Clinical Autonomic Disorders: A Training Protocol
Clinical Autonomic Disorders: A Training Protocol
Background:

 - The autonomic nervous system helps control things that happen automatically in the body,
 like blood pressure, heart rate, and digestion. When it doesn t work properly, it s called
 dysautonomia. Researchers want to learn more about dysautonomias and train doctors to learn
 how to better diagnose them.

 Objectives:

 -  To allow people with autonomic disorders to be evaluated.

 -  To help fellows in the NIH Autonomic Disorders fellowship program see a variety of
 diseases in their training.

 Eligibility:

 -  People age 18 and older with dysautonomia; children over age 2 if they might benefit

 -  Healthy adult volunteers

 Design:

 -  Participants will have different procedures depending on their symptoms. They will have
 one or more visits. They will have some but not all of the tests below.

 -  Participants will have a medical history and physical exam. They may be tested for
 problems thinking. They may complete a symptom questionnaire. They may have their
 temperature and blood pressure taken.

 -  Participants may have an intravenous line placed. A needle will guide a thin plastic
 tube into an arm vein.

 -  Participants may have blood drawn several times. They may give a urine sample.

 -  Participants may have an electrocardiogram.

 -  How much blood a participant s heart is pumping and/or how much blood is flowing through
 their arm may be measured. The total amount of blood in their bloodstream may be
 measured.

 -  Participants may have their ability to sweat and/or sense of smell tested.

 -  Breathing, bowel sounds, and/or body functions may be monitored.

 -  Pupil size, response to environmental temperature changes, and/or breathing may be
 measured.

 -  Participants may have a bladder ultrasound.

 -  Small pieces of skin may be taken for study.
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.