Pseudo-Foster Kennedy syndrome is a rare condition characterized by unilateral papilledema with contralateral optic atrophy in the absence of an intracranial mass causing direct compression of the optic nerve. We here report the case of a 75-year-old overweight female patient (BMI 30 kg/m[2]), with poorly controlled hypertension, who presented with decreased visual acuity. The patient reported headaches evolving over 2 months. Ophthalmologic examination showed papilledema in the right eye and optic nerve atrophy in the left eye. Physical examination revealed poorly controlled arterial hypertension with a peak blood pressure of 200/110 mmHg. The patient underwent neurological examination and brain imaging, both of which revealed no abnormalities. A lumbar puncture was performed and revealed increased resistance to CSF flow (34 cmH2O). Other tests were normal. The diagnosis of pseudo-Foster Kennedy syndrome secondary to idiopathic intracranial hypertension (IIH) was established. The patient was treated with oral acetazolamide. The evolution was marked by complete resolution of papilledema and headaches, with gradual recovery of visual acuity in the right eye. Idiopathic intracranial hypertension is a rare cause of pseudo-Foster Kennedy syndrome. Only a few cases have been reported in the literature. It should be considered when this syndrome is present in order to preserve both visual and vital prognosis.

Optical coherence tomography (OCT) and OCT-angiography (OCTA) have emerged as useful tools for noninvasive imaging in the neurologic field. This chapter elucidates their utility in detecting biomarkers for the diagnosis and monitoring of progression and response to treatment in different prevalent neurologic conditions. The retina, an extension of the central nervous system, presents a unique opportunity for monitoring brain pathology due to its accessibility. Through highly resolved scans of the retina and optic nerve, OCT and OCTA facilitate the identification of subtle clinical changes occurring during neuroinflammatory, neurodegenerative, and ischemic processes in disorders like multiple sclerosis, Alzheimer and Parkinson disease, anterior ischemic optic neuropathies, and papilledema. Peripapillary nerve fiber layer and macular internal retinal layer thickness on OCT, and vessel density in the superficial retina on OCTA seem to be the most sensitive parameters in detecting axonal injury and neurodegeneration. These metrics hold promise as surrogate markers for cerebral alterations. While OCT and OCTA show considerable potential, continued research is necessary to validate their reliability and clinical significance, considering potential confounding factors such as concurrent ophthalmic pathologies. Nonetheless, these advancements represent significant progress toward enhancing the diagnosis, management, and prediction of outcomes in various neuro-ophthalmic disorders.

Purpose: To report a case of POEMS syndrome presenting with unilateral optic disc edema and unexpectedly low systemic vascular endothelial growth factor (VEGF) levels in the setting of contralateral intravitreal (IVT) anti-VEGF therapy. Methods: A single case was reviewed. Results: A 65-year-old man with branch retinal vein occlusion in the right eye receiving IVT anti-VEGF injections presented with optic disc edema in the left eye. Systemic symptoms included skin hyperpigmentation; shortness of breath; and peripheral neuropathy characterized by numbness, tingling, swelling, and weakness of his extremities. Imaging demonstrated pachymeningeal enhancement, mixed sclerotic and lytic bone lesions, lymphadenopathy, and splenomegaly. Laboratory evaluation workup revealed a monoclonal gammopathy, and lymph node biopsy findings were consistent with the plasma cell variant of Castleman disease, supporting a diagnosis of POEMS syndrome. Notably, systemic VEGF levels were unexpectedly low (8.7 pg/mL, normal <96.2 pg/mL), likely secondary to the IVT anti-VEGF injection. Conclusions: This case highlights an atypical presentation of POEMS syndrome with unilateral disc edema and suppressed systemic VEGF levels, potentially confounded by contralateral IVT anti-VEGF treatment. Awareness of this interaction is important for accurate interpretation of VEGF levels and timely diagnosis.

Spaceflight associated neuro-ocular syndrome (SANS) is a significant ophthalmic complication observed in astronauts during and after long-duration missions, characterized by optic disc edema, globe flattening, choroidal folds, and hyperopic shifts. Unlike papilledema in terrestrial idiopathic intracranial hypertension, optic disc edema in SANS is often asymmetric. The mechanisms underlying this asymmetry remain poorly understood. In this narrative review, we synthesize and critically interpret existing clinical observations, anatomical studies, neuroimaging findings, and experimental evidence, and propose that uneven ocular venous congestion, arising from microgravity-induced cephalad fluid shifts, pre-existing transverse sinus asymmetry, and orbital venous overload, leads to asymmetric optic disc edema by differentially disrupting anterograde ocular glymphatic transport between the eyes. This mechanistic framework highlights the interplay between venous hemodynamics and ocular glymphatic flow as a key factor in SANS pathophysiology. Targeted in-flight monitoring and ground-based analog studies will be essential to rigorously test this hypothesis. To this end, we outline a feasible experimental approach that prospectively integrates preflight cerebral magnetic resonance venography, providing data on transverse sinus dominance, with serial in-flight ophthalmic imaging on the International Space Station. This combined strategy could directly determine whether dural venous sinus anatomy predisposes to uneven ocular venous congestion and asymmetric optic disc edema in microgravity. Insights gained from this work may guide the development of effective countermeasures against SANS and broaden our understanding of ocular fluid dynamics under conditions of altered venous physiology on Earth.

Papilledema due to elevated intracranial pressure may indicate life-threatening conditions such as intracranial mass lesions or cerebral sino-venous thrombosis. This bicentric retrospective cohort study evaluated neuroimaging outcomes and clinical predictors in adults presenting with suspected papilledema to emergency departments between 2009 and 2022. Diagnostic outcomes were categorized as confirmed papilledema (due to idiopathic intracranial hypertension, space-occupying lesions, hydrocephalus or secondary intracranial causes) or papilledema ruled out. Clinical features, visual acuity, and frequency of focal neurological deficits were compared between groups. Diagnostic yield and number needed to scan (NNS) for CT and MRI were calculated, and univariable logistic regression was performed to identify predictors of secondary intracranial causes. Among 225 patients (mean age 43.0 ± 16.8 years; 57% female), papilledema was confirmed in 124 (55%), including 44 (35.5%) with secondary intracranial causes. Papilledema was excluded in 73 patients (32.4%). Primary CT and MRI identified secondary pathology in 13.3% (NNS 7.5) and 16.7% (NNS 6) of patients, respectively. Diplopia, nausea/vomiting, and focal neurological deficits were associated with secondary causes but were not consistently present. Secondary intracranial pathology is common in suspected papilledema. Given the limited discriminatory value of clinical features, timely neuroimaging should be performed in the diagnostic evaluation.

Chiari malformation type I is a structural anomaly of the posterior fossa characterized by cerebellar tonsillar herniation through the foramen magnum, often associated with disrupted cerebrospinal fluid dynamics and elevated intracranial pressure. Papilledema is a rare ophthalmic manifestation in Chiari malformation type I, typically linked to increased intracranial pressure. Isolated visual symptoms without signs of intracranial pressure in pediatric Chiari malformation type I are particularly rare and poorly understood. We report a rare pediatric case of Chiari malformation type I in a 9-year-old girl presenting solely with bilateral papilledema and visual impairment, without typical symptoms of raised intracranial pressure such as headache. Magnetic resonance imaging revealed cerebellar tonsillar descent and cervical syringomyelia. Due to progressive visual decline and the absence of alternative diagnoses, the patient underwent foramen magnum decompression with C1 laminectomy. Postoperatively, visual acuity significantly improved, although papilledema persisted at 9 months. This case challenges the conventional view that papilledema in Chiari malformation type I is solely caused by elevated intracranial pressure. Alternative mechanisms, such as localized venous congestion, mechanical stress on the optic nerve, or regional cerebrospinal fluid flow disturbances, may contribute to papilledema. Persistent papilledema despite clinical improvement supports the hypothesis of localized rather than global intracranial pressure elevation. Pediatric presentations may differ from adult cases in symptomatology and pathophysiology, requiring careful diagnostic and therapeutic considerations. Surgical decompression may lead to functional visual recovery even if anatomical signs such as papilledema persist. This case underscores the need for further research into atypical presentations and underlying mechanisms of Chiari malformation type I, especially in pediatric populations.

Increased intracranial pressure (ICP) in the pediatric population represents a critical and potentially life-threatening condition that may lead to severe neurological sequelae, including brain herniation, cerebral ischemia, and irreversible neurological impairment, such as visual loss. The diagnosis of elevated ICP in children is particularly complex, as clinical manifestations are frequently subtle and non-specific, especially in infants and young children. Symptoms such as irritability, poor feeding, lethargy, and failure to thrive often overlap with a wide range of common pediatric disorders, thereby contributing to delayed diagnosis and an increased risk of adverse neurological outcomes. Within this context, ophthalmologic evaluation constitutes a key non-invasive tool in the early detection of increased ICP. Fundoscopic examination provides critical diagnostic information, with optic disc abnormalities among the most sensitive markers of elevated ICP. However, these findings may be less pronounced or exhibit distinct characteristics in children compared to adults, and their interpretation is further complicated by age-related variability in ocular anatomy and intracranial pressure norms. This review provides an integrated synthesis of current diagnostic strategies for assessing elevated ICP in children, with a particular focus on ophthalmologic assessment. It examines the association between specific ocular findings and ICP, with the aim of improving early recognition, guiding clinical management, and enhancing patient outcomes. Furthermore, it addresses the inherent challenges of pediatric ICP diagnosis and highlights key gaps in the literature that warrant further investigation.