Idiopathic Intracranial Hypertension Research Articles

Can optic disc vessel density help in cases of residual disc elevation after shunt surgery in cases of idiopathic intracranial hypertension?

To detect if we can use the reduction in the optic disc vessel density as an indicator to the reduction in intracranial tension in patients with residual optic disc elevation after shunt surgery as fundus examination in those cases is not conclusive. 21 patients with papilledema due to idiopathic intracranial hypertension underwent shunt surgery. Full neurological and ophthalmological assessments were done. The optic disc vessel density was measured before and 3 months after surgery. Patients were then divided according to the resolution of papilledema into 2 groups: 1) Residual disc elevation group. 2) Completely resolved disc edema group. CSF pressure was measured via lumber puncture preoperative for all patients and 3 months post-operative only for patients with residual disc edema. A comparison between both groups was done. There was a highly statistically significant difference between the two groups as regard the papilledema grade (the residual disc elevation group had a higher grade of papilledema) with P-value=0.000. As regard the difference in the preoperative optic disc vessel density between the two groups, there were statistically significant differences (optic disc vessel density was more in the residual disc elevation group). As regard the postoperative optic disc vessel density, there were non-significant differences between the two groups in whole image, inside disc and peripapillary vessel density (either in macro or microvasculature). The optic disc vessel density decreased with normal postoperative CSF opening pressure in cases with residual disc elevation postoperatively. Thus, in cases of residual optic disc swelling after shunt surgery, we can detect the reduction of intracranial pressure by the reduction in the optic disc vessel density which is a safe non-invasive technique. That may help in cases of residual disc elevation.

The additive value of complementing diagnostic idiopathic intracranial hypertension criteria by MRI – an external validation study

BackgroundRecently, diagnostic criteria including a standardized MRI criterion were presented to identify patients suffering from idiopathic intracranial hypertension (IIH) proposing that IIH might be defined by two out of three objective findings (papilledema, ≥ 25 cm cerebrospinal fluid opening pressure (CSF-OP) and ≥ 3/4 neuroimaging signs).MethodsTo provide independent external validation, we retrospectively applied the proposed diagnostic criteria to our cohort of patients with clinical suspicion of IIH from the Vienna IIH database. Neuroimaging was reevaluated for IIH signs according to standardized definitions by a blinded expert neuroradiologist. We determined isolated diagnostic accuracy of the neuroimaging criterion (≥ 3/4 signs) as well as overall accuracy of the new proposed criteria.ResultsWe included patients with IIH (n = 102) and patients without IIH (no-IIH, n = 23). Baseline characteristics were balanced between IIH and no-IIH groups, but papilledema and CSF-OP were significantly higher in IIH. For the presence of ≥ 3/4 MRI signs, sensitivity was 39.2% and specificity was 91.3% with positive predictive value (PPV) of 95.2% and negative predictive value (NPV) 25.3%. Reclassifying our cohort according to the 2/3 IIH definition correctly identified 100% of patients without IIH, with definite IIH and suggested to have IIH without papilledema by Friedman criteria, respectively.ConclusionThe standardized neuroimaging criteria are easily applicable in clinical routine and provide moderate sensitivity and excellent specificity to identify patients with IIH. Defining IIH by 2/3 criteria significantly simplifies diagnosis without compromising accuracy.

Cognıtıve functıons in idiopathic intracranial hypertensıon.

Cognitive problems in idiopathic intracranial hypertension (IIH) is generally overlooked in the presence of disabling headache and threat to visual function. The aim of this study was to search for cognitive deficits in patients with IIH using neuropsychologic tests in addition to P300 potential recordings to assess cognition related brain activity. Fifty IIH patients were examined using Montreal Cognitive Assessment Test, Stroop Test and Visual Aural Digit Span Test to measure different domains of cognition at the time of diagnosis. P300 potentials were recorded by using an oddball paradigm. Hospital Anxiety and Depression Scalewas used to determine anxiety and depression. Quality of life (QoL) was assessed by SF-36. The results were compared with fifty healthy controls with matching age, gender and body mass index. Neuropsychologic tests revealed wide cognitive impairment including attention, working memory, executive function, naming, language, delayed recall and orientation in IIH patients. In addition, quality of life was affected in the sub-parameters of general health perceptions, emotional role functioning, vitality, mental health and bodily pain. P300 potential latencies were long and the amplitudes were reduced indicating deficits in attention and working memory. Anxiety scores were high, and health-related QoL was low mainly involving vitality, emotional and mental health. Cognitive dysfunction was not correlated with the levels of anxiety and the correlation with headache severity was mild. A multidomain cognitive decline mainly involving attention and working memory was recorded in IIH patients. It was not correlated with anxiety and only a mild correlation with headache severity was present which may indicate a casual relationship between raised intracranial pressure and cognitive deficits. Screening is important as neuropsychological rehabilitation might be relevant in these patients.

Pseudotumor Cerebri Secondary to All-Trans Retinoic Acid/Arsenic Trioxide Therapy in Acute Promyelocytic Leukemia: A Case Report and Review of the Literature.

Pseudotumor Cerebri Secondary to All-Trans Retinoic Acid/Arsenic Trioxide Therapy in Acute Promyelocytic Leukemia: A Case Report and Review of the Literature.

Neurofilament light chain is elevated in patients with newly diagnosed idiopathic intracranial hypertension: A prospective study.

Idiopathic intracranial hypertension is a secondary headache disorder potentially causing visual loss. Neurofilament light chain is a candidate, prognostic biomarker, but further studies of neuronal biomarkers are needed. Our objective was to investigate neurofilament light chain in cerebrospinal fluid (cNfL) and plasma (pNfL), amyloid-beta 42 (Aβ-42), total-tau and phosphorylated-tau in cerebrospinal fluid in new-onset idiopathic intracranial hypertension. Prospective case-control study including new-onset idiopathic intracranial hypertension and age, sex and BMI matched controls. Biomarkers were compared between patients and controls and related to papilledema, visual fields and opening pressure. We included 37 patients and 35 controls. Patients had higher age-adjusted cNfL (1.4 vs. 0.6 pg/mL, p-adjusted < 0.001), pNfL (0.5 vs. 0.3 pg/mL, p-adjusted < 0.001) and total-tau/Aβ-42 (0.12 vs. 0.11, p-adjusted = 0.039). Significant, positive linear correlations were found between cNfL, pNfL, total-tau/Aβ-42 and opening pressure. Patients with severe papilledema had elevated cNfL compared to mild-moderate papilledema (median cNfL: 4.3 pg/mL (3.7) versus 1.0 pg/mL (1.4), p-adjusted = 0.009). cNFL was inversely associated with perimetric mean deviation (r = -0.47, p-adjusted < 0.001). cNfL, pNfL and total-tau/Aβ-42 were elevated in new-onset idiopathic intracranial hypertension. cNfL was associated with severity of papilledema and visual field defects at diagnosis. This indicates early axonal damage. Neurofilament light chain is a candidate biomarker for disease severity.

Vogt-Koyanagi-Harada Disease Presented with Bilateral Optic Disc Edema

Vogt-Koyanagi-Harada (VKH) disease is a multisystem inflammatory disease that occurs when body tissues including melanocytes are damaged by autoimmunity. The typical symptoms of VKH are bilateral multifocal uveitis and serous retinal detachment. Our patient was misdiagnosed as idiopathic intracranial hypertension because there were no other characteristic neurological symptoms other than bilateral optic disc edema. However, intracranial pressure was normal and pleocytosis was found, so VKH disease could be diagnosed. Bilateral optic disc edema may be a initial manifestation of VKH disease.

Clinico demographic profile and short-term outcome of Pediatric Idiopathic Intracranial Hypertension in a tertiary care hospital of Bangladesh

Background: Diagnosis of IIH (idiopathic intracranial hypertension) in children is challenging. The pathogenesis of IIH is still largely unknown. The aim of this study to evaluate the clinico-demographic profile and short-term outcome of IIH among Bangladeshi children. Methodology: This observational cohort study was done in pediatric Neurology Department, National Institute of Neurosciences and Hospital Bangladesh from January 2018 to July 2019. Total 19 cases were enrolled who were clinically suspected and/or had normal CSF with opening pressure (OP) above 250 mmH2O. They were further categorized as “definite IIH”, definite IIH without papilledema, and “probable IIH”. Clinical, radiological profile and short-term outcome were analyzed by SPSS version 22. Result: Among them most were probable IIH (74%). Episodic (57.9%), moderate, diffuse headache was the predominant symptom along with vomiting, double vision, unilateral VI nerve palsy and papilledema. Obesity was found in 21% cases. Mean CSF pressure was 21.84+7.62cm H2O and found high in definite IIH. All the cases were treated with acetazolamide and serial LP was done in 4(15.8%) cases. Definite IIH requires longer hospital stay (6.42 ± 2.87days). Recurrence was observed in 10.5% cases and visual impairment was found in 15.8% cases. Conclusion: This study found wide range of diversity between clinical feature and CSF opening pressure in Pediatric IIH and diagnostic confirmation was challenging. Initial treatment response was satisfactory with a low recurrence rate. J Bangladesh Coll Phys Surg 2024; 42: 161-168

A Rare Case Report of Benign Intracranial Hypertension Caused by Hypervitaminosis A

A Rare Case Report of Benign Intracranial Hypertension Caused by Hypervitaminosis A

A Case Report Detailing a Rare Presentation of Idiopathic Intracranial Hypertension With Atypical Symptoms

A Case Report Detailing a Rare Presentation of Idiopathic Intracranial Hypertension With Atypical Symptoms

Idiopathic intracranial hypertension associated with SARS-CoV-2 infection in an adult male patient: a case report and review of the literature

BackgroundHeadache is a frequent symptom in coronavirus disease 2019 (COVID-19) patients, and idiopathic intracranial hypertension (pseudotumor cerebri) has been reported among patients who underwent lumbar puncture for persistent headaches.Case presentationA 45-year-old black man presented with dyspnea, cough, fever and headache for 05 days followed by blurring of vision associated with worsening of the headache. Physical examination was significant for tachypnea and oxygen desaturation and there were no abnormal neurologic findings. He tested positive for SARS-CoV-2 with nasopharyngeal swab PCR. His CSF opening pressure appeared high with normal CSF analysis and brain magnetic resonance imaging (MRI) revealed prominent subarachnoid space around the optic nerves and bilateral papilledema. He had significant improvement with medical therapy alone.ConclusionIdiopathic intracranial hypertension (IIH) may occur in association with SARS-CoV-2 infection and should be considered when making a differential diagnosis for headache and blurring of vision. COVID-19 may play a role in the development of intracranial hypertension, even in the absence of known risk factors. Early diagnosis and treatment of IIH has paramount importance to prevent vision loss and other morbidities.

Novel Approach to Venous Stenting: Tigertriever Temporary Stenting for the Evaluation of Pulsatile Tinnitus

OBJECTIVE Pulsatile tinnitus can be significantly functionally debilitating, with venous sinus stenosis being a largely underrecognized etiology. Establishing a direct cause‐effect relationship can be particularly challenging in pulsatile tinnitus cases without idiopathic intracranial hypertension, and with multifocal, bilateral venous sinus stenoses. CASE PRESENTATION Herein, we present a patient with pulsatile tinnitus and bilateral transverse‐sigmoid sinus stenoses on diagnostic angiogram. Temporary stenting using a Tigertreiver was utilized to localize the symptomatic segment. Patient reported resolution of pulsatile tinnitus after deploying the temporary stentriever within the left sigmoid sinus. Subsequently, permanent stenting of the left sigmoid sinus was preformed resulting in complete resolution of symptoms. CONCLUSION We highlight the diagnostic challenges of patients presenting with pulsatile tinnitus and venous sinus stenoses, without idiopathic intracranial hypertension. While more experience is needed, temporary stenting may provide an improved method for assessing venous stenting efficacy in patients with pulsatile tinnitus.

Single cell transcriptomics of cerebrospinal fluid cells from patients with recent-onset narcolepsy

Narcolepsy is a rare cause of hypersomnolence and may be associated or not with cataplexy, i.e. sudden muscle weakness. These forms are designated narcolepsy-type 1 (NT1) and -type 2 (NT2), respectively. Notable characteristics of narcolepsy are that most patients carry the HLA-DQB1*06:02 allele and NT1-patients have strongly decreased levels of hypocretin-1 (synonym orexin-A) in the cerebrospinal fluid (CSF). The pathogenesis of narcolepsy is still not completely understood but the strong HLA-bias and increased frequencies of CD4+ T cells reactive to hypocretin in the peripheral blood suggest autoimmune processes in the hypothalamus. Here we analyzed the transcriptomes of CSF-cells from twelve NT1 and two NT2 patients by single cell RNAseq (scRNAseq). As controls, we used CSF cells from patients with multiple sclerosis, radiologically isolated syndrome, and idiopathic intracranial hypertension. From 27,255 CSF cells, we identified 20 clusters of different cell types and found significant differences in three CD4+ T cell and one monocyte clusters between narcolepsy and multiple sclerosis patients. Over 1000 genes were differentially regulated between patients with NT1 and other diseases. Surprisingly, the most strongly upregulated genes in narcolepsy patients as compared to controls were coding for the genome-encoded MTRNR2L12 and MTRNR2L8 peptides, which are homologous to the mitochondria-encoded HUMANIN peptide that is known playing a role in other neurological diseases including Alzheimer's disease.

Pediatric Idiopathic Intracranial Hypertension: Clinical Presentations, Risk Factors, and Prognostic Indicator

Pediatric Idiopathic Intracranial Hypertension: Clinical Presentations, Risk Factors, and Prognostic Indicator

1216 Resolution of Headaches Related to Idiopathic Intracranial Hypertension After Treatment with Lemborexant

1216 Resolution of Headaches Related to Idiopathic Intracranial Hypertension After Treatment with Lemborexant

Does a Healthy Weight Body Mass Index at Onset of Idiopathic Intracranial Hypertension Change the Outcomes? A United Kingdom Prospective Cohort Study

ABSTRACT Idiopathic intracranial hypertension (IIH) predominantly affects reproductive-aged females with obesity. However, the prevalence and impact of a healthy weight body mass index (BMI) at disease presentation is not known. This study aimed to evaluate the visual and headache outcomes stratified by the presenting BMI. This was a longitudinal prospective cohort study (IIH Life) based on a tertiary neuro-ophthalmology IIH service, University Hospitals Birmingham NHS Trust, United Kingdom, recruiting consecutive patients living with IIH between 2012 and 2021. Those with a presenting BMI were included. The outcome measures included visual outcomes of LogMAR visual acuity, Humphrey visual field perimetric mean deviation (PMD), optical coherence tomography (OCT) measurements, and headache outcomes of frequency, severity, and Headache Impact Test-6 score. Three hundred seventy-five people with IIH and a documented baseline BMI. About 3.7% of the entire cohort had a healthy weight BMI at IIH presentation and 15.5% BMI < 30 kg/m2. The baseline PMD was worse in patients without obesity; however, OCT papilloedema measures were similar. The presence of obesity was associated with a small but significant greater worsening in visual acuity but slower macular ganglion cell layer loss. There was no impact on PMD or papilloedema prognosis related to baseline obesity. The headache outcomes showed heterogeneity, with worse baseline headache frequency in patients with obesity. No BMI group was associated with worse headache outcomes. Patients with a healthy weight BMI or those without obesity at baseline make up a small proportion of IIH patients. BMI at presentation does not appear to influence long-term visual or headache outcomes.

Longitudinal optical coherence tomography indices in idiopathic intracranial hypertension

Idiopathic intracranial hypertension (IIH) may result in optic nerve fiber loss and even atrophy. The timing of the optical coherence tomography (OCT) indices reaching the lowest point (nadir) and the factors that predict the patient's anatomical outcome are not known. We aimed to determine the timing and the factors that affect nadir retinal nerve fiber layer (RNFL) thickness. The medical records of 99 IIH patients who were treated from December 2009 to January 2020 were retrospectively reviewed. The mean RNFL thickness at presentation was 263.5 ± 106.4 µm. The mean time to nadir was 7.9 ± 6.3 months. The average RNFL and ganglion cell complex (GCC) thickness at the nadir were 92.6 ± 14.5 µm (47% showed thinning) and 77.9 ± 27.8 µm (70% showed thinning), respectively. The Frisén disc edema stage and average RNFL thickness at baseline correlated with a longer time to nadir, (r = 0.28 P = 0.003 and r = 0.24, P = 0.012, respectively). The nadir average RNFL thickness and the nadir average GCC thickness (r = 0.32, P = 0.001, r = 0.29, P = 0.002, respectively) correlated with the baseline visual field mean deviation. The final anatomical outcome of IIH episodes in this study resulted in RNFL and GCC thinning. The time to RNFL nadir and its values correlated with IIH severity at presentation.

Idiopathic intracranial hypertension as the initial presenting manifestation of systemic lupus erythematosus in a child.

Idiopathic intracranial hypertension (IIH) is a diagnosis of exclusion characterized by features of raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. Commonly used other terms for this entity include benign intracranial hypertension (BIH) or pseudotumor cerebri. Few case reports of systemic lupus erythematosus (SLE) presenting as IIH are available in the literature. We report a 12-year-old girl presented with chronic holocranial headache and occasional episodes of projectile vomiting for the last 6 months and then developed blurring of vision for the last month. She fulfilled the criteria for IIH. Subsequent evaluation revealed a diagnosis of SLE. The occurrence of IIH in SLE is not coincidental and is reported in 1%-5.4% of patients with SLE. Though corticosteroids have not been widely used in IIH, underlying SLE warranted administering corticosteroids with subsequent complete resolution of IIH. Pediatricians, neurologists, intensivists, and ophthalmologists should consider SLE as a differential diagnosis in children presenting with IIH.

Facilitators and Barriers to Weight Loss Among Patients With Idiopathic Intracranial Hypertension.

Little is known about motivation for weight loss and barriers to weight loss among patients with idiopathic intracranial hypertension (IIH). Such information is crucial for developing tailored weight management recommendations and novel interventions. We administered a survey to patients with IIH presenting to neuro-ophthalmology clinics at The University of Michigan Kellogg Eye Center (Michigan, USA) and St. Thomas' Hospital (London, England). Participants rated importance and motivation to lose weight (1-10 scale; 10 = extremely important/motivated). Facilitators and barriers to weight loss were assessed using open-ended survey questions informed by motivational interviewing methodology. Open-ended responses were coded by 2 team members independently using a modified grounded theory approach. Demographic data were extracted from medical records. Descriptive statistics were used to analyze quantitative responses. Of the 221 (43 Michigan and 178 London) patients with IIH (Table 1), most were female (n = 40 [93.0%] Michigan and n = 167 [94.9%] London). The majority of patients in the United States were White (n = 35 [81.4%] Michigan), and the plurality were Black in the United Kingdom (n = 67 [37.6%] London]) with a mean (SD) BMI of 38.9 kg/m2 (10.6 kg/m2) Michigan and 37.5 kg/m2 (7.7 kg/m2) London. Participants' mean (SD) level of importance to lose weight was 8.5 (2.2) (8.1 [2.3] Michigan and 8.8 [2.1] London), but their mean (SD) level of motivation to lose weight was 7.2 (2.2) (6.8 [2.4] Michigan and 7.4 [2.1] London). Nine themes emerged from the 992 open-ended coded survey responses grouped into 3 actionable categories: self-efficacy, professional resources (weight loss tools, diet, physical activity level, mental health, and physical health), and external factors (physical/environmental conditions, social influences, and time constraints). Most responses (55.6%; n = 551) were about barriers to weight loss. Lack of self-efficacy was the most discussed single barrier (N = 126; 22.9% total, 28.9% Michigan, and 20.4% London) and facilitator (N = 77; 17.5% total, 15.9% Michigan, and 18.7% London) to weight loss. Other common barriers were related to physical activity level (N = 79; 14.3% total, 13.2% Michigan, and 14.8% London) and diet (N = 79; 14.3% total, 9.4% Michigan, and 16.3% London). Commonly reported facilitators included improvements in physical activity level (N = 73; 16.6% total, 18.5% Michigan, and 15.1% London) and dietary changes (N = 76; 17.2% total, 16.4% Michigan, and 17.9% London). Patients with IIH believe weight loss is important. Self-efficacy was the single most mentioned important patient-identified barrier or facilitator of weight loss, but professional resource needs and external factors vary widely at the individual level. These factors should be assessed to guide selection of weight loss interventions that are tailored to individual patients with IIH.

Apparent Diffusion Coefficient of the Optic Nerve Head in Idiopathic Intracranial Hypertension

ABSTRACT Idiopathic Intracranial Hypertension (IIH) is a condition in which patients have elevated intracranial pressure which does not have an apparent cause. To diagnose IIH, evaluation excluding other causes of elevated pressure must be performed. This typically includes magnetic resonance imaging (MRI) of the brain and venous sinuses. Despite there being known radiographic signs suggestive of IIH on MRI, there currently are no established correlations between radiographic findings and visual outcomes. Previous work revealed diffusion weighted imaging (DWI), a qualitative measurement on MRI, correlated with clinical findings (i.e presence and grade of papilloedema), but not visual outcomes. We hypothesized that the apparent diffusion coefficient (ADC), a quantitative value obtained during clinical MRI, may correlate with visual outcomes. We conducted a retrospective chart review to correlate findings on the ADC sequence on routine brain MRIs in patients with papilloedema with visual outcomes. In 49 patients with IIH, this study shows the ADC in the retrobulbar optic nerve to be 1,487 ± 559 × 10−6 mm2 /s, 15% lower than reported value of 1744 ± 413 in healthy controls. This suggests that there is true restricted diffusion in patients with IIH and papilloedema, as previously reported visually by MRI. However, there was no significant correlation with clinical outcomes of papilloedema grade, mean deviation on standard perimetry, and retinal nerve fibre layer (RNFL) on optical coherence tomography (OCT). We discuss reasons why the ADC measurement may be confounded by motion and partial volume and propose methods that may reduce these confounders for future studies.

Emergence of De Novo Conditions Following Remission of Cushing Syndrome: A Case Report and Scoping Review.

Onset and exacerbation of autoimmune, inflammatory or steroid-responsive conditions have been reported following the remission of Cushing syndrome, leading to challenges in distinguishing a new condition versus expected symptomatology following remission. We describe a case of a 42-year-old man presenting with new-onset sarcoidosis diagnosed 12 months following the surgical cure of Cushing syndrome and synthesise existing literature reporting on de novo conditions presenting after Cushing syndrome remission. A scoping review was conducted in Medline, Epub, Ovid and PubMed. Case reports and case series detailing adult patients presenting with new-onset conditions following Cushing syndrome remission were included. In total, 1641 articles were screened, 138 full-text studies were assessed for eligibility, and 43 studies were included, of which 84 cases (including our case) were identified. Most patients were female (85.7%), and the median reported age was 39.5 years old (IQR = 13). Thyroid diseases were the most commonly reported conditions (48.8%), followed by sarcoidosis (15.5%). Psoriasis, lymphocytic hypophysitis, idiopathic intracranial hypertension, multiple sclerosis, rheumatoid arthritis, lupus and seronegative arthritis were reported in more than one case. The median duration between Cushing remission and de novo condition diagnosis was 4.1 months (IQR = 3.75). Of those patients, 59.5% were receiving corticosteroid therapy at the time of onset. Our scoping review identified several cases of de novo conditions emerging following the remission of Cushing syndrome. They occurred mostly in women and within the year following remission. Clinicians should remain aware that new symptoms, particularly in the first year following the treatment of Cushing syndrome, may be manifestations of a wide range of conditions aside from adrenal insufficiency or glucocorticoid withdrawal syndrome.