Prenatal or Perinatal Injury? Diagnosing the Cortically Blind Infant.

PURPOSE: To document the association of prenatal brain disruption with secondary perinatal distress in children diagnosed as having cortical visual impairment (CVI). DESIGN: Retrospective case series. METHODS: Eight children with severe CVI and clinical history of perinatal events were included. Case histories and neuroimaging studies were reviewed. The main outcome measures were perinatal history, visual and neurologic findings, and magnetic resonance (MR) imaging. RESULTS: In our patient cohort, MR imaging showed signs of cortical dysgenesis leading to congenital brain malformations such as polymicrogyria consistent with a prenatal timing of CNS injury. Although subcortical white matter changes were common, signs of watershed injury to the visual cortex were absent, suggesting that the visual loss was attributable to a prenatal etiology with secondary birth complications. CONCLUSION: Some children with CVI and a history of perinatal distress have prenatal dysgenesis of the developing brain. Therefore, a clinical history of perinatal hypoxia-ischemia is nonspecific and merits neuroimaging to identify antecedent brain malformations and timing of injury, which can influence patient diagnosis and management.

Cortical blindness secondary to posterior reversible encephalopathy syndrome, recovered by successful blood pressure management / Cegueira cortical secundária à síndrome de encefalopatia reversível posterior recuperada pelo controle bem sucedido da pressão arterial

ABSTRACT We report a case of cortical blindness secondary to posterior leukoencephalopathy syndrome, which was successfully treated by blood pressure management. A 66-year-old white man presented at the emergency room complaining of severe frontal headache, disorientation, and progressive blurred vision. The initial physical examination disclosed a blood pressure of 200/176 mmHg. One hour later the patient exhibited mental confusion and bilateral blindness. Computed tomography and magnetic resonance imaging were performed, and a systemic workup was conducted, resulting in a diagnosis of posterior leukoencephalopathy syndrome. This syndrome is a rare manifestation of systemic hypertension that requires proper diagnosis and management to avoid irreversible brain damage. Early recognition of this condition and prompt control of the patient's blood pressure are essential because they may bring about a reversal of the syndrome, which may otherwise result in permanent brain damage.

RESUMO Relatamos um caso de cegueira cortical secundário à síndrome de leucoencefalopatia posterior recuperado pelo controle bem sucedido da pressão arterial. Um homem branco de 66 anos de idade compareceu à emergência com queixa de dor de cabeça frontal severa, desorientação e embaçamento progressivo da visão. O exame físico inicial revelou uma pressão arterial de 200/176 mmHg. Uma hora depois, o paciente apresentou confusão mental e cegueira bilateral. Após a tomografia e a ressonância nuclear magnética, o tratamento sistêmico foram realizados e o diagnóstico de síndrome da leucoencefalopatia posterior foi realizado. A síndrome de leucoencefalopatia posterior é uma manifestação rara de hipertensão sistêmica que requer um diagnóstico e gerenciamento adequados para evitar danos cerebrais irreversíveis. O reconhecimento precoce desta condição e o controle imediato da pressão arterial são essenciais porque podem levar à reversão da síndrome, o que, de outra forma, pode resultar em dano cerebral permanente.

Strabismus in patients with cortical visual impairment: outcomes of surgery and observations of spontaneous resolution.

PURPOSE: To investigate the rate of spontaneous resolution of strabismus in patients with cortical visual impairment (CVI) at a single center over a 10-year period and to evaluate the success rate of strabismus surgery. METHODS: The medical records of patients with CVI and strabismus seen between October 2003 and October 2013 were reviewed retrospectively. Patients were classified into 4 outcome groups: (1) those who experienced spontaneous resolution of strabismus, (2) those with persistent strabismus who did not undergo surgery, (3) those who achieved postoperative alignment of ≤10(Δ); and (4) those whose final postoperative alignment was 11(Δ)-25(Δ) or >25(Δ). RESULTS: A total of 70 patients were included. Of these, 11 patients (16%) experienced spontaneous resolution of strabismus, 27 (38%) were observed without receiving surgery, and 32 (46%) underwent strabismus surgery. Of these 32, 18 (56%) achieved alignment of ≤10(Δ); 9 (28%), alignment of 11(Δ)-25(Δ); and 5 (16%), alignment >25(Δ). The patients who did not undergo surgery were significantly older at presentation (36 months vs 12-15 months; P = 0.03); otherwise, there were no significant differences between groups in age at surgery or spontaneous resolution, type of strabismus, or underlying cause of CVI. CONCLUSIONS: In our study cohort, a minority of patients with CVI and strabismus (16%) experienced spontaneous resolution of strabismus. Only 16% of patients undergoing surgery had poor final alignment (>25(Δ)). Strabismus surgery can be reasonably successful in properly selected patients with CVI and strabismus. These patients show considerable rates of resolution of their strabismus, either spontaneously or through surgery.

Transient cortical visual impairment after video-assisted thoracic surgery: a case report.

BACKGROUND: Visual loss associated with thoracic surgery has been reported mostly after coronary angiography or bypass surgery. The position of video-assisted thoracic surgery (VATS) is usually lateral, thus not compressive to the globe. Visual loss after VATS has not been reported. Herein we report a patient without any cardiovascular risk factors who experienced transient cortical blindness after an uneventful VATS. CASE PRESENTATION: A 40-year-old man noticed a visual loss at the recovery room after VATS. He showed normal pupillary reflex, normal optic disc appearance, and homonymous hemianopia respecting the vertical meridian, thus was typical for cortical visual impairment. CONCLUSIONS: Transient cortical visual impairment could be encountered after an uneventful VATS in a patient without any cardiovascular risk factors.

Progressive cortical visual failure associated with occipital calcification and coeliac disease with relative preservation of the dorsal 'action' pathway.

We describe the first reported case of a patient with coeliac disease and cerebral occipital calcification who shows a progressive and seemingly selective failure to recognise visual stimuli. This decline was tracked over a study period of 22 years and occurred in the absence of primary sensory or widespread intellectual impairment. Subsequent tests revealed that although the patient was unable to use shape and contour information to visually identify objects, she was nevertheless able to use this information to reach, grasp and manipulate objects under central, immediate vision. This preservation of visuo-motor control was echoed in her day-to-day ability to navigate and live at home independently. We conclude that occipital calcification following coeliac disease can lead to prominent higher visual failure that, under prescribed viewing conditions, is consistent with separable mechanisms for visual perception and action control.

Acute visual loss with ophthalmoplegia after spinal surgery: report of a case and review of the literature.

We report a case of a 15-year-old boy who presented with profound visual loss and complete ophthalmoplegia after an uneventful spinal surgery for removal of cervical osteoblastoma. Postoperative visual loss following nonocular surgery is, fortunately rare, yet a devastating complication. The most common cause is ischemic optic neuropathy, but it can also be due to central retinal occlusion or cortical blindness. Visual loss in conjunction with ophthalmoplegia is rarely seen, and there are very few reports in the literature. We also review the related literature and highlight the mechanism and preventive measures.

[Eye and the pregnacy]. / Ochiul si sarcina.

Pregnancy is associated with changes in many organs and systems including the eyes. Sometimes in pregnancy may appear physiological and pathological ocular changes that may be associated with pre-existing problems. In such cases it is very important interdisciplinary collaboration gynecologist, ophthalmologist.

First description of posterior reversible encephalopathy syndrome as a complication of glycerolnitrate patch following open cardiac surgery.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) affects predominantly the parietal and occipital lobes. Frequent clinical features are epileptic seizure, altered mental status and visual disturbances. CLINICAL PRESENTATION: We present the first case of a patient with pericarditis and mitral valve insufficiency, who developed PRES after application of a glycerolnitrate patch day three post-operatively and whose neurological deficits improved within 2 days after withdrawal of patch therapy. CONCLUSION: The precise pathomechanism of PRES is unknown. The lower sympathetic innervation of the posterior circulation may be one explanation for its particular vulnerability to vasodilatation caused by glycerolnitrate.

Transient bilateral cortical visual loss after coronary artery bypass grafting in a normotensive risk-free patient.

BACKGROUND: Postoperative blindness has been mentioned as a rare complication of heart surgery. The majority of reported cases are caused by retinal artery occlusion or ischemic optic neuropathy. We report a case of transient visual loss due to cortical ischemia after coronary artery bypass grafting (CABG). CASE REPORT: A 52-year-old nondiabetic man developed complete bilateral visual loss immediately after CABG. He had been normotensive throughout the operation. An ophthalmologic exam detected no causative ocular damage. The results of a Doppler study of the carotid, vertebral, and ophthalmic arteries were completely normal, and an echocardiography examination showed no left ventricular clot. Magnetic resonance imaging of the brain, however, showed several ischemic plaques in watershed areas and a small subacute infarct in the occipital lobe. Recovery began on postoperative day 4, and the patient's vision was restored in 6 months. CONCLUSION: Although most cases of visual loss after open heart surgery have been caused by injuries to the peripheral optic system, cortical blindness may occur following open heart surgery in the absence of any preexisting risk factor. Fortunately, the course of recovery is promising one, as it was for our patient.

[Transient blindsight (type 2) after surgery for posterior cerebral artery aneurysm--case report].

In a 47-year-old woman experiencing pulsatile headache, radiological examination revealed a large right posterior cerebral artery (PCA) aneurysm. Preoperative neurological examination showed bilateral papilledema, but no visual disturbance. Cerebral angiography revealed that the aneurysm originated from the ambient segment of the PCA, and the posterior temporal, calcarine, and parieto-occipital arteries were all branched from the aneurysmal dome. Clipping of the feeding PCA trunk was performed via a left subtemporal approach. As a result of left occipital lobe infarction due to calcarine artery occlusion, right upper quadrant hemianopsia appeared after surgery, as confirmed by Goldman's perimetry. Other cerebral infarctions were identified in the junction between the left posterior internal capsule and thalamus, in the left posterior corpus callosum, and in the posterior base of the left temporal lobe due to the occlusion of the posterolateral thalamoperforating, posterior pericallosal, and posterior temporal arteries, respectively. The aneurysm was thrombosed and headache and papilledema subsided. The patient was able to detect the motion of a subject in a blind field, but consciousness of sight was absent. She was able to unconsciously move her finger toward a small penlight, and insert a paper into a slot with variable angles in the blind field. These phenomena disappeared within 4 months of the surgery. The results to forced choice tasks with figures (circle, cross, square, triangle, and star) and colors (red, blue, yellow, and green) were below chance levels. The present case was thus diagnosed as blindsight type 2 (gnosopsia) associated with awareness, probably due to transient activation of the dorsal "what" pathway among numerous visual processes.

Vision loss due to coincident ocular and central causes in a patient with Heidenhain variant Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease (CJD) is a degenerative disease of the brain associated with a rapidly progressive spongiform encephalopathy. Visual symptoms and neuro-ophthalmological signs are not infrequent, and presentation to an ophthalmologist may result. A case is reported of an 89-years-old gentleman who presented with a short history of isolated deterioration in vision. He underwent ocular intervention but subsequently developed progressive dementia, asterixis, myoclonus, cerebellar and extrapyramidal signs, and cortical blindness. An electroencephalogram was consistent with CJD. The patient progressively deteriorated and died 9 weeks after symptom onset. Limited post-mortem examination confirmed CJD.

Reversible posterior leukoencephalopathy syndrome after bevacizumab/FOLFIRI regimen for metastatic colon cancer.

OBJECTIVE: To describe a patient with reversible posterior leukoencephalopathy syndrome following the administration of bevacizumab (Avastin), a monoclonal antibody against vascular endothelial growth factor. DESIGN: Case report/literature review. SETTING: University hospital. PATIENT: A 52-year-old man receiving chemotherapy for stage IV rectal carcinoma. RESULTS: Clinical and radiographic evidence consistent with reversible posterior leukoencephalopathy syndrome was found following the administration of irinotecan hydrochloride, leucovorin calcium, and fluorouracil (FOLFIRI) regimen chemotherapy and bevacizumab. CONCLUSIONS: Reversible posterior leukoencephalopathy syndrome following treatment with angiogenesis modulators can occur. In addition to raising clinical suspicion in appropriate patients, this report may yield clues to the pathophysiologic underpinnings of reversible posterior leukoencephalopathy syndrome.

Anton's syndrome due to a giant anterior fossa meningioma. The problem of routine use of advanced diagnostic imaging in psychiatric care.

We present a case of blindness and Anton's syndrome in a psychiatric patient with late diagnosis of a giant frontal meningioma. The criteria for advanced diagnostic imaging in the psychiatric population are discussed. We conclude that MR or CT scan is indicated in psychiatric in-patients who fail to improve with standard psychiatric treatment. This strategy should be submitted to a cost-benefit analysis.

Cortical blindness: clinical and radiologic findings in reversible posterior leukoencephalopathy syndrome: case report and review of the literature.

PURPOSE: To alert ophthalmologists to the recognition of cortical visual loss as the presenting feature in patients with reversible posterior leukoencephalopathy syndrome (RPLES). Unique radiologic findings are paramount to the diagnosis. DESIGN: Interventional case report. METHODS: A patient was seen with perioperative bilateral cerebral visual loss that was misinterpreted initially as an irreversible ischemic event. Further detailed analysis of the radiologic findings and clinical history led to the correct diagnosis. MAIN OUTCOME MEASURES: Visual acuity and magnetic resonance imaging (MRI) of the brain. RESULTS: Recognition of the correct diagnosis of RPLES led to the institution of antihypertensive therapy and recovery of normal vision. CONCLUSIONS: The diagnosis of RPLES should be considered in all patients with acute cerebral visual loss, especially in the setting of recent surgery, blood transfusion, chemotherapy, immunosuppressant use, hypertension, eclampsia, or seizures. Prompt diagnosis requires close collaboration with a radiologist and an emergent MRI study, which ideally should include diffusion-weighted imaging with calculation of an apparent diffusion coefficient map. Differentiation from acute cerebral ischemia is important in order to avoid permanent visual loss by prompt and vigorous treatment of exacerbating factors such as intermittent hypertension. Prompt diagnosis will also help to avoid potentially dangerous invasive procedures such as thrombolytic therapy.

"Other" neurologic complications after cardiac surgery.

Compared to the neurologic morbidity of stroke and cognitive dysfunction, "other" neurologic complications involving injuries to the brachial plexus, phrenic nerve, cranial nerves, other peripheral nerves, as well as the visual pathways, have been disproportionately underrepresented in the cardiac surgery and anesthesiology literature. These injuries are often missed in the early postoperative period when attention is focused principally on recovery from the acute trespass of cardiac surgery and cardiopulmonary bypass. However, when these problems do become apparent, they can cause considerable discomfort and morbidity. An overview of the current concepts of injury mechanisms/etiology, diagnosis, prognosis, and when possible, prevention of these injuries is presented.

Posterior reversible encephalopathy syndrome due to severe hypercalcemia.

Posterior reversible encephalopathy syndrome (PRES) is a leukoencephalopathy clinically characterized by headache, altered mental status, visual loss and seizures. Neuroimaging demonstrates symmetrical posterior cortical and subcortical lesions. The exact pathophysiology is unknown but there is a strong association with immunosuppressants and hypertension. We report two cases of PRES in normotensive patients with severe hypercalcemia as the only identifiable cause. Possible pathophysiological mechanisms are discussed.