RESUMEN
Primitive neuroectodermal tumors (PNET) of the central nervous system (CNS) are a heterogeneous group of embryonal malignancies that are composed of undifferentiated or poorly differentiated neuroepithelial cells. Supratentorial PNET is the second most common CNS embryonal malignancy in children, but it is rare in adults. We report the case of a 31-year-old woman with bilateral vision loss and a bitemporal hemianopia. Neuroimaging revealed a suprasellar mass, and pathology was consistent with PNET. After surgical debulking of the tumor followed by radiation therapy and chemotherapy, the patient had significant visual recovery and remained stable over 14 months of follow-up.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Tumores Neuroectodérmicos Primitivos/diagnóstico , Trastornos de la Visión/etiología , Agudeza Visual , Adulto , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/cirugía , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Tumores Neuroectodérmicos Primitivos/complicaciones , Tumores Neuroectodérmicos Primitivos/cirugía , Procedimientos Neuroquirúrgicos/métodos , Trastornos de la Visión/diagnóstico , Trastornos de la Visión/fisiopatologíaRESUMEN
A 75-year-old woman with new onset headaches and left vision loss, temporal scalp tenderness, and jaw claudication was found to have biopsy-proven giant cell arteritis (GCA). Despite treatment and improvement with prednisone, she later developed left orbital apex syndrome, and an orbital biopsy revealed aspergillosis. After antifungal treatment, extraocular motility improved although vision in the left eye remained no light perception. Clinicians should be aware that fungal orbital apex disease may mimic or complicate steroid-treated GCA.
Asunto(s)
Aspergilosis/complicaciones , Infecciones Fúngicas del Ojo/complicaciones , Arteritis de Células Gigantes/complicaciones , Enfermedades Orbitales/complicaciones , Anciano , Aspergilosis/diagnóstico , Biopsia , Infecciones Fúngicas del Ojo/diagnóstico , Infecciones Fúngicas del Ojo/microbiología , Femenino , Arteritis de Células Gigantes/diagnóstico , Humanos , Órbita/microbiología , Enfermedades Orbitales/diagnóstico , Enfermedades Orbitales/microbiología , Arterias Temporales/patología , Tomografía Computarizada por Rayos XRESUMEN
Central nervous system involvement from chronic lymphocytic leukemia (CLL) occurs infrequently, and manifestations include cognitive and cerebellar dysfunction and cranial nerve palsies. We report a 45-year-old man with CLL believed to be in clinical remission, who presented with vision loss and bilateral optic disc edema. His optic neuropathy due to CLL was proven by optic nerve sheath biopsy, and he experienced visual recovery after treatment with ibrutinib and intrathecal methotrexate.
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Leucemia Linfocítica Crónica de Células B/complicaciones , Nervio Óptico/patología , Papiledema/etiología , Adenina/análogos & derivados , Antimetabolitos Antineoplásicos/uso terapéutico , Biopsia , Quimioterapia Combinada , Humanos , Leucemia Linfocítica Crónica de Células B/diagnóstico , Leucemia Linfocítica Crónica de Células B/tratamiento farmacológico , Imagen por Resonancia Magnética , Masculino , Metotrexato/uso terapéutico , Persona de Mediana Edad , Papiledema/diagnóstico , Papiledema/tratamiento farmacológico , Piperidinas , Pirazoles/uso terapéutico , Pirimidinas/uso terapéutico , Punción EspinalRESUMEN
Papillary thyroid carcinoma (PTC) is a type of well-differentiated thyroid cancer that accounts for the majority of thyroid malignancies. The prognosis of PTC is very good and distant metastases are rare, especially to the skull base. The authors report the case of a 47-year-old woman with biopsy-proven PTC treated with surgery and radiation therapy who presented with headache and diplopia after 5 years and was found to have clivus and cavernous sinus metastasis. Following radiation therapy for her skull base and cavernous sinus lesion, she subsequently developed sixth nerve ocular neuromyotonia. Possible causes and treatments are reviewed.
RESUMEN
PURPOSE OF REVIEW: Giant cell arteritis (GCA) remains a potentially blinding inflammatory vasculitis of the elderly. Because prolonged doses of corticosteroids remain the best established treatment, side-effects during treatment are common and potentially serious. This review addresses the challenges clinicians face in managing this disease. RECENT FINDINGS: High-dose corticosteroids with slow tapering and close monitoring are the mainstay of treatment. Investigations into adjunctive treatment have yet to establish other agents as beneficial, but further research is ongoing with some promising results. SUMMARY: GCA represents a challenging illness to clinicians because of its potential for causing blindness and the need for prolonged high doses of corticosteroids with their many complications.
Asunto(s)
Arteritis de Células Gigantes , Ceguera/etiología , Glucocorticoides/uso terapéutico , Humanos , EsteroidesRESUMEN
A 39-year-old woman with a history of Behçet disease presented for evaluation of oscillopsia that began postpartum. Examination showed oculopalatal tremor (OPT), documented videographically. Brain magnetic resonance imaging revealed bilateral pseudohypertrophy of the inferior olivary nuclei. Treatment with gabapentin was initiated for OPT presumed secondary to neuro-Behçet disease.
Asunto(s)
Síndrome de Behçet/complicaciones , Temblor/complicaciones , Enfermedades Vestibulares/complicaciones , Adulto , Aminas/uso terapéutico , Anticonvulsivantes/uso terapéutico , Síndrome de Behçet/tratamiento farmacológico , Ácidos Ciclohexanocarboxílicos/uso terapéutico , Femenino , Gabapentina , Humanos , Imagen por Resonancia Magnética , Examen Neurológico , Nistagmo Patológico/complicaciones , Núcleo Olivar , Temblor/tratamiento farmacológico , Ácido gamma-Aminobutírico/uso terapéuticoRESUMEN
A 25-year-old woman with dermatomyositis suffered a right central retinal vein occlusion (CRVO) with visual acuity of 20/40. Examination of the right eye showed vitreous cells, suggesting inflammation of the central retinal vein leading to a CRVO as the presumed mechanism. She was admitted to hospital, and extensive evaluation was negative. She was maintained on corticosteroids to manage her dermatomyositis. One month later, she had macular edema and elevated intraocular pressure. Both resolved with dorzolamide, timolol, and intravitreal bevacizumab, and vision returned to 20/20 in the right eye.
Asunto(s)
Dermatomiositis/complicaciones , Oclusión de la Vena Retiniana/complicaciones , Adulto , Femenino , Lateralidad Funcional , Humanos , Papiledema/etiología , Tomografía de Coherencia ÓpticaRESUMEN
Autoimmune-related retinopathy and optic neuropathy is characterized by visual loss in the presence of antibodies against retina or optic nerve antigens in the absence of neoplasia. We report a case with progressive central visual loss accompanied by latent autoimmune diabetes in an adult. Visual fields, multimodal imaging and electrophysiological testing showed characteristic changes without evidence of neoplasia on positron emission tomography. Immunologic testing revealed abnormal reactivity against a 20 kDa optic nerve antigen and 40 kDa and 62 kDa retina antigens along with antibodies against anti-glutamic acid decarboxylase, a constellation which we believe to be unique.
RESUMEN
We present a woman with acute onset of bilateral ophthalmoparesis and ataxia occurring 4 weeks after gastroenteritis. Serum antibody titers against asialo-GM1 and GD1a, typically associated with inflammatory axonal neuropathies, were elevated but titers against anti-GQ1b, the most commonly found antibody found in the Miller Fisher variant of Guillain-Barre syndrome were not. No other etiology for ophthalmoparesis was found despite extensive patient evaluation. Intravenous immunoglobulin was administered, and the patient gradually improved over subsequent months. This case is unique for its antiganglioside antibody profile associated with Miller Fisher syndrome.
Asunto(s)
Autoanticuerpos/sangre , Gangliósido G(M1)/inmunología , Gangliósidos/inmunología , Síndrome de Miller Fisher/sangre , Adulto , Femenino , HumanosRESUMEN
PURPOSE: Proton radiation therapy is commonly used in young children with brain tumors for its potential to reduce late effects. However, some proton series report higher rates of brainstem injury (0%-16%) than most photon series (2.2%-8.6%). We report the incidence of brainstem injury and a risk factor analysis in pediatric patients with posterior fossa primary tumors treated with proton radiation therapy at our institution. METHODS AND MATERIALS: The study included 216 consecutive patients treated between 2000 and 2015. Dosimetry was available for all but 4 patients. Grade 2 to 5 late brainstem toxicity was assessed by the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.0. RESULTS: The histologies include medulloblastoma (n=154, 71.3%), ependymoma (n=56, 25.9%), and atypical teratoid rhabdoid tumor (n=6, 2.8%). The median age at irradiation was 6.6 years (range, 0.5-23.1 years); median dose, 54 gray relative biological effectiveness (Gy RBE) (range, 46.8-59.4 Gy RBE); and median follow-up period, 4.2 years (range, 0.1-15.3 years) among 198 survivors. Of the patients, 83.3% received chemotherapy; 70.4% achieved gross total resection. The crude rate of injury was 2.3% in all patients, 1.9% in those with medulloblastoma, 3.6% in those with ependymoma, and 0% in those with atypical teratoid rhabdoid tumor. The 5-year cumulative incidence of injury was 2.0% (95% confidence interval, 0.7%-4.8%). The median brainstem dose (minimum dose received by 50% of brainstem) in the whole cohort was 53.6 Gy RBE (range, 16.5-56.8 Gy RBE); maximum point dose within the brainstem (Dmax), 55.2 Gy RBE (range, 48.4-60.5 Gy RBE); and mean dose, 50.4 Gy RBE (range, 21.1-56.7 Gy RBE). In the 5 patients with injury, the median minimum dose received by 50% of the brainstem was 54.6 Gy RBE (range, 50.2-55.1 Gy RBE); Dmax, 56.2 Gy RBE (range, 55.0-57.1 Gy RBE); mean dose, 51.3 Gy RBE (range, 45.4-54.4 Gy RBE); and median volume of the brainstem receiving ≥55 Gy RBE (V55), 27.4% (range, 0%-59.4%). Of the 5 patients with injury, 4 had a brainstem Dmax in the highest quartile (≥55.8 Gy RBE, P = .016) and a V55 in the highest tertile (>6.0%) of the cohort distribution (P = .047). Of the 5 patients with injury, 3 were aged >6 years (age range, 4.1-22.8 years), and 4 of 5 patients received chemotherapy and achieved gross total resection. CONCLUSIONS: The incidence of injury in pediatric patients with posterior fossa tumors is consistent with previous reports in the photon setting. Our data suggest that when Dmax and V55 are kept <55.8 Gy RBE and ≤6.0%, respectively, the 5-year rate of radiation brainstem injury would be <2%.
Asunto(s)
Tronco Encefálico/efectos de la radiación , Neoplasias Infratentoriales/radioterapia , Terapia de Protones/efectos adversos , Traumatismos por Radiación/epidemiología , Adolescente , Niño , Preescolar , Intervalos de Confianza , Ependimoma/tratamiento farmacológico , Ependimoma/mortalidad , Ependimoma/radioterapia , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Neoplasias Infratentoriales/tratamiento farmacológico , Neoplasias Infratentoriales/mortalidad , Masculino , Meduloblastoma/tratamiento farmacológico , Meduloblastoma/mortalidad , Meduloblastoma/radioterapia , Supervivencia sin Progresión , Traumatismos por Radiación/mortalidad , Traumatismos por Radiación/patología , Dosificación Radioterapéutica , Efectividad Biológica Relativa , Tumor Rabdoide/tratamiento farmacológico , Tumor Rabdoide/mortalidad , Tumor Rabdoide/radioterapia , Medición de Riesgo , Teratoma/tratamiento farmacológico , Teratoma/mortalidad , Teratoma/radioterapia , Adulto JovenRESUMEN
A 51-year-old man with rheumatoid arthritis and diabetes mellitus presented with new onset left-sided hemiparesis, left-sided neglect, and left-sided incongruous, denser inferiorly, homonymous hemianopsia. Magnetic resonance image of the brain showed prominent swelling of the right frontal, parietal, and occipital lobes greater than on the left with significant change in the fluid-attenuated inversion recovery signal in the gray matter. Perinuclear antineutrophil cytoplasmic antibodies titers were elevated, and skin biopsy demonstrating leukocytoclastic vasculitis. He showed marked clinical and radiographic improvement in association with recovery of vascular abnormalities after the initiation of prednisone, pulse therapy with methylprednisolone, and methotrexate. Clinicians should be aware of the possibility of neuro-ophthalmic manifestations of rheumatoid arthritis, including rheumatoid arthritis-related vasculitis causing homonymous hemianopsia.
Asunto(s)
Artritis Reumatoide/diagnóstico , Hemianopsia/diagnóstico , Vasculitis del Sistema Nervioso Central/diagnóstico , Vasculitis Leucocitoclástica Cutánea/diagnóstico , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Artritis Reumatoide/tratamiento farmacológico , Artritis Reumatoide/inmunología , Diabetes Mellitus/diagnóstico , Diagnóstico Diferencial , Quimioterapia Combinada , Glucocorticoides/uso terapéutico , Hemianopsia/tratamiento farmacológico , Hemianopsia/inmunología , Humanos , Inmunosupresores/uso terapéutico , Imagen por Resonancia Magnética , Masculino , Metotrexato/uso terapéutico , Metilprednisolona/uso terapéutico , Persona de Mediana Edad , Prednisona/uso terapéutico , Quimioterapia por Pulso , Vasculitis del Sistema Nervioso Central/tratamiento farmacológico , Vasculitis del Sistema Nervioso Central/inmunología , Vasculitis Leucocitoclástica Cutánea/tratamiento farmacológico , Vasculitis Leucocitoclástica Cutánea/inmunología , Pruebas del Campo Visual , Campos VisualesRESUMEN
Papilledema is optic disc swelling due to high intracranial pressure. Possible conditions causing high intracranial pressure and papilledema include intracerebral mass lesions, cerebral hemorrhage, head trauma, meningitis, hydrocephalus, spinal cord lesions, impairment of cerebral sinus drainage, anomalies of the cranium, and idiopathic intracranial hypertension (IIH). Irrespective of the cause, visual loss is the feared morbidity of papilledema, and the main mechanism of optic nerve damage is intraneuronal ischemia secondary to axoplasmic flow stasis. Treatment is directed at correcting the underlying cause. In cases where there is no other identifiable cause for intracranial hypertension (ie, IIH) the available options include both medical and surgical modalities. Weight loss and diuretics remain the mainstays for treatment of IIH, and surgery is typically reserved for patients who fail, are intolerant to, or non-compliant with maximum medical therapy.
RESUMEN
Idiopathic Intracranial Hypertension (IIH) is a disorder of increased intracranial pressure without any identifiable etiology. It is defined by elevated intracranial pressure (ICP) with normal neuroimaging and normal cerebrospinal fluid (CSF) contents. IIH typically affects young obese women and produces symptoms and signs related to high ICP. Headache and blurred vision are the most common symptoms, and papilledema is the major clinical sign. In this review we examine the epidemiology and demographic features of IIH in Middle Eastern countries and compare and contrast them with the published IIH literature from Western countries. The incidence of IIH in several Middle East countries has been estimated at 2.02-2.2/100,000 in the general population, which is higher than the Western rate. Obesity is a major risk factor globally and it is associated with an increased risk of severe vision loss due to IIH. There has been an increase in obesity prevalence in the Middle East countries mainly affecting the Gulf Council Countries (GCC), which parallels increased industrial development. This rise may be contributing to the increasing incidence of IIH in these countries. Other risk factors may also be contributing to IIH in Middle East countries and the differences and similarities to Western IIH merit further study.
RESUMEN
OBJECTIVE: To define the efficacy, safety, and cost-effectiveness of a single centre's approach to evaluating Horner syndrome (HS) including a simplified single neuroimaging protocol. DESIGN: Case series study. PARTICIPANTS: Medical records of 34 patients diagnosed with HS at Houston Methodist Hospital (HMH) were reviewed after obtaining Institutional Review Board approval. METHODS: A retrospective chart review was performed for all patients presenting with the diagnosis of HS at the HMH from January 2010 to November 2013. All patients had diagnostic imaging with contrast-enhanced brain magnetic resonance imaging (MRI) extending to the T2 level in the chest. They had either documented causative diagnosis for HS or were "idiopathic." Efficacy and cost-effectiveness of the proposed neuroimaging technique were analyzed compared with other recommended protocols. RESULTS: We initially reviewed 34 charts with presumed diagnosis of HS; 27 charts were included in the analysis. The average age of patients was 46.6 years. Eleven patients (41%) had a final diagnosis of HS secondary to a proven cause, and 16 patients (59%) were diagnosed as "idiopathic." Ten patients (63%) in the idiopathic group had follow-up, and none of those with follow-up had an alternative cause. The estimated cost of our recommended MRI protocol was US$667.76 without magnetic resonance angiography (MRA) or US$1501.71 with MRA. CONCLUSIONS: A single contrast-enhanced brain MRI extending to the T2 level in the chest is an effective and simple means of ruling out life-threatening and other causative factors of HS. Compared with previous imaging recommendations, this proposed protocol may be simpler for clinicians to use and more cost-effective.
Asunto(s)
Síndrome de Horner/diagnóstico , Imagen por Resonancia Magnética/métodos , Adolescente , Agonistas de Receptores Adrenérgicos alfa 2 , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Clonidina/análogos & derivados , Cocaína , Análisis Costo-Beneficio , Inhibidores de Captación de Dopamina , Femenino , Síndrome de Horner/economía , Humanos , Lactante , Imagen por Resonancia Magnética/economía , Masculino , Persona de Mediana Edad , Neuroimagen/métodos , Estudios RetrospectivosRESUMEN
A 59-year-old woman with late-onset diabetes mellitus presented with a 4-week history of oscillopsia and vertigo. Physical examination revealed downbeating nystagmus in the primary position that worsened on right gaze and left gaze. Magnetic resonance imaging of the brain and orbits showed enhancement and signal abnormality in the right temporal lobe without evidence of a cervicomedullary junction lesion. Serum anti-glutamic acid decarboxylase 65 (GAD65) antibody titer was markedly elevated. Given these findings, her vertigo and downbeat nystagmus were likely secondary to elevated anti-GAD antibodies.
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Autoanticuerpos/sangre , Enfermedades Autoinmunes/complicaciones , Nistagmo Patológico/etiología , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Vértigo/etiologíaRESUMEN
OBJECTIVE: To illustrate three different ophthalmic presentations of cryptococcal meningitis (CM). INTRODUCTION: CM is the most common manifestation of extra-pulmonary cryptococcosis. Intracranial hypertension occurs in up to 75% of patients with CM and is associated with increased mortality. CM can present to the ophthalmologist as vision loss, papilledema, abducens palsy, and/or other cranial neuropathies. PARTICIPANTS AND METHODS: We report three cases, two C. neoformans and one C. gattii, highlighting the various CM presentations. The first was a woman immunosuppressed following kidney transplantation in whom idiopathic intracranial hypertension (IIH) was initially suspected. The second was a man immunocompromised by previously undiagnosed HIV/AIDS who presented with signs and symptoms of increased intracranial pressure. The third case is an immunocompetent man with bilateral disc edema and an incomplete macular star diagnosed with presumed neuroretinitis. Further evaluation revealed positive CSF cryptococcal antigen with culture positive for C. gattii. CONCLUSIONS: Ophthalmologists should be aware that cryptococcosis can mimic more benign etiologies including IIH and neuroretinitis. Additionally, C. gattii, an emerging organism, can infect immunocompetent patients. In contrast to the typical treatment of increased ICP, serial lumbar punctures are recommended while acetazolamide and surgical CSF shunting may be harmful.
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Antifúngicos/uso terapéutico , Criptococosis/diagnóstico , Infecciones Fúngicas del Ojo/diagnóstico , Hipertensión Intracraneal/diagnóstico , Meningitis Criptocócica/diagnóstico , Neuritis Óptica/diagnóstico , Papiledema/diagnóstico , Anfotericina B/uso terapéutico , Anticuerpos Antifúngicos/líquido cefalorraquídeo , Antígenos Fúngicos/inmunología , Criptococosis/tratamiento farmacológico , Cryptococcus gattii/inmunología , Cryptococcus gattii/aislamiento & purificación , Cryptococcus neoformans/inmunología , Cryptococcus neoformans/aislamiento & purificación , Quimioterapia Combinada , Infecciones Fúngicas del Ojo/tratamiento farmacológico , Infecciones Fúngicas del Ojo/microbiología , Femenino , Flucitosina/uso terapéutico , Humanos , Hipertensión Intracraneal/tratamiento farmacológico , Hipertensión Intracraneal/microbiología , Presión Intracraneal , Masculino , Meningitis Criptocócica/tratamiento farmacológico , Meningitis Criptocócica/microbiología , Neuritis Óptica/tratamiento farmacológico , Neuritis Óptica/microbiología , Papiledema/tratamiento farmacológico , Papiledema/microbiologíaRESUMEN
The aim of this study was to update the practicing ophthalmologist on the English-language neuro-ophthalmology literature from the prior year. This study is a review of English-language literature from August 1, 2012, to August 1, 2013. The authors searched PubMed articles published from August 1, 2012, to August 1, 2013, limited to English-language publications including original articles, review articles, and case reports and excluding letters to the editor, unpublished work, and abstracts. We researched the following topics: pupillary abnormalities, eye movement dysfunction, neuromuscular diseases, optic neuropathies, optic neuritis and demyelinating disease including multiple sclerosis, lesions of the chiasm and posterior primary visual pathways, elevated intracranial pressure, tumors and aneurysms affecting the visual pathways, vascular diseases, higher visual functions, and neuroimaging advances. We intend to share clinically relevant literature of the past year with the practicing ophthalmologist. We aimed to highlight remarkable and interesting literature rather than exhaustively including all new neuro-ophthalmological publications of the year. We reviewed literature in the past year with a focus on relevance and novelty. This review updates the comprehensive ophthalmologist on neuro-ophthalmic topics.