Progress and recognition of idiopathic intracranial hypertension: A narrative review.

BACKGROUND: Idiopathic intracranial hypertension (IIH) mainly affects obese young women, causing elevated intracranial pressure, headaches, and papilledema, risking vision loss and severe headaches. Despite weight loss as the primary treatment, the underlying mechanisms remain unclear. Recent research explores novel therapeutic targets. AIMS: This review aimed to provide a comprehensive understanding of IIH's pathophysiology and clinical features to inform pathogenesis and improve treatment strategies. METHODS: Recent publications on IIH were searched and summarized using PubMed, Web of Science, and MEDLINE. RESULTS: The review highlights potential pathomechanisms and therapeutic advances in IIH. CONCLUSION: IIH incidence is rising, with growing evidence linking it to metabolic and hormonal disturbances. Early diagnosis and treatment remain challenging.

Regulation of brain fluid volumes and pressures: basic principles, intracranial hypertension, ventriculomegaly and hydrocephalus.

The principles of cerebrospinal fluid (CSF) production, circulation and outflow and regulation of fluid volumes and pressures in the normal brain are summarised. Abnormalities in these aspects in intracranial hypertension, ventriculomegaly and hydrocephalus are discussed. The brain parenchyma has a cellular framework with interstitial fluid (ISF) in the intervening spaces. Framework stress and interstitial fluid pressure (ISFP) combined provide the total stress which, after allowing for gravity, normally equals intracerebral pressure (ICP) with gradients of total stress too small to measure. Fluid pressure may differ from ICP in the parenchyma and collapsed subarachnoid spaces when the parenchyma presses against the meninges. Fluid pressure gradients determine fluid movements. In adults, restricting CSF outflow from subarachnoid spaces produces intracranial hypertension which, when CSF volumes change very little, is called idiopathic intracranial hypertension (iIH). Raised ICP in iIH is accompanied by increased venous sinus pressure, though which is cause and which effect is unclear. In infants with growing skulls, restriction in outflow leads to increased head and CSF volumes. In adults, ventriculomegaly can arise due to cerebral atrophy or, in hydrocephalus, to obstructions to intracranial CSF flow. In non-communicating hydrocephalus, flow through or out of the ventricles is somehow obstructed, whereas in communicating hydrocephalus, the obstruction is somewhere between the cisterna magna and cranial sites of outflow. When normal outflow routes are obstructed, continued CSF production in the ventricles may be partially balanced by outflow through the parenchyma via an oedematous periventricular layer and perivascular spaces. In adults, secondary hydrocephalus with raised ICP results from obvious obstructions to flow. By contrast, with the more subtly obstructed flow seen in normal pressure hydrocephalus (NPH), fluid pressure must be reduced elsewhere, e.g. in some subarachnoid spaces. In idiopathic NPH, where ventriculomegaly is accompanied by gait disturbance, dementia and/or urinary incontinence, the functional deficits can sometimes be reversed by shunting or third ventriculostomy. Parenchymal shrinkage is irreversible in late stage hydrocephalus with cellular framework loss but may not occur in early stages, whether by exclusion of fluid or otherwise. Further studies that are needed to explain the development of hydrocephalus are outlined.

Application of novel non-invasive ophthalmic imaging to visualize peripapillary wrinkles, retinal folds and peripapillary hyperreflective ovoid mass-like structures associated with elevated intracranial pressure.

Background: Elevated intracranial pressure (ICP) is a serious and potentially life-threatening condition, for which clinically useful non-invasive measures have been elusive, in some cases due to their inadequate sensitivity and specificity. Our aim was to evaluate novel non-invasive ophthalmic imaging of selected pathological features seen in elevated ICP, namely peripapillary hyperreflective ovoid mass-like structures (PHOMS), peripapillary wrinkles (PPW) and retinal folds (RF) as potential biomarkers of elevated ICP. Methods: This single-center pilot study included subjects with untreated or incompletely treated high ICP. The retinas of these subjects were evaluated with averaged en-face optical coherence tomography (OCT), OCT retinal cross-sections (OCT B-scans), adaptive optics scanning light ophthalmoscopy (AOSLO), and fundus photos. Results: Seven subjects were included in the study. 6 subjects with high ICP (5 idiopathic intracranial hypertension, 1 medication induced, 30.8 ± 8.6 years, 75% female, 5 with papilledema) and 1 control (20-25 years) were included. PHOMS, PPW and RF were present in all subjects with papilledema, but neither in the high ICP subject without papilledema nor in the control subject. Averaged en-face OCT scans and AOSLO were more sensitive for PPW and RF than OCT B-scans and commercial fundus photos. Conclusion: PPW, RF and PHOMS volume have potential as non-invasive biomarkers of ICP. Novel imaging modalities may improve sensitivity. However, lack of automated image acquisition and processing limits current widespread adoption in clinical settings. Further research is needed to validate these structures as biomarkers for elevated ICP and improve clinical utility.

A Case Report Detailing a Rare Presentation of Idiopathic Intracranial Hypertension With Atypical Symptoms.

Idiopathic intracranial hypertension (IIH), formerly known as pseudotumor cerebri, represents a challenging diagnostic entity in neurology, characterized by elevated intracranial pressure of unknown origin. The classical clinical triad of headache, visual disturbances, and papilledema provides a well-established framework for diagnosis; however, the heterogeneity of IIH presentations, combined with the absence of an overt causative factor, continues to perplex clinicians. This case report delves into the complexities of a rare IIH presentation in a 32-year-old male, highlighting the need for a nuanced understanding of this condition beyond its traditional confines.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: A Case Report.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a newly discovered autoimmune demyelinating disorder. The clinical manifestations of MOGAD are divergent but often characterized by inflammatory central nervous system (CNS) deficits such as optic neuritis, encephalitis, or transverse myelitis that predominantly affect the pediatric population. Despite the distinct features often associated with MOGAD, the disease exhibits a diverse range of clinical manifestations, making timely diagnosis and treatment challenging. In particular, distinguishing MOGAD from multiple sclerosis (MS) is important for adequate treatment and the prevention of relapsing disease. In this report, we present a rare case of MOGAD in a 57-year-old male who initially exhibited symptoms of bilateral optic nerve edema and flame hemorrhage. This led to an initial misdiagnosis of pseudotumor cerebri. Serological analysis at a tertiary care center ultimately led to the diagnosis of MOGAD after multiple visits to the ophthalmologist with worsening vision deficits.

Assessment of glymphatic dysfunction in pediatric idiopathic intracranial hypertension: insights from quantitative diffusivity and perivascular spaces analysis-a case-control study.

Background: The impaired drainage of cerebrospinal fluid through the glymphatic system is thought to play a role in the idiopathic intracranial hypertension (IIH) pathophysiology. Limited data exist regarding the glymphatic system's involvement in pediatric patients with IIH. Therefore, the study's objective was to quantitatively evaluate alterations in parenchymal diffusivity and magnetic resonance imaging (MRI)-visible dilated perivascular spaces (PVS) as imaging indicators of glymphatic dysfunction in pediatric patients with IIH. Methods: Patients diagnosed with IIH in 2017-2022 in a single tertiary center (Sheba Medical Center, Israel) were retrospectively reviewed. Twenty-four pediatric patients were enrolled. All patients underwent clinical 3-T brain MRI. The control group included 24 age- and gender-matched healthy subjects with a normal-appearing brain on imaging. We used automatic atlas-based diffusion-weighted imaging analysis to determine regional diffusivity of the thalamus, caudate, putamen, globus pallidus, hippocampus, amygdala, and brain stem. PVS were evaluated using a semi-quantitative rating scale on T2-weighted images. Variables were compared using the Mann-Whitney test. Multivariate analysis of covariance was used to test for differences between controls and IIH patients. Results: No significant differences in regional brain diffusivity were observed between individuals with IIH and healthy controls (P=0.14-0.91 for various brain regions). The number of visible PVS was comparable between patients with IIH and the control group across all evaluated sites (P=0.12-0.74 for various brain regions). Conclusions: Pediatric IIH patients exhibited similar patterns of parenchymal diffusivity and PVS compared to age-matched controls. These findings do not support the hypothesis that the glymphatic system may play a role in the pathophysiology of pediatric IIH, although previously postulated. However, employing more sophisticated magnetic resonance (MR) techniques could enhance the sensitivity in uncovering underlying glymphatic dysfunction. Further research is warranted to validate and explore this association in larger cohorts and investigate the underlying mechanisms involved in IIH.

Pseudotumour Cerebri Induced by All-Trans Retinoic Acid: A Case Report.

Acute promyelocytic leukaemia (APL) is an aggressive type of leukaemia associated with severe coagulopathy and haemorrhage. Treatment with all-trans retinoic acid (ATRA) combined with arsenic trioxide (ATO) therapy is life-saving and induces excellent remission rates. However, ATRA can, on rare occasions, cause pseudotumour cerebri, which is characterised by an elevation in intracranial pressure without evidence of infection or vascular or structural abnormalities. We describe a case of pseudotumour cerebri that was precipitated by ATRA therapy. Intracranial hypertension should always be considered in patients with headaches and visual complaints with normal neuroimaging. Early identification and management are essential to preventing visual loss.

A Rare Case of Extraction of Ventriculoperitoneal Shunt From the Left Main Bronchus.

We present the case of a 40-year-old female who underwent several insertions of ventriculoperitoneal (VP) shunts as a part of the treatment for idiopathic intracranial hypertension (IIH). Several years after the insertion of the last VP shunt, the patient started experiencing shortness of breath (SOB) and cough; after further assessment, it was noted on computed tomography (CT) scan that the VP shunt had migrated into the right lower lobe of the lung and perforated the distal left main bronchus. The shunt was successfully retrieved using bronchoscopy under general anesthesia, after which the patient had a complete resolution of symptoms. Shunt migration is one of the rare complications that can happen years after shunt insertion. Therefore, we present this rare case of shunt migration into the thorax cavity to highlight the presentation of this complication and its successful management.

Case report: Novel transtemporal transverse sinus decompression surgery to alleviate transverse sinus stenosis in a pulsatile tinnitus patient with restricted bilateral venous outflow.

Transverse sinus (TS) stenosis is common in individuals with venous pulsatile tinnitus (PT). While PT can be addressed by endoluminal or extraluminal methods, the former has shown promise in alleviating symptoms associated with increased intracranial pressure. This study explores the potential of extraluminal methods to alleviate TS stenosis and eliminate PT caused by sigmoid sinus diverticulum. A 31-year-old male patient presenting with left-sided PT, attributed to a large, pedunculated sigmoid sinus diverticulum along with severe ipsilateral TS stenosis and contralateral TS hypoplasia, underwent ipsilateral extraluminal TS decompression surgery following sigmoid sinus wall reconstruction under local anesthesia. Postoperative CT and MR angiography revealed a significant increase in the TS lumen from 0.269 to 0.42 cm2 (56.02%) 2 years after surgery. Cervical Doppler ultrasound demonstrated a 36.07% increase in ipsilateral outflow volume to 16.6 g/s and a 77.63% increase in contralateral outflow volume to 1.35 g/s. In conclusion, this pioneering study showcases the potential of transtemporal TS decompression surgery in creating space for adaptive expansion of the TS lumen. However, the procedure should be reserved for individuals with severely compromised venous return.

Benign Intracranial Hypertension: A Rare Manifestation of Neurosarcoidosis.

Sarcoidosis is an immune-mediated disease that can involve multiple systems. Sarcoidosis of the nervous system or neurosarcoidosis may present as cranial mononeuropathy, hypothalamic involvement, aseptic meningitis, granulomatous inflammation in the brain parenchyma or spinal cord, peripheral neuropathy, and, in rare cases, as myopathy and benign intracranial hypertension. The most common cranial nerve involvement is the facial nerve, which can present as unilateral or bilateral facial nerve palsy, often with recurrent episodes. Involvement of other cranial nerves such as the second and eighth cranial nerves has also been reported. Granulomatous inflammation in the spinal cord presents as myelopathy or radiculopathy. Peripheral neuropathy can manifest as mononeuropathy, mononeuritis multiplex, or generalized sensory-motor neuropathy. Carpal tunnel syndrome is more common in patients with sarcoidosis compared to the general population. Here, we describe the case of a 40-year-old female who presented with heaviness of the head and blurred vision, with a prior history of left-sided Bell's palsy. Bilateral papilledema was observed during the fundus examination. MRI of the brain revealed signs suggestive of benign intracranial hypertension. The cerebrospinal fluid (CSF) opening pressure was measured at 40 cmH2O. Biopsy of bilateral hilar lymphadenopathy indicated granulomatous inflammation consistent with sarcoidosis. The patient was started on steroids and acetazolamide, and she had a dramatic improvement in symptoms.

A Man's Struggle With Idiopathic Intracranial Hypertension: A Unique Case Study.

We report a unique case of a 53-year-old male with idiopathic intracranial hypertension (IIH), predominantly affecting overweight young women. The patient, known to have diabetes mellitus, familial Mediterranean fever, and dyslipidemia, presented with blurred vision and throbbing headaches. Clinical examination, brain MRI/MRV, and a lumbar puncture confirmed the IIH diagnosis. Management with acetazolamide improved the patient's symptoms significantly. This case highlights the potential for IIH occurrence in men and underscores the need for early diagnosis and intervention to prevent potential visual impairment, typically more severe in male patients.

B-mode transorbital ultrasonography for the diagnosis of idiopathic intracranial hypertension: an updated systematic review and meta-analysis.

OBJECTIVES: This systematic review and meta-analysis aimed to evaluate the role of B-mode transorbital ultrasonography (TOS) for the diagnosis of idiopathic intracranial hypertension (IIH) in adults. METHODS: MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) (1966-May 2022) were searched to identify studies reporting ultrasonographic data about the optic nerve sheath diameter (ONSD) and optic disc elevation (ODE) in adults with IIH compared to subjects without IIH. The quality of the included studies was evaluated by the Newcastle-Ottawa Quality. RESULTS: Fifteen studies were included (total of 439 patients). The values of ODE ranged from 0.6 to 1.3 mm in patients with IIH. The values of ONSD ranged from 4.7 to 6.8 mm in IIH patients and from 3.9 to 5.7 mm in controls. In IIH patients, the ONSD was significantly higher compared to controls (standardized mean difference: 2.5 mm, 95% confidence interval (CI): 1.6-3.4 mm). Nine studies provided data about the presence of papilledema and the pooled prevalence was 95% (95% CI, 92-97%). CONCLUSIONS: In adults, the thickness of ONSD and the entity of ODE were significantly associated with IIH. B-mode TOS enables to noninvasively detect increased ICP and should be performed, potentially routinely, in any patient with suspected IIH.

Management of a Growth Hormone-Secreting Pituitary Macroadenoma Associated With Idiopathic Intracranial Hypertension and an Empty Sella.

Idiopathic intracranial hypertension (IIH) is a -condition associated with elevated intracranial pressure (ICP) and frequently presents with headaches, papilledema, and visual loss. Rarely, IIH has been reported in association with acromegaly. Although removal of the tumor may reverse this process, elevated ICP, especially in the setting of an otherwise empty sella, may result in a cerebrospinal fluid (CSF) leak that is exceedingly difficult to manage. We present the first case of a patient with a functional pituitary adenoma causing acromegaly associated with IIH and an otherwise empty sella and discuss our management paradigm for this rare condition.

Sleep Disturbances in Adolescents With Idiopathic Intracranial Hypertension.

BACKGROUND: We aimed to assess the presence of sleep disturbances in adolescents with idiopathic intracranial hypertension (IIH) and to determine whether demographic, anthropometric, and clinical factors are associated with disrupted sleep. METHODS: Sleep disturbances and patterns were evaluated in a cohort of adolescents (aged 12 to 18 years) with ongoing IIH and compared with a healthy age- and sex-matched control group. All participants responded to three self-rating questionnaires: the School Sleep Habits Survey (SSHS), the Pediatric Sleep Questionnaire (PSQ), and the Depression, Anxiety, and Stress Scale. The study group's demographic, clinical, laboratory, and radiological data were documented, and their association with sleep patterns was examined. RESULTS: Thirty-three adolescents with ongoing IIH and 71 healthy controls were included. There was a significantly higher prevalence of sleep disturbances in the IIH group compared with the controls (SSHS, P < 0.001 and PSQ, P < 0.001), as well as of their independent subscales: sleep-related breathing disorders (P = 0.006), daytime sleepiness (P = 0.04), sleep/wake disruptions (P < 0.001), and sleep-related depressive tendencies (P < 0.001). According to subgroup analyses, these differences were also present between the normal-weight adolescents but not between the overweight IIH and control adolescents. No differences were found in the demographic, anthropometric, and IIH disease-related clinical measures between individuals with IIH with disrupted and normal sleep patterns. CONCLUSIONS: Sleep disturbances are common among adolescents with ongoing IIH, irrespective of their weight and disease-related characteristics. Screening adolescents with IIH for sleep disturbances is recommended as part of their multidisciplinary management.

Evaluation of peripheral and central olfactory regions by MRI in patients with idiopathic intracranial hypertension.

OBJECTIVES: We investigated the peripheral and central smell regions in patients with idiopathic intracranial hypertension (IIH) by cranial MRI. METHODS: In this retrospective study, cranial MRI images of 43 adult patients with IIH (Group 1) and 43 healthy adults without IIH (Group 2) were included. In both groups, peripheral [Olfactory bulb (OB) volume and Olfactory sulcus (OS) depth] and central smell regions (insular gyrus and corpus amygdala area, and thalamus volume) were measured in cranial MRI. RESULTS: Bilateral OB volume and insular gyrus area, and right corpus amygdala and thalamus volumes of the IIH group were significantly lower than those of the control group (p < 0.05). In the IIH group, OB volume of the right side was significantly lower, and insular gyrus area of the right side was significantly higher than those of the left side (p < 0.05). In the IIH group, there were positive correlations between OB volumes; OS depths; insular gyrus areas; corpus amygdala areas; and thalamus volumes bilaterally (p < 0.05). In older patients, right OS depth and right corpus amygdala area decreased (p < 0.05). CONCLUSION: In conclusion, IIH may be related to olfactory impairment. Cranial MRI images showed a decrease in peripheral (OB volume) and central (insular gyrus and corpus amygdala area and thalamus volume) smell regions. To prevent olfactory impairment in IIH patients, treatment should be done in IIH patients to decrease intracranial pressure. It is very important to prevent the circulation of CSF with increased pressure between the sheets of the olfactory nerve in IIH patients.

Idiopathic Intracranial Hypertension in a Morbidly Obese Young Female Managed With Bariatric Surgery.

Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology characterized by raised intracranial pressure (ICP) and papilledema, also known as pseudotumor cerebri. Morbid obesity mainly affects women of childbearing age, which makes it a consistent risk factor for the development of IIH in these individuals. Consequently, the higher the body mass index (BMI) the higher the risk of IIH. We report a case of a 30- year-old female with symptomatic idiopathic IIH and malfunctioning ventriculoperitoneal (VP) shunt, associated with obesity, who underwent a bariatric sleeve gastrectomy, which led to weight loss and a significant improvement in her IIH. Our objective is to better understand the efficacy of bariatric surgery as a treatment for IIH.

Cisternotomy and Liliequist's Membrane Fenestration as a Surgical Treatment for Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): A Case Report.

Subarachnoid basal cistern opening (cisternotomy) is used during many microsurgical operations to relax the brain by removing or diverting cerebrospinal fluid (CSF). Recently, cisternotomy has been used in patients with traumatic brain injury to improve outcomes due to its ability to decrease intracranial pressure (ICP) and brain edema by diverting CSF. Theoretically, another condition that can benefit from cisternotomy is idiopathic intracranial hypertension (IIH) as it presents with manifestations of increased ICP, such as headache, vomiting, and papilledema. Here, we discuss the case of a 39-year-old woman with IIH who presented with headache, nausea, and papilledema in the setting of maximally tolerated medical management after five months of shunt removal due to infection. The patient did not want to proceed with the replacement of her shunt and therefore underwent a right eyebrow craniotomy for cisternotomy, lamina terminals fenestration, and Liliequist's membrane opening. Postoperatively, her symptoms improved completely. She was off acetazolamide altogether at the three-month follow-up and no longer had pseudotumor cerebri headaches. This case report demonstrates the use of cisternotomy to relieve the manifestations of increased ICP and its potential as a surgical option for patients with IIH.

A case report of cerebral venous sinus thrombosis presenting with rapidly progressive dementia.

Background: Cerebral venous sinus thrombosis (CVST) is a rare but serious and treatable cause of neurologic symptoms. Due to the variable clinical presentation, CVST was often misdiagnosed. According to published case reports, common clinical manifestations of CVST include headache, focal neurological deficit, epilepsy, papilledema, etc. It is rare, nevertheless, to mention cases of rapidly progressive dementia (RPD). Case presentation: We reported a case of a 62-year-old retired male accountant, a Han Chinese from eastern China, who initially presented with slow response and memory decline. Until 2 months later, his memory declined and slow response deteriorated significantly, and he could not even complete simple tasks like brushing his teeth, washing his face, washing his feet, and dressing himself, and sometimes developed fecal incontinence. His neuropsychological test demonstrated severe cognitive decline. The cerebrospinal fluid (CSF) studies revealed markedly high opening pressure (260 mm of water), and coagulation tests indicated a mild elevation of D-Dimer of 1.19 mg/L. The magnetic resonance venography (MRV) showed thrombosis of the left transverse sinus, sigmoid sinus, and jugular venous bulb and was diagnosed as CVST. He switched from subcutaneous low molecular weight heparin (LMWH) and transitioned to oral anticoagulants at the time of discharge. The repeated CSF studies revealed normal opening pressure. After 5 days of anticoagulant treatment, his symptoms considerably improved, and a 1-month follow-up revealed that he had fully healed with no signs of recurrence. Conclusion: This case demonstrated the clinical heterogeneity of CVST, which should be taken into account for differential diagnosis of RPD. This case study also offered fresh data for the categorization of the clinical traits and the diagnosis of CVST.

Micronutrient Deficiencies Presenting with Optic Disc Swelling Associated with or without Intracranial Hypertension: A Systematic Review.

Idiopathic intracranial hypertension (IIH) is a neurological disorder characterised by optic disc swelling secondary to raised intracranial pressure (ICP) of unknown cause. Obesity is the most established and prevalent risk factor in developed countries. As obesogenic diets are high in calories and nutrient-poor, there may be associated nutritional deficiencies that contribute to the clinical presentation of IIH. Yet none, aside from iron deficiency, are currently included in the inclusion or exclusion criteria for the diagnosis of IIH. Our primary aim was to determine which micronutrient deficiencies, aside from iron deficiency, could present with optic disc swelling associated with or without intracranial hypertension that could potentially meet current IIH diagnostic criteria. To this end, we conducted a systematic search of articles published between 1 January 1980 and 18 December 2020 reporting cases of optic disc swelling associated with micronutrient deficiencies. In total, 65 cases met the eligibility criteria from initial searches: all were case reports and case series with a high risk of bias. Our findings suggest that patients with IIH or unexplained optic disc swelling ought to be screened, investigated, and treated for associated micronutrient deficiencies in vitamin A, B1 and B12; and weight loss interventions in IIH patients ought to promote better nutrition in addition to overall calorie restriction.

Papilledema and Anemia: A Rare Association.

Papilledema is the swelling of the optic disc due to the transmission of intracranial pressure through the optic nerve. It can occur as a symptom of various intracranial pathologies that elevate the intracranial pressure. Even though anemia has been described as an association with raised intracranial pressure, the exact causal relationship between the two has not been well established in the literature. We present one such unusual case wherein a 21-year-old female patient, who complained of headaches, was found to have papilledema and severe anemia. After an unyielding workup for secondary causes, the rare association between papilledema and anemia was considered and prompt hemoglobin correction was ensued. Subsequently, resolution of papilledema and improvement in the visual acuity of the patient was observed. Although the causal relationship between anemia and papilledema has not been thoroughly explained, appropriate workup and correction of anemia play an integral role in the management of patients with papilledema and the prevention of long-term ocular complications.