Autonomic Small Fiber Neuropathy and Ehlers Danlos Syndromes - Prospective Study and Registry (ProANS)

RWTH Aachen University

Status: Enrolling

Conditions: Autonomic Neuropathy; Ehlers-Danlos Syndrome Hypermobility Type; Postural Tachycardia Syndrome; Small Fiber Neuropathy

Treatments: Other: No intervention planned, but all patients get our standart treatment

Study type: Observational

Funder types: Other

Identifiers

NCT04310644

19-016

Details and patient eligibility

About

We examine patients with different autonomic neuropathies and Ehlers Danlos syndromes compared to healthy controls at three different points over time (baseline, after 3 months and after 1.5 years) to gain knowledge about the course of this disease and understand its pathophysiology, with a focus on Small Fiber neuropathy. Moreover we will validate the german version of the Malmö POTS Score and establish an easy diagnostic scheme for patients in outpatient care.

Full description

Inclusion of patients with autoimmune autonomic neuropathies/ pure autonomic failure, postural orthostatic tachycardia syndrome, small fiber neuropathies, Ehlers-Danlos syndromes, Mast cell activation syndrom, Chronic fatigue syndrome and PostCOVID syndrome in our Registry study with follow up visits. Comparison to healthy controls concerning selected examinations.

Planned examinations are laboratory tests, questionnaires on mental and physical health status and circulatory disorders, attention tests, tilt table testing, standing test, sweat function, investigation of small fiber function via quantitative sensory testing as well as the density of nerve fibers in the skin. Measurements are performed at baseline mostly in clinical routine and follow up visits are offered.

Enrollment: 200 estimated patients

Sex: All

Ages: 18 to 80 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• autonomic neuropathy
• Postural orthostatic tachycardia syndrome
• hypermobile or classical Ehlers Danlos syndromes
• Chronic fatigue syndrome, mast cell activation syndrome and/or PostCOVID
• healty controls
• between 18-80 years
• in patients: diagnosis and clinical testing in our outpatient clinic
• German speaking

Exclusion criteria

• pregnancy
• Pacemaker or Deep brain Stimulation
• sensory or motor Polyneuropathy
• neurodegenerative disease

Trial design

200 participants in 5 patient groups

Postural Orthostatic Tachycardia Syndrome

Description:

Patients with orthostatic intolerance because of Postural orthostatic tachycardia syndrome diagnosed in our outpatient clinic by tilt table examination.

Treatment:

Other: No intervention planned, but all patients get our standart treatment

Ehlers Danlos Syndrome

Description:

Patients with hypermobile or classical EDS who are already diagnosed including genetical testing for classical or vascular EDS and Marfan Syndromes

Treatment:

Other: No intervention planned, but all patients get our standart treatment

Autoimmune autonomic neuropathy/Pure autonomic failure

Description:

Patients who have an autoimmune autonomic neuropathy based on clinical diagnosis and antibody testing in our outpatient clinic. Cardial MIBG Scintigraphy should have been performed.

Treatment:

Other: No intervention planned, but all patients get our standart treatment

Healthy controls

Description:

Healthy controls with no documented cardiovascular or neurological disorders and no symtoms of autonomic failure/dizziness/fainting

others

Description:

Patients who have comorbidities as mast cell activation syndrome, chronic fatigue and/or Post COVID syndrome, based on clinical diagnosis in our outpatient clinic.