Valérie Biousse's research while affiliated with Emory University and other places

Publications (331)

Article
Hypertensive eye disease includes a spectrum of pathological changes, the most well known being hypertensive retinopathy. Other commonly involved parts of the eye in hypertension include the choroid and optic nerve, sometimes referred to as hypertensive choroidopathy and hypertensive optic neuropathy. Together, hypertensive eye disease develops in...
Article
Objectives Diffusion weighted imaging hyperintensity (DWI-H) has been described in the retina and optic nerve during acute central retinal artery occlusion (CRAO). We aimed to determine whether DWI-H can be accurately identified on standard brain magnetic resonance imaging (MRI) in non-arteritic CRAO patients at two tertiary academic centers. Mate...
Article
Full-text available
Background Leber Hereditary Optic Neuropathy (LHON) is a rare, maternally-inherited mitochondrial disease that primarily affects retinal ganglion cells (RGCs) and their axons in the optic nerve, leading to irreversible, bilateral severe vision loss. Lenadogene nolparvovec gene therapy was developed as a treatment for patients with vision loss from...
Article
Purpose of review: The aging world population accounts for the increasing prevalence of neurodegenerative diseases such as Alzheimer's and Parkinson's which carry a significant health and economic burden. There is therefore a need for sensitive and specific noninvasive biomarkers for early diagnosis and monitoring. Advances in retinal and optic ne...
Article
Background: Radiologic findings of intracranial hypertension (RAD-IH) are common in idiopathic intracranial hypertension (IIH) patients. Paralleling the increasing rates of obesity, the burden of IIH is growing. Urgent neuro-ophthalmology consultations for possible IIH in patients with incidentally detected RAD-IH are increasing, with many patient...
Article
Background Patients with idiopathic intracranial hypertension (IIH) have elevated intracranial pressure (ICP) of unclear etiology. This study evaluated the ability of quantitative intracranial Hounsfield unit (HU) histogram analysis to detect pathophysiological changes from elevated ICP in the setting of a normal head CT. Methods Retrospective ana...
Article
Background: The Neurological Pupil index (NPi) provides a quantitative assessment of pupil reactivity and may have prognostic value in patients with subarachnoid hemorrhage (SAH). We aimed to explore associations between the NPi and clinical outcomes in patients with SAH. Methods: A retrospective analysis of 79 consecutive patients with acute SA...
Article
Background: The examination of the optic nerve head (optic disc) is mandatory in patients with headache, hypertension or any neurological symptoms, yet it is rarely or poorly performed in general clinics. We recently developed a deep learning system (BONSAI-DLS) capable of accurately detecting optic disc abnormalities including papilledema (swellin...
Article
Background: Central retinal artery occlusion (CRAO) is a medical emergency, and patients who present acutely should be immediately referred to the nearest stroke center. We evaluated practice patterns for CRAO management at one academic center over the last decade. Methods: This was a retrospective study on all adult patients diagnosed with a CR...
Article
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PurposeBrain tumors are the leading cause of death from childhood cancer. Although overall survival has improved due to earlier detection, better therapies, and improved surveillance, visual dysfunction and impaired vision-related quality-of-life (VR-QOL) are often unrecognized in children. This project investigated VR-QOL in pediatric brain tumor...
Article
Objective Acute central retinal artery occlusion (CRAO) is an emergency with poor visual outcome. Intravenous thrombolysis within 4.5 h of vision loss is safe and may improve vision, but is rarely administered because of frequent delays in presentation. We describe a subgroup of CRAO patients presenting within 24 h of vision loss to a tertiary care...
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Full-text available
Background: RESCUE and REVERSE were 2 Phase 3 clinical trials that assessed the efficacy and safety of intravitreal gene therapy with lenadogene nolparvovec (rAAV2/2-ND4) for the treatment of Leber hereditary optic neuropathy (LHON). RESTORE is the long-term follow-up study of subjects treated in the RESCUE and REVERSE trials. Methods: In RESCUE...
Conference Paper
Full-text available
Objectives MRI signs of intracranial hypertension (MRI-IH) are classically associated with idiopathic intracranial hypertension (IIH), but also detected in asymptomatic individuals without papilledema. The objective of this study was to determine the prevalence of MRI-IH in consecutive outpatients undergoing brain MRI for any clinical indication, a...
Chapter
Visual loss is a common symptom in neurologic emergencies. Although ocular causes of visual loss are usually identified by eye care specialists, many patients appear in the emergency department or a neurologist’s office when the ocular examination is normal or when it suggests a neurologic disorder. Indeed, many causes of monocular or binocular acu...
Article
Objective: This report presents a cross-sectional analysis of the baseline characteristics of subjects with Leber hereditary optic neuropathy enrolled in the gene therapy trials RESCUE and REVERSE, to illustrate the evolution of visual parameters over the first year after vision loss. Methods: RESCUE and REVERSE were 2 phase III clinical trials...
Article
Central retinal artery occlusion (CRAO) is a form of acute ischemic stroke which affects the retina. Intravenous thrombolysis is emerging as a compelling therapeutic approach. However, it is not known which patients may benefit from this therapy because there are no imaging modalities that adequately distinguish viable retina from irreversibly infa...
Article
Background Central retinal artery occlusion (CRAO) causes sudden, irreversible blindness and is a form of acute ischemic stroke. In this study, we sought to determine the proportion of patients in whom atrial fibrillation (AF) is detected by extended cardiac monitoring after CRAO. Methods We performed a retrospective, observational cohort study us...
Article
Objective To evaluate the performance of a deep learning system (DLS) in classifying the severity of papilledema associated with increased intracranial pressure, on standard retinal fundus photographs. Methods A DLS was trained to automatically classify papilledema severity in 965 patients (2103 mydriatic fundus photographs), representing a multie...
Article
Full-text available
Objective: This work aimed to compare the evolution of visual outcomes in Leber hereditary optic neuropathy (LHON) patients treated with intravitreal gene therapy to the spontaneous evolution in prior natural history (NH) studies. Design: A combined analysis of two phase three randomized, double-masked, sham-controlled studies (REVERSE and RESCUE)...
Article
Full-text available
Background/objectives REALITY is an international observational retrospective registry of LHON patients evaluating the visual course and outcome in Leber hereditary optic neuropathy (LHON). Subjects/methods Demographics and visual function data were collected from medical charts of LHON patients with visual loss. The study was conducted in 11 stud...
Article
Importance: Magnetic resonance imaging (MRI) signs of intracranial hypertension (IH) are traditionally associated with idiopathic intracranial hypertension (IIH), but these signs are also detected among individuals with primary headaches and among asymptomatic individuals without papilledema. Objective: To examine the prevalence of MRI signs of IH...
Article
Background: A relationship between idiopathic intracranial hypertension and spontaneous skull base cerebrospinal fluid (CSF) leaks has been proposed, by which CSF leak decreases intracranial pressure (ICP) and masks the symptoms and signs of elevated ICP. These patients are at risk of developing papilledema, symptoms of elevated ICP, or a recurren...
Article
Objective: To prospectively examine diagnostic error of neuro-ophthalmic conditions and resultant harm at multiple sites. Design: Prospective cross-sectional study. Subjects: 496 consecutive adult new patients seen at three university-based neuro-ophthalmology clinics in the United States in 2019-2020. Methods: Collected data regarding demog...
Article
Purpose Central retinal artery occlusion (CRAO) is a form of acute ischemic stroke that causes severe visual loss and is a harbinger of further cerebrovascular and cardiovascular events. There is a paucity of scientific information on the appropriate management of CRAO, with most strategies based on observational literature and expert opinion. In t...
Article
A 55-year-old female presented with binasal visual field defects and normal neuro-ophthalmic examination. Slit-lamp examination demonstrated posterior subcapsular cataracts that were located temporally in the visual axis. Due to the location of the nodal point in the eye, her visual field defect was contralateral to the opacity. Her visual field de...
Article
Inner retinal thinning on optical coherence tomography (OCT) occurring through retrograde trans-synaptic degeneration is an increasingly recognized phenomenon, even in acquired retro-chiasmal brain lesions. We describe a man with stable visual field defects from multiple bilateral posterior circulation infarctions, who had ganglion cell complex (GC...
Article
A 35-year-old myopic woman developed right-eye optic disc edema with normal visual function. The presence of a subtle crescent-shaped peripapillary subretinal hemorrhage in addition to the disc edema raised concern for a peripapillary choroidal neovascular membrane, which was confirmed by enhanced depth optical coherence tomography.
Article
Full-text available
Purpose: To evaluate the efficacy of a single intravitreal injection of rAAV2/2-ND4 in subjects with visual loss from Leber hereditary optic neuropathy (LHON). Design: RESCUE is a multicenter, randomized, double-masked, sham-controlled, phase 3 clinical trial. Participants: Subjects with the m.11778G>A mitochondrial DNA mutation and vision los...
Article
Background: Unilateral isolated optic disc edema (UIODE) represents a challenging clinical presentation that frequently precipitates an extensive diagnostic work-up. Patients without an apparent diagnosis despite appropriate investigations are often categorized as having "papillophlebitis," an entity that is poorly defined in the existing literatu...
Article
Full-text available
REVERSE is a randomized, double-masked, sham-controlled, multicenter, phase 3 clinical trial that evaluated the efficacy of a single intravitreal injection of rAAV2/2- ND4 in subjects with visual loss from Leber hereditary optic neuropathy (LHON). A total of 37 subjects carrying the m.11778G>A ( MT-ND4 ) mutation and with duration of vision loss be...
Article
Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology that results in isolated raised intracranial pressure. Classic symptoms and signs of IIH include headache, papilledema, diplopia from sixth nerve palsy and divergence insufficiency, and pulsatile tinnitus. Atypical presentations include: (1) highly asymmetric or even unila...
Article
Acute retinal vascular occlusions are common causes of visual impairment. Although both retinal artery occlusions and retinal vein occlusions are associated with increased age and cardiovascular risk factors, their pathophysiology, systemic implications, and management differ substantially. Acute management of retinal artery occlusions involves a m...
Article
Perineural spread (PNS) to cranial nerves (CNs) by cutaneous malignancies is difficult to diagnose given the indolent course and often late or absent findings on brain imaging. A 68-year-old white man with multiple cranial neuropathies secondary to PNS by squamous cell carcinoma had negative high-quality neuroimaging for 5.25 years. He first develo...
Article
Acute central retinal arterial occlusion has a very poor visual prognosis. Unfortunately, there is a dearth of evidence to support the use of any of the so-called “conservative” treatment options for CRAO, and the use of thrombolytics remains controversial. In this review, we address a variety of these “conservative” pharmacologic treatments (pento...
Article
Purpose: The purpose of this study was to evaluate whether papilledema severity is associated with specific demographic or clinical factors in patients with idiopathic intracranial hypertension (IIH). Materials and methods: A retrospective cohort study of consecutive IIH patients seen at one tertiary care institution between 1989 and March 31, 2...
Article
Background: Ocular fundus abnormalities, especially intraocular hemorrhage, may represent a clinically useful prognostic marker in patients with acute subarachnoid hemorrhage (SAH). Objective: To evaluate associations between ocular fundus abnormalities and clinical outcomes in acute SAH. Methods: Prospective evaluation of acute SAH patients w...
Article
Objective: To determine the prevalence of fundus pathology in metropolitan Australian EDs utilising a non-mydriatic fundus photography screening programme. Secondary objectives include diagnostic accuracy among emergency physicians compared to telehealth ophthalmologist review. Methods: Prospective cross-sectional study investigating non-mydriat...
Article
Vision problems from giant cell arteritis (GCA) can be difficult to diagnose as patients may present with vision loss in the absence of systemic symptoms, have other comorbidities that affect inflammatory blood markers, or have other ocular diagnoses. We present 3 cases illustrating this point including a patient with advanced glaucoma with worseni...
Article
Background: Diagnostic error is prevalent and costly, occurring in up to 15% of US medical encounters and affecting up to 5% of the US population. One-third of malpractice payments are related to diagnostic error. A complex and specialized diagnostic process makes neuro-ophthalmologic conditions particularly vulnerable to diagnostic error. Eviden...
Article
Background: Acute nonarteritic central retinal artery occlusion (CRAO) is an eye stroke with poor visual prognosis and no proven effective therapies. Given advances in acute stroke care, thrombolysis in CRAO merits critical re-examination. We review the evidence for intravenous (IV) and intra-arterial (IA) tissue plasminogen activator (tPA) in CRA...
Article
Very poor (hand motion or worse) visual acuity at presentation is highly unusual in non-arteritic anterior ischaemic optic neuropathy. We retrospectively reviewed the medical records of 151 consecutive non-arteritic anterior ischaemic optic neuropathy patients seen at our institution between July 2014 and April 2016 to evaluate the frequency and ch...
Article
Full-text available
Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a new retinal optical coherence tomography (OCT) finding. The Optic Disc Drusen Studies Consortium had made recommendations to distinguish PHOMS from true optic disc drusen (ODD) in 2018. While publications on PHOMS have increased since then, the accuracy of the definition of PHOM...
Article
Objective To compare the diagnostic performance of an artificial intelligence deep learning system with that of expert neuro‐ophthalmologists in classifying optic disc appearance. Methods The deep learning system was previously trained and validated on 14,341 ocular fundus photographs from 19 international centers. The performance of the system wa...
Article
Background The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. Objective To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. Methods Systematic collection of demographics, neuro-ophthalmic...
Article
Background: The tortuosity of the optic nerve can be quantified radiologically by measuring the angle of optic nerve deformation (the “optic nerve angle” [ONA]). In patients with idiopathic intracranial hypertension (IIH), lowering the intracranial pressure (ICP) to a normal range by lumbar puncture leads to straightening of the optic nerve and an...
Article
Objectives: To determine whether optic disc hemorrhages (ODH) and cotton wool spots (CWS) at presentation are associated with worse visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH). Methods: Retrospective institutional review of 100 eyes of 50 consecutive pediatric IIH patients (aged 16 years or less) who had...
Article
We report a case of ophthalmic artery occlusion (OAO) in a young patient with Covid-19 infection that was on therapeutic anticoagulation with apixaban for deep venous thrombosis (DVT). A 48-year-old man with obesity was hospitalized with a severe form of Covid-19 infection, complicated with acute respiratory failure, septic shock, dilated cardiomyo...
Article
Involvement of the pituitary gland by leukemic infiltration is exceedingly rare. Here, we describe a very late recurrence of B-cell acute lymphoblastic leukemia masquerading as a pituitary tumor and review the literature for previously reported cases. Our female patient presented 13 years after completion of therapy for B-ALL with headache, amenorr...
Article
Background: Nonophthalmologist physicians do not confidently perform direct ophthalmoscopy. The use of artificial intelligence to detect papilledema and other optic-disk abnormalities from fundus photographs has not been well studied. Methods: We trained, validated, and externally tested a deep-learning system to classify optic disks as being norm...
Article
Objective: To determine the feasibility of nonmydriatic fundus photography in the neurology outpatient setting and to record frequency of clinically relevant fundus findings. Methods: Over 5 weeks, fundus photographs were obtained using a nonmydriatic fundus camera in both eyes of adult patients attending our general neurology and headache clini...
Article
Background: Idiopathic intracranial hypertension (IIH) mostly affects overweight and obese women. Severe obesity is associated with poorer visual outcomes in IIH, and weight gain can precipitate IIH and increase the risk of recurrence. Conversely, weight loss can decrease intracranial pressure and is an effective IIH treatment. Therefore, accurate...
Article
Background: Neuro-ophthalmologists specialize in complex, urgent, vision- and life-threatening problems, diagnostic dilemmas, and management of complex work-ups. Access is currently limited by the relatively small number of neuro-ophthalmologists, and consequently, patients may be affected by incorrect or delayed diagnosis. The objective of this s...
Article
Purpose of review: To summarize recent developments in the classification, investigation and management of pediatric optic neuritis (PON). Recent findings: A recent surge in interest surrounding antibodies to myelin oligodendrocyte glycoprotein antibody (MOG-Ab) has instigated a paradigm shift in our assessment of children with PON. This serolog...
Chapter
The visual pathways traverse more than one-third of the brain, explaining why visual function is frequently affected by intracranial lesions and a wide range of neurological disorders. Visual disturbances such as monocular or binocular visual loss, visual hallucinations and diplopia may reveal a neurological disorder. Correct interpretation and eva...
Article
Translaminar pressure gradient abnormalities have been implicated in the pathogenesis and progression of glaucoma. A “reversed” translaminar pressure gradient may be related to papilloedema severity in idiopathic intracranial hypertension. Central corneal thickness is related to intraocular pressure measurements and, by extension, to translaminar p...
Article
Background: The association between cerebrospinal fluid (CSF) leaks at the skull base and raised intracranial pressure (ICP) has been reported since the 1960s. It has been suggested that spontaneous CSF leaks might represent a variant of idiopathic intracranial hypertension (IIH). We review the evidence regarding the association between spontaneou...
Chapter
Central retinal artery occlusion (CRAO) is the ocular equivalent of an acute cerebral ischemic event. It is most commonly caused by an embolus from the ipsilateral carotid artery, aortic arch or heart, leading to partial or complete occlusion of the central retinal artery. Patients with a CRAO need a thorough stroke evaluation to search for the cau...
Chapter
Non-arteritic anterior ischemic optic neuropathy (NAION) is the most common acute optic neuropathy in older individuals and usually occurs in patients with vascular risk factors and a “disc at risk” (i.e., a small optic nerve head and an absent or small physiologic cup). Potential areas for intervention in NAION include correction of underlying ris...
Article
Background: Acute visual loss is a common chief complaint in emergency department (ED) patients, but the scarcity of ophthalmologists in most EDs limits its evaluation. Introduction: Our objective was to evaluate whether nonmydriatic fundus photography (NMFP) in the ED helps triage patients with acute visual loss. Materials and Methods: We included...
Article
Importance Diagnostic errors can lead to the initial misdiagnosis of optic nerve sheath meningiomas (ONSM), which can lead to vision loss. Objective To identify factors contributing to the initial misdiagnosis of ONSM. Design, Setting, and Participants We retrospectively reviewed 35 of 39 patients with unilateral ONSM (89.7%) who were seen in the...
Article
Background/aims It remains unclear whether the presence of optic disc haemorrhages (ODH) or cotton wool spots (CWS) at presentation in patients with papilloedema from idiopathic intracranial hypertension (IIH) has prognostic value. The aim of this study was to determine if optic disc appearance at presentation predicts visual outcome in patients wi...
Article
Purpose of review: This review summarizes the recent literature on diagnostic error relevant to the practice of neuro-ophthalmology, and evaluates the potential for increased access to neuro-ophthalmology to decrease rates of diagnostic error, thereby reducing medical harm and spending on unnecessary tests and treatments. Recent findings: Despit...