Quality of Life in Patients with Idiopathic Intracranial Hypertension and the Impact of the COVID-19 Pandemic

ABSTRACT

The COVID-19 pandemic became a challenge to maintain care for patients with idiopathic intracranial hypertension (IIH). We aimed to find out how they were affected during lockdown. Thirty IIH patients admitted to hospital during the COVID-19 pandemic were studied. Their demographic and neuro-ophthalmological findings were evaluated. The World Health Organization – Five Well-Being Index (WHO-5), the EUROHIS Quality of Life (QOL) 8-item index, National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25), Headache Impact Test (HIT-6), and COVID-19 Fear Scale were used to assess QOL and pandemic-associated fear. Thirty age, sex, and body mass index matched volunteers constituted the control group. Apart from the COVID-19 Fear Scale and colour vision subscale of the NEI-VFQ-25, all scale scores were worse in IIH patients than in healthy control subjects. Patients with severe visual field defects had higher HIT-6 scores (p = .036). Both vision-specific and overall QOL was reduced in patients with IIH. Headache severity and disability were more prominent in patients with severe visual loss. Fear caused by the COVID pandemic was not different in IIH patients than in healthy control subjects.

Introduction

Coronavirus disease 2019 (COVID-19) was identified as a global pandemic by the World Health Organization in March 2020. COVID-19 is caused by the novel coronavirus severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a single-stranded ribonucleic acid virus that is now one of the seven coronaviruses known to infect humans.¹ A sizeable subset of patients with SARS-CoV-2 manifest neurological symptoms, often including headache.² The COVID-19 pandemic and lockdown has caused disruption in health services, caused post-traumatic stress disorder, and caused a deterioration in quality of life (QOL) in patients with chronic neurological disorders including Parkinson’s disease, headache, multiple sclerosis, epilepsy, polyneuropathy, and stroke.³

Idiopathic intracranial hypertension (IIH) is characterised by raised intracranial pressure without any identifiable pathology in the brain and with normal cerebrospinal fluid (CSF) composition.⁴ Patients with IIH must be monitored closely to assess visual function and to take interventions into consideration when vision is under threat. The COVID-19 lockdown prohibited patients from their usual follow-up visits and caused lifestyle changes.

The main aim of this study was to assess QOL in patients with IIH diagnosed during the COVID-19 pandemic. The QOL scores of patients with severely impaired visual function were compared with the ones showing less severe visual impairment.

Methods

Patients meeting the diagnostic criteria for IIH⁵ and admitted for the first time to the Neuro-ophthalmology Unit of Ege University Medical School, Department of Neurology between June 2020 and December 2020 were included in this cross-sectional cohort. The study protocol was approved by the Ege University Medical School Ethics Committee and Turkish Ministry of Health (reference number: 99166796-050.06.04782) and was performed in accordance with the ethical standards outlined in the Declaration of Helsinki. Written informed consent was obtained from all participants.

All patients had contrast-enhanced magnetic resonance imaging (MRI) of the brain including venography to exclude intracranial abnormalities and cerebral venous sinus thrombosis. All had a CSF opening pressure ≥250 mmCSF in the lateral decubitus position with normal CSF composition.

Data collected included demographics, body mass index (BMI) (weight/height²), CSF opening pressure, visual acuity (logarithm of the minimum angle of resolution [logMAR]), perimetric mean deviation (PMD) (Humphrey 24–2 Swedish Interactive Testing Algorithm [SITA]) central threshold), and papilloedema grade (Frisén grade) detected by fundus photography.⁶ The worse affected eye of each patient was chosen for the statistical analyses. Patients with other chronic diseases, known eye diseases, and pregnancy were excluded as well as ones with SARS-CoV-2 infection.

QOL during the COVID-19 lockdown was assessed by using the scales given below by a senior neurologist. The scales were administered to patients on headache-free days.

Healthy volunteers who had no complaints including headache and who were matched for sex, age, and BMI constituted the control group.

Scales

1. World Health Organization – Five Well-Being Index (WHO-5): This is a short self-reported measure of current mental wellbeing within the last month assessed with five simple and non-invasive questions. The raw score ranges from 0 to 25, with 0 representing the worst possible and 25 representing the best possible QOL. To obtain a percentage score ranging from 0 to 100, the raw score is multiplied by 4. A percentage score of 0 represents the worst possible, whereas a score of 100 represents the best possible QOL.⁷

2. The EUROHIS-QOL 8-item index: This is an eight-item measure of QOL. The domains are general, physical, psychological, social, and environmental aspects of QOL. The overall QOL score is formed by a simple summation of scores on the eight items, with higher scores indicating better QOL.⁸

3. National Eye Institute Visual Functioning Questionnaire (NEI-VFQ) 25: This measures the dimensions of self-reported vision-targeted health status that are most important for persons with chronic eye diseases. The 12 sub-scales (number of items) that are included are as follows: general health (1); general vision (1); near (3), distance (3), peripheral (1), and colour vision (1); vision-specific role difficulties (2); dependency (3); mental health (4); social function (2); ocular pain (2); and driving (2).⁹ The driving subscale was not used in our group as very few of our participants were drivers.

4. Headache Impact Test (HIT-6): The HIT-6 items measure the adverse impact of headache on social functioning, role functioning, vitality, cognitive functioning, and psychological distress. The HIT-6 also measures the severity of headache pain. The four headache impact severity categories are little or no impact (49 or less), some impact (50–55), substantial impact (56–59), and severe impact (60–78).¹⁰

5. COVID-19 Fear Scale: This is a seven-item scale. The participants indicate their level of agreement with the statements using a five-item Likert-type scale (1 = strongly disagree to 5 = strongly agree). A total score is calculated by adding up each item score (ranging from 7 to 35). The higher the score, the greater the fear of COVID-19. It has been shown to have good internal consistency, test–retest reliability, and good construct validity.¹¹

All the scales have had validity and reliability studies performed on Turkish populations.^12–16

The scale scores of the healthy controls were compared with the scores of the IIH patients. Depending on the PMD, patients were grouped into two as having mild-to-moderate (PMD −2 to −14 dB) or severe (PMD worse than −14 dB) visual loss.¹⁷ BMI, mean CSF opening pressure, logMAR visual acuity, papilloedema grade, and the scale scores of the two groups were compared.

Statistical analysis

Statistical Product and Service Solutions (SPSS) 21 for Windows (SPSS Inc., Chicago, IL, USA) was used for the statistical analysis. Descriptive statistics were expressed as mean ± standard error (SE) or median (range) for numeric variables. The Mann–Whitney U-test was used for comparison of independent variables. Categorical variables were compared with the chi-square test. The Mann–Whitney U-test was also used for comparisons between the two groups regarding the severity of their visual field defects. The difference between the groups was considered statistically significant when the p-value was <.05.

Results

Thirty patients meeting the diagnostic criteria for IIH and 30 healthy controls were included in the study. They were all female. The mean age of the patients was 29.8 ± 8.2 years (range: 18–43 years), and the mean age of the healthy controls was comparable at 30.8 ± 5.3 years (range: 18–38 years) (p = .573). The mean BMI of the patients was 31.93 ± 5.58 (range: 22–42) kg/m², and the healthy controls were comparable at 31.20 ± 2.44 (range: 25–34) kg/m² (p = .727).

The mean duration of symptoms prior to diagnosis with IIH was 3.4 ± 1.9 months (range: 1–7 months). The delay in admission to hospital was mainly due to the transformation of the clinics to COVID-19 clinics between March 2020 and June 2020. The mean CSF opening pressure was 402.2 ± 153.7 (range: 270–740) mmCSF. The visual acuity at onset was 0.0 logMAR in 12, 0.1 logMAR in 10, 0.2 logMAR in two, 0.3 logMAR in three, 0.4 logMAR in one, and 0.5 logMAR in two patients. Funduscopic examination revealed grade 1 papilloedema in nine, grade 2 papilloedema in seven, grade 3 papilloedema in 10, grade 4 papilloedema in two, and grade 5 papilloedema in two patients. The mean PMD at onset was −9.5 ± 4.2 (range: −1.3 to −17.4) dB. There were 22 patients with mild-to-moderate and eight patients with severe visual field loss.

When the scale results of the patients were compared with the healthy controls, it was found that apart from the COVID-19 Fear Scale (p = .157) and the NEI-VFQ 25 colour vision subscale (p = .671), all scores were significantly different, indicating impaired scores in all scales in patients with IIH (p < .05) (Table 1).

Table 1.

Comparison of the scale results of patients with idiopathic intracranial hypertension and healthy controls.

SCALES		Patients with IIH (n = 30) ± SE (range)	Healthy controls (n = 30) ± SE (range)	p values
*WHO-5		48.40 ± 14.11 (16.00–80.00)	59.86 ± 10.58 (40.00–80.00)	.001
*EUROHIS-QOL 8		57.77 ± 10.68 (35.00–75.00)	70.36 ± 10.03 (50.00–84.00)	<.010
*HIT-6		56.23 ± 9.37 (38.00–67.00)	49.90 ± 8.29 (38.00–64.00)	.006
*COVID-19 Fear Scale		19.60 ± 6.26 (9.00–32.00)	17.10 ± 4.85 (7.00–26.00)	.157
NEI-VFQ 25	*Near vision	81.94 ± 14.69 (41.67–100.00)	95.55 ± 7.49 (75.00–100.00)	<.001
	*Distance vision	73.06 ± 16.48 (33.33–91.67)	95.00 ± 7.45 (75.00–100.00)	<.001
	*Vision-specific role difficulties	80.00 ± 16.61 (25.00–100.00)	96.66 ± 7.99 (75.00–100.00)	<.001
	*Mental health	70.42 ± 18.12 (37.50–93.75)	96.25 ± 3.88 (87.50–100.00)	<.001
	*Ocular pain	74.58 ± 18.12 (50.00–100.00)	89.10 ± 13.02 (50.00–100.00)	.002
	*Driving	54.49 ± 29.78 (0–83.33)	85.33 ± 12.33 (41.67–100.00)	<.001
	**General health n (25–50/75+)	27/3	17/13	.009
	**General vision n (20–60/80+)	24/6	21/9	<.001
	**Peripheral vision n (50–75/100)	15/15	2/28	<.001
	**Colour vision n (75/100)	4/26	2/28	.671
	**Social function n (<100/100)	13/17	4/26	.022
	**Dependency n (<100/100)	12/18	0/30	<.001

*Mann–Whitney U-test **Chi-square test

EUROHIS-QOL 8 = EUROHIS Quality of Life 8-item index; HIT-6 = Headache Impact Test; NEI-VFQ 25 = National Eye Institute Visual Functioning Questionnaire; SE = standard error; WHO-5 = World Health Organization – Five Well-Being Index

Comparison of the two groups with mild-to-moderate and severe visual field defects regarding BMI and mean CSF opening pressure did not reveal significant difference (p = .70 and p = .10, respectively). On the other hand, logMAR visual acuity and papilloedema grade were significantly worse in those patients with severe visual field defects compared with those with mild-to-moderate visual field defects (p = .004 and p ≤ .001, respectively) (Table 2). The two groups were also compared regarding the QOL scale scores. Only the HIT-6 scores showed significant difference with higher scores noted in patients with severe visual field defects compared with those with mild-to-moderate visual field defects (p = .036). Other scale scores were not significantly different between the two groups (p ≥ .05) (Table 3).

Table 2.

Comparison of the clinical features of patients with mild-to-moderate and severe visual loss.

	PMD –2 to −14 dB (n = 22) ± SE (range)	PMD worse than −14 dB (n = 8) ± SE (range)	p values*
BMI (kg/m2)	35.73 ± 5.97 (21.00–42.00)	32.50 ± 4.66 (26.00–41.00)	.707
CSF opening pressure (mmCSF)	370.45 ± 131.63 (250.00–740.00)	491.88 ± 180.43 (270.00–680.00)	.103
Visual acuity (logMAR)	0.073 ± 0.103 (0.00–0.40)	0.263 ± 0. 177 (0.00–0.50)	.004
Papilloedema Frisén grade	1.86 ± 0.83 (1–3)	3.75 ± 0.89 (3–5)	<.001

*Mann–Whitney U-test

BMI = Body mass index; CSF = Cerebrospinal fluid; PMD = Perimetric mean deviation; SE = standard error

Table 3.

Comparison of the scale results of patients with mild-to-moderate and severe visual loss.

		PMD –2 to −14 dB (n = 22) ± SE (range)	PMD worse than −14 dB (n = 8) ± SE (range)	p values*
WHO-5		50.91 ± 13.70 (20.00–80.00)	41.50 ± 13.68 (16.00–64.00)	.073
EUROHIS-QOL 8		58.23 ± 11.19 (35.00–75.00)	56.50 ± 9.74 (44.00–73.00)	.586
HIT		54.05 ± 9.62 (38.00–67.00)	62.25 ± 5.44 (50.00–67.00)	.036
COVID-19 Fear Scale		20.09 ± 7.03 (9.00–32.00)	18.25 ± 3.33 (14.00–23.00)	.653
NEI-VFQ 25	Near vision	83.71 ± 14.88 (41.67–100.00)	77.08 ± 13.91 (50.00–91.67)	.223
	Distance vision	73.86 ± 16.53 (41.67–91.67)	70.83 ± 17.25 (33.33–91.67)	.667
	Vision-specific role difficulties	80.68 ± 17.98 (25.00–100.00)	78.13 ± 12.94 (50.00–87.50)	.609
	Mental health	72.16 ± 18.17 (37.50–93.75)	65.63 ± 18.30 (43.75–87.50)	.393
	Ocular pain	75.57 ± 19.47 (50.00–100.00)	71.88 ± 14.56 (50.00–100.00)	.683
	General health	44.32 ± 17.13 (25.00–75.00)	40.63 ± 12.94 (25.00–50.00)	.656
	General vision	59.09 ± 16.88 (20.00–80.00)	47.50 ± 14.88 (20.00–60.00)	.097
	Peripheral vision	86.36 ± 16.77 (50.00–100.00)	81.25 ± 17.68 (50.00–100.00)	.435
	Colour vision	97.76 ± 7.36 (75.00–100.00)	93.75 ± 11.57 (75.00–100.00)	.265
	Social function	90.91 ± 12.31 (62.50–100.00)	87.50 ± 22.16 (37.50–100.00)	.917
	Dependency	93.18 ± 11.10 (66.67–100.00)	86.46 ± 20.38 (50.00–100.00)	.426

*Mann–Whitney U-test

EUROHIS-QOL 8 = EUROHIS quality of life (QOL) 8-item index; HIT-6 = Headache Impact Test; NEI-VFQ 25 = National Eye Institute Visual Functioning Questionnaire; PMD = Perimetric mean deviation; SE = Standard error; WHO-5 = World Health Organization – Five Well-Being Index

Discussion

Headache is the most common symptom,¹⁸ and visual loss is a serious complication that may occur either early or late in the course of IIH.¹⁹ Both are associated with reduced QOL. There are few studies that have assessed QOL in IIH patients by using different scales, including the Short Form-36 (SF-36), NEI-VFQ-25, and HIT-6.^20,21

A strong association between IIH and obesity is well known, and obese patients have been shown to have lower QOL scores than non-obese controls.^22–25 Therefore, in order to rule out the effect of obesity, we chose BMI-matched volunteers as healthy controls for this study.

Apart from the colour vision sub-scale of visual functioning, all other subscale scores of vision-targeted health status were significantly lower in the IIH patients compared with the healthy controls. Additionally, all other QOL scores including mental wellbeing assessed by the WHO-5; general, physical, psychological, social, and environmental aspects of QOL assessed by the EUROHIS-QOL 8-item index; and headache impact on daily living assessed by the HIT-6 scale were impaired in the IIH group. Similar results have been reported in previous studies. Kleinschmidt et al. found that patients with IIH had significantly lower scores for social functioning and had worse scores for both depression and anxiety.²⁶ Daniels et al. found that NEI-VFQ-25 and SF-36 subscale scores were lower in IIH.²¹ Mulla et al. reported that headache was the main determinant of reduced QOL needing effective treatment.²⁷ The IIH Treatment Trial demonstrated QOL reduction at disease onset with mild visual loss, linked with raised intracranial pressure.²⁰ On the NEI-VFQ-25, ocular pain and distance driving subscale scores were prominently affected. Headache severity was associated with the NEI-VFQ-25 composite score, the Neuro-Ophthalmic Supplement score, and scores on SF-36. Visual loss, headache, neck pain, transient visual obscurations, and binocular diplopia were all independently associated with poorer QOL. Improvement was noted after treatment with acetazolamide at the 6 month follow-up visit.²⁸

In our study, depending on the PMD, patients were divided into two groups, as those with mild-to-moderate and severe visual field defects, to find out if severe visual loss was associated with more impairment of QOL. However, this was true just for the HIT-6 scores (p = .036). Headache severity and the impact of headache on daily living were higher in patients with severe visual loss (Table 3). Other aspects of QOL, including visual functioning, were similarly affected in all patients regardless of the severity of the visual field defect. Comparison of the two groups regarding BMI and mean CSF opening pressure did not reveal significant difference (p > .05). Similarly, in a previous study, CSF pressure elevation was not associated with PMD.²⁹ On the other hand, logMAR visual acuity was significantly lower (p = .004) and papilloedema grade was significantly higher (p ≤ .001) in patients with severe visual field defects compared with those with mild-to-moderate visual field defects (Table 2).

The interesting finding was that there was the same level of fear of COVID-19 in patients with IIH and healthy controls. We were expecting to find increased levels of COVID-19 fear in IIH patients as they had to go into public more often than the healthy controls and come to hospital regularly for follow-up. In the only study to date assessing the impact of COVID-19 lockdown on patients with IIH, an increased risk of disease deterioration and CSF shunting has been reported with raised anxiety and depression scores.³⁰ Increased levels of anxiety, depression, sleep disruption, and reduced QOL have been reported in patients with different neurological disorders during the COVID-19 pandemic including migraine,³¹ multiple sclerosis,³² epilepsy,³³ and Parkinson’s disease.³⁴ However, controversial results are also present. In patients with multiple sclerosis, no worsening of anxiety or depression levels has been reported.³⁵

The limitation of our study is the low number of patients coming from one centre and it being a cross-sectional study. A longitudinal study is still going on to see if the disease course of these patients will be different from the patients followed up during pre-COVID-19 pandemic times.

In conclusion, both vision-specific and overall QOL was reduced in patients with IIH at disease onset. Headache disability was more prominent in patients with severe visual field defects. Fear caused by the COVID-19 pandemic was not different in IIH patients compared with healthy controls.

Funding Statement

The authors reported that there is no funding associated with the work featured in this article.

Data availability statement

All data generated or analysed during this study are included in this published article.

Declaration of interest statement

No potential conflict of interest was reported by the authors.

Patient consent and ethics statement

A written informed consent was obtained from all participants. The study protocol was approved by Ege University Medical School Ethics Committee and Turkish Ministry of Health (reference number: 99166796-050.06.04782) and was performed in accordance with the ethical standards outlined in the Declaration of Helsinki.