Idiopathic intracranial hypertension and visual function in North Indian population

2021 ◽  
pp. 112067212110065
Author(s):  
Obaidur Rehman ◽  
Parul Ichhpujani ◽  
Suresh Kumar ◽  
Richa Saroa ◽  
Nishit Sawal

Background: Idiopathic Intracranial Hypertension (IIH) is a neurological disorder with varied presentation, visual morbidity being the most important one. Literature is sparse as regards the effects of IIH on the visual system in Asians. Objective: Assessment of visual morbidity and change with treatment in IIH patients in North India through a prospective interventional study. Materials and methods: Sixty eyes of newly diagnosed IIH patients were evaluated for functional and structural tests of visual system. Lumbar puncture was performed to establish IIH diagnosis and received oral Carbonic anhydrase inhibitor thereafter. Change in visual function was studied from baseline to 6-month follow-up visit. Results: Mean age of participants at presentation was 33.27 ± 10.68 years and majority were females (73.3%). Seventy percent eyes ( n = 42) had visual acuity 20/20 at presentation. Average Pelli Robson contrast sensitivity (CS) improved from 1.86 ± 0.34 to 2.11 ± 0.24 (Friedman Test: X2 = 59.2, p ⩽ 0.001) while number of eyes with detectable visual field deficits reduced from 70% to 43.7%. Retinal nerve fibre layer (RNFL) thickness reduced from 176.27 to 114.97 µ (Friedman Test: X2 = 69.3, p ⩽ 0.001). Conclusions: The socio-demographic profile in our study showed higher male incidence than noted in previous studies. Visual function deficits were present in patients, even in those with 20/20 visual acuity. Significant improvement was seen after lumbar puncture and initiation of medical treatment. CS and RNFL thickness showed significant improvement even after 1 month of treatment and can be used for monitoring IIH patients.

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.


2020 ◽  
pp. 088307382097223
Author(s):  
Erin Yamamoto ◽  
Darren Farber ◽  
David Rothner ◽  
Manikum Moodley

Pseudotumor cerebri also known as idiopathic intracranial hypertension is a relatively uncommon disorder of unknown pathophysiology. Although pseudotumor cerebri occurs in both children and adults, the pseudotumor cerebri literature is heavily dominated by adult studies. The aim of this study is to retrospectively describe the clinical presentation, imaging, treatment, and outcomes of a large pediatric pseudotumor cerebri population over a 23-year period. We also discuss secondary pseudotumor cerebri (44%) as well as the increasingly recognized patient subgroups without headache (13.3%) and without papilledema (7.3%). Female sex, obesity, and initial symptoms were consistent with the literature; however radiographic findings were surprisingly low in this cohort. Headache outcomes at 1 week, 1 month, and 3 months following initial lumbar puncture/treatment and visual function outcomes are reported.


2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Priscilla Fernandes Nogueira ◽  
Gustavo Coelho Caiado ◽  
Carolina P. B. Gracitelli ◽  
Fernando Meister Martins ◽  
Felipe Chaves Duarte Barros ◽  
...  

Purpose. To correlate optical coherence tomography (OCT) measurements with clinical parameters in idiopathic intracranial hypertension (IIH). Methods. A cross-sectional study was conducted with 22 patients with IIH and 11 controls. All participants underwent comprehensive ophthalmological examination followed by spectral-domain OCT (SD-OCT) and standard automated perimetry using the 30–2 program of the Humphrey visual field analyzer. Correlations between ganglion cell complex (GCC) thickness and retinal nerve fiber layer (RNFL) thickness, as measured by SD-OCT, and clinical parameters were assessed using generalized estimating equations. Result. The mean age of the participants was 35.0 ± 10.83 years. The groups were similar regarding age, but were significantly different regarding sex and visual acuity ( p = 0.001 and p = 0.038 , respectively). The GCC was significantly thinner in the IIH group, with a mean of 90.535 ± 9.766 μm compared to 98.119 ± 6.988 μm for the controls ( p = 0.023 ). There was a significant association between GCC thickness and optic disc pallor ( p = 0.016 ) and between edema and visual acuity ( p = 0.037 ). No significant difference was found in RNFL thickness between patients and controls. Conclusion. The GCC was thinner in the patients with IIH compared to the controls, and there was an association between GCC and optic disc pallor. This might suggest a role for OCT parameters when the structural changes that occur in IIH are investigated, possibly guiding clinical decision making.


Author(s):  
Mahmoud M. Allam ◽  
Hatem A. Almasry ◽  
Sandra M. Ahmed ◽  
Youssuf G. Taha ◽  
Mohammed I. Oraby

Abstract Background Idiopathic intracranial hypertension (IIH) is a disorder of increased intracranial pressure without a clear cause and can have serious visual effects. Previous research work suggests that transcranial Doppler measurements of pulsatility index correlate accurately with elevated intracranial pressure. Objective To assess the cerebrovascular hemodynamic changes in patients with IIH using transcranial Doppler before and after lumbar puncture and CSF withdrawal. Methods An interventional study conducted on 40 patients (31 females and 9 males) fulfilling the modified Dandy criteria for diagnosis of idiopathic intracranial hypertension, MRI brain, and MRV was done to the patients. Lumbar puncture was done for all included patients to measure intracranial pressure and CSF withdrawal. Transcranial Doppler was performed for all included before and after lumbar puncture and CSF withdrawal and the following parameters were measured: peak systolic velocity (PSV), end diastolic velocity (EDV), resistivity index (RI), and pulsatility index (PI). Results Significant relation was found between grades of papilledema and PSV, RI, and PI (p value 0.012, 0.025, 0.016) but no significant relation was found between grades of papilledema and EDV (0.102). Significant changes occurred in parameters of TCD pre- and post-CSF withdrawal including PSV, EDV, and PI (p value 0.001, 0.015, 0.019) denoting a significant change in cerebral hemodynamics after CSF withdrawal which denotes a decrease in intracranial pressure. Conclusion Increased intracranial pressure significantly affects cerebral blood flow. A normalization of transcranial Doppler parameters occurs following lowering of intracranial pressure through lumbar puncture and CSF withdrawal.


2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Keira Markey ◽  
James Mitchell ◽  
Hannah Botfield ◽  
Ryan S Ottridge ◽  
Tim Matthews ◽  
...  

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 (&gt;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.


Author(s):  
Gad Dotan ◽  
Natalie Hadar Cohen ◽  
Hanya M. Qureshi ◽  
Mika Shapira Rootman ◽  
Yoram Nevo ◽  
...  

OBJECTIVE Pediatric idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure despite normal cerebrospinal fluid and neuroimaging findings. Initial management is typically medical; however, nearly 10% of children will eventually require surgery for persistent headache and/or vision loss. External lumbar drainage, which is a considerably safer treatment option, has not been adequately analyzed in children with medically refractory IIH. METHODS The authors conducted a single-institution retrospective analysis of children with medically refractory IIH who had undergone external lumbar drain (ELD) placement because of worsening papilledema, reflected as increased retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT) testing. The main outcome measures were effects of external lumbar drainage on papilledema resolution, symptoms, and vision. RESULTS The authors analyzed the medical records of 13 children with IIH (11 girls, mean age 15.0 ± 2.3 years) whose mean CSF opening pressure was 45.5 ± 6.8 cm H2O. In all children, the average global RNFL thickness in both eyes significantly increased at ELD placement (right eye 371.8 ± 150.2 μm, left eye 400.3 ± 96.9 μm) compared with presentation thickness (right eye 301.6 ± 110.40 μm, left eye 350.2 ± 107.7 μm) despite acetazolamide medical therapy (20–30 mg/kg/day), leading to ELD placement after 9.5 ± 6.9 days (range 3–29 days). After ELD insertion, there was headache resolution, gradual and continuous improvement in optic disc thickness, and preservation of good vision. CONCLUSIONS ELD placement in children with medically refractory IIH who demonstrated worsening papilledema with increased RNFL thickening on OCT testing typically results in symptom relief and disc edema resolution with good visual outcome, often preventing the need for additional definitive surgeries that carry greater failure and morbidity risks.


2020 ◽  
Vol 13 (5) ◽  
pp. e234741
Author(s):  
Ayman Alboudi ◽  
Emily C Johnson

Idiopathic intracranial hypertension typically presents with holocephalic headache associated with nausea, vomiting and bilateral papilledema. Involvement of the sixth cranial nerve is relatively common. The involvement of other cranial nerves, however, is rare in this disorder. We describe a patient with idiopathic intracranial hypertension who presented with episodic unilateral retro-orbital pain and multiple cranial nerve abnormalities without papilledema. Imaging studies excluded alternate diagnoses, and the immediate resolution of symptoms after lumbar puncture confirmed that these symptoms were due to intracranial hypertension. Atypical presentations of such a disabling yet treatable disorder is very important to recognise and address.


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