Ischaemic optic neuropathy in pulseless disease.
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Biomedical subjects
Publications and source records attributed to M D Sanders.
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Seven Ridley Mark I anterior chamber lenses were removed from patients at St Thomas's Hospital. The lenses had been in place for an average of 15 years. The indication for removal was corneal endothelial decompensation with painful bullous keratopathy. Examination of the lenses under the scanning electron microscope showed remarkably similar morphology in each case. A well organised fibrin membrane covered the implants to which iris pigment epithelial cells were adherent. Red blood cells, scattered fibroblasts and pigment granules were also found. Giant cells were not identified while macrophages and lymphocytes were rare. These findings suggest that corneal endothelial decompensation was not due to chronic inflammation but was probably caused by movement of the implants within the eyes.
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Sera from 44 patients with isolated retinal vasculitis (RV), 38 patients with retinal vasculitis accompanying systemic inflammatory diseases (RV + SID), and 33 patients with a similar range of systemic inflammatory diseases without eye involvement (SID alone) were assayed for circulating immune complexes (CIC) and for anti-retinal autoantibodies. CIC were present in 41% of patients with isolated RV and 55% of patients with RV + SID, whilst anti-retinal antibodies were present in about 70% of all patients with RV. 42% of those with SID alone had CIC and 30% of those with SID alone had retinal autoantibodies. Titres of anti-retinal antibodies were higher in patients with RV than in those with SID alone. In isolated RV there was an inverse relation between pronounced retinal autoimmunity and the occurrence of CIC--i.e., the more severe autoimmune retinal disease occurred in CIC-negative patients. Most patients with RV + SID tended to have mild or moderate retinal disease accompanied by both retinal autoantibodies and CIC, but severe retinal disease occurred in CIC-positive patients who did not have circulating anti-retinal antibodies. Patients with SID alone had high titres of retinal antibodies only when they were CIC-positive. It is suggested that the formation of CIC, possibly of an idiotype/anti-idiotype nature, may be a compensatory mechanism accompanying anti-retinal autoimmunity and that an imbalance between autoimmunity and immune complex formation may be an important predisposing factor in the development of retinal inflammatory disease.
Seventy cases of retinal embolism showed cholesterol and platelet-fibrin emboli, usually from a carotid source, and calcific emboli, usually from a cardiac source, in that order of frequency. A marked preference for the temporal circulation, and particularly for the posterior pole, was observed with all the types of emboli. Only patients with cholesterol embolism complained of amaurosis fugax, whereas all the patients with calcific or stationary platelet-fibrin emboli experienced permanent visual loss. Visual field defects were characteristic of those seen with degeneration of the retinal axons. Collateral vessels usually developed with emboli to the arterioles of the disc and peripapillary region. Periarteriolar sheathing, as well as late fluorescein leakage from the impacted site, seemed to follow the cases of more severe endothelial damage due to cholesterol embolism. Subtotal nonprogressive ischemia ensued in relationship to post-embolic sheathing, which eventually disappeared, leaving a narrowed arteriole.
Five patients with optic neuropathy, four vascular and one demyelinating, are described who each complained of an unusual symptom. Bright flashes of light (phosphenes) occurred in the affected eyes and were evoked by sudden unexpected sounds. Movement of the eye alone did not reproduce the symptom. In all patients the phenomenon was sufficiently prominent to interfere with sleep and was the main complaint of one patient. An anticonvulsant (phenytoin) greatly reduced the frequency and intensity of the phosphene in one patient.
A 48-year-old man presented with a vertical gaze palsy associated with secondary syphilis. It is suggested that the eye movement disorder is due to syphilitic endarteritis in the mesodiencephalic region.
We reviewed the clinical features, natural history and visual prognosis of 26 patients with histologically confirmed sarcoidosis. Compatible chest x-ray changes were found in 75% of patients. Periphlebitis was the commonest fundus feature. Disc changes were seen in a substantial number of patients, and the condition can present as unilateral disc oedema. The disease seems primarily to involve equatorial retinal veins, and occlusions of a hemisphere branch vein or central retinal vein did not occur. Changes in the subretinal pigment epithelium were noted in a substantial number of patients but did not produce visual morbidity, and these patients seemed to have less florid periphlebitis than others. The disease has a low visual morbidity unless neovascularisation develops; the treatment of this remains controversial.
Eight patients who had limited forms of Wegener's granulomatosis are described, with details of their pathology. Ocular pathological data were available for 2 of them. The condition carries a serious ocular risk; useful vision was lost in 6 out of 16 eyes (37%). An indolent but slowly progressive marginal keratitis and scleritis was a prominent feature in 4 patients and was helpful in suggesting the diagnosis. Limited froms of Wegener's granulomatosis carry a better prognosis and response to treatment than the classical disease.
Immunological investigations have been performed in eighty patients with retinal vasculitis. 50 per cent of patients with retinal vasculitis associated with systemic inflammatory disease have raised circulating immune complexes, abnormal complement abnormalities, and antibodies to retinal S antigen. Patients with isolated retinal vasculitis demonstrate similar abnormalities when vascular leakage is the predominant manifestation, but a higher incidence (89 per cent) of retinal antibodies and a lower incidence of immune complexes (22 per cent) are found if reduced retinal perfusion is present. A rise in titre of antibodies to smooth muscle occurred in several patients before the recurrence of uveitis, indicating that this test may predict a clinical relapse.
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This paper presents the results of a point-prevalence study of circulating autoantibodies and immune complexes in 44 patients with isolated retinal vasculitis (RV alone), 38 patients with retinal vasculitis accompanied by systemic inflammatory disease (RV + SID), and 33 patients with a similar range of systemic inflammatory disease but without eye involvement (SID alone). In isolated retinal vasculitis, antiretinal antibodies, non-retinal antibodies, and circulating immune complexes each showed a prevalence of about 50 per cent, but there was an inverse relationship between marked antiretinal autoimmunity and the occurrence of circulating immune complexes. In SID alone, antiretinal antibodies (ret-AB) were of lower prevalence (about 20 per cent) and occurred only in patients who had circulating immune complexes (CIC). Patients with RV + SID fell into two principal categories: (a) ret-AB with CIC, and (b) ret-AB without CIC. In isolated retinal vasculitis, the occurrence of high levels of retinal autoimmunity in the absence of circulating immune complexes was associated with the more severe retinal disease, while in those RV + SID patients who did not express autoimmunity, the more severe retinal disease was associated with the presence of circulating immune complexes. It is suggested that the formation of circulating autoimmune complexes, possibly of an idiotypic: anti-idiotypic character, may be a 'risk' mechanism for limiting retinal-specific autoimmunity, and that certain patients with severe retinal vasculitis may undercompensate or overcompensate in this way.
Twenty-six children with juvenile Batten's disease are reviewed. On clinical and histological evidence they appear to represent a specific disease entity, which though rare is a substantial cause of blindness in children aged 5--15 years. Children present with rapid progressive visual loss at age 6--7 years, early mental deterioration, and fits about 2--4 years later, and this is the stage at which the diagnosis is usually made. Macular degeneration appears to be a consistent early feature, and peripheral retinal changes become more marked as the disease progresses. Phototoxicity may possibly play a part in the retinal degeneration.
Six cases are presented with macular changes in association with papilloedema; 4 suffered permanent visual loss. The present paper emphasises this previously infrequent finding and discusses the haemodynamic and mechanical factors responsible. The macular changes consisted of haemorrhages situated in front, within, or behind the retina, and occasionally the results of neovascular membrane formation produced secondary visual loss. Changes in the pigment epithelium were seen in 3 cases associated with choroidal folds. Macular stars rarely produce visual loss. Recognition of these changes is important in the assessment of the visual loss in papilloedema.
Two cases are described with severe intracranial hypertension, papilloedema, and a hitherto unreported haemorrhagic peripheral retinopathy. The marked disc swelling in these patients has probably contributed to a venous occlusive element resulting in the haemorrhagic retinopathy.
Seven cases are presented in which prolonged papilloedema led to the development of acquired optociliary shunt vessels. These vessels may also be found with optic nerve tumours, particularly spheno-orbital meningiomas, optic nerve drusen, glaucoma, and after central retinal vein occlusion. Two patients had intracranial tumours, 4 benign intracranial hypertension, and one Crouzon's disease. Three had marked atrophic changes of the disc. The pathophysiology of the disc changes is discussed. The triad of long-standing poor vision, acquired optociliary shunts, and optic atrophy with blurred disc margins should not be regarded as specific for spheno-orbital meningioma.
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