ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Bilateral optic neuropathy and subacute cerebellar ataxia were manifestations of a paraneoplastic neurologic disorder in a woman found to have small cell carcinoma of the lung. Serologic tests revealed a neuronal autoantibody specific for CRMP-5, a 62-kd member of the collapsin response-mediating protein family. Unexplained optic neuropathy in the setting of subacute cerebellar ataxia should cause suspicion of a paraneoplastic disorder and prompt testing for this autoantibody, especially in patients at risk for lung carcinoma.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectivesTo report a novel antibody associated with paraneoplastic retinopathy and to characterize the retinal autoantigen.MethodsImmunohistochemistry of rat and human tissues was used to identify antiretinal antibodies. Serologic screening of a bovine retinal cDNA expression library was performed to clone the target antigen.ResultsA 72-year-old woman presented with a 6-month history of progressive visual loss, bilateral central scotomas, light flashes, and night blindness. Visual acuity was 20/40 OD and 20/30 OS. There was generalized loss of retinal pigment and narrow arterioles; discs were normal in appearance. The electroretinogram showed no response. Chest computed tomograph scan demonstrated a right lung mass; biopsy revealed poorly differentiated carcinoma. The patients' serum contained antibodies that immunolabeled nuclei of cells of the outer—and to a lesser extent, the inner—nuclear layer of the adult rat retina. No reactivity was identified with nonretinal adult human or rat tissues. Reactivity was seen in the developing rat embryo. Serologic screening of a bovine retinal library resulted in the isolation of three overlapping clones, encoding a protein highly homologous to the human photoreceptor cell-specific nuclear receptor gene product.ConclusionsThe target antigen of an antibody associated with paraneoplastic retinopathy is the photoreceptor cellspecific nuclear receptor, a member of a conserved family of nuclear receptors involved in photoreceptor cell development or maintenance.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo evaluate the signs, symptoms, and immune responses of patients with melanoma-associated retinopathy (MAR) syndrome.Materials and MethodsWe reviewed the clinical and immunologic findings of 62 MAR syndrome patients. They include 25 patients from our institution (11 not previously reported) and 37 patients reported from other institutions.ResultsThere were 33 men and seven women (no gender information is available for the remaining 22 cases). Age at onset of the visual disturbance averaged 57.5 years (range, 30–78). Visual acuity of 20/60 or better was initially present in 82%. Fundus examination was normal in 44%, optic disc pallor was present in 23%, and retinal vessel attenuation was present in 30%. Vitreous cells were present in 30%. The latency from melanoma diagnosis to recognition of MAR syndrome averaged 3.6 years (range, 2 months to 19 years). Seven patients sustained visual improvement with various treatment regimens, especially with intravenous immunoglobulin and cytoreductive surgery (metastasectomy). Indirect immunohistochemical staining of the bipolar layer was typical, but several other retinal elements were also reactive. Tissue from a metastatic melanoma excised from one of the patients expressed antigens that reacted with antiretinal antibodies.ConclusionMAR syndrome demonstrates diverse clinical and immunologic features. Treatment, especially intravenous immunoglobulin and cytoreductive surgery (metastasectomy), improves vision in some cases.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

We conducted an unmasked evaluation of transdermal scopolamine in seven patients with acquired nystagmus for whom other treatments had been unsatisfactory. We measured eye speed and visual acuity before and several hours after starting treatment. Median eye speed decreased slightly in three patients but increased in two; no change in visual acuity occurred in any patient. One patient was unable to tolerate the side effects of scopolamine after two hours, but the others continued the scopolamine treatment for 48 hours; only one reported minor improvement. We conclude that transdermal scopolamine is not likely to be an effective treatment of acquired nystagmus. Patients should be monitored during the first few hours of treatment to determine whether vision is improved or made worse and whether side effects occur.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

PurposeTo report the clinical presentation of acute visual loss in six patients who were ultimately diagnosed with systemic lupus erythematosus (SLE).MethodsRetrospective case series.ResultsAll patients had a positive antinuclear antibody and elevated anti–double stranded DNA titers. Five of six patients demonstrated gadolinium enhancement of the optic nerve and/or chiasm on magnetic resonance imaging (MRI). Most patients showed initial improvement after treatment with high-dose systemic corticosteroids, but five experienced recrudescences during steroid taper, requiring further treatment with immunosuppressive or cytotoxic medications.ConclusionsVisual loss owing to optic neuropathy or chiasmopathy may be the presenting sign of SLE or the event that leads to this diagnosis. Gadolinium-enhanced MRI is useful for identifying anterior visual pathway lesions in these patients. Corticosteroids are effective in the treatment of this condition; however, relapses requiring further treatment are common.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo elucidate the cause of monocular blur or diplopia after reading in downgaze.MethodsCorneal topography was obtained before and after a 15-to 30-minute reading effort in downgaze in three symptomatic patients and in nine asymptomatic control subjects.ResultsChanges in corneal topographic color maps, corneal uniformity index, and predicted corneal acuity were found in the symptomatic patients but not in the control subjects before and after reading.ConclusionChanges in corneal topography can occur after prolonged reading in downgaze and may produce symptoms of blur or monocular diplopia.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

We report a 38-year-old woman with bilateral tonic (Adie's) pupils who was diagnosed as having Vogt-Koyanagi-Harada syndrome. The tonic pupils persisted after other clinical features of this syndrome had disappeared.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

We report a case of relative pupil-sparing oculomotor paresis initially attributed to ischemia because weakness of other cranial nerves was minimal and dismissed as insignificant. Neuroimaging eventually revealed a posterior fossa meningioma. The neurologic symptoms and signs disappeared immediately after resection of the tumor. The third nerve palsy was attributed to deformation of the brainstem. This case reinforces the importance of neuroimaging even in patients who have apparently isolated oculomotor palsy with features not classic for an ischemic etiology.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

A 52-year-old patient developed an eye movement disorder first resembling a left internuclear ophthalmoplegia and subsequently a “one-and-a-half syndrome” as the presenting symptoms of ocular myasthenia gravis. No accompanying myasthenic features were present except for the fluctuation in the amplitude of dissociated nystagmus. This patient shows that an oculomotor disorder considered a typical pontine lesion may instead be caused by myasthenia gravis, even in the absence of other clinical and electrophysiologic features of neuromuscular deficit.

ISSN:1070-8022    

出版商:OVID    

年代:2001

数据来源: OVID