Predictors of visual outcome in Idiopathic Intracranial Hypertension: observations from a retrospective cohort in Germany

doi:10.21203/rs.3.rs-4875880/v1

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Research Article

Predictors of visual outcome in Idiopathic Intracranial Hypertension: observations from a retrospective cohort in Germany

https://doi.org/10.21203/rs.3.rs-4875880/v1

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Background

Idiopathic intracranial hypertension (IIH) is a rare secondary headache disorder. It is characterized by increased intracranial pressure without an identifiable cause and can potentially cause visual impairment due to damage to the optic nerve. This study aimed to evaluate clinical characteristics and to identify risk factors of permanent and progressive deterioration of visual function in a German IIH cohort.

Methods

This is a retrospective observational study of IIH patients who were diagnosed between 2004 and 2020 at our tertiary care neurological department. Ophthalmologic findings, clinical characteristics and treatment strategies were recorded. Visual outcomes were assessed at a minimum follow-up of 6 months after IIH diagnosis. Regression analysis was utilized to evaluate potential risk-factors of poor visual outcome, defined as 1) worsening of ophthalmologic findings and/or 2) persistent visual impairment.

Results

The patient cohort comprised 191 consecutive IIH patients. Cohort characteristics and clinical findings were consistent with prior studies. Ophthalmologic follow-up was available in 90 patients. Here, poor visual outcome occurred in 36% of patients. In regression models, male sex was independently associated with a greater risk for poor visual outcome (OR: 7.2, p = 0.028). Patients with moderate papilledema at baseline had a lower likelihood for poor visual outcome than patients with lower degree papilledema (OR: 0.2, p = 0.016). Surgical intervention (primarily by ventriculo-peritoneal shunt) was associated with a lower risk of poor visual outcome (OR: 0.21, p = 0.09), mediating the relation between papilledema and visual outcome. Neither age, BMI nor cerebrospinal fluid opening pressure were associated with the visual prognosis.

Conclusions

One third of patients in the follow-up cohort experienced worsening of ophthalmologic findings or visual impairment. Male sex may predispose IIH patients to visual impairment. Moderate papilledema at baseline reduced the risk of poor visual outcome, partially due to surgical therapy during the follow-up period. This study highlights the importance of timely intervention in patients with moderate papilledema and the need for reliable outcome predictors in IIH.

Trial registrations: Trial registration is not applicable: This observational study reports retrospective documentations of clinical routine data. It does not classify as a clinical trial.

idiopathic intracranial hypertension

outcome

prognosis

visual outcome

papilledema

Idiopathic intracranial hypertension (IIH) is characterized by visual disturbance and burdening headaches due to increased intracranial pressure (ICP). Its etiology is unknown. IIH primarily affects young overweight and obese women, with the dynamic of weight gain being a relevant risk factor for the disease [1]. The therapeutic strategy primarily aims to prevent persistent visual impairment next to achieving freedom of symptoms. However, predicting the visual prognosis is challenging due to the rarity and heterogenic course of the disease between individuals. While some patients achieve remission under dietary measures and ICP-lowering medication alone, more severely affected patients may receive neurosurgical or endovascular procedures. Considering the risk of permanent visual impairment, identifying prognostic factors at the time of diagnosis is vital for an adapted treatment strategy. Poor visual prognosis has been associated with male sex, longer disease duration, with severe papilledema and decreased visual acuity at baseline [2–4]. Some studies found cerebrospinal fluid opening pressure (CSF) to be predictive of visual outcome[5], whereas more recent studies could not validate this [2, 6]. Magnetic resonance imaging (MRI) signs do not seem to be predictive of headache or visual outcomes [6]. Neurofilament light chain is a promising CSF-biomarker for axonal damage and neurodegeneration, that in IIH is associated with the severity of papilledema and visual field defects [7, 8]. The literature describing the prospective longitudinal course of IIH is still restricted to few large cohorts, mainly focusing on specific interventions in and with older studies applying heterogenous diagnostic criteria. Friedman et al. proposed novel diagnostic criteria for IIH, which have been accepted in current guidelines [9, 10]. To the best of our knowledge, baseline characteristics, visual outcomes, and risk factors of visual loss have not yet been evaluated in a German IIH cohort.

This study aimed to evaluate the clinical characteristics and visual outcome in our retrospectively collected cohort of IIH patients in Germany and sought to identify possible predictors of permanent or progressive visual loss.

This is a single-center cohort study evaluating factors that predict poor visual outcome in a retrospectively collected cohort of IIH patients. All patients were treated at the neurological department of Charité – Universitätsmedizin Berlin, which is a tertiary care university hospital. Ethical approval was obtained. This study complies with the reporting guidelines within the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) Statement.

Patient Selection

A medical database inquiry identified all consecutive patients treated under the definite or suspected diagnosis of IIH (ICD-10 code: G93.2) at our tertiary care center between January 2004 and October 2020.

Individual patient charts were reviewed to determine whether the diagnosis of IIH could be verified based on the revised Friedman criteria for IIH [10]. For the present study, we included all patients with definite IIH and IIH-WOP (IIH without papilledema) according to Friedman criteria. Patients were excluded if data to determine the diagnosis of IIH was insufficient (i.e., lack of fundoscopy or lack of MRI for exclusion of secondary causes or measurement of CSF opening pressure (CSF-OP)) or if the IIH diagnosis was not consistent with Friedman criteria. Patients with probable IIH or suggested IIH-WOP according to Friedman criteria were excluded as well as any patients with secondary causes of intracranial hypertension. We excluded pediatric IIH patients, defined as all patients diagnosed < 14 years of age, as pediatric IIH is considered a distinct entity of the disease [11]. The patient selection process is shown in Fig. 1.

Figure 1. Flow chart of the inclusion/exclusion process.

Data collection

Data were retrospectively extracted from electronically archived reports. We recorded the dates of clinical presentations, the date of the first diagnostic lumbar puncture (as a surrogate marker of the disease duration, defined as the time from the first diagnostic lumbar puncture to the date of the last visit). Baseline parameters were age, sex, weight, height, BMI and CSF-OP, as measured during lumbar puncture. All patients had CSF analysis, magnetic resonance imaging (MRI) of the brain and ophthalmological as well as neurological examination to exclude alternative causes of visual loss or intracranial hypertension. All patients were treated according to best practice including recommendation of weight loss for overweight or obese patients. We recorded the treatment strategy during the follow-up period, defining two groups of patients: 1) those who were treated pharmacologically (who received acetazolamide, topiramate and/or furosemide) and 2) those who were surgically managed by implantation of a ventriculoperitoneal (VP) shunt or by bariatric surgery. Symptoms and signs included the presence of 1) headache, 2) visual disturbance, 3) vertigo, 4) diplopia, 5) tinnitus, and 6) abducens palsy. Headache severity at baseline was recorded using the numeric rating scale (NRS, ranging from 0 to 10, where 0 is no pain and 10 refers to the most severe pain). Symptoms and signs, BMI, ophthalmologic findings and CSF-OP were recorded for follow-up visits.

Ophthalmologic Findings

Best corrected visual acuity (BCVA) of the most severely affected eye was transformed to logMAR (logarithm of the minimum angle of resolution) for statistical analysis (logMAR = − log (decimal visual acuity)). Visual field perimetric mean deviation (MD) in decibel [dB] was recorded on Humphrey visual field analysis using the 30 − 2° tendency-oriented perimetry. Visual field MD of > 6.0dB in at least one eye was considered abnormal, analogous to the IIH treatment trial [12]. Funduscopic papilledema grading of the most severely affected eye (i.e. the higher value) was extracted from ophthalmologic reports. Papilledema was graded according to the modified Frisén Scale from grade 0 (normal optic disc), 1 (minimal degree of edema), 2 (low degree of edema), 3 (moderate edema), 4 (marked edema) to grade 5 (severe papilledema)[13, 14]. The presence of optic nerve atrophy was noted.

Long-term visual follow-up was recorded, if an ophthalmologic dataset including BCVA and the grade of papilledema, of at least 6 months after initial diagnosis was available. If more than one ophthalmologic dataset was available, the most recent dataset was included. Analogous to our prior research [7], Frisén grades at follow-up were transformed into the following three categories for the regression analysis:

no papilledema & mild papilledema (Frisén grades 0 and 1)
moderate papilledema (Frisén grades 2 and 3)
severe papilledema (Frisén grades 4 and 5)

Visual outcomes were assessed applying the following definitions:

persistent visual impairment: visual acuity ≥ 0.2 logMAR and/or perimetric MD of > 6.0 dB in at least one eye
ophthalmologic worsening: decline of visual acuity by ≥ 0.2 logMAR and/or increase of papilledema by one category (as defined above) and/or occurrence of optic disc atrophy

As the presence of optic disc atrophy defined a poor visual outcome, patients with optic disc atrophy at baseline were excluded from the regression analysis.

Statistical analysis

Statistical analyses were performed using IBM SPSS Statistics software (IBM SPSS Statistics, Version 29.0. Armonk, NY: IBM Corp.). Categorical variables were expressed in frequencies and percentages, continuous normally distributed variables as mean ± standard deviation and non-parametric variables as median with interquartile range (IQR), as appropriate. Missing data was handled by listwise deletion and reporting of valid percentages.

Q-Q plots and histograms were used to check for normal distribution of continuous variables. Univariate group comparisons were computed using Fisher’s exact test or two-sided t-test. Univariable correlation was analysed by Pearson test. Linked group comparisons of continuous variables were conducted with paired samples t-test or Wilcoxon-test, if not normally distributed. Univariable regression was applied to explore the association between baseline parameters and visual outcome. Backward stepwise binary logistic regression (likelihood-ratio) was conducted to identify possible predictors of a poor visual outcome (as defined above). The following candidate variables were included into the saturated backward regression model: baseline BMI, age at diagnosis, sex, disease duration, baseline CSF-OP, baseline severity of papilledema). At each step, variables that had the lowest contribution were removed from the model, i.e. with an elimination criterion set at a probability ≥ 0.1. Results of the regression analysis are presented as odds-ratio (OR) and 95% confidence intervals (95%CI). Goodness-of-fit was assessed using the Hosmer-Lemeshow-test. The level of significance was set at a two-sided p-value < 0.05.

Cohort characteristics

The IIH study cohort comprised 191 patients (Fig. 1). Seven patients (4%, n = 7/191) were diagnosed with IIH-WOP and 184 with IIH (96%, n = 184/191). Cohort characteristics at baseline are presented in Table 1. Patients were predominantly female (86.4%, n = 165/191, males: 13.6%, n = 26/191). Mean age at diagnosis was 35.5 ± 12.5 years (range: 14–75). Men were significantly older than women at the time of diagnosis (p = 0.001).

Baseline data on weight and height was available in 99% (n = 189/191) of patients. The mean BMI at baseline was 34.4 ± 8.4 kg/m² (range: 20–80). Nineteen patients (10%) had a normal BMI (i.e. <25kg/m²) at baseline. Forty patients were overweight (21%, n = 40/189, BMI: 25–30 kg/m²). Out of 69% (130/189) obese patients (BMI > 30 kg/m²), 42 patients had a BMI > 40 kg/m². Mean CSF-OP at diagnosis was 36.2 ± 8.1 cmCSF. BMI and CSF-OP did not significantly differ between women and men. Baseline BMI and CSF-OP showed a moderate correlation (Pearson correlation, r = 0.325, p = 0.002). Visual disturbance (89%) and headache (84%) were the most common symptoms at baseline. Headache was more frequently reported by women (p = 0.007). Median baseline headache intensity on the NRS was 6 (IQR: 0–8). CSF-OP did not differ between patients with or without headaches (p = 0.347).

Diplopia was reported by 39%, vertigo by 45% of patients and 9% had abducent nerve palsy, each symptom or sign similarly frequent between women and men (Table 1). Headache and visual disturbance were less frequent at follow-up (headache: 68%, p = 0.016, visual disturbance: 55%, p = < 0.001, Wilcoxon-test). Follow-up BMI was only available for 36 out of 90 patients, with the mean follow-up BMI being lower compared to baseline (p = 0.031). Mean CSF-OP at follow-up was lower than at baseline (p < 0.001, follow-up CSF-OP available in 42 of 90 patients).

Table 1. Baseline characteristics of the study cohort.
	all patients n = 191	females n = 165	males n = 26	*p-value*
age at diagnosis	35.5 ± 12.5	34.4 ± 11.6	42.8 ± 15.2	0.001¹
BMI [kg/m²]	34.4 ± 8.4	34.7 ± 8.3	32.4 ± 8.8	0.2¹
CSF-OP [cmCSF]	36.2 ± 8.1	36.3 ± 8.4	34.8 ± 7.6	0.40¹
symptoms at baseline headache - visual disturbance - abducens palsy - vertigo - diplopia	150/179 (84%) 155/174 (89%) 18 (9%) 32/71 (45%) 31/80 (39%)	137 (83%) 135/152 (89%) 16 (9.7%) 28/63 (44%) 27/72 (38%)	13 (50%) 20/22 (90%) 2 (7%) 4/8 (50%) 4/8 (50%)	0.007² 1.0² 0.9² 1.0² 0.7²
ophthalmologic findings ■ BCVA [logMAR] ■ Frisén grade (median & IQR) ■ optic disc atrophy	0.17 ± 0.38 2 (1) 2	0.17 ± 0.41 2 (1) 2	0.15 ± 0.15 2 (1) 0	0.9¹ - -
¹two-sided p-values calculated by unpaired samples t-test. ²two-sided p-values obtained by Fisher’s exact test.

Figure 1. Flow chart of the inclusion/exclusion process.

Treatment

For the analysis of visual outcomes, follow-up data of 90 patients was available. During the follow-up period 17% were managed surgically by either implantation of a VP shunt (14%, 13/90) or by bariatric surgery (2%, 2/90). None of the patients were treated by sinus stenting. The group of surgically treated patients constituted of two men and 13 women. Surgical treatment was similarly frequent in men and women (men: 22%, n = 2/9, and women: 16%, 13/81; p = 0.712). Surgical treatment was more frequent in patients with moderate papilledema at baseline (22%) than in patients with mild (5%) and with severe papilledema (14%) at baseline.

Data on medication were available in 83/90 (92%) patients. Of those, 62% were treated pharmacologically (i.e. received either/or a combination of: acetazolamide, topiramate and furosemide). Forty-five percent (54%) of the pharmacologically treated patients received acetazolamide in a median daily dose (MDD) of 1000mg, 23% received topiramate (MDD: 100mg) and 8% furosemide (MDD: 40mg).

Ophthalmologic findings and visual outcome

At baseline 184 of 191 patients had papilledema. In the whole study cohort, median Frisén grade was 2 (range: 0–5, IQR: 2) and median decimal BCVA at baseline was 0.8 (IQR: 0.4). Two patients had optic disc atrophy at baseline.

Assessment of visual outcomes required the availability of ophthalmologic follow-up examinations at a minimum of 6 months after IIH diagnosis. Visual outcomes were available in 90 patients (i.e. the follow-up cohort), which represented 47% of the whole study cohort. Patients who were lost to follow up were older than patients in the follow-up cohort. Other baseline characteristics (sex, BMI, CSF-OP, BCVA, Frisén score) of the patients who were lost to follow-up did not significantly differ from those in the follow-up cohort (Supplemental Table 2).

The median time between baseline to follow-up was 3.4 years (range: 14 years, IQR: 3.5 years). Overall visual acuity was significantly better at follow-up than at baseline (p = 0.029). Follow-up perimetric MD was documented in 52% (47/90) of patients and exceeded 6dB in 40% (19/47). Perimetric MD was lower at follow-up, compared to baseline data, although not statistically significant (p = 0.155). Median papilledema grades at follow-up were lower than at baseline (p < 0.001). One patient in the follow-up cohort had optic disc atrophy at baseline, eleven patients (12%) had optic disc atrophy at follow-up. Ophthalmologic characteristics of the follow-up cohort are presented in Table 2.

Poor visual prognosis occurred in 36% (n = 32/90) of patients. This was defined as either 1) ophthalmologic worsening, which occurred in 22% and/or 2) persistent visual impairment, which occurred in 32% of patients. Worsening of BCVA occurred in 2 patients (3%) and worsening of papilledema occurred in 13 of 73 patients (i.e. 14%), who had follow-up documentation of papilledema grading.

In univariate group comparisons, neither sex, age, disease duration nor BMI baseline CSF-OP were significantly different between the visual outcome groups (Supplemental Table 1). Presence of headache at baseline (p = 0.8) and presence of abducens palsy (p = 1.0) were not associated with the visual outcome. Pharmacologic treatment was similarly frequent in the group of patients with poor and without poor outcome (p = 1.0, Suppl. Table 1) and was therefore not included into regression analysis.

In univariate regression analyses, the presence of moderate papilledema at baseline was associated with a lower likelihood of poor visual outcome (OR: 0.31, p = 0.043. Table 3). Here, surgical intervention was also strongly associated with a lower risk of poor visual outcome (OR: 0.21, p = 0.009, Table 3). Being a potential causal mediator of the papilledema-outcome relation, it was included into a multiple regression model as a sensitivity analysis, which yielded significant ORs for both moderate papilledema (OR: 0.13, p = 0.008) and for surgical intervention (OR: 14.5, p = 0.042), indicating partial mediation. Surgical intervention was therefore not included into the main multiple regression model in order to not bias the effect estimate.

Multivariable stepwise backward regression evaluated the influence of possible predictors to the visual outcome: The saturated model included the following independent variables: sex, baseline BMI, age at diagnosis, baseline CSF-OP, disease duration in months, and severity of papilledema at baseline. Due to listwise deletion of missing data, multivariable analysis included 71 patients. The final model included papilledema grades at baseline, sex and disease duration (Table 3, Nagelkerke R-square: 0.34). In multivariate analysis, the presence of moderate papilledema at baseline was associated with a lower likelihood of poor visual outcome compared to patients in the mild papilledema group (OR: 0.21, p = 0.016). The presence of severe papilledema at baseline showed no significant association with poor visual outcome compared to patients in the mild papilledema group (OR: 5.5, p = 0.157). Hosmer-Lemeshow-test indicated a good model fit (p = 0.537).

Table 2

Ophthalmologic findings at baseline and at follow-up in the follow-up cohort (90 patients).
	baseline	follow-up	p-value
BCVA [logMAR]^a (mean ± SD)	0.13 ± 0.20	0.077 ± 0.13	0.029¹
Frisén grade^b median (IQR)	2 (2)	0 (2)	< 0.001²
visual field MD^c (mean ± SD)	7.3 ± 7.4	4.8 ± 4.9	0.155¹
optic disc atrophy n	1	11	-
SD: standard deviation. IQR: interquartile range. ¹two-sided p-values calculated by paired samples t-test. ²two-tailed p-values obtained by Wilcoxon-test. ^adocumented in 68 patients. ^bdocumented in 79 patients. ^cdocumented in 17 patients.

Table 3

Regression analysis of the follow-up cohort (n = 90).
	poor visual outcome
	univariable		multivariable
	OR (95% CI) ¹	p-value	OR (95% CI)²	p-value
age at diagnosis	1.02 (0.98–1.16)	0.283	not retained	-
baseline BMI	0.99 (0.93–1.04)	0.600	not retained	-
baseline CSF-OP	1.02 (0.97–1.08)	0.391	not retained	-
disease duration	1.01 (1.00–1.02)	0.174	1.01 (0.99–1.03)	0.075
male sex	2.50 (0.62–10.07)	0.198	7.17 (1.2–41.7)	0.028
surgical therapy	0.21 (0.06–0.68)	0.009	not included
baseline papilledema - moderate - severe	0.31 (0.10–0.97) 6.67 (0.67–66.5)	0.043 0.106	0.21 (0.06–0.75) 5.47 (0.52–57.3)	0.016 0.157
CSF-OP: cerebrospinal fluid opening pressure, OR: odds ratio, 95%CI: 95% confidence interval. ¹Obtained by univariable binary regression. ²Obtained by multivariable stepwise backward regression (likelihood ratio). Bold values indicate significant likelihood of the independent variables contributing to a poor visual prognosis. (Nagelkerke R-square: 0.34; Hosmer-Lemeshow test: p = 0.537)

The aim of this study was to evaluate baseline characteristics, the visual prognosis and predictors of poor visual outcome in a well-characterized cohort of IIH patients in Germany.

In our study, the mean age at diagnosis was 35.5 years, which lies above that of other observational studies (i.e. 27–34 years) [4, 6, 15, 16]. Our study excluded pediatric IIH patients, which is in line with prior studies [4]. Apart from that, the demographic profile of our cohort was consistent with that of other studies: As IIH is most common in obese females, 69% of our patients were obese and males constituted 7% of the study cohort. The frequency of IIH signs and symptoms, was comparable to other cohorts [17, 18]. Elevated BMI and CSF-OP were also consistent with previous observations [15, 19].

In this cohort, patients experienced significant improvement of papilledema and of visual acuity. However, ten patients developed optic disc atrophy during the follow-up period. Poor visual outcome occurred in 36% of patients, which lies within the previously described range of 10–60%, albeit definitions of visual outcomes vary [2, 15, 20, 21]. In terms of visual acuity, none of our patients became blind. With 17%, the frequency of surgical therapy in our follow-up cohort was comparable to that of other studies, which report a surgical intervention rate between 2 and 20% [15, 18, 22].

In multivariable analyses, backward regression suggested a model including sex and the degree of papilledema at baseline as a model to predict visual outcome. The model also included disease duration, however, not with statistical significance (p = 0.075). Male sex and longer disease duration have priorly been associated with poor visual prognosis in IIH [4, 23] Also consistent with recent literature, neither BMI nor CSF-OP at baseline were associated with the visual outcome [6, 18]. In prior reports, patients with severe papilledema had a greater risk of poor visual prognosis [24]. In our model, this association was not statistically significant (OR: 5.47 (0.52–57.3), p = 0.157), and the large confidence interval may reflect the small number of patients (n = 7) with severe papilledema at baseline. Notably, moderate papilledema was significantly associated with a lower risk for poor visual outcome compared to patients in the mild papilledema group (OR: 0.21, p = 0.016). Conversely, these patients received surgical therapy more frequently than patients with mild or severe papilledema. However, multivariable analysis showed that the lower odds for poor visual outcome were only partially mediated by surgical therapy. There may be other factors mediating the outcome in patients with moderate papilledema. Beyond that, one third of our patients experienced worsening of ophthalmologic findings or visual impairment. Reliable predictors of the visual outcome and IIH-specific treatment strategies to prevent visual loss are to be desired.

There are several limitations given the retrospective nature of our study. All data was collected during routine clinical work-up and many patients were lost to follow-up. Especially asymptomatic patients or patients with a mild disease course were more likely to discontinue management than to be expected in a prospectively collected cohort. The proportion of more severely affected patients may therefore be overestimated. The visual outcomes in our cohort do not necessarily reflect long term outcome, as many patients were not followed for more than 6 months. A further limitation is that individual follow-up periods were variable. We strove to mitigate those limitations by standardizing data collection and listwise deletion in case of missing data. We diligently applied the current diagnostic criteria and minimized the risk of confounders, choosing a backward regression model and taking treatment strategies and disease duration into account. As this is a real-world cohort, treatment regimens may have varied, potentially introducing bias. Adjusting regression models for more detailed treatment parameters (i.e. specific drugs and dosages) would have caused overfitting. Moreover, patient reported outcomes were not assessed in this cohort. Although headache affects the quality of life in IIH, studies investigating headache outcome and the burden of other symptoms throughout the course of the disease are rare.

In our retrospective, real-world IIH cohort, visual outcomes were generally good. However, a proportion of patients retained persistent visual impairment or experienced deterioration of visual acuity. In line with prior research, men had a greater risk of poor visual outcome. However, the presence of moderate papilledema at baseline lowered the likelihood for a poor visual outcome, partially due to employment of surgical therapy in these patients. We hypothesize that patients with moderate papilledema received a more intensive treatment regimen than patients with mild papilledema, which effectively prevented visual impairment. This emphasizes the importance of timely intervention in patients with moderate papilledema. Further baseline characteristics, such as age, BMI and CSF-OP were not associated with the visual outcome. Therefore, all patients should be closely monitored after IIH diagnosis. With increasing incidence of IIH [1], there is an unmet need to identify outcome predictors and to prevent visual impairment.

BCVA

best corrected visual acuity

BMI

Body-Mass-Index

CSF-OP

cerebrospinal fluid opening pressure

CSF

cerebrospinal fluid

ICP

intracranial pressure

IIH-WOP

IIH without papilledema

IIH

Idiopathic intracranial hypertension

IQR

interquartile range

logMAR

logarithm of the minimum angle of resolution

mean deviation

MDD

median daily dose

MRI

magnetic resonance imaging

NRS

numeric rating scale

VP shunt

ventriculo-peritoneal shunt

Ethics approval and consent to participate: This research study was conducted retrospectively from data obtained for clinical purposes. The need for informed consent was waived and retrospective data extraction and analysis was performed in approval with the ethics committee of Charité - Universitätsmedizin Berlin, Germany, on 12 February 2021 (Application No. EA4/004/21). The study was conducted in accordance with the Declaration of Helsinki.

Data availability: The datasets generated for the current study are not publicly available but are available from the corresponding author on reasonable request.

Consent for publication: Informed consent was waived due to the retrospective nature of the study.

Competing interest: The authors declare that they have no financial or non-financial conflicts of interest to disclose.

Funding: No sources of funding were received for this work.

Author contributions: TK oversaw study design and conception. Data collection was performed by TK, PH and LV. Data analysis and visualization was performed by TK and LV with counselling for statistical analysis and interpretation by AP and LAD. AK gave critical input regarding study design and conception. The draft of the manuscript was written by TK and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript and gave consent for publication.

Acknowledgements: None.

Shaia JK, Sharma N, Kumar M, et al (2024) Changes in Prevalence of Idiopathic Intracranial Hypertension in the United States Between 2015 and 2022, Stratified by Sex, Race, and Ethnicity. Neurology 102:. https://doi.org/10.1212/WNL.0000000000208036
Agarwal A, Vibha D, Prasad K, et al (2017) Predictors of poor visual outcome in patients with Idiopathic Intracranial Hypertension (IIH): An ambispective cohort study. Clin Neurol Neurosurg 159:13–18. https://doi.org/10.1016/j.clineuro.2017.05.009
Thaller M, Homer V, Mollan SP, Sinclair AJ (2023) Disease Course and Long-term Outcomes in Pregnant Women With Idiopathic Intracranial Hypertension: The IIH Prospective Maternal Health Study. Neurology 10.1212/WNL.0000000000206854. https://doi.org/10.1212/wnl.0000000000206854
Hatem CF, Yri HM, Sørensen AL, et al (2018) Long-term visual outcome in a Danish population of patients with idiopathic intracranial hypertension. Acta Ophthalmol 96:719–723. https://doi.org/10.1111/aos.13664
Bruce BB, Preechawat P, Newman NJ, et al (2008) Racial Differences in Idiopathic Intracranial Hypertension. Neurology 70:861–867. https://doi.org/10.1212/01.wnl.0000304746.92913.dc
Bsteh G, Marik W, Krajnc N, et al (2023) MRI features of idiopathic intracranial hypertension are not prognostic of visual and headache outcome. Journal of Headache and Pain 24:. https://doi.org/10.1186/s10194-023-01641-x
Knoche T, Gaus V, Haffner P, Kowski A (2023) Neurofilament light chain marks severity of papilledema in idiopathic intracranial hypertension. Neurological Sciences 44:2131–2135. https://doi.org/10.1007/s10072-023-06616-z
Beier D, Korsbæk JJ, Madsen JS, et al (2020) Neurofilament light chain as biomarker in idiopathic intracranial hypertension. Cephalalgia 40:1346–1354. https://doi.org/10.1177/0333102420944866
Hoffmann J, Mollan SP, Paemeleire K, et al (2018) European headache federation guideline on idiopathic intracranial hypertension. Journal of Headache and Pain 19:. https://doi.org/10.1186/s10194-018-0919-2
Friedman DI, Liu GT, Digre KB (2013) Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 81:1159–1165. https://doi.org/10.1212/WNL.0b013e3182a55f17
Gaier ED, Heidary G (2019) Pediatric Idiopathic Intracranial Hypertension. Semin Neurol 39:704–710. https://doi.org/10.1055/s-0039-1698743
Wall M, Johnson CA, Cello KE, et al (2016) Visual Field Outcomes for the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). Investigative Opthalmology & Visual Science 57:805. https://doi.org/10.1167/iovs.15-18626
Frisen L (1982) Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry 45:13–18. https://doi.org/10.1136/jnnp.45.1.13
Sheils CR, Fischer WS, Hollar RA, et al (2018) The Relationship Between Optic Disc Volume, Area, and Frisén Score in Patients With Idiopathic Intracranial Hypertension. Am J Ophthalmol 195:101–109. https://doi.org/10.1016/j.ajo.2018.07.032
Xu W, Prime Z, Papchenko T, Danesh-Meyer H V (2021) Long term outcomes of idiopathic intracranial hypertension: Observational study and literature review. Clin Neurol Neurosurg 205:106463. https://doi.org/10.1016/j.clineuro.2020.106463
Tata G, Kisabay A, Gokcay F, Celebisoy N (2019) Idiopathic intracranial hypertension: Are there predictors for visual outcome or recurrences? Clin Neurol Neurosurg 183:105378. https://doi.org/10.1016/j.clineuro.2019.105378
Wall M, Kupersmith MJ, Kieburtz KD, et al (2014) The idiopathic intracranial hypertension treatment trial clinical profile at baseline. JAMA Neurol 71:693–701. https://doi.org/10.1001/jamaneurol.2014.133
Rosenblatt A, Klein A, Roemer S, et al (2016) Idiopathic intracranial hypertension - A comparison of clinical characteristics between 4 medical centers in different geographic regions of the world. Journal of Neuro-Ophthalmology 36:280–284. https://doi.org/10.1097/WNO.0000000000000402
Yri HM, Wegener M, Sander B, Jensen R (2012) Idiopathic intracranial hypertension is not benign: A long-term outcome study. J Neurol 259:886–894. https://doi.org/10.1007/s00415-011-6273-9
Wall M, Kupersmith MJ, Thurtell MJ, et al (2017) The Longitudinal Idiopathic Intracranial Hypertension Trial: Outcomes From Months 6–12. Am J Ophthalmol 176:102–107. https://doi.org/10.1016/j.ajo.2017.01.004
Thaller M, Homer V, Hyder Y, et al (2023) The idiopathic intracranial hypertension prospective cohort study: evaluation of prognostic factors and outcomes. J Neurol 270:851–863. https://doi.org/10.1007/s00415-022-11402-6
Mikkilineni S, Trobe JD, Cornblath WT, De Lott L (2019) Visual Field Mean Deviation at Diagnosis of Idiopathic Intracranial Hypertension Predicts Visual Outcome. Journal of Neuro-Ophthalmology 39:186–190. https://doi.org/10.1097/WNO.0000000000000709
Donaldson L, Jhaveri A, Micieli J, Margolin E (2022) Idiopathic intracranial hypertension in atypical demographics. J Neurol Sci 437:. https://doi.org/10.1016/j.jns.2022.120271
Wall M, Falardeau J, Fletcher WA, et al (2015) Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension. Neurology 85:799. https://doi.org/10.1212/WNL.0000000000001896

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Predictors of visual outcome in Idiopathic Intracranial Hypertension: observations from a retrospective cohort in Germany

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Abstract

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Background

Methods

Patient Selection

Data collection

Ophthalmologic Findings

Statistical analysis

Results

Cohort characteristics

Treatment

Ophthalmologic findings and visual outcome

Discussion

Conclusions

Abbreviations

Declarations

References

Additional Declarations

Supplementary Files

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