Life | Special Issue : Idiopathic Intracranial Hypertension

Editorial

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4 pages, 181 KiB

Open AccessEditorial

Evolving Evidence in Idiopathic Intracranial Hypertension

by Susan P. Mollan, Heather E. Moss and Steffen Hamann

Life 2021, 11(11), 1225; https://doi.org/10.3390/life11111225 - 12 Nov 2021

Cited by 3 | Viewed by 2870

Abstract

Idiopathic intracranial hypertension (IIH) is an enigmatic disorder characterized by raised intracranial pressure (ICP) with no known cause and it affects both children and adults [...] Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

Research

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7 pages, 180 KiB

Open AccessArticle

Autism Spectrum Disorder in Pediatric Idiopathic Intracranial Hypertension

by Anne K. Jensen, Claire A. Sheldon, Grace L. Paley, Christina L. Szperka, Geraldine W. Liu, Grant T. Liu and Shana E. McCormack

Life 2021, 11(9), 972; https://doi.org/10.3390/life11090972 - 15 Sep 2021

Cited by 2 | Viewed by 3267

Abstract

In recent years, the substantial burden of medical comorbidities in autism spectrum disorder (ASD) populations has been described. We report a retrospective observational case series of pediatric patients with suspected idiopathic intracranial hypertension (IIH) and concurrent ASD. Pediatric subjects with suspected IIH aged [...] Read more.

In recent years, the substantial burden of medical comorbidities in autism spectrum disorder (ASD) populations has been described. We report a retrospective observational case series of pediatric patients with suspected idiopathic intracranial hypertension (IIH) and concurrent ASD. Pediatric subjects with suspected IIH aged 2–18 years were identified by review of a pediatric neuro-ophthalmologist’s database spanning from July 1993 to April 2013. ASD diagnoses were identified within this cohort by an ICD-9 diagnosis code search and database review. Three subjects had concurrent ASD diagnoses; all were non-obese males. Since the retrospective observational case series was performed in April 2013, we identified three additional IIH cases in boys with ASD. Our experience suggests that IIH may be a comorbidity of ASD, particularly in non-obese boys. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

10 pages, 693 KiB

Open AccessArticle

Unilateral Optic Nerve Sheath Fenestration in Idiopathic Intracranial Hypertension: A 6-Month Follow-Up Study on Visual Outcome and Prognostic Markers

by Snorre Malm Hagen, Marianne Wegener, Peter Bjerre Toft, Kåre Fugleholm, Rigmor Højland Jensen and Steffen Hamann

Life 2021, 11(8), 778; https://doi.org/10.3390/life11080778 - 31 Jul 2021

Cited by 10 | Viewed by 2767

Abstract

Loss of vision is a feared consequence of idiopathic intracranial hypertension (IIH). Optic nerve sheath fenestration (ONSF) may be an effective surgical approach to protect visual function in medically refractory IIH. In this study, we evaluate the impact of unilateral superomedial transconjunctival ONSF [...] Read more.

Loss of vision is a feared consequence of idiopathic intracranial hypertension (IIH). Optic nerve sheath fenestration (ONSF) may be an effective surgical approach to protect visual function in medically refractory IIH. In this study, we evaluate the impact of unilateral superomedial transconjunctival ONSF on bilateral visual outcome using a comprehensive follow-up program. A retrospective chart review of IIH patients who underwent unilateral ONSF between January 2016 and March 2021 was conducted. Patients fulfilling the revised Friedman criteria for IIH and who had exclusively received ONSF as a surgical treatment were included. Main outcomes were visual acuity (VA); perimetric mean deviation (PMD); papilledema grade; and optic nerve head elevation (maxONHE) 1 week, 2 weeks, and 1, 3, and 6 months after surgery. VA (p < 0.05), PMD (p < 0.05), papilledema grade (p < 0.01), and maxOHNE (p < 0.001) were improved after 6 months on both the operated and non-operated eye. Prolonged surgical delay impedes PMD improvement (r = −0.78, p < 0.01), and an increasing opening pressure initiates a greater ganglion cell loss (r = −0.79, p < 0.01). In this small case series, we demonstrate that unilateral superonasal transconjunctival ONSF is a safe procedure with an effect on both eyes. Optic nerve head elevation and PMD are feasible biomarkers for assessing early treatment efficacy after ONSF. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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9 pages, 925 KiB

Open AccessArticle

OCT Based Interpretation of the Optic Nerve Head Anatomy and Prevalence of Optic Disc Drusen in Patients with Idiopathic Intracranial Hypertension (IIH)

by Elisabeth Arnberg Wibroe, Lasse Malmqvist and Steffen Hamann

Life 2021, 11(6), 584; https://doi.org/10.3390/life11060584 - 19 Jun 2021

Cited by 18 | Viewed by 8301

Abstract

We aimed to systematically examine the optic nerve head anatomy in patients with idiopathic intracranial hypertension (IIH) using a standardized optical coherence tomography (OCT) protocol. The study retrospectively included 32 patients diagnosed from 2014 to 2021 with IIH. Using OCT, in accordance with [...] Read more.

We aimed to systematically examine the optic nerve head anatomy in patients with idiopathic intracranial hypertension (IIH) using a standardized optical coherence tomography (OCT) protocol. The study retrospectively included 32 patients diagnosed from 2014 to 2021 with IIH. Using OCT, in accordance with a standardized scanning protocol for patients with optic disc drusen, the presence of optic disc drusen, prelaminar hyperreflective lines, peripapillary hyperreflective ovoid mass-like structures, the retinal nerve fiber layer thickness, and macular ganglion cell layer volume was obtained. Optic disc drusen were found in 3.1%, hyperreflective lines in 31.3%, and peripapillary hyperreflective ovoid mass-like structures in 81.3% of all IIH patients at least three months after the time of diagnosis. We found no significant differences in retinal nerve fiber layer thickness or macular ganglion cell layer volume in patients with hyperreflective lines or PHOMS respectively compared to patients without hyperreflective lines (p = 0.1285 and p = 0.1835). In conclusion, the prevalence of optic disc drusen in IIH patients is similar to the reported prevalence in the general population. The high prevalence of hyperreflective lines and peripapillary hyperreflective ovoid mass-like structures in IIH patients suggest these structures be a result of crowding in the optic nerve head caused by papilledema. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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10 pages, 1028 KiB

Open AccessArticle

Abnormal Intracranial Pulse Pressure Amplitude Despite Normalized Static Intracranial Pressure in Idiopathic Intracranial Hypertension Refractory to Conservative Medical Therapy

by Per Kristian Eide

Life 2021, 11(6), 537; https://doi.org/10.3390/life11060537 - 9 Jun 2021

Cited by 8 | Viewed by 2544

Abstract

Idiopathic intracranial hypertension (IIH) incorporates symptoms and signs of increased intracranial pressure (ICP) and is diagnosed by increased lumbar cerebrospinal fluid pressure. However, our knowledge about the characteristics of ICP abnormality, e.g., changes in pulsatile versus static ICP, remains scarce. This study questioned [...] Read more.

Idiopathic intracranial hypertension (IIH) incorporates symptoms and signs of increased intracranial pressure (ICP) and is diagnosed by increased lumbar cerebrospinal fluid pressure. However, our knowledge about the characteristics of ICP abnormality, e.g., changes in pulsatile versus static ICP, remains scarce. This study questioned how overnight pulsatile ICP (mean ICP wave amplitude, MWA) associates with static ICP (mean ICP) in IIH patients who were refractory to conservative medical treatment. The material included 80 consecutive IIH patients undergoing ICP monitoring prior to shunt, as part of work-up for failed conservative medical therapy. In this group, the overnight mean ICP was normalized in 52/80 patients, but with abnormal overnight MWA in 45 of the 52 patients. Even though there was a positive correlation between MWA and mean ICP at group level and within individual ICP recordings, the levels of MWA were abnormal in a high proportion of patients despite normalized mean ICP. Taken together, the present results disclosed lasting abnormal pulsatile ICP despite normalized static ICP in IIH patients refractory to conservative medical therapy, which may reflect the underlying pathophysiology. It is tentatively suggested that abnormal pulsatile ICP in IIH may reflect alterations at the glia–neurovascular interface, resulting in impaired astrocytic pulsation absorber mechanisms. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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9 pages, 854 KiB

Open AccessArticle

Magnetic Resonance Imaging Features and Clinical Findings in Pediatric Idiopathic Intracranial Hypertension: A Case–Control Study

by Aubrey L. Gilbert, Jennifer Vaughn, Sarah Whitecross, Caroline D. Robson, David Zurakowski and Gena Heidary

Life 2021, 11(6), 487; https://doi.org/10.3390/life11060487 - 27 May 2021

Cited by 6 | Viewed by 4623

Abstract

The purpose of this study is to identify salient magnetic resonance imaging (MRI) findings of pediatric IIH, to determine the relevance of these findings with regard to disease pathogenesis, and to relate these findings to the clinical presentation towards identification of risk factors [...] Read more.

The purpose of this study is to identify salient magnetic resonance imaging (MRI) findings of pediatric IIH, to determine the relevance of these findings with regard to disease pathogenesis, and to relate these findings to the clinical presentation towards identification of risk factors of disease. A retrospective, a case–control study of 38 pediatric patients with and 24 pediatric patients without IIH from the ophthalmology department at a tertiary care center was performed. Clinical data, including ophthalmic findings and lumbar puncture results, were recorded. Neuroimaging, including both MRI and magnetic resonance venography (MRV), was evaluated for perioptic subarachnoid space diameter enlargement, posterior globe flattening, optic nerve head protrusion, empty or partially empty sella turcica, dural venous sinus abnormalities, skull base crowding, and prominent arachnoid granulations. Compared with controls, IIH patients had larger perioptic subarachnoid space diameters, higher incidences of posterior globe flattening, protrusion of the optic nerve heads, an empty sella turcica, and dural venous sinus abnormalities. A perioptic subarachnoid space diameter of ≥5.2 mm was identified as an independent predictor of IIH (p < 0.001) with sensitivity of 87% and specificity of 67%. Several significant MRI findings in pediatric IIH were identified. Using a model that uniquely incorporated clinical and MRI findings at presentation, we provide a framework for risk stratification for the diagnosis of pediatric IIH which may be utilized to facilitate diagnosis. Future prospective work is needed to further validate the model developed in this study. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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10 pages, 1547 KiB

Open AccessArticle

Exploratory Study of the Association between the Severity of Idiopathic Intracranial Hypertension and Electroretinogram Photopic Negative Response Amplitude Obtained Using a Handheld Device

by Antony Raharja, Shaun M. Leo, Isabelle Chow, Mathura Indusegaran, Christopher J. Hammond, Omar A. Mahroo and Sui H. Wong

Life 2021, 11(5), 437; https://doi.org/10.3390/life11050437 - 13 May 2021

Cited by 6 | Viewed by 2938

Abstract

The photopic negative response (PhNR) is a negative component of the photopic flash electroretinogram that follows the b-wave and is thought to arise from the retinal ganglion cells. Reduction in its amplitude in idiopathic intracranial hypertension (IIH) has been previously documented using formal [...] Read more.

The photopic negative response (PhNR) is a negative component of the photopic flash electroretinogram that follows the b-wave and is thought to arise from the retinal ganglion cells. Reduction in its amplitude in idiopathic intracranial hypertension (IIH) has been previously documented using formal electroretinography. This study explored the use of a handheld device (RETeval, LKC technologies, Gaithersburg, MD, USA) in 72 IIH patients of varying stages and severity (and seven controls) and investigated associations between PhNR parameters and disease severity. PhNR amplitudes at 72 ms (P₇₂) and p-ratio (ratio to b-wave peak value) differed significantly across groups, with a trend towards smaller amplitudes in those with severe IIH, defined as papilloedema with Modified Frisén Scale (MFS) ≥ 3, retinal nerve fibre layer (RNFL) ≥ 150 μm or atrophic papilloedema (p = 0.0048 and p = 0.018 for P72 and p-ratio, respectively). PhNR parameters did not correlate with MFS, RNFL thickness, standard automated perimetry mean deviation or macular ganglion cell layer volume. This study suggests that PhNR measurement using a handheld device is feasible and could potentially augment the assessment of disease severity in IIH. The clinical utility of PhNR monitoring in IIH patients requires further investigation. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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14 pages, 2681 KiB

Open AccessArticle

Idiopathic Intracranial Hypertension: Evaluation of Admissions and Emergency Readmissions through the Hospital Episode Statistic Dataset between 2002–2020

by Susan P. Mollan, Jemma Mytton, Georgios Tsermoulas and Alex J. Sinclair

Life 2021, 11(5), 417; https://doi.org/10.3390/life11050417 - 5 May 2021

Cited by 40 | Viewed by 4081

Abstract

With increasing incidence and prevalence of Idiopathic intracranial hypertension in the UK, the aim of this study was to explore emerging themes in Idiopathic intracranial hypertension using the Hospital Episode Statistics dataset and to quantify recent change in hospital admissions and surgeries performed [...] Read more.

With increasing incidence and prevalence of Idiopathic intracranial hypertension in the UK, the aim of this study was to explore emerging themes in Idiopathic intracranial hypertension using the Hospital Episode Statistics dataset and to quantify recent change in hospital admissions and surgeries performed within England. Methods: Hospital Episode Statistics national data was extracted between 1 April 2002 and 31 March 2019, and followed up until 31 March 2020. All those within England with a diagnosis of Idiopathic Intracranial Hypertension were included. Those with secondary causes of raised intracranial pressure such as tumors, hydrocephalus and cerebral venous sinus thrombosis were excluded. Results: 28,794 new IIH cases were diagnosed between 1 January 2002 and 31 December 2019. Incidence rose between 2002 to 2019 from 1.8 to 5.2 per 100,000 in the general population. Peak incidence occurred in females aged 25–29 years. Neurosurgical shunt was the commonest procedure performed (6.4%), followed by neovascular venous sinus stenting (1%), bariatric surgery (0.8%) and optic nerve sheath fenestration (0.5%). The portion of the total IIH population requiring a shunt fell from 10.8% in 2002/2003 to 2.46% in 2018/2019. The portion of the total IIH population requiring shunt revision also reduced over time from 4.84% in 2002/2003 to 0.44% in 2018/2019. The mean 30 days emergency readmissions for primary shunt, revision of shunt, bariatric surgery, neurovascular stent, and optic nerve sheath fenestration was 23.1%, 23.7%, 10.6%, 10.0% and 9.74%, respectively. There was a peak 30 days readmission rate following primary shunt in 2018/2019 of 41%. Recording of severe visual impairment fell to an all-time low of 1.38% in 2018/19. Conclusions: Increased awareness of the condition, specialist surgery and expert guidance may be changing admissions and surgical trends in IIH. The high 30 readmission following primary shunt surgery for IIH requires further investigation. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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9 pages, 508 KiB

Open AccessArticle

The Health Economic Evaluation of Bariatric Surgery Versus a Community Weight Management Intervention Analysis from the Idiopathic Intracranial Hypertension Weight Trial (IIH:WT)

by Magda Aguiar, Emma Frew, Susan P. Mollan, James L. Mitchell, Ryan S. Ottridge, Zerin Alimajstorovic, Andreas Yiangou, Rishi Singhal, Abd A. Tahrani and Alex J. Sinclair

Life 2021, 11(5), 409; https://doi.org/10.3390/life11050409 - 30 Apr 2021

Cited by 10 | Viewed by 2951

Abstract

Background: The Idiopathic Intracranial Hypertension Weight Trial (IIH:WT) established the efficacy of bariatric surgery as compared to a community weight management intervention in reducing intracranial pressure in active IIH. The aim of this cost-effectiveness analysis was to evaluate the economic impact of these [...] Read more.

Background: The Idiopathic Intracranial Hypertension Weight Trial (IIH:WT) established the efficacy of bariatric surgery as compared to a community weight management intervention in reducing intracranial pressure in active IIH. The aim of this cost-effectiveness analysis was to evaluate the economic impact of these weight loss treatments for IIH. Methods: IIH:WT was a five-year randomised, controlled, parallel group, multicentre trial in the United Kingdom, where participants with active IIH and a body mass index ≥35 kg/m² were randomly assigned (1:1) to receive access to bariatric surgery or a community weight management intervention. All clinical and quality of life data was recorded at baseline, 12 and 24 months. Economic evaluation was performed to assess health-care costs and cost-effectiveness. Evaluations were established on an intention to treat principle, followed by a sensitivity analysis using a per protocol analysis. Results: The mean total health care costs were GBP 1353 for the community weight management arm and GBP 5400 for the bariatric surgery arm over 24 months. The majority of costs for the bariatric surgery arm relate to the surgical procedure itself. The 85% who underwent bariatric surgery achieved a 12.5% reduction in intracranial pressure at 24 months as compared to 39% in the community weight management arm; a mean difference of 45% in favour of bariatric surgery. The cost effectiveness of bariatric surgery improved over time. Conclusions: The IIH:WT was the first to compare the efficacy and cost-effectiveness of bariatric surgery with community weight management interventions in the setting of a randomised control trial. The cost-effectiveness of bariatric surgery improved over time and therefore the incremental cost of surgery when offset against the incremental reduction of intracranial pressure improved after 24 months, as compared with 12 months follow up. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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12 pages, 2004 KiB

Open AccessEditor’s ChoiceArticle

Numerical Investigation on the Role of Mechanical Factors Contributing to Globe Flattening in States of Elevated Intracranial Pressure

by Jafar A. Mehr, Heather E. Moss and Hamed Hatami-Marbini

Life 2020, 10(12), 316; https://doi.org/10.3390/life10120316 - 28 Nov 2020

Cited by 6 | Viewed by 3776

Abstract

Flattening of the posterior eye globe in the magnetic resonance (MR) images is a sign associated with elevated intracranial pressure (ICP), often seen in people with idiopathic intracranial hypertension (IIH). The exact underlying mechanisms of globe flattening (GF) are not fully known but [...] Read more.

Flattening of the posterior eye globe in the magnetic resonance (MR) images is a sign associated with elevated intracranial pressure (ICP), often seen in people with idiopathic intracranial hypertension (IIH). The exact underlying mechanisms of globe flattening (GF) are not fully known but mechanical factors are believed to play a role. In the present study, we investigated the effects of material properties and pressure loads on GF. For this purpose, we used a generic finite element model to investigate the deformation of the posterior eyeball. The degree of GF in numerical models and the significance of different mechanical factors on GF were characterized using an automated angle-slope technique and a statistical measure. From the numerical models, we found that ICP had the most important role in GF. We also showed that the angle-slope graphs pertaining to MR images from five people with high ICP can be represented numerically by manipulating the parameters of the finite element model. This numerical study suggests that GF observed in IIH patients can be accounted for by the forces caused by elevation of ICP from its normal level, while material properties of ocular tissues, such as sclera (SC), peripapillary sclera (PSC), and optic nerve (ON), would impact its severity. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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Review

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14 pages, 968 KiB

Open AccessReview

Awareness, Diagnosis and Management of Idiopathic Intracranial Hypertension

by Rigmor Højland Jensen, Vlasta Vukovic-Cvetkovic, Johanne Juhl Korsbaek, Marianne Wegener, Steffen Hamann and Dagmar Beier

Life 2021, 11(7), 718; https://doi.org/10.3390/life11070718 - 20 Jul 2021

Cited by 10 | Viewed by 5759

Abstract

The diagnosis and management of idiopathic intracranial hypertension (IIH) can be difficult and multiple medical subspecialities are often involved. Several national and international guidelines regarding the investigations and management of IIH have been published in recent years but still there is no consensus [...] Read more.

The diagnosis and management of idiopathic intracranial hypertension (IIH) can be difficult and multiple medical subspecialities are often involved. Several national and international guidelines regarding the investigations and management of IIH have been published in recent years but still there is no consensus about the optimal organization of IIH-care. The objective of this review was to propose and describe a referral pathway and an organization scheme for diagnosis and management of IIH. An extensive search of existing literature was conducted and summarized. In total, 237 IIH-articles were identified and hereof 43 included. The clinical practice in our specialized IIH-clinic is characterized and described. We conclude that an educational campaign involving medical care providers and patients with chronic headaches is necessary. A detailed organizational proposal for a referral pathway and management of IIH patients based on the literature search and our clinical experience from a highly specialized IIH outpatient clinic is suggested and discussed. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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12 pages, 2713 KiB

Open AccessReview

Paediatric Idiopathic Intracranial Hypertension (IIH)—A Review

by Andrew Malem, Twishaa Sheth and Brinda Muthusamy

Life 2021, 11(7), 632; https://doi.org/10.3390/life11070632 - 29 Jun 2021

Cited by 14 | Viewed by 4205

Abstract

Paediatric idiopathic intracranial hypertension (IIH), is a rare but important differential diagnosis in children presenting with papilloedema. It is characterised by raised intracranial pressure in the absence of an identifiable secondary structural or systemic cause and is, therefore, a diagnosis of exclusion. In [...] Read more.

Paediatric idiopathic intracranial hypertension (IIH), is a rare but important differential diagnosis in children presenting with papilloedema. It is characterised by raised intracranial pressure in the absence of an identifiable secondary structural or systemic cause and is, therefore, a diagnosis of exclusion. In the adult population, there is a strong predilection for the disease to occur in female patients who are obese. This association is also seen in paediatric patients with IIH but primarily in the post-pubertal cohort. In younger pre-pubertal children, this is not the case, possibly reflecting a different underlying disease aetiology and pathogenesis. Untreated IIH in children can cause significant morbidity from sight loss, chronic headaches, and the psychological effects of ongoing regular hospital monitoring, interventions, and medication. The ultimate goal in the management of paediatric IIH is to protect the optic nerve from papilloedema-induced optic neuropathy and thus preserve vision, whilst reducing the morbidity from other symptoms of IIH, in particular chronic headaches. In this review, we will outline the typical work-up and diagnostic process for paediatric patients with suspected IIH and how we manage these patients. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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15 pages, 267 KiB

Open AccessReview

Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema

by Susan P. Mollan, Yu Jeat Chong, Olivia Grech, Alex J. Sinclair and Benjamin R. Wakerley

Life 2021, 11(6), 472; https://doi.org/10.3390/life11060472 - 24 May 2021

Cited by 20 | Viewed by 5844

Abstract

The pseudotumor cerebri syndrome embraces disorders characterised by raised intracranial pressure, where the commonest symptom is headache (90%). Idiopathic intracranial hypertension without papilloedema (IIHWOP) is increasingly recognised as a source of refractory headache symptoms and resultant neurological disability. Although the majority of patients [...] Read more.

The pseudotumor cerebri syndrome embraces disorders characterised by raised intracranial pressure, where the commonest symptom is headache (90%). Idiopathic intracranial hypertension without papilloedema (IIHWOP) is increasingly recognised as a source of refractory headache symptoms and resultant neurological disability. Although the majority of patients with IIHWOP are phenotypically similar to those with idiopathic intracranial hypertension (IIH), it remains uncertain as to whether IIHWOP is nosologically distinct from IIH. The incidence, prevalence, and the degree of association with the world-wide obesity epidemic is unknown. Establishing a diagnosis of IIHWOP can be challenging, as often lumbar puncture is not routinely part of the work-up for refractory headaches. There are published diagnostic criteria for IIHWOP; however, some report uncertainty regarding a pathologically acceptable cut off for a raised lumbar puncture opening pressure, which is a key criterion. The literature provides little information to help guide clinicians in managing patients with IIHWOP. Further research is therefore needed to better understand the mechanisms that drive the development of chronic daily headaches and a relationship to intracranial pressure; and indeed, whether such patients would benefit from therapies to lower intracranial pressure. The aim of this narrative review was to perform a detailed search of the scientific literature and provide a summary of historic and current opinion regarding IIHWOP. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

15 pages, 655 KiB

Open AccessReview

The Role of Metabolism in Migraine Pathophysiology and Susceptibility

by Olivia Grech, Susan P. Mollan, Benjamin R. Wakerley, Daniel Fulton, Gareth G. Lavery and Alexandra J. Sinclair

Life 2021, 11(5), 415; https://doi.org/10.3390/life11050415 - 1 May 2021

Cited by 23 | Viewed by 5446

Abstract

Migraine is a highly prevalent and disabling primary headache disorder, however its pathophysiology remains unclear, hindering successful treatment. A number of key secondary headache disorders have headaches that mimic migraine. Evidence has suggested a role of mitochondrial dysfunction and an imbalance between energetic [...] Read more.

Migraine is a highly prevalent and disabling primary headache disorder, however its pathophysiology remains unclear, hindering successful treatment. A number of key secondary headache disorders have headaches that mimic migraine. Evidence has suggested a role of mitochondrial dysfunction and an imbalance between energetic supply and demand that may contribute towards migraine susceptibility. Targeting these deficits with nutraceutical supplementation may provide an additional adjunctive therapy. Neuroimaging techniques have demonstrated a metabolic phenotype in migraine similar to mitochondrial cytopathies, featuring reduced free energy availability and increased metabolic rate. This is reciprocated in vivo when modelling a fundamental mechanism of migraine aura, cortical spreading depression. Trials assessing nutraceuticals successful in the treatment of mitochondrial cytopathies including magnesium, coenzyme q10 and riboflavin have also been conducted in migraine. Although promising results have emerged from nutraceutical trials in patients with levels of minerals or vitamins below a critical threshold, they are confounded by lacking control groups or cohorts that are not large enough to be representative. Energetic imbalance in migraine may be relevant in driving the tissue towards maximum metabolic capacity, leaving the brain lacking in free energy. Personalised medicine considering an individual’s deficiencies may provide an approach to ameliorate migraine. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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15 pages, 2628 KiB

Open AccessReview

Neurosurgical CSF Diversion in Idiopathic Intracranial Hypertension: A Narrative Review

by Geraint J. Sunderland, Michael D. Jenkinson, Elizabeth J. Conroy, Carrol Gamble and Conor L. Mallucci

Life 2021, 11(5), 393; https://doi.org/10.3390/life11050393 - 26 Apr 2021

Cited by 14 | Viewed by 3617

Abstract

The prevalence of idiopathic intracranial hypertension (IIH), a complex disorder, is increasing globally in association with obesity. The IIH syndrome occurs as the result of elevated intracranial pressure, which can cause permanent visual impairment and loss if not adequately managed. CSF diversion via [...] Read more.

The prevalence of idiopathic intracranial hypertension (IIH), a complex disorder, is increasing globally in association with obesity. The IIH syndrome occurs as the result of elevated intracranial pressure, which can cause permanent visual impairment and loss if not adequately managed. CSF diversion via ventriculoperitoneal and lumboperitoneal shunts is a well-established strategy to protect vision in medically refractory cases. Success of CSF diversion is compromised by high rates of complication; including over-drainage, obstruction, and infection. This review outlines currently used techniques and technologies in the management of IIH. Neurosurgical CSF diversion is a vital component of the multidisciplinary management of IIH. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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Other

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8 pages, 195 KiB

Open AccessCommentary

Intracranial Venous Hypertension and Venous Sinus Stenting in the Modern Management of Idiopathic Intracranial Hypertension

by Robert K. Townsend and Kyle M. Fargen

Life 2021, 11(6), 508; https://doi.org/10.3390/life11060508 - 31 May 2021

Cited by 14 | Viewed by 4056

Abstract

Idiopathic intracranial hypertension (IIH) is a debilitating condition that has traditionally been difficult to treat. In recent years, there has been increasing focus on the role of intracranial venous hypertension in the pathophysiology of IIH. Based on increased understanding of this pathophysiology, venous [...] Read more.

Idiopathic intracranial hypertension (IIH) is a debilitating condition that has traditionally been difficult to treat. In recent years, there has been increasing focus on the role of intracranial venous hypertension in the pathophysiology of IIH. Based on increased understanding of this pathophysiology, venous sinus stenting (VSS) has emerged as a safe and reliable treatment for a certain population of patients with IIH. Stratifying patients with IIH based on the status of their venous outflow can provide insight into which patients may enjoy reduction in their symptoms after VSS and provides information regarding why some patients may have symptom recurrence. The traditional view of IIH as a disease due to obesity in young women has been cast into doubt as the understanding of the role of intracranial venous hypertension has improved. Full article

(This article belongs to the Special Issue Idiopathic Intracranial Hypertension)

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