Search

A 30-years-old third gravida with previous normal pregnancies and an unremarkable prenatal course had an emergency lower segment caesarean section at a periphery hospital for failure of labour to progress. She developed bilateral cortical blindness immediately after recovery from anesthesia due to cerebral angiopathy shown by CT and MR scan as cortical infarct cerebral angiopathy, which is a rare complication of a normal pregnancy.

Objectives: Hypoglycemia is a significant problem in neonates, and a pattern of parietooccipital diffusion restriction on MRI scans has been reported. The purpose of this study was to determine whether hypoglycemic injury, as indicated by diffusion restriction in the occipital lobes, correlated with visual evoked potentials and long-term cortical visual dysfunction., Methods: A cohort of 45 neonates from 2000-2005 with diffusion-weighted MRI studies after hypoglycemia was studied retrospectively. Perinatal history and follow-up data were analyzed, and results were correlated with diffusion-weighted imaging findings.The presence of occipital diffusion restriction was assessed qualitatively, and the mean apparent diffusion coefficients of mesial occipital lobes were calculated., Results: Among 25 patients who underwent diffusion-weighted imaging within 6 days after the onset of hypoglycemia, restricted diffusion in the occipital lobes was found in 8 (50%) of 16 term infants but not in preterm infants. For the remaining 20 patients, who had diffusion-weighted imaging performed >6 days after the initial onset of hypoglycemia, occipital diffusion restriction was not seen, even if hypoglycemia was ongoing. Restricted diffusion was associated with abnormal visual evoked potentials detected within 1 week after birth. Cortical visual deficits were seen in a significant proportion of patients with recurrent hypoglycemia and were correlated significantly with low mesial occipital apparent diffusion coefficient values., Conclusions: Diffusion-weighted imaging studies performed within 6 days after initial hypoglycemia were sensitive in term but not preterm neonates. Diffusion restriction, with low apparent diffusion coefficient values, in the mesial occipital poles may indicate the prognosis for visual outcomes in acute settings after neonatal hypoglycemia.

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.

Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs. We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage. Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable.

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.

Unlabelled: The goal of the research is the evaluation of a complex and multidisciplinary assessment model medical and psychological, of children age 0 and 9 years old with neurological disorders with risk for cerebral visual impairments (CVI), in the frame of longitudinal and prospectively studies. The methods of complex assessment of children with CVI are the following: ophthalmological assessment; psychological assessment of general development on different area; functional vision assessment. These clinical methods are completed by para-clinical ones through magnetic resonance imaging and visual evoked potential. The results revealed the existence of a powerful and significant correlation among the level of brain damage and functional vision level involved in communication and achievement of different and concrete visual tasks., Conclusions: The cerebral visual impairment represents a severe degradation of vision, in spite of visual clinical behaviours, which can not explain and justify the severity of functional vision loss. But the complex assessment method proposed in the frame of this research underlined relevant information related with the general developmental level of children, the functional vision level and disease prognostic.

Study Design: Case report., Objective: To present and discuss the case of a patient who sustained a significant flexion compression injury of the cervical spine with resulting tetraplegia and development of cortical blindness., Setting: National Spinal Injuries Unit and Institute of Neurological Sciences, Southern General Hospital, Glasgow, Scotland, UK., Methods: Clinical and radiological follow-up of the patient., Results: Cortical blindness resulted from vertebral artery dissection associated with blunt cervical spine trauma. The patient is registered blind and is ventilator dependent., Conclusion: The potential complications of blunt vertebral artery injury remain poorly recognised. Screening is routinely not performed. Advances in noninvasive radiological techniques may result in recognition of asymptomatic disease and the potential for therapeutic intervention.

Purpose: To evaluate the etiology, prognosis, and associated neurological and ophthalmologic findings of children with cortical visual impairment (CVI) at a tertiary care referral facility., Methods: Records from patients visiting the Vanderbilt University Pediatric Ophthalmology Center during 2002 to 2005 were reviewed, and 98 patients were identified with an International Classification of Disease (9th ed.) coding of CVI (377.75). The charts were reviewed to assess presenting symptoms. The clinic and imaging notes were correlated with visual function (graded on a scale of I to VI)., Results: The most common etiologies were perinatal hypoxia (35%), prematurity (29%), hydrocephalus (19%), structural central nervous system abnormalities (11%), and seizures (10%). Many children (69%) had multiple etiologies. Associated ophthalmic abnormalities included esotropia (19%), exotropia (40%), nystagmus (21%), and optic atrophy (42%). Significant refractive error (> +3.00 D or < -2.00 D) was common (20%). Associated neurological findings included seizures (60%), cerebral palsy (37%), periventricular leukomalacia (12%), hemiparesis (21%), and hearing loss (11%). Fifty-three percent of children initially diagnosed with CVI were followed for a period of 0.5 to 10 years. Forty percent of the patients showed no improvement in visual function; 34% had minimal improvement, and 17% had mild improvement. Only 6% of the patients had significant improvement in visual function. Eight patients had fixing and following or better acuity at last follow-up., Conclusions: The major risk factors for CVI are perinatal hypoxia, premature birth, and hydrocephalus. Most patients have associated serious neurological and ophthalmologic abnormalities. While many patients have some recovery in vision acuity, most never see well. Patients with the most improvement in visual function were those having better initial acuity.

We present a patient with Posterior Reversible Encephalopathy Syndrome (PRES). A 74-year-old woman was admitted with sepsis, which originated from erysipelas on her neck the following day. She developed respiratory obstruction due to oedema, septic shock, disseminated intravascular coagulation (DIC), acute renal failure and atrial fibrillation. She responded well to treatment and improved rapidly, despite of her serious condition. When she had almost fully recovered after 15 days, her general condition worsened, and she developed confusion, blindness and pareses. MRI showed vasogenic oedema in the parietooccipital regions of the brain and in the cerebellum, consistent with PRES. PRES is a clinical and radiological diagnosis consisting of headache, confusion, cortical blindness, convulsions and sometimes pareses. MRI of the cerebrum with diffusion-weighted imaging (DWI) and Apparent Diffusion Coefficient (ADC) map are decisive to the diagnosis, and usually shows a characteristic bilateral vasogenic oedema in the parietooccipital region. This can distinguish PRES from brain infarction, which shows a cytotoxic oedema on MRI. We discuss our patient in the light of different conditions leading to PRES, possible pathophysiological factors and treatment options.

Background: We report a case of an adult with MMD who presented initially with left visual field defect that had suddenly progressed to bilateral cortical blindness 1 year and 9 months later., Case Description: A 33-year-old male presented with visual blurring and mild right hemiparesis that developed suddenly. He was regarded as having MMD based on an imaging study. An inverted encephalodurogaleosynangiosis using the left occipital artery and inverted encephaloduroarteriogaleosynangiosis with the parietal branch of the superficial temporal artery were done. The postoperative course was uneventful and without any complication. Several months later, he visited again because of sudden bilateral blindness preceded by repeated transient blindness in both eyes for 4 days. The right posterior temporal artery, which was well visualized at the time of the first postoperative follow-up angiography, was no longer seen on repeated 4-vessel angiography. Biochemical analysis including prothrombin and thrombin time, activated partial thromboplastin time, fibrinogen, d-dimer, plasminogen, antithrombin, platelet, protein C and S, lupus anticoagulant, and cardiolipin antibodies; VDRL was within the reference range., Conclusion: The authors suggest abnormal thromboembolism as a presumed mechanism of the pathogenesis of MMD in this patient.

Anosognosia for cortical blindness, also called Anton's syndrome, is a rare neurological disorder usually following bilateral lesions to occipital cortices. Neuropsychological, morphological and functional neuroimaging (SPECT and fMRI) findings are reported in a patient who incurred Anton's syndrome after an ischaemic lesion confined to the left occipital lobe involving the corpus callosum. The present case study suggests that Anton's syndrome may also follow from lesions disconnecting the occipital cortices.

Aim: To review the medical records of seven children with a delayed diagnosis of cerebral visual impairment., Methods: The charts of a consecutive series of children examined in a university based ophthalmology clinic with a delayed diagnosis of cerebral visual impairment were reviewed. Their neuroimaging studies were evaluated by a paediatric neuroradiologist., Results: The seven patients ranged in age from 2 years to 10 years at the time of presentation and had acuities ranging from 20/40 to 20/400. Their visual fields ranged from full visual fields to homonymous hemianopias. Neuroimaging findings ranged from subtle occipital lobe abnormalities to obvious cerebral volume loss., Conclusions: Cerebral visual impairment can be cryptic in children with mild visual impairment. Neuroimaging studies and visual field testing can help to differentiate this condition from other causes of visual impairment.

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.

Purpose: To explain the rare phenomenon of acute transient bilateral blindness without additional ophthalmological or neurological symptoms and signs., Methods: Six patients with isolated bilateral visual loss lasting 1-15 mins and occurring simultaneously in both eyes were evaluated. Clinical observation, neuroimaging (CT, MRI, MR-angiography), extra- and transcranial Doppler and vascular risk factors assessment were performed., Results: Cortical blindness due to bilateral occipital lobe transient ischaemic attacks (TIAs) was established as the most likely cause of acute transient visual loss in all patients., Conclusion: We suggest that in cases of transient bilateral blindness with sudden onset, appearing simultaneously in both visual fields, a bilateral ischaemia of the visual cortex should be suspected even if other neurological symptoms are lacking. Patients should be managed in the same way as patients with vertebrobasilar TIAs.

Neurocysticercosis (NCC) remains a major public health problem in developing countries as it is the most common helminthic infection of the central nervous system. Clinical manifestations are non-specific and pleomorphic. Case reports on uncommon presentations of NCC are few. We report six interesting cases of NCC with unusual clinical presentation that demonstrate this spectrum of pleomorphism. These include extrapyramidal disease (parkinsonism and focal dystonia), Kluver-Bucy syndrome, Weber's syndrome, dementia and cortical blindness. The clinical details and possible mechanisms for the uncommon presentations are also discussed. Thus, a high level of suspicion should be kept for NCC, especially in endemic zones and developing countries.

Background: Posterior Reversible Encephalopathy (PRES) is characterized by acute-onset headache, altered mental status, cortical blindness and seizures, with parietal-occipital involvement. We report all cases of PRES diagnosed in our intensive care unit during the last 4 years, and evaluate their outcome in terms of the different medical treatments used. Even if usually reversible, PRES can sometimes result in death or irreversible neurological deficit, such as chronic epilepsy., Material/methods: From January 2001 to January 2005, we identified 8 female patients with PRES. All patients underwent basal and follow-up brain MRI. Patients referred to Epilepsy Center (about 3000 patients) were reviewed in order to identify subjects with a clinical history of PRES. These latter were clinically re-evaluated and underwent video-EEG, MRI study and neuropsychological testing., Results: Of the 8 patients, 5 had hypertensive encephalopathy during pregnancy; 2 had eclampsia during the postpartum period; 1 patient with chronic renal failure developed symptoms after immunosuppressive treatment. In all patients but 1, neurological and radiological abnormalities resolved after appropriate treatment. In addition, we found 2 patients with temporal lobe epilepsy subsequent to a previous PRES. MRI revealed cortical-subcortical malacia in the parietal-occipital regions., Conclusions: The widespread use of MRI technology has made PRES familiar to many clinicians. Although PRES is reversible when treatment is instituted, delayed diagnosis and therapy can result in chronic neurological sequelae. The relationship between hypertensive encephalopathy and chronic epilepsy needs to be confirmed by longitudinal studies. Normalization of blood pressure and treatment of seizures deserves particular attention.

The most common neurologic manifestations of acute intermittent porphyria (AIP) are autonomic visceral neuropathy, peripheral motor neuropathy, and CNS dysfunctions including seizures and neuropsychiatric disturbances. In rare instances, however, AIP patients have presented with acute cortical blindness. We present a 20-year-old woman who suffered her first attack of AIP. Following 1 week of abdominal pain, she was transferred from a surgical department because of sudden visual loss and deterioration of consciousness. On admission, she developed several generalized seizures. Magnetic resonance imaging showed bilateral DWI lesions occipitally and in the left anterior circulation. Cerebrospinal fluid, MR angiography, and duplex ultrasound were normal. On the following day, sedation and intubation became necessary because of a generalized status epilepticus. Analysis of porphyrinogens in blood, urine and stool showed significantly elevated values. Intravenous therapy with häm-arginate was initiated and antiepileptic therapy was changed to gagabentine. Under this therapeutical regime she remained stable and extubation was possible 48 h later.

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.

We report the clinical and radiologic features of a 31-year-old woman who suffered incongruous binasal and bitemporal visual field defects and severe sudden visual loss due to hypoperfusion of bilateral lateral geniculate bodies following anaphylactic shock induced by 500 mg amoxicillin per os. Complete neuroophthalmologic examinations were performed regularly for visual acuity, color vision, pupillary reflexes, and visual fields. Additional testing was performed by means of MR imaging of the brain and CSF analysis. Follow-up was performed for 12 months. Vision loss was acute and severe, its onset bilateral and simultaneous. The patient recovered visual acuity of 1.0 within 7 weeks. Color vision was abnormal in both eyes but gradually improved to normal. Visual fields were characterized by incongruous binasal and bitemporal defects, but they reduced progressively. Cerebral MR imaging confirmed the presence of symmetrical lesions confined exclusively within both lateral geniculate bodies. These lesions were best seen on T1-weighted and fluid-attenuated inversion recovery images as high-signal-intensity areas suggestive of hemorrhagic ischemia. CSF analysis was normal and aseptic. Blood tests and cultures excluded any microbial infection. We conclude that shock may induce a bilateral isolated ischemia of the lateral geniculate bodies, resulting in incongruous binasal and bitemporal visual field defects and severe visual loss. MR imaging is the optimal imaging technique to confirm the diagnosis and for follow-up., (Copyright American Society of Neuroradiology)

A 4-year-old male presented with only acute vision loss. His neurologic examination, funduscopic examination, and pupils were normal. Cranial magnetic resonance imaging revealed abnormal hyperintense, bilaterally symmetric lesions (on T(2)-weighted and fluid-attenuated inversion recovery, images) in bilateral optic radiations, pulvinar region in the thalami, crus posterior of internal capsules, periventricular white matter, and unilaterally left anterior pons. Elevated measles antibody titers in the cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. Vision loss improved and cranial magnetic resonance imaging findings regressed,but myoclonic jerks and deterioration began 7 months later. The diagnosis of subacute sclerosing panencephalitis should be considered in cases with acute vision loss resulting from cortical blindness even when classical findings of the central nervous system do not exist.

The clinical assessment of vision by means of optotypes does by no means test just two-point resolution, since a correct naming of the letters or digits requires a preceding visual object recognition. Cortical lesions can massively deteriorate vision up to a "Seelenblindheit" in spite of intact optics and retina. There are different processing levels involved in the analysis which can be individually defective, leading to disorders from visual indiscrimination to agnosia or anomia.

We present a case of eclampsia complicated by alexia without agraphia. To our knowledge, this syndrome has never before been described in the literature.

We report on a 36-year-old man with a history of mild head trauma. The initial clinical findings and the CT-scan of the brain revealed no pathological result, although the patient suffered from weakness of the right arm and bilateral blindness. Those findings were interpreted as psychogenic disorder. Nine days later he developed an instable gait, a child like attitude, amnesia and enuresis. The CT-scan revealed a subacute bilateral occipital stroke in the region of the arteriae cerebri posteriors. No cause for the stroke was found. In spite of the rareness of cortical blindness in young people as a cause of stroke, a detailed medical history and clinical examination should always be performed, and by unclearness additional investigations should be considered.

Purpose: To describe the clinical features of a visual variant Alzheimer's disease, a disorder that might be seen by the ophthalmologist with visual symptoms and signs., Design: Retrospective case series., Participants: Eight patients with Alzheimer's disease presenting with predominantly visual complaints., Methods: A retrospective review of patients at a tertiary care academic center seen by the authors from 1999 to 2001 with Alzheimer's disease and predominantly visual complaints., Results: Eight patients with the visual variant of Alzheimer's disease were reviewed. All had seen eye care providers before referral to the neuro-ophthalmology clinic for visual complaints but without a diagnosis. Four patients had homonymous visual field loss, and two had presumed cortical visual impairment. Neuroimaging showed either normal brain (1 patient) or atrophy of the parietal or occipital areas but no structural lesions (7 patients). Fluoro-18-deoxyglucose positron emission tomography scans were performed in five of the cases, and all showed hypoperfusion in the parietooccipital areas. Neuropsychologic testing revealed visuospatial deficits in all 5 patients tested., Conclusions: Visual symptoms might be the presenting (or rarely only) manifestation of Alzheimer's disease. Clinicians should be aware of the visual variant of Alzheimer's disease. Neuropsychological testing and magnetic resonance imaging (MRI) provide supporting evidence for the diagnosis. Positron emission tomography scans might be helpful in selected cases, especially those with a normal MRI.

We describe MR findings in three patients who experienced transient cortical blindness following cerebral angiograms. All angiograms were performed by using the same nonionic contrast medium. On the basis of similar clinical and radiologic findings, we believe that this entity is closely related to and probably has the same pathophysiology as posterior reversible leukoencephalopathy.

Focal ischemic brain injury, or stroke, is an important cause of later handicap in children. Early assessment of structure-function relationships after such injury will provide insight into clinico-anatomic correlation and potentially guide early intervention strategies. We used combined functional MRI (fMRI) with diffusion tensor imaging (DTI) in a 3-month-old infant to explore the structure-function relationship after unilateral perinatal stroke that involved the visual pathways. With visual stimuli, fMRI showed a negative BOLD activation in the visual cortex of the intact right hemisphere, principally in the anterior part, and no activation in the injured hemisphere. The functional activation in the intact hemisphere correlated clearly with the fiber tract of the optic radiation visualized with DTI. DTI confirmed the absence of the optic radiation in the damaged left hemisphere. In addition, event-related fMRI (ER-fMRI) experiments were performed to define the characteristics of the BOLD response. The shape is that of an inverted gamma function (similar to a negative mirror image of the known positive adult BOLD response). The maximum decrease was reached at 5-7 s with signal changes of -1.7 +/- 0.4%.Thus, this report describes for the first time the combined use of DTI and event-related fMRI in an infant and provides insight into the localization of the fMRI visual response in the young infant and the characteristics of the BOLD response.

Visual disturbances occur in up to 25 % of the patients with preeclampsia. However, blindness remains a rare phenomenon. A 39 year old primigravida was admitted for observation at 30 weeks gestation with signs of preeclampsia. After 11 days she suffered a complete loss of vision. The blindness reversed completely after cesarean section and antihypertensive treatment. Blindness in preeclamptic patients is mostly caused by hypertensive encephalopathy. We discuss pathophysiological aspects as well as diagnostic approaches and therapeutic options with respect to the available literature.

Cortical blindness is an uncommon, but dramatic, complication of preeclampsia. We present a case in which diffusion-weighted magnetic resonance imaging played a critical role in determining the etiology of the cortical blindness and its subsequent management.

A 21-year-old male presented with occipital lobes that were extensively damaged by bilateral infarcts present at birth. The absence of the striate cortex was confirmed with anatomic and functional MRI and high-resolution EEG. His cortical visual impairment was severe, but he retained a remarkable ability to see fast-moving stimuli. Horizontal optokinetic nystagmus could be elicited from either eye. Resolution acuity was close to normal providing the patient was allowed to move his head and eyes. The direction of motion in random-dot patterns could be discriminated with perfect accuracy at speeds above 2 deg/s, and the patient reported that he could 'see' the motion at fast but not at slow speeds. This conscious residual vision for motion is known as Riddoch's phenomenon, but it has never been reported in the complete absence of the striate cortex. Functional neuroimaging revealed activation that was outside the motion-responsive regions of the extrastriate cortex. This case demonstrates remarkable plasticity in the human visual system and may have implications for understanding the functional organization of the motion pathways.

Purpose: To present a case of permanent cortical blindness (CB) following hepatic encephalopathy., Methods: Systematic clinical examination and further investigations to elicit the diagnosis., Conclusion: Complete CB is an uncommon neurological disorder and is rarely persistent. In rare cases of hepatic encephalopathy, the visual cortex may be affected and CB may occur, which resolves following treatment of the hepatic encephalopathy. We report a previously unreported complication involving permanent CB following hepatic encephalopathy in a young man.

We describe the case of a female patient affected by migraine and untreated adult celiac disease who presented with a state of acute migraine accompanied by multiple neurological deficits, including transient cortical blindness with ischemic CT and MRI alterations, and hypocoagulation due to factor VII deficiency. She was receiving estroprogestin therapy. There was a prompt response to cortisone therapy followed by a state of complete well-being, which also led to the disappearance of migraine attacks after five years of dietary treatment alone.

Cyclosporine (CYA) is a calcineurin inhibitor widely used in immunosuppressive regimens after organ transplantation. Several neurologic side effects are frequently associated with CYA use; however, reversible cortical blindness is a rare manifestation of CYA toxicity traditionally seen after liver and bone marrow transplantation. This report presents a case of reversible cortical blindness after lung transplantation, then details the risk factors and clinical course of 28 previously well-documented cases of CYA-induced cortical blindness after transplantation. Identification of known risk factors, clinical clues, and typical radiographic findings may aid in the diagnosis of CYA-induced cortical blindness, since reduction in CYA dose or cessation of CYA therapy usually permits resolution of the neurologic effects.

Transient cortical blindness is an uncommon but well-known complication following cerebral angiography. One possible cause of this complication is an adverse reaction to contrast agent, resulting in an osmotic disruption of the blood-brain barrier that seems to be selective for the occipital cortex. We report the case of a 16-year-old male patient with cortical blindness after intravenous application of nonionic contrast agent during CT angiography performed because of seizure that was attributed to thrombosis of the basilar artery on the basis of clinical findings. To our knowledge, the development of cortical blindness after CT angiography has not been described in the literature. The patient's symptoms were triggered by hyponatriemia and diabetes insipidus.

Although unilateral epidural hematomas are well known, bilateral presentation, especially at the confluens sinuum, is uncommon, and its diagnosis may be delayed, thus causing morbidity. We report a case of acute posttraumatic cortical blindness due to epidural hematoma at the confluens sinuum caused by venous sinus tearing. The epidural hematoma was evacuated and the dural tear was repaired. The patient remained alert throughout the hospitalization. Examination of the visual field revealed recovery postoperatively, and the patient was discharged. We discuss the pathological mechanisms of transient blindness. The importance of anopsia as an early sign of epidural hematoma is emphasized., (Copyright 2003 S. Karger AG, Basel)

Transient visual loss may occur with occipital seizures as an ictal or post-ictal phenomenon. Its duration varies from less than one minute to days, or can be permanent. We describe a 61-year-old man presenting with headache, vomiting and bilateral visual loss. EEG revealed persistent spike discharge in the occipital lobes suggesting occipital seizures. His vision improved with carbamazepine.

Post-infectious acute glomerulonephritis with hypertensive encephalopathy is characterized by episodic hypertension with headache, vomiting, and hematuria. The association between hypertensive encephalopathy and cortical blindness in children with acute glomerulonephritis is extremely rare. We report the case of a 10-year-old boy with acute glomerulonephritis who presented with gross hematuria, headache, vomiting, and oliguria, and developed transient cortical blindness as a complication of hypertensive encephalopathy. No occurrence of seizure was observed during the clinical course. T2-weighted cranial magnetic resonance imaging showed a high-intensity signal over cortical and subcortical areas of bilateral occipital regions. His vision recovered fully, 2 days after receiving antihypertensive therapy. The patient was well without complaint at 1-year follow-up. This case highlights the possibility that cortical blindness may develop as a complication of acute glomerulonephritis in children. Prevention of the occurrence of neurological deficits in children with acute glomerulonephritis and hypertensive encephalopathy requires careful evaluation and appropriate management of hypertension.

Acute blindness is a rare presentation of epileptic seizures, referring to loss of sight without loss of consciousness associated with electroencephalographic (EEG) epileptic discharges, mainly representing an ictal phase but also either pre- or postictal. We report a series of 14 children with documented epileptic blindness, describing the accompanying fits and thereafter the response to therapy to resolve the blindness and control associated seizures. All patients experienced episodes of acute complete visual obscuration lasting for 1 to 10 minutes. Seven patients hadaccompanying generalized seizures, with a photosensitive response recorded in three of them. All of these seven children were treated with valproic acid, regaining full vision, and six of them became seizure free. Three patients with acute blindness who had accompanying focal motor seizures and unilateral temporooccipital posterior epileptic discharges were treated with carbamazepine regained full vision and complete seizure control. Four additional children had the constellation of migrainous headaches, focal motor phenomena, and complete blindness, along with occipital discharges compatible with Gastaut syndrome, benign childhood epilepsy with occipital paroxysms. All four patients were started on carbamazepine and became asymptomatic. Our overall experience suggests that epileptic blindness in children is associated with a favorable outcome when promptly diagnosed and treated appropriately, resulting in complete resolution of blindness in all children and satisfactory control of seizures in most of them. We therefore recommend performing a prompt EEG in any child presenting with acute visual obscuration, even in the absence of other epileptic phenomena.

Objectives: To characterise the nature of cognitive change in Creutzfeldt-Jakob disease (CJD)., Methods: Case histories are reported of four patients with sporadic (sCJD) and two with familial CJD (fCJD), with postmortem pathological findings in four cases. The data derived from cognitive examination are examined with respect to the presence or absence of a variety of characteristics to elicit performance profiles across cognitive domains., Results: Three patients with sCJD exhibited clear focal cortical deficits. One patient had visual impairment leading to cortical blindness, associated with posterior hemisphere abnormalities on single photon emission computed tomography (SPECT) imaging; two others had impairments in language, mirrored by left hemisphere SPECT abnormalities. The remaining three patients showed no specific cortical symptomatology. Despite these differences all six patients shared common qualitative characteristics: episodic unresponsiveness, interference effects, and profound verbal and motor perseveration. These common features are interpreted in terms of impaired activation and regulation of neocortex from subcortical structures. Findings from postmortem pathological examination and from the published literature provide converging evidence to implicate a critical role of the thalamus., Conclusion: These preliminary findings suggest that sCJD and fCJD may be associated with distinct neuropsychological characteristics.

Cortical blindness (or more generally, cerebral blindness) consists of bilateral visual loss with normal ocular examination and normal pupillary function, related to bilateral occipital lesions. Its most frequent cause in adults is hypoperfusion in the vertebral-basilar arteries, although other curable causes are described. Other syndromes, related to lesions of associative cortical areas or occipital-fugal connections may cause poorly defined visual complaints. Their recognition by the ophthalmologist is mandatory, as they may concern a potentially curable underlying disease.

In two patients, a 60-year-old woman and a 63-year-old man, cerebral blindness was diagnosed. The first patient developed the disorder shortly after cerebral angiography was carried out to diagnose a carotid-cavernous fistula. The symptoms disappeared after three days. The second patient suffered from a reversible posterior leuko-encephalopathy syndrome. He recovered his vision without specific treatment. Cortical or cerebral blindness is a form of blindness caused by a lesion in the cerebral part of the visual pathways. Although in itself rare, cerebral blindness occurs frequently as a complication of non-neurologic diseases or interventions. Physicians who are unaware of this syndrome tend to diagnose these patients as 'hysteric', since normal pupillary reactions and normal eye movements accompany the loss of vision. If the blindness is not caused by an ischaemic infarct, it is usually reversible.