RESUMEN
BACKGROUND: There have been limited data on idiopathic intracranial hypertension (IIH) in Asians and there remain uncertainties whether a cerebrospinal fluid (CSF) pressure of 250 mm CSF is an optimum diagnostic cutoff. The aims of the present study included (1) characterization of IIH patients in Taiwan, (2) comparisons among different diagnostic criteria for IIH, and (3) comparisons between patients with CSF pressures of > 250 and 200-250 mm CSF. METHODS: This retrospective study involved IIH patients based on the modified Dandy criteria from two tertiary medical centers in Taiwan. Clinical manifestations were retrieved from electronic medical records, and findings on ophthalmologic examination and magnetic resonance images (MRIs) were reviewed. RESULTS: A total of 102 patients (71 F/31 M, mean age 33.4 ± 12.2 years, mean CSF pressure 282.5 ± 74.5 mm CSF) were identified, including 46 (45.1%) with obesity (body-mass index ≥ 27.5), and 57 (62.6%) with papilledema. Overall, 80 (78.4%), 55 (53.9%), 51 (50.0%), and 58 (56.9%) patients met the Second and Third Edition of International Classification of Headache Disorders, Friedman, and Korsbæk criteria, respectively. Patients in the 200-250 mm CSF group (n = 40) were less likely to have papilledema (48.5% vs. 70.7%, p = 0.035), transient visual obscuration (12.5% vs. 33.9%, p = 0.005), and horizontal diplopia (10.0% vs. 30.6%, p = 0.006), and had fewer signs on MRIs (2.2 ± 1.3 vs. 2.8 ± 1.0, p = 0.021) when compared with those with CSF pressures > 250 mm CSF (n = 62). However, the percentages of patients with headache (95.0% vs. 87.1%, p = 0.109) at baseline, chronic migraine at six months (31.6% vs. 25.0%, p = 0.578), and visual field defect (86.7% vs. 90.3%, p = 0.709) were similar. CONCLUSIONS: It was found that obesity and papilledema were less common in Asian IIH patients when compared with Caucasian patients. Although patients with CSF pressures of 200-250 mm CSF had a less severe phenotype, the risks of having headache or visual loss were comparable to those in the > 250 mm CSF group. It is possible that a diagnostic cutoff of > 200 mm CSF could be more suitable for Asians, although further studies are still needed.
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Seudotumor Cerebral , Humanos , Femenino , Masculino , Adulto , Estudios Retrospectivos , Seudotumor Cerebral/epidemiología , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/diagnóstico , Taiwán/epidemiología , Pueblo Asiatico , Adulto Joven , Persona de Mediana Edad , Imagen por Resonancia Magnética , Presión del Líquido Cefalorraquídeo/fisiología , Papiledema/diagnósticoRESUMEN
BACKGROUND: Acute acquired comitant esotropia caused by prolonged near work, such as the use of digital devices, has been frequently reported in recent years. However, intracranial examination is necessary even for patients with nonparalytic comitant esotropia. Lhermitte-Duclos disease is a rare tumor that grows in layers in the cerebellum. Among those with this disease, cases of esotropia have been reported due to abduction limitation of the eye, but there have been no reports of comitant esotropia. Here, we report the case of a young woman with acute acquired comitant esotropia who was found to have Lhermitte-Duclos disease. CASE PRESENTATION: A 16-year-old Japanese female patient, whose ethnicity was Asian, was referred to our hospital for acute acquired comitant esotropia. Fundus examination revealed papilledema in both eyes, and magnetic resonance imaging of the head revealed a cerebellar tumor in the right cerebellum with obstructive hydrocephalus. She underwent partial tumor resection, and a histopathological diagnosis of Lhermitte-Duclos disease was obtained. However, comitant esotropia status remained unchanged, and she underwent strabismus surgery. Finally, diplopia disappeared completely. CONCLUSION: Neurological and intracranial imaging examinations are essential when acute acquired comitant esotropia is observed. Acute acquired comitant esotropia by Lhermitte-Duclos disease did not improve with partial tumor resection and required strabismus surgery, but good surgical results were obtained.
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Esotropía , Síndrome de Hamartoma Múltiple , Imagen por Resonancia Magnética , Humanos , Femenino , Esotropía/etiología , Esotropía/diagnóstico , Adolescente , Síndrome de Hamartoma Múltiple/complicaciones , Síndrome de Hamartoma Múltiple/diagnóstico , Síndrome de Hamartoma Múltiple/cirugía , Neoplasias Cerebelosas/complicaciones , Neoplasias Cerebelosas/cirugía , Enfermedad Aguda , Diplopía/etiología , Papiledema/etiología , Papiledema/diagnósticoRESUMEN
Bilateral edematous optic disc swelling from papilledema is caused by elevated intracranial pressure (ICP). Idiopathic intracranial hypertension (IIH), a clinical syndrome with elevated ICP of unclear etiology, is a frequent cause of this condition. IIH typically affects obese middle-aged females. Papilledema usually has a fairly symmetrical bilateral pattern. Unilateral papilledema is a rare disorder that must be detected early to avoid optic nerve damage. However, the etiology of unilateral papilledema remains unclear. Based on bilateral optic nerve sheath diameter measurements, we aimed to find an explanation for the unilaterality in this rare case.
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Nervio Óptico , Papiledema , Humanos , Papiledema/diagnóstico , Papiledema/etiología , Femenino , Nervio Óptico/patología , Nervio Óptico/diagnóstico por imagen , Disco Óptico , Adulto , Presión Intracraneal , Agudeza Visual , Tomografía de Coherencia Óptica/métodos , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/complicaciones , Imagen por Resonancia MagnéticaRESUMEN
BACKGROUND: Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a recently defined optical coherence tomography (OCT) finding. The purpose of this study was to characterize the presence of PHOMS and their visual significance in pediatric patients with and without optic nerve pathologies. METHODS: This retrospective study evaluated 400 patients (<18 years of age) including normal control subjects and patients with optic neuritis, papillitis, optic nerve head drusen (ONHD), and papilledema. Information on demographics, visual function, and structural parameters were obtained. RESULTS: PHOMS were found in 7 of 258 normal control eyes (2.7%), 9 of 59 eyes with optic neuritis (15.3%), 58 of 76 eyes with ONHD (76.3%), 3 of 11 eyes with papillitis (27.3%), and 180 of 308 eyes with papilledema (58.4%). PHOMS were more prevalent in the papilledema (P < 0.001), ONHD (P < 0.001), and optic neuritis (P = 0.028) eyes than in control eyes. We identified 5 cases where PHOMS developed de novo. This occurred over an average of 2.3 years (range, 0.2-7.4 years). Sixteen cases of PHOMS resolved over an average of 1.1 years (range, 0.3-4.0 years). Cross-sectionally, PHOMS were not associated with visual acuity (P = 0.551), retinal nerve fiber layer thickness (P = 0.068), ganglion cell volume (P = 0.375), or visual field mean deviation (P = 0.795). CONCLUSIONS: PHOMS are present in a majority of children with papilledema or ONHD. PHOMS are dynamic and may form de novo over time with optic nerve pathology and may resolve either through treatment or atrophy. There was no relationship between the presence of PHOMS and poor visual function in our study cohort.
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Drusas del Disco Óptico , Disco Óptico , Enfermedades del Nervio Óptico , Papiledema , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Estudios Retrospectivos , Tomografía de Coherencia Óptica/métodos , Niño , Femenino , Masculino , Agudeza Visual/fisiología , Adolescente , Prevalencia , Papiledema/diagnóstico , Papiledema/fisiopatología , Disco Óptico/patología , Disco Óptico/diagnóstico por imagen , Preescolar , Enfermedades del Nervio Óptico/diagnóstico , Enfermedades del Nervio Óptico/fisiopatología , Drusas del Disco Óptico/fisiopatología , Drusas del Disco Óptico/diagnóstico , Campos Visuales/fisiología , Fibras Nerviosas/patología , Células Ganglionares de la Retina/patología , Neuritis Óptica/fisiopatología , Neuritis Óptica/diagnóstico , LactanteRESUMEN
INTRODUCTION: Idiopathic intracranial hypertension (IIH) or benign intracranial hypertension is a rare disease in childhood. The clinical presentation in pediatric patients can be very variable, being more unespecific in younger patients. PATIENTS AND METHODS: A retrospective descriptive study was carried out on patients diagnosed of IIH in the last eight years (2016-2023) in the neuropediatrics unit of a tertiary hospital. In the present study, the clinical-epidemiological characteristics and the diagnostic-therapeutic procedure carried out in each case were analyzed. RESULTS: We studied 14 patients, 57% were women. The average age at diagnosis was 9 years, headache was the most common reason for consultation. In all patients, papilledema was found in the fundus and neuroimaging didn´t find alterations. Optical coherence tomography has been carried out in 78.5% of the sample, > 80% of patients showed thickening of the retinal nerve fiber layer. All patients had a high cerebrospinal fluid opening pressure (>25 cmH2O). 57% of patients required treatment with acetazolamide, a carbonic anhydrase inhibito. In all patients the resolution was complete, however almost 30% of them have presented recurrences during follow-up. CONCLUSIONS: In recent years there has been an increase in the incidence of this entity, making early diagnosis and treatment essential to avoid possible irreversible damage.
TITLE: Hipertensión intracraneal idiopática. Revisión de nuestra experiencia en los últimos ocho años (2016-2023).Introducción. La hipertensión intracraneal idiopática (HII), o hipertensión intracraneal benigna, es una enfermedad poco frecuente en la infancia. La presentación clínica en pacientes pediátricos puede ser muy variable, y es más inespecífica a menor edad. Pacientes y métodos. Se ha realizado un estudio descriptivo retrospectivo de los pacientes diagnosticados de HII en los últimos ocho años (2016-2023) en la consulta de neuropediatría de un hospital de tercer nivel. En el presente estudio se analizaron las características clinicoepidemiológicas y el procedimiento diagnosticoterapéutico llevado a cabo en cada caso. Resultados. Se estudió a 14 pacientes, de los cuales el 57% eran mujeres. La edad media en el momento del diagnóstico fue de 9 años, y la cefalea fue el motivo de consulta más habitual. En todos los pacientes se constató papiledema en el fondo de ojo y una prueba de neuroimagen sin alteraciones. Se llevó a cabo una tomografía de coherencia óptica en el 78,5% de la muestra, y >80% de los pacientes presentaba engrosamiento de la capa de fibras nerviosas retiniana. La totalidad de los pacientes presentaba una presión de apertura de líquido cefalorraquídeo elevada (> 25 cmH2O). El 57% de los pacientes precisó como tratamiento acetazolamida, un inhibidor de la anhidrasa carbónica. En todos los pacientes la resolución fue completa; sin embargo, casi el 30% de ellos presentó recurrencias durante el seguimiento. Conclusiones. En los últimos años se ha mostrado un aumento en la incidencia de esta entidad, y es fundamental un diagnóstico y un tratamiento precoces para evitar posibles secuelas irreversibles.
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Seudotumor Cerebral , Humanos , Femenino , Estudios Retrospectivos , Masculino , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/complicaciones , Niño , Adolescente , Preescolar , Acetazolamida/uso terapéutico , Factores de Tiempo , Tomografía de Coherencia Óptica , Papiledema/etiología , Papiledema/diagnósticoRESUMEN
BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare autoimmune demyelinating disorder of the central nervous system. Optic neuritis (ON) is the most common clinical manifestation of MOGAD in adults. In 2023, new MOGAD diagnostic criteria were proposed, highlighting the importance of supplemental criteria when MOG-immunoglobulin G (IgG) titers are unavailable. OBJECTIVES: To investigate the applicability of the 2023 MOGAD criteria in patients diagnosed with MOGAD and treated before the availability of MOG-IgG titers. METHODS: We conducted a retrospective chart review of patients classified as MOGAD between 2010 and 2023 at Rabin Medical Center. Patient demographics as well as clinical and imaging data were collected, including visual acuity, expanded disability status score, core demyelinating events, antibody status, and brain and optic nerve magnetic resonance imaging data. Patients fulfilling the 2023 MOGAD criteria were reported as definite MOGAD. RESULTS: Fifteen patients met the 2023 MOGAD diagnostic criteria despite lack of MOG-IgG titer. The most common supplemental criterion meeting the 2023 MOGAD criteria was optic disc edema (n=12, 80%), followed by longitudinal optic nerve involvement (53%), bilateral ON (40%), and perineural optic sheath enhancement (33%). CONCLUSIONS: All patients with a clinical diagnosis of MOG-ON in our cohort fulfilled the 2023 MOGAD criteria despite the lack of antibody titers. The 2023 MOGAD criteria can be reliably applied to Israeli cohorts, prior to availability of MOGAD IgG titers, with particular attention to additional supplemental criteria. Since the 2023 MOGAD criteria were published, MOGAD IgG titers have been added to routine testing at our facility.
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Inmunoglobulina G , Imagen por Resonancia Magnética , Glicoproteína Mielina-Oligodendrócito , Neuritis Óptica , Humanos , Glicoproteína Mielina-Oligodendrócito/inmunología , Femenino , Israel/epidemiología , Masculino , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Imagen por Resonancia Magnética/métodos , Neuritis Óptica/diagnóstico , Neuritis Óptica/inmunología , Inmunoglobulina G/sangre , Autoanticuerpos/sangre , Enfermedades Autoinmunes Desmielinizantes SNC/diagnóstico , Enfermedades Autoinmunes Desmielinizantes SNC/inmunología , Estudios de Cohortes , Anciano , Papiledema/diagnósticoRESUMEN
This case highlights the atypical presentation of Foster-Kennedy syndrome (FKS) associated with Neurocysticercosis (NCC), a prevalent cause of space-occupying lesions in areas endemic to the parasite. We report a newly diagnosed case of NCC in a 13-year-old boy who presented with a one-day history of abnormal movements of the left side of the body and no ocular complaints. Fundus examination of the patient revealed temporal disc pallor and a cup disc ratio (CDR) of 0.6 in the right eye suggesting unilateral optic disc atrophy and a hyperaemic disc with CDR 0.3 and blood vessel tortuosity in the left eye suggesting contralateral impending disc edema, mimicking the classic triad of FKS. He was diagnosed with NCC based on clinical features and radiological findings and was started on Carbamazepine (400 mg), Prednisolone (60 mg), Albendazole (400 mg), Acetazolamide (750 mg), and Vitamin B12 complex. Abbreviations: BCVA = Best Corrected Visual Acuity, CDR = Cup-Disc Ratio, CT = Computed Tomography, FKS = Foster Kennedy Syndrome, IDSA = Infectious Diseases Society of America, ICP = Intracranial Pressure, IOP = Intraocular Pressure, MRI = Magnetic Resonance Imaging, NCC = Neurocysticercosis, OOC = Orbital/Ocular Cysticercosis, OD = Right Eye, OS = Left Eye, OU = Both Eyes, RNFL = Retinal Nerve Fibre Layer, WNL = Within Normal Limits.
Asunto(s)
Neurocisticercosis , Humanos , Masculino , Neurocisticercosis/diagnóstico , Neurocisticercosis/complicaciones , Neurocisticercosis/tratamiento farmacológico , Adolescente , Imagen por Resonancia Magnética , Infecciones Parasitarias del Ojo/diagnóstico , Infecciones Parasitarias del Ojo/parasitología , Agudeza Visual , Tomografía Computarizada por Rayos X , Papiledema/diagnóstico , Papiledema/etiología , Papiledema/tratamiento farmacológico , Papiledema/parasitología , Atrofia Óptica/diagnóstico , Atrofia Óptica/etiologíaRESUMEN
BACKGROUND: Papilledema is the optic disc swelling caused by increased intracranial pressure (ICP) that can damage the optic nerve and cause subsequent vision loss. Pseudopapilledema refers to optic disc elevation without peripapillary fluid that can arise from several optic disc disorders, with optic disc drusen (ODD) being the most frequent cause. Occasionally, pseudopapilledema patients are mistakenly diagnosed as papilledema, leading to the possibility of unneeded procedures. We aim to thoroughly examine the most current evidence on papilledema and pseudopapilledema causes and several methods for distinguishing between both conditions. METHODS: An extensive literature search was conducted on electronic databases including PubMed and google scholar using keywords that were relevant to the assessed pathologies. Data were collected and then summarized in comprehensive form. RESULTS: Various techniques are employed to distinguish between papilledema and pseudopapilledema. These techniques include Fundus fluorescein angiography, optical coherence tomography, ultrasonography, and magnetic resonance imaging. Lumbar puncture and other invasive procedures may be needed if results are suspicious. CONCLUSION: Papilledema is a sight-threatening condition that may lead to visual affection. Many disc conditions may mimic papilledema. Accordingly, differentiation between papilledema and pseudopailledema is crucial and can be conducted through many modalities.
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Enfermedades Hereditarias del Ojo , Angiografía con Fluoresceína , Disco Óptico , Papiledema , Tomografía de Coherencia Óptica , Papiledema/diagnóstico , Humanos , Diagnóstico Diferencial , Tomografía de Coherencia Óptica/métodos , Angiografía con Fluoresceína/métodos , Disco Óptico/diagnóstico por imagen , Disco Óptico/patología , Enfermedades Hereditarias del Ojo/diagnóstico , Enfermedades del Nervio Óptico/diagnóstico , Fondo de Ojo , Imagen por Resonancia Magnética/métodos , Drusas del Disco Óptico/diagnóstico , Drusas del Disco Óptico/fisiopatología , Drusas del Disco Óptico/complicacionesAsunto(s)
Astrocitoma , Angiografía con Fluoresceína , Hamartoma , Papiledema , Esclerosis Tuberosa , Humanos , Esclerosis Tuberosa/complicaciones , Esclerosis Tuberosa/diagnóstico , Papiledema/diagnóstico , Papiledema/etiología , Hamartoma/diagnóstico , Hamartoma/complicaciones , Astrocitoma/complicaciones , Astrocitoma/diagnóstico , Enfermedades de la Retina/diagnóstico , Enfermedades de la Retina/etiología , Fondo de Ojo , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/diagnóstico , Astrocitos/patología , Femenino , Masculino , Imagen por Resonancia MagnéticaRESUMEN
Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological condition characterised by a raised intracranial pressure and papilloedema that causes disabling headaches. The main risk factors of female sex and living with obesity have been known for some time, however the knowledge of the underlying pathophysiology is evolving. Papilloedema can impact the visual function, and the majority of people are offered acetazolamide. Those with sight threatening disease need urgent management, though there is little high quality evidence to recommend any particular surgical intervention. Headache treatment is an unmet clinical need and simple medication overuse advice has the potential to reduce the chronification of migraine-like headaches. IIH is emerging as a systemic metabolic disease distinct from people living with obesity alone. While weight loss is the main stay of disease modifying therapy this is challenging to access and many healthcare professionals that manage the condition have no formal training or accessible pathways for weight management. The aim of this "how to do it" article is to present the latest advances in knowledge of IIH that we pragmatically included in routine clinical care for people living with the condition.
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Papiledema , Seudotumor Cerebral , Humanos , Seudotumor Cerebral/fisiopatología , Seudotumor Cerebral/terapia , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/complicaciones , Papiledema/diagnóstico , Papiledema/fisiopatología , Papiledema/terapia , Papiledema/etiología , Acetazolamida/uso terapéutico , Factores de Riesgo , Obesidad/complicaciones , Obesidad/fisiopatología , Inhibidores de Anhidrasa Carbónica/uso terapéutico , Manejo de la EnfermedadRESUMEN
A 71-year-old woman developed sudden, painful, decreased vision in the left eye accompanied by progressive instability. Initial examination revealed left optic disc edema, and macular optical coherence tomography confirmed the presence of intraretinal and subretinal fluid, as well as hyperreflective material under the retinal pigment epithelium. Subsequent investigations, including brain magnetic resonance imaging and a comprehensive serological analysis, ruled out infectious and autoimmune causes, further complicating the diagnostic picture. The patient's vision in both eyes continued to deteriorate, prompting empirical corticosteroid treatment. While the vision improved, the case took an unexpected turn with worsening neurological symptoms. Ultimately a brain biopsy was consistent with diffuse large B-cell lymphoma.
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Neuropatía Óptica Isquémica , Tomografía de Coherencia Óptica , Humanos , Femenino , Anciano , Neuropatía Óptica Isquémica/diagnóstico , Tomografía de Coherencia Óptica/métodos , Imagen por Resonancia Magnética , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/complicaciones , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/complicaciones , Papiledema/diagnóstico , Papiledema/etiología , Diagnóstico Diferencial , Agudeza VisualRESUMEN
PURPOSE: To report clinical and imaging features of optic nerve and retinal involvement in a patient with mucopolysaccharidosis (MPS) type II B. METHODS: A 27-year-old man, diagnosed with MPS type II B and undergoing enzymatic substitution therapy for the past 19 years, was referred to the retina service. An ophthalmological evaluation, which included multimodal imaging, was conducted to investigate potential retinal and optic disc involvement. RESULTS: The eye examination revealed a pigmentary retinopathy with a predominant loss of the outer retinal loss, primarily in the parafoveal and perifoveal regions. Notably, multimodal imaging identified macular edema without any signs of leakage, implying an association between macular edema and retinal neurodegeneration. Additionally, both eyes exhibited an optic disc with blurred margins. CONCLUSION: We herein describe the multimodal imaging findings of retinal and optic disc involvement in a patient with MPS type II B. This report describes for the first-time the presence of macular edema without leakage alongside photoreceptor damage and optic disc swelling.
Asunto(s)
Angiografía con Fluoresceína , Edema Macular , Mucopolisacaridosis II , Disco Óptico , Papiledema , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Masculino , Adulto , Edema Macular/diagnóstico , Edema Macular/etiología , Papiledema/diagnóstico , Papiledema/etiología , Angiografía con Fluoresceína/métodos , Disco Óptico/patología , Disco Óptico/diagnóstico por imagen , Mucopolisacaridosis II/complicaciones , Mucopolisacaridosis II/diagnóstico , Mucopolisacaridosis II/tratamiento farmacológico , Retinitis Pigmentosa/diagnóstico , Retinitis Pigmentosa/complicaciones , Imagen Multimodal , Fondo de OjoRESUMEN
BACKGROUND: Recently, diagnostic criteria including a standardized MRI criterion were presented to identify patients suffering from idiopathic intracranial hypertension (IIH) proposing that IIH might be defined by two out of three objective findings (papilledema, ≥ 25 cm cerebrospinal fluid opening pressure (CSF-OP) and ≥ 3/4 neuroimaging signs). METHODS: To provide independent external validation, we retrospectively applied the proposed diagnostic criteria to our cohort of patients with clinical suspicion of IIH from the Vienna IIH database. Neuroimaging was reevaluated for IIH signs according to standardized definitions by a blinded expert neuroradiologist. We determined isolated diagnostic accuracy of the neuroimaging criterion (≥ 3/4 signs) as well as overall accuracy of the new proposed criteria. RESULTS: We included patients with IIH (n = 102) and patients without IIH (no-IIH, n = 23). Baseline characteristics were balanced between IIH and no-IIH groups, but papilledema and CSF-OP were significantly higher in IIH. For the presence of ≥ 3/4 MRI signs, sensitivity was 39.2% and specificity was 91.3% with positive predictive value (PPV) of 95.2% and negative predictive value (NPV) 25.3%. Reclassifying our cohort according to the 2/3 IIH definition correctly identified 100% of patients without IIH, with definite IIH and suggested to have IIH without papilledema by Friedman criteria, respectively. CONCLUSION: The standardized neuroimaging criteria are easily applicable in clinical routine and provide moderate sensitivity and excellent specificity to identify patients with IIH. Defining IIH by 2/3 criteria significantly simplifies diagnosis without compromising accuracy.
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Imagen por Resonancia Magnética , Seudotumor Cerebral , Humanos , Femenino , Imagen por Resonancia Magnética/normas , Imagen por Resonancia Magnética/métodos , Masculino , Adulto , Seudotumor Cerebral/diagnóstico por imagen , Seudotumor Cerebral/diagnóstico , Estudios Retrospectivos , Sensibilidad y Especificidad , Persona de Mediana Edad , Papiledema/diagnóstico por imagen , Papiledema/diagnósticoAsunto(s)
Papiledema , Humanos , Papiledema/diagnóstico , Papiledema/etiología , Anciano , Femenino , Masculino , Anciano de 80 o más AñosRESUMEN
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.
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Hipertensión Intracraneal , Papiledema , Seudotumor Cerebral , Humanos , Seudotumor Cerebral/diagnóstico , Seudotumor Cerebral/etiología , Seudotumor Cerebral/terapia , Hipertensión Intracraneal/complicaciones , Cefalea/diagnóstico , Cefalea/etiología , Cefalea/terapia , Trastornos de la Visión/terapia , Síndrome , Papiledema/complicaciones , Papiledema/diagnósticoRESUMEN
A 60-year-old man presented to an outside ophthalmology clinic with 1 month of progressive vision loss in the right eye (OD). Right optic disc edema was noted. Brain and orbit magnetic resonance imaging revealed right optic nerve and left occipital lobe enhancement. He was seen initially by neurology and neurosurgery and subsequently referred to neuro-ophthalmology for consideration of optic nerve biopsy. He was seen 3 months after his initial symptom onset where vision was light perception OD and a relative afferent pupillary defect with optic nerve edema. OS was unremarkable. A lumbar puncture with flow cytometry was negative for multiple sclerosis and lymphoma. At his oculoplastic evaluation for optic nerve biopsy, his vision was noted to be no light perception OD. Optic nerve biopsy demonstrated non-caseating granulomatous inflammation consistent with neurosarcoidosis. The patient was started on high-dose oral steroids with improvement of disc edema, as well as significant improvement in optic nerve and intracranial parenchymal enhancement, although his vision never improved.
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Imagen por Resonancia Magnética , Papiledema , Sarcoidosis , Humanos , Masculino , Persona de Mediana Edad , Imagen por Resonancia Magnética/métodos , Papiledema/diagnóstico , Papiledema/etiología , Sarcoidosis/diagnóstico , Sarcoidosis/complicaciones , Enfermedades del Sistema Nervioso Central/diagnóstico , Biopsia , Nervio Óptico/patología , Nervio Óptico/diagnóstico por imagen , Glucocorticoides/uso terapéutico , Enfermedades del Nervio Óptico/diagnóstico , Enfermedades del Nervio Óptico/etiología , Agudeza VisualRESUMEN
We report the case of an 8-year-old boy who presented with an optic disk pit and subsequently developed optic disk pit maculopathy, consisting of cystoid retinal edema in the peripapillary space and in the papillomacular bundle, which slowly and spontaneously resolved without intervention.
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Disco Óptico , Remisión Espontánea , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Masculino , Niño , Disco Óptico/anomalías , Disco Óptico/diagnóstico por imagen , Agudeza Visual/fisiología , Angiografía con Fluoresceína/métodos , Edema Macular/diagnóstico , Edema Macular/etiología , Anomalías del Ojo/diagnóstico , Anomalías del Ojo/complicaciones , Enfermedades de la Retina/diagnóstico , Papiledema/diagnóstico , Papiledema/etiologíaRESUMEN
BACKGROUND: Idiopathic intracranial hypertension (IIH) occurs more frequently in obese females of childbearing age. A link between eating disorders and poor outcome has been suggested but remains unproven. METHODS: This prospective field study at two tertiary headache centers included patients with clinically suspected IIH after standardized diagnostic work-up. Eating disorders were evaluated using validated questionnaires (EDQs). Primary outcome was the impact of eating disorders on IIH severity and outcome, secondary outcome was the prevalence and type of eating disorders in IIH compared to controls. RESULTS: We screened 326 patients; 143 patients replied to the EDQs and were classified as 'IIH' or 'non-IIH' patients. The demographic profile of EDQ-respondents and non-respondents was similar. Presence of an eating disorder did not impact IIH severity (lumbar puncture opening pressure (p = 0.63), perimetric mean deviation (p = 0.18), papilledema (Frisén grad 1-3; p = 0.53)) nor IIH outcome (optic nerve atrophy (p = 0.6), impaired visual fields (p = 0.18)). Moreover, we found no differences in the prevalence and type of eating disorders when comparing IIH with non-IIH patients (p = 0.09). CONCLUSION: Eating disorders did not affect IIH severity or outcome. We found the same prevalence and distribution pattern of eating disorders in IIH and non-IIH patients advocating against a direct link between IIH and eating disorders.