Manejo de la trombosis venosa cerebral en España: estudio descriptivo MOTIVATE / Management of cerebral venous thrombosis in Spain: MOTIVATE descriptive study

Introducción: La trombosis venosa cerebral (TVC) es una causa poco común de ictus que afecta principalmente a adultos jóvenes. Un diagnóstico precoz y preciso puede reducir la tasa y gravedad de las complicaciones. Objetivo: Analizar las características clínicas, manejo y tratamiento de la TVC en diferentes centros de nuestro país. Métodos: Estudio descriptivo retrospectivo multicéntrico de pacientes hospitalizados por TVC entre 2008 y 2017 en 11 centros sanitarios en nuestro país. Resultados: Se incluyeron 256 pacientes, edad media 49,8 ± 18,7 años y el 51% fueron mujeres. Los síntomas más frecuentes fueron: cefalea (73%), déficits focales (50%), crisis epilépticas (33%) y encefalopatía (21%). Las localizaciones más frecuentes fueron: seno longitudinal superior (12,5%), transverso (10,9%) y afectación de dos o más senos o venas (66,4%). La etiología conocida más frecuente fue la trombofilia (24%), siendo la mutación de la protrombina G20210A la más común (19%). El 46% fue tratado con antitrombóticos durante 3-6 meses, el 21% durante un año y un 22,6% de los pacientes requirieron anticoagulación indefinida. En un 5% de los sujetos fue preciso terapia endovascular y un 33% requirió neurocirugía. En relación al pronóstico, el 75% fueron independientes a los 3 meses con una puntuación en la escala de Rankin modificada (mRS) ≤ 2 y la presencia de papiledema (p = 0,03), déficit focal (p = 0,001) y encefalopatía (p < 0,001) se relacionaron significativamente con mal pronóstico (mRS > 3). La tasa de mortalidad intrahospitalaria fue del 4,3% y el 6,3% de los pacientes fallecieron a los 3 meses. Conclusión:La diversidad de factores de riesgo y la presentación variable suponen un desafío en el diagnóstico y tratamiento de la TVC. Para mejorar el pronóstico y reducir la mortalidad es fundamental la instauración de protocolos en el manejo de esta patología.(AU)

Introduction: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults. Early, accurate diagnosis can reduce the rate and severity of complications. Objective: The aim of this study was to analyse the clinical characteristics, management, and treatment of CVT in different centres in Spain. Methods: We conducted a multicentre, retrospective, descriptive study of patients hospitalised due to CVT between 2008 and 2017 at 11 Spanish centres. Results: We included 256 patients, with a mean age (SD) of 49.8 (18.7) years; 51% of patients were women. The most frequent symptoms were headache (73%), focal deficits (50%), epileptic seizures (33%), and encephalopathy (21%). The most frequent localisations were the superior sagittal sinus (12.5%), the transverse sinus (10.9%), and 2 or more sinuses or veins (66.4%). Thrombophilia was the most frequent known aetiology (24%), and was most commonly associated with the prothrombin G20210A mutation (19%). Forty-six percent of patients were treated with antithrombotics for 3-6 months, 21% for one year, and 22.6% required indefinite anticoagulation. Endovascular therapy was performed in 5% of cases, and 33% required neurosurgery. Regarding outcomes, 75% of patients were independent at 3 months (modified Rankin Scale [mRS] score ≤ 2), with papilloedema (P = .03), focal deficits (P = .001), and encephalopathy (P < .001) showing a statistically significant association with poor prognosis (mRS > 3). The in-hospital mortality rate was 4.3%, with a 3-month mortality rate of 6.3%. Conclusion: The diverse risk factors and variable presentation of CVT represent a challenge in the diagnosis and treatment of this condition. To improve prognosis and reduce mortality, it is essential to establish management protocols for this entity.(AU)

Diagnosis and treatment of disorders of intracranial pressure: consensus statement of the Spanish Society of Neurology's Headache Study Group.

Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the diagnosis and treatment of these 2entities in recent years. Therefore, the Spanish Society of Neurology's Headache Study Group (GECSEN) deemed it necessary to prepare this consensus statement, including diagnostic and therapeutic algorithms to facilitate and improve the management of these disorders in clinical practice. This document was created by a committee of experts belonging to GECSEN, and is based on a systematic review of the literature, incorporating the experience of the participants, and establishes practical recommendations with levels of evidence and grades of recommendation.

Management of cerebral venous thrombosis in Spain: MOTIVATE descriptive study.

INTRODUCTION: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults. Early, accurate diagnosis can reduce the rate and severity of complications. OBJECTIVE: The aim of this study was to analyse the clinical characteristics, management, and treatment of CVT in different centres in Spain. METHODS: We conducted a multicentre, retrospective, descriptive study of patients hospitalised due to CVT between 2008 and 2017 at 10 Spanish centres. RESULTS: We included 256 patients, with a mean age (SD) of 49.8 (18.7) years; 51% of patients were women. The most frequent symptoms were headache (73%), focal deficits (50%), epileptic seizures (33%), and encephalopathy (21%). The most frequent localisations were the superior sagittal sinus (12.5%), the transverse sinus (10.9%), and 2 or more sinuses or veins (66.4%). Thrombophilia was the most frequent known aetiology (24%), and was most commonly associated with the prothrombin G20210A mutation (19%). Forty-six percent of patients were treated with antithrombotics for 3 to 6 months, 21% for one year, and 22.6% required indefinite anticoagulation. Endovascular therapy was performed in 5% of cases, and 33% required neurosurgery. Regarding outcomes, 75% of patients were independent at 3 months (modified Rankin Scale [mRS] score ≤ 2), with papilloedema (P =  .03), focal deficits (P = .001), and encephalopathy (P < .001) showing a statistically significant association with poor prognosis (mRS > 3). The in-hospital mortality rate was 4.3%, with a 3-month mortality rate of 6.3%. CONCLUSION: The diverse risk factors and variable presentation of CVT represent a challenge in the diagnosis and treatment of this condition. To improve prognosis and reduce mortality, it is essential to establish management protocols for this entity.

Papiledema como posible signo guía en el diagnóstico del síndrome periódico asociado a la criopirina: a propósito de un caso / Papilledema as a possible key sign for diagnosis of cryopyrin-associated periodic syndromes (CAPS): A case report

El síndrome periódico asociado a la criopirina es una enfermedad rara y probablemente infradiagnosticada. Se presenta con manifestaciones sistémicas, entre ellas oftalmológicas, muy diversas, por lo que su diagnóstico supone un reto para el clínico. Presentamos el caso de una niña de 4 años en la que la identificación de papiledema en el examen oftalmológico constituyó el signo guía para el diagnóstico de síndrome periódico asociado a la criopirina. Pretendemos así concienciar sobre esta enfermedad de graves implicaciones y cuyo diagnóstico precoz resulta esencial para los afectados, para que sea tenido en cuenta con mayor frecuencia como diagnóstico diferencial (AU)

Cryopyrin-associated periodic syndrome is a rare and probably underdiagnosed disease. It presents with various systemic manifestations, including ophthalmological, making its diagnosis a challenge for the clinician. We present the case of a 4-year-old girl for which the identification of papilledema in the ophthalmological examination was the key sign for the diagnosis of cryopyrin-associated periodic syndrome. Our aim is to raise awareness of this syndrome with serious implications for affected patients, so that it is taken into account more frequently as a differential diagnosis, allowing an early diagnosis (AU)

Papilledema as a possible key sign for diagnosis of cryopyrin-associated periodic syndromes (CAPS): A case report.

Cryopyrin-associated periodic syndrome is a rare and probably underdiagnosed disease. It presents with various systemic manifestations, including ophthalmological, making its diagnosis a challenge for the clinician. We present the case of a 4-year-old girl for which the identification of papilledema in the ophthalmological examination was the key sign for the diagnosis of cryopyrin-associated periodic syndrome. Our aim is to raise awareness of this syndrome with serious implications for affected patients, so that it is taken into account more frequently as a differential diagnosis, allowing an early diagnosis.

Papiledema bilateral por síndrome do pseudotumor cerebral em paciente com achado fortuito de microadenoma hipofisário / Bilateral papilledema by cerebral pseudotumor syndrome in a patient with a fortuitous finding of pituitary microadenoma

RESUMO O presente relato descreve um raro caso de papiledema bilateral decorrente da síndrome do pseudotumor cerebral relacionado com achado de microadenoma hipofisário. Relatamos um caso de paciente do sexo feminino, 21 anos, referindo baixa acuidade visual para longe, associada à cefaleia de início há 2 meses. Ao exame oftalmológico, a fundoscopia mostrou presença de papiledema bilateral significativo. A ressonância magnética de encéfalo evidenciou imagem nodular na hipófise, medindo 7 mm, sem qualquer evidência de compressão intracraniana, insuficiente para causar os sintomas do quadro clínico. Após diagnóstico de síndrome do pseudotumor cerebral, instituiu-se tratamento clínico com acetazolamida e orientou-se perda de peso, com evolução satisfatória. A descrição de ambas as patologias presentes de forma mútua foi citada apenas uma vez durante extensa revisão da literatura. Dessa forma, chamamos a atenção para essa ocorrência incomum, enfatizando as características de ambas as patologias, a fim de facilitar o diagnóstico diferencial, bem como elucidar a melhor abordagem terapêutica. Ressaltamos que o achado de pequenos adenomas hipofisários não deve confundir o diagnóstico etiológico em pacientes com queixas visuais e papiledema bilateral.

ABSTRACT The present report describes a rare case of bilateral papilledema due to cerebral pseudotumor syndrome (CPT) related to an occasional finding of pituitary microadenoma. We report the case of a 21-year-old female patient presenting low far visual acuity, associated with headache beginning 2 months ago. On ophthalmological examination, fundoscopy showed significant bilateral papilledema. Magnetic resonance imaging of the brain showed symmetrical and normal-sized ventricles, besides a nodular imaging in the pituitary, measuring 7 mm, without any evidence of intracranial compression, insufficient to cause the symptoms of the clinical presentation. After the diagnosis of cerebral pseudotumor syndrome, clinical treatment with acetazolamide was instituted and weight loss was advised, with satisfactory evolution. The description of both pathologies mutually present was mentioned only once during extensive literature review. Thus, we call attention to this unusual occurrence, emphasizing the characteristics of both pathologies in order to facilitate the differential diagnosis, as well as to elucidate the best therapeutic approach. We emphasize that the finding of small pituitary adenomas should not confuse the etiological diagnosis in patients with visual complaints and bilateral papilledema.

Idiopathic intracranial hypertension: an illustrated guide for the trainee radiologist / Hipertensão intracraniana idiopática: um guia ilustrado para o residente de radiologia

Abstract Idiopathic intracranial hypertension is characterized by increased intracranial pressure, headache, and visual perturbations. Although the pathophysiology of idiopathic intracranial hypertension is obscure, several mechanisms have been proposed, such as increased cerebral blood volume, excessive cerebrospinal fluid volume (due to high production or impaired resorption), and inflammatory mechanisms as a likely cause of or contributor to impaired cerebrospinal fluid circulation. It predominantly affects women of reproductive age who are overweight or obese. The most common symptoms are daily headache, synchronous pulsatile tinnitus, transient visual perturbations, and papilledema with visual loss. The main neuroimaging findings are a partially empty sella turcica; flattening of the posterior sclera; transverse sinus stenosis (bilateral or in the dominant sinus); a prominent perioptic subarachnoid space, with or without optic nerve tortuosity; and intraocular protrusion of the optic nerve head. The main complication of idiopathic intracranial hypertension is visual loss. Within this context, neuroimaging is a crucial diagnostic tool, because the pathology can be reversed if properly recognized and treated early.

Resumo A hipertensão intracraniana idiopática é caracterizada por aumento da pressão intracraniana, cefaleia e manifestações visuais. Apresenta fisiopatologia incerta, porém, alguns mecanismos já foram propostos, como o aumento do volume sanguíneo cerebral, o excesso de líquor por aumento da produção ou a redução da reabsorção, e mecanismos inflamatórios como fator causal ou mesmo determinando limitação na circulação do líquor. Predomina em mulheres obesas em idade reprodutiva. Os sintomas e sinais mais comuns são cefaleia diária, zumbido síncrono ao pulso, obscurecimentos visuais transitórios e papiledema com perda visual. Os principais achados em neuroimagem são: sela turca vazia, achatamento posterior do globo ocular/esclera, estenose do seio transverso bilateral ou do seio dominante, distensão do espaço liquórico perióptico com ou sem tortuosidade do nervo óptico e protrusão intraocular da cabeça do nervo óptico. A principal complicação da hipertensão intracraniana idiopática é a perda visual. Nesse contexto, o papel da neuroimagem no diagnóstico é fundamental, pois a doença pode ser revertida se devidamente reconhecida e precocemente tratada.

An uncommon case of brucellosis with ocular presentation / Um caso incomum de brucelose com apresentação ocular

ABSTRACT We report an unusual case of brucellosis presented with headache, diminished vision, papillitis and multiple peripapillary hemorrhages accompanied by subretinal fluid extending up to macula. Diagnosis of brucellosis was made based on positive polymerase chain reaction of cerebrospinal fluid sample for Brucella species DNA, accompanied by a raised titer of anti-brucella antibodies. Patient showed remarkable improvement on triple drug therapy in form of doxycycline, rifampicin and ceftriaxone.

RESUMO Relatamos um caso incomum de brucelose apresentada com cefaleia, visão diminuída, papilite e múltiplas hemorragias peripapilares acompanhadas por fluido sub-retinal, estendendo-se até a mácula. O diagnóstico de brucelose foi feito com base na reação em cadeia da polimerase positiva de amostra de líquido cefalorraquidiano para DNA de espécies de Brucella, acompanhada por um título elevado de anticorpos antibrucela. O paciente apresentou melhora notável com a terapia tripla com drogas na forma de doxiciclina, rifampicina e ceftriaxona.

Unilateral papilledema and peripapillary polypoidal choroidal vasculopathy as the presenting manifestations of intracranial hypertension / Papiledema unilateral e vasculopatia polipoidal da coroide peripapilar como sinais de apresentação de hipertensão intracraniana

ABSTRACT We have reported here the case of a 54-year-old woman with intracranial hypertension that presented with the unique features of unilateral papilledema and peripapillary polypoidal choroidal vasculopathy. Our investigations lead to the diagnosis of idiopathic intracranial hypertension and an incidental small right frontal meningioma. The patient was accordingly treated with oral acetazolamide, followed by three consecutive monthly intravitreal injections of bevacizumab, which resulted in the inactivation of the polypoidal choroidal vasculopathy, marked reduction of lipid exudation, and complete absorption of the subretinal fluid. This case serves as the first documentation of polypoidal choroidal vasculopathy associated with papilledema. It also demonstrates that choroidal vascular abnormalities may occur even when optic disk edema is unilateral, which is an uncommon manifestation of increased intracranial pressure. Prompt recognition of such findings and its appropriate management are essential for adequate treatment and prevention of irreversible visual loss.

RESUMO Relatamos um caso de uma paciente de 54 anos com hipertensão intracraniana que apresentava achados atípicos de papiledema unilateral e vasculopatia polipoidal da coroide peripapilar. A investigação levou ao diagnóstico de hipertensão intracraniana idiopática e de um pequeno meningioma incidental. A paciente foi tratada com acetazolamida por via oral, seguida de três injeções intravítreas mensais de bevacizumabe, resultando em inatividade da vasculopatia polipoidal da coroide, redução da exsudação e completa absorção do líquido subretiniano. A apresentação deste caso serve para documentar pela primeira vez vasculopatia polipoidal da coroide associada a papiledema. Ele também demonstra que podem ocorrer anormalidades vasculares da coroide mesmo quando o edema do disco óptico é unilateral, uma manifestação incomum do aumento da pressão intracraniana. O reconhecimento imediato desses achados e seu manejo adequado são essenciais para o tratamento adequado e para prevenção da perda visual irreversível.

Management of cerebral venous thrombosis in Spain: MOTIVATE descriptive study. / Manejo de la trombosis venosa cerebral en España: estudio descriptivo MOTIVATE.

INTRODUCTION: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults. Early, accurate diagnosis can reduce the rate and severity of complications. OBJECTIVE: The aim of this study was to analyse the clinical characteristics, management, and treatment of CVT in different centres in Spain. METHODS: We conducted a multicentre, retrospective, descriptive study of patients hospitalised due to CVT between 2008 and 2017 at 11 Spanish centres. RESULTS: We included 256 patients, with a mean age (SD) of 49.8 (18.7) years; 51% of patients were women. The most frequent symptoms were headache (73%), focal deficits (50%), epileptic seizures (33%), and encephalopathy (21%). The most frequent localisations were the superior sagittal sinus (12.5%), the transverse sinus (10.9%), and 2 or more sinuses or veins (66.4%). Thrombophilia was the most frequent known aetiology (24%), and was most commonly associated with the prothrombin G20210A mutation (19%). Forty-six percent of patients were treated with antithrombotics for 3-6 months, 21% for one year, and 22.6% required indefinite anticoagulation. Endovascular therapy was performed in 5% of cases, and 33% required neurosurgery. Regarding outcomes, 75% of patients were independent at 3 months (modified Rankin Scale [mRS] score ≤ 2), with papilloedema (P=.03), focal deficits (P=.001), and encephalopathy (P <.001) showing a statistically significant association with poor prognosis (mRS> 3). The in-hospital mortality rate was 4.3%, with a 3-month mortality rate of 6.3%. CONCLUSION: The diverse risk factors and variable presentation of CVT represent a challenge in the diagnosis and treatment of this condition. To improve prognosis and reduce mortality, it is essential to establish management protocols for this entity.

Coexistence of idiopathic intracranial hypertension and optic disc drusen in a latin american patient: case report.

Optic nerve drusen are acellular concretions of calcium located in the parenchyma of the optical nerve head described as the most common cause of pseudo-papilloedema, which makes it difficult to differentiate from a true optical disc oedema. Despite it being rare, the drusen of the optic nerve and the papilloedema secondary to idiopathic intracranial hypertension can coexist in the same patient. The case is presented of a 34 year-old woman referred to the Ophthalmology Department with visual discomfort, headaches, and pulsatile tinnitus of two months onset. In the physical examination there was bilateral blurring of the optic disc margin with absence of spontaneous venous pulsation. The diagnosis of idiopathic intracranial hypertension was made by lumbar puncture with the measurement of the opening pressure, and due to the findings of the magnetic resonance scan of the brain and the eye sockets. The optic nerve drusen was an incidental finding in an optical coherence tomography angiography, which would later be confirmed with an eye ultrasound. To our knowledge, this the first case reported in Latin America of the coexistence of optic nerve drusen and papilloedema secondary to idiopathic intracranial hypertension.

Alterações retinianas em pacientes com leucemia linfocítica aguda (lla): discussão de tema a partir de relato de caso / Retinal changes in patients with acute lymphocytic leukemia (all): discussion of topic from a case report

RESUMO A Leucemia Linfocitica Aguda (LLA) é uma doença caracterizada por uma alta taxa de sobrevida, porém o número absoluto de crianças que morrem por ela representa uma grande parcela dos casos de óbitos infantis por câncer. A morbidade decorrente de seu tratamento pode deixar sequelas em pessoas com grande expectativa de vida, tornando-se extremamente necessário o entendimento da patogênese desta doença, possibilitando o desenvolvimento de novos tratamentos e diminuição de sequelas provocadas pela doença. O diagnóstico precoce é importante para se evitar complicações oculares que possam levar a baixa de acuidade visual em longo prazo e para avaliação de recaídas de tratamento sendo determinante no direcionamento de condutas.

ABSTRACT The Acute Lymphocytic Leukemia (ALL) is a disease characterized by a high survival rate, but the absolute number of children who die from it represents a large proportion of cases of infant deaths from cancer. The morbidity resulting from its treatment can leave sequelae in people with high life expectancy, making it extremely necessary to understand the pathogenesis of this disease, enabling the development of new treatments and reduction of sequelae caused by the disease. This early diagnosis is important to avoid ocular complications that may lead to low long-term visual acuity and to evaluate treatment relapses and determine the conducts.

Viagem espacial: Um desafio sob o ponto de vista da oftalmologia / Space travel: A challenge from the point of view of ophthalmology

RESUMO No final do século vinte, com o surgimento de novas tecnologias e de novos programas espaciais, a medicina aeroespacial ganhou destaque no meio científico uma vez que os estudos relacionados às alterações da fisiologia humana no espaço tornaram-se cada vez mais necessário para a manutenção da saúde de cosmonautas. Os olhos são considerados uma das estruturas mais sensíveis do corpo às alterações vasculares, estruturais e bioquímicas provocadas pela microgravidade e radiação cósmica. Nesse sentido, essa revisão narrativa busca identificar e explicar as principais alterações morfológicas e funcionais que ocorrem no sistema visual em decorrência de missões espaciais.

ABSTRACT At the end of the twentieth century, with the emergence of new technologies and new space programs, aerospace medicine gained prominence in the scientific community since studies related to changes in human physiology in space have become increasingly necessary for the maintenance of cosmonaut health. The eyes are considered one of the most sensitive structures in the body to vascular, structural and biochemical changes caused by microgravity and cosmic radiation. In this sense, this narrative review seeks to identify and explain the main morphological and functional changes that occur in the visual system as a result of space missions.

Uncommon progression of toxoplasmic papillitis: patient perception and case report / Papilite por toxoplasmose ocular com evolução incomum: percepção do paciente e relato de caso

ABSTRACT Ocular toxoplasmosis frequently presents as necrotizing retinochoroiditis and, less often, as peripapillary chorioretinitis and/or papillitis. The progression from papillitis to peripapillary retinochoroiditis has been rarely described. We report the case of a 52-year-old patient living in southern Brazil, who developed papillitis in the right eye and was treated with systemic corticosteroids (prednisone 0.6 mg/kg/day and pulse therapy with methylprednisolone 15 mg/kg/day, for 3 days). After 14 days, the patient developed peripapillary retinochoroiditis with vitritis and decreased visual acuity (20/60), and was immediately initiated on the classic oral treatment for toxoplasmosis, consisting of pyrimethamine (50 mg/day), sulfadiazine (4 g/day), folinic acid (15 mg every 3 days) and prednisone (0.6 mg/kg/day). The visual acuity of the right eye normalized after treatment (20/20), which lasted approximately 70 days, but scotomas were detected on visual field examination, especially in the lower nasal quadrant. Although two studies mentioned this presentation, our report emphasizes the possible manifestation of ocular toxoplasmosis as papillitis in the initial phase, with progression to peripapillary retinochoroiditis and permanent visual field defects, which justifies early treatment for toxoplasmosis in suspected cases, especially in endemic regions.

RESUMO A toxoplasmose ocular manifesta-se com maior frequência por um quadro de retinocoroidite necrotizante e, com menor frequência, por coriorretinite justapapilar e/ou papilite. A evolução de papilite para retinocoroidite justapapilar raramente foi descrita. Apresenta-se o relato de caso de uma paciente de 52 anos, habitante da Região Sul do Brasil, que iniciou com quadro de papilite em olho direito, sendo tratada com corticoides sistêmicos (prednisona 0,6/mg/kg ao dia e pulsoterapia com metilprednisolona 15mg/kg ao dia, por 3 dias), mas, após 14 dias, evoluiu para retinocoroidite justapapilar, com vitreíte e diminuição de acuidade visual (20/60), sendo imediatamente instituído o tratamento via oral clássico para toxoplasmose, com pirimetamina (50 mg ao dia), sulfadiazina (4 g ao dia) e ácido folínico (15 mg a cada 3 dias), e mantida a prednisona (0.6 mg/kg/dia). A acuidade visual do olho direito normalizou após o tratamento (20/20), que durou em torno de 70 dias, porém desenvolveu escotomas ao exame de campo visual, sobretudo de quadrante nasal inferior. Embora tenham sido encontrados dois trabalhos que mencionam essa forma de apresentação, o presente relato destaca-se por enfatizar a possibilidade de manifestação da toxoplasmose ocular por meio de papilite na fase inicial, que evolui com retinocoroidite justapapilar, causando defeito permanente de campo visual, justificando que se avalie a instituição de tratamento precoce para toxoplasmose dos casos suspeitos, sobretudo em região endêmica.

Optic disc drusen in children: a diagnostic challenge / Drusas de disco óptico em criança um desafio diagnóstico

Abstract In children, optic disc drusen (ODD) are often mistaken for papilledema, this being the prin-cipal differential diagnosis. This report describes the case of an 11-year old patient with ODD, in which the condition was initially diagnosed as papilledema, and the patient referred for pulse therapy. Fundoscopic examination is important because it is the first examination conducted by the ophthalmologist that is capable of revealing some characteristics of ODD that will aid in the differentiation between this disease and papilledema. In cases of ODD, the optic disc presents blurred margins and elevation of the disc borders, with clearly defined vessels at the papilla border. The differential diagnosis of ODD in children is challenging and requires appropriate management and follow-up to avoid iatrogenesis.

Resumo As drusas de disco óptico (DDO) em crianças são frequentemente confundidas com papiledema, sendo este o principal diagnóstico diferencial. Este artigo relata o caso de uma paciente de 11 anos com DDO, no qual o quadro foi inicialmente diagnosticado como papiledema, e a paciente encaminhada para pulsoterapia. O exame fundoscópico é importante por ser o primeiro exame realizado pelo oftalmologista que é capaz de revelar algumas características das DDO que auxiliarão na diferenciação do papiledema. Nos casos de DDO, o disco óptico apresenta margens mal definidas e bordas elevadas, com vasos na margem da papila bem definidos. O diagnóstico diferencial das DDO em crianças é desafiador e requer conduta e seguimento adequados para evitar iatrogenias.

Uso terapéutico de la hialuronidasa y la triamcinolona en el pseudotumor orbitario / Therapeutic use of hyaluronidase and triamcinolone in orbital pseudotumor

RESUMEN El origen del pseudotumor orbitario no es del todo conocido. Se admite su naturaleza inflamatoria granulomatosa e inespecífica en diferentes localizaciones. El pseudotumor orbitario se define como una respuesta inflamatoria celular pleomórfica, que está usualmente confinado a estructuras de la órbita y tiene una evolución limitada. En este trabajo se presenta una paciente femenina de 16 años, con diagnóstico de pseudotumor orbitario corroborado por biopsia y tomografía axial computarizada, refractaria al tratamiento con esteroides sistémicos, por lo que se decide iniciar con la aplicación de hialuronidasa y triamcinolona en el espacio peribulbar. Los casos agudos casi siempre responden rápidamente al tratamiento con cortocoesteroides, como prednisona, pero debemos tener en cuenta que existen pacientes que son refractarios al tratamiento, por lo que es necesario buscar procedimientos alternativos. Una opción es el uso de hialuronidasa para destruir las uniones extracelulares, y difundir un esteroide de manera local, como la triamcinolona, más efectiva dentro del tejido inflamatorio para provocar un efecto localizado de este. A los tres meses del tratamiento hubo una regresión total del cuadro en esta paciente(AU)

ABSTRACT The exact etiology of orbital pseudotumor is unknown, but its granulomatous unspecific inflammatory nature at various locations has been recognized. Orbital pseudotumor is defined as a cellular pleomorphic inflammatory response of limited evolution often confined to orbital structures. A case is presented of a female 16-year-old patient diagnosed with orbital pseudotumor confirmed by biopsy and computerized axial tomography, refractory to treatment with systemic steroids, due to which it is decided to start treatment with hyaluronidase and triamcinolone in the peribulbar space. Acute cases often respond fast to treatment with corticosteroids such as prednisone. It should be borne in mind that there are patients who are refractory to treatment for whom alternative treatments should be sought. An option is the use of hyaluronidase to destroy extracellular junctions and locally spread a steroid such as triamcinolone, most effectively within the inflammatory tissue to ensure its localized effect. Total regression of the patient's status was observed at three months of treatment(AU)

Coexistence of papilledema and pseudopapilledema after remission of idiopathic intracranial hypertension by bariatric surgery / Coexistência de papiledema e pseudopapiledema após remissão de hipertensão intracraniana idiopática por cirurgia bariátrica

ABSTRACT A 37-year-old woman complained of headaches following bilateral visual loss in the past two years. She was obese and had undergone bariatric surgery three months earlier, followed by a considerable weight loss. Neuro-ophthalmic examination revealed a bilateral swollen optic disk. After a computerized analysis of the visual fields and magnetic resonance imaging of the brain and orbits, a diagnosis of idiopathic intracranial hypertension was made. At six months after the bariatric surgery, the patient reported no further headaches and exhibited better findings on computerized analysis of visual fields. However, fundus examination revealed persistent mild papilledema in both eyes. Ocular B-scan ultrasonography showed bilateral optic disk drusen. This report highlights the coexistence of true papilledema and pseudopapilledema due to optic disk drusen, following remission of idiopathic intracranial hypertension after a bariatric surgery.

RESUMO Uma mulher de 37 anos queixou-se de cefaleia após perda visual bilateral nos últimos dois anos. Apresentava história de obesidade e havia sido submetida à cirurgia bariátrica três meses antes, seguida de considerável perda de peso. O exame neuro-oftálmico revelou um disco óptico inchado bilateral. Após uma análise computadorizada dos campos visuais e ressonância magnética do crânio e órbitas, foi feito um diagnóstico de hiper tensão intracraniana idiopática. Após seis meses da cirurgia bariátrica, a paciente não relatou mais cefaleia e foram descobertas melhoras na análise computadorizada dos campos visuais. No entanto, o exame de fundo de olho revelou papiledema leve persistente em ambos os olhos. A ultrassonografia ocular B-scan mostrou drusas do disco óptico bilateralmente. Este relato destaca a coexistência de papiledema verdadeiro e pseudopapiledema devido à drusa de disco óptico após remissão da hipertensão intracraniana idiopática após uma cirurgia bariátrica.

Neuro-ophthalmological manifestations of POEMS syndrome. / Manifestaciones neurooftalmológicas del síndrome de POEMS.

The cases is presented of a 38 year-old male with a constitutional syndrome, fever, multiple swollen lymph nodes, and hepatosplenomegaly of 2 months onset. There was also mention of headache, bilateral blurred vision, and myiodesopsias. Best correct visual acuity was 20/50 and 20/200. The anterior segment was unremarkable. The fundus of both eyes showed raised and erased discs, accompanied by serous detachment, greater in the left eye. Complementary studies of both eyes showed a visual field with increased blind spot; fluorescein angiography indicated late disc hyperfluorescence. The electroretinogram showed compromise of cones and rods, and the visual evoked potential detected alteration in the perception and conduction of stimuli. The imaging studies were essentially normal. During his hospital admission he presented with paraparesis, renal failure, endocrinopathy, skin alterations, and osteoblastic lesions with negative biopsies. Monoclonal gammopathy was documented, and the diagnosis of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) syndrome was made. The chemotherapy started with dexamethasone/melphalan, with a good response at 3 months.

Optic nerve involvement in ocular toxoplasmosis: 12 year data from a tertiary referral center in Turkey / Acometimento de nervo óptico na toxoplasmose ocular: um relato de 12 anos de um centro de referência terciário na Turquia

ABSTRACT Purpose: To evaluate the prevalence, clinical characteristics, and types of optic nerve involvement in patients with ocular toxoplasmosis. Methods: For this retrospective cross-sectional study, we examined all patients with active ocular toxoplasmosis referred to our Uveitis Section during the last 12 years, and we included patients with optic nerve involvement in the study. The primary outcome was the prevalence of optic nerve involvement, and secondary outcomes included the types of optic nerve involvement and the final best-corrected visual acuity after treatment. Results: The prevalence of optic nerve involvement was 14.4%, with the leading cause being the activation of a juxtapapillary lesion (70.5%). We found papillitis in two eyes and neuroretinitis in two eyes (11.7% for each). We only detected one optic nerve involvement secondary to a distant active lesion (5.8%). Sixteen patients (94.1%) had unilateral ocular toxoplasmosis. The overall final best-corrected visual acuity after treatment was 10/10 (LogMAR = 0.0) excluding the three patients with a juxtapapillary scar involving the macula. Conclusions: Optic nerve involvement was common in patients with ocular toxoplasmosis. The main type of optic nerve involvement was caused by activation of an old juxtapapillary lesion. Treatment was quickly effective, but the best-corrected visual acuity was dependent on the presence of a scar in the papillomacular bundle.

RESUMO Objetivos: Avaliar a prevalência, características clínicas e tipos de acometimento do nervo óptico em pacientes com toxoplasmose ocular. Métodos: Para este estudo retrospectivo transversal, examinamos todos os pacientes com toxoplasmose ocular ativa encaminhados ao nosso Setor de Uveíte nos últimos 12 anos, e incluímos pacientes com comprometimento do nervo óptico no estudo. O resultado primário foi a prevalência do envolvimento do nervo óptico, e os resultados secundários incluíram os tipos de envolvimento do nervo óptico e a acuidade visual final melhor corrigida após o tratamento. Resultados: A prevalência de acometimento do nervo óptico foi 14,4%, sendo a principal causa a ativação de uma lesão justapapilar (70,5%). Encontramos papilite em dois olhos e neuroretinite em dois olhos (11,7% para cada um). Apenas detectamos um comprometimento do nervo óptico secundário a uma lesão ativa distante (5,8%). Dezesseis pacientes (94,1%) apresentavam toxoplasmose ocular unilateral. A acuidade visual final com melhor correção após o tratamento foi 10/10 (LogMAR= 0,0) excluindo os três pacientes com uma cicatriz justapapilar envolvendo a mácula. Conclusões: O comprometimento do nervo óptico foi comum em pacientes com toxoplasmose ocular. O principal tipo de comprometimento do nervo óptico foi causado pela ativação de uma lesão justapapilar antiga. O tratamento foi rapidamente eficaz, mas a acuidade visual final com melhor correção foi dependente da presença de uma cicatriz no feixe papilomacular.

Pseudotumour cerebri in children: Aetiology, clinical features, and progression. / Seudotumor cerebri en niños: etiología, características clínicas y evolución.

INTRODUCTION: The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. OBJECTIVES AND METHODS: Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure>25cmH2O) and absence of space-occupying lesions in brain MR images. RESULTS: A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. CONCLUSIONS: The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP.