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At least 523 records · Page 29Linked to original sources

Methyl bromide optic atrophy.

A 32-year-old fumigation assistant developed systemic and neuro-ophthalmic manifestations of methyl bromide poisoning, including increased serum bromide level (6.6 mg/100 ml), paresthesias and burning dysesthesia on his hands and feet, and visual impairment. Ocular examination showed mild bilateral decrease in vision, temporal optic nerve head pallor, severely attenuated visual-evoked response amplitudes and normal latencies, a normal electroretinogram, an abnormal electrooculogram, and a severe deuteranomalous (green) defect on Farnsworth-Munsell 100-hue testing. His vision had not improved 12 months after the initial exposure.

Adult↗

Conjunctival necrosis after administration of topical fortified aminoglycosides.

We treated 11 episodes of bulbar conjunctival necrosis that occurred in ten patients after therapy for suppurative keratitis with topical fortified aminoglycosides. Chemosis and mucous discharge preceded the development of an area of conjunctival pallor, which stained with fluorescein and was 5 to 10 mm from the corneoscleral limbus. Typical lesions developed in the inferior bulbar conjunctiva after a mean of 4.8 days and 112 mg of gentamicin sulfate (109 drops). The fortified aminoglycoside was the only agent common to all cases. The conjunctival defects healed completely between five and 13 days after treatment was modified to reduce or eliminate aminoglycoside exposure.

Administration, Topical↗

Loss of electroretinographic oscillatory potentials, optic atrophy, and dysplasia in congenital stationary night blindness.

New criteria for diagnosing congenital stationary night blindness include loss of the oscillatory potentials in the photopic and bright-flash dark-adapted electroretinogram, and atrophy or dysplastic changes, or both, in the optic nerve head. Ten patients (seven male and three female, ranging in age from 6 to 19 years) had typical findings of congenital stationary night blindness including congenital nonprogressive nyctalopia, no pigmentary retinopathy, and full visual fields consistent with myopia. Visual acuities ranged from 20/30 to 20/60, though one patient had a visual acuity of 20/200. Most patients had histories of strabismus. The photopic electroretinograms were subnormal. Of the male patients, five had tilted optic disks with temporal portions of the nerve missing, and two had misshapen nerve heads. The three female patients had pallor of the optic disk without evidence of tilt.

Adolescent↗

A clinicopathologic study of optic neuropathies associated with intracranial mass lesions with quantification of remaining axons.

We examined three patients with intracranial mass lesions that damaged the anterior visual pathway and studied this damage histologically after the patients died. Estimation of the number of surviving axons in the six optic nerves generally showed the greatest atrophy to be in the temporal sector in cross sections immediately behind the globe. More posterior to the globe, atrophy appeared greatest in the center of several nerves. This pattern of damage may result from particular vulnerability of macular axons to damage along the anterior visual pathway. Optic disk pallor was present in two eyes that had less than 40% of the normal number of axons remaining, while one optic disk appeared to be normal with 70% of the axons intact. Afferent pupillary defects were observed in two patients who had two to three times as many remaining axons in the contralateral optic nerve as in the nerve on the side of the defect.

Adenoma↗

Autosomal dominant Stargardt-like macular dystrophy: I. Clinical characterization, longitudinal follow-up, and evidence for a common ancestry in families linked to chromosome 6q14.

PURPOSE: Characterize the phenotype of autosomal dominant Stargardt-like macular dystrophy in two families linked to chromosome 6q14 and determine whether they share a common ancestry. METHODS: Two families spanning 10 generations were identified and studied independently. Participating members were examined and genetic linkage and genotyping performed. RESULTS: Presenting symptoms included decreased vision, hemeralopia, and mild photophobia. The subjective onset of visual loss ranged from age 3 to 50 with a mean of 14 years. A Snellen acuity of 20/200 occurred at a mean age of 22 years. Over decades, the macular lesion enlarged and visual acuity decreased to 20/300 to 20/800. The typical phenotype was well-circumscribed, homogenous atrophy of the retinal pigment epithelium and choriocapillaris in the macula, with surrounding yellow flecks and temporal optic nerve pallor. The phenotypic spectrum included a pattern dystrophy-like appearance, diffuse geographic atrophy, and extensive fundus flecks. Genotyping revealed that the two families were linked to chromosome 6q14 and shared a common haplotype spanning 21 cM between D6S430 and D6S300. The two families were subsequently shown by genealogic investigation to represent different branches of a common kindred. CONCLUSIONS: Families with autosomal dominant Stargardt-like macular dystrophy linked to chromosome 6q14 share a common phenotype and in some cases can be distinguished from similar dystrophies by inheritance pattern and clinical features. The finding that these two families shared a common ancestor suggests the existence of a founder effect. Characterization of the gene for autosomal dominant Stargardt-like macular dystrophy may enable better understanding of this condition and elucidation of its potential role in other forms of macular degeneration.

Adolescent↗

Bilateral posterior ischemic optic neuropathy after spinal surgery.

PURPOSE: To report the association between bilateral posterior ischemic optic neuropathy and spinal surgery. METHOD: Case report. RESULTS: After prone-position spinal surgery of 8 hours' duration, a 68-year-old woman was completely blind in both eyes. Moderate periorbital edema and temporal conjunctival chemosis were present bilaterally. Ophthalmic examination disclosed normal-appearing optic nerve heads, except for bilateral nasal fullness related to bilateral optic nerve drusen, and no retinal edema. Immediate cerebral arteriography, magnetic resonance imaging, and electroretinography were normal. Visual-evoked response was not detectable, and 7 weeks later, severe bilateral optic nerve head pallor developed. CONCLUSIONS: Severe selective hypoperfusion of the retrobulbar optic nerves may occur after spinal surgery. Pressure to the periorbital region may be a contributing factor.

Aged↗

Topless optic disk syndrome without maternal diabetes mellitus.

PURPOSE: To describe four cases of topless optic disk syndrome without maternal diabetes mellitus. METHOD: Four patients had incidentally discovered inferior visual field defects. RESULTS: Ophthalmoscopic examinations in all four patients disclosed superiorly displaced entrances of the central retinal artery and thinning of the superior peripapillary nerve fiber layers. One patient had a superior peripapillary crescent with pallor of the superior disk. These clinical findings were consistent with a diagnosis of superior segmental optic hypoplasia, the topless disk. None of the patients had mothers who had diabetes. CONCLUSIONS: The topless optic disk syndrome can occur in the absence of maternal diabetes mellitus.

Adult↗

Morbidity after procurement of radial arteries in diabetic patients and the elderly undergoing coronary revascularization.

BACKGROUND: The use of radial arteries for coronary revascularization is increasing. There remain concerns regarding alteration of upper extremity function after radial artery procurement. This study evaluates the functional morbidity in higher risk patients. METHODS: Between April 1997 and September 1999, 374 patients underwent unilateral or bilateral radial artery procurement. A questionnaire was used to evaluate symptoms related to motor and sensory function and changes in appearance after radial artery harvest. RESULTS: Two hundred eighty-nine patients were successfully interviewed. The average age was 63 years. Median follow-up was 9.5 months (range, 2 to 23 months). No patient suffered limb loss. Altered gross and fine motor function, residual pain, paresthesias, numbness, pallor, swelling, and altered temperature sensation were compared among diabetic patients, patients older than 70 years, and patients without these characteristics. CONCLUSIONS: Radial artery procurement for elective coronary revascularization can be done with minimal serious morbidity in higher risk patients. The most common symptoms were numbness and paresthesia. Despite the finding of greater residual pain in diabetic patients, we do not believe the use of radial artery conduits is contraindicated in these patients.

Adult↗

Increased blood-brain barrier permeability in LP-BM5 infected mice is mediated by neuroexcitatory mechanisms.

Serum protein levels in LP-BM5 infected mouse brains were investigated to gain insight into the contribution of blood-brain barrier (BBB) patency to the pathogenesis of retroviral encephalopathy. Evans blue uptake by the forebrain and cerebellum was significantly increased between 8-12 weeks post inoculation. Immunohistochemistry revealed foci of albumin, transferrin, alpha(2)-macroglobulin and IgG transudation around blood vessels particularly in the cerebral cortex and cerebellar vermis. These leaks were often associated with astrocytosis and apoptotic cells. Unlike the other serum proteins, IgG immunoreactivity extended from the circumventricular organs and disseminated throughout the brain parenchyma, accumulating on the plasma membranes of hippocampal and cortical neurons. Consistent with the chronic elevation of free glutamate levels in LP-BM5 infected mice, the increase in Evans blue uptake into the forebrain was completely reversed following dizocilpine administration. Thus, the chronic increase in free glutamate levels in LP-BM5 infected mouse brain contributes to BBB disruption. Furthermore, the CNS accumulation of serum proteins, particularly IgG, observed in these mice may increase osmotic load, impair neuronal function, and cause white matter pallor. Administration of NMDA receptor antagonists may prove useful in managing BBB permeability in those neuropathologies, such as HIV-associated dementia/cognitive/motor complex, having a glutamatergic component.

Animals↗

"Cherry-red spot" or "perifoveal white patch"?

The color of the normal fovea, red in Caucasians, contrasts with the surrounding retina, whose colour is altered by accumulation of metabolic products, causing retinal pallor. In this photo-essay, we present the fundus photographs of 3 patients of different race, all with metabolic disease, each of whose fovea contrasts with the surrounding retina, and for whom the expression "cherry red spot" is not necessarily accurate. We suggest that the term "perifoveal white patch" describes this characteristic finding more accurately. We also discuss the pathological entities that may produce this striking appearance, along with its histological and anatomical basis.

Child↗

Prognostic DNA testing and counselling for dominant optic atrophy due to a novel OPA1 mutation.

CASE REPORT: To report the case of a 26-year-old woman with a family history of dominant optic atrophy who requested DNA testing and counselling. Ophthalmologic examination showed her affected father had bilateral temporal papillary pallor. Direct genomic sequencing of the OPA1 gene revealed a novel heterozygous nonsense mutation (Arg879stop). Because no mutation in OPA1 was detected in the daughter, we could counsel her that the possibility was very low that she was a carrier or would pass the disease-causing gene to her children. COMMENTS: Our study provides evidence of the apparent value of molecular genetic analysis of OPA1 gene as predictive DNA testing, although the exact risk and benefit of this type of analysis awaits further study.

Adult↗

The 1994 Von Sallman Lecture. The optic nerve head circulation in health and disease.

This is a brief overview of multifaceted anatomical, experimental and clinical studies conducted by the author since 1955 on the optic nerve head circulation in health and disease. Conclusions, based on the accumulated information provided by these studies, are summarized. The studies on the pattern of blood supply of the optic nerve head have shown that: (a) its main source of blood supply is the posterior ciliary artery circulation, with retinal circulation supplying only the surface nerve fiber layer, (b) there is marked interindividual variation in the blood supply pattern, and (c) the blood supply in the optic nerve head has a sectorial distribution. The various factors which produce interindividual variation in the blood supply of the optic nerve head are discussed, particularly those in the posterior ciliary artery circulation; this is because all available evidence indicates that it is derangement in the posterior ciliary circulation in the optic nerve head that is primarily responsible for the common ischemic disorders of the optic nerve head, e.g. anterior ischemic optic neuropathy and glaucomatous optic neuropathy. Factors that may derange the blood flow in the optic nerve head include defective autoregulation of blood flow in it, vascular changes in its feeding arteries, hematologic abnormalities, systemic arterial hypertension and hypotension, and intraocular pressure; their roles are discussed. For better understanding and management of optic nerve head ischemic disorders, there is an urgent need for an accurate clinical method of assessment of blood flow in the posterior ciliary circulation in optic nerve head, since no satisfactory method is currently available. Redness or pallor of the optic disk on ophthalmoscopy is not a true guide to the optic nerve head vascularity as it gives no information about the state of the posterior ciliary circulation. Fluorescein fundus angiography, though far superior to the optic disk color for evaluation of optic nerve head vascularity, has a number of limitations. All these topics and various controversies about them are discussed briefly.

Animals↗

Colonoscopy in ischemic colitis.

Fifteen patients with ischemia of the colon are presented. The majority showed a similar clinical presentation with hematochezia, abdominal pain, and diarrhea in an elderly patient population having associated disease. Colonoscopy was abnormal in all patients studied. Three endoscopic stages were recognized; (1) acute stage characterized by petechiae, pallor, and hyperemia; (2) subacute stage consisting of ulceration and exudation; and (3) chronic stage characterized by stricture, decrease in haustrations, and mucosal granularity. Conventional barium enemas were abnormal and suggested ischemic colitis in six of 15 patients. Rigid proctoscopy was normal or demonstrated nonspecific proctitis in 12 of 15 patients studied. Colonoscopic biopsies demonstrated superficial inflammatory changes in all patients. Thirteen patients had complete mucosal healing endoscopically in 2 weeks to 3 months with stricture developing in four patients. Because ischemic colitis is a distinct subtype of ischemic bowel disease most often limited to the superficial mucosa, colonoscopy is an alternative and usually safe modality in the diagnosis of this entity and proved more accurate that conventional x-ray and proctoscopy.

Aged↗

A novel mutation of the OPA1 gene in a Japanese family with optic atrophy type 1.

PURPOSE: To report a novel mutation of the OPA1 gene in a Japanese family with optic atrophy type 1 (OPA1) and to describe the clinical features of this family. METHODS: Standard ocular examinations were performed on the proband and his two affected sons. The DNA sequence of all exons and splice sites of the OPA1 gene was determined to detect mutations. RESULTS: The proband and his sons had a heterozygous mutation of the OPA1 gene in the third nucleotide of intron 12 (IVS12+3A-->T). Clinically, each patient had reduced visual acuity (onset within the first 6 years of life) and optic nerve pallor. The proband showed bilateral central scotomas and generalized dyschroatopsia. This is the first report of OPA1 gene mutation in Japanese patients with familial optic atrophy. CONCLUSIONS: A mutation of the OPA1 gene was detected in a Japanese family with OPA1, which follows the same pattern as reported in Western countries. It is suggested that mutations of the OPA1 gene contribute to the development of optic nerve atrophy regardless of ethnic groups. Screening for the OPA1 gene mutation will be useful for diagnosis of OPA1 in Japanese patients.

Adult↗

Histopathology of oesophagogastric lesions in pigs.

The development of parakeratotic lesions and ulcers of the oesophagogastric epithelium was studied in pigs from a variety of sources and of ages ranging from 10 to 22 weeks. In general, the severity, as assessed by gross appearance, paralleled the depth to which parakeratotic change extended into the epithelium. However, some early degenerative change and erosion was observed in epithelium graded as macroscopically normal. Separation and erosion usually occurred beneath a band of epithelial cells with cytoplasmic pallor and nuclear degeneration underlying the parakeratotic layers. These findings are discussed in relation to the possible role of diet and gastric acidity in the pathogenesis of the lesions.

Animals↗

Studies on experimental Jembrana disease in Bali cattle. II. Clinical signs and haematological changes.

The clinical and haematological changes which occurred in 18 Bali cattle (Bos javanicus) experimentally infected with Jembrana disease are described. The major clinical signs were an elevated rectal body temperature persisting for 7 days (range 5 to 12 days), lethargy, anorexia, enlargement of the superficial lymph nodes, a mild ocular and nasal discharge, diarrhoea with blood in the faeces and pallor of the mucous membranes. Not all of these changes occurred in all affected cattle. The major haematological changes included leucopenia, lymphopenia, eosinopenia and a slight neutropenia, a mild thrombocytopenia, a normocytic normochromic anaemia, elevated blood urea concentrations and reduced total plasma protein. The mortality rate in the experimentally infected cattle was 17 per cent. The similarity of Jembrana disease to malignant catarrhal fever and to diseases of cattle associated with Ehrlichia is discussed.

Animals↗

Ileal dysgenesis in infants and children.

Ileal dysgenesis describes the segmental dilatation of the terminal ileum treated in seven patients presenting from birth to 15 yr. Manifestations included saccular segmental ileal dilatation adherent to the peritoneal surface of an omphalocele sac in two neonates, a radiographically delineated enterolith in a child studied for an unrelated disorder, and a syndrome including pallor, fatigue, and anemia in four children aged 10 to 15 yr. Literature review uncovered 11 similar patients to 38 yr of age. Other symptoms in that group included intestinal obstruction and recurrent abdominal pain. At laparotomy all patients were found to have segmental saccular or tubular dilatation of the terminal ileum. The dilated segments varied in length from 8 to 25 cm. Meckel's diverticula were present in two patients. Segmental resection and anastomosis was performed in all patients. No lumenal stenosis was present in any specimen to account for the dilatation. Previous reports have referred to this abnormality as "giant Meckel's diverticulum" or "segmental ileal dilatation." Evaluation of these 18 patients suggests that this entity is an intrinsic abnormality of ileal development at the morphologically active site of juncture with the yolk stalk.

Adolescent↗

Splenic hemorrhage in a neonate of a mother on anticonvulsant therapy.

A 24-hour-old male infant was referred to the Eastern Maine Medical Center (EMMC) because of pallor, lethargy, and abdominal distention. Diagnostic abdominal paracentesis revealed free blood. At surgery hemisplenectomy removed the damaged part of the spleen followed by complete recovery of the infant. A liver-spleen scan, performed before discharge, revealed normal uptake in the remaining portion of the spleen.

Female↗