympathetic activities
Sympathetic
-Ganglion system is located in a continuous and
interconnected longitudinal chain
paravertebrally - Sympathetic chain
-main neurotransmitter of th postganglionic
connection to the organ is NorEpinephrine
EXCEPTION: sweat glands (Sudomotor),
which are cholinergic
-Functionally, the two divisions are
complementary in maintaining a balance in the
tonic activities of many visceral structures and
organs
-The rigid separation into sympathetic and
parasympathetic parts is physiologically NOT
absolute
-the two divisions are often affected together
Normal ANS Activation
-Responses to stimulation are antagonistic
-Highly coordinated interactions

Clinical Evaluation

-disorders of the ANS may result from pathology

of either the CNS or the PNS

-Signs and symptoms may result from the

interruption of the afferent limb, CNS
processing centers, or efferent limb of reflex
arcs controlling autonomic responses

Symptoms of Autonomic Dysfunction

Clinical manifestations can result from loss of

function, overactivity, or dysregulation of
autonomic circuits

-unexplained orthostatic hypertension

-syncope

-sleep dysfunction

-altered sweating

-impotence

-constipation or other GI problems

-bladder disorders
HISTORY TAKING
- Self-report questionnaire
- Focuses on systemic functions (BP, HR, sleep,
fever, sweating) and involvement of individual
organ systems (pupils, bowel, bladder, sexual
function)
- Autonomic symptoms may vary dramatically

- Orthostatic hypotension - most disabling

feature
* Aging, diabetes mellitus
* Dimming or loss of vision, lightheadedness
* Diaphoresis, diminished hearing, pallor,
and weakness
* Syncope

- Other manifestations of impaired baroreflexes:

* Supine hypertension
* Heart rate that is fixed regardless of
posture
* Postprandial hypotension
* Excessively high nocturnal BP

- Many patients with OH have a preceding

diagnosis of hypertension or have concomitant
supine hypertension
* Importance of baroreflexes in maintaining
postural and supine normotension
* OH in antihypertensive treatment may
indicate overtreatment or the onset of an
autonomic disorder

- The most common causes of OH are not

neurologic in origin; these must be
distinguished from the neurogenic causes. Approach to the Patient

> 1st step in the evaluation of symptomatic OH

is the exclusion of treatable causes

- Review of medications

- precipitation of OH by medications may

also be the 1st sign of an underlying autonomic
disorder

- Underlying cause of symptoms

- e.g. diabetes, Parkinson’s disease

 - specific underlying mechanisms (e.g.
cardiac pump failure, reduced intravascular
volume)
Physical Examination

> Measurement of supine and standing pulse

and BP

- OH – sustained drop in systolic (>20

mmHg) or diastolic (>10 mmHg) BP after
2-3min of standing

- In nonneurogenic causes of OH (such as

hypovolemia) – BP drop and compensatory
increase in heart

rate of >15 beats/min

> Autonomic stressors: meal, hot bath, exercise

Neurologic Examination

> Mental status

- neurodegenerative disorders

> Cranial nerves

- impaired downgaze with progressive

supranuclear palsy

- abnormal pupils with Horner’s or Adie’s

syndrome

> Motor tone

- Parkinson’s disease and parkinsonism

> Sensation

- Polyneuropathies
AUTONOMIC TESTING
- Document abnormalities when findings
on hx and PE are inconclusive; to
detect subclinical involvement
- Heart rate variation with deep
breathing
o Parasympathetic function
- Valsalva response
o Parasympathetic (beat-to-beat
HR variability) and
sympathetic (BP changes)
- Sudomotor Function
o QSART- sympathetic
postganglionic fibers
o Thermoregulatory Sweat
Testing- sweat production in
response to controlled
elevation of body temperature
- Orthotatic BP recordings
o Beat-to-beat BP in supine, tilt,
tilt-back
o Quantitates orthostatic failure of
BP control
- Tilt Table Testing for Syncope
o For diagnosing vasovagal
syncope
MULTIPLE SYSTEMS ATROPHY
-Combination of parkinsonian, cerebellar and
autonomic features
- Predominant parkinsonian (MSA-P)
- Cerebellar (MSA-C)
- Shy-drager syndrome (MSA-A)
-Atypical parkinsonism in conjunction with
cerebellar signs and/or early and prominent
autonumic dysfunction
-Degeneration of Snc, striatum, cerebellum, and
inferior olivary glial cytoplasmic inclusions (GCIs)
that stain for alpha synuclein
-Iron accumulation in the striatum on T2
weighted scans, high signal change in the
region of external surface of the putamen
( putaminal rim) in MSA- P
-Cerebellar and brain stem atrophy ( the
pontine “hot cross buns” sign )
2-5 per 100,000 individuals
Onset is typically on mid fifties
-Men > women
-Sporadic
-Should be considered in adults > 30 years old
who present with OH or urinary incontinence
and either parkinsonism that is poorly
responsive to dopamine replacement or a
cerebellar syndrome
-Mortality: 7-10 years after onset
Spinal cord lesions
-Focal autonimic deficits or autonomi
hyperreflexia affecting boweo, bladder, sexual,
temperature regulation, or cardiovascular
functions E.g. spinal cord transection or
hemisection
-Autonomic dysreflexia Describes a dramatic
increase in BP in patients with traumatic spinal
cord lesions above the T6 level, often in
response to stimulation of the bladder, skin or
muscle
PERIPHERAL NERVE DISORDERS
- most common cause of chronic
autonomic insufficiency
- Diabetes Mellitus
 o Increased mortality rate
independent of other factors
- Amyloidosis
o Distal painful neuropathy;
search for amyloid deposits
- Alcoholic Neuropathy
o Can occur with brainstem
involvement, Wernicke’s
- Porphyria
- Guillain-Barre Syndrome
o Demyelination in vagus,
sympathetic chain; less
favorable diagnosis
Other Disorders
Inherited disorders: HSAN I to V

Primary Hyperhidrosis

Infections, malignancy, organophosphate

poisoning, aging

Autonomic Storm:
-Acute sympathetic overactivity
-Alterations in vital signs
-Brain and spinal cord injury, large strokes,
autonomic neuropathy, toxins, drugs,
Pheochormocytoma
-Neuroleptic malignant syndrome
Treatment of Autonomic failure
-aimed at specific treatment of cause and
alleviation of symptoms
E.g. removal of offending agents
-patient education
-symptomatic treatement

Symptomatic treatment

Pharmacologic:

-Midrodine - direct acting a1 agonist that does

does NOT cross BBB

-Droxidopa - converted to NE
-Fludrocortisone -exacerbates supine HPN

Investigational:

-Atomoxetine + Yohimbine