Susac Syndrome: a Vasospastic Disorder?
Overview
Authors
Affiliations
Purpose: The Susac syndrome is a microangiopathy that leads to visual symptoms, hearing loss and neurological symptoms.
Case Report: We report on a young woman suffering from this syndrome who also presented the following signs and symptoms typical of a vasospastic syndrome; 1) a history of cold hands, low blood pressure and migraine; 2) a typical alteration of conjunctival vessels; 3) prolonged flow arrest time after cooling in nailfold capillaromicroscopy; 4) increased resistivity in the orbital vessels measured by color Doppler imaging; and 5) an increased plasma level of endothelin-1.
Conclusions: We postulate that the Susac syndrome is a manifestation of the vasospastic syndrome.
Neurodegeneration in Alzheimer's disease and glaucoma: overlaps and missing links.
Sen S, Saxena R, Tripathi M, Vibha D, Dhiman R Eye (Lond). 2020; 34(9):1546-1553.
PMID: 32152519 PMC: 7608361. DOI: 10.1038/s41433-020-0836-x.
Transient visual field impairment after cold provocation in glaucoma patients with Flammer syndrome.
Terelak-Borys B, Grabska-Liberek I, Schoetzau A, Konieczka K Restor Neurol Neurosci. 2019; 37(1):31-39.
PMID: 30741709 PMC: 6484275. DOI: 10.3233/RNN-180866.
Diseases potentially related to Flammer syndrome.
Konieczka K, Erb C EPMA J. 2017; 8(4):327-332.
PMID: 29209435 PMC: 5700007. DOI: 10.1007/s13167-017-0116-4.
The discovery of the Flammer syndrome: a historical and personal perspective.
Flammer J, Konieczka K EPMA J. 2017; 8(2):75-97.
PMID: 28725290 PMC: 5486542. DOI: 10.1007/s13167-017-0090-x.
Multiple sclerosis and primary vascular dysregulation (Flammer syndrome).
Konieczka K, Koch S, Binggeli T, Schoetzau A, Kesselring J EPMA J. 2016; 7:13.
PMID: 27307797 PMC: 4908696. DOI: 10.1186/s13167-016-0062-6.