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"Twin peaks" papilledema: the appearance of papilledema with optic tract atrophy.

A woman with a right middle fossa meningioma causing right optic tract atrophy and papilledema had distinctive funduscopic changes. The ipsilateral eye showed temporal disc pallor and nasal edema. The contralateral disc showed edema of the upper and lower poles which was separated by a horizontal band of optic atrophy. The fluorescein angiographic changes clearly illustrate this pattern. Apart from its theoretical interest this appearance of papilledema and atrophy provides definite localizing information.

Adult

Papilledema: its recognition and relation to increased intracranial pressure.

This has been a review on the subject of papilledema from a clinical, pathologic and experimental point of view. Terminology has been clarified. The term papilledema should be reserved for those patients with optic disc edema caused by increased intracranial pressure. The forms of papilledema which are identifiable are: 1. Early (incipient) form which develops into an acute type and then into a fully developed form; 2. Fully developed form, characterized by obscured disc margins, hemorrhages and ischemic infarcts; 3. Chronic papilledema which may persist (the name "vintage" papilledema may be applied to those patients affected in varying degrees up to several years); and 4. Chronic atrophic papilledema which, as the name implies, illustrates a form we do not see as frequently as in years past. It is the type we are now trying to prevent and in this effort we are fortunately achieveing some success. The importance of the intracranial expanding lesions and the influence of the rapidity of elevation and duration of intracranial hypertension on the optic nerve has been considered, based on the clinical and experimental work to date. The pathogenesis of papilledema has been considered in some detail. A unified whole is difficult to arrive at in such a complex situation. Recent advances in our understanding of anatomic, mechanical (sheath space pressure), tissue and vascular pressure as related to increased intracranial pressure have been described. A hydrostatic mechanism brings these factors together in a reasonable, although admittedly not completely proven concept of a mechanism for the development of papilledema. Clarification of terminology and clinical appearance of the various forms of disc edema related to intracranial pressure are of practical value in the diagnosis and management of each patient we see with this clinical entity.

Acute Disease

Optic disc edema in raised intracranial pressure. IV. Axoplasmic transport in experimental papilledema.

Tritiated leucine was injected intravitreously into the eyes of rhesus monkeys that had developed papilledema secondary to implantation of intracranial balloons. Autoradiographic studies of the optic nerve head showed that six hours after intravitreous injection of the isotope the fast component of axoplasmic transport accumulated in the regions of the lamina choroidalis and lamina scleralis. The slow component arrived at the optic nerve head two to four days after injection, and the swollen axons of the entire optic nerve head were filled with radioactive isotopes. Twelve days after injection of isotope, the axons in the optic nerve head were still diffusely labeled. Disturbance of axoplasmic transport was one of the primary events resulting in swelling of axons in papilledema. The pattern of axoplasmic disturbances in papilledema secondary to raised intracranial pressure was similar to that observed in papilledema secondary to ocular hypotony or increased intraocular pressure. Ocular hypotony, raised intracranial pressure, and increased intraocular pressure appear to share a final common pathway. All these conditions apparently converge into this final common pathway of disturbance of axoplasmic transport to give rise to papilledema.

Animals

Experimental papilledema produced by cyclocryotherapy.

Cyclocryotherapy was a reliable method of producing prolonged decreased intraocular pressure and papilledema in the rhesus monkey. Histologic changes in papilledema caused by decreased pressure were indistinguishable from those in papilledema from increased intracranial pressure. Electron microscopic observations in papilledema after cyclocryotherapy suggested that the principal morphologic alteration in the optic disk is axonal swelling. Additionally, many mitochondria accumulated in some swollen axons. These changes, observed at the ultrastructural level, suggest that alternations of axoplasmic transport may play a significant role in the pathogenesis of swelling of the disk.

Animals

Optic nerve axoplasm and papilledema.

A detailed review of optic nerve axoplasm is presented. A number of hypotheses have been postulated for the pathogenesis of papilledema associated with increased intracranial pressure. These hypotheses, mechanical and nonmechanical, are critically evaluated in relation to five essential features of papilledema. Theories, as well as clinical and experimental studies, of axonal transport are reviewed, and a new hypothesis is proposed: Papilledema is primarily a mechanical, nonvascular phenomenon in which an excess amount of extracellular fluid is present in the prelaminar region of the optic disc and the accumulation of that fluid results from the leakage of axoplasm from optic nerve fibers which are compressed posterior to the lamina cribrosa of the optic disc. The authors believe that this is the only existing hypothesis consistent with all the known facts about papilledema. Discussions by Drs. J. Terry Ernest, Thomas R. Hedges, and S. S. Hayreh follow the review.

Animals

A light microscopic, autoradiographic study of axoplasmic transport in the optic nerve head during ocular hypotony, increased intraocular pressure, and papilledema.

Cyclocryotherapy of the monkey eye reliably produces transient increased intraocular pressure followed by prolonged hypotony, during which papilledema occurs. Axoplasmic transport was studied while the intraocular pressure was both increased and decreased after cyclocryotherapy by autoradiography following the intravitreal injection of tritiated leucine and proline. Surgical fistulization of the anterior chamber was also used to produce hypotony and papilledema. Significant alterations of both the rapid and the slow components of axoplasmic transport were demonstrated in the nerve head during increased intraocular pressure, and in ocular hypotony with papilledema.

Animals

Funduscopic appearance of papilledema with optic tract atrophy.

In a child with a hypothalamic glioma, elevated intracranial pressure and retrograde optic tract atrophy produced distinctive patterns of papilledema and disc pallor in each eye. On the side of the tract lesion, the disc was swollen nasally and was pale temporally. In the contralateral fundus, the swollen optic disc was bisected horizontally by a pale atrophic band. These funduscopic signs exemplify a rare instance in neuro-ophthalmologic diagnosis wherein specific lateralizing and localizing information can be deduced solely from the appearance of papilledema.

Astrocytoma

Papilledema in endemic typhus.

A 21-year-old women developed severe bilateral papilledema during an acute febrile disease. Her optic disk margins were blurred and the disks were elevated up to 5 diopters. Splinter hemorrhages, cotton-wool exudates, cytoid bodies, and sheathing of veins were also present. The pyrexia was caused by murine typhus diagnosed by serologic tests. These tests revealed that Proteus OX-19 agglutination titer rose to 1:12800, and a positive complement fixation test titer was 1:640 with Rickettsia mooseri antigens. Neurological examination results, skull roentgenograms, brain scan, electroencephalogram, and the cerebrospinal fluid were all within normal range, thereby excluding intracranial hypertension. After the patient's recovery from the rickettsial disease, the papilledema abated gradually until her fundi reverted to normal.

Adult

Subretinal neovascularization and papilledema associated with pseudotumor cerebri.

A 31-year-old man, on routine ocular examination, was found to have bilateral papilledema. Neurologic evaluation confirmed elevated cerebrospinal pressure with no mass lesion and a diagnosis of pseudotumor cerebri was made. A tiny subretinal hemorrhage adjacent to the right optic disk was found to be secondary to subretinal neovascularization. Over the course of several months, the papilledema resolved. However, the neovascular membrane extended further toward the fovea and was subsequently obliterated with argon laser photocoagulation.

Adult

Computed tomography of the optic nerve: part II. Size and shape modifications in papilledema.

Increase in the caliber and course tortuosity of the optic nerve (ON) can be demonstrated by computed tomography (CT) in cases of papilledema due to intracranial tumors, hydrocephalus, pseudotumor cerebri, and other conditions causing increased intracranial pressure. The enlargement of the ON in these conditions is generally bilateral, although one of the nerves, usually of the side of the tumoral pathology, may be thicker. Enlargement on the ON may also be demonstrated by CT in papilledema due to optic neuritis. Occasionally, the thickened ON also display increased attenuation coefficient values.

Humans

Syphilitic meningitis with papilledema.

Reported is a patient with associated papilledema and syphilitic meningitis. The clinical picture initially suggested an intracranial mass lesion. The cause of the papilledema in this patient was not clear; it did not appear to be related to increased intracranial pressure. Patients with syphilitic meningitis should receive prompt penicillin therapy to avoid serious neurologic sequelae.

Adult

Papilledema associated with a sacral intraspinal cyst.

A rare case of papilledema associated with a large sacral intraspinal cyst is described in a 34-year-old male. Symptoms were aggravated by heavy work and consisted of low back pain, headache, dizziness and episodic vomiting. Papilledema was observed on ophthalmological examination. A valvular mechanism was found to exist between the normal spinal sac and the huge sacral cyst. Division of the valvular fistula combined with a dural plastic operation brought complete relief of all symptoms.

Adult

Cysticercosis: an unusual cause of papilledema.

A case of cerebral cysticercosis with papilledema is presented in a 44-year-old Mexican woman who progressed to optic atrophy despite a ventriculoatrial shunt. The diagnosis was made on testing the cerebral spinal fluid for the indirect hemagglutination response to cysticercosis. The problems of the pork tape-worm as well as several references from the literature are discussed pertaining to ocular and cerebral involvement. Although this disease is not common in the United States we should think of it in cases of papilledema in patients from endemic areas and in those who have traveled in those areas and eaten pork not properly cooked or frozen.

Adult

Asymptomatic unilateral papilledema in pseudotumor cerebri.

A 32-year-old asymptomatic woman was found to have unilateral papilledema on routine ophthalmological examination. Subsequent visual field, neuroimaging, and cerebrospinal fluid examinations were consistent with the diagnosis of pseudotumor cerebri. This case demonstrates that pseudotumor cerebri may present as unilateral papilledema in any asymptomatic patient and illustrates the need for thorough neuro-ophthalmological evaluations to allow early detection of cases with atypical presentations to increase the efficacy of therapeutic intervention and prevent progressive visual loss.

Acetazolamide

[POEMS syndrome and papilledema].

The Crow-Fukase syndrome or POEMS syndrome (Polyneuropathy, Organomegaly, Edema, Monoclonal protein, Skin changes) is an uncommon plasma cell dyscrasia leading to a multisystemic disorder. An optic disc swelling is often related to this syndrome. We report the case of a 51-year-old man with a papilledema. We presume that vasculitis or plasma cell infiltration is the cause of the papilledema.

Adrenal Cortex Hormones

Benign intracranial hypertension without papilledema: case report.

An obese young man presented the typical features of benign intracranial hypertension (BIH), including raised intracranial pressure. Despite elevated intracranial pressure, papilledema did not develop. The syndrome resolved after medical treatment and, on examination 6 months later, the patient was normal. The absence of papilledema in this case is discussed together with data available from the literature.

Adult

The Foster Kennedy sign: a case with papilledema contralateral to a sphenoid wing meningioma but without apparent optic atrophy.

A 28 year old lady with a one year history of mental changes and headaches presented with right fundus normal but with marked papilledema on the left. Subsequent evaluation demonstrated a right sphenoid wing meningioma which was successfully removed as an emergency procedure. The case illustrates the well known clinical dictum that the side of unilateral papilledema offers little information concerning tumor localization.

Adult

[Thoraco-lumbar spinal tumor associated with papilledema (author's transl)].

Increased intracranial pressure and papilledema are occasionally observed in patients harboring spinal tumors in the cervical region or at the craniocervical junction, and the mechanical obstruction to the cerebrospinal fluid circulation is assumed to be responsible for such symptoms and signs. However, increased intracranial pressure is very rare in spinal tumors locating in the dorso-lumbar region; only 44 such cases having been reported in the literature. Recently we saw a 58-year-old female who presented with three brief episodes of loss of consciousness associated with nausea and vomiting, progressive dementia and insomnia. Neurologic examination disclosed an early papilledema, weakness of both legs and dementia. A left carotid angiogram revealed a small aneurysm arising from C2 segment of the internal carotid artery. Right carotid and bilateral vertebral angiograms were not contributory. The aneurysm was clipped at the first operation. The aneurysm was found apparently unruptured. A ventriculoperitoneal shunt failed to improve her dementia. Finally, a total myelographic block was found at L1 level, and a neurinoma arising from the right D12 was removed. After this, all symptoms and signs disappeared within 3 weeks. Pertinent literature on the low spinal cord tumor associated with an intracranial pressure was reviewed and the mechanism of the elevation of intracranial pressure in such cases were discussed.

Diagnosis, Differential