Abstract:Purpose: To analyze how far an ischemic component might have been involved in optic neuritis. Methods: Case report: a 32-year-old man with symptoms characteristic for optic neuritis underwent extensive clinical, laboratory/serological and vascular examination for systemic associations and vascular involvement. Results: The patient was found to have a temporary ocular blood flow dysregulation and increased plasma endothelin-1 levels which decreased after the acute phase of the optic nerve. Conclusions: We concl… Show more
“…One may, however, hypothesize that this increase in perfusion is related to an indirect effect of the drug. A case with acute optic neuritis has previously been reported showing a pronounced transient increase in endothelin-1 (ET-1) plasma levels associated with a reduction in ocular blood flow [17]. This is in good agreement with another study indicating increased plasma levels of ET-1 in patients with multiple sclerosis [18].…”
A small but significant increase in ONH blood flow resulted from infusion of high-dose prednisolone. Further studies are required to investigate whether this effect contributes to the therapeutic efficacy of cortisone in patients with optic neuritis.
“…One may, however, hypothesize that this increase in perfusion is related to an indirect effect of the drug. A case with acute optic neuritis has previously been reported showing a pronounced transient increase in endothelin-1 (ET-1) plasma levels associated with a reduction in ocular blood flow [17]. This is in good agreement with another study indicating increased plasma levels of ET-1 in patients with multiple sclerosis [18].…”
A small but significant increase in ONH blood flow resulted from infusion of high-dose prednisolone. Further studies are required to investigate whether this effect contributes to the therapeutic efficacy of cortisone in patients with optic neuritis.
“…5a). We found increased ET levels in giant cell arteritis [208], rheumatoid arthritis [209], fibromyalgia syndrome [210], multiple sclerosis [211, 212], optic neuritis [213], retinal vein occlusions [214], retinitis pigmentosa [215], Susac syndrome [216] and in patients with cystic macula oedema that responded poorly to anti-VEGF therapy [217]. The border between primary and secondary vascular dysregulation is sometimes vague, which we will discuss by way of the example of multiple sclerosis (MS) further below.Fig.…”
Section: From Vasospasm To Vascular Dysregulationmentioning
confidence: 99%
“…MS patients without a history of retrobulbar neuritis had subclinical visual field defects [226], narrower retinal arterioles and wider retinal venules [227], increased rigidity of these retinal vessels [228] and thinning of the macula [229]. During optic neuritis, we observed a transient raise of the ET plasma levels, a reduction of ocular blood flow [213], an improvement of visual function after intake of red wine [230] and a distension of the optic nerve sheaths [231]. A controlled study confirmed the presence of FS symptoms in MS patients [232].…”
Section: From Vasospasm To Vascular Dysregulationmentioning
This review describes the clinical and basic research that led to the description of Flammer syndrome. It is narrated from a personal perspective. This research was initiated by the observation of an increased long-term fluctuation of visual fields in a subgroup of glaucoma patients. As these patients had strikingly cold hands, peripheral blood flow was tested with a capillary microscopy, and vasospastic syndrome (VS) was diagnosed. Further studies on these patients revealed frequently weakened autoregulation of ocular blood flow and increased flow resistivity in retroocular vessels. Their retinal vessels were more rigid and irregular and responded less to flickering light. Holistic investigation demonstrated low blood pressure, silent myocardial ischaemia, altered beat-to-beat variation, altered gene expression in the lymphocytes, slightly increased plasma endothelin level and increased systemic oxidative stress. This combination of signs and symptoms was better described by the term primary vascular dysregulation (PVD) than by VS. Subsequent studies showed additional symptoms frequently related to PVD, such as low body mass index, cold extremities combined with slightly increased core temperature, prolonged sleep onset time, reduced feelings of thirst, increased sensitivity to smell and also for certain drugs and increased retinal venous pressure. To better characterise this entire syndrome, the term Flammer syndrome (FS) was introduced. Most subjects with FS were healthy. Nevertheless, FS seemed to increase the risk for certain eye diseases, particularly in younger patients. This included normal-tension glaucoma, anterior ischaemic optic neuropathy, retinal vein occlusions, Susac syndrome and central serous chorioretinopathy. Hereditary diseases, such as Leber’s optic neuropathy or retinitis pigmentosa, were also associated with FS, and FS symptoms and sings occurred more frequent in patients with multiple sclerosis or with acute hearing loss. Further research should lead to a more concise definition of FS, a precise diagnosis and tools for recognizing people at risk for associated diseases. This may ultimately lead to more efficient and more personalised treatment.
“…33,34 Transient increases in plasma endothelin may be associated with ischaemic optic neuropathy (case report). 35 Endothelin receptors have been found on optic nerve head astrocytes 36 that regulate astrocyte proliferation in disease states and directly influence axon function. Elevated endothelin levels can also decrease the anterograde transport of mitochondria in animal models 37 thereby compromising local supplies of ATP.…”
Retinal ganglion cells are the output cells of the retina whose axons are under considerable metabolic stress in both health and disease states. They are highly polarised to ensure that mitochondria and enzymes involved in the generation of ATP are strategically concentrated to meet the local energy demands of the cell. In passing from the eye to the brain, axons are protected and supported by glial tissues and the blood supply of the optic nerve head is regulated to maintain the supply of oxygen and nutrients to the axons.In spite of this, the optic nerve head remains the point at which retinal ganglion cell axons are most vulnerable to the effects of increased intraocular pressure or ischaemia. Considerable work has been undertaken in this area to advance our understanding on the pathophysiology of axon damage and to develop new strategies for the prevention of retinal ganglion cell death.
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