The Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.

Severe papilloedema with vision loss secondary to Guillain-Barré syndrome.

We discuss a patient who presented with bilateral VI and VII cranial nerve palsies, symmetric upper and lower limb weakness and areflexia, 2 weeks following an flu-like illness. At presentation, there was no papilloedema, and her visual function was normal. Cerebrospinal fluid analysis and electrophysiology supported the diagnosis of Guillain-Barré Syndrome (GBS). She received intravenous immunoglobulins. She subsequently developed headaches and vision loss. Funduscopy demonstrated severe papilloedema with visual acuity of 6/18 right eye, 6/12 left eye with bitemporal visual field depression. Lumbar puncture revealed elevated opening pressure with high protein and normal cell count. She received acetazolamide. There was resolution of papilloedema and normal visual function at 3 months. Of note, the patient's body mass index was 17 kg/m2Our case highlights the rare occurrence of papilloedema in GBS, reiterating the importance of performing funduscopy on patients with any neurological diagnosis. Early detection and prompt management of papilloedema can prevent permanent vision loss.

Surgical management of pseudotumor cerebri syndrome: A single center experience with endoscopic optic nerve decompression and CSF diversion procedures.

OBJECTIVES: Pseudotumor cerebri syndrome (PTC) is a chronic disorder, which is initially treated by conservative measures, yet surgery is inevitable in case of progressive worsening of vision or headache despite medical treatment. The surgical management is controversial including CSF diversion procedures and optic decompression. The purpose of this study was to evaluate the efficacy of different surgeries in PTC and to present surgical outcomes in a single center. METHODS: This retrospective study included the patients with PTC who were operated by endoscopic optic nerve decompression (EOND), ventriculoperitoneal (VP) and lumboperitoneal (LP) shunting. Surgical outcomes, i.e. visual acuity, visual field, papilledema and headache were compared according to type of surgery. Surgical complications were noted. RESULTS: Seventeen of 36 patients were treated with shunting, 14 with EOND and 5 patients with both EOND and shunting. No statistical significance was observed between CSF diversion procedures and EOND concerning clinical outcomes. The improvement rate of papilledema was higher with VPS (p = 0574) while more patients benefitted from LPS regarding visual field and acuity (p = 0471 and p = 0718, respectively). The best treatment response for headache was in shunt implemented patients (VPS and LPS) with a rate of 88.2% followed by EOND (78.6%) and both surgeries (60%)(p = 0.294). Gender and BMI were significant predictors of improved papilledema (p = 0.034). CONCLUSIONS: Our study demonstrated comparable results between shunting and EOND, regarding the efficacy on surgical outcomes. EOND is quite effective for headache besides its advantage on treatment-refractory visual loss. Shunting may offer sustained relief of symptoms when compared with EOND.

Systemic lupus erythematosus with chronic persistent intracranial hypertension: A case report.

OBJECTIVE: The aim is to investigate the clinical characteristics of systemic lupus erythematosus with intracranial hypertension. METHODS: The clinical characteristics of one case of systemic lupus erythematosus with chronic persistent intracranial hypertension were analyzed, and related literature was reviewed by searching Medline and Wanfang databases. RESULTS: Intracranial hypertension in SLE patients may occur at the onset or during the course of the disease. Our patient was diagnosed with IH 3 years after the onset of SLE. Headache and papilledema were the most common symptoms of intracranial hypertension, followed by nausea or vomiting, vision changes, and cerebral palsy. Our patient had a headache and cranial hypertension that lasted for years, but no papilledema was found. Corticosteroid is currently the mainstay of the treatment of IIH in patients with SLE, and immunosuppressive agents, acetazolamide, intravenous mannitol and furosemide are also used. However, our patient did not respond to these treatments and presents the characteristics of chronic persistent intracranial hypertension. CONCLUSION: Systemic lupus erythematosus with intracranial hypertension is a rare manifestation of SLE, which is not completely parallel to SLE activity. Headache and papilledema were the most common presenting symptoms. Different from previous reported cases, our patient had poor response to treatments, showing chronic and persistent characteristics.

Coexistencia de hipertensión intracraneal idiopática y drusas del nervio óptico en una paciente latinoamericana: reporte de caso / Coexistence of idiopathic intracranial hypertension and optic disc drusen in a latin american patient: case report

Las drusas del nervio óptico son concreciones acelulares de calcio localizadas en el parénquima de la cabeza del nervio óptico, descritas como la causa más frecuente de pseudopapiledema, lo cual las hace difícil de diferenciar de un verdadero edema del disco óptico. A pesar de su poca frecuencia, las drusas del nervio óptico y el papiledema secundario a una hipertensión intracraneal idiopática pueden coexistir en el mismo paciente. Reportamos el caso de una mujer de 34 años de vida remitida al servicio de oftalmología con molestias visuales, cefalea, y tinnitus pulsátil de dos meses de evolución; al examen físico se encontró borramiento del margen del disco óptico de manera bilateral sin pulsación venosa espontánea. El diagnóstico de hipertensión intracraneal idiopática se realizó por una punción lumbar con medición de la presión de apertura, y por los hallazgos de resonancia magnética cerebral y de órbitas. Se observó incidentalmente las drusas del nervio óptico en una tomografía de coherencia óptica con angiografía, que luego se confirmarían con una ecografía ocular. Este es el primer caso reportado en América Latina de la coexistencia de drusas del nervio óptico y papiledema secundario a hipertensión intracraneal idiopática para nuestro conocimiento (AU)

Optic nerve drusen are acellular concretions of calcium located in the parenchyma of the optic 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 optic nerve drusen and the papilloedema secondary to idiopathic intracranial hypertension can coexist in the same patient. We present a case 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 orbits. 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 (AU)

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.

Membrana neovascular yuxtapapilar asociada a drusas del nervio óptico / Juxtapapillary neovascular membrane associated with optic nerve drusen

CASO CLÍNICO: Paciente de 14 años de edad concurre refiriendo una disminución progresiva de la agudeza visual del ojo izquierdo de 3 meses de evolución. Al examen presenta drusas de papila bilateral, asociadas a membrana neovascular yuxtapapilar, que comprometen seriamente la visión y el campo visual del ojo izquierdo. RESULTADO: El tratamiento con 3 inyecciones consecutivas de ranibizumab intravítreo resultó en la inactivación de la membrana neovascular con reabsorción de líquido subretiniano y mejora de la agudeza visual mejor corregida del ojo izquierdo. Después de 9 meses de seguimiento, esta fue 20/20 y estable. CONCLUSIÓN: Si bien las drusas de la cabeza del nervio óptico son consideradas benignas, las membranas neovasculares pueden ser una complicación. Los anti-VEGF son una alternativa eficaz para el tratamiento

CLINICAL CASE: Forteen year old patient presenting progressive decrease in visual acuity of the left eye after 3 months of evolution. On examination he presents bilateral drusen of papilla, associated with juxtapapillary neovascular membrane, which seriously compromises the vision and visual field of the left eye. RESULT: Treatment with 3 consecutive injections of intravitreal ranibizumab resulted in the inactivation of the neovascular membrane with reabsorption of subretinal fluid and improvement of the best corrected visual acuity of the left eye. After 9 months of follow-up, it was 20/20 and stable. CONCLUSION: Although optic nerve head drusen are considered benign, neovascular membranes can be a complication. Anti-VEGFs are an effective alternative for treatment

Neurorretinitis estrellada idiopática de Leber: conflictos diagnósticos y terapéuticos / Leber's idiopathic stellate neuroretinitis: diagnostic and therapeutic conflicts

Presentamos 3 pacientes con clínica de disminución de agudeza visual, papilitis y desarrollo de estrella macular al cabo de unas semanas. En todos ellos, las pruebas complementarias arrojaron resultados irrelevantes. Dado este contexto, fueron diagnosticados de neurorretinitis estrellada idiopática de Leber (NEIL) y se pautó tratamiento con corticoides, así como antibióticos en 2de ellos. Evolucionaron favorablemente, aunque con signos de atrofia papilar en los ojos afectos. El diagnóstico de la neurorretinitis estrellada idiopática de Leber puede resultar complejo, dado el amplio espectro de enfermedades que descartar. A esto se suma un período de tiempo en el que puede no haber aparecido la estrella macular, motivo que no debe hacer excluir su diagnóstico. El uso de antibióticos o corticoides como medida terapéutica es controvertido dado su buen curso clínico, aunque parece razonable en casos severos. Otro punto controvertido es el uso rutinario de pruebas complementarias, principalmente aquellas que pueden resultar agresivas o caras, que deben ser utilizadas de forma racional

Three cases of patients with decreased visual acuity and papillitis at onset with subsequent macular star development after a few weeks are presented. Complementary tests were unremarkable in all included patients. Based on this clinical context, they were diagnosed with Leber's idiopathic stellate neuroretinitis, were treated with corticosteroids, as well as with antibiotics in 2 cases. All patients showed favourable outcomes, although signs of papillary atrophy were observed in the affected eyes. Leber's idiopathic stellate neuroretinitis diagnosis can be challenging due to the wide spectrum of conditions that have to be ruled out. In addition, macular star may appear later on, which should not exclude its diagnosis. Use of antibiotics and/or corticosteroids is controversial considering its benign nature, but should be considered in selected severe cases. Moreover, the routine use of complementary tests should be carefully evaluated, mainly those that can be aggressive and/or expensive, which should be rationally used

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

Introducción: El síndrome de hipertensión intracraneal idiopática o seudotumor cerebri (STC) en niños está en constante revisión, respecto a su definición, etiologías asociadas, diagnóstico y terapéutica más apropiada. Objetivos y métodos: Se revisaron los casos de STC < 15 años de edad en un hospital de referencia en los últimos 12 años. Se estudiaron las características clínico-epidemiológicas y el procedimiento diagnóstico-terapéutico empleado. Se definió STC como presión intracraneal > 25cmH2O por punción lumbar (PL), con estudio de resonancia magnética cerebral sin lesión ocupante de espacio. Resultados: Se registró a 12 niños con STC, media de edad de 10 años, 90% mujeres. Todos presentaban peso normal. El 82% manifestaba síntomas: cefalea (66%), diplopía (8%) o baja visión (8%). Todos asociaban papiledema (17% unilateral). La PL fue diagnóstica en el 100% y la neuroimagen fue normal en el 91%. Se evidenció un posible desencadenante en 5 casos (2 farmacológico y 3 infeccioso por Mycoplasma pneumoniae [M. pneumoniae]). El 91% recibió tratamiento médico: en el 75% consistió en PL repetidas y en el 42% solo acetazolamida y/o prednisona. La evolución fue favorable en todos ellos. Conclusiones: La incidencia de STC fue de aproximadamente 1/100.000 niños/año, similar a estudios previos. En esta población, el sobrepeso no es un factor de riesgo. La infección por M. pneumoniae podría actuar como desencadenante de STC y favorecer recurrencias tardías. La ausencia de síntomas parece independiente del grado de presión intracraneal. El tratamiento con acetazolamida es eficaz en la mayoría de los casos, desterrando el uso de PL repetidas

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> 25 cmH2O) 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

Infiltración leucémica y retinitis por citomegalovirus en paciente con leucemia linfoblástica aguda tipo T en remisión completa / Leukaemic infiltration and cytomegalovirus retinitis in a patient with acute T-cell lymphoblastic leukaemia in complete remission

CASO CLÍNCO:Mujer de 43 años con leucemia linfoblástica T en remisión completa, remitida por sospecha de necrosis retiniana herpética/retinitis leucémica en el ojo izquierdo (OI). La agudeza visual era de unidad y el fondo de ojo presentaba retinitis y hemorragias en periferia. Ante estudio hematológico negativo, recibió tratamiento por retinitis por citomegalovirus. Tras mejoría inicial, aparece papilitis en el OI y restricción de la motilidad en el ojo derecho. La resonancia y punción lumbar confirman la recidiva leucémica. DISCUSIÓN: La afectación ocular puede preceder a la recaída hematológica, por eso debe sospecharse ante sintomatología ocular. Además, son frecuentes las infecciones oportunistas en inmunodeprimidos

CLINICAL CASE: A 43-year-old woman in remission from T- cell acute lymphoblastic leukaemia was referred to our hospital with suspected leukaemic retinitis. The funduscopic examination of her left eye revealed multifocal yellow-white peripheral retinitis and retinal haemorrhage. The patient was treated for cytomegalovirus retinitis after an extended haematological investigation showed no abnormalities. Initial improvement was followed by papillitis in the left eye and motility restriction in the right eye. Magnetic resonance and lumbar puncture confirmed leukaemia relapse. Specific treatment was initiated with complete resolution. DISCUSSION: Ocular involvement may precede haematological leukaemia relapse. Physicians should be alerted when ocular symptoms appear in these cases

Alteraciones visuales como signo de neurosífilis precoz. Dos casos de neurosífilis / Visual disturbances as a sign of early neurosyphilis. Two cases of neurosyphilis

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Papiledema secundario a trombosis del seno sagital superior. Cuadro paraneoplásico de un linfoma del manto / Papilledema secondary to a superior sagittal sinus thrombosis. Mantle cell lymphoma paraneoplastic syndrome

CASO CLÍNICO: Se presenta el caso de un varón de 46 años con disminución de la visión del ojo izquierdo de meses de evolución. Se diagnostica de papiledema por trombosis del seno sagital superior gracias a la angiorresonancia. En busca de la etiología de la trombosis se descubre un linfoma del manto. DISCUSIÓN: La trombosis venosa cerebral es una causa poco frecuente de papiledema. Puede deberse a cuadros de hiperviscosidad en el contexto de un síndrome paraneoplásico

CLINICAL CASE: A 46 year old patient presented with visual loss in the left eye during the previous months. Ophthalmoscopic examination and magnetic resonance angiography found the presence of papilledema due to thrombosis in superior sagittal sinus. The examination findings revealed a mantle cell lymphoma. DISCUSSION: Cerebral venous thrombosis is an unusual cause of papilledema. This type of thrombosis may be secondary to hyper-viscosity within a context of a paraneoplastic síndrome

De la tortuosidad vascular fisiológica a la tortuositas vasorum retinae / From physiological vascular tortuosity to the tortuositas vasorum retinae

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Canal de Cloquet (II) / Cloquet's canal (II)

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Papiledema secundario a linfoma de Burkitt / Papilledema secondary to Burkitt lymphoma

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Neuropatía optica isquémica anterior no arterítica como primera manifestación de síndrome antifosfolipídico en un paciente joven / Non-arteritic anterior ischemic optic neuropathy as first manifestation of antiphospholipid syndrome in a young patient

CASO CLÍNICO: Se presenta el caso clínico de un paciente joven con neuropatía óptica anterior isquémica unilateral sin factores de riesgo cardiovasculares conocidos y agudeza visual conservada con anticuerpos anticardiolipina positivos como único hallazgo. DISCUSIÓN: El inicio clínico como neuropatía isquémica anterior no arterítica en el síndrome antifosfolipídico es un hallazgo infrecuente, pero debe tenerse en cuenta en el diagnóstico de la neuropatía óptica anterior isquémica atípicap

CASE REPORT: We report the case of a young patient with unilateral anterior ischemic optic neuropathy, with no known cardiovascular risk factors and visual acuity preserved with positive anticardiolipin antibodies as a unique find. DISCUSSION: Non-arteritic anterior ischemic optic neuropathy in the context of antiphospholipid syndrome is an uncommon finding, but it must be considered in the diagnosis of the atypical anterior ischemic optic neuropathy

Ranibizumab intravítreo para el tratamiento de la membrana neovascular peripapilar secundaria a papiledema por hipertensión endocraneana idiopática: informe de un caso / Intravitreal ranibizumab for peripapillary neovascular membrane associated to papiledema due to idiopathic intracranial hypertension: a case report

Las membranas neovasculares peripapilares son una complicación rara del papiledema crónico, y son aún más infrecuentes en el contexto del papiledema por hipertensión endocraneana idiopática (HEI). Dada su escasez, es difícil plantear estudios para definir la alternativa terapéutica más adecuada. Se presenta el caso de una mujer con papiledema por HEI quien desarrolló membrana neovascular peripapilar, y cuyo tratamiento con ranibizumab intravítreo fue exitoso.

Peripapillary neovascular membrane is a rare complication of chronic papiledema. When the latter is due to idiopathic intracranial hypertension, neovascularization is even rarer. Because of this low occurrence, it is difficult to design studies to define the most appropriate treatment. We report the case of a woman who developed papiledema by idiopathic intracranial hypertension with peripapillary neovascular membrane, and who was successfully treated with intravitreal ranibizumab.

Papiledema bilateral como primera manifestación de una carcinomatosis meníngea / Bilateral papilledema as first sign of a meningeal carcinomatosis

Introducción. La carcinomatosis meníngea (CM) es una complicación rara de las neoplasias en estadios avanzados. El 5% de las neoplasiasse presentan como una CM. Clínicamente se manifiestan con diplopía, papiledema y/o déficit visual. Ante su sospecha es necesario la realización de una resonancia magnética nuclear y una punción lumbar para su diagnóstico. Caso clínico. Paciente de 54 años diagnosticado de adenocarcinoma de próstata con oscurecimientos visuales y papiledema como primera manifestación de una carcinomatosis meníngea. Discusión. Se discute como proceder ante un edema de papila bilateral secundario a hipertensión intracraneal. Conclusiones. Se resalta la importancia de considerar la posibilidad de invasión meníngea de una neoplasia, cuando nos encontramos un paciente con déficit neurológico y/o visual sin datos de infección o lesión ocupante de espacio (AU)

Introduction. Meningeal carcinomatosis (MC) is a rare complication of late-stage tumors. The 5% of the tumors are presented as a MC. Clinically it was manifested with diplopia, papilledema and / or visual deficits. MC is usually diagnosed by magnetic resonance imaging and cerebrospinal fluid analysis. Case report. A 54-year old man with prostate adenocarcinoma,presented to the emergency service with papilledema, and visual obscurations as first manifestation of meningeal carcinomatosis. Discussion. We discuss how to proceed before a bilateral papilledema due to intracranial hypertension. Conclusions. The importance of considering the possibility of a malignant meningeal invasion, when we find a patient with neurologicaland / or visual deficits without evidence of infection or space occupying lesion (AU)

Pseudotumor cerebral en dos pacientes pediátricos / Pseudotumor Cerebri on two pediatric patients

Antecedentes: El pseudotumor cerebral es una condición que se caracteriza por presión intracraneal elevada en ausencia de manifestaciones clínicas, evidencia radiológica o laboratorial de lesión ocupativa. Es una entidad clínica bien definida en adultos, pero puede afectar a niños de cualquier edad. Casos clínicos: Paciente 1: Femenino de 8 años con cuadro de cefalea generalizada y vómitos dos días después, presenta desviación nasal de la mirada del ojo derecho, paresia bilateral de los rectos externos, edema bilateral de la papila óptica grado III. Campimetría computada reportó discreto escotoma superior en ojo izquierdo y nasal en ojo derecho. Se realizó punción lumbar donde se registró una presión de apertura de 45 cm de agua. Paciente 2: femenino de 11 años, con historia de un año de evolución de cefalea frontal intermitente, pulsátil y disminución de la agudeza visual referida por la paciente;a la evaluación neurológica edema bilateral de la papila óptica grado III. En la punción lumbar se registró inicialmente una presión de apertura de 43 cm de agua. Ambos casos fueron tratados con acetazolamida oral, con buena respuesta clínica y normalización de la presión intracraneal.Conclusión: el pseudotumor cerebral es una enfermedad infrecuente en pediatría que requiere de su identificación y manejo oportuno con el fin de evitar una lesión visual irreversible...

Síndrome de cefalea y déficits neurológicos transitorios con linfocitosis en líquido cefalorraquídeo asociado a papiledema / Syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis associated to papilloedema

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