Vasospasm can have many different causes and can occur in a variety of dise
ases, including infectious, autoimmune, and ophthalmic diseases, as well as
in otherwise healthy subjects. We distinguish between the primary vasospas
tic syndrome and secondary vasospasm. The term "vasospastic syndrome" summa
rizes the symptoms of patients having such a diathesis as responding with s
pasm to stimuli like cold or emotional stress. Secondary vasospasm can occu
r in a number of autoimmune diseases, such as multiple sclerosis, lupus ery
thematosus, antiphospholipid syndrome, rheumatoid polyarthritis, giant cell
arteritis, Behcet's disease, Buerger's disease and preeclampsia, and also
in infectious diseases such as AIDS. Other potential causes for vasospasm a
re hemorrhages, homocysteinemia, head injury, acute intermittent porphyria,
sickle cell disease, anorexia nervosa, Susac syndrome, mitochondriopathies
, tumors, colitis ulcerosa, Crohn's disease, arteriosclerosis and drugs. Pa
tients with primary vasospastic syndrome tend to suffer from cold hands, lo
w blood pressure. and even migraine and silent myocardial ischemia. Valuabl
e diagnostic tools for vasospastic diathesis are nailfold capillary microsc
opy and angiography, but probably the best indicator is an increased plasma
level of endothelin-1. The eye is frequently involved in the vasospastic s
yndrome, and ocular manifestations of vasospasm include alteration of conju
nctival vessels, corneal edema, retinal arterial and venous occlusions, cho
roidal ischemia, amaurosis fugax, AION, and glaucoma. Since the clinical im
pact of vascular dysregulation has only really been appreciated in the last
few years, there has been little research in the according therapeutic fie
ld. The role of calcium channel blockers, magnesium, endothelin and glutama
te antagonists, and gene therapy are discussed. (C) 2001 Elsevier Science L
td. All rights reserved.