Severe papilloedema with vision loss secondary to Guillain-Barré syndrome.

We discuss a patient who presented with bilateral VI and VII cranial nerve palsies, symmetric upper and lower limb weakness and areflexia, 2 weeks following an flu-like illness. At presentation, there was no papilloedema, and her visual function was normal. Cerebrospinal fluid analysis and electrophysiology supported the diagnosis of Guillain-Barré Syndrome (GBS). She received intravenous immunoglobulins. She subsequently developed headaches and vision loss. Funduscopy demonstrated severe papilloedema with visual acuity of 6/18 right eye, 6/12 left eye with bitemporal visual field depression. Lumbar puncture revealed elevated opening pressure with high protein and normal cell count. She received acetazolamide. There was resolution of papilloedema and normal visual function at 3 months. Of note, the patient's body mass index was 17 kg/m2Our case highlights the rare occurrence of papilloedema in GBS, reiterating the importance of performing funduscopy on patients with any neurological diagnosis. Early detection and prompt management of papilloedema can prevent permanent vision loss.

A case report: co-occurrence of probable Vogt-Koyanagi-Harada disease and diabetic retinopathy.

BACKGROUND: Bilateral retinal detachment and choroidal detachment in a patient are rare occurrences. The presence of bilateral diabetic retinopathy (DR) in such a case is even rarer and complicates the condition. CASE PRESENTATION: In this study, we document a case of unconventional VKH. Manifestations in this patient included intense peripheral retinal detachment and choroidal detachment, along with vitreous opacities akin to cotton wool spots, concurrent with DR. The diagnosis was considered as probable VKH with DR. Treatment according to VKH protocols, including high-dose corticosteroids, yielded positive results. CONCLUSIONS: VKH can co-occurrence with DR. VKH manifestations vary, and early, aggressive, and long-term treatment is essential. The complexity of treatment increases with concurrent DR, necessitating the use of immunosuppressants.

Navigating Uncharted Waters: Comparative Analysis of Clinical Progression and Outcomes in Vestibular Schwannoma Patients with Papilledema and without Hydrocephalus, Versus Those without Papilledema and Hydrocephalus: A Comprehensive Institutional Insight.

BACKGROUND: Papilledema's association with hydrocephalus (HCP)-linked larger vestibular schwannoma (VS) is established but cases lacking concurrent HCP require further investigation. METHODS: This retrospective comparative observational study, conducted from July 2018 to July 2023, examined 120 VS patients undergoing surgery. Patients were categorized into Group 1 (papilledema without HCP) and Group 2 (no papilledema or HCP), with comprehensive data analyzed. RESULTS: In this study, Group 1 (14 patients with papilledema) and Group 2 (106 patients without papilledema or HCP) were compared. Group 1 was younger (mean age 27.21 ± 11.73 years) than Group 2 (mean age 54.66 ± 11.44 years). Both groups had similar symptom durations and tumor detection times. Group 1 had increased vascularity (P = 0.001), elevated cisterna magna protein levels (P = 0.001), and a higher incidence of neurofibromatosis 2 (P = 0.003). They also experienced longer surgeries (P = 0.001) and more blood loss (P = 0.001), leading to extended postoperative complications. Group 2 showed improved postsurgery visual outcomes (P = 0.001), better Glasgow Outcome Scores (P = 0.001), enhanced facial nerve preservation (P = 0.002), and improved hearing on follow-up (P = 0.003). Logistic regression analysis highlighted prolonged surgery duration (P = 0.057) and papilledema (P = 0.0001) as significant factors influencing visual improvement. CONCLUSIONS: Patients with VS require preoperative fundoscopy evaluation due to potential visual loss and papilledema, even without HCP. Early treatment initiation enhances visual and hearing outcomes. Meticulous surgery is vital given the lesion's hypervascular nature and adherence to surrounding structures. Preoperative embolization may aid in preserving neurovascular structures. In developing countries with higher blindness rates, judicious noncontrast computed tomography brain evaluation is crucial for timely detection and treatment initiation of lesions like VS.

Idiopathic intracranial hypertension in two twin sisters.

Idiopathic intracranial hypertension (IIH) is a condition of unknown aetiology characterised by an increase in the intracranial pressure. Familial cases of IIH are rare and not well-understood. We present two monozygotic twins who developed IIH two years apart. The case involves two monozygotic female twins developing IIH in their 50s. They presented with a history of blurry vision and headaches. The diagnosis included the neurological, radiological and ophthalmological examination, excluding other causes. Both patients received treatment with acetazolamide, successfully resolving the papilloedema and restoring a normal visual field. This case highlights the occurrence of IIH among twins presenting at similar periods, emphasising the potential genetic influence. Clinicians should alert and educate the family regarding the risk factors and potential symptoms of this condition in the unlikely occurrence that other family members are affected.

Concomitant optic disk drusen and papilledema due to idiopathic intracranial hypertension in a pediatric cohort.

BACKGROUND: Optic disk drusen (ODD) in pediatric patients typically presents with pseudopapilledema. Diagnosing concomitant papilledema due to idiopathic intracranial hypertension (IIH) in these patients can be challenging. The purpose of this study was to evaluate the incidence and clinical features of papilledema due to IIH among pediatric patients with a new diagnosis of ODD and to discuss the clinical and paraclinical findings that helped diagnose this group. METHODS: The medical records of children <15 years of age with ODD confirmed by B-scan ultrasound at their first visit over a 4-year period (2019-2022) were reviewed retrospectively. Patients with concurrent IIH were identified, and the demographic and clinical characteristics were reviewed. RESULTS: A total of 83 children with confirmed ODD at the initial presentation were included, of whom 4 (4.8%) were diagnosed with concomitant IIH. Patients ranged in age from 7 to 15 years; 3 of the 4 were female, and 3 had IIH-related symptoms at presentation (1 was asymptomatic). None of the 4 patients had papilledema greater than Frisen grade 2. CONCLUSIONS: We recommend that clinicians review pertinent IIH symptoms and risk factors in children with ODD and follow the standard workup for IIH in suspicious cases. In asymptomatic patients with a new diagnosis of ODD, we recommend obtaining a follow-up optic nerve evaluation and optical coherence tomography scan to detect any significant interval change that might serve as a possible indicator of concomitant papilledema.

[An analysis of the etiology composition and clinical characteristics of bilateral optic disc swelling].

Objective: To investigate the etiology composition and clinical characteristics of bilateral optic disc swelling(ODS). Methods: The medical records of all newly diagnosed bilateral ODS patients admitted to the neurology ward of Beijing Tongren Hospital from January 2017 to June 2021 were retrospectively searched to classify the etiology, obtain demographic and clinical information, and compare the differences in clinical characteristics. Results: A total of 131 patients with bilateral ODS were included, including 56 males and 75 females, aged 15-73 (39±14) years. The most common cause of the bilateral ODS was increased intracranial pressure (ICP)(56/131, 42.7%), followed by optic neuritis (ON)(40/131, 30.5%). Other causes included vascular optic neuropathy (13/131, 9.9%), pseudopilledema (9/131, 6.9%); uveitis (6/131, 4.6%), toxic optic neuropathy (3/131, 2.3%) and unknown causes (4/131, 3.1%). Idiopathic intracranial hypertension (IIH) (43/56, 76.8%) was the most common etiology for papilledema. In the comparison of the two main causes of intracranial hypertension and the clinical characteristics of ON in ODS, there was no statistically significant difference in the incidence of age, gender, complaints of ocular pain or headache, and hemorrhage of optic disc(P>0.05). Visual acuity abnormalities and low vision were more common in ON group than the increased ICP group[36/40(90%) vs 33/56(58.9%), P=0.001; 35/80(43.8%) vs 22/112(19.6%), P<0.001], while severe papilledema was more common in increased ICP group[38/112(33.9%)vs 9/80(11.3%), P<0.001]. Conclusions: The most common cause for bilateral ODS is increased ICP, but it can also be triggered by a variety of other causes. Optic neuritis(ON) is the most important differentiating disease in the study of Chinese patients.

Papilloedema: a highly specific predictor of raised intracranial pressure in a complex neurosurgical paediatric cohort.

PURPOSE: Papilloedema is recognised as an indicator of raised intracranial pressure, although there is a paucity of literature describing the utility of fundoscopy in screening for raised ICP in children with craniofacial synostosis, particularly young children. We sought to investigate the association of optic disc morphology with ICP in children, and to define the sensitivity and specificity of papilloedema as a clinical indicator of raised ICP and determine if age, or underlying conditions impact the findings. METHOD: Retrospective analysis of all patients undergoing ICP monitoring at a designated paediatric neurosurgical and craniofacial unit in the United Kingdom between October 2009 and October 2018. The fundoscopy findings and ICP monitoring data were analysed for 31 children with craniosynostosis and 29 children without craniosynostosis. RESULTS: All children who had papilloedema had raised ICP confirmed with monitoring. Across the 60-patient cohort, confirmed papilloedema on fundoscopy had Positive Predictive Value (PPV) of 1.00, Negative Predictive Value (NPV) of 0.64 with sensitivity 48% and specificity 100% for the presence of raised ICP (p = < 0.0001). In the craniosynostosis group, PPV was 1.00, NPV was 0.39, sensitivity 48% and specificity 100% (p = < 0.03). There is no correlation between severity of optic disc swelling using Frisen grading and elevation of ICP. Age did not affect the presence of papilloedema in those with raised ICP. CONCLUSION: The presence of papilloedema is a strong indicator of raised ICP in a child, regardless of underlying aetiology. Detailed fundoscopy can prevent the need for further investigations including imaging-related radiation and invasive CSF pressure monitoring.

A multi-centre case series of patients with coexistent intracranial hypertension and malignant arterial hypertension.

OBJECTIVE: To describe the clinical characteristics, outcomes, and management of a large cohort of patients with concomitant malignant arterial hypertension and intracranial hypertension. METHODS: Design: Retrospective case series. SUBJECTS: Patients aged ≥ 18 years with bilateral optic disc oedema (ODE), malignant arterial hypertension and intracranial hypertension at five academic institutions. Patient demographics, clinical characteristics, diagnostic studies, and management were collected. RESULTS: Nineteen patients (58% female, 63% Black) were included. Median age was 35 years; body mass index (BMI) was 30 kg/m2. Fourteen (74%) patients had pre-existing hypertension. The most common presenting symptom was blurred vision (89%). Median blood pressure (BP) was 220 mmHg systolic (IQR 199-231.5 mmHg) and 130 mmHg diastolic (IQR 116-136 mmHg) mmHg), and median lumbar puncture opening pressure was 36.5 cmH2O. All patients received treatment for arterial hypertension. Seventeen (89%) patients received medical treatment for raised intracranial pressure, while six (30%) patients underwent a surgical intervention. There was significant improvement in ODE, peripapillary retinal nerve fibre layer thickness, and visual field in the worst eye (p < 0.05). Considering the worst eye, 9 (47%) presented with acuity ≥ 20/25, while 5 (26%) presented with ≤ 20/200. Overall, 7 patients maintained ≥ 20/25 acuity or better, 6 demonstrated improvement, and 5 demonstrated worsening. CONCLUSIONS: Papilloedema and malignant arterial hypertension can occur simultaneously with potentially greater risk for severe visual loss. Clinicians should consider a workup for papilloedema among patients with significantly elevated blood pressure and bilateral optic disc oedema.

Analysis of intracranial pressure waveform using a non-invasive method in individuals with craniosynostosis.

PURPOSE: Craniosynostosis can lead to symptoms resulting from cranial compliance (CC) changes and intracranial hypertension (ICH), which may cause cognitive and visual impairment. Non-invasive methods have emerged, including a new device that captures and processes the intracranial pressure waveform (ICPw) by the skull's oscillation. The present study evaluates ICPw obtained non-invasively (NIICPw) in patients with craniosynostosis. METHODS: This prospective, cross-sectional, and descriptive study was conducted at a single center. Patients diagnosed with craniosynostosis and who provided informed consent were included. A US Food and Drug Administration-approved mechanical extensometer device (Brain4Care Corp.) was used to obtain a NIICPw. An ophthalmologist did a point-of-care retinography to check the optic nerve papilla. The P2/P1 ratio and the morphology of the NIICPw were analyzed, as well as the retinography. RESULTS: Thirty-five patients were evaluated, and 42 registers were obtained because seven were assessed before and after the surgery. The two patients who presented papilledema had low CC (NIICPw shape Class 3 or 4). There was a significant association between NIICPw and papilledema. CONCLUSION: The ratio P2/P1 and the NIICPw morphology provided by a non-invasive monitor are related to CC changes before papilledema occurs. This is especially useful in patients with craniosynostosis because invasive ICP monitoring is not always feasible. Further studies are warranted to establish the clinical utility of NIICPw in patients with craniosynostosis.

Technical considerations and long-term results of endovascular venous stenting to control venous hypertension from meningiomas invading intracranial venous sinuses.

OBJECTIVE: Meningiomas invading the intracranial venous sinuses may cause intracranial venous hypertension, papilledema, and visual compromise. Sinus resection and graft reconstructions, however, add significant complexity to tumor surgery, with the potential for increased morbidity. In this study, the authors explored whether venous sinus stenting might provide an alternative means of controlling venous hypertension that would be sustainable over the long term. METHODS: The authors performed a retrospective review of all 16 patients with intracranial meningiomas who underwent stenting at their institution for venous sinus compromise. At presentation, all had headache and 9 had papilledema. Thirteen patients had 1 meningioma and 3 had 2 or more. Three patients had had previous tumor resection and radiotherapy. One patient had been treated with a lumboperitoneal shunt and radiotherapy. The median length of clinical follow-up was 8 years (range 4 months-18 years). RESULTS: Venous sinus narrowing was often not confined to the site of meningioma, and bilateral transverse sinus narrowing, reminiscent of that seen in idiopathic intracranial hypertension, was present in 7 patients with sagittal sinus meningiomas. Eleven patients had stents placed solely across sinus narrowing caused by meningioma. Five patients had additional stents placed at other sites of venous narrowing at the same time: in one of these patients, a stent was placed across a defect in the sagittal sinus caused by previous surgery, and in the 4 other patients, stents were placed across nontumor narrowings of the transverse sinuses. In 1 patient, the jugular vein was also stented. Nine patients developed symptomatic in-stent restenosis at the meningioma site. Eight had further stenting procedures with variable success in restoring the in-stent lumen. The remaining patient, with a late partial relapse, is being reinvestigated. Papilledema resolved in all patients after stenting. Six patients experienced prolonged and very substantial relief of all symptoms. Five patients had persistent headache despite restoration of the sinus lumen. Five had persistent symptoms associated with resistant in-stent stenosis. There were no significant complications from any of the diagnostic or therapeutic procedures. CONCLUSIONS: In patients who are symptomatic with meningiomas obstructing the venous sinuses, successful stenting of the affected segment can give a good outcome, especially in terms of relieving papilledema. However, further procedures are often necessary to maintain stent patency, other areas of venous compromise frequently coexist, and some patients remain symptomatic despite apparently successful treatment of the index lesion. Long-term surveillance is a requirement.

An optical coherence study on optic disc parameters and peripapillary retinal nerve fiber layer thickness in patients with optic disc edema.

PURPOSE: This study aimed at evaluation of patients diagnosed with optic disc edema (ODE) in terms of demographics, etiology, clinical presentation, and optical coherence tomography parameters. METHODS: An observational cross-sectional study was conducted from August 2021 to July 2022. Patients diagnosed with ODE underwent comprehensive ophthalmic evaluation. Peripapillary retinal nerve fiber layer (RNFL) thickness and optic nerve head parameters were assessed using optical coherence tomography. Radiologic investigations were done as deemed necessary. RESULTS: One hundred and forty-eight eyes with ODE (134 eyes from 67 patients with bilateral ODE and 14 eyes from 14 patients with unilateral ODE) and 148 eyes of age- and gender-matched control group were included in the study. The mean age of the study population was 31.51 ± 13.33 years, with 44 (54.3%) males and 37 (45.6%) females. Bilateral ODE was seen in 82.7% of patients. Idiopathic intracranial hypertension was the most common cause of bilateral ODE, and nonarteritic anterior ischemic optic neuropathy was the most common cause of unilateral presentation. Decreased vision and headache were the main symptoms reported by a majority of patients. Peripapillary RNFL thickness was significantly increased in the ODE group compared to controls. CONCLUSION: The current study provides the clinical profile and optic disc and RNFL measurements of ODE patients in an Eastern Indian population. Detailed history and careful evaluation are necessary, as the treatment strategy is highly dependent on the underlying etiologies.

Incidental multiple myeloma in a patient with neuroretinitis: a case report.

BACKGROUND: Neuroretinitis is classically defined as a clinical triad of unilateral, painless vision loss, accompanied by optic disc edema and characteristic macular star formation. The causes of neuroretinitis can be categorized as infectious, non-infectious, and idiopathic, therefore differential diagnosis and careful evaluation are required, owing to the various etiologies and masqueraders. CASE PRESENTATION: A 54-year-old woman presented to the clinic with blurred vision in both eyes. A complete ophthalmic examination revealed optic disc edema with blurred margins and macular exudates, intraretinal edema in the temporal peripapillary area, and subretinal fluid with neurosensory retinal detachment in the macular area. Systemic laboratory investigations showed no signs of infection or inflammation. However, bone marrow suppression was suspected based on the results of the complete blood count test, and the patient was diagnosed with multiple myeloma. CONCLUSION: Although neuroretinitis is rarely accompanied by hematological malignancy, it is important to be mindful of the latter because ophthalmic manifestations are a common feature of hematological malignancies and lesions occur in nearly every ocular structure.

Severe bilateral papilledema after sigmoid sinus constriction surgery: a case report.

BACKGROUND: Papilledema is a common sign of various diseases in the eye. It could result from any conditions of increased intracranial pressure (ICP). Underlying the etiology of papilledema and appropriate treatment in time is essential. CASE REPORT: We present a case of severe bilateral papilledema after sigmoid sinus constriction surgery. A 25-year-old female presented with a 1-month history of bilateral blurred vision, headache, and vomiting. The patient had a history of right-side sigmoid sinus constriction surgery for pulsatile tinnitus (PT) one month before in another hospital. Fundus examination showed severe bilateral papilledema. Lumbar puncture showed an elevated cerebrospinal fluid (CSF) opening pressure of 29 cm H2O. Neuroimaging examination demonstrated the right sigmoid sinus filling defect as changes after surgery. We referred the patient to the initial surgeon, who repaired the sigmoid sinus on the right side by removing the implanted gelatin sponge, as diuretic treatment could not be effective. Intracranial hypertension symptoms and signs improved soon after eliminating sigmoid sinus stenosis. Neuroimaging showed resolved right sigmoid sinus stenosis after the second surgery. CSF opening pressure was 14.5 cm H2O at the 1-month follow-up. Fundus examination showed entirely resolved papilledema. Three years of follow-up showed no recurrence. CONCLUSIONS: This is the first clinical report of intracranial hypertension associated with sigmoid sinus constriction surgery. Although rare, rapid detection and adequate etiology management could lead to a good prognosis. It highlights the need for ophthalmologists to be aware of the diagnostic approach to papilledema and enhance cooperation with multidisciplinary departments. The most likely cause of the intracranial hypertension was dominant sinus surgical constriction by mechanical external compression, as confirmed by the complete clinical remission following the second operation to remove the implanted gelatin sponge. Thus, this case also highlights the importance of selecting the appropriate therapeutic option for PT. Surgical sinus constriction should no longer be considered a viable option for PT treatment.

SARS-CoV-2 Parainfectious Optic Neuropathy: 3 Case Reports and a Review of the Literature.

BACKGROUND: Parainfectious optic neuritis is an inflammatory reaction that occurs shortly after an infection without direct invasion by a pathogen. The clinical profile depends on the infectious organism. Cases of SARS-CoV-2 parainfectious optic neuritis have been reported in the literature, but there are no reviews that have applied strict inclusion criteria to more definitively establish the clinical profile associated with SARS-CoV-2. METHODS: We present 3 new cases of SARS-CoV-2 parainfectious optic neuritis. We also review the literature for definite cases by selecting only those with unambiguous clinical features and MRI findings of optic neuritis, positive SARS-CoV-2 polymerase chain reaction or serology, and the absence of myelin oligodendrocyte-glycoprotein or aquaporin-4 antibodies or other diseases associated with optic neuritis. RESULTS: We report 2 cases of monophasic, unilateral SARS-CoV-2 parainfectious optic neuritis with optic disc edema and nadir visual acuities of finger counting. We report 1 case of mild SARS-CoV-2 parainfectious optic neuritis that featured cotton wool spots, peripapillary wrinkles and hemorrhages, and recurrence after an initial steroid taper. We identified 6 cases of unambiguous SARS-CoV-2 parainfectious optic neuritis from the literature. Combining our case series with the case reports in the literature, the average age was 42.8 years, 3/9 had bilateral disease, 6/8 had optic disc edema, 8/9 had nadir visual acuity of finger counting or worse, and all recovered visual acuity to 20/40 or better after therapy with steroids. CONCLUSIONS: SARS-CoV-2 parainfectious optic neuritis has a clinical profile that is atypical for idiopathic optic neuritis but fairly typical of parainfectious forms of optic neuritis with a severely reduced nadir visual acuity, high likelihood of bilaterality, high incidence of optic disc edema, and prompt and significant response to corticosteroids. Further study with long-term follow-up and epidemiologic investigation will be needed to further characterize this clinical entity.

Experimental drugs for the treatment of idiopathic intracranial hypertension (IIH): shedding light on phase I and II trials.

INTRODUCTION: Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema that causes debilitating headaches. While the extent of the pathophysiology is being discovered, the condition is emerging as a systemic metabolic disease distinct to people living with obesity alone. Idiopathic intracranial hypertension is becoming more common and therefore establishing licensed therapeutics is a key priority. AREA COVERED: The translation of preclinical work in idiopathic intracranial hypertension is evident by the two early phase trials evaluating 11-ß-hydroxysteroid dehydrogenase inhibitor, AZD4017, and a glucagon like peptide-1 receptor agonist, Exenatide. This review summarizes these two early phase trials evaluating targeted medicines for the treatment of intracranial pressure. The modulation of these two distinct mechanisms have potential for therapeutic intervention in people living with idiopathic intracranial hypertension. EXPERT OPINION: The clinical trial landscape in idiopathic intracranial hypertension is a challenge due to the rarity of the disease and the lack of agreed meaningful trial outcomes. Further preclinical work to fully understand the pathogenesis is required to enable personalized targeted drug treatment.

Diagnosis and management of Neuro-Behçet disease with isolated intracranial hypertension: a case report and literature review.

BACKGROUND: Neuro-Behçet's disease (NBD), characterized by isolated intracranial hypertension, is a rarely encountered condition, especially in children. In this study, we describe the treatment of a pediatric patient with NBD, who exhibited isolated intracranial pressure elevation as indicated by the initial manifestation of diplopia and physical examination finding of papilledema. CASE PRESENTATION: An 8-year-old boy was admitted to the hospital with a confirmed diagnosis of Behçet's disease (BD) over eight months. The patient also presented with the symptom of diplopia for three days. The evaluation of the patient's nervous system did not reveal any apparent abnormalities. The measurement of cerebrospinal fluid pressure yielded a reading of 470 mm H2O. The examination of the fundus indicated papilledema, and imaging scans revealed evidence of focal demyelination. The symptoms of the child showed alleviation after the administration of mannitol, methylprednisolone, and azathioprine. Furthermore, this study involved a comprehensive analysis of 18 cases of NBD with isolated intracranial hypertension, comprising one case reported herein and 17 cases from the literature review. Three cases were children, and an equal distribution of males and females 9:9 was noted. The average age at the onset of symptoms was 24.7 years (8-38 years). Headache (90%) was the most commonly reported clinical manifestation, followed by blurred vision or diplopia (80%). The ocular manifestations included papilledema (100%), abducent nerve paralysis (20%) and local eye hemorrhages in the retina (30%). Notably, 88.9% of these ocular manifestations were relieved or cured after treatment. CONCLUSION: This study presents the first reported case of NBD with isolated intracranial hypertension in the pediatric population of China. In a child with Bechet's disease presenting with features of raised intracranial pressure, it is important to be aware of neuro Bechet's presenting with intracranial hypertension without other neurological abnormalities. This will help make early diagnosis, institute treatment and prevent sequelae resulting from untreated raised intracranial pressure.