1/1916. Cancer-associated retinopathy in a patient with non-small-cell lung carcinoma. Cancer-associated retinopathy (CAR) is a paraneoplastic syndrome most often associated with small-cell lung carcinoma (SCLC), and it has been reported in patients with other malignancies. Antibodies against recoverin, a 23-kDa protein, have been found in patients with CAR suggesting an autoimmune phenomenon. Herein is the first report of a patient with non-small-cell lung cancer (NSCLC) in whom anti-recoverin antibodies were detected in the serum. Steroid therapy, chemotherapy, and radiation therapy did not help the patient's vision. Progressive loss of vision in patients with lung cancer should, potentially, be tested for CAR. ( info) |
2/1916. vitrectomy in sickling retinopathy: report of five cases. The unique problems encountered in sickle patients include the need to remove peripheral vitreous if perfusing sea fans-which can bleed after vitrectomy-are present at the time of surgery. However, peripheral vitrectomy is riskier than central vitrectomy alone. If visualization of the sea fans is sufficient, it is safer to close the sea fans prior to vitrectomy in order to obviate the need for peripheral vitrectomy; then, only central vitrectomy is performed. When sea fans cannot be closed prior to vitrectomy, peripheral vitreous is removed to allow early photocoagulation of the sea fans before they bleed again. Four case presentations illustrate these principles. vitrectomy relieved severe vitreous traction that complicated retinal detachment in the fifth patient, thereby eliminating the need for a tight encircling structure, which can be poorly tolerated in patients with SC hemoglobin. ( info) |
3/1916. Immune-mediated retinopathy in a patient with stiff-man syndrome. BACKGROUND: Stiff-man syndrome is a rare neurological disorder characterised by rigidity and violent spasms of the body musculature. In the majority of patients, presence of antibodies against glutamic acid decarboxylase (GAD), the enzyme synthesizing gamma-aminobutyric acid (GABA), suggests an autoimmune attack against GABA-ergic inhibitory neurons. We report a 32-year-old patient with stiff-man syndrome and anti-GAD antibodies who developed subacute progressive loss of vision in the right eye, and in the left eye 18 months thereafter. methods: Ophthalmological work-up included electro-retinogram (ERG), visual evoked potentials (VEP) and fluorescein angiography. Antiretinal antibodies were investigated using an indirect immunofluorescence technique on frozen sections of macaque retina with patients serum and FITC-conjugated goat antihuman immunoglobulin. Staining with monoclonal anti-GAD65 antibodies and with serum from three healthy normals served as controls. RESULTS: visual acuity of both eyes decreased to 0.16 within a span of 6 weeks. Perimetry revealed a central scotoma in the visual field of both eyes. VEP and flash ERG were progressively disturbed on the right eye. On the left eye, initially only pattern ERG and photopic responses were abnormal. Follow-up recordings revealed widespread pathology of photopic single and flicker responses. Immunofluorescence revealed strong reactivity of the inner plexiform layer and to a lesser extent staining of the outer plexiform layer at dilutions of 1:1000 with patients serum. The same retinal staining pattern was obtained with monoclonal anti-GAD65 antibodies. CONCLUSIONS: These findings suggest autoimmune retinopathy, mediated by anti-GAD65 autoantibodies as the underlying cause of visual loss. ( info) |
4/1916. Human psychophysical analysis of receptive field-like properties: V. Adaptation of stationary and moving windmill target characteristics to clinical populations. This paper describes the application of several key parameters of a windmill-shaped target (used in determinations of the psychophysical transient-like function) to clinical populations as a diagnostic tool for static perimetry. A technique for independently analyzing sustained- (Westheimer function) and transient-like retinally-based psychophysical responses is outlined, and stimulus characteristics, reliability and diagnostic potential of the tests are examined. Several particularly interesting clinical cases (one closure of a branch retinal arteriole) and two senile macular degeneration patients) are presented for illustrative purposes. In addition, a tentative 'working hypothesis' is presented as a basis for extensive future analysis of various clinical populations. ( info) |
5/1916. Purtscher's retinopathy in acute pancreatitis. A patient who became blind following an episode of alcohol-induced pancreatitis is described. The clinical appearance of this patient's retinal changes corresponds most closely to post-traumatic (Purtscher's) retinopathy. The etiology of the retinopathy is best explained by retinal vascular occlusion due either to fat embolism or thrombosis. ( info) |
6/1916. Pars plana vitrectomy and subretinal surgery for ocular toxocariasis. The clinical course of ocular toxocariasis and the chronological development of peripheral retinal and macular granulomas are reported. Removing the epiretinal as well as subretinal component of the granuloma via pars plana vitrectomy and retinotomy techniques yielded an excellent clinical result. Clinicopathologic correlation of the specimen confirmed the diagnosis with histological evidence of degenerated larval structures in granulomatous inflammation. ( info) |
| PURPOSE: To report an unusual case of familial exudative vitreoretinopathy in an infant. methods: Case report. A 6-day-old girl had unilateral microphthalmia in the right eye, with a retrolental plaque initially diagnosed as persistent hyperplastic primary vitreous. Three months later, peripheral retinal vascular changes and a fibrovascular ridge were noted in the left eye, suggesting familial exudative vitreoretinopathy as the cause in both eyes. RESULTS: The microphthalmic right eye was unsalvageable. The left eye developed an exudative retinal detachment despite photocoagulation of the peripheral avascular retina. Additional cryotherapy resulted in resolution of the detachment and regression of the vascular changes. CONCLUSIONS: With highly asymmetric involvement, neonatal familial exudative vitreoretinopathy can mimic persistent hyperplastic primary vitreous. Fellow eye involvement can progress rapidly. ( info) |
8/1916. Progression of myelinated retinal nerve fibers. PURPOSE: To present a case demonstrating progression of retinal nerve fiber myelin and to suggest that myelinated retinal nerve fibers be considered among the peripapillary choristomas. METHOD: Case report. A 46-year-old woman demonstrated progression of myelinated retinal nerve fibers and associated retinal vascular anomalies. RESULTS: Myelinated retinal nerve fibers can be progressive. We confirm earlier reports of vascular anomalies associated with retinal nerve fiber myelin and show that they can also be progressive. CONCLUSIONS: The rarely progressive nature of myelinated retinal nerve fibers and the histopathologic studies published to date suggest that myelinated retinal nerve fibers represent a striking ophthalmoscopic feature resulting from ectopic oligodendrocytes; thus, the entity may be described as an oligodendrocytic choristoma. ( info) |
9/1916. Extensive brain calcification in two children with bilateral Coats' disease. We report two children with bilateral Coats' disease associated with cerebral calcifications in the basal ganglia and deep white matter, asymptomatic at the time of their discovery. cerebellar ataxia developed secondarily in one of them. Both children were born small for date and had febrile convulsive seizures. Three similar patients have been previously reported, two of them in the same sibship; the third reported patient died of aplastic anemia. Bilateral Coats' disease in children should prompt systematic CT scan in search of cerebral calcifications. If present, neurological and genetic prognosis should be cautious. ( info) |
10/1916. Upward extension of an atrophic tract of the retinal pigment epithelium associated with congenital macular toxoplasmosis. PURPOSE: To report an unusual case of gravitational atrophic tract of the retinal pigment epithelium in a 20-year old woman. methods: Case Report. RESULTS: The patient had macular cicatricial congenital toxoplasmic chorioretinitis in both eyes. In the right eye, an atrophic tract of the retinal pigment epithelium originating from the upper margin of the macular scar extended upwards toward the retinal periphery. CONCLUSION: The unusual upward direction of the atrophic tract of retinal pigment epithelium may be explained by the in utero head position during the active phase of the chorioretinal disease. ( info) |