Case 3-2001: unilateral visual loss.
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Biomedical subjects
Publications and source records attributed to J W Gittinger.
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PURPOSE: To describe the retinal histopathology of paraneoplastic retinopathy associated with cutaneous melanoma. METHODS: A 59-year-old man had visual loss attributable to paraneoplastic retinopathy and died of metastatic cutaneous melanoma. His eyes were studied by conventional histopathologic techniques. RESULTS: Histopathologic examination of both eyes disclosed a marked reduction in the density of bipolar neurons in the inner nuclear layer; photoreceptor cell neurons in the outer nuclear layer were normal. Ganglion cells were present, although many showed evidence of transsynaptic atrophy. CONCLUSION: The histopathologic changes observed are consistent with clinical, immunologic, and electrophysiologic data that implicate the bipolar cell as the major site of the paraneoplastic process in cutaneous melanoma-associated retinopathy.
Three patients with renal failure caused by atheroembolism following cardiac vascular procedures had multiple, bilateral retinal cholesterol emboli. Ophthalmoscopy in such patients represents a noninvasive diagnostic technique that is underutilized.
BACKGROUND: The mechanism leading to visual loss in paraneoplastic retinopathy is not known. An autoimmune process has been imputed based on immunologic investigations of several patients and by analogy to certain other paraneoplastic syndromes. METHODS: Two patients with documented small cell carcinoma of the lung who had clinical evidence of paraneoplastic retinopathy are described. Histopathologic examination of the retina from one patient and immunohistochemical staining of human retina with serum from control subjects and both patients were performed. RESULTS: Electroretinograms demonstrated dysfunction of photoreceptors in both patients, with predominant loss of rod function in one patient. Post mortem examination showed patchy loss of photoreceptors of the extramacular retina and relative sparing of cones, findings consistent with the clinical and electrophysiologic test results. Serum from both patients stained the retina in an identical manner, with restriction of the stain to the outer retina. Stain was present over the outer plexiform layer, the outer nuclear layer, and the inner and outer segments of most photoreceptors. A sharp demarcation was present between those areas that did and did not stain. All rod inner and outer segments appeared to stain, and many cone inner segments were not stained. Immunologic tests obtained elsewhere did not show serum antibody to the 23 kD protein. CONCLUSION: These findings support the concept of an autoimmune pathogenesis by showing selectivity of the immune response and correlation between the apparent target of the immune response and the clinical and pathologic findings. The mechanism by which cell loss occurs in the retina is not answered by this study. The absence of antibody to the 23 kD protein does not exclude the diagnosis of paraneoplastic retinopathy.
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A 21-year-old man with known Castleman's disease, a lymphoproliferative disorder with both local and systemic manifestations, presented with decreased vision in the left eye and chromatopsia. He had infiltration of the left disc and choroid with elevation of the retina, multiple bilateral depigmented areas at the level of the choroid or retinal pigment epithelium (RPE) (also thought to represent infiltrates), and left exophthalmos. A serious retinal detachment (RD) evolved, but then responded to radiotherapy, with recovery of good vision. Occult leptomeningeal involvement resolved during a period of observation. There was no systemic evidence of malignant lymphoma.
Three young women with papilledema secondary to pseudotumor cerebri evolved mottled macular pigmentation with preservation of normal visual acuity. Choroidal folds or macular star figures were observed. These macular changes could represent the sequelae of either macular edema or a mechanical disruption of the retinal pigment epithelium. The mechanism of choroidal folds in papilledema remains uncertain.
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Disc swelling and hemorrhages appeared in three eyes of two men receiving amiodarone hydrochloride for cardiac arrhythmias. The initial diagnosis was ischemic optic neuropathy, but visual acuity was preserved, and visual field changes were present in only one eye. Amiodarone is a cationic amphiphilic compound, and other drugs with this property have keratopathy, retinopathy, and optic neuropathy as side effects. We believe our patients had an amiodarone-induced papillopathy.
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Four patients, two men 25 and 43 years old and two women 20 and 40 years old, had complete monocular temporal hemianopsias, suggesting that it may be a more common functional deficit than has been recognized. The initial symptoms were visual loss and headache, and eye pain also occurred. Correct diagnosis is possible if the absence of a relative afferent pupillary defect and the persistence of a hemianopsia on binocular testing are demonstrated.
Trauma in an 8-year-old Black-Vietnamese boy caused a medial wall blow-out fracture, which presented as an acquired retraction syndrome. Computed tomography demonstrated both the fracture and entrapment of the medial rectus and surrounding tissues. Surgical relief of the entrapment resulted in improvement of ocular motility, but retraction persisted. This is the sixth young black male reported with a medial wall blow-out fracture, which suggests that this group may have an orbital structure vulnerable to this relatively uncommon fracture.
A 44-year-old man developed a complete homonymous hemianopia 2 days after undergoing chiropractic cervical manipulation. Thromboembolism from the vertebrobasilar circulation--as the consequence of trauma to the vertebral arteries by adjacent bones, muscles, and ligaments during twisting and extension of the neck--is the probable mechanism for occipital infarction and other strokes in this and previous cases. Other neuroophthalmic manifestations reported include Horner's syndrome and sixth nerve and gaze palsies.
The one-and-a-half syndrome, produced by a lesion in the tegmentum at the level of the pons, rarely has been reported as the result of a solitary metastasis. We report a case of adenocarcinoma of the lung metastatic to the pontine tegmentum with clinical, radiological, and pathological confirmation of the anatomy of this syndrome.
Ischemic optic neuropathy developed in five eyes of four patients with optic nerve drusen. Two of the patients were in their 20s, a decade when idiopathic ischemic optic neuropathy is rare. This argues against a chance concurrence of drusen and idiopathic ischemic optic neuropathy. Infarction of the distal portion of the optic nerve in patients with drusen may result from mechanical distortion of blood vessels in the laminar and prelaminar regions.
Cavernous sinus syndrome due to a pituitary tumor is quite rare. Our patient had a large prolacinoma that extended superiorly, causing bitemporal visual field defects, and laterally into the right cavernous sinus, causing tearing, dysesthesia in the distribution of the fifth cranial nerve, a loss of the corneal reflex, and a sixth cranial nerve palsy. Prolactin levels ranged between 800 and 1000 ng/mL. Treatment with 7.5 mg of bromocriptine daily caused a marked reduction in the size of the tumor and resolution of her visual field defects and cranial nerve dysfunction over 6 months. We feel that bromocriptine should be considered as initial therapy for patients with prolactinomas extending into the middle or posterior cranial fossae.