Fulminant Idiopathic Intracranial Hypertension Research Articles

Very asymmetric disc edema in fulminant idiopathic intracranial hypertension: A rare and an unusual clinical finding

Fulminant idiopathic intracranial hypertension (IIH) can present as a rapid and severe vision-threatening condition, particularly in young females. We present a distinctive case of fulminant IIH characterized by asymmetrical vision loss with unilateral disc edema with loss of vision in the eye without disc edema. The patient exhibited an unusual serial sequence of radiological signs of raised intracranial pressure, emphasizing the need for emergent treatment. Resolution of the radiological signs was achieved following theco-peritoneal shunt placement. This case highlights the occurrence of fulminant IIH in unilateral disc edema and cause–effect relation between raised intracranial pressure and radiological signs and educative emphasis on the pathophysiology of the condition.

A surge in fulminant idiopathic intracranial hypertension cases within 1 year from the coronavirus disease 2019 pandemic lockdown: A case series

A surge in fulminant idiopathic intracranial hypertension cases within 1 year from the coronavirus disease 2019 pandemic lockdown: A case series

Clinical, radiological profile and outcomes of patients with idiopathic intracranial hypertension: a prospective study in the South Indian population

Objective: To describe the clinical, radiological characteristics and outcomes of patients with Idiopathic intracranial hypertension (IIH) at 6 months in a cohort of South Indian patients. Methods: We did a prospective study on patients presented to our institute with the symptomatology of IIH. A detailed history regarding headache, visual symptoms were noted. A complete neurological examination including examination of the fundus, CSF opening pressure, MRI brain, and MR cerebral venography were performed for all subjects. The patients were followed-up for 6 months to assess treatment response. Results: We evaluated 49 patients, the majority were females, and 38.7% had grade 1 obesity. Headache was the most common clinical presentation. The most common MRI abnormality was empty sella turcica, noted in 47%. There was a significant positive correlation between CSF opening pressure and headache severity (R2= 0.165, p = 0.018), as well as between CSF opening pressure and the grade of papilledema (R2=0.245, p=0.001). Of the cases, 95% responded to medical management, and three patients of fulminant IIH underwent lumbar-peritoneal shunting. At 6 months, 60% had persistent headaches. Only 8.7% had persistent visual symptoms, and only one had optic atrophy. There was no significant correlation between the follow-up grade of papilledema and the patient’s BMI. No significant correlation was found between the amount of weight lost and the improvement in the Numerical Rating Scale (NRS) score at 6 months. Conclusion: Headache in IIH is multifactorial and a significant cause of disability. The overall prognosis for vision is good. IIH should be suspected in any obese woman with headaches since it is one of the preventable causes of blindness.

A rare case of fulminant idiopathic intracranial hypertension

Background: Idiopathic intracranial hypertension (IIH) is characterised by raised intracranial pressure and normal cerebrospinal fluid composition with no detectable intracranial aetiology. Fulminant IIH is rare and is defined by the acute onset of symptoms and signs (less than 4 weeks between onset of symptoms and severe vision loss) with progressive vision loss over days.
 Case presentation: A 22-year-old woman with body mass index of 27.6 was diagnosed with fulminant IIH. Her presenting vision was hand movement and it progressed rapidly to no light perception by day 4. She was managed with oral acetazolamide and intermittent lumbar punctures. Nevertheless, she did not regain her vision.
 Conclusion: Fulminant IIH is a vision-threatening condition that must be recognised and treated early to prevent irreversible vision loss.

Coronavirus Disease 2019-Related Fulminant Idiopathic Intracranial Hypertension

AbstractIdiopathic intracranial hypertension or pseudotumor cerebri syndrome (PTCS) is defined as an increase in intracranial pressure (ICP) without an identifiable and/or structural cause or abnormal cerebrospinal fluid content. The most common symptoms of PTCS include headache, vomiting, pulsatile tinnitus, blurred vision, and diplopia. In 2 to 3% of PTCS patients, severe and rapidly progressive vision loss may develop within 1 month since symptoms begin; this clinical condition is usually defined as fulminant idiopathic intracranial hypertension (FIIH). This study presented a patient admitted to the hospital with headache and blurred vision, who also had high severe acute respiratory syndrome coronavirus 2 infections total antibody level and was treated with a lumboperitoneal shunt due to medical treatment-resistant increased ICP. We also reviewed similar cases previously reported in the literature. To the best of our knowledge, no children affected with coronavirus 2019 related to FIIH and requiring surgical treatment have been yet reported in the literature.

Fulminant Idiopathic Intracranial Hypertension Refractory to Treatment Associated to SARS-CoV-2: A Case Report (P14-4.004)

<h3>Objective:</h3> NA <h3>Background:</h3> Idiopathic Intracranial Hypertension (IIH) is characterized by increased intracranial pressure, which most frequently occurs in childbearing-age obese women. Scarce reports of COVID-19 infection associated IIH exist and have only described this in women and children. “Fulminant IIH” is defined as intracranial hypertension without secondary cause, with severe vision loss within 4 weeks of onset, and progressive vision loss over days. A 36-year-old man with obesity came to the Ophthalmology clinic for evaluation of vision loss and headache, the patient tested positive for SARS-CoV-2 one month before this. He was identified to have bilateral grade V papilledema, confirmed with optical coherence tomography (OCT), and admitted to Neurology for IIH management. The patient was started intravenous therapy with high-dose steroids and acetazolamide without relief of headache or vision improvement (20/100). Lumbar puncture was performed, revealing an opening pressure of ≥55 cm H20. The MRI only showed findings of increased intracranial hypertension, and the MRV ruled out thrombosis. The lack of improvement in his headaches and vision loss ultimately required ventriculoperitoneal shunt placement. The patient had immediate subjective visual improvement after this, with unchanged visual acuity. On follow-up 4 weeks later, the patient continued to have headaches and the opening pressure was 30 cm H20. Lumboperitoneal shunt was placed. Both optic nerves became completely atrophic. Despite finally reaching an opening pressure of 15 cm H20, headaches did not resolve, and the patient currently only perceives light. <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> This case represents, to our best knowledge, the first description of fulminant IIH associated with SARS-CoV-2. The previous reports described mild to moderate cases in women and children with response to treatment. This occurred to an adult male who despite receiving promptly treatment, had a poor response. Further studies are needed to determine if atypical clinical phenotypes are associated with refractory forms. <b>Disclosure:</b> Dr. Murguia Fuentes has nothing to disclose. Mr. Murguia Fuentes has nothing to disclose. Dr. Adamu has nothing to disclose. Dr. Carvajal- Gonzalez has nothing to disclose. Dr. Arvas has nothing to disclose.

Interpretation of telemetric intracranial pressure recordings in people with idiopathic intracranial hypertension after shunt implantation.

The M.scio telesensor (Aesculap-Miethke, Germany) is a device integrated within a ventriculoperitoneal (VP) shunt for non-invasive measurement of the intracranial pressure (ICP). The purpose of this study was to analyze the telemetric recordings with the M.scio system in shunted patients with idiopathic intracranial hypertension (IIH), in order to determine reference values and assist the interpretation of telemetric data. This was a cohort study of consecutive patients with fulminant IIH who underwent primary VP shunt insertion between July 2019 and June 2022. The first telemetric measurements after surgery in the sitting and supine positions were analyzed. Telemetric ICP values, wave morphology, and pulse amplitude were determined for functioning and malfunctioning shunts. Fifty-seven out of 64 patients had available telemetric recordings. The mean ICP was - 3.8mmHg (standard deviation (SD) = 5.9) in the sitting and 16.4mmHg (SD = 6.3) in the supine position. The ICP curve demonstrated pulsatility in 49 (86%) patients. A pulsatile curve with mean ICP in the above ranges indicated a functioning shunt, whereas the lack of pulsatility was challenging to interpret. There was a significant positive correlation between ICP versus amplitude, ICP versus body mass index (BMI), and amplitude versus BMI. This clinical study defined ICP values and curves in IIH patients with a shunt. The results will assist the interpretation of telemetric ICP recordings in clinical decision making. More research is required to model longitudinal recordings and explore the link between telemetric measurements with clinical outcomes.

Fulminant Idiopathic Intracranial Hypertension with Atypical Presentation.

Fulminant Idiopathic Intracranial Hypertension with Atypical Presentation.

Developing a "Fast-Track" Strategy for Interventional Management of Patients With Idiopathic Intracranial Hypertension.

Idiopathic intracranial hypertension (IIH) has an increasing incidence worldwide over the past decade, with a high economic burden on patients and society. Up to 10% of patients with IIH have progressive visual decline requiring an invasive intervention (including cerebrospinal fluid shunting, cerebral dural sinus stenting, or optic nerve sheath fenestration [ONSF]). IIH patients with visual decline usually undergo evaluation and initial management through the emergency department (ED) and commonly have a long hospital stay due to the lack of a dedicated methodology for evaluation and management, particularly in patients who present with visual loss (i.e., fulminant IIH). An innovative practice approach is needed to improve the means of multidisciplinary communication in care and evaluation of IIH patients. This paper aims to discuss the need for the development and implementation of a multidisciplinary "fast-track" strategy for the evaluation and management of patients with fulminant IIH or those with a suboptimal response to maximum tolerated medical treatment at risk for visual loss. We suggest that such a program could reduce hospital stay and ED visits and therefore reduce healthcare costs and improve patient outcomes by accelerating the management process.

Clinical Manifestations, Neuroimaging Findings, and Treatment of Idiopathic Intracranial Hypertension in a Nepalese Tertiary Centre

Introduction: Idiopathic Intracranial Hypertension is characterized by headache, visual impairment, papilledema, and increased cerebrospinal fluid opening pressure. We aim to evaluate clinical manifestations, neuroimaging findings, and treatment of Idiopathic Intracranial Hypertension in a Nepalese tertiary center. Materials and Methods: We retrospectively included patients with the diagnosis of Idiopathic Intracranial Hypertension who were admitted to the Neurology department of Tribhuvan University Teaching Hospital from 2019 June to 2021 May and presented to the Neuro-ophthalmology outpatient clinic of the hospital for follow-up. Results: Out of 16 Idiopathic Intracranial Hypertension patients, 12(75%) patients had either headache or ocular pain. Reduced visual acuity and progressive visual loss were found in 44% of patients. Normal CSF opening pressure was found in 19%, 44% had CSF opening pressure at a range of 20-30 cm H20 and &gt; 30 cm H20 in 37.5%. 15 Idiopathic Intracranial Hypertension patients (93.7%) were subtyped as Typical and 1 patient was diagnosed as Fulminant Idiopathic Intracranial Hypertension. Acetazolamide was used for treatment in 15 patients and 1 patient needed ventriculoperitoneal shunting. 56% Seven patients (44%) had some abnormal findings: partial empty sella (44%), and tortuous optic nerve (31%), flattened posterior eyeball (31%), and hypoplastic transverse sinus (19%). Conclusions: Idiopathic Intracranial Hypertension is an uncommon diagnosis but should be suspected in patients with chronic headaches with visual impairment. In low-resource settings, proper history along with neurological and ophthalmological examinations can even detect the early features and timely referral can save the vision and disability of Idiopathic Intracranial Hypertension patients.

Fulminant idiopathic intracranial hypertension and reversible splenial lesion syndrome (RESLES) : A case report

Reversible splenial lesion syndrome (RESLES) is a rare clinico–radiological entity characterized by transient lesions involving the splenium of the corpus callosum (SCC). It has been described in a broad spectrum of conditions. We report the case of a RESLES in a young woman ultimately diagnosed with fulminant idiopathic intracranial hypertension (IIH). To the best of our knowledge, this is the first such association reported.

Efficacy of dural venous sinus stenting in treating idiopathic intracranial hypertension with acute vision loss.

Dural venous sinus stenting (VSS) is an effective, durable treatment for patients with idiopathic intracranial hypertension (IIH) due to underlying venous sinus stenosis. However, the use of venous sinus stenting to treat IIH with acute vision loss has rarely been described. A retrospective chart analysis identified patients who received VSS for fulminant IIH, defined as acute (< 8 weeks) visual field loss to within the central 5° and/or a decrease in visual acuity to less than or equal to 20/50 in either eye in the presence of papilledema. Ten patients were identified with average patient age of 31.0 years, and all except one were female. Mean body mass index was 41.2 kg/m2. All patients presented with vision loss and some with headache and tinnitus. The average trans-stenotic gradient pre-stenting was 28.7 mmHg (range 9-62 mmHg). All patients had diminished or resolved venous gradients immediately following the procedure. At mean follow-up of 60.5 weeks, 100% had improvements in papilledema, 80.0% had subjective vision improvement, 55.6% had headache improvement and 87.5% had tinnitus improvement. 90.0% had stable or improved visual acuity in both eyes with a mean post-stenting Snellen acuity of 20/30 and an average gain of 3 lines Snellen acuity post-stenting (95% confidence intervals 0.1185-0.4286, p = 0.0018). Two patients (20.0%) required further surgical treatment (cerebrospinal shunting and/or stenting) after their first stenting procedure. This series suggests that VSS is feasible in patients presenting with IIH and acute vision loss with a fairly low complication rate and satisfactory clinical outcomes.

Superior Ophthalmic Vein Flow Patterns as a Marker of Venous Sinus Stenosis and Hypertension in Idiopathic Intracranial Hypertension: A Case of Emergent Transverse Sinus Stenting as Treatment of Fulminant Idiopathic Intracranial Hypertension

Fulminant idiopathic intracranial hypertension (IIH) can cause rapid vision loss. Transverse sinus stenosis is a finding commonly associated with IIH, and transverse sinus stenting has been used to rapidly reduce intracranial pressure and improve visual symptoms. Our objective was to describe a case of immediate alteration in reversed superior ophthalmic vein (SOV) flow in a fulminant IIH patient who underwent venous sinus stenting. All charts, imaging, and notes spanning from the initial presentation to the post-intervention follow-up were reviewed and summarized for inclusion. A 24-year-old woman presented with several weeks of severe headaches and progressive vision loss. She was found to have severe papilledema and the opening pressure on lumbar puncture (LP) was 70 mm Hg. Computed tomography (CT) and magnetic resonance imaging demonstrated findings consistent with elevated intracranial pressure, and CT venography revealed stenosis of the right transverse sinus. She underwent an uncomplicated diagnostic cerebral angiogram, right venous sinus manometry, and right transverse to sigmoid sinus stenting procedure. Prior to deployment of the stent, a trans-stenotic pressure gradient of 12 mm Hg was observed within the right transverse-sigmoid junction, and flow through the bilateral SOVs was retrograde. Following stent placement, the pressure gradient normalized, and SOV flow was bidirectional. She subjectively reported improved vision, and there was improving papilledema. Repeat LP yielded an opening pressure of 21.6 mm Hg. This case demonstrates reversed SOV flow should be considered an indicator of severe venous sinus stenosis, and restoration to normal or near normal state following stenting for IIH is likely indicative of procedural success.

Complete bilateral ophthalmoplegia in malignant intracranial hypertension in a child.

To describe a case of fulminant idiopathic intracranial hypertension (IIH) in a child with "malignant" presentation. A 16-year-old, previously healthy, girl presented with bilateral visual loss and bilateral global limitation of eye movements in the absence of headache. Extensive laboratory evaluation for infectious, inflammatory, autoimmune, and neoplastic conditions was negative. Magnetic resonance imaging (MRI) of the brain and lumbar puncture findings were consistent with a diagnosis of IIH. Extraocular motility improved in the next few days as well as optic disc edema but visual acuity remained poor. The authors believe that the acute, severe, and fulminant ("malignant") presentation with markedly elevated intracranial pressure may produce the unique presentation of severe vision loss and bilateral complete ophthalmoplegia. Interestingly, there was no headache. To our knowledge this is the first such case to be reported in the English language ophthalmic literature.

Demographics, Clinical Characteristics, and Management of Idiopathic Intracranial Hypertension in Kuwait: A Single-Center Experience.

Background: Idiopathic intracranial hypertension (IIH) affects predominantly obese females during their reproductive age period. The demographics of this condition has not been studied in Kuwait before.Objectives: To determine the demographics, clinical features, risk factors, and treatment modalities of IIH in the main neurology tertiary referral hospital in Kuwait and to compare our data with literature.Methods: A retrospective study was conducted to identify cases of IIH seen between January 1, 2018, and December 31, 2018. Patients were diagnosed in and referred from the neurology and neuro-ophthalmology clinics.Results: Our cohort consisted of 139 patients. We estimated a crude annual incidence rate of IIH of 3.28 per 100,000 population. Female-to-male ratio was 9.6:1. Mean age was 32.1 ± 10.8 years. Mean age of males was 31.46 ± 12.63 and that of females was 32.11 ± 10.67. The median of the duration from the first symptoms till diagnosis was 6 weeks (2–10 weeks). Headache was the most common symptom in 134 (96.4%) patients, followed by visual disturbances in 85 (61.2%) patients, transient visual obscurations (TVOs) in 84 (60.4%) patients, pulsatile tinnitus in 72 (51.8%) patients, diplopia in 22 (15.8%) patients, other symptoms (e.g., nausea, vomiting, radicular neck, and back pain) in 19 (13.7%) patients, and 1 (0.7%) patient had facial weakness. High body mass index (BMI) was seen in 89.9% of patients, either overweight or obese, and it was the most common risk factors in both males (46.2%) and females (61.1%). Anemia was found in 38.1%; 21.6% of patients used OCPs and 7.9% used vitamin A. Bilateral transverse sinus stenosis (BTSS) was detected in 47 (33.8%) patients. Only 2 (1.4%) patients developed “fulminant IIH” characterized by rapidly progressive disease. All the patients received medical treatment and only 12 (8.6%) needed surgical management.Conclusion: Incidence of IIH in Kuwait is similar to other regional studies but higher than Western studies. Demographics and clinical features of IIH in our study are comparable to international and regional figures. Most of our patients had a benign course. IIH is more prevalent in females and strongly associated with obesity.

Fulminant Idiopathic Intracranial Hypertension.

The goal of this review is to describe the presenting features of fulminant idiopathic intracranial hypertension (IIH) and outline the multimodal approach to its treatment. Venous sinus stenting may be an appropriate alternative to optic nerve sheath fenestration or cerebrospinal fluid shunting in select patients with fulminant IIH. Prompt surgical intervention maximizes the chance of visual recovery in patients with fulminant IIH. "Fulminant IIH" is defined as intracranial hypertension with no secondary cause, severe vision loss within 4weeks of symptom onset, and progressive vision loss over days. Rapid recognition of the fulminant phenotype of IIH by emergency department physicians, neurologists, and ophthalmologists is critical. Without appropriate triage and rapid medical and surgical intervention, patients with fulminant IIH are at high risk for profound, permanent vision loss. Prompt surgical intervention with optic nerve sheath fenestration, cerebrospinal fluid shunting, or venous sinus stenting minimizes the chance of poor visual outcome. If a delay is anticipated, serial lumbar punctures or temporary cerebrospinal fluid drainage and medical therapy may forestall irreversible vision loss.

Rapid visual loss associated with fulminant idiopathic intracranial hypertension.

Rapid visual loss associated with fulminant idiopathic intracranial hypertension.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Comment.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Comment.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Response.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Response.

Forgetting the fundoscope – A case of fulminant idiopathic intracranial hypertension causing rapid visual loss

Idiopathic intracranial hypertension (IIH) is a syndrome of elevated intracranial pressure without evidence of intracranial mass lesion or venous thrombosis, and bland cerebrospinal fluid examination. It mostly affects overweight women of childbearing age and if left untreated, can lead to permanent visual loss. Visual decline in this condition is generally slow, over months to years. However, a small proportion of patients develop rapidly progressive visual loss within days or weeks of onset of headaches. We describe a 29 year-old patient with fulminant IIH in whom inadequate fundoscopy and assessment of vision contributed to a delay in diagnosis and poor outcome. Unfortunately, competence with the bedside examination technique of fundoscopy is declining. We emphasise the importance of thorough assessment of vision and fundi in patients presenting with new-onset headache.