Panhipopituitarismo y escotoma perimacular como presentación de enfermedad relacionada a IgG4 / IgG4 related disease presenting as panhypopituitarism and perimacular scotoma

La enfermedad relacionada a IgG4 (IgG4-RD) constituye una entidad sistémica recientemente descrita, de causa desconocida. Afecta predominantemente a hombres mayores y presenta características histopatológicas distintivas, como fibrosis estoriforme, flebitis obliterante y denso infiltrado linfoplasmocitario con inmunomarcación para IgG4, pudiendo estar asociada a elevación sérica de dicha inmunoglobulina. Si bien cualquier órgano puede estar afectado, el compromiso de la hipófisis es infrecuente. Describimos el caso de un hombre de 36 años que se presentó con cefaleas, alteración del campo visual, panhipopituitarismo, diabetes insípida y una imagen que mostraba una lesión infiltrativa infundíbulo-panhipofisaria extendida. Arribamos al diagnóstico de IgG4-RD a través de biopsia hipofisaria. La respuesta al tratamiento con dosis inmunosupresoras de corticoides fue exitosa.

IgG4-related disease (IgG4-RD) is a recently described systemic entity of unknown origin. It predominantly affects older men and has distinctive histopathologic features as storiform fibrosis, obliterative phlebitis, dense lymphoplasmacytic infiltrate with immunostaining for IgG4, and it may be associated with elevated serum levels of IgG4. Although any organ can be affected, pituitary gland is rarely involved. We describe the case of a 36-year-old man who presented with headaches, impaired vision, panhypopituitarism with diabetes insipidus and an infiltrative lesion mainly of infundibulum and pituitary. We arrived at diagnosis of IgG4-RD by pituitary biopsy. A successful response to treatment with immunosuppressive doses of corticosteroids was achieved.

Acute Zonal Occult Outer Retinopathy (AZOOR) in a patient with Hashimoto's thyroiditis / Retinopatia aguda zonal oculta externa em uma paciente com tireoidite de Hashimoto

ABSTRACT Acute zonal occult outer retinopathy (AZOOR) is a rare disease characterized by an acute damage of one or more external retinal zones leading to the visual field or the visual acuity impairment associated with small or no changes in the fundus examination.The main clinical symptoms are scotomas and the sudden onset of photopsias. Abnormal findings on electroretinography and visual field defects are critical for the diagnosis. Central vision is usually preserved and the stabilization occurs in six months in most cases. The objective of this article is to describe a 24-month follow-up of a patient with AZOOR and correlate the findings with the typical features of this disease.

RESUMO A retinopatia aguda zonal oculta externa (AZOOR) é uma doença rara, caracterizada por um dano agudo de uma ou mais zonas da retina externa que levam ao comprometimento do campo visual ou da acuidade visual, estando associada a pequenas ou nenhumas alterações no exame fundoscópico. Os principais sintomas clínicos são escotomas e o aparecimento súbito de fotopsias. Resultados anormais de defeitos de campo visual e eletrorretinografia são críticos para o diagnóstico. A visão central é geralmente preservada e a estabilização do quadro ocorre em seis meses na maioria dos casos. O objetivo deste artigo é descrever o "follow-up" de 24 meses de uma paciente com AZOOR e correlacionar os achados com as características típicas desta doença.

Comparison of Risk Factors for Initial Central Scotoma versus Initial Peripheral Scotoma in Normal-tension Glaucoma

PURPOSE: To investigate the risk factors for initial central scotoma (ICS) compared with initial peripheral scotoma (IPS) in normal-tension glaucoma (NTG). METHODS: Fifty-six NTG patients (56 eyes) with an ICS and 103 NTG patients (103 eyes) with an IPS were included. Retrospectively, the differences were assessed between the two groups for baseline characteristics, ocular factors, systemic factors, and lifestyle factors. Also, the mean deviation of visual field was compared between the two groups. RESULTS: Patients from both ICS and IPS groups were of similar age, gender, family history of glaucoma, and follow-up periods. Frequency of disc hemorrhage was significantly higher among patients with ICS than in patients with IPS. Moreover, systemic risk factors such as hypotension, migraine, Raynaud's phenomenon, and snoring were more prevalent in the ICS group than in the IPS group. There were no statistical differences in lifestyle risk factors such as smoking or body mass index. Pattern standard deviation was significantly greater in the ICS group than in the IPS group, but the mean deviation was similar between the two groups. CONCLUSIONS: NTG Patients with ICS and IPS have different profiles of risk factors and clinical characteristics. This suggests that the pattern of initial visual field loss may be useful to identify patients at higher risk of central field loss.

Evaluation of Hemifield Sector Analysis Protocol in Multifocal Visual Evoked Potential Objective Perimetry for the Diagnosis and Early Detection of Glaucomatous Field Defects

PURPOSE: Multifocal visual evoked potential (mfVEP) is a newly introduced method used for objective visual field assessment. Several analysis protocols have been tested to identify early visual field losses in glaucoma patients using the mfVEP technique, some were successful in detection of field defects, which were comparable to the standard automated perimetry (SAP) visual field assessment, and others were not very informative and needed more adjustment and research work. In this study we implemented a novel analysis approach and evaluated its validity and whether it could be used effectively for early detection of visual field defects in glaucoma. METHODS: Three groups were tested in this study; normal controls (38 eyes), glaucoma patients (36 eyes) and glaucoma suspect patients (38 eyes). All subjects had a two standard Humphrey field analyzer (HFA) test 24-2 and a single mfVEP test undertaken in one session. Analysis of the mfVEP results was done using the new analysis protocol; the hemifield sector analysis (HSA) protocol. Analysis of the HFA was done using the standard grading system. RESULTS: Analysis of mfVEP results showed that there was a statistically significant difference between the three groups in the mean signal to noise ratio (ANOVA test, p < 0.001 with a 95% confidence interval). The difference between superior and inferior hemispheres in all subjects were statistically significant in the glaucoma patient group in all 11 sectors (t-test, p < 0.001), partially significant in 5 / 11 (t-test, p < 0.01), and no statistical difference in most sectors of the normal group (1 / 11 sectors was significant, t-test, p < 0.9). Sensitivity and specificity of the HSA protocol in detecting glaucoma was 97% and 86%, respectively, and for glaucoma suspect patients the values were 89% and 79%, respectively. CONCLUSIONS: The new HSA protocol used in the mfVEP testing can be applied to detect glaucomatous visual field defects in both glaucoma and glaucoma suspect patients. Using this protocol can provide information about focal visual field differences across the horizontal midline, which can be utilized to differentiate between glaucoma and normal subjects. Sensitivity and specificity of the mfVEP test showed very promising results and correlated with other anatomical changes in glaucoma field loss.

Evaluation of Hemifield Sector Analysis Protocol in Multifocal Visual Evoked Potential Objective Perimetry for the Diagnosis and Early Detection of Glaucomatous Field Defects

PURPOSE: Multifocal visual evoked potential (mfVEP) is a newly introduced method used for objective visual field assessment. Several analysis protocols have been tested to identify early visual field losses in glaucoma patients using the mfVEP technique, some were successful in detection of field defects, which were comparable to the standard automated perimetry (SAP) visual field assessment, and others were not very informative and needed more adjustment and research work. In this study we implemented a novel analysis approach and evaluated its validity and whether it could be used effectively for early detection of visual field defects in glaucoma. METHODS: Three groups were tested in this study; normal controls (38 eyes), glaucoma patients (36 eyes) and glaucoma suspect patients (38 eyes). All subjects had a two standard Humphrey field analyzer (HFA) test 24-2 and a single mfVEP test undertaken in one session. Analysis of the mfVEP results was done using the new analysis protocol; the hemifield sector analysis (HSA) protocol. Analysis of the HFA was done using the standard grading system. RESULTS: Analysis of mfVEP results showed that there was a statistically significant difference between the three groups in the mean signal to noise ratio (ANOVA test, p < 0.001 with a 95% confidence interval). The difference between superior and inferior hemispheres in all subjects were statistically significant in the glaucoma patient group in all 11 sectors (t-test, p < 0.001), partially significant in 5 / 11 (t-test, p < 0.01), and no statistical difference in most sectors of the normal group (1 / 11 sectors was significant, t-test, p < 0.9). Sensitivity and specificity of the HSA protocol in detecting glaucoma was 97% and 86%, respectively, and for glaucoma suspect patients the values were 89% and 79%, respectively. CONCLUSIONS: The new HSA protocol used in the mfVEP testing can be applied to detect glaucomatous visual field defects in both glaucoma and glaucoma suspect patients. Using this protocol can provide information about focal visual field differences across the horizontal midline, which can be utilized to differentiate between glaucoma and normal subjects. Sensitivity and specificity of the mfVEP test showed very promising results and correlated with other anatomical changes in glaucoma field loss.

A Case of Syphilitic Outer Retinitis Mimicking Acute Zonal Occult Outer Retinopathy

No abstract available.

A Congruous Superior Quadrantanopsia Following a Junctional Scotoma Induced by Asperogillosis

A 69-year old man presented to us with decreased vision in his right eye and a relative afferent pupillary defect. Under the presumption that he was suffering from retrobulbar optic neuritis or ischemic optic neuropathy, visual field tests were performed, revealing the presence of a junctional scotoma. Imaging studies revealed tumorous lesions extending from the sphenoid sinus at the right superior orbital fissure, with erosion of the right medial orbital wall and optic canal. Right optic nerve decompression was performed via an endoscopic sphenoidectomy, and histopathologic examination confirmed the presence of aspergillosis. The patient did not receive any postoperative antifungal treatment; however, his vision improved to 20 / 40, and his visual field developed a left congruous superior quadrantanopsia 18 months postoperatively. A junctional scotoma can be caused by aspergillosis, demonstrating the importance of examining the asymptomatic eye when a patient is experiencing a loss of vision in one eye. Furthermore, damage to the distal optic nerve adjacent to the proximal optic chiasm can induce unusual congruous superior quadrantanopsia.

Detecção de perda neural localizada através da redução da espessura macular na tomografia de coerência óptica: relato de caso / Localized neural loss detected by macular thickness reduction using optical coherence tomography: case report

Paciente de 22 anos, sexo masculino, notou mancha central no olho esquerdo durante um episódio da cefaléia de forte intensidade há aproximadamente 6 meses. Referia diagnóstico de enxaqueca com aura, apresentando crises recorrentes desde os 15 anos de idade. O exame oftalmológico revelou perda da camada de fibras nervosas da retina na região do feixe papilomacular do olho esquerdo. A tomografia de coerência óptica demonstrou importante redução da espessura macular, acompanhada de redução localizada da camada de fibras nervosas da retina no setor temporal do disco óptico no olho esquerdo. Avaliação sistêmica, exames de imagem e avaliação cardiovascular nada revelaram e a causa da perda da perda visual foi atribuída a possível enxaqueca retiniana, embora um evento isquêmico de outra natureza não pudesse ser descartado. Nosso caso é interessante porque demonstra que a tomografia de coerência óptica pode ser útil na detecção de perda neural localizada não apenas pela medida da camada de fibras nervosas da retina, mas também pela avaliação da espessura macular.

A 22-year-old man developed a central scotoma in the left eye during an episode of severe headache 6 months previously. He had a diagnosis of migraine headaches with aura since age 15. Ophthalmic examination showed retinal nerve fiber layer thickness reduction on the papilomacular bundle in the left eye and unremarkable in the right. Optical coherence tomography documented severe macular thickness reduction and a mild and localized retinal nerve fiber layer loss on the temporal side of the optic disc in the left eye. Systemic investigation, neuroimaging and cardiovascular studies were non-revealing. The cause of his visual loss was possibly related to retinal migraine, although an ischemic episode of another origin could not be excluded. Our case is interesting because it draws attention to the fact that optical coherence tomography may be able to identify localized neural loss not only by measuring peripapillary retinal nerve fiber layer but also by means of macular thickness measurements.

Unilateral dysphotopsia after bilateral intraocular lens implantation using the AR40e IOL model: case report

The authors report a recent complication during the postoperative period of cataract surgery. A patient was submitted to cataract surgery in both eyes with IOL implantation (Sensar®) inside the capsular bag. The postoperative period of right eye was uneventful, however, in the left eye the patient noted a dark shadow at the temporal visual field at the first postoperative week. This diagnosis was confirmed with the presence of a temporal scotoma revealed by the computerized visual field (first reported in this study) and also reducing the pupil area with miotic drops. The treatment of this complication was performed by using brimonidine tartrate and after 6 months this symptom completely disappeared as confirmed by the computerized visual field. The treatment should be based on reducing the pupil area with miotic drops and we also recommend to observe these cases until the 6th postoperative month before indicating an IOL exchange since the capsular edge that overlaps the IOL may opacify creating an optical barrier reducing or eliminating negative dysphotopsia.

Os autores relatam uma complicação no período pós-operatório relativamente recente na cirurgia de catarata. Trata-se de paciente que foi submetido à cirurgia de catarata bilateral não simultânea com implante sacular da lente intra-ocular (Sensar®). O pós-operatório do olho direito evoluiu sem nenhuma queixa, entretanto, no olho esquerdo o paciente referiu a presença de uma sombra em campo visual temporal na primeira semana de pós-operatório sendo diagnosticado disfotopsia negativa após exclusão de outras causas oculares, principalmente retinianas. Este diagnóstico foi confirmado com a presença de um escotoma temporal na campimetria visual computadorizada (relatado na literatura pela primeira vez neste estudo) e também com a redução medicamentosa do tamanho da pupila. Seu tratamento foi realizado com a administração de tartarato de brimonidina e após 6 meses o sintoma desapareceu sendo também documentado com o campo visual (ausência de escotoma). A disfotopsia negativa deve ser tratada visando reduzir a área pupilar e observar por pelo menos 6 meses antes de indicar a troca do lente intra-ocular, uma vez que a cápsula anterior que cobre a LIO pode opacificar criando uma barreira óptica reduzindo ou eliminando esta complicação.

An unusual chiasmal visual defect in a patient with neuromyelitis optica: case report

PURPOSE: To report the unusual visual field finding due to a chiasmal neuritis in a 33-year-old female with the diagnosis of optic neuromyelitis optica (Devic's syndrome). METHODS: We report a case of a 33 years old female with limb paraesthesias, weakness in the legs, bowel and bladder dysfunction that was referred to the "Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo" in October 1995. Six years and four months later she had an acute visual involvement. Ophthalmologic examination, laboratory studies, magnetic resonance imaging (MRI) and a 24-2 threshold visual field in the Humphrey field analyzer were performed. RESULTS: The MRI scan showed enlargement and cavitation on the spinal cord and chiasmal involvement (thickening of the chiasm with contrast enhancement) and no demyelinating lesions in the brain, brainstem, or cerebellum. The central 24-degree threshold field examination showed an inferior visual field defect bitemporally, disclosing a chiasmal involvement. CONCLUSION: Chiasmal involvement may occur in neuromyelitis optica, probably due to a plaque within the chiasm. The authors call attention to the importance of visual field examination with particular regard to quantifying the visual impairment and follow-up of these patients.

OBJETIVO: Relatar o caso de uma mulher de 33 anos de idade, com o diagnóstico de neuromielite óptica (síndrome de Devic) acometendo o quiasma óptico que apresentou um escotoma incomum no exame de campo visual. MÉTODOS: Uma paciente do sexo feminino, portadora de parestesias nos membros inferiores, fraqueza nas pernas, disfunção da defecação e disfunção urinária, foi encaminhada para o Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo em outubro do ano de 1995. Seis anos e quatro meses mais tarde ela sofreu acometimento visual agudo. Foram realizados exame oftalmológico completo, exame de líquor, ressonância nuclear magnética e um exame de campo visual no perímetro de Humphrey. RESULTADOS: A ressonância magnética revelou espessamento e imagens de cavitações na medula espinal, assim como espessamento do quiasma óptico, acompanhado de aumento na captação do contraste. Não apareceu imagem sugestiva de processo desmielinizante no cérebro, tronco cerebral ou cerebelo. O exame 24-2 (campo visual central) demonstrou defeito bitemporal inferior, revelando assim o comprometimento do chiasma. CONCLUSÃO: Comprometimento do quiasma óptico pode ocorrer nos casos de neuromielite óptica, provavelmente devido a uma placa de desmielinização ocorrendo no quiasma. Os autores enfatizam a importância do exame de campo visual para quantificar o comprometimento das vias ópticas e acompanhar a evolução destes pacientes.

Alterações oculares associadas ao "bungee jumping": relato de caso / Ocular alterations associated with bungee jumping: case report

Com a popularização do "bungee jumping" vem se observando aumento na ocorrência de lesões associadas a sua prática, entre elas lesões oculares. O objetivo deste relato é descrever um caso de diminuição aguda da acuidade visual e alterações campimétricas após "bungee jumping". Os autores apresentam o caso de paciente de sexo feminino, 48 anos, sem história de doença ocular ou sistêmica, que chega a consulta em emergência oftalmológica com queixa de baixa da acuidade visual após "bungee jumping", apresentando ao exame oftalmológico inicial, hemorragias em pólo posterior de ambos os olhos. A angiografia fluoresceínica apresentava áreas hipofluorescentes, por bloqueio do contraste, correspondendo às hemorragias, sem outras alterações vasculares. Avaliada após 14 semanas, observou-se reabsorção das hemorragias e rarefação do epitélio pigmentar da retina em pólo posterior; evoluiu clinicamente com melhora da visão, mas permaneceu com queixa de escotoma e alterações campimétricas mesmo cinco meses após o evento inicial. A ocorrência de lesões corporais, entre elas lesões oculares, com risco de diminuição da acuidade visual deve ser informada aos candidatos à prática deste esporte, sendo papel do oftalmologista prover informações à população em geral sobre possíveis afecções oculares, neste esporte e no cotidiano.

Optic disc imaging by Heidelberg retinal tomogram in congenital optic disc anomaly.

We evaluated two cases of congenital optic disc anomaly with the Heidelberg Retinal Tomograph (HRT) that could be mistaken for glaucomatous optic disc. One was an optic disc coloboma with a visual field defect and the other had an optic disc pit without a visual field defect. HRT was abnormal only in the eye with optic disc pit with normal fields. While HRT can be a valuable adjunct to disc evaluation and follow-up, it cannot be used in isolation in the differentiation of abnormal from normal optic discs.

Junctional scotoma in giant cerebral aneurysm

A brain lesion located at the lateral side of the sella turcica can produce a junctional scotoma by compressing the ipsilateral optic nerve and the contralateral inferonasal nerve fiber. This study reports a female patient with a junctional scotoma caused by a cerebral aneurysm. At the initial visit, she complained of visual disturbance in both eyes and the right optic disc was atrophied. The visual field showed right blindness and left superotemporal quadrantopsia. A brain CT indicated an approximately 3 cm sized brain mass located superolateral to the sella turcica. The brain MRI showed the lesion to be more like an aneurysm than a pituitary adenoma. Therefore, 4 vessels angiography was done, and this lesion was confirmed to be a sellar variant of an aneurysm located at the right carotid siphon. Like a tumor of the optic chiasm, a cerebral aneurysm can cause visual disturbance and visual field defects. Therefore, an early differential diagnosis is important because the prognosis and treatment of an aneurysm differ.

Escotoma juncional e neuropatia óptica compressiva intracraniana / Junctional scotoma and intracranial compressive optic neuropathy

O autor apresenta um caso de escleromalácia perfurante em paciente com artrite reumatóide