Primary: To explore the diagnostic value to distinguish PAF and MSA of a variety of tests: corneal confocal microscopy optical coherence tomography (OCT) to measure the retinal nerve fibre layer thickness and total macular volume, corneal…
ID
Source
Brief title
Condition
- Ocular structural change, deposit and degeneration NEC
- Peripheral neuropathies
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
- CCM : Difference in nerve fiber density in the cornea between patients with
PAF, patients with MSA and controls. A difference of 14.2 fibers / mm2 between
groups is regarded as a minimal clinically relevant difference .
- OCT: Difference in the RNFL (Retinal Nerve Fiber Layer) thickness between
patients with PAF, patients with MSA and controls.
- WISW : Difference in the degree of wrinkling of the skin in the fingertips
between patients with PAF, patients with MSA and controls. A difference of 1 '
grade ' between goups is considered a minimal clinically relevant difference .
- Sudoscan: Difference in electrochemical skin conductance in microsiemens (µS)
between patients with PAF, patients with MSA and controls. A difference in skin
conductance of 10 µS between groups is considered the minimal clinically
relevant difference.
Secondary outcome
ROX: difference in the arterial and venous oxygenation during a fall in blood
pressure among patients with PAF, patients with MSA and controls.
Background summary
Two such rare disorders, pure autonomic failure (PAF) and multiple system
atrophy (MSA,) cause very severe autonomic failure as well as major diagnostic
difficulties due to many clinical similarities: while both exhibit progressive
autonomic failure, patients with MSA additionally develop movement disorders
during the course of their disease and suffer the burden of a much less
favourable prognosis than patients with PAF, with a mean survival of six years.
Consensus criteria for MSA stipulate that a definite diagnosis of MSA can still
only be made after death, leaving patients with autonomic failure to face years
of uncertainty.
Study objective
Primary: To explore the diagnostic value to distinguish PAF and MSA of a
variety of tests: corneal confocal microscopy optical coherence tomography
(OCT) to measure the retinal nerve fibre layer thickness and total macular
volume, corneal sensitivity, a test of sweat production (Sudoscan) and water
induced skin wrinkling (WISW).
Secondary: To explore whether measuring retinal perfusion in patients with
autonomic failure using retinal oximetry (ROX) is suited as a
pathophysiological tool.
With the help of one or more of these methods can be made a diagnosis hopefully
earlier in the course of the disease in the future for PAF and MSA patients and
thus years of uncertainty to be removed.
Study design
Observational prospective study.
Study burden and risks
There is a single, low risk for the participants, since all tests are
non-invasive. PAF and MSA patients can get enough rest between the tests and at
any time the tests can be paused.
There are hardly any risks associated with the study, only a very low risk of
glaucoma. The pupil dilation with Phenylephrine HCL 2.5% en Tropicamide 0.5% is
necessary to view the posterior segment of the eye. Side effects of these drops
include photophobia and reduced accommodative power during 2 hours. We do not
expect any adverse events or serious adverse events. There is a very low risk
of acute angle-closure glaucoma due to pupil dilation: 3:10.000. If this
occurs, we will examine and treat the patient according to standard car at
Dept. Ophthalmology at LUMC.
Albinusdreef 2
Leiden 2333ZA
NL
Albinusdreef 2
Leiden 2333ZA
NL
Listed location countries
Age
Inclusion criteria
All participants: older than 18 years and the ability to provide informed consent.
Patients with PAF are defined according to the consensus of the American Autonomic Society and the American Acedemy of Neurology (AAN):
- Presence of NOH AND
- Evidence of more widespread autonomic failure AND
- No other neurological features
Patients with MSA are defined according to the second consensus criteria of the AAN.
Healty controls: no signs of autonomic failure or other relevant neurological or other disorders.
Exclusion criteria
For all participants: younger than 18 years or not able to provide informed consent.
Furthermore, a potential subject who meets any of the following criteria will be excluded from participation in this study:
- Diabetes Mellitus
- Inability to sustain the investigations due to the nature of their disease
- Ophtalmological morbidity: AMD, macular dystrophies, glaucoma, herpes keratitis, history of cornea surgery like refractive surgery
Design
Recruitment
Medical products/devices used
metc-ldd@lumc.nl
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL54904.058.16 |