Management Of Idiopathic Intracranial Hypertension Research Articles

Idiopathic intracranial hypertension; research progress and emerging themes

Idiopathic intracranial hypertension (IIH) is a condition characterised by increased intracranial pressure of unknown cause predominantly seen in obese women of childbearing age and associated with a history of recent weight gain. The aetiology is poorly understood and there are no evidence-based guidelines on the management of the disease. We aim to provide a review of the recent literature outlining the latest advances in this field over the past few years. Areas of emerging interest related to the pathophysiology of IIH will be discussed, such as the role of obesity, adipose tissue and 11β-hydroxysteroid dehydrogenase type 1. We consider the latest research on the role of venous sinus stenosis in IIH and ex vivo advances into cerebrospinal fluid drainage via the arachnoid granulation tissue. The latest techniques for optic nerve head evaluation and the role of optical coherence tomography will be summarised. Finally, we will discuss recent advances in the management of IIH, including weight loss, and medical and surgical treatment strategies.

Long-Term Results of Optic Nerve Sheath Fenestration for Idiopathic Intracranial Hypertension: Earlier Intervention Favours Improved Outcomes

The role of optic nerve sheath fenestration (ONSF) in the management of idiopathic intracranial hypertension remains controversial, with indications, risks, and benefits compared to cerebro-spinal fluid diversion procedures not fully elucidated. We report a retrospective record review of 37 patients (50 eyes) which had undergone ONSF by a single surgeon. Visual acuity (VA) improved in 22% of operated eyes and 17% of fellow eyes; stabilized in 54% of operated and 74% of fellow eyes; and deteriorated in 24% of operated and 9% of fellow eyes. Better pre-operative VA (p = 0.01), colour vision (p = 0.002), and earlier intervention (p = 0.04) were associated with stabilization. We conclude that ONSF often stabilizes vision and visual fields. Our results were best in patients with better pre-operative vision and in those with earlier intervention.

The Girl With the Headache and Double Vision

Idiopathic intracranial hypertension (IIH), previously referred to as pseudotumor cerebri is a disorder that is characterized by increased intracranial pressure caused by buildup or poor absorption of cerebrospinal fluid (CSF) without a space-occupying intracranial lesion or hydrocephalus. We report a case of a 17 year old female who presented to her primary care physician (PCP) for evaluation of a headache. Upon performance of computed tomography (CT scan) of the head and magnetic resonance imaging (MRI) of the brain: no evidence of mass lesion or abnormal enhancement in the brain parenchyma, neither evidences of acute intracranial pathology was present. Lumbar puncture (LP) was done resulting in improvement of the patient’s symptoms and decreased intensity of headache. We will discuss the management of idiopathic intracranial hypertension (IIH) with emphasis on the different etiologies and new diagnostic criteria for this disease. J Med Cases. 2013;4(3):121-124 doi: https://doi.org/10.4021/jmc913w

Visual Loss without Headache in Children with Pseudotumor Cerebri and Growth Hormone Treatment

We report on two prepubescent girls with visual loss due to idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, both treated with recombinant human growth hormone for growth failure. The interval from starting hormone therapy to diagnosis of IIH was 3 and 18 months, respectively. Both girls did not complain of headache and nausea. They were neither obese nor did they suffer from renal insufficiency. In both patients, we observed bilateral optic disc edema with visual loss and elevated cerebrospinal fluid (CSF) pressures. Other causes of IIH were excluded with neuroimaging and CSF examination. Cessation of drug administration is often sufficient for symptom resolution in cases of hormone therapy-associated IIH. However, visual field defects in one girl remained unchanged during follow-up of 8 months. In children with IIH, the spectrum of neurologic and visual manifestations might be variable and unspecific. Diagnosis and management of IIH can be difficult in the absence of headache. Blurred or double vision due to cranial nerve palsy might be the only symptom rather than complaints about reduced visual acuity. Therefore, regular clinical monitoring of visual function and fundus appearance is essential for early diagnosis, efficient management, and improvement of visual outcome in children receiving recombinant human growth hormone.

The Girl With the Headache and Double Vision

Idiopathic intracranial hypertension (IIH), previously referred to as pseudotumor cerebri is a disorder that is characterized by increased intracranial pressure caused by buildup or poor absorption of cerebrospinal fluid (CSF) without a space-occupying intracranial lesion or hydrocephalus. We report a case of a 17 year old female who presented to her primary care physician (PCP) for evaluation of a headache. Upon performance of computed tomography ( CT scan) of the head and magnetic resonance imaging ( MRI) of the brain: no evidence of mass lesion or abnormal enhancement in the brain parenchyma, neither evidences of acute intracranial pathology was present. Lumbar puncture (LP) was done resulting in improvement of the patient’s symptoms and decreased intensity of headache. We will discuss the management of idiopathic intracranial hypertension (IIH) with emphasis on the different etiologies and new diagnostic criteria for this disease. doi: http://dx.doi.org/10.4021/jmc913w

Management of Idiopathic Intracranial Hypertension in Parturients

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Pathophysiology of Idiopathic Intracranial Hypertension

Investigators at Emory University, Atlanta, GA review the epidemiology, pathophysiology and management of idiopathic intracranial hypertension (IIH), sometimes called pseudotumor cerebri or benign intracranial hypertension, terms now considered inappropriate.

Traitement et pronostic de l’hypertension intracrânienne idiopathique de l’enfant. Étude rétrospective (1995–2009) et revue de la littérature

Idiopathic intracranial hypertension (IIH) may cause severe visual loss due to the optic nerve damage. Routine management involves mainly medical treatment. The aim of this study was to improve diagnosis and management of IIH in children. The medical records of all patients with definite IIH seen at the children's hospital of Toulouse between 1995 and 2009 were reviewed. Cases of secondary intracranial hypertension were included because they did not present any cerebral lesions and underwent a similar therapeutic approach. The clinical and ophthalmological data at the beginning and at the end of their treatment was collected. Eighteen children were included in this study. The average age was 10 years and the sex-ratio was equal to 1. There were 3 cases of secondary idiopathic intracranial hypertension in this pediatric group. The main features encountered were headache (15 children) and diplopia (8 children). Abnormal neurological examination was found for 11 patients with abducens nerve paresis in 8 cases, rachialgia in 6 cases, and neurogenic pains (neuralgia, dysesthesia, paresthesia, hyperesthesia) in the other cases. Papilledema was noted in 16 patients. At the initial phase, loss of visual acuity was documented in 6 patients and altered visual field in nine patients. All patients had a medical treatment. When recurrence occurred, each new treatment was documented, for a total of 23 treatments analyzed. Lumbar puncture was the only treatment for 2 patients. In 16 cases, first-line treatment was acetazolamide and it was the second choice in 1 case, with an average dosage of 11.2mg/kg and a mean duration of 2.5 months (15 treatments could be analyzed). This treatment was effective in 11 cases out of 15. Steroids were the initial treatment in 4 cases and second-line treatment in 4 cases (after failed acetazolamide therapy). The dosage was 1.5-2mg/kg for a mean duration of 1.5 months (6 treatments could be analyzed). This treatment was effective in 5 patients out of 6. One patient had dual therapy. No surgical procedure was necessary in this pediatric cohort. Three patients presented relapses of IIH. The outcome was good with no residual visual impairment in the 13 patients analyzed. One patient was still under medication. Therapeutic management of IIH in a pediatric population is essentially medical, in some cases limited to lumbar puncture. The first-line treatment is acetazolamide, but this study shows that low doses and short duration are usually chosen. Doses must be increased and treatment prolonged to avoid the use of corticosteroids as a second-line treatment and prevent possible relapses that require close monitoring of visual function. The visual prognosis is generally better for this age group compared to adults and no risk factors for visual sequelae were identified. A standardized protocol for management of IIH was proposed.

Short-Term Continuous Intraparenchymal Intracranial Pressure Monitoring in Presumed Idiopathic Intracranial Hypertension

The management of idiopathic intracranial hypertension (IIH) depends on a reliable assessment of intracranial pressure (ICP), particularly when visual function measures or ophthalmoscopic indicators are confusing and when invasive surgical procedures are being considered. Although ICP monitoring has been widely applied in many neurologic conditions as a more reliable measure of ongoing ICP than lumbar puncture (LP), it has not often been widely used in the management of IIH. We searched the records of the University of Michigan between 2001 and 2008 for patients with IIH who had undergone LP and continuous ICP monitoring with an intraparenchymal Codman ICP Monitoring System and in whom at least 1 year of follow-up information was available. Ten patients met entry criteria. There were no complications from the ICP monitoring. ICP monitoring influenced management in all 10 patients. In 8 patients, LP had shown elevated opening pressures; in 7 of them, ICP monitoring failed to confirm a consistently high ICP. In these patients, the decision to withdraw ICP-lowering agents or shunts, or not to revise indwelling shunts, produced no change in visual function or optic disc appearance over a follow-up period of at least 1 year. In 1 patient, ICP monitoring confirmed the high ICP suggested by LP, justifying placement of a ventriculoperitoneal shunt. In 1 patient, ICP monitoring was performed instead of LP because a petroclival mass posed a danger to the performance of LP; a shunt was subsequently placed due to elevated ICP. In providing more accurate information about ICP than about LP, short-term continuous ICP intraparenchymal monitoring may be a useful adjunct in the management of IIH when clinical data are confusing and invasive interventions are under consideration.

Management of idiopathic intracranial hypertension in parturients: anesthetic considerations

Idiopathic intracranial hypertension (IIH) is a rare condition characterized by raised intracranial pressure (ICP) without related pathology in either the brain or the composition of cerebrospinal fluid (CSF). Herein, we provide a brief review of the clinical presentation of IIH and the anesthetic considerations in parturients diagnosed with the disorder. We conducted a MEDLINE® literature search for all types of articles published in English with restriction for year of publication, and we used the search terms "idiopathic intracranial hypertension", "pseudotumor cerebri", "benign intracranial hypertension", "pregnancy", "cesarean section", "labour analgesia", "epidural", and "anesthesia". Idiopathic intracranial hypertension affects primarily obese women of childbearing age. The main symptom is headache, and the cardinal sign is papilledema. The main goal of management is to preserve visual function. Treatment lies in the administration of diuretics and corticosteroids, control of excessive weight gain, and surgical management, such as cerebrospinal fluid diversion or optic nerve sheath fenestration for refractory cases. For the parturient with IIH, Cesarean delivery is not necessarily indicated. Neuraxial anesthesia has been used uneventfully for both labour analgesia and for Cesarean delivery. There are reports describing successful use of both spinal and epidural anesthesia, even in IIH patients with CSF diversion devices in situ. Although IIH is rare, there are special considerations for anesthetic management in the parturient. Despite the presence of raised ICP in these patients, there are no specific contraindications to neuraxial techniques, and uncal herniation has not been reported to occur in patients with IIH.

Idiopathic Intracranial Hypertension in Children: A Review and Algorithm

This updated review of pediatric idiopathic intracranial hypertension focuses on epidemiology, clinical presentations, diagnostic criteria, evaluation, clinical course, and treatment. General guidelines for the clinical management of idiopathic intracranial hypertension are discussed. A new algorithm outlines an efficient management strategy for the initial diagnostic evaluation of children with signs or symptoms of intracranial hypertension. This algorithm provides a systematic approach to initial evaluation and management, and identifies important decision-making factors. The risk of permanent visual loss with idiopathic intracranial hypertension necessitates a prompt, thorough collaborative approach in the management of patients. Although idiopathic intracranial hypertension has been recognized for over a century, the need remains for prospectively collected data to promote a better understanding of the etiology, risk factors, evaluative methods, and effective treatments for children with this syndrome.

PO30-FR-03 Management of idiopathic intracranial hypertension: a Tunisian experience

PO30-FR-03 Management of idiopathic intracranial hypertension: a Tunisian experience

The 18F-FDG PET Cingulate Island Sign and Comparison to 123I-β-CIT SPECT for Diagnosis of Dementia with Lewy Bodies

Neuroimaging is increasingly used to supplement the clinical diagnosis of dementia with Lewy bodies (DLB) by showing reduced occipital metabolism and perfusion and reduced striatal dopaminergic innervation. We aimed to optimize the interpretation of (18)F-FDG PET images for differentiating DLB from Alzheimer disease (AD) and to compare the results with dopamine transporter imaging using (123)I-beta-carbomethoxy-3ss-(4-iodophenyl)tropane ((123)I-beta-CIT) SPECT. Fourteen subjects with a clinical diagnosis of DLB and 10 with AD underwent both (18)F-FDG PET and (123)I-beta-CIT SPECT. Four DLB and 1 AD diagnoses were subsequently confirmed at autopsy. Diagnostic accuracy was calculated for visual interpretation by 3 readers of standard 3-plane and stereotactic surface projection (18)F-FDG PET images, receiver-operating-characteristic analysis of regional (18)F-FDG uptake, and a cutoff value for the striatal-to-occipital binding ratio of beta-CIT defined by receiver-operating-characteristic analysis. Visual interpretation of 3-plane (18)F-FDG PET images had a sensitivity of 83% and specificity of 93% for DLB, slightly higher than the results with the stereotactic surface projection images. Regionally, hypometabolism in the lateral occipital cortex had the highest sensitivity (88%), but relative preservation of the mid or posterior cingulate gyrus (cingulate island sign) had the highest specificity (100%). Region-of-interest analysis revealed that occipital hypometabolism and relative preservation of the posterior cingulate both had a sensitivity of 77% and specificity of 80%. beta-CIT achieved 100% accuracy and greater effect size than did (18)F-FDG PET (Cohen d = 4.1 vs. 1.9). Both (18)F-FDG PET and (123)I-beta-CIT SPECT appear useful for the diagnosis of DLB, although the latter provides more robust results. The cingulate island sign may enhance the specificity of (18)F-FDG PET.

Idiopathic intracranial hypertension (pseudotumor cerebri)

To review the current standard of care in the diagnosis and treatment of idiopathic intracranial hypertension (IIH). We also discuss recent advances in the understanding of the pathogenesis of IIH with emerging trends in management of this syndrome. There has been increasing literature suggesting a role of vitamin A, adipokines, sleep disorders, and venous sinus stenosis in the pathogenesis of IIH. Newer medical treatment options like topiramate and surgical treatment options like stereotactic ventriculoperitoneal shunting have been found to be attractive alternatives. There has also been an emerging interest in cerebral venous sinus stenting, though its role and utility remain debatable. This article seeks to present a systematic approach to the management of IIH. Many newer treatment modalities are being explored for IIH refractory to standard medical therapy, but their efficacy and safety must be demonstrated in large studies before they can be adopted as part of standard treatment.

Idiopathic Intracranial Hypertension in a Child After Hemodialysis

Idiopathic intracranial hypertension remains a disease of unknown etiology. Epidemiology, associations, features, and prognosis in children have changed over the years. The main clinical complaint of pediatric idiopathic intracranial hypertension is headache with visual disturbance. We report on a patient with childhood idiopathic intracranial hypertension, who presented without headache after hemodialysis for acute renal failure. Idiopathic intracranial hypertension without headache is believed to have a poor prognosis. However, this child demonstrated a favorable outcome with medical therapy. The child was followed with serial optical coherence tomography of retinal nerve fiber layer thickness around the optic nerve head. In the absence of a reliable procedure to evaluate the therapeutic efficacy in the follow-up of patients with idiopathic intracranial hypertension, the importance of optical coherence tomography, a noninvasive, objective, and reproducible procedure, is highlighted as a useful adjunct in the management of idiopathic intracranial hypertension.

O.096 A novel impedance sensor to monitor and control ventricular size

To evaluate the clinical outcomes and complications rate among idiopathic intracranial hypertension (IIH) patients who underwent lumboperitoneal (LP) shunt insertion with a programmable Strata valve.We retrospectively evaluated patients who underwent LP shunt with a programmable Strata valve insertion at the University of Ottawa Civic Hospital from November 2012 to June 2013. The demographic data, clinical symptoms, opening pressure, pre-operative and post-operative visual fields, neuroimaging, visual acuity, disc status, and complications were recorded and analyzed.Seven female patients with IIH underwent insertion of an LP shunt with a programmable Strata valve. The mean opening pressure was 35.8 cm H2O. The initial valve setting was 1.5, and four patients required post-operative valve pressure adjustment. All patients showed significant improvement in objective visual testing at follow-up as well as less frequent headaches. None of the patients developed intra- or post-operative complications.LP shunts with programmable Strata valve systems are a potential alternative to conventional LP and programmable ventriculoperitoneal shunt systems as well as optic nerve sheath fenestration, due to their potential in avoiding brain injury, lower failure and complication rates, lower intracranial hypotension incidence, and flexibility in adjusting valve pressure settings post-operatively evading under- and overdrainage complications. They should be considered for the management of IIH instead of early design LP systems and VP shunts. A randomized multi-center trial should be conducted to compare the efficacy of these surgical techniques.

Acetazolamide and Symptoms Due to Low Intracranial Pressure in Idiopathic Intracranial Hypertension

This report is aimed at highlighting an important issue which may arise during the management of Idiopathic Intracranial Hypertension (IIH). Once the diagnosis of IIH has been confirmed by a combination of intracranial imaging and an elevated opening pressure at lumbar puncture, routine management involves the use of oral acetazolamide therapy. There is, however, a risk of a headache of high intracranial pressure being converted to one resulting from low intracranial pressure. This needs to be considered when reviewing these patients immediately following any diagnostic or therapeutic lumbar puncture (LP) that is performed, specifically ascertaining whether change in posture modulates the headache. Continuing acetazolamide in the presence of a low pressure headache can cause significant morbidity and should be avoided. Furthermore chronic acetazolamide therapy can induce low pressure symptoms. We describe four cases of IIH who have suffered as a result of over-treatment with acetazolamide.

Visual outcomes comparing surgical techniques for management of severe idiopathic intracranial hypertension

The object of this study was to compare surgical techniques for management of visual loss in idiopathic intracranial hypertension (IIH) unresponsive to medical treatment. The published literature was reviewed using electronic and manual search techniques. Articles were categorized based upon the surgical procedure performed, including optic nerve sheath decompression (ONSD), also called optic nerve sheath fenestration; intracranial venous sinus stent placement; ventriculoperitoneal (VP) shunt placement; and lumboperitoneal (LP) shunt placement. Demographic data and visual outcome of the cumulative experience were compiled and compared for each type of surgical intervention. Seventeen patients treated by stent placement, 31 by VP shunt placement, 44 by LP shunt placement, and 252 patients by ONSD were identified in the literature; average follow-up ranged from 11.8 months for patients treated with stents to 57.2 months for those treated with LP shunts. Improved or resolved vision deficit was noted in 38.7% of patients after VP shunt placement, 47% of patients after stent placement, 44.6% of patients after LP shunt placement, and 80% of eyes after ONSD. Visual worsening was rare for all procedures evaluated. There is a paucity of information regarding visual outcomes from intracranial venous stent placement and cerebrospinal fluid diversion procedures. Visual outcomes from ONSD are better documented and appear to be superior to other surgical techniques for management of IIH. Further studies with improved data collection regarding visual outcomes are needed for surgical procedures other than ONSD in order to assess their possible value as treatments for visual loss in IIH.

Endoscopic Endonasal Management of Pseudotumor Cerebri: Is it Effective?

To study the efficacy and safety of endoscopic endonasal optic nerve fenestration for the management of idiopathic intracranial hypertension (IIH). A prospective study at a tertiary care center. All patients with a final diagnosis of IIH from July 2001 to March 2005 were included and subjected to detailed neuro-ophthalmologic examination and endoscopic endonasal optic nerve fenestration. Postoperative visual acuity and the perimetry was compared with the preoperative status, and the results were analyzed using the chi2 test. : Of the 18 patients included in the study, 17 had improvement in vision postoperatively. Fifteen patients had visual deterioration in the other eye as well, and of these, 12 had improvement, obviating the need for surgery on the other side. Complications were minimal and in the form of synechiae in two of the cases. A number of procedures have been described for the management of this entity, and each is associated with a significant morbidity; therefore, there was a need for a minimally invasive procedure. The procedure adopted in the series is minimally invasive and is associated with a 94.5% success rate and minimal morbidity. Endoscopic endonasal optic nerve fenestration is a safe, minimally invasive, and extremely effective procedure for the management of IIH.

Presentation, investigation and management of idiopathic intracranial hypertension in children

Summary Idiopathic intracranial hypertension is characterised by increased intracranial pressure in the presence of normal cerebrospinal fluid, normal neuroimaging and no localising signs on neurologic exam except cranial nerve VI palsy. It is common in adults, particularly obese young women, but also occurs in children and adolescents. We discuss the clinical presentation of idiopathic intracranial hypertension in the pediatric population and how the presenting signs and symptoms may be different from those seen in adult patients. We discuss the differential diagnosis for idiopathic intracranial hypertension, as well as underlying conditions that may be associated with it. As in adults, therapy most often involves the use of carbonic anhydrase inhibitors, diuretics, and steroids. Children with vision loss due to idiopathic intracranial hypertension who not respond to aggressive medical therapy should be promptly referred for surgical intervention, including optic nerve fenestration and cerebrospinal (CSF) shunting procedures such as ventriculoperitoneal or lumboperitoneal shunting.