Acute Bilateral Visual Loss Research Articles

A Case of Systemic Lupus Erythematosus with Myelitis and Recurrent Neuromyelitis Optica

Neuromyelitis optica (NMO) is an idiopathic, severe inflammatory demyelinating disease of the central nervous system targeting optic nerves and the spinal cord. It is characterized by acute bilateral visual loss (optic neuritis), acute transverse myelitis, and tends to spare brain early in the disease course. NMO can occur as an isolated condition or secondary to infection, toxin exposure, and autoimmune disease including systemic lupus erythematosus (SLE), sarcoidosis, and Behcet’s disease. We experienced a case of SLE with myelitis and recurrent optic neuritis in a 28-year-old woman who presented with recurrent visual disturbance and sudden onset of paraplegia, and report here on this case along with a review of the relevant literature.

Acute Bilateral Visual Loss Related to Orthostatic Hypotension

A 50-year-old man had undergone lumbar vertebral surgery and was confined to bed in the supine position for three months. When he sat up from the prolonged supine position, he showed clinical signs of orthostatic hypotension and reported decreased vision in both eyes. He also had underlying anemia. Ophthalmologic findings suggested bilateral anterior ischemic optic neuropathy (ION) as the cause of the visual loss. Although there are numerous reports of ION in the setting of hemodynamic compromise, such as systemic hypotension, cases of ION-associated orthostatic hypotension are very rare.

Bilateral Visual Loss: More Than Meets The Eye

A 72-year-old woman presented with acute onset bilateral visual loss. She had no other symptoms or signs, but had a complex past medical history including blood transfusions and immunosuppression. T2-weighted magnetic resonance imaging demonstrated bilateral occipital lobe vasogenic edema, consistent with posterior reversible encephalopathy syndrome (PRES). Her vision improved with conservative management.

Acute bilateral blindness as a presenting symptom of Non-Hodgkin's lymphoma

NHL usually presents with lymphadenopathy or symptoms related to compression by the primary tumor of surrounding structures. While the head and neck region is a common site of involvement, blindness is rarely a presenting symptom. We report here the case of a child who presented to the emergency room with acute bilateral loss of vision and no other symptoms. Cranial imaging studies revealed a solid mass of the skull base with compression on optic nerves. Diagnosis of Burkitt's lymphoma was confirmed after biopsy. The patient had partial vision improvement two days after optic nerve decompression which was done immediately at the night of presentation.

Methanol poisoning: two case studies of blindness in Indonesia

We report two recent cases of methanol intoxication in French patients living in Bali. These intoxications were secondary to the consumption of adulterated liquor. Both patients presented acute bilateral loss of vision a few days after methanol ingestion with no sign of recovery. The fundus showed bilateral optic atrophy that was well correlated with retinal nerve fibre layer thickness measured with spectral-domain optical coherence tomography and compatible with toxic optic neuropathy. In one of the patients, macular swelling in one eye was observed. Electroretinograms were normal contrasting with abolished visual evoked potentials. Methanol was isolated from a sample of the beverage with gas chromatography. Methanol intoxication may occur in Asia and therefore adequate information for tourists and the local population is required.

Retinale Arteriitis in der Schwangerschaft

The case of a 35-year-old pregnant patient is described who presented with acute bilateral visual loss. As the reason for this a retinal arteritis could be demonstrated. The patient also suffered from acute unilateral hearing loss in combination with cerebral changes detectable by MRI. After exclusion of other causes the diagnosis of Susac syndrome (retinocochleocerebral microangiopathy) was made and appropriate therapy was initiated.

Central retinal artery occlusion in Wegener's granulomatosis: a diagnostic dilemma

PurposeTo report a case of central retinal artery occlusion (CRAO) in a patient with biopsy-verified Wegener's granulomatosis (WG) with positive C-ANCA.MethodsA 55-year-old woman presented with a 3-day history of acute painless bilateral loss of vision; she also complained of fever and weight loss. Examination showed a CRAO in the left eye and angiographically documented choroidal ischemia in both eyes.ResultsThe possibility of systemic vasculitis was not kept in mind until further studies were carried out; methylprednisolone pulse therapy was then started. Renal biopsy disclosed focal and segmental necrotizing vasculitis of the medium-sized arteries, supporting the diagnosis of WG, and cyclophosphamide pulse therapy was administered with gradual improvement, but there was no visual recovery.ConclusionCRAO as presenting manifestation of WG, in the context of retinal vasculitis, is very uncommon, but we should be aware of WG in the etiology of CRAO. This report shows the difficulty of diagnosing Wegener's granulomatosis; it requires a high index of suspicion, and we should obtain an accurate medical history and repeat serological and histopathological examinations. It emphasizes that inflammation of arteries leads to irreversible retinal infarction, and visual loss may occur

Acute Bilateral Visual Loss with Idiopathic Hypertrophic Pachymeningitis

Acute Bilateral Visual Loss with Idiopathic Hypertrophic Pachymeningitis

A case of Charles Bonnet syndrome following syphilitic optic neuritis

Charles Bonnet syndrome refers to visual hallucinations in patients with visual acuity loss or visual field loss without dementia. We report a case of Charles Bonnet syndrome following syphilitic optic neuritis. A 62-year-old man was admitted to our hospital suffering acute bilateral visual loss in a few months. On admission, he was almost blind and his optic discs were found to be atrophic on fundoscopy. In addition to increased cell counts and protein concentration in cerebrospinal fluid (CSF), serum and CSF rapid plasma reagin tests were positive. A diagnosis of syphilitic optic neuritis was made and he was treated with intravenous penicillin G (24 million units per day for 14 days) without any recovery. After treatment finished, he began to experience complex, vivid, elaborate and colored visual hallucinations. He recognized these visions as unreal and felt distressed by them. No cognitive impairment was observed on several neuropsychological tests. We diagnosed the patient as suffering from Charles Bonnet syndrome. Brain MRI revealed diffuse mild atrophy of the cerebral cortex and multiple T2 high signal intensity lesions in the deep cerebral white matter. Single photon emission computed tomography revealed decreased regional cerebral blood flow in bilateral medial occipital lobes. Administration of olanzapine resulted in a partial remission of visual hallucinations. Charles Bonnet syndrome following syphilitic optic neuritis is rare. In the present case, visual loss and dysfunction of bilateral medial occipital lobes may have triggered the visual hallucinations, which were alleviated by olanzapine.

Multiple Sclerosis on Steroids

A 57-year-old man developed acute bilateral vision loss clinically consistent with bilateral optic neuritis. Within 1 month of diagnosis, he developed progressive and severe neurologic dysfunction, and repeat MRI demonstrated enhancement of the optic chiasm and optic tracts, as well as a large enhancing lesion within the right parieto-occipital lobe. Stereotactic-guided brain biopsy demonstrated demyelination consistent with multiple sclerosis. A diagnosis of fulminant multiple sclerosis was made. The patient died within 2 months of diagnosis. Multiple sclerosis and a fulminant subtype known as Marburg disease are discussed.

Idiopathic Hypertrophic Pachymeningitis Presenting with Uveitic Glaucoma.

A 73-year-old man presented with uveitic glaucoma associated with headache. Because there were no active inflammatory signs, synechiolysis and cataract extraction were performed. Even after lowering intraocular pressure, he repeatedly complained of headache, but no neuroimaging was performed. Acute bilateral visual loss was occurred 3 months after cataract surgery. Ophthalmologic examination revealed bilateral optic neuritis and a Gd-DPTA-enhanced cranial magnetic resonance imaging (MRI) showed thickened enhancement of the dura and optic nerve sheath. A final clinical diagnosis of idiopathic hypertrophic pachymeningitis associated with optic neuritis was made.

Infliximab and anterior optic neuropathy: case report and review of the literature

Anti-tumor necrosis factor alpha (TNF alpha) agents have been increasingly used to treat patients with Crohn's disease, psoriatic arthritis, rheumatoid arthritis, ankylosing spondylitis, and persistent uveitis. We describe a 68-year-old man with Crohn's esophagitis who developed a possible bilateral toxic anterior optic neuropathy during infliximab infusion. This is an observational case report, with review of the PubMed literature from 1977 to present. This 68-year-old man with a 2-year history of Crohn's esophagitis developed acute bilateral visual loss during his third infliximab infusion. His clinical features and laboratory tests were characteristic for a bilateral anterior optic neuropathy. Only three cases of possible infliximab-associated anterior optic neuropathy have been reported in the literature to date. It is important to consider the possibility of anterior optic neuropathy, in addition to retrobulbar optic neuritis, in patients who experience sudden-onset visual loss while being treated with infliximab.

Acute bilateral vision loss as the presenting feature of subacute sclerosing panencephalitis

A 12-yr-old boy with an atypical presentation of subacute sclerosing panencephalitis (SSPE) is described. Bilateral macular chorioretinitis preceded the neurological symptoms by 3 weeks. Both visual and neurological features had an acute onset. Clinicians need to be aware that macular chorioretinitis in a child may be the heralding feature of SSPE.

Cortical blindness due to sagittal sinus thrombosis in acute lymphoblastic leukaemia

A 73-year-old patient undergoing treatment for relapsed acute lymphoblastic leukaemia (ALL) was admitted to our unit with severe pneumonia. He had just completed 4 weeks of reinduction chemotherapy using prednisolone, vincristine, daunorubicin and asparaginase and was in morphological remission from ALL. During the course of admission he developed acute complete bilateral visual loss with preserved pupillary responses. His conscious level was not impaired and there was no apparent neurological deficit in the limbs. These features suggested bilateral pathology affecting the occipital and/or posterior parietal cortices and we suspected venous infarction from a sagittal sinus thrombosis. This was confirmed on a contrast computed tomography (CT) scan (left), which showed the characteristic ‘delta sign’ a triangle of non-enhancing thrombus in the sagittal sinus (long arrow). CT (right) also demonstrated peri-ventricular white matter oedema (thin arrows) and atypical posterior parietal lobe infarction bilaterally (thick arrows). These venous infarcts had damaged both optic radiations and were responsible for the cortical blindness. We considered anticoagulation and antithrombin replacement but with established cortical infarcts the likelihood of significant visual recovery was low. We elected for supportive care only, because of his progressive respiratory failure. Venous thrombosis is a recognized complication of asparaginase therapy, having been reported in up to 37% of patients undergoing treatment with asparaginase for ALL. It is attributed to depletion of natural anticoagulants, particularly antithrombin. It tends to affect unusual areas, such as cerebral venous sinuses and upper limb veins. The risk appears to be higher in adults, in patients receiving Escherichia colirather than Erwinia-derived asparaginase and in patients receiving concurrent prednisolone therapy. Other common risk factors, such as immobility, sepsis and central venous catheters, can play a role in increasing this risk. Where venous sinus thrombosis is suspected it is important to perform a venous phase contrast CT where scanning starts at least 60 s post-injection. A thrombus is identified as an intra-sinus filling defect amidst the contrast in the venous sinus blood. A standard post-contrast CT is normally imaged at 20–30 s; this may be too early for contrast to reach cerebral venous sinuses and this life-threatening condition may be overlooked.

Demyelinating disease masquerading as a surgical problem: a case series.

IntroductionWe report three cases of demyelinating disease with tumor-like presentation. This information is particularly important to both neurosurgeons and neurologists who should be aware that inflammatory demyelinating diseases can present as a mass lesion, which is indistinguishable from a tumor, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease.Case presentationThe first patient was a 42-year-old Malay woman who developed subacute onset of progressive quadriparesis with urinary incontinence. Magnetic resonance imaging of her spine showed an intramedullary lesion at the C5-C7 level. She was operated on and biopsy was suggestive of a demyelinating disease. Retrospective history discovered two episodes of acute onset of neurological deficits with partial recovery and magnetic resonance imaging of her brain revealed demyelinating plaques in the centrum semiovale.The second patient was a 16-year-old Malay boy who presented with symptoms of raised intracranial pressure. A computed tomography brain scan revealed obstructive hydrocephalus with a lesion adjacent to the fourth ventricle. An external ventricular drainage was inserted. Subsequently, a stereotactic biopsy was taken and histopathology was reported as demyelination. Retrospective history revealed similar episodes with full recovery in between episodes.The third case was a 28-year-old Malay man who presented with acute bilateral visual loss and confusion. Magnetic resonance imaging of his brain showed a large mass lesion in the right temporoparietal region. Biopsy was consistent with demyelinating disease. Reexamination of the patient revealed bilateral papillitis and not papilledema. Visual evoked potential was prolonged bilaterally. In all three cases, lumbar puncture for cerebrospinal fluid study was not carried out due to lack of patient consent.ConclusionsThese cases illustrate the importance of considering a demyelinating disease in the differential diagnosis of a mass lesion. Critical analyses of clinical presentations coupled with good physical examination are vital in assisting clinicians to reach the correct diagnosis.

Bilateral central retinal artery occlusion in a patient with acute pancreatitis.

To illustrate a case of bilateral simultaneous central retinal artery occlusion in a patient with acute pancreatitis. This patient presented to the emergency room with a 4-day history of acute painless bilateral loss of vision and was then immediately referred to the on-call ophthalmologist. This patient is a 47-year-old white man with a chronic history of alcohol abuse. Laboratory workup and computed tomography abdominal imaging were diagnostic of acute pancreatitis. It is postulated that the resultant complement activation with subsequent leukoembolization along with the combined effect of other variables led to microvascular damage resulting in bilateral central retinal artery occlusion.

A case of early Creutzfeldt-Jakob disease presenting with acute bilateral visual loss

A case of early Creutzfeldt-Jakob disease presenting with acute bilateral visual loss

The Genetics of Leber Hereditary Optic Neuropathy—Prototype of an Inherited Optic Neuropathy with Mitochondrial Dysfunction

Leber Hereditary Optic Neuropathy is a maternally inherited condition that is characterized by acute or subacute bilateral loss of vision, usually in otherwise healthy young individuals. Several point mutations in the mitochondrial genome have been identified in patients with the condition. Scientific advances into a better understanding of the molecular pathogenesis have been hampered by the lack of an animal model for the disease. This article summarizes what is known about the clinical features, epidemiology and genetics of Leber Hereditary Optic Neuropathy and reviews recent experiments scientists have used in addressing the many unanswered questions that remain about the disease.

Primary Sinonasal Undifferentiated Carcinoma Presenting With Bilateral Retrobulbar Optic Neuropathy

A 43-year-old man presented with acute bilateral visual loss. Ophthalmologic examination revealed no light perception in the right eye and a visual acuity of 20/50 in the left eye with a right afferent pupillary defect. Ophthalmoscopic examination was normal. Brain MRI showed an intracranial but extra-axial mass in the floor of the anterior cranial fossa extending along the olfactory groove and into the sinonasal vault. Endoscopic biopsy showed a high-grade neoplasm consistent with sinonasal undifferentiated carcinoma. This case report highlights an unusual clinical presentation for this rare and aggressive neoplasm.

Transmission of heteroplasmic G11778A in extensive pedigrees of Thai Leber hereditary optic neuropathy

Leber hereditary optic neuropathy (LHON) is characterized by the acute or subacute bilateral painless loss of central vision, predominantly in young males. G11778A is the most common mitochondrial DNA mutation responsible for the disease. Thirty-seven percent of our LHON pedigrees (which is a much higher prevalence than that generally found) carried heteroplasmic G11778A. Analyses of four large Thai LHON pedigrees spanning four to six generations strongly suggested that the transmission of the heteroplasmic G11778A mutation is under selective pressure in favour of the mutated allele and that heteroplasmy influences the disease expression.