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4.
Arch Soc Esp Oftalmol ; 93(11): 555-557, 2018 Nov.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-29859732

RESUMO

CLINICAL CASE: A 22 year-old woman complained about blurred vision after an episode of recovered cardiorespiratory arrest. She had bilateral low visual acuity («count fingers¼) and no ophthalmological or visual pathways changes. She also had an apparent lack of awareness of the deficit. The Magnetic Resonance Imaging (MRI) showed ischaemic changes in both occipital lobes. As a result, she was diagnosed with Anton-Babinski syndrome. DISCUSSION: This is a rare disease that should be suspected in strange or poorly congruent visual loss. It is usually due to an ischaemic injury in this region of brain, manifesting itself with low vision not perceived by the patient (visual confabulation). It can simulate a non-organic visual loss or psychiatric disease.


Assuntos
Cegueira Cortical/diagnóstico , Isquemia Encefálica/diagnóstico , Córtex Visual/irrigação sanguínea , Agnosia/etiologia , Cegueira Cortical/diagnóstico por imagem , Cegueira Cortical/psicologia , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/psicologia , Feminino , Parada Cardíaca/complicações , Humanos , Hipestesia/etiologia , Hipóxia-Isquemia Encefálica/etiologia , Imagem por Ressonância Magnética , Paresia/etiologia , Tomografia de Coerência Óptica , Córtex Visual/diagnóstico por imagem , Adulto Jovem
5.
Medicine (Baltimore) ; 97(16): e0332, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29668582

RESUMO

RATIONALE: Splenic artery embolization (SAE) is a common procedure in trauma patients with blunt splenic injuries. We report a case of acute ischemic stroke following orthopedic surgery in a patient with post-SAE reactive thrombocytosis. PATIENT CONCERNS: A 37-year-old woman with idiopathic thrombocytopenic purpura (ITP) suffered from multiple trauma scheduled for open reduction and internal fixation for right tibial and left radius fracture five days after SAE. The patient did not have any thromboembolic complications, although the platelet counts increased from 43 × 10/L to 568 × 10/L within two days after SAE. Surgery was completed under general anesthesia with tracheal intubation without complications. The patient complained of visual loss followed by limb weakness on the fourth and eighth hour postoperatively. DIAGNOSES: Magnetic resonance imaging (MRI) of head demonstrated ischemic change over bilateral basal ganglia, and occipital areas, suggesting the diagnosis of cortical blindness. INTERVENTIONS: To suppress platelet count and avoid platelet hyper-aggregation, anti-platelet drug (i.e., oral aspirin 100 mg daily), hydration, and hydroxyurea (i.e., 20 mg/kg daily) were used for the treatment of reactive thrombocytosis. OUTCOMES: Although right-sided hemiparesis persisted, the patient reported mild visual recovery. She was discharged four months after SAE with active rehabilitation. LESSONS: Our report highlights an increased risk of acute arterial thromboembolic events in patients with reactive thrombocytosis, especially those undergoing surgery.


Assuntos
Aspirina/administração & dosagem , Cegueira Cortical , Isquemia Encefálica , Embolização Terapêutica , Púrpura Trombocitopênica Idiopática/complicações , Baço , Acidente Vascular Cerebral , Trombocitose/tratamento farmacológico , Ferimentos não Penetrantes/terapia , Adulto , Cegueira Cortical/diagnóstico , Cegueira Cortical/tratamento farmacológico , Cegueira Cortical/etiologia , Isquemia Encefálica/etiologia , Isquemia Encefálica/fisiopatologia , Embolização Terapêutica/efeitos adversos , Embolização Terapêutica/métodos , Feminino , Humanos , Imagem por Ressonância Magnética/métodos , Traumatismo Múltiplo/complicações , Traumatismo Múltiplo/cirurgia , Procedimentos Ortopédicos/efeitos adversos , Inibidores da Agregação de Plaquetas/administração & dosagem , Contagem de Plaquetas/métodos , Baço/irrigação sanguínea , Baço/lesões , Artéria Esplênica/diagnóstico por imagem , Artéria Esplênica/patologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/fisiopatologia , Trombocitose/diagnóstico , Trombocitose/etiologia , Resultado do Tratamento
6.
Indian J Ophthalmol ; 66(2): 279-284, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29380776

RESUMO

PURPOSE: A pilot study was done to evaluate knowledge regarding "cortical visual impairment (CVI) in children" among ophthalmologists. METHODS: This study was conducted during the annual conference of a zonal ophthalmological society. All ophthalmologists who attended the conference were requested to participate in this study. Those who agreed were given a validated questionnaire to assess knowledge regarding CVI. Cronbach's alpha of the questionnaire was 0.6. Participants were asked to respond to multiple choice questions by choosing the single best option. The responses obtained were then evaluated. RESULTS: The total number of registered delegates in the conference was 448. A total of 103 ophthalmologists showed interest to participate in the study with a response rate of 22.9%. Only 89/103 interested delegates were included in the study as remaining were unaware of CVI. No participant gave correct answers to all questions. Although more than 80% of them knew the most common association (87%) and site of pathology (84%), only 52% were sure about clinical features and even lesser respondents (39%) knew that magnetic resonance imaging is the correct investigation of choice. The majority responded correctly that these children need eye examination (89%) and can be managed by rehabilitation through multidisciplinary approach (82%), but only 58% could recognize differential diagnoses and had a correct idea regarding the prognosis of CVI. There was no correlation between the number of patients diagnosed per month by the respondent with knowledge of the disease. CONCLUSION: In this pilot study, ophthalmologists were found to have limited knowledge regarding clinical features, investigation, differential diagnosis, and visual prognosis of CVI in children. There is a need to improve awareness regarding CVI among ophthalmologists.


Assuntos
Cegueira Cortical/diagnóstico , Conhecimentos, Atitudes e Prática em Saúde , Oftalmologistas/normas , Oftalmologia/educação , Sociedades Médicas , Inquéritos e Questionários , Córtex Visual/diagnóstico por imagem , Criança , Diagnóstico Diferencial , Feminino , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Projetos Piloto , Prognóstico
7.
Br J Ophthalmol ; 102(4): 424-432, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29146757

RESUMO

The childhood condition of visual difficulties caused by brain damage, commonly termed cortical or cerebral visual impairment (CVI), is well established but has no internationally accepted definition. Clarification of its core features is required to advance research and clinical practice. This systematic review aimed to identify the definitions of childhood CVI in the original scientific literature to describe and critically appraise a consensual definition of the condition. MEDLINE, EMBASE, PsychINFO, CINAHL and AMED databases were searched in January 2017. Studies were included if they (1) were published original research, (2) contained a childhood CVI sample, (3) contained a definition of CVI and (4) described their CVI identification/diagnostic method. Thematic analysis identified concepts within definitions and narrative synthesis was conducted. Of 1150 articles, 51 met inclusion criteria. Definitions were subdivided according to detail (descriptive definition, description not reaching definition status and diagnostic/operationalising criteria). Three themes concerning visual deficits, eye health and brain integrity were identified (each containing subthemes) and analysed individually across definitions. The most common themes were 'visual impairment' (n=20), 'retrochiasmatic pathway damage'(n=13) and 'normal/near normal eye health' (n=15). The most consensual definition identified here may not be the best quality for advancing our understanding of CVI. We argue for the alternative definition: CVI is a verifiable visual dysfunction which cannot be attributed to disorders of the anterior visual pathways or any potentially co-occurring ocular impairment. We propose reporting guidelines to permit comparison across studies and increase the evidence base for more reliable clinical assessment and diagnosis.


Assuntos
Cegueira Cortical/diagnóstico , Encefalopatias/diagnóstico , Terminologia como Assunto , Cegueira Cortical/fisiopatologia , Encefalopatias/fisiopatologia , Consenso , Humanos , Vias Visuais/fisiopatologia , Percepção Visual/fisiologia
8.
BMJ Case Rep ; 20172017 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-29084739

RESUMO

Cerebral hyperperfusion syndrome (CHS) is a well-documented complication after carotid endarterectomy or stenting. In contrast, CHS following vertebral revascularization is extremely rare. Here we present a case of a 77-year-old man with high-grade vertebral stenosis who subsequently underwent balloon angioplasty, complicated by hemorrhagic CHS manifesting as cortical blindness, although strict postoperative blood pressure control was administered. To our knowledge, cortical blindness as a presentation of hemorrhagic CHS has not previously been reported. This study highlights the fact that identifying high-risk patients, as well as making an individual therapeutic plan, is important prior to revascularization. Further studies are needed to elucidate the exact mechanism of this condition and thereby prevent it.


Assuntos
Cegueira Cortical/diagnóstico , Constrição Patológica/cirurgia , Hemorragia Intracraniana Hipertensiva/diagnóstico , Stents , Artéria Vertebral , Idoso , Angioplastia com Balão/efeitos adversos , Cegueira Cortical/diagnóstico por imagem , Cegueira Cortical/etiologia , Constrição Patológica/diagnóstico por imagem , Diagnóstico Diferencial , Humanos , Hemorragia Intracraniana Hipertensiva/diagnóstico por imagem , Hemorragia Intracraniana Hipertensiva/etiologia , Imagem por Ressonância Magnética , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Tomografia Computadorizada por Raios X
10.
Medicine (Baltimore) ; 96(34): e7454, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28834867

RESUMO

RATIONALE: The conception that multiple sclerosis may be challenging to distinguish from demyelinating manifestations of Sjögren's syndrome (SS) was introduced more than 30 years ago. However, it is now recognized that the neuromyelitis optica spectrum disorder (NMOSD) may occur more frequently in SS as opposed to multiple sclerosis. Characteristic NMOSD features can include severe attacks of optic neuritis, myelitis which is frequently longitudinally-extensive (spanning at least three vertebral segments on magnetic resonance imaging [MRI]), and an association with anti-aquaporin-4 antibodies. In addition, whereas NMOSD was initially thought to spare the brain, it is now recognized that brain lesions occur in a majority of NMOSD patients. Therefore, it is important for the multi-disciplinary team of physicians who care for SS patients to understand this widening spectrum of NMOSD as encompassing brain lesions. In this case-report we describe clinical features, radiographic findings, and treatment of a SS NMOSD patient presenting with severely decreased visual acuity, visual hallucinations, and encephalopathy. PATIENT CONCERNS: The SS NMOSD patient presented with rapid, bilateral onset of severely decreased visual acuity and was therefore suspected as having bilateral optic neuritis. DIAGNOSIS: However, the patient lacked stigmata of optic neuritis, instead had visual hallucinations and encephalopathy suggestive of cortical blindness, and was noted to have occipital lobe lesions on brain MRI. Other radiographic findings included simultaneous enhancement of brainstem and periventricular lesions. INTERVENTIONS: The patient was initially treated with methylprednisolone with no change in her neurological deficits. She was then treated with plasma exchange therapy. OUTCOMES: The patient had resolution of decreased visual acuity, visual hallucinations, encephalopathy, and contrast-enhancing brain lesions in response to plasma exchange therapy. LESSON: We provide the first example of severely decreased visual acuity in a NMOSD patient due to cortical blindness and not bilateral optic neuritis. This finding expands the spectrum of central nervous system syndromes and brain lesions which may occur in NMOSD. The synchronous enhancement of a brainstem lesion (known to occur in NMOSD) with occipital lobe lesions also suggests that our patient's occipital lobe findings were due to NMOSD. All of our patient's findings had an excellent clinical and radiographic response to plasma exchange therapy.


Assuntos
Cegueira Cortical/diagnóstico , Cegueira Cortical/etiologia , Neuromielite Óptica/complicações , Síndrome de Sjogren/complicações , Idoso , Cegueira Cortical/diagnóstico por imagem , Feminino , Humanos , Imagem por Ressonância Magnética , Neurite Óptica/diagnóstico
11.
Eur J Hum Genet ; 25(6): 775-778, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28378817

RESUMO

Mutations of the cystatin B gene (CSTB; OMIM 601145) are known to cause Unverricht-Lundborg disease or progressive myoclonic epilepsy-1A (EPM1A, MIM #254800). Most patients are homozygous for an expanded (>30) dodecamer repeat in the promoter region of CSTB, or are compound heterozygotes for the dodecamer repeat and a point mutation. We report two adolescent sisters born to consanguineous parents of Sri Lankan descent who presented with profound global developmental delay, microcephaly, cortical blindness and axial hypotonia with appendicular hypertonia. Neither sibling ever developed head control, independent sitting or ambulation, and never developed speech. The elder sister had a seizure disorder. Both sisters had profound microcephaly and distinct facial features. On serial brain imaging, they had progressive atrophy of the corpus callosum and supratentorial brain, and diffuse hypomyelination with progressive loss of myelin signal. Exome sequencing revealed both siblings to be homozygous for a c.218dupT (p.His75Serfs*2) mutation in exon 3 of CSTB. The neuroimaging features of our patients are consistent with those observed in Cstb-knockout mice, which supports the hypothesis that disease severity is inversely correlated with the amount of residual functional cystatin B protein.


Assuntos
Cegueira Cortical/genética , Cistatina B/genética , Deficiências do Desenvolvimento/genética , Mutação da Fase de Leitura , Doenças Desmielinizantes Hereditárias do Sistema Nervoso Central/genética , Microcefalia/genética , Adolescente , Cegueira Cortical/diagnóstico , Criança , Corpo Caloso/diagnóstico por imagem , Corpo Caloso/patologia , Deficiências do Desenvolvimento/diagnóstico , Feminino , Doenças Desmielinizantes Hereditárias do Sistema Nervoso Central/diagnóstico , Homozigoto , Humanos , Masculino , Microcefalia/diagnóstico , Bainha de Mielina/patologia , Linhagem , Síndrome
12.
Neurodiagn J ; 57(1): 47-52, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28436816

RESUMO

A middle-aged man with a history of type 2 diabetes mellitus, hypertension, hyperlipidemia, prior cerebral vascular accident, and remote history of generalized seizure disorder presented with worsening right-sided visual deficits and focal seizures. On examination the patient had dense homonymous hemianopsia on the right side. He was initially diagnosed with a stroke and underwent further investigation for potential causes. However, upon further review, magnetic resonance imaging (MRI) findings and repeat examination were more consistent with seizure-related effects on cortical brain matter as the cause of his visual disturbances rather than stroke. An EEG confirmed focal seizures from the left posterior quadrant of the brain, and the diagnosis of status epilepticus amauroticus (SEA) was made. The hemianopsia resolved with antiepileptic treatment.


Assuntos
Cegueira Cortical , Estado Epiléptico , Anticonvulsivantes/uso terapêutico , Cegueira Cortical/diagnóstico , Cegueira Cortical/etiologia , Diabetes Mellitus Tipo 2/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Estado Epiléptico/complicações , Estado Epiléptico/diagnóstico , Estado Epiléptico/tratamento farmacológico
13.
Diving Hyperb Med ; 47(1): 59-61, 2017 03.
Artigo em Inglês | MEDLINE | ID: mdl-28357826

RESUMO

We present a case of a patient with Anton's syndrome due to decompression illness (DCI) after recreational scuba diving. Visual anosognosia, or denial of loss of vision, which is associated with lack of awareness regarding visual loss in the setting of cortical blindness, is known as Anton's syndrome (also termed Anton-Babinski syndrome). Our patient presented with progressive neurological DCI treated with repeated recompression. The anosogosia resolved after 48 h. Subsequent echocardiography revealed a persistent (patent) foramen ovale.


Assuntos
Agnosia/etiologia , Cegueira Cortical/etiologia , Doença da Descompressão/complicações , Agnosia/diagnóstico , Cegueira Cortical/diagnóstico , Encéfalo/diagnóstico por imagem , Doença da Descompressão/diagnóstico por imagem , Forame Oval Patente/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade
14.
Clin Neurophysiol ; 128(5): 770-779, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28319878

RESUMO

OBJECTIVE: We investigated neuronal correlates of faces versus non-faces processing in a cortically blind patient (TN) and a group of healthy age-matched controls in order to test electrophysiological correlates of the processing of pertinent stimuli in this patient. METHODS: An EEG paradigm was used, in which intact and scrambled faces were displayed on a screen. First, time-frequency transforms were conducted on the patients' data alone. These oscillations were then compared to the frontal activity of six control participants. RESULTS: Post stimulus oscillatory modulations (synchronisation in theta and alpha frequency bands) of both intact and scrambled faces at frontal scalp sites were observed in TN. These modulations were different for correct and incorrect responses. A more important increase in the theta band for incorrect responses was observed. The oscillatory rhythms highlighted in blindsight and in frontal regions differ from the ones observed in control participants. CONCLUSION: Despite the destruction of the visual cortex, oscillatory rhythms are not cancelled out but are shifted to anterior regions, revealing the activity of an alternate pathway for residual visual function. SIGNIFICANCE: The results provide evidence for a top-down cognitive control process in blindsight.


Assuntos
Cegueira Cortical/fisiopatologia , Lobo Frontal/fisiopatologia , Ritmo Teta , Percepção Visual , Cegueira Cortical/diagnóstico , Estudos de Casos e Controles , Humanos , Masculino , Pessoa de Meia-Idade , Vias Visuais/fisiopatologia
16.
Pediatr Diabetes ; 18(2): 159-162, 2017 03.
Artigo em Inglês | MEDLINE | ID: mdl-26764016

RESUMO

Development of acute neurological symptoms secondary to cerebral oedema is well described in diabetic ketoacidosis (DKA) and often has a poor prognosis. We present the clinical and radiological data of a 17-yr-old girl who developed cortical blindness, progressive encephalopathy, and seizures caused by posterior reversible encephalopathy syndrome (PRES) that developed after her DKA had resolved. Vasogenic oedema in PRES resolves if the underlying trigger is identified and eliminated. In this case, hypertension was identified as the likely precipitating factor and following treatment her vision and neurological symptoms rapidly improved. We suggest how recent DKA may have contributed to the development of PRES in this patient.


Assuntos
Cetoacidose Diabética/complicações , Cetoacidose Diabética/terapia , Síndrome da Leucoencefalopatia Posterior/complicações , Síndrome da Leucoencefalopatia Posterior/terapia , Adolescente , Cegueira Cortical/diagnóstico , Cegueira Cortical/etiologia , Cegueira Cortical/terapia , Edema Encefálico/diagnóstico , Edema Encefálico/etiologia , Edema Encefálico/terapia , Cetoacidose Diabética/diagnóstico , Eletrólitos/uso terapêutico , Feminino , Hidratação , Humanos , Hipertensão/diagnóstico , Hipertensão/etiologia , Hipertensão/terapia , Labetalol/uso terapêutico , Imagem por Ressonância Magnética , Síndrome da Leucoencefalopatia Posterior/diagnóstico
17.
Tunis Med ; 95(12): 229-231, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29878292

RESUMO

Neurological manifestations of preeclampsia are serious and very variable. We report the case of a woman at 34 weeks gestation with preeclampsia who developed blindness within hours without alteration of consciousness. The imagery was in favor of posterior reversible encephalopathy. The therapeutic management consisted of emergency fetal extraction, the administration of antihypertensive drugs and magnesium sulfate. The outcome was favorable with complete recovery of visual function. This case reflects the heterogeneity of the clinical presentation of preeclampsia, as the only neurological manifestation was cortical blindness, with no associated consciousness disorder.


Assuntos
Cegueira Cortical/diagnóstico , Cegueira Cortical/etiologia , Pré-Eclâmpsia/diagnóstico , Complicações na Gravidez/diagnóstico , Adulto , Encéfalo/diagnóstico por imagem , Feminino , Humanos , Imagem por Ressonância Magnética , Gravidez , Acuidade Visual
19.
J Neuroophthalmol ; 36(3): 264-9, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27295602

RESUMO

BACKGROUND: The vulnerable brain areas in hypoxic-ischemic encephalopathy (HIE) following systemic hypotension are typically the neocortex, deep cerebral gray nuclei, hippocampus, cerebellum, and the parieto-occipital arterial border zone region. The visual cortex is not commonly recognized as a target in this setting. METHODS: Single-institution review from 2007 to 2015 of patients who suffered cortical visual loss as an isolated clinical manifestation following systemic hypotension and whose brain imaging showed abnormalities limited to the occipital lobe. RESULTS: Nine patients met inclusion criteria. Visual loss at outset ranged from hand movements to 20/20, but all patients had homonymous field loss at best. In 1 patient, imaging was initially normal but 4 months later showed encephalomalacia. In 2 patients, imaging was initially subtle enough to be recognized as abnormal only when radiologists were advised that cortical visual loss was present. CONCLUSIONS: The occipital lobe may be an isolated target in HIE with cortical visual loss as the only clinical manifestation. Imaging performed in the acute period may appear normal or disclose abnormalities subtle enough to be overlooked. Radiologists informed of the clinical manifestations may be more attune to these abnormalities, which will become more apparent months later when occipital volume loss develops.


Assuntos
Cegueira Cortical/etiologia , Hipóxia-Isquemia Encefálica/complicações , Imagem por Ressonância Magnética/métodos , Lobo Occipital/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Acuidade Visual , Adolescente , Adulto , Idoso , Cegueira Cortical/diagnóstico , Cegueira Cortical/fisiopatologia , Feminino , Seguimentos , Humanos , Hipóxia-Isquemia Encefálica/diagnóstico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Tempo , Adulto Jovem
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