Idiopathic intracranial hypertension leading to bilateral optic atrophy in a patient with recent COVID-19 infection: a case report

Neurologic complications are common in patients hospitalised with COVID-19 infection. Most common complications are myalgias, headaches, encephalopathy and dizziness. Uncommon complications are stroke, motor and sensory deficits, seizures, ataxia and movement disorders. Multiple neuro-ophthalmological manifestations have also been reported in association with COVID-19. These complications may be the result of a range of pathophysiological mechanisms like hypoxic neuronal injury during active COVID-19 infection, RAS dysfunction, immune dysfunction and direct injury by the virus etc throughout the course of the disease. Here we reported a case of neuro-ophthalmic complication of Idiopathic intracranial hypertension (IIH) followed by bilateral optic atrophy in a middle-aged man with recent COVID-19 infection. He presented to the emergency with complaints of headache, dizziness and sudden painless bilateral diminution of vision for 3 days. His fundus examination was suggestive of bilateral papilledema, his MRI brain was normal and opening pressure of CSF was raised on lumbar puncture. His MRV was normal, there was no evidence of CSVT. He was started on steroids and acetazolamide. His headache improved but there was no improvement in visual acuity. Repeat fundus showed pale disc and MRI orbit was suggestive of bilateral optic atrophy.

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Fulminant Idiopathic Intracranial Hypertension and Venous Stasis Retinopathy Resulting in Severe Bilateral Visual Impairment

European Journal of Ophthalmology ◽

10.5301/ejo.5000918 ◽

2017 ◽

Vol 27 (2) ◽

pp. e25-e27 ◽

Cited By ~ 2

Author(s):

Alexander Voldman ◽

Breanna Durbin ◽

John Nguyen ◽

Brian Ellis ◽

Monique Leys

Keyword(s):

Optic Nerve ◽

Magnetic Resonance ◽

Intracranial Hypertension ◽

Optic Atrophy ◽

Idiopathic Intracranial Hypertension ◽

Venous Stasis ◽

Fundus Examination ◽

History Of ◽

Venous Stasis Retinopathy ◽

Central Retinal Vein

Purpose To report a complicated case of fulminant idiopathic intracranial hypertension and concomitant venous stasis retinopathy leading to postpapilledema optic atrophy. Methods Case report. Results A 34-year-old morbidly obese woman with a history of idiopathic intracranial hypertension (IIH) presented with a 1-month history of bilateral vision loss, diplopia, and left eye pain after being lost to follow-up for 6 years. Fundus examination revealed florid papilledema with venous tortuosity bilaterally. Brain and orbit magnetic resonance imaging showed bilateral globe flattening, intraocular optic nerve swelling in both eyes, and no abnormality on magnetic resonance venography. After additional workup including lumbar puncture with an opening pressure of 55 cm H2O, a diagnosis of IIH was confirmed. Medical treatment with oral carbonic anhydrase inhibitors was initiated, followed by same-day bilateral optic nerve sheath decompression and ventriculoperitoneal shunt placement the following week. Fundus examination 2 months later revealed a persistent blood and thunder fundus suggestive of bilateral central retinal vein occlusions. Over the course of 6 months, both eyes displayed postpapilledema optic atrophy with light perception and hand motion vision in the right and left eyes, respectively. On Goldmann perimetry, the patient had vague limited isolated responses in both eyes to the largest target. Conclusions Fulminant IIH can present with profoundly blinding complications recalcitrant to aggressive medical and surgical intervention. Central retinal vein occlusion is an uncommon blinding complication of IIH.

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“Benign intracranial hypertension” is a misleading name for a more serious disease: analysis of the visual outcome in cases with idiopathic intracranial hypertension

Egyptian Journal of Neurosurgery ◽

10.1186/s41984-019-0064-5 ◽

2019 ◽

Vol 34 (1) ◽

Author(s):

Ali R. Hamdan ◽

Amr M. Tayel ◽

Eslam El Sayed El Khateeb ◽

Osama Elsoghiar

Keyword(s):

Cerebrospinal Fluid ◽

Visual Acuity ◽

Magnetic Resonance ◽

Visual Field ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

The Other ◽

Benign Intracranial Hypertension ◽

Fundus Examination ◽

Opening Pressure

Abstract Objective Assessment of visual field outcomes following the medical or surgical management of increased intracranial pressure in patients with idiopathic intracranial hypertension. Patient and methods Thirty-two patients diagnosed with idiopathic intracranial hypertension who met all the modified Dandy criteria were included in this study. Magnetic resonance brain imaging with contrast and magnetic resonance venography were performed to exclude the presence of secondary causes of intracranial hypertension. Following normal neuroimaging, a lumbar puncture was performed for all patients with papilledema to measure the cerebrospinal fluid opening pressure. The assessment of visual function, including visual acuity, fundus examination (dilated fundus examination to confirm the presence and the grade of papilledema and to exclude the other ocular causes of optic disc swelling), and formal visual field assessment by perimetry when visual acuity was more than 1/60 were performed by the ophthalmologist before and after medical or surgical treatment. Results Thirty-two patients were included, the majority of whom were females of childbearing age (29/32; 90.6%). All patients complained of headache. Visual assessment revealed the presence of blurry vision in all patients. Papilledema with different grades was observed in 31/32 (96.9%) patients, while the other patient (3.1%) showed optic atrophy; 30/32 (93.8%) patients had visual acuity ˃ 1/60. The average visual field was − 18 dB ± 9.97 SD in the right eye and − 19.47 dB ± 5.43 SD in the left eye. Twenty-nine patients received medical treatment in the form of acetazolamide, which succeeded in controlling idiopathic intracranial hypertension in 24/29 (82.7%) patients, while a theco-peritoneal shunt was inserted in 8/32 (25%) patients. After management, statistically significant improvements in cerebrospinal fluid opening pressure, presenting manifestation, visual acuity, and field were noticed. Conclusions Permanent visual loss is the most feared complication of idiopathic intracranial hypertension; therefore, early diagnosis and multidisciplinary treatment are necessary to improve visual acuity and visual field.

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11β-Hydroxysteroid dehydrogenase type 1 inhibition in idiopathic intracranial hypertension: a double-blind randomized controlled trial

Brain Communications ◽

10.1093/braincomms/fcz050 ◽

2020 ◽

Vol 2 (1) ◽

Cited By ~ 7

Author(s):

Keira Markey ◽

James Mitchell ◽

Hannah Botfield ◽

Ryan S Ottridge ◽

Tim Matthews ◽

...

Keyword(s):

Intracranial Hypertension ◽

Lumbar Puncture ◽

Idiopathic Intracranial Hypertension ◽

Controlled Trial ◽

Hydroxysteroid Dehydrogenase ◽

Opening Pressure ◽

Double Blind ◽

Randomized Controlled

Abstract Treatment options for idiopathic intracranial hypertension are limited. The enzyme 11β-hydroxysteroid dehydrogenase type 1 has been implicated in regulating cerebrospinal fluid secretion, and its activity is associated with alterations in intracranial pressure in idiopathic intracranial hypertension. We assessed therapeutic efficacy, safety and tolerability and investigated indicators of in vivo efficacy of the 11β-hydroxysteroid dehydrogenase type 1 inhibitor AZD4017 compared with placebo in idiopathic intracranial hypertension. A multicenter, UK, 16-week phase II randomized, double-blind, placebo-controlled trial of 12-week treatment with AZD4017 or placebo was conducted. Women aged 18–55 years with active idiopathic intracranial hypertension (>25 cmH2O lumbar puncture opening pressure and active papilledema) were included. Participants received 400 mg of oral AZD4017 twice daily compared with matching placebo over 12 weeks. The outcome measures were initial efficacy, safety and tolerability. The primary clinical outcome was lumbar puncture opening pressure at 12 weeks analysed by intention-to-treat. Secondary clinical outcomes were symptoms, visual function, papilledema, headache and anthropometric measures. In vivo efficacy was evaluated in the central nervous system and systemically. A total of 31 subjects [mean age 31.2 (SD = 6.9) years and body mass index 39.2 (SD = 12.6) kg/m2] were randomized to AZD4017 (n = 17) or placebo (n = 14). At 12 weeks, lumbar puncture pressure was lower in the AZD4017 group (29.7 cmH2O) compared with placebo (31.3 cmH2O), but the difference between groups was not statistically significant (mean difference: −2.8, 95% confidence interval: −7.1 to 1.5; P = 0.2). An exploratory analysis assessing mean change in lumbar puncture pressure within each group found a significant decrease in the AZD4017 group [mean change: −4.3 cmH2O (SD = 5.7); P = 0.009] but not in the placebo group [mean change: −0.3 cmH2O (SD = 5.9); P = 0.8]. AZD4017 was safe, with no withdrawals related to adverse effects. Nine transient drug-related adverse events were reported. One serious adverse event occurred in the placebo group (deterioration requiring shunt surgery). In vivo biomarkers of 11β-hydroxysteroid dehydrogenase type 1 activity (urinary glucocorticoid metabolites, hepatic prednisolone generation, serum and cerebrospinal fluid cortisol:cortisone ratios) demonstrated significant enzyme inhibition with the reduction in serum cortisol:cortisone ratio correlating significantly with reduction in lumbar puncture pressure (P = 0.005, R = 0.70). This is the first phase II randomized controlled trial in idiopathic intracranial hypertension evaluating a novel therapeutic target. AZD4017 was safe and well tolerated and inhibited 11β-hydroxysteroid dehydrogenase type 1 activity in vivo. Reduction in serum cortisol:cortisone correlated with decreased intracranial pressure. Possible clinical benefits were noted in this small cohort. A longer, larger study would now be of interest.

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Asymptomatic idiopathic intracranial hypertension post female to male gender transition

BMJ Case Reports ◽

10.1136/bcr-2021-246454 ◽

2021 ◽

Vol 14 (12) ◽

pp. e246454

Author(s):

Geoffrey Peter Ronan ◽

Brian Sweeney

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Brain Mri ◽

Male Gender ◽

Raised Intracranial Pressure ◽

Gender Transition ◽

Mri Brain ◽

Ct Brain ◽

Bilateral Papilloedema

A 23- year-man post female to male (FTM) gender transition was found to have bilateral papilloedema at a routine optician visit. The patient was referred on for formal ophthalmological and neurological assessments. Optical coherence tomography (OCT) confirmed the presence of bilateral papilloedema. The patient was entirely asymptomatic and had no medical history. He took testosterone intramuscularly once per month. Neurological examination was otherwise normal. Investigations including routine blood panels, CT brain, MRI brain and cerebral MR venogram were all normal. Lumbar puncture yielded cerebrospinal fluid (CSF) normal in appearance but demonstrated raised intracranial pressure. In the absence of other causative aetiologies a diagnosis of idiopathic intracranial hypertension (IIH) was made. Treatment was commenced with acetazolamide and the patient was discharged with outpatient ophthalmological and neurological follow-up.

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Therapeutic lumbar puncture for headache in idiopathic intracranial hypertension: Minimal gain, is it worth the pain?

Cephalalgia ◽

10.1177/0333102418782192 ◽

2018 ◽

Vol 39 (2) ◽

pp. 245-253 ◽

Cited By ~ 14

Author(s):

Andreas Yiangou ◽

James Mitchell ◽

Keira Annie Markey ◽

William Scotton ◽

Peter Nightingale ◽

...

Keyword(s):

Intracranial Hypertension ◽

Lumbar Puncture ◽

Idiopathic Intracranial Hypertension ◽

Rating Scale ◽

Acute Effect ◽

Numeric Rating Scale ◽

Severe Exacerbation ◽

Opening Pressure ◽

Headache Severity ◽

Numeric Rating

Background Headache is disabling and prevalent in idiopathic intracranial hypertension. Therapeutic lumbar punctures may be considered to manage headache. This study evaluated the acute effect of lumbar punctures on headache severity. Additionally, the effect of lumbar puncture pressure on post-lumbar puncture headache was evaluated. Methods Active idiopathic intracranial hypertension patients were prospectively recruited to a cohort study, lumbar puncture pressure and papilloedema grade were noted. Headache severity was recorded using a numeric rating scale (NRS) 0–10, pre-lumbar puncture and following lumbar puncture at 1, 4 and 6 hours and daily for 7 days. Results Fifty two patients were recruited (mean lumbar puncture opening pressure 32 (28–37 cmCSF). At any point in the week post-lumbar puncture, headache severity improved in 71% (but a small reduction of −1.1 ± 2.6 numeric rating scale) and exacerbated in 64%, with 30% experiencing a severe exacerbation ≥ 4 numeric rating scale. Therapeutic lumbar punctures are typically considered in idiopathic intracranial hypertension patients with severe headaches (numeric rating scale ≥ 7). In this cohort, the likelihood of improvement was 92% (a modest reduction of headache pain by −3.0 ± 2.8 numeric rating scale, p = 0.012, day 7), while 33% deteriorated. Idiopathic intracranial hypertension patients with mild (numeric rating scale 1–3) or no headache (on the day of lumbar puncture, prior to lumbar puncture) had a high risk of post- lumbar puncture headache exacerbation (81% and 67% respectively). Importantly, there was no relationship between lumbar puncture opening pressure and headache response after lumbar puncture. Conclusion Following lumbar puncture, the majority of idiopathic intracranial hypertension patients experience some improvement, but the benefit is small and post-lumbar puncture headache exacerbation is common, and in some prolonged and severe. Lumbar puncture pressure does not influence the post-lumbar puncture headache.

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080 The incidence, diagnosis and outcomes of idiopathic intracranial hypertension in the southern tasmanian catchment

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2019-anzan.68 ◽

2019 ◽

Vol 90 (e7) ◽

pp. A25.3-A26

Author(s):

Natasha Krishnadas ◽

Bruce Taylor

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Incidence Rates ◽

Regional Studies ◽

Opening Pressure ◽

Exclusion Criteria ◽

Human Ethics ◽

Regional Population ◽

Australian Bureau Of Statistics ◽

The University

IntroductionThis study aimed to identify the incidence of idiopathic intracranial hypertension (IIH) in Southern Tasmania, Australia. Secondary aims were to elucidate demographics, current approaches to investigation, treatment and outcomes. To our knowledge, similar regional studies have not been performed.MethodsThe study was approved by the University of Tasmania Human Ethics Committee. Patients presented between June 2016-June 2018 to Royal Hobart Hospital, the single tertiary Neurology service in Tasmania. Cases were identified by screening lumbar punctures (LP) performed by Neurology services (inpatient, outpatient, Radiologically-assisted) and by surveying all regional Neurologists. Medical records were used to corroborate LP results and determine whether patients met Modified Dandy Criteria (MDC) (used to define IIH in current literature). Regional population statistics were obtained from the Australian Bureau of Statistics (ABS). Duplicate records were excluded. Exclusion criteria included age <18 at data collection, LP opening pressure <20cm of water or secondary causes for IIH.Results45 cases identified; 30 within the Greater Hobart region, population of 229,088 (June 2016-June 2017). Cumulative incidence was 6.55/100,000 (incidence rate 0.06/1000) with classification based on Neurologist diagnosis and 5.46/100,000 (0.05/1000) with classification according to MDC. 100% of the cohort were female. Mean age was 26.7 (range 17–45) and mean weight was 105.3kg (range 78–170). Headache was the most commonly reported symptom. 8.9% (4/45) of the total cohort were medically refractory (requiring VP/LP shunting). Ophthalmology services initiated 51% (23/45) of the referrals.ConclusionsOur incidence rates are higher than rates in previous studies for population subsets of young women.

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Cerebrospinal fluid protein and opening pressure in idiopathic intracranial hypertension (pseudotumor cerebri)

Neurology ◽

10.1212/wnl.41.7.1040 ◽

1991 ◽

Vol 41 (7) ◽

pp. 1040-1040 ◽

Cited By ~ 16

Author(s):

P. K. Johnston ◽

J. J. Corbett ◽

C. E. Maxner

Keyword(s):

Cerebrospinal Fluid ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Pseudotumor Cerebri ◽

Opening Pressure ◽

Cerebrospinal Fluid Protein

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Chiari I - Idiopathic Intracranial Hypertension Association After Failed Posterior Fossa Surgery. Case Report

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v30i3.1852 ◽

2019 ◽

Vol 30 (3) ◽

pp. 252-260

Author(s):

Felix Pastor Escartín ◽

Vicent Quilis Quesada ◽

Pau Capilla Guasch ◽

Diego Tabarés Palacín ◽

Esteban Vega Torres ◽

...

Keyword(s):

Intracranial Hypertension ◽

Lumbar Puncture ◽

Posterior Fossa ◽

Idiopathic Intracranial Hypertension ◽

Decompression Surgery ◽

Posterior Fossa Decompression ◽

Type I ◽

Posterior Fossa Surgery ◽

Opening Pressure ◽

Chiari I

Objectives: Several papers have been published relating the Idiopathic Intracranial Hypertension Syndrome (HTII) to the Arnold Chiari type I malformation (AC1M). Both entities have clinical and demographic similarities, a poorly defined etiology and, sometimes common therapeutic posibilities. A correlation between both entities has been suggested, especially in a subgroup of patients in whom posterior fossa decompression surgery fails. With regard to a case, we reviewed the literature and proposed our hypothesis about the origin of Chiari-HTII syndrome and its therapeutic possibilities. Case presentation: A 41year-old patient with mild obesity, menstrual abnormalities and empty Sella Turcicae, was operated on with an AC1M associating basilar impression and syringomyelia causing all together a centromedullary syndrome. After posterior fossa decompression surgery and successful arthrodesis, she improved in the immediate postoperative period. Nevertheless, she soon developed symptoms of intracranial hypertension (ICH), and showed increased opening pressure in lumbar puncture compatible with HTII syndrome. A ventriculoperitoneal shunt (VPS) was implanted with clinical improvement and 12 months later the syringomyelia was absent on in the magnetic resonance (MRI). Conclusion: The Chiari I-HTII syndrome is described as the coexistence of ICH symptoms after failed posterior fossa surgery, in patients with no flow MRI anomalies, and increased opening pressure at the lumbar puncture. In our experience, both entities seem to overlap in a common syndrome and must be taken into account, especially in patients with atypical onset of symptoms or patients in whom conservative treatment fails.

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The patients’ perspective on the burden of idiopathic intracranial hypertension

10.21203/rs.3.rs-542088/v1 ◽

2021 ◽

Author(s):

Max Witry ◽

Christine Kindler ◽

Johannes Weller ◽

Andreas Linder ◽

Ullrich Wüllner

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Sleep Disturbances ◽

Online Survey ◽

Cross Sectional Study ◽

Increased Intracranial Pressure ◽

Opening Pressure ◽

Cross Sectional ◽

Patients Perspective ◽

Visual Disturbances

Abstract Background: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure without evidence of a tumor or any other underlying cause. Headache and visual disturbances are frequent complaints of IIH patients, but little is known about other symptoms. In this study, we evaluated the patients’ perspective on the burden of IIH. Methods: For this cross-sectional study, we developed an online survey for patients with IIH containing standardized evaluations of headache (HIT-6), sleep (PROMIS Sleep Disturbance Scale) and depression (MDI) in relation to BMI, lumbar puncture opening pressure (LP OP) and treatment. Results: Between December 2019 and February 2020, 306 patients completed the survey. 285 (93%) were female, mean age was 36.6 years (± 10.8), mean BMI 34.2 (± 7.3) and mean LP OP at diagnosis was 37.8 cmH2O (± 9.5). 219 (72%) of the participants were obese (BMI ≥ 30); 251 (82%) reported severe impacting headaches, 140 (46%) were suffering from sleep disturbances and 169 (56%) from depression. Higher MDI scores correlated with higher BMI and increased sleep disturbances. Patients with a normalized LP opening pressure reported less headaches, less sleep disturbances and less depression than those with a constantly elevated opening pressure. Conclusion: In addition to headaches and visual disturbances, sleep disturbances and depression are frequent symptoms in IIH and contribute to the patients’ burden. Structured questionnaires can help to identify IIH patients’ needs and can lead to personalized and better treatment.

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