scholarly journals Clinical Manifestations, Neuroimaging Findings, and Treatment of Idiopathic Intracranial Hypertension in a Nepalese Tertiary Centre

2021 ◽  
Vol 4 (2) ◽  
pp. 468-472
Author(s):  
Niraj Gautam ◽  
Rajeev Ojha ◽  
Sanjeev Kharel ◽  
Bikram Prasad Gajurel ◽  
Sanjeeta Sitaula ◽  
...  
Keyword(s):  
Visual Impairment ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Clinical Manifestations ◽  
Transverse Sinus ◽  
Empty Sella ◽  
University Teaching ◽  
Opening Pressure ◽  
Ventriculoperitoneal Shunting ◽  
Tertiary Center

Introduction: Idiopathic Intracranial Hypertension is characterized by headache, visual impairment, papilledema, and increased cerebrospinal fluid opening pressure. We aim to evaluate clinical manifestations, neuroimaging findings, and treatment of Idiopathic Intracranial Hypertension in a Nepalese tertiary center. Materials and Methods: We retrospectively included patients with the diagnosis of Idiopathic Intracranial Hypertension who were admitted to the Neurology department of Tribhuvan University Teaching Hospital from 2019 June to 2021 May and presented to the Neuro-ophthalmology outpatient clinic of the hospital for follow-up. Results: Out of 16 Idiopathic Intracranial Hypertension patients, 12(75%) patients had either headache or ocular pain.  Reduced visual acuity and progressive visual loss were found in 44% of patients. Normal CSF opening pressure was found in 19%, 44% had CSF opening pressure at a range of 20-30 cm H20 and > 30 cm H20 in 37.5%. 15 Idiopathic Intracranial Hypertension patients (93.7%) were subtyped as Typical and 1 patient was diagnosed as Fulminant Idiopathic Intracranial Hypertension. Acetazolamide was used for treatment in 15 patients and 1 patient needed ventriculoperitoneal shunting. 56% Seven patients (44%) had some abnormal findings: partial empty sella (44%), and tortuous optic nerve (31%), flattened posterior eyeball (31%), and hypoplastic transverse sinus (19%). Conclusions: Idiopathic Intracranial Hypertension is an uncommon diagnosis but should be suspected in patients with chronic headaches with visual impairment. In low-resource settings, proper history along with neurological and ophthalmological examinations can even detect the early features and timely referral can save the vision and disability of Idiopathic Intracranial Hypertension patients.

scholarly journals Infantile idiopathic intracranial hypertension: case report and review of the literature

2022 ◽  
Vol 48 (1) ◽  
Author(s):  
Francesco Del Monte ◽  
Laura Bucchino ◽  
Antonia Versace ◽  
Irene Tardivo ◽  
Emanuele Castagno ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Symptom Relief ◽  
Clinical Manifestations ◽  
Initial Symptom ◽  
Anterior Fontanel ◽  
Opening Pressure ◽  
Cerebral Mri

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.

scholarly journals Familial Idiopathic Intracranial Hypertension in Two Non-obese Chinese Sisters

2020 ◽  
Vol 11 ◽  
Author(s):  
Lei Qiao ◽  
Yanping Wei
Keyword(s):  
Magnetic Resonance Imaging ◽  
Visual Impairment ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Clinical Course ◽  
Rare Condition ◽  
Empty Sella ◽  
Resonance Imaging ◽  
Magnetic Resonance Imaging Mri ◽  
The Brain

Background: Familial idiopathic intracranial hypertension (FIIH) is a rare condition, the etiology of which is unclear.Aims: To describe two non-obese Chinese sisters who met the criteria of FIIH and to analyze the clinical features and prognosis of FIIH.Methods: The clinical course, treatment, and prognosis of these two patients were analyzed retrospectively. Meanwhile, all the literature of familial IIH (FIIH) was reviewed.Results: These two sisters presented with headaches and visual impairment in their mid-thirties. Magnetic resonance imaging (MRI) of the brain was unremarkable except for partial empty sella. No comorbidities or defined causes were detected. Headaches were partially relieved by dehydrated medicine, whereas the visual impairment persisted.Conclusion: In cases where patients present with headaches, empty sella are found on an MRI, and there is visual impairment with or without papilla edema, intracranial hypertension should be excluded. Furthermore, we should pay more attention to the relatives of those patients with increased intracranial hypertension.

scholarly journals Intracranial Venous Sinus Stenting in Idiopathic Intracranial Hypertension: A Case Report and Review of the Literature

2021 ◽  
Vol 11 (3) ◽  
pp. 382
Author(s):  
Dinesh Ramanathan ◽  
Zachary D. Travis ◽  
Emmanuel Omosor ◽  
Taylor Wilson ◽  
Nikhil Sahasrabudhe ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Transverse Sinus ◽  
Venous Sinus ◽  
Subdural Hygroma ◽  
Presenting Symptoms ◽  
Minimal Improvement ◽  
Sinus Stenosis ◽  
Work Up

We describe a case of severe headaches, double vision, and progressive vision loss secondary to a ruptured intracranial cyst (IAC) in a 31-year-old woman with no relevant past medical history. The case is peculiar because drainage of the subdural hygroma led to a minimal improvement in vision with persistent elevated intracranial pressure (ICP). Further exploration revealed transverse sinus stenosis necessitating stenting. Evaluation post-stenting showed marked reduction of ICP and improvement in symptoms. This report underscores the importance of comprehensive work-up and suspicion of multiple underlying etiologies that may be crucial to complete resolution of presenting symptoms in some cases. We provide an overview of the clinical indications and evidence for venous sinus stenting in treating idiopathic intracranial hypertension (IIH).

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

2020 ◽  
Vol 2 (1) ◽  
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.

scholarly journals Intracranial hypertension due to spinal cord tumor misdiagnosed as pseudotumor cerebri syndrome: case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Wanglu Hu ◽  
Chun Wang ◽  
Qun Wu ◽  
Yike Chen ◽  
Wei Gao ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Spinal Cord Tumor ◽  
Tumor Resection ◽  
Pseudotumor Cerebri ◽  
Transverse Sinus ◽  
Pathologic Evaluation ◽  
Spinal Imaging ◽  
One Year

Abstract Background Isolated onset of intracranial hypertension due to spinal cord tumor is rare, thus, easily leading to misdiagnosis and delay in effective treatment. Case presentation Herein, we describe a 45-year-old female patient who manifested isolated symptoms and signs of intracranial hypertension and whose condition was initially diagnosed as idiopathic intracranial hypertension and transverse sinus stenosis. The patient received a stent implantation; however, no improvements were observed. One year later her symptoms exacerbated, and during rehospitalization a spinal imaging examination revealed a lumbar tumor. Pathologic evaluation confirmed schwannoma, and tumor resection significantly improved her symptoms, except for poor vision. Conclusions Space-occupying lesions of the spine should be considered in the differential diagnosis of idiopathic intracranial hypertension, even in the absence of spine-localized signs or symptoms.

STEREOTACTIC VENTRICULOPERITONEAL SHUNTING FOR REFRACTORY IDIOPATHIC INTRACRANIAL HYPERTENSION

Neurosurgery ◽  
2007 ◽  
Vol 60 (6) ◽  
pp. 1039-1044 ◽  
Author(s):  
Basel Abu-Serieh ◽  
Keyvan Ghassempour ◽  
Thierry Duprez ◽  
Christian Raftopoulos
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Long Term Results ◽  
Ventricular Catheter ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Visual Deficits ◽  
Ventriculoperitoneal Shunting

Abstract OBJECTIVE Recent reports have shown promising short- to medium-term results in patients with refractory idiopathic intracranial hypertension (IIH) treated using the stereotactic ventriculoperitoneal shunting (SVPS) technique. However, the long-term clinical efficacy of this technique remains questionable. This report provides the long-term results of SVPS in treating refractory IIH patients. METHODS We reviewed the medical charts of nine consecutive patients (mean age, 26.4 yr; range, 4–63 yr) treated using either a frame-based or frameless SVPS technique for IIH. RESULTS The mean postoperative follow-up period was 44.3 months (range, 6–110 mo). Before shunting procedures were performed, each patient presented with intractable headache, and five patients (55.6%) had mild to moderate visual deficits. The last follow-up assessment showed that after shunting was performed, eight patients (89%) were headache-free. Only one patient had recurrent headache; however, this patient's pain was much less frequent and severe than before the shunting procedure was completed and was concomitant with recent weight increase. Visual deficits were resolved in three patients and remained stable in two who already had optic nerve atrophy before shunting was completed. Twelve SVPS procedures were performed on our patients. Nine shunt revisions were needed in six patients because of infection (n = 5, including two revisions in one patient), valve dysfunction (n = 2), distal obstruction (n = 1), and ventricular catheter malpositioning (n = 1). No patient had proximal catheter obstruction. CONCLUSION Given the favorable long-term outcome of the SVPS technique for refractory IIH, we are encouraged to apply this procedure on our patients. More invasive approaches should be reserved for patients who have SVPS failure.

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

Cephalalgia ◽  
2018 ◽  
Vol 39 (2) ◽  
pp. 245-253 ◽  
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.

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

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.

Pattern of pressure gradient alterations after venous sinus stenting for idiopathic intracranial hypertension predicts stent-adjacent stenosis: a proposed classification system

2017 ◽  
Vol 10 (4) ◽  
pp. 391-395 ◽  
Author(s):  
Daniel Raper ◽  
Thomas J Buell ◽  
Dale Ding ◽  
Ching-Jen Chen ◽  
Robert M Starke ◽  
...  
Keyword(s):  
Pressure Gradient ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Venous Pressure ◽  
Patient Characteristics ◽  
Transverse Sinus ◽  
Venous Sinus ◽  
Type I ◽  
Type Ii ◽  
Type Iii

ObjectiveVenous sinus stenting (VSS) is a safe and effective treatment for idiopathic intracranial hypertension (IIH) with angiographic venous sinus stenosis. However, predictors of stent-adjacent stenosis (SAS) remain poorly defined.MethodsWe performed a retrospective review of 47 patients with IIH and intracranial venous stenosis who underwent VSS with pre- and post-stent venography. Patient characteristics, treatments and outcomes were reviewed. Changes in pressure gradient after VSS were classified according to pattern of gradient resolution into types I–III.ResultsType I gradient resolution, in which mean venous pressure (MVP) in the transverse sinus (TS) decreases towards MVP in the sigmoid sinus (SS), occurred in 18 patients (38.3%). Type II gradient resolution pattern, in which SS MVP increases towards that in the TS, occurred in 7patients (14.9%). Type III pattern, in which MVP equilibrates to a middle value, occurred in 22patients (46.8%). SAS occurred in 0%, 28.6%, and 22.7% of patients in types I, II and III, respectively. Compared with patients with type I gradient resolution, SAS was more common in those with type II (p=0.0181) and type III (p=0.0306) patterns.ConclusionsThe pattern of change in the trans-stenosis venous pressure gradient may be predictive of SAS and is a useful tool for classifying the response of the venous obstruction to stenting. A type I pattern appears to represent the ideal response to VSS. Some patients with type II and III changes, particularly if they have other predictors of recurrent stenosis, may benefit from longer initial stent constructs.

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