Diseases of the autonomic nervous system
Conditions
Interventions
Group 1: On examination day 1: LBNP + cerebral fMRI and after a break LBNP + cardio-MRI.
On examination day 2: plasma volume and active standing test. After a break, microneurography + TT.
Group 2: Th
Sponsors
Uniklinik RWTH AachenKlinik für Neurologie
Eligibility
Sex/Gender
All
Age
18 Years to 60 Years
Inclusion criteria
Inclusion criteria: 1. age between 18 - 60 years (register: at least 18 years old). 2. written declaration of consent 3. persons who are legally competent and mentally capable of to follow the instructions of the study personnel 4. healthy persons OR: Patients with POTS, hypermobile or classic EDS, SFN or PAF/AAN (Diagnosis made in the ANS outpatient clinic on the basis of the clinical examinations including autonomic function testing and skin biopsy) Exclusion of other causes (including by means of cranial MRI and basic laboratory). 5. in all POTS, EDS and PAF/AAN patients at least the following examinations have been performed in clinical routine in the last 6 months before inclusion in the ProANS study or will be completed otherwise completed (will not be added to the registry!) - Quantitative sensory testing (QST) - skin biopsy - Blood sampling (noradrenaline, adrenaline and specific antibodies) - Tilt table (KT) incl. heart rate analysis - Quantitative sensorimotor axon reflex testing (QSART)
Exclusion criteria
Exclusion criteria: 1. pregnancy (anamnestic, not in the register) 2. minority 3. pacemaker (HSM) or deep brain stimulation (TSH) (exclusion for electrophysiological examinations) (not in the registry) 4. moderate and severe sensorimotor polyneuropathy (not in the registry) 5. other neurodegenerative diseases such as Parkinson's, Alzheimer's disease or inflammatory diseases such as multiple sclerosis (not in the registry) 6. severe heart failure (not in registry) 7. diabetic rentinopathy (exclusion for Valsalva maneuvers, not in registry) 8. poorly controlled diabetes mellitus (not in the registry) 9.drug use (not in the register) 10.persons who are institutionalized by official or judicial order are placed in an institution - Claustrophobia - Metal implants, active medical implants (e.g. Infusion pump, etc.), or other conditions that preclude an MRI examination
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - Difference in flow and time-volume responses of the inferior vena cava to LBNP in the supine position between patients with EDS and healthy controls. | — |
Secondary
| Measure | Time frame |
|---|---|
| - Difference in flow and volume response of the truncus pulmonalis during supine LBNP between patients with EDS compared with healthy subjects. - Differences in flow and volume response of the ascending aorta during supine LBNP between patients with EDS compared with healthy subjects - Differences in heart size and function between EDS patients and healthy controls; - Difference in plasma volume between EDS patients and healthy controls; - Differences in sympathetic nerve activity as well as release of catecholamines during the tilt table test between EDS patients and healthy controls. - Difference in the extent of fascicle dynamics between EDS patients and healthy controls; | — |
Countries
Germany
Contacts
Public ContactLaura de Boni
DLR Deutsches Zentrum für Luft- und RaumfahrtInstitut für Luft- und Raumfahrtmedizin
Outcome results
None listed