Eletrorretinograma de padrão reverso (PERG) de campo total na esclerose múltipla e na neuromielite óptica e PERG multifocal na atrofia em banda do nervo óptico: correlação com os achados da tomografia de coerência óptica e da perimetria computadorizada / Pattern-reversal electroretinogram (PERG) full field in multiple sclerosis and optic neuromyelitis and multifocal PERG in band atrophy of the optic nerve: correlation with the findings of optical coherence tomography and computerized perimetry

OBJETIVO: avaliar a capacidade do eletrorretinograma de padrão reverso de campo total (PERG) e multifocal (PERGmf) de detectar alterações funcionais da retina em pacientes com afecções desmielinizantes e compressivas da via óptica anterior; estudar a correlação entre as amplitudes do PERG e PERGmf com as espessuras das camadas internas da retina obtidas pela tomografia de coerência óptica (TCO) e com a perda de campo visual avaliada pela perimetria computadorizada padrão (PCP). MÉTODOS: cento e cinquenta e dois olhos de 28 pacientes com esclerose múltipla (EM), 38 com espectro da neuromielite óptica (NMO) com ou sem neurite óptica (NO) prévia e 30 olhos de 26 controles normais foram submetidos ao PERG, à TCO para avaliação da camada de fibras nervosas da retina (CFNR) peripapilar e camadas internas da retina na mácula e à PCP. Os olhos estudados foram divididos em 4 grupos: grupo 1. EM com NO; grupo 2. EM sem NO; grupo 3. NMO; grupo 4. Mielite transversa longitudinal extensa. Os achados foram comparados utilizando-se as equações de estimativas generalizadas. As correlações entre os achados do PERG, da TCO e da PCP foram avaliadas pela correlação de Pearson ou Spearman e pela análise de regressão linear. Para avaliação dos pacientes com doenças compressivas da via óptica anterior, foram estudados 25 olhos de pacientes com defeito campimétrico temporal e atrofia em banda (AB) do nervo óptico decorrente de compressões quiasmáticas já tratadas e 25 olhos de 25 controles normais pelo PERGmf, a TCO e a PCP. As comparações entre olhos com AB e controles foram feitas usando análise de variância (ANOVA). As correlações entre achados do PERGmf, da TCO e da PCP foram avaliadas pela correlação de Pearson ou Spearman e pela análise de regressão linear. RESULTADOS: comparado com controles, as amplitudes do PERG foram significativamente reduzidas em olhos de pacientes com NMO e EM com NO, mas não mostraram diferenças significativas em olhos de pacientes com EM sem NO...

PURPOSE: To evaluate the ability of full-field and multifocal (mf) pattern electroretinogram (PERG) parameters to detect functional changes of the retina in patients with demyelinating and compressive diseases of the anterior visual pathway; to study the relationship between the PERG (full field and multifocal) amplitudes, optical coherence tomography (OCT)-measured inner retinal layers measurements and visual field sensitivity loss on standard automated perimetry (SAP). METHODS: one hundred fifty-two eyes of 28 patients with multiple sclerosis (MS), 38 with neuromyelitis optica (NMO) spectrum with or without previous episodes of optic neuritis (ON) and 30 eyes of 26 normal controls underwent PERG, OCT macular and peripapillary retinal nerve fiber layer measurements and SAP. The eyes of the patients were divided into 4 groups: group 1. EM with ON; group 2. EM without ON; group 3. NMO; group 4. Longitudinally extensive transverse myelitis (LETM). The findings were compared using the generalized estimating equations (GEE). The correlations between the findings of the PERG, OCT and SAP were assessed by Pearson correlation coefficients or Spearman's rank correlation coefficients and linear regression analysis. For evaluation of patients with compressive diseases of the anterior visual pathway, 25 eyes of patients with temporal visual field defect and band atrophy (BA) of the optic nerve due to chiasmal compression and 25 eyes of 25 normal controls were studied by mfPERG, OCT and SAP. Comparisons between BA and control eyes were made using variance analysis (ANOVA). The correlations between the findings mfPERG, OCT and SAP were evaluated by Pearson correlation coefficients or Spearman's rank correlation coefficients and linear regression analysis. RESULTS: compared to controls, PERG amplitudes were significantly reduced in eyes of patients with MS with ON and NMO, but not in eyes with MS without ON and longitudinal extensive transverse myelitis...

A Case of Idiopathic Granulomatous Hypophysitis

Granulomatous hypophysitis is a rare pituitary condition that commonly presents with enlargement of the pituitary gland. A 31-year-old woman was admitted to the hospital with a severe headache and bitemporal hemianopsia. Magnetic resonance imaging (MRI) showed an 18 x 10-mm sellar mass with suprasellar extension and compression of the optic chiasm. Interestingly, brain MRI had shown no abnormal finding 4 months previously. On hormonal examination, hypopituitarism with mild hyperprolactinemia was noted. The biopsy revealed granulomatous changes with multinucleated giant cells. We herein report this rare case and discuss the relevant literature.

Correlação entre as espessuras da mácula e da camada de fibras nervosas da retina, medidas pelas tomografias de coerência óptica de dominio Fourier e de domínio do tempo, e a perimetria automatizada na atrofia em banda do nervo óptico / Correlation between macular and retinal nerve fiber layer Fourier domain and time domain optical coherence tomography measurements and visual field loss in band atrophy of the optic nerve

OBJETIVO: Investigar a correlação entre as espessuras da mácula e da camada de fibras nervosas da retina (CFNR), medidas pelas tomografias de coerência óptica de domínio Fourier (FD - OCT) e de domínio do tempo (TD - OCT) e a perda de sensibilidade no campo visual (CV) em pacientes com atrofia em banda do nervo óptico. Comparar a habilidade diagnóstica dos dois instrumentos. MÉTODOS: 36 olhos de 36 pacientes com perda de CV permanente por compressão do quiasma óptico e 36 controles normais foram submetidos ao exame de CV pela perimetria automatizada padrão (Humphrey Field Analyzer TM; Carl Zeiss Meditec, Dublin, CA), ao FD - OCT (3 D OCT-1000TM Topcon Corp., Tokyo, Japan) e ao TD - OCT (StratusTM ;Carl Zeiss Meditec Inc, Dublin, California, USA). Foram comparados os protocolos análogos de ambos os equipamentos para avaliação da espessura macular.Foi realizado a divisão macular em quatro quadrantes e em metades, sendo calculado a média da espessura macular global, a média da espessura dos quadrantes e a média da espessura das metades maculares. A média global e setorial da medida da espessura da CFNR peripapilar também foi analisada. A perda de sensibilidade no CV foi inicialmente avaliada pelo defeito temporal médio. O CV foi ainda dividido em 6 setores de acordo com a distribuição da CFNR e em 16 pontos centrais para a realização da correlação estrutura-função entre os parâmetros medidos pelo FD - OCT e a perda de sensibilidade no CV em decibéis e 1/Lambert. Foi calculado o coeficiente de correlação de Sperman e a análise de regressão linear. As áreas sobre a curva ROC e valores fixos de sensibilidade e especificidade foram calculados para cada parâmetro estudado. RESULTADOS: As medidas da espessura macular e da CFNR pelo FD - OCT e TD - OCT foram capazes de discriminar olhos com atrofia em banda do nervo óptico dos controles normais. A espessura global e setorial dos parâmetros maculares e da CFNR mostraram diferenças significativas (p<0,001) entre os...

PURPOSE: To investigate the relationship between fourier-domain optical coherence tomography (FD - OCT) measured macular and retinal nerve fiber layer thickness (RNFL) and visual field sensitivity loss on standard automated perimetry in eyes with permanent temporal hemianopia from chiasmal compression and compare the ability of FD - OCT and time-domain optical coherence tomography (TD - OCT) to detect axonal loss in eyes with band atrophy of the optic nerve. METHODS: One eye of each of 36 patients with permanent temporal visual field defects and 36 age- and sex-matched healthy subjects. Subjects underwent standard automated perimetry and macular and RNFL thickness measurements with FD - OCT (3 D OCT-1000® Topcon Corp., Tokyo, Japan) and TD - OCT (Stratus; Carl Zeiss Meditec Inc, Dublin, California, USA). Macular thickness measurements as a global average, divided in four quadrants and in two halves as well as average and sectoral RNFL thickness around the optic disc were calculated. Visual field sensitivity loss was evaluated by the temporal mean defect; as deviations from normal in six sectors of the visual field and in 16 central visual field test points. Relationship between visual field sensitivity loss in decibel and 1/Lambert units and optical coherence tomography measurements were evaluated using Spearman correlation coefficients and by linear regression analysis. Receiver operating characteristic curves and sensitivities at fixed specificities were calculated for each parameter. RESULTS: Global and sectoral macular and RNFL thickness parameters showed a significant difference in eyes with band atrophy compared with controls. The strongest correlations were seen between visual field sensitivity loss and quadrantic or hemianopic nasal macular thickness measurements than with sectoral retinal nerve fiber layer thickness measurements. The highest correlation was observed between the inferonasal quadrant macular thickness and the visual field...

Relationship between visual field sensitivity loss and quadrantic macular thickness measured with Stratus-Optical coherence tomography in patients with chiasmal syndrome / Correlação entre defeito de campo visual e espessura macular quadrântica avaliada através da tomografia de coerência óptica em pacientes com compressão quiasmática

PURPOSE: To correlate visual field sensitivity (VFS) loss on standard automated perimetry (SAP) and quadrantic macular thickness on optical coherence tomography (OCT) in patients with permanent temporal hemianopia from chiasmal compression. METHODS: Forty eyes from 40 patients with chiasmal compression and 40 healthy eyes were submitted to standard automated perimetry and Stratus-OCT scanning. Raw data of the fast macular thickness scanning protocol were exported and macular thickness measurements were recorded and averaged for each quadrant and half of the central area. The correlation between visual field sensitivity loss and optical coherence tomography measurements was tested with Pearson's correlation coefficients and with linear regression analysis. RESULTS: A significant association was found between each macular thickness parameter and the corresponding central VF mean sensitivity. The strongest association was observed between superonasal macular thickness and the inferotemporal mean defect measured both in decibel (R=0.47; p=0.001) and in 1/Lambert (R=0.59; p<0.0001) units. CONCLUSION: Stratus-OCT-measured macular thickness was topographically related with visual field sensitivity loss in patients with temporal hemianopia from chiasmal compression. Such measurements could prove clinically useful in the diagnosis and follow-up of patients with chiasmal compression. ClinicalTrial.gov identifier number: NCT0039122.

OBJETIVO: Avaliar a correlação entre o defeito de campo visual ao exame de perimetria computadorizada e a espessura macular quadrântica ao exame de tomografia de coerência óptica (OCT) em pacientes com hemianopsia temporal permanente causada por compressão quiasmática. MÉTODOS: Quarenta olhos de 40 pacientes com compressão quiasmática e 40 olhos de 40 indivíduos controles foram submetidos aos exames de perimetria computadorizada e tomografia de coerência óptica. Dados não processados foram exportados e as medidas de espessura macular foram calculadas para cada quadrante e metade da área macular central. A correlação entre o defeito campimétrico e as medidas de espessura macular foi avaliada por coeficiente de correlação de Pearson e por análise de regressão linear. RESULTADOS: Associação significante foi encontrada entre os parâmetros de espessura macular e seus respectivos defeitos campimétricos. A correlação mais forte foi encontrada entre o parâmetro espessura macular nasal superior e o defeito campimétrico médio temporal inferior medido em decibel (R=0,47; p=0,001) e em 1/Lambert (R=0,59; p<0,0001). CONCLUSÃO: Medidas de espessura macular avaliada através da tomografia de coerência óptica foi topograficamente relacionada ao defeito campimétrico em pacientes com hemianopsia temporal por compressão quiasmática. Estas medidas podem provar a importância clínica no diagnóstico e seguimento dos pacientes com compressão quiasmática. ClinicalTrial.gov identifier number: NCT0039122.

Correlação entre o eletrorretinograma de padrão reverso, a tomografia de coerência óptica e a perimetria automatizada na detecção da perda neural na atrofia em banda do nervo óptico / Relationship between pattern electroretinogram, optical coherence tomography and automated perimetry for detection of neural loss in eyes with band atrophy of the optic nerve

OBJETIVO: Avaliar a capacidade dos parâmetros do eletrorretinograma de padrão reverso de campo total e hemianópico em diferenciar olhos com atrofia em banda do nervo óptico e, a correlação entre as amplitudes do eletrorretinograma de padrão reverso, a espessura da camada de fibras nervosas da retina e macular obtidas pela tomografia de coerência óptica e a perda de campo visual nestes pacientes. MÉTODOS: Quarenta e um olhos de 41 pacientes com perda de campo visual temporal permanente por compressão do quiasma óptico e 41 controles normais foram submetidos ao eletrorretinograma de padrão reverso de estimulação de campo total e hemianópicos (temporal e nasal), a tomografia de coerência óptica, para avaliação das medidas da espessura da camada de fibras nervosas da retina e macular e, ao exame de campo visual, pela perimetria automatizada padrão. O desvio do normal da sensibilidade dos 18º centrais do campo visual foram expressos em decibéis e unidades 1/Lambert. As comparações foram feitas pelo teste t de Student. A correlação entre os parâmetros do campo visual central, do eletrorretinograma de padrão reverso e da tomografia de coerência óptica foi avaliada pela correlação de Pearson e análise de regressão linear. RESULTADOS: Os valores das amplitudes P50, N95, and P50+N95 do eletrorretinograma de padrão reverso de campo total, e de estimulação hemianópica e os valores das medidas da espessura macular e da camada de fibras nervosas da retina obtidas pela tomografia de coerência óptica foram significativamente menores nos olhos com atrofia em banda do que nos controles (P<0,001). Uma correlação significativa foi encontrada entre a perda de sensibilidade no campo visual central e as amplitudes do eletrorretinograma de padrão reverso de estimulação de campo total e nasal, mas não para o temporal. Uma correlação significativa positiva foi observada entre os parâmetros de perda de sensibilidade no campo visual e a maioria dos parâmetros da espessura da...

PURPOSE: To evaluate the ability of full-field and hemifield pattern electroretinogram parameters to differentiate between healthy eyes and eyes with band atrophy of the optic nerve and also to evaluate the relationship between pattern electroretinogram amplitude, macular and retinal nerve fiber layer thickness by optical coherence tomography, and visual field loss on standard automated perimetry in eyes with BA of optic nerve. METHODS: Forty-one eyes from 41 patients with permanent temporal visual field defects from chiasmal compression and 41 healthy subjects underwent transient fullfield and hemifield (temporal or nasal) stimulation pattern electroretinogram, standard automated perimetry and time domain- optical coherence tomography macular and retinal nerve fiber layer thickness measurements. Comparisons were made using Students t-test. Deviation from normal visual field sensitivity for the central 18° was expressed in dB and 1/Lambert units. Correlations between measurements were verified by Pearsons correlations and linear regression analysis. RESULTS: Full-field P50, N95, and P50+N95 amplitude values were significantly smaller in eyes with band atrophy than in control eyes (P<0.001). Nasal and temporal hemifield pattern electroretinogram studies revealed significant differences in N95 and P50+N95 amplitudes measurements. Pattern electroretinogram and optical coherence tomography measurements were significantly lower in eyes with temporal hemianopia than in normal eyes. A significant correlation was found between visual field sensitivity loss and full-field or nasal, but not temporal, hemifield pattern electroretinogram amplitude. Likewise a significant correlation was found between visual field sensitivity loss and most optical coherence tomography parameters. No significant correlation was observed between optical coherence tomography and pattern electroretinogram parameters, except for nasal hemifield amplitude. A significant correlation was...

Pituitary macroadenomas; demographic, visual, and neuro-radiological patterns

To describe the demographic profile, patterns of visual disturbance and imaging studies of patients with Macroadenoma. Retrospectively. Reviewed the clinical charts and neuroradiologic imaging of 125 patients who were diagnosed as cases of Macroadenoma. 2000 to 2009. 100 patients were selected who had visual disturbances along with Macroadenomas. Age, sex, visual symptoms and other associated systemic problems of these patients were reviewed. The Neuroimaging data [Magnetic Resonance Imaging] was correlated with the clinical picture. The data was analysed using Statistical package for social sciences [SPSS]. The Descriptive statistics were calculated for age and MR findings. The age ranged from 9 to 85 years [mean 42.92]. Male to female ratio was 1.4:1.90% patients had visual disturbances including visual field defects and 10% had ocular motor nerve palsies. Tumour extension on MR studies showed optic chiasma compression in 69% patients, cavernous sinus invasion in 57% and Sphenoidal sinus invasion in 14%. Haemorrhagic foci were seen in 8% and intra tumour necrosis was found in 9% patients. The most common path for the extension of pituitary macroadenomas is towards the optic chiasma. Hence majority of these patients present with visual disturbances. MRI is an excellent aid to see the extent and invasion of Macroadenomas to the surrounding structures

Suprasellar ganglioglioma with unusual diffuse involvement of the entire optico-chiasmal hypothalamic pathway.

Gangliogliomas (GG) are mixed glioneuronal tumors of the central nervous system (CNS), occurring mostly in the pediatric population, with common sites being temporal lobes and less commonly in the frontal and parietal lobes. We report a case of a 7-year-old child who presented with bilateral visual defects for 6 months. Magnetic resonance imaging (MRI) of the brain revealed an intensely enhancing mass lesion with calcification in the sellar and suprasellar region involving the optic chiasm and the left optic nerve. The mass showed almost bilaterally symmetrical diffuse spread along the optic tracts posteriorly and hypothalamus, temporal lobes, thalami and the basal ganglia. The lesion was radiologically indistinguishable from chiasmatic astrocytoma or a germ cell tumor but histopathological features were of a ganglioglioma. While a few optic apparatus gangliogliomas have been reported in the literature, such widespread diffuse involvement of the entire optico-chiasmal hypothalamic pathway is unusual.

Acute Marchiafava-Bignami Disease with Widespread Callosal and Cortical Lesions

Marchiafava-Bignami disease (MBD) is a rare alcohol-related disorder that results in progressive demyelination and necrosis of the corpus callosum. The process may extend to the optic chiasm and tracts, cerebellar peduncle, subcortical resion, neighboring white matter, and rarely, cortical gray matter. We report a case of MBD in which fluid-attenuated inversion recovery and diffusion magnetic resonance imaging studies revealed symmetrical hyperintense lesions in the cerebral cortex in addition to the callosal lesions.

Suprasellar malignant mixed germ cell tumour presenting as craniopharyngioma.

A 15 year old boy presented with diminution in vision of both eyes, diabetes insipidus and hypopituitarism. MRI was suggestive of a large suprasellar and retrosellar craniopharyngioma with stretching of the optic chiasma. Histopathological findings on the first surgical specimen were interpreted as a craniopharyngioma. He was reoperated on account of clinical deterioration and increase in tumour size. Histological examination this time revealed derivatives of all three germ cell lineages along with areas of embryonal carcinoma, and yolk sac tumour besides squamous cysts, establishing the diagnosis of malignant mixed germ cell tumour. Serum and CSF were strongly positive for alpha foetoproteins.

Tumores sellares y suprasellares que afectan al quiasma y nervios ópticos. III. Gliomas diencefálicos de nervios y quiasma ópticos, ependimomas y subependimomas, espongioneuroblastomas, hemangionas diencefálicos, alteraciones inflamatorias / Sellar and suprasellar tumors afecting chiasma and optic nerves. Part III. Diencephalic gliomas of the chiasma and optic nerve, ependimomas ans subepondimomas, spongioneuroblastomas, diencephalic hemangiomas and inflamatory alterations

Las vías visuales anteriores pueden dañarse por: Tumores que se originen del quiasma y nervio óptico (astrocitoma y glioblastoma multiforme), adenomas pituitarios y arqueoblastomas, gliomas diencefálicos, espongioneuroblastoma, ependimomas y excepcionalmente por parásitos o procesos inflamatorios, granulomatosos que actúan como una masa ocupativa intracraneana. Es necesario tener en mente estos padecimientos en el diagnóstico diferencial. Es frecuentemente que los pacientes acudan primero con el optometrista o el oftalmólogo, en este último es en quien recae la responsabilidad más importante en la prontitud del diagnóstico

Tumores sellares y suprasellares que afectan al quiasma y nervios ópticos. II. Tumores derivados de la hipófisis y de los restos de la bolsa de Rathke / Sellar and suprasellar tumors afecting optic chiasm II. Pituitary derived tumors and rathkes pouch remainders

Los tumores derivados de la hipófisis y de la bolsa de Rathke incluyen los adenomas (cromófilos y cromófobos) y los arqueoblastomas (craneogaringiomas, quistes coloides, tumor de Erdheim, etc.) Estos tumores en su evolución biológica lesionan a la vía visual anterior y las manifestaciones clínicas más tempranas son visuales y le siguen las endócrinas y mecánicas. El tratamiento depende de la prontitud del diagnóstico. Los métodos diagnósticos actuales de imagenología permiten un diagnóstico temprano (microadernomas). Se mencionan los hechos histológicos más sobresalientes en relación con la clínica

Tumores sellares y suprasellares que afectan al quiasma y nervios ópticos. I. Consideraciones generales / Sellar and suprasellar tumors affecting the chiasma and optic nerves. General considerations

Las neoplasias sellares y suprasellares representan únicamente el 7.5 por ciento de los tumores intracraneanos. Las variedades más importantes son los craneofaringiomas y los adenomas. Estas neoplasias se manifiestan clínicamente por el daño a las vías visuales anteriores. Los adenomas son neoplasias funcionales específicas mientras que los craneofanrigiomas cursan con hipopituitarismo. La resonancia nuclear magnética y la topografía axial computada substituyen con muchas ventajas a los procedimiento radiológicos anteriores. El tratamiento puede ser médico, quirúrgico, con radiaciones ionizantes y recientemente con una variante que es la radiocirugía o el bisturí de rayos gamma. Hisotológicamente tienen un patrón característico cada una de sus variedades y actualmente se puede demostrar su estirpe con toda precisión. El oftalmólogo juega el papel más importante en la detección de estas neoplasias