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.
SusacJ.O., HardmanJ.M., SelhorstJ.B.. Microangiopathy of the brain and retina.Neurology1979; 29: 313–6.
2.
MonteiroM.L., SwansonR.A., CoppetoJ.R., CuneoR.A., DeArmondS.J., PrusinerS.B.. A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions.Neurology1985; 35: 1113–21.
3.
MacFadyenD.J., SchneiderR.J., ChisholmI.A.. A syndrome of brain, inner ear and retinal microangiopathy.Can J Neurol Sci1987; 14: 315–8.
4.
HeiskalaH., SomerH., KovanenJ., PoutiainenE., KarliH., HaltiaM.. Microangiopathy with encephalopathy, hearing loss and retinal arteriolar occlusions: two new cases.J Neurol Sci1988; 86: 239–50.
5.
SchwitterJ., AgostiR., OttP., KalmanA., WaespeW.. Small infarctions of cochlear, retinal, and encephalic tissue in young women.Stroke1992; 23: 903–7.
6.
NotisC.M., KiteiR.A., CaffertyM.S., OdelJ.G., MitchellJ.P.. Microangiopathy of brain, retina, and inner ear.J Neuroophthalmol1995; 15: 1–8.
O'HalloranH.S., PearsonP.A., LeeW.B., SusacJ.O., BergerJ.R.. Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature.Ophthalmology1998; 105: 1038–44.
9.
RaynaudM.. De l'asphyxie locale et de la gangrène des extrémités. Thèse. Paris, 1862.
10.
SanerH., WurbelH., MahlerF., FlammerJ., GasserP.. Microvasculatory evaluation of vasospastic syndromes.Adv Exp Med Biol1987; 220: 215–8.
11.
MahlerF., SanerH., WurbelH., FlammerJ.. Local cooling test for clinical capillaroscopy in Raynaud's phenomenon, unstable angina, and vasospastic visual disorders.Vasa1989; 18: 201–4.
12.
ZimmermannM.. Endothelin in cerebral vasospasm. Clinical and experimental results.J Neurosurg Sci1997; 41: 139–51.
13.
GasserP.. Video-nailfold-microscopy and local cold test: morphological and hemodynamic correlates in 124 healthy subjects.Vasa1991; 20: 244–51.
14.
GasserP., FlammerJ.. Blood-cell velocity in the nailfold capillaries of patients with normal-tension and high-tension glaucoma.Am J Ophthalmol1991; 111: 585–8.
15.
GasserP., OrgulS., DublerB., BucheliB., FlammerJ.. Relation between blood flow velocities in the ophthalmic artery and in nailfold capillaries.Br J Ophthalmol1999; 83: 505.
16.
GuthauserU., FlammerJ., MahlerF.The relationship between digital and ocular vasospasm.Graefes Arch Clin Exp Ophthalmol1988: 226: 224–6.
17.
FlammerJ., GasserP., PrunteC.H., YaoK.. The probable involvement of factors other than ocular pressure in the pathogenesis of glaucoma. In: DranceS.M., Buskirk VanE.M., NeufeldA.H., eds. Pharmacology of glaucoma.Baltimore: Williams and Wilkins, 1992; 273–83.
18.
FlammerJ., GuthauserU., MahlerF.. Do ocular vasospasm help cause low tension galucoma?Doc Ophthalmol Proc Ser1987; 49: 397–9.
19.
O'BrienC.. Vasospasm and glaucoma.Br J Ophthalmol1998; 82: 855–7.
20.
KaiserH.J., FlammerJ., MesserliJ.. Vasospasm - A risk factor for nonarteritic anterior ischemic optic neuropathy?J Neuroophthalmol1996; 16: 5–10.
21.
MesserliJ., FlammerJ.. Central vein thrombosis in younger patients. [Article in German].Klin Monatsbl Augenheilkd1996; 208: 303–5.
22.
PrunteC., FlammerJ.. Choroidal capillary and venous congestion in central serous chorioretinopathy.Am J Ophthalmol1996; 121: 26–34.
23.
OrgülS., FlammerJ.. Perilimbal aneurysms of conjunctival vessels in glaucoma patients.Ger J Ophthalmol1995; 4: 94–6.
KaiserH.J., SchötzauA., FlammerJ.. Blood-flow velocities in the extraocular vessels in normal volunteers.Am J Ophthalmol1996; 122: 364–70.
26.
MiyauchiT., MasakiT.. Pathophysiology of endothelin in the cardiovascular system.Annu Rev Physiol1999; 61: 391–415.
27.
FlammerJ., PacheM., ResinkT.H.. Vasospasm, its role in the pathogenesis of diseases with particular reference to the eye.Prog Retin Eye Res2001; 20: 319–49.
28.
GravesonC.S.. Retinal artery occlusion in migraine.Br J Ophthalmol1949; 2: 838–40.
29.
WeinsteinJ.M., FemanS.S.. Ischemic optic neuropathy in migraine.Arch Ophthalmol1982; 100: 1097–100.
30.
ViirreE.S., BalohR.W.. Migraine as a cause of sudden hearing loss.Headache1996; 36: 24–8.
31.
PrunteC.H., FlammerJ.. Mikrozirkulationsstörungen als pathogenetischer Faktor des Glaukoms - wird der Verdacht zur Gewissheit? In: PrunteC., FlammerJ., Hrsg. Das Glaukom in der Praxis.Basel: Karger, 1997; 39–52.
32.
ArnoldG., ReuterU., KinzeS., WolfT., EinhauplK.M.. Migraine with aura shows gadolinium enhancement which is reversed following prophylactic treatment.Cephalalgia1998; 18: 644–6.
33.
StromanG.A., StewartW.C., GolnikK.C., CureJ.K., OlingerR.E.. Magnetic resonance imaging in patients with low-tension glaucoma.Arch Ophthalmol1995; 113: 168–72.
34.
SahinO., GoldsteinD.A., TesslerH.H.. Findings typical of Susac's syndrome in a patient with scleroderma.Retina1999; 19: 476–7.
35.
GasserP., FlammerJ.. Short-and long-term effect of nifedipine on the visual field in patients with presumed vasospasm.J Int Med Res1990; 18: 334–9.
36.
DettmannE.S., LüscherT.H.F., FlammerJ., HaefligerI.O.. Modulation of endothelin-1-induced contractions by magnesium/calcium in porcine ciliary arteries.Graefes Arch Clin Exp Ophthalmol1998; 236: 47–51.
37.
MeyerP., FlammerJ., LuscherT.H.F.. Endothelium-dependent regulation of the ophthalmic microcirculation in the perfused porcine eye: role of nitric oxide and endothelins.Invest Ophthalmol Vis Sci1993; 34: 3614–21.
