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Nature Reviews. Neurology Dec 2023The understanding of idiopathic intracranial hypertension (IIH) has evolved over the past few years. Previously, IIH was considered a disease exclusively affecting the... (Review)
Review
The understanding of idiopathic intracranial hypertension (IIH) has evolved over the past few years. Previously, IIH was considered a disease exclusively affecting the neuro-ophthalmic axis, characterized by raised intracranial pressure, headache and papilloedema, and resulting in the risk of severe and permanent visual loss and life-changing disabling headaches. Recent advances have begun to redefine IIH as a probable metabolic disease involving a range of systemic manifestations. More than 95% of individuals affected by the disease are women of reproductive age with obesity. The incidence is rapidly rising and parallels the escalating worldwide obesity rates. Contemporary insights identify associations with insulin resistance, type 2 diabetes and a twofold increased risk of cardiovascular disease in excess of that driven by obesity alone. Adipose distribution in people with IIH, like that in other metabolic diseases, is preferentially centripetal and is associated with changes in intracranial pressure. Evidence now demonstrates adipose tissue dysfunction in people with IIH, involving transcriptional and metabolic priming for lipogenesis and weight gain. Hormonal perturbations are also observed, including a unique phenotype of androgen excess that promotes cerebrospinal fluid secretion. Knowledge of these additional disease features is driving research into novel therapeutic targets and altering the approach to multidisciplinary care.
Topics: Female; Humans; Male; Pseudotumor Cerebri; Diabetes Mellitus, Type 2; Obesity; Headache; Vision Disorders
PubMed: 37957260
DOI: 10.1038/s41582-023-00893-0 -
World Neurosurgery Dec 2023This retrospective study aimed at determining the dimension of the interthalamic adhesion (ITA) in patients with the idiopathic intracranial hypertension (IIH) for...
OBJECTIVE
This retrospective study aimed at determining the dimension of the interthalamic adhesion (ITA) in patients with the idiopathic intracranial hypertension (IIH) for assisting in preoperative radiologic diagnosis.
METHODS
The study universe consisted of magnetic resonance images of 20 patients with IIH (age: 22.70 ± 4.04 years, sex: 14 females and 6 males) and 20 normal subjects (age 22.30± 2.94 years, sex: 14 females and 6 males). To determine the morphology of ITA, its height (vertical diameter) and width (horizontal diameter) were measured on the coronal and axial planes, respectively.
RESULTS
The height and width of ITA in IIH were measured as 2.58 ± 0.71 mm (range: 1.40-4.20 mm) and 2.73 ± 0.77 mm (range: 1.70-4.40 mm), respectively. Its height and width in controls were measured as 4.99 ± 1.04 mm (range: 2.70-6.30 mm) and 4.92 ± 1.11 mm (range: 2.60-6.50 mm), respectively. ITA height and width in IIH was significantly smaller compared with controls (P < 0.001). For an arbitrary cutoff of 3.85 mm, the sensitivity of the height of ITA was 85% with 95% specificity. For an arbitrary cutoff of 4.45 mm, the sensitivity of the width of ITA was 75% with 100% specificity.
CONCLUSIONS
The height and width of ITA are approximately 50% smaller in IIH than controls; therefore alterations in the dimension of ITA may be a valuable radiologic sign for the diagnosis of IIH.
Topics: Male; Female; Humans; Adolescent; Young Adult; Adult; Pseudotumor Cerebri; Retrospective Studies; Magnetic Resonance Imaging; Thalamus; Radiology; Intracranial Hypertension
PubMed: 37769844
DOI: 10.1016/j.wneu.2023.09.080 -
Neurologia Mar 2024Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this...
INTRODUCTION
Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status.
PATIENTS AND METHODS
We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up.
RESULTS
We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients.
CONCLUSION
Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.
Topics: Male; Humans; Child; Infant; Child, Preschool; Adolescent; Female; Pseudotumor Cerebri; Overweight; Retrospective Studies; Prognosis; Obesity
PubMed: 38272258
DOI: 10.1016/j.nrleng.2024.01.004 -
Acta Neurochirurgica Feb 2024CSF diversion is a recognised intervention in idiopathic intracranial hypertension (IIH), particularly in the presence of vision-threatening papilledema. Although... (Review)
Review
PURPOSE
CSF diversion is a recognised intervention in idiopathic intracranial hypertension (IIH), particularly in the presence of vision-threatening papilledema. Although ventriculo-atrial (VA) shunt insertion is a routine neurosurgical procedure, ventriculoperitoneal and lumboperitoneal shunts have been mostly used in this particular indication. This study aims to look at a single centre's experience with VA shunts in idiopathic intracranial hypertension (IIH).
METHODS
Retrospective case series with a review of electronic records over a 10-year period; exclusion criteria were duplication of same shunt insertion, no VA shunt insertion, paediatric patients and indication other than IIH. Notes were reviewed for demographics, shunt survival (defined by time prior to revision) and reasons for revision.
RESULTS
Eight VA shunt procedures were identified in 6 patients (mean age at insertion 34 ± 10 years) with a mean follow-up of 58 ± 25 months. All shunts were secondary procedures; 2 revisions from lumbo-pleural, 2 from ventriculopleural, 2 from ventriculoatrial and one each from ventriculoperitoneal and combined lumbo-/ventriculoperitoneal. At 50 months, 75% of VA shunts had survived, compared to only 58.3% of VPleural shunts in patients with IIH. Revisions were required due to acute intracranial bleed (1 case)-revised at day 1, and thrombus at distal site (1 case)-revised at day 57. Both shunts were later reinserted. From the latest clinic letters, all patients had their treatment optimised with this procedure, although only two patients had documented resolved papilloedema post-procedure.
CONCLUSIONS
Ventriculo-atrial shunts are a safe and efficacious alternative option for CSF diversion in IIH. In this series, only 1 shunt was revised for a VA shunt-specific complication.
Topics: Humans; Child; Young Adult; Adult; Pseudotumor Cerebri; Retrospective Studies; Intracranial Hemorrhages; Prostheses and Implants
PubMed: 38386079
DOI: 10.1007/s00701-024-05985-4 -
Current Pain and Headache Reports Nov 2023Migraine and idiopathic intracranial hypertension (IIH) are increasingly encountered but remain enigmatic. This review compares the similarities and differences of the... (Review)
Review
PURPOSE OF REVIEW
Migraine and idiopathic intracranial hypertension (IIH) are increasingly encountered but remain enigmatic. This review compares the similarities and differences of the diagnostic criteria, pathophysiology, and risk factors for chronic migraine and IIH.
RECENT FINDINGS
While migraine and IIH are distinct diseases, both conditions are frequently found concurrently and may share a link. Increased intracranial pressure (ICP) in those with or without pre-existing migraine may present with migraine-like headaches and contribute to migraine chronification. Increased intracranial pressure may be a coincidental occurrence in patients with migraine and normalization of pressure does not always translate to headache improvement. Limited information is available regarding the standard of treatment for patients with chronic migraine and IIH without papilledema. There continues to be controversy over the normal range of cerebral spinal fluid (CSF) values. Recognizing the concurrence of both conditions advances our understanding of headache pathology and demonstrates a striking need for more research.
Topics: Humans; Pseudotumor Cerebri; Intracranial Hypertension; Migraine Disorders; Papilledema; Headache
PubMed: 37656318
DOI: 10.1007/s11916-023-01166-7 -
Cephalalgia : An International Journal... Aug 2023The quality of clinical trials is essential to advance treatment, inform regulatory decisions and meta-analysis. With the increased incidence of idiopathic intracranial...
The quality of clinical trials is essential to advance treatment, inform regulatory decisions and meta-analysis. With the increased incidence of idiopathic intracranial hypertension and the emergence of clinical trials for novel therapies in this condition, the International Headache Society aims to establish guidelines for designing state-of-the-art controlled clinical trials for idiopathic intracranial hypertension.
Topics: Humans; Headache; Pseudotumor Cerebri; Controlled Clinical Trials as Topic
PubMed: 37661711
DOI: 10.1177/03331024231197118 -
Brain Communications 2023Idiopathic intracranial hypertension, a disease classically occurring in women with obesity, is characterized by raised intracranial pressure. Weight loss leads to the...
Idiopathic intracranial hypertension, a disease classically occurring in women with obesity, is characterized by raised intracranial pressure. Weight loss leads to the reduction in intracranial pressure. Additionally, pharmacological glucagon-like peptide-1 agonism reduces cerebrospinal fluid secretion and intracranial pressure. The potential mechanisms by which weight loss reduces intracranial pressure are unknown and were the focus of this study. Meal stimulation tests (fasted plasma sample, then samples at 15, 30, 60, 90 and 120 min following a standardized meal) were conducted pre- and post-bariatric surgery [early (2 weeks) and late (12 months)] in patients with active idiopathic intracranial hypertension. Dynamic changes in gut neuropeptides (glucagon-like peptide-1, gastric inhibitory polypeptide and ghrelin) and metabolites (untargeted ultra-high performance liquid chromatography-mass spectrometry) were evaluated. We determined the relationship between gut neuropeptides, metabolites and intracranial pressure. Eighteen idiopathic intracranial hypertension patients were included [Roux-en-Y gastric bypass (RYGB) = 7, gastric banding = 6 or sleeve gastrectomy = 5]. At 2 weeks post-bariatric surgery, despite similar weight loss, RYGB had a 2-fold (50%) greater reduction in intracranial pressure compared to sleeve. Increased meal-stimulated glucagon-like peptide-1 secretion was observed after RYGB (+600%) compared to sleeve (+319%). There was no change in gastric inhibitory polypeptide and ghrelin. Dynamic changes in meal-stimulated metabolites after bariatric surgery consistently identified changes in lipid metabolites, predominantly ceramides, glycerophospholipids and lysoglycerophospholipids, which correlated with intracranial pressure. A greater number of differential lipid metabolites were observed in the RYGB cohort at 2 weeks, and these also correlated with intracranial pressure. In idiopathic intracranial hypertension, we identified novel changes in lipid metabolites and meal-stimulated glucagon-like peptide-1 levels following bariatric surgery which were associated with changes in intracranial pressure. RYGB was most effective at reducing intracranial pressure despite analogous weight loss to gastric sleeve at 2 weeks post-surgery and was associated with more pronounced changes in these metabolite pathways. We suggest that these novel perturbations in lipid metabolism and glucagon-like peptide-1 secretion are mechanistically important in driving a reduction in intracranial pressure following weight loss in patients with idiopathic intracranial hypertension. Therapeutic targeting of these pathways, for example with glucagon-like peptide-1 agonist infusion, could represent a therapeutic strategy.
PubMed: 37901040
DOI: 10.1093/braincomms/fcad272 -
Journal of Child Neurology Oct 2023Lyme disease is the most common vector-borne disease in the United States and has been associated with secondary intracranial hypertension. We reviewed 11 pediatric... (Review)
Review
Lyme disease is the most common vector-borne disease in the United States and has been associated with secondary intracranial hypertension. We reviewed 11 pediatric patients with Lyme-associated secondary intracranial hypertension. All patients presented with headache, ten had papilledema, 7 with a rash, and 5 with a cranial nerve palsy. All patients were treated with acetazolamide, and 3 received combination therapy with furosemide. Three patients were considered to have fulminant intracranial hypertension because of the severity in their presenting courses. Two of the fulminant intracranial hypertension patients were treated with a temporary lumbar drain in addition to medications, whereas 1 fulminant intracranial hypertension patient was treated exclusively with medical therapy alone. The addition of a lumbar drain decreased the time to resolution of papilledema compared to medical management alone. Final visual acuity was 20/20 in each eye of all patients, suggesting that a titrated approach to therapy depending on the severity of presentation can result in good visual outcomes in these cases. Additionally, symptoms can recur after medication wean, so patients should be monitored closely with any discontinuation of intracranial pressure lowering medications.
Topics: Humans; Child; Papilledema; Intracranial Hypertension; Intracranial Pressure; Meningitis; Lyme Disease; Pseudotumor Cerebri
PubMed: 37691308
DOI: 10.1177/08830738231197873 -
Cureus Mar 2024An unidentified source of increased intracranial pressure is a hallmark of idiopathic intracranial hypertension (IIH), also referred to as pseudotumor cerebri. It mainly... (Review)
Review
An unidentified source of increased intracranial pressure is a hallmark of idiopathic intracranial hypertension (IIH), also referred to as pseudotumor cerebri. It mainly affects young, obese women, yet it can happen to anyone, regardless of age, gender, or weight. IIH presents with symptoms such as headaches, visual disturbances, and pulsatile tinnitus and can lead to severe complications, including vision loss, if left untreated. Diagnosis involves clinical evaluation, neuroimaging, and lumbar puncture, while management options include medical interventions and surgical procedures. This review provides a comprehensive overview of IIH, including its etiology, clinical presentation, epidemiology, complications, management approaches, and challenges. Increased awareness among healthcare professionals, standardized diagnostic criteria, and further research efforts are essential for improving outcomes and quality of life for individuals with IIH.
PubMed: 38623134
DOI: 10.7759/cureus.56256 -
Ophthalmology Sep 2023
Topics: Humans; Pseudotumor Cerebri; Papilledema
PubMed: 36229275
DOI: 10.1016/j.ophtha.2022.09.006