RESUMEN
PURPOSE: To evaluate the ability of confocal near-infrared reflectance (NIR) to diagnose retrograde microcystic maculopathy (RMM) in eyes with temporal visual field (VF) loss and optic atrophy from chiasmal compression. To compare NIR findings with optical coherence tomography (OCT) findings in the same group of patients. METHODS: Thirty-four eyes (26 patients) with temporal VF loss from chiasmal compression and 41 healthy eyes (22 controls) underwent NIR fundus photography, and macular OCT scanning. VF loss was estimated and retinal layers thickness were measured on OCT. Two examiners blinded to the diagnosis randomly examined NIR images for the presence of hyporeflective abnormality (HA) and OCT scans for the presence of microcystic macular abnormalities (MMA). The total average and hemi-macular HA area and number of microcysts were determined. The groups were compared and the level of agreement was estimated. RESULTS: The OCT-measured macular retinal nerve fiber and ganglion cell layers were thinner and the inner nuclear layer was thicker in patients compared to controls. HA and MMA were detected in 22 and 12 patient eyes, respectively, and in 0 controls (p<0.001, both comparisons). HA was significantly more frequent than MMA in patients with optic atrophy, and agreement between HA and MMA (both total and hemi-macular) was fair (kappa range: 0.24-0.29). The mean HA area was significantly greater in the nasal than temporal hemi-macula. A re-analysis of the 14 eyes with discrepant findings allowed to confirm RMM in 20 eyes (20/34) indicating that OCT detected RMM in 12 and missed it in 8 eyes. On the other hand, NIR correctly detected 18 out of 20 eyes, overcalled 4 and missed 2. CONCLUSIONS: RMM is a frequent finding in eyes with severe VF loss from long-standing chiasmal compression. NIR photography appears to be more sensitive than OCT for detecting RMM and may be useful as screening method for its presence.
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Fondo de Ojo , Degeneración Macular , Síndromes de Compresión Nerviosa , Quiasma Óptico/diagnóstico por imagen , Enfermedades del Nervio Óptico , Tomografía de Coherencia Óptica , Adulto , Técnicas de Diagnóstico Oftalmológico , Femenino , Humanos , Degeneración Macular/diagnóstico por imagen , Degeneración Macular/etiología , Masculino , Persona de Mediana Edad , Síndromes de Compresión Nerviosa/complicaciones , Síndromes de Compresión Nerviosa/diagnóstico por imagen , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/diagnóstico por imagen , Estudios ProspectivosRESUMEN
BACKGROUND: Detection of a relative afferent pupillary defect (RAPD) by the swinging-light test can be challenging in clinical practice (dark eyes, anisocoria, dark environment). We developed a new method of RAPD quantification based on the recording of the infrared pupillary asymmetry (IPA) with a standard optical coherence tomography (OCT) device. METHODS: The diagnostic value of the IPA for detection of the RAPD was determined by receiver-operating characteristic (ROC) curves and area under the curve (AUC). RESULTS: Twenty-nine subjects were included in this study (17 controls and 12 unilateral optic neuropathies). The IPA was significantly greater in unilateral optic neuropathies (0.39) compared to controls (0.18, p = 0.001). The diagnostic value was good with a ROC-AUC of 0.843. Importantly, the IPA correlated significantly with the inter-eye percentage difference of the macular ganglion cell-inner plexiform layer (mGCIPL) thickness (R = 0.53, p = 0.01). Assessment of the IPA took less than 30 s. CONCLUSION: The present data show that the IPA is a practical and rapid test that can be applied in a clinical setting. The IPA may be a valuable functional outcome measure for clinical trials, complementing structural retinal OCT data in a biological meaningful way. The IPA should be further investigated for suitability for optic neuritis treatment trials.
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Técnicas de Diagnóstico Oftalmológico , Trastornos de la Pupila/diagnóstico , Adulto , Área Bajo la Curva , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/diagnóstico , Trastornos de la Pupila/complicaciones , Curva ROC , Reflejo Anormal , Estudios Retrospectivos , Factores de Tiempo , Tomografía de Coherencia ÓpticaAsunto(s)
Esotropía/complicaciones , Enfermedades del Nervio Óptico/complicaciones , Nervio Óptico/anomalías , Vasos Sanguíneos , Niño , Esotropía/diagnóstico , Fondo de Ojo , Humanos , Imagen por Resonancia Magnética , Masculino , Oftalmología , Disco Óptico/fisiopatología , Nervio Óptico/patología , Enfermedades del Nervio Óptico/diagnóstico , Pupila , Visión OcularRESUMEN
PURPOSE: Glaucomatous eyes with disc hemorrhage (DH) have a greater risk of paracentral visual field (VF) loss. However, not every DH eye presents with parafoveal scotoma (PFS), and contributing factors are still to be determined. In the present study, we investigated clinical and ocular factors associated with the presence of PFS in glaucomatous eyes with DH. METHODS: A case-control study was carried out. One hundred thirty glaucomatous patients with DH were enrolled. They were divided into two groups based on two reliable 24-2 VF tests: those with PFS (defined as ≥3 adjacent points with p < 5% within the central 10 degrees of fixation, ≥1 point with p < 1% lying at the innermost paracentral points, in the same hemifield) and those without PFS. Clinical and ocular data from the time of DH detection were compared between groups. Factors associated with the presence of PFS were investigated through logistic regression. RESULTS: The PFS group had a higher prevalence of Caucasian patients (82 vs. 47%; p < 0.01). Eyes with PFS had a more negative spherical equivalent and worse VF mean deviation (MD) index (p ≤ 0.01). There was a marginally significant intraocular pressure (IOP) difference between eyes with (15 mmHg) and without PFS (18 mmHg) at the time of DH detection (p = 0.10). Univariable analysis revealed PFS to be significantly associated with Caucasian race (OR, 3.02; p = 0.004), myopia (<-3 diopters; OR, 3.44; p = 0.039), and lower IOP (≤16 mmHg; OR, 2.10; p ≤ 0.047). Multivariable analysis, controlling for VF MD, revealed that only Caucasian race and myopia (as a continuous or categorical variable) remained significant in this model (p ≤ 0.038). CONCLUSIONS: Caucasian race and the presence and magnitude of myopia were found to be significantly associated with the presence of PFS in glaucomatous eyes with DH. Our results may help clinicians in the identification and surveillance of these eyes at higher risk of central VF loss.
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Glaucoma/complicaciones , Enfermedades del Nervio Óptico/complicaciones , Hemorragia Retiniana/complicaciones , Escotoma/etiología , Adulto , Anciano , Estudios de Casos y Controles , Etnicidad/estadística & datos numéricos , Femenino , Humanos , Presión Intraocular/fisiología , Modelos Logísticos , Masculino , Persona de Mediana Edad , Factores de Riesgo , Escotoma/fisiopatología , Pruebas del Campo Visual , Campos Visuales/fisiologíaRESUMEN
Resumo Nós descrevemos uma rara associação entre estafiloma peripapilar congênito e drusa de disco óptico em uma mulher de 47 anos de idade e visão normal.
Abstract We described a rare association between peripapillary staphyloma and optic disk drusen in a woman with 47 years old and normal vision.
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Humanos , Femenino , Persona de Mediana Edad , Disco Óptico/anomalías , Drusas del Disco Óptico/etiología , Anomalías del Ojo/complicaciones , Informes de Casos , Angiografía con Fluoresceína , Agudeza Visual , Drusas del Disco Óptico/diagnóstico por imagen , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/congénito , Ultrasonografía , Pruebas del Campo VisualRESUMEN
PURPOSE: We measured macular inner retinal layer thicknesses using frequency-domain optical coherence tomography (fd-OCT) and correlated these measures with visual field (VF) in eyes with temporal hemianopia from chiasmal compression and band atrophy (BA) of the optic nerve. METHODS: Macular fd-OCT scans and VFs were obtained from 33 eyes of 33 patients with temporal hemianopia and 36 control eyes. The macular retinal nerve fiber layer (mRNFL), combined retinal ganglion cell and inner plexiform layers (RGCL+), and the inner nuclear layer (INL) were segmented. Measurements were averaged for each macula quadrant. Scans were assessed qualitatively for microcysts in the INL. The VF was estimated from the central 16 test points. The two groups were compared. Correlations between VF and OCT measurements were assessed. RESULTS: The mRNFL, RGCL+, and total retinal (TR) macular thickness measurements were significantly smaller in BA eyes than controls. In the nasal quadrants, INL measurements were significantly greater in BA eyes than controls. The mRNFL and RGCL+ measurements had greater discrimination ability than TR measurements in the temporal quadrants. A significant correlation was found between most OCT parameters and their corresponding VF parameters. The strongest association was observed between RNFL and RGCL+ thickness, and VF loss in the corresponding area. The INL microcysts were found in seven eyes with BA, but not in controls. CONCLUSIONS: Band atrophy leads to mRNFL and RGCL+ thinning, and INL thickening, and mRNFL and RGCL+ measurements are correlated strongly with VF loss. Segmented macular thickness measurements may be useful for quantifying neuronal loss in chiasmal compression.
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Hemianopsia/diagnóstico , Mácula Lútea/patología , Quiasma Óptico/patología , Enfermedades del Nervio Óptico/diagnóstico , Células Ganglionares de la Retina/patología , Tomografía de Coherencia Óptica/métodos , Campos Visuales/fisiología , Adulto , Constricción Patológica , Estudios Transversales , Femenino , Estudios de Seguimiento , Hemianopsia/etiología , Hemianopsia/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/fisiopatología , Estudios Prospectivos , Pruebas del Campo Visual , Adulto JovenRESUMEN
PURPOSE: To evaluate 5 patients with serous macular detachment due to optic disc pit that were submitted to pars plana vitrectomy and were followed for at least 7 years. METHODS: Patients were submitted to pars plana vitrectomy, posterior hyaloid removal, autologous serum injection and gas-fluid exchange, without laser photocoagulation, and were evaluated pre and post-operatively with visual acuity and Amsler grid testing, retinography, and recently, with autofluorescence imaging and high resolution OCT. RESULTS: All 5 eyes improved visual acuity significantly following the surgical procedure maintaining good vision throughout the follow-up period. Mean pre-operative visual acuity was 20/400 and final visual acuity was 20/27 with a mean follow-up time of 13.6 years. No recurrences of serous detachments were observed. OCT examinations demonstrated an attached retina up to the margin of the pit. CONCLUSION: Serous macular detachments due to optic disc pits were adequately treated with pars plana vitrectomy and gas fluid exchange, without the need for laser photocoagulation, maintaining excellent visual results for a long period of time.
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Mácula Lútea/cirugía , Disco Óptico/anomalías , Enfermedades del Nervio Óptico/complicaciones , Desprendimiento de Retina/cirugía , Vitrectomía/métodos , Adolescente , Adulto , Femenino , Humanos , Masculino , Desprendimiento de Retina/etiología , Factores de Tiempo , Tomografía de Coherencia Óptica , Resultado del Tratamiento , Agudeza Visual , Adulto JovenRESUMEN
OBJETIVO: Avaliar 5 olhos com descolamento seroso da mácula devido à fosseta de disco óptico que foram submetidos à vitrectomia via pars plana e seguidos por pelo menos 7 anos. MÉTODOS: Os pacientes foram submetidos à vitrectomia via pars plana, remoção da membrana hialoide posterior, injeção de soro autólogo e troca fluido-gasosa, sem aplicação de fotocoagulação a laser, e foram testados quanto à acuidade visual, tela de Amsler, retinografia e, recentemente, retinografia com autofluorescência e OCT de alta resolução. RESULTADOS: Todos os 5 olhos operados tiveram significativa melhora da visão após o procedimento cirúrgico, mantendo boa visão durante todo período de acompanhamento. A acuidade visual pré-operatoria média foi de 20/400 enquanto a acuidade visual final foi de 20/27 com um tempo médio de seguimento de 13,6 anos. Não foram observadas recorrências do descolamento seroso da mácula e os exames de OCT mostraram a retina perfeitamente aplicada até a margem da fosseta de disco óptico. CONCLUSÃO: Descolamentos serosos da mácula causados por fosseta de disco óptico são adequadamente tratados com vitrectomia via pars plana e troca fluido-gasosa, sem a necessidade de fotocoagulação da retina, mantendo excelente acuidade visual por vários anos após o procedimento, sem o aparecimento de recorrências.
PURPOSE: To evaluate 5 patients with serous macular detachment due to optic disc pit that were submitted to pars plana vitrectomy and were followed for at least 7 years. METHODS: Patients were submitted to pars plana vitrectomy, posterior hyaloid removal, autologous serum injection and gas-fluid exchange, without laser photocoagulation, and were evaluated pre and post-operatively with visual acuity and Amsler grid testing, retinography, and recently, with autofluorescence imaging and high resolution OCT. RESULTS: All 5 eyes improved visual acuity significantly following the surgical procedure maintaining good vision throughout the follow-up period. Mean pre-operative visual acuity was 20/400 and final visual acuity was 20/27 with a mean follow-up time of 13.6 years. No recurrences of serous detachments were observed. OCT examinations demonstrated an attached retina up to the margin of the pit. CONCLUSION: Serous macular detachments due to optic disc pits were adequately treated with pars plana vitrectomy and gas fluid exchange, without the need for laser photocoagulation, maintaining excellent visual results for a long period of time.
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Adolescente , Adulto , Femenino , Humanos , Masculino , Adulto Joven , Mácula Lútea/cirugía , Disco Óptico/anomalías , Enfermedades del Nervio Óptico/complicaciones , Desprendimiento de Retina/cirugía , Vitrectomía/métodos , Desprendimiento de Retina/etiología , Factores de Tiempo , Tomografía de Coherencia Óptica , Resultado del Tratamiento , Agudeza VisualRESUMEN
PURPOSE: To evaluate the correlation between multifocal pattern electroretinography (mfPERG) and Fourier-domain optical coherence tomography (FD-OCT) with regard to macular and retinal nerve fiber layer (RNFL) thickness in eyes with temporal hemianopia from chiasmal compression. METHODS: Twenty-five eyes from 25 patients with permanent temporal visual field defects from chiasmal compression and 25 healthy eyes were submitted to mfPERG using a stimulus pattern of 19 rectangles, standard automated perimetry and FD-OCT measurements. The mfPERG response was determined for groups of three rectangles for the nasal and temporal hemifields and for each quadrant. Macular thickness measurements were registered according to an overlaid OCT-generated checkerboard with 36 checks and averaged for the central area, and for each scanned quadrant and hemifield. RNFL thickness was determined for all twelve 30-degree segments around the disc, and averaged for the segments corresponding to the 6, 7, 8, 9, 10, 11 and 12 o'clock position. Correlations were verified with Pearson's correlation coefficients and linear regression analysis. RESULTS: Both mfPERG amplitudes and OCT measurements were significantly smaller in eyes with temporal visual field defects than in normals. A significant and strong correlation was found between most mfPERG and macular or RNFL thickness OCT parameters. CONCLUSIONS: mfPERG amplitudes and OCT measurements are significantly correlated in patients with chiasmal compression. Both technologies can quantify neuronal loss and, if used in combination, may help clarify structure-function relationships in this patient population.
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Electrorretinografía , Hemianopsia/fisiopatología , Síndromes de Compresión Nerviosa/fisiopatología , Quiasma Óptico/fisiopatología , Enfermedades del Nervio Óptico/fisiopatología , Células Ganglionares de la Retina/patología , Tomografía de Coherencia Óptica , Adenoma/patología , Adenoma/cirugía , Adulto , Estudios Transversales , Femenino , Análisis de Fourier , Hemianopsia/etiología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Síndromes de Compresión Nerviosa/complicaciones , Fibras Nerviosas/patología , Enfermedades del Nervio Óptico/complicaciones , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/cirugía , Estudios Prospectivos , Estadística como Asunto , Pruebas del Campo Visual , Campos VisualesRESUMEN
INTRODUCTION: Non-Hodgkin lymphomas (NHLs) constitute a group of heterogeneous diseases that can arise in lymphatic nodal or extranodal sites. Ocular lymphomas account for 1% of all NHLs. Tumor of the orbit, which can lead to compression of the optic nerve, is the most frequent presentation of the disease. Primary infiltration of the optic nerve and its sheath remains exceptional. OBSERVATION: We report the case of a 51-year-old female patient treated for a NHL. While she was considered to be in remission after four courses of chemotherapy, she presented a right visual loss with hand motion acuity. Her examination revealed a right afferent pupillary defect. Brain MRI emphasized an infiltration of her right optic nerve with no other orbit abnormality. Cerebrospinal fluid analysis showed lymphomatous meningitis. She was then considered to have lymphomatous optic neuropathy (LON). Despite initial improvement of the visual acuity with treatment, the patient died of bone marrow aplasia 6 weeks later. CONCLUSION: LON can be suspected in a painful and sudden visual loss in a context of neoplasia. The diagnosis is confirmed by MRI and cerebrospinal fluid analysis. LON may occur as the sole ocular manifestation of disease recurrence in a patient with systemic NHL, otherwise thought to be in clinical remission.
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Neoplasias del Sistema Nervioso Central/secundario , Linfoma no Hodgkin/complicaciones , Linfoma no Hodgkin/patología , Neoplasias Meníngeas/secundario , Enfermedades del Nervio Óptico/etiología , Neoplasias del Sistema Nervioso Central/complicaciones , Neoplasias del Sistema Nervioso Central/diagnóstico , Femenino , Humanos , Neoplasias Meníngeas/complicaciones , Neoplasias Meníngeas/diagnóstico , Persona de Mediana Edad , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/diagnóstico , Neuritis Óptica/diagnóstico , Neuritis Óptica/etiología , Agudeza VisualRESUMEN
La pérdida transitoria de la visión monocular (PTVM), es un síntoma alarmante relacionado frecuentemente con alteraciones vasculares retinales y puede tener consecuencias importantes desde el punto de vista ocular y vital. Es por ello, que se requiere de un manejo precoz y adecuado. El presente artículo tiene por objeto revisar las principales causas, su presentación, diagnóstico y manejo, como enfermedades oclusivas vasculares y alteraciones el nervio óptico entre otras
Transient monocular vision loss (TMVL) is an alarming symptom often in relation with retinal vascular anomalies that may have severe consequences for vision and life, so it should be evaluated urgently and a prompt approach is needed. This article will review the main causes, its presentation, diagnosis and management such as vascular occlusive diseases and optic nerve abnormalities among others
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Humanos , Trastornos de la Visión/diagnóstico , Trastornos de la Visión/etiología , Trastornos de la Visión/terapia , Visión Monocular , Ataque Isquémico Transitorio/complicaciones , Enfermedades del Nervio Óptico/complicaciones , Amaurosis Fugax/diagnóstico , Amaurosis Fugax/etiología , Amaurosis Fugax/terapia , Isquemia/complicaciones , Trastornos Migrañosos/complicacionesRESUMEN
BACKGROUND: Cryptococcosis is one of the most frequent and severe AIDS defining illnesses. AIMS: We present a patient with advanced HIV/AIDS disease and a diffuse meningoencephalitis due to Cryptococcus neoformans. The patient developed an acute and bilateral blindness associated with high cerebrospinal fluid pressure and optic neuropathy. METHODS: Post-mortem anatomopathologic study revealed a high number of Cryptococcus in the central nervous system, including the optic nerves and the optic chiasm. CONCLUSION: The patient's sudden visual loss appeared to be related to the perineuritic arachnoiditis and the massive invasion of the optic nerves by the fungus.
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Síndrome de Inmunodeficiencia Adquirida/complicaciones , Ceguera/etiología , Meningitis Criptocócica/complicaciones , Enfermedades del Nervio Óptico/complicaciones , Adulto , Humanos , MasculinoRESUMEN
PURPOSE: To evaluate the relationship between pattern electroretinogram (PERG) amplitude, macular and retinal nerve fiber layer (RNFL) thickness by optical coherence tomography (OCT), and visual field (VF) loss on standard automated perimetry (SAP) in eyes with temporal hemianopia from chiasmal compression. METHODS: Forty-one eyes from 41 patients with permanent temporal VF defects from chiasmal compression and 41 healthy subjects underwent transient full-field and hemifield (temporal or nasal) stimulation PERG, SAP and time domain-OCT macular and RNFL thickness measurements. Comparisons were made using Student's t-test. Deviation from normal VF sensitivity for the central 18 degrees of VF was expressed in 1/Lambert units. Correlations between measurements were verified by linear regression analysis. RESULTS: PERG and OCT measurements were significantly lower in eyes with temporal hemianopia than in normal eyes. A significant correlation was found between VF sensitivity loss and full-field or nasal, but not temporal, hemifield PERG amplitude. Likewise a significant correlation was found between VF sensitivity loss and most OCT parameters. No significant correlation was observed between OCT and PERG parameters, except for nasal hemifield amplitude. A significant correlation was observed between several macular and RNFL thickness parameters. CONCLUSIONS: In patients with chiasmal compression, PERG amplitude and OCT thickness measurements were significant related to VF loss, but not to each other. OCT and PERG quantify neuronal loss differently, but both technologies are useful in understanding structure-function relationship in patients with chiasmal compression. (ClinicalTrials.gov number, NCT00553761).
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Electrorretinografía , Hemianopsia/diagnóstico , Síndromes de Compresión Nerviosa/diagnóstico , Quiasma Óptico/patología , Enfermedades del Nervio Óptico/diagnóstico , Tomografía de Coherencia Óptica , Pruebas del Campo Visual , Adulto , Anciano , Axones/patología , Femenino , Hemianopsia/etiología , Humanos , Masculino , Persona de Mediana Edad , Síndromes de Compresión Nerviosa/complicaciones , Disco Óptico/patología , Enfermedades del Nervio Óptico/complicaciones , Células Ganglionares de la Retina/patología , Escotoma/diagnóstico , Campos Visuales , Adulto JovenRESUMEN
PURPOSE: To determine causes of visual impairment in children at the Low Vision Service of the Ophthalmic Clinic at the University of São Paulo and at the Brazilian Association for the Visually Impaired People (Laramara), located in São Paulo, Brazil. PATIENTS AND METHODS: This study evaluated 3,210 visually impaired children (49% female, 51% male; average age, 5.9 years). Visual impairment was present in 57% (visually impaired group) and 43% presented another associated disability (multiple disability group). RESULTS: The main causes of visual impairment in the visually impaired group were toxoplasmic macular retinochoroiditis (20.7%), retinal dystrophies (12.2%), retinopathy of prematurity (11.8%), ocular malformation (11.6%), congenital glaucoma (10.8%), optic atrophy (9.7%), and congenital cataracts (7.1%). The main causes of visual impairment in the multiple disability group were optic atrophy (37.7%), cortical visual impairment (19.7%), toxoplasmic macular retinochoroiditis (8.6%), retinopathy of prematurity (7.6%), ocular malformation (6.8%), congenital cataracts (6.1%), and degenerative disorders of the retina and macula (4.8%). The retina was the most frequently affected anatomic site in the visually impaired group (49.2%) and the optic nerve in the multiple disability group (39%). CONCLUSION: Primary, secondary, and tertiary prevention efforts for childhood blindness and visual rehabilitation must be considered in Latin America.
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Ceguera Cortical/complicaciones , Catarata/complicaciones , Anomalías del Ojo/complicaciones , Glaucoma/complicaciones , Enfermedades del Nervio Óptico/complicaciones , Enfermedades de la Retina/complicaciones , Baja Visión/etiología , Adolescente , Ceguera Cortical/epidemiología , Brasil/epidemiología , Catarata/congénito , Catarata/epidemiología , Niño , Preescolar , Evaluación de la Discapacidad , Anomalías del Ojo/epidemiología , Femenino , Glaucoma/epidemiología , Humanos , Lactante , Recién Nacido , Masculino , Enfermedades del Nervio Óptico/epidemiología , Prevalencia , Pronóstico , Enfermedades de la Retina/epidemiología , Estudios Retrospectivos , Baja Visión/diagnóstico , Baja Visión/rehabilitaciónRESUMEN
OBJECTIVE: To determine the prevalence of endocrinopathies, neuroradiographical findings, and growth derangements in young children with optic nerve hypoplasia (ONH). STUDY DESIGN: A prospective observational study examined the prevalence of endocrinopathies at study enrollment and growth patterns in children with ONH. Subjects (n = 47, mean +/- SD 15.2 +/- 10.6 months) were followed until 59.0 +/- 6.2 months of age. RESULTS: The prevalence of endocrinopathies was 71.7%: 64.1% of subjects had growth hormone (GH) axis abnormalities, 48.5% hyperprolactinemia, 34.9% hypothyroidism, 17.1% adrenal insufficiency, and 4.3% diabetes insipidus (DI). Endocrinopathies were not associated with ONH laterality, absence of the septum pellucidum, or pituitary abnormalities on neuroimaging. End height standard deviation score (SDS) was similar to start length SDS independent of GH surrogate status. A significant increase in end weight SDS was found for the cohort (p < .001). A body mass index (BMI) >85th percentile was noted in 44.4% of the cohort and in 52.1% of subjects with GH axis abnormalities. Initial hyperprolactinemia was positively associated with increased end BMI SDS (p = .004). CONCLUSIONS: These prospective findings confirm the high prevalence of pituitary endocrinopathies in children with ONH reported in previous retrospective studies. Our data reveal that some of these children maintain normal height velocity despite GH axis abnormalities, and, as a group, they are at high risk for increased BMI.
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Enfermedades del Sistema Endocrino/etiología , Hormona de Crecimiento Humana/metabolismo , Enfermedades del Nervio Óptico/complicaciones , Índice de Masa Corporal , Preescolar , Enfermedades del Sistema Endocrino/epidemiología , Enfermedades del Sistema Endocrino/metabolismo , Femenino , Trastornos del Crecimiento/epidemiología , Trastornos del Crecimiento/etiología , Trastornos del Crecimiento/terapia , Terapia de Reemplazo de Hormonas , Hormona de Crecimiento Humana/sangre , Hormona de Crecimiento Humana/uso terapéutico , Humanos , Lactante , Los Angeles/epidemiología , Masculino , Hipófisis/fisiopatología , Prevalencia , Estudios Prospectivos , Resultado del TratamientoRESUMEN
Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The patient, a 45 year old woman was submitted to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis.
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Meningitis/etiología , Biopsia , Duramadre/patología , Femenino , Gadolinio , Humanos , Hipertrofia , Angiografía por Resonancia Magnética , Meningitis/patología , Persona de Mediana Edad , Tabique Nasal/patología , Enfermedades del Nervio Óptico/complicacionesRESUMEN
En la presente exposición se narra vívidamente, una experiencia personal obtenida durante un viaje a Cuba en mayo de 1993. Durante una semana, formamos parte de una misión multidisciplinaria de expertos enviados por la OMS/OPS para atender un urgente llamado de ayuda de las autoridades de la isla. Se trataba de una severa epidemia de ceguera asociada a otros síntomas neurológicos, que llegó afectar unas cincuenta mil personas, por lo que se consideró "una nueva enfermedad", e inicialmente se dió en llamar "neuritis óptica" y posteriormente redenominada, "neupatía óptica cubana". La situación social prevalente, los factores predisponentes, la situación política agravante, las diversas causas invocadas y descartadas durante los primeros 2 años de su irrupción , la presencia de una etiología viral y el arribo final a un consenso, son detalladamente narradas. El hambre y la miseria fueron la razón etiológica última. Antecedentes de epidemias similares, de origen nutricional casencial, se encontraron a menudo en la literatura internacional destacando la evidencia recogida durante la Segunda Guerra Mundial en campos de concentración japoneses ubicados en zonas tropicales. En la segunda parte de la exposición se discutirán las características nutricionales, clínicas, neurológicas y neuro-oftalmológicas de la "neuropatía óptica cubana"
Asunto(s)
Enfermedades del Nervio Óptico/complicaciones , Acontecimientos que Cambian la Vida , Oftalmología , Cuba , Medicina , VenezuelaRESUMEN
Foi realizado o estudo retrospectivo dos prontuários de 155 pacientes encaminhados para avaliaçäo neuro-oftalmológica, com o objetivo de identificar causas freqüentes de transtornos neuro-oftalmológicos, visando facilitar o diagnóstico diferencial, em serviços de atendimento primário. Foram obtidos dados referentes a hipótese diagnóstica final, doenças associadas e hábitos pessoais. A hipertensäo arterial sistêmica foi o antecedente mórbido pessoal mais freqüente. O etilismo foi o principal hábito pessoal. As principais causas de transtornos neuro-oftalmológicos foram: neuropatia óptica isquêmica näo-arterítica, neuropatia tóxico-carencial por alcoolismo, retinopatia associada à doença sistêmica e/ou simulando doença do nervo óptico, traumatismo crânio-encefálico, alteraçöes decorrentes de tumores cerebrais e anomalias congênitas. Várias causas freqüentes de distúrbios neuro-oftalmológicos podem ser identificadas mediante exame oftalmológico cuidadoso e exames complementares de fácil acesso e custo relativamente baixo.
Asunto(s)
Humanos , Alcoholismo/complicaciones , Traumatismos Craneocerebrales/complicaciones , Enfermedades del Nervio Óptico/complicaciones , Hipertensión/complicaciones , Traumatismos del Nervio Óptico/diagnóstico , Traumatismos del Nervio Óptico/etiología , Estudios RetrospectivosRESUMEN
Nearly 51,000 Cubans were afflicted during an outbreak of an optic neuropathy (ON) and peripheral neuropathy (PN) between 1991 and 1993. We re-examined 14 of 20 affected individuals 16 months after an initial evaluation. The optic features were painless symmetric vision loss with poor visual acuity, color vision loss, central or cecocentral scotoma, optic disc pallor, and nerve fiber layer drop-out. The neurologic symptoms included stocking-glove sensory changes, hearing loss, leg cramps, sensory ataxia, hyperactive or absent reflexes, and complaints of memory loss. Two of 11 ON probands tested harbored Leber's hereditary optic neuropathy (LHON)-associated mitochondrial DNA mutations. All patients had received multivitamin therapy. We performed comparisons using the paired two-tailed t test. On re-examination, 12 of 14 patients demonstrated improvement. One patient remained unchanged. One woman with the nt-3460 mtDNA mutation showed a decline in vision. In patients not harboring mtDNA mutations, overall visual acuity, color vision, and peripheral neuropathy manifestations improved significantly (p < 0.001 for each manifestation). Most of the patients with Cuban ON and PN improved on multivitamin therapy. The significance of the mtDNA mutations is unclear. In the 2 LHON patients, manifestation of the disease may have been precipitated by nutritional deficiency. Patients with poor recovery or further deterioration should be evaluated for other factors, including poor vitamin therapy compliance and alternative diagnoses.