Clinical Autonomic Disorders: A Training Protocol

Objective:

This Protocol is to facilitate training of Fellows in evaluation of clinical disorders of the
autonomic nervous system. The training is necessary to enable Fellows to sit for and pass the
certifying examination in autonomic disorders administered by the United Council for
Neurologic Subspecialties (UCNS) and to maintain UCNS accreditation of the Fellowship. A
secondary objective is to include results of evaluations and testing with those from other
Protocols, for future data analyses.

Study population:

Referred patients with known or suspected abnormalities of one or more components of the
autonomic nervous system and healthy adult volunteers. Volunteers will be studied for Fellows
to practice and gain normative values for pupillometric function.

Design:

Clinical physiologic, neuropharmacologic, and neurochemical testing will be performed during
outpatient clinic visits or during inpatient admissions, depending on the individual testing
schedule. Inpatient testing may be done in conjunction with other Protocols (e.g.,
03-N-0004). Most of the testing under this Protocol can be completed in a single outpatient
visit over the course of a workday. Testing procedures under this Protocol will be performed
as needed for adequate Fellow training. In addition to autonomic medical history and physical
examination, clinical testing procedures may include (A) monitoring of hemodynamics,
sweating, and other physiologic parameters associated with the Valsalva maneuver,
orthostasis, or external temperature manipulation; (B) venous blood sampling for assays of
levels of catecholamines and related neurochemicals; (C) administration of autonomic test
drugs; (D) skin biopsies; (E) blood volume measurement; (F) pupillometry; (G)
gastrointestinal phonography; (H) urinary bladder ultrasound; and (I) neurobehavioral rating
scales.

Outcome measures:

The main outcome measure is competency in clinical and laboratory evaluation of autonomic
disorders, based on assessment of the Fellow by the Principal Investigator at approximately
6-month intervals. A secondary outcome measure is results obtained under this Protocol, which
may be analyzed and reported as research data along with data from other Protocols.

- INCLUSION CRITERIA - PATIENTS:

Patients may be included if they are at least 18 years old and are known or suspected to
have failure of one or more components of the autonomic nervous system, small fiber
neuropathy, inherited dysautonomia, orthostatic intolerance, neurocardiogenic syncope,
status post endoscopic thoracic sympathectomy, or disorders of catecholamine synthesis or
fate.

Children greater than two years old may be enrolled in this Protocol if they might
personally benefit from the tests in the Protocol through the establishment of a diagnosis.

EXCLUSION CRITERIA - PATIENTS:

There are no exclusion criteria for the study as a whole.

INCLUSION CRITERIA - HEALTHY VOLUNTEERS:

Healthy adult Volunteers (at least 18 years old) may be included, pending normal or
medically insignificant results of history and physical examination. A repeat history and
physical examination is required if more than 6 months have gone by from a previous workup.

EXCLUSION CRITERIA - HEALTHY VOLUNTEERS:

A candidate Healthy Volunteer will be excluded if the person has any of the following:
symptoms of autonomic dysfunction, including lightheadedness, fainting, abnormalities of
sweating, frequent nausea, vomiting, constipation, or diarrhea, or urinary frequency or
retention. If the Volunteer has a condition requiring medication that in the judgment of
the Investigators will affect results of the pupillometric tests under this Protocol, the
Volunteer may be included for training purposes.