CHAPTER
Evaluation of the Autonomic
Nervous System
MICHAEL J. AMINOFF
ANATOMY
Afferent Pathways and Central Structures
Sympathetic Efferent Pathways
Parasympathetic Efferent Pathways
CLINICAL ASPECTS OF DYSAUTONOMIA
TESTS OF AUTONOMIC FUNCTION
Cardiovascular Tests
Heart Rate Variation
Response to the Valsalva Maneuver
Blood Pressure Variation
Cutaneous Vasomotor Control
Tests of Baroreflex Sensitivity at Rest
Myocardial Scintigraphy
Sweat Tests
Autonomic disturbances are a characteristic feature of
certain neurologic disorders; may be a cause of death in
others; and sometimes complicate general medical disor-
ders, such as diabetes mellitus. For most clinical purposes,
autonomic function is evaluated by a number of simple
noninvasive tests. These tests have an important role in
several clinical contexts. First, they help to confirm a clin-
ical diagnosis of dysautonomia and to exclude other causes
of symptoms. Second, they provide an indication of the
extent and severity of autonomic involvement and indicate
whether the sympathetic and parasympathetic divisions
are affected equally or whether one is involved selectively.
Third, they may permit the site of the lesion to be localized
more precisely, although this sometimes requires more
sophisticated or invasive studies. Finally, autonomic func-
tion tests may be helpful in the evaluation of small-fiber
neuropathies. In this chapter, attention is directed primar-
ily at investigations that can be undertaken conveniently in
a clinical neurophysiology laboratory. The enteric compo-
nent of the autonomic nervous system is also important,
but it will not be discussed here because it is usually not
the focus of clinical neurophysiologists.
21
Thermoregulatory Sweat Test
Sympathetic Skin Response and Related
Responses
Sudomotor Axon Reflex Testing
Plasma Catecholamine Levels and Infusions
Tests of Pupillary Function
Intraneural Recordings
Other Investigative Techniques
SELECTION OF TESTS
TEST FINDINGS IN SPECIFIC DISORDERS
Central Nervous System
Peripheral Nervous System
Acute Peripheral Dysautonomias
Chronic Autonomic Neuropathies
ANATOMY
Details of the anatomy of the autonomic nervous system
are provided in standard textbooks; only a short sum-
mary of certain aspects of clinical relevance is provided
here to facilitate understanding of clinical test procedures
and their interpretation.
Afferent Pathways and
Central Structures
Autonomic afferent fibers pass along autonomic or
somatic peripheral nerves to the central nervous system
(CNS), but their precise pathways have not been well
defined. Fibers from the retina pass along the optic nerve
and tract to the pretectal nucleus and then to pupillocon-
strictor neurons in the Edinger–Westphal nucleus. The
trigeminal nerve carries afferent fibers from the cornea
and the nasal and oropharyngeal mucosa to the trigem-
inal nuclei and the nucleus tractus solitarius; their activa-
tion causes lacrimation and nasal and oral secretions.
The glossopharyngeal and vagus nerves carry afferent
455