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

2019 ◽  
Vol 34 (1) ◽  
Author(s):  
Ali R. Hamdan ◽  
Amr M. Tayel ◽  
Eslam El Sayed El Khateeb ◽  
Osama Elsoghiar

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.

2021 ◽  
Vol 14 (5) ◽  
pp. e242455
Author(s):  
James Trayer ◽  
Declan O'Rourke ◽  
Lorraine Cassidy ◽  
Basil Elnazir

A 13-year-old male asthmatic presented to the general paediatric clinic with papilloedema identified following a check-up with his optician due to blurred vision. His asthma was well controlled on a moderate dose of inhaled corticosteroid and there had been no recent increase or decrease in the dose. A diagnosis of benign intracranial hypertension (BIH) was made based on a raised cerebrospinal fluid opening pressure, papilloedema, a normal neurological examination and normal neuroimaging. The only associated risk factor was his inhaled corticosteroids. He was commenced on acetazolamide and the inhaled corticosteroid dose was reduced, resulting in resolution of his papilloedema. This case serves to highlight that steroid side effects including BIH may occur at moderate doses of inhaled corticosteroids and that inhaled corticosteroid dose should be regularly reviewed and decreased to the lowest dose that maintains asthma control.


Cephalalgia ◽  
2011 ◽  
Vol 31 (16) ◽  
pp. 1627-1633 ◽  
Author(s):  
Alexandra J Sinclair ◽  
Sarin Kuruvath ◽  
Diptasri Sen ◽  
Peter G Nightingale ◽  
Michael A Burdon ◽  
...  

Background: The role of cerebrospinal fluid (CSF) diversion in treating idiopathic intracranial hypertension (IIH) is disputed. Method: We conducted a 10-year, retrospective case note review to evaluate the effects of CSF diversion in IIH. Symptoms, signs and details of shunt type, complications and revisions were documented at baseline, 6, 12 and 24 months post-operatively. Results: Fifty-three IIH patients were shunted [predominantly lumboperitoneal (92%)]. The most common symptom pre-surgery was headache (96%). Post-operatively, significantly fewer patients experienced declining vision and visual acuity improved at 6 ( p = 0.001) and 12 months ( p = 0.016). Headache continued in 68% at 6 months, 77% at 12 months and 79% at 2 years post-operatively. Additionally, post-operative low-pressure headache occurred in 28%. Shunt revision occurred in 51% of patients, with 30% requiring multiple revisions. Conclusion: We conclude that CSF diversion reduces visual decline and improves visual acuity. Unfortunately, headache remained in the majority of patients and low-pressure headache frequently complicated surgery. Over half of the patients required shunt revision with the majority of these requiring multiple revisions. We suggest that CSF shunting should be conducted as a last resort in those with otherwise untreatable, rapidly declining vision. Alternative treatments, such as weight reduction, may be more effective with less associated morbidity.


2018 ◽  
Vol 103 (10) ◽  
pp. 1429-1435 ◽  
Author(s):  
Jonathan A Micieli ◽  
Beau B Bruce ◽  
Caroline Vasseneix ◽  
Richard J Blanch ◽  
Damian E Berezovsky ◽  
...  

Background/aimsIt remains unclear whether the presence of optic disc haemorrhages (ODH) or cotton wool spots (CWS) at presentation in patients with papilloedema from idiopathic intracranial hypertension (IIH) has prognostic value. The aim of this study was to determine if optic disc appearance at presentation predicts visual outcome in patients with IIH.MethodsRetrospective study of 708 eyes of 360 consecutive patients with IIH who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardised manner. Visual function was assessed using Snellen converted to logMAR visual acuity, Humphrey mean deviation and visual field grade.ResultsAt least one ODH was found in 201 (28.4%) eyes, at least one CWS was found in 101 (14.3%) eyes and 88 eyes had both ODH and CWS (12.4%). At presentation, Frisén grade was associated with the presence and severity of ODH and CWS (p<0.001). ODH were associated with a worse visual acuity and CWS were associated with a worse visual field grade and mean deviation at presentation (p<0.05). Frisén grade was associated with worse visual function at presentation and final follow-up (p<0.001). Neither ODH nor CWS at presentation were associated with visual function at final follow-up when controlling for the Frisén grade.Conclusions and relevanceODH and CWS at baseline are not independent predictors of final visual function in IIH when controlling for the severity of papilloedema.


2021 ◽  
Vol 11 (3) ◽  
pp. 324
Author(s):  
Ethem Göksu ◽  
Baran Bozkurt ◽  
Deniz İlhan ◽  
Ahmet Özak ◽  
Musa Çırak ◽  
...  

Objective: To evaluate the results of bilateral endoscopic optic nerve decompression (EOND) with the opening nerve sheath (ONS) technique in patients with idiopathic intracranial hypertension (IIH). Methods: Between the years of 2017 and 2019, we retrospectively evaluated nine IIH patients with progressive visual impairment despite medical treatment and who were treated with the EOND and ONS techniques. We also demonstrated our surgical technique recipe on postmortem human heads in a stepwise manner. Results: There were 9 patients (7 females and 2 males) between the ages of 21 and 72 included in this study, and the mean age was 40.8. All patients had an impairment in visual acuity and/or their visual field, with signs of papilledema and/or optic atrophy. The patients were followed up with for 9–48 months. Improvements in visual acuity were observed in 7 out of 9 patients (78%). Visual field defects improved in 5 out of 8 patients (62.5%). Papilledema was resolved in all patients (100%). Headaches improved in all symptomatic patients (100%). No intraoperative or postoperative complications were observed. Conclusions: EOND is a safe and effective surgical procedure in selected patients with IIH. Bilateral wide bony decompression and nerve fenestration can also be an additional benefit for headache relief. Further clinical series and long-term follow-up are needed for more precise results.


2021 ◽  
Vol 9 (1) ◽  
pp. 46
Author(s):  
Anu Yarky ◽  
Vipan Kumar ◽  
Nidhi Chauhan ◽  
Priyesh Sharma

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.


2017 ◽  
Vol 27 (2) ◽  
pp. e25-e27 ◽  
Author(s):  
Alexander Voldman ◽  
Breanna Durbin ◽  
John Nguyen ◽  
Brian Ellis ◽  
Monique Leys

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.


2020 ◽  
pp. 112067212092020
Author(s):  
Angeliki Androu ◽  
Dimitrios Alonistiotis ◽  
Dimitrios Isaakidis ◽  
Konstantinos Ananikas ◽  
Panagiotis Stavrakas

Both idiopathic intracranial hypertension and intracranial cerebrospinal fluid hypotension, spontaneous or iatrogenic, display visual disturbances, including visual loss, visual field deficit, transient visual obscurations, and diplopia. We present a case of a long-standing idiopathic intracranial hypertension, primarily manifesting with visual disturbances and documented pre-existing concentric visual field deficit, treated with a lumboperitoneal shunt after conservative treatment failure, leading to an unexpected exacerbation of visual field deficit and acute visual loss, probably due to shunt catheter malfunction and subsequent intracranial hypotension. We provide a step-by-step documentation of cerebrospinal fluid volume and consequent intracranial pressure fluctuations effect on visual field alterations over a significant period of time, attempting an insight on pathogenetic mechanisms implicated in the relationship between intracranial pressure and optic nerve functionality. Interdepartmental collaboration in such cases could ensure prompt diagnosis and treatment, leading to the possibility of either halting the progression or even reversing established visual loss, sparing our patients from a lifetime disability and improving their quality of life.


Cephalalgia ◽  
2020 ◽  
Vol 40 (12) ◽  
pp. 1346-1354
Author(s):  
Dagmar Beier ◽  
Johanne Juhl Korsbæk ◽  
Jonna Skov Madsen ◽  
Dorte Aalund Olsen ◽  
Laleh Dehghani Molander ◽  
...  

Background Damage of the optic nerve is the major complication of idiopathic intracranial hypertension. A biomarker indicative for optic nerve damage would help identifying high-risk patients requiring surgical procedures. Here, we studied the potential of cerebrospinal fluid neurofilament to predict idiopathic intracranial hypertension-induced optic nerve damage. Methods In two centers, serum and cerebrospinal fluid of 61 patients with clinically suspected idiopathic intracranial hypertension were prospectively collected. Neurofilament concentrations were measured and related to ophthalmological assessment. Results The average cerebrospinal fluid neurofilament concentration in patients with moderate and severe papilledema was increased compared to patients with minor and no papilledema (1755 ± 3507 pg/ml vs. 244 ± 102 pg/ml; p < 0.001). Cerebrospinal fluid neurofilament concentrations correlated with the maximal lumbar puncture opening pressure (r = 0.67, p < 0.001). In patients fulfilling the Friedman criteria for idiopathic intracranial hypertension with or without papilledema (n = 35), development of bilateral visual field defects and bilateral atrophy of the optic nerve were associated with increased average age-adjusted cerebrospinal fluid neurofilament concentrations. At last follow-up (n = 30), 8/13 of patients with increased, but only 3/17 with normal, cerebrospinal fluid neurofilament had developed bilateral visual field defects and/or bilateral optic nerve atrophy resulting in a sensitivity of 72.7% and a specificity of 73.7% of cerebrospinal fluid neurofilament to detect permanent optic nerve damage. Conclusions Cerebrospinal fluid neurofilament is a putative biomarker for optical nerve damage in idiopathic intracranial hypertension.


2022 ◽  
Vol 48 (1) ◽  
Author(s):  
Francesco Del Monte ◽  
Laura Bucchino ◽  
Antonia Versace ◽  
Irene Tardivo ◽  
Emanuele Castagno ◽  
...  

Abstract Background Idiopathic intracranial hypertension is an infrequent condition of childhood, and is extremely rare in infants, with only 26 cases described. The etiology is still unknown. Typical clinical manifestations change with age, and symptoms are atypical in infants, thus the diagnosis could be late. This is based on increased opening pressure at lumbar puncture, papilloedema and normal cerebral MRI. The measurement of cerebrospinal fluid opening pressure in infants is an issue because many factors may affect it, and data about normal values are scanty. The mainstay of treatment is acetazolamide, which allows to relieve symptoms and to avoid permanent visual loss if promptly administered. Case presentation We report the case of an 8-month-old infant admitted because of vomit, loss of appetite and irritability; later, also bulging anterior fontanel was observed. Cerebral MRI and cerebrospinal fluid analysis resulted negative and after two lumbar punctures he experienced initial symptom relief. Once the diagnosis of idiopathic intracranial hypertension was made, he received oral acetazolamide, and corticosteroids, with progressive symptom resolution. Conclusions Infantile idiopathic intracranial hypertension is extremely rare, and not well described yet. Bulging anterior fontanel in otherwise healthy infants with normal neuroimaging should be always considered suggestive, but can be a late sign, while irritability and anorexia, especially if associated with vomiting, may represent an early sign. In such cases, lumbar puncture should be always done, hopefully with cerebrospinal fluid opening pressure measurement, which is among coded diagnostic criteria, but whose threshold is controversial in infants. Early diagnosis, timely treatment and strict follow-up help to prevent vision loss or death of affected infants.


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