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Idiopathic intracranial hypertension ((IIH) is characterized by increased cerebrospinal fluid pressure of unknown cause. It is predominantly a disease of women in the childbearing years. Although the cause of IIH remains obscure, it has become clear that loss of visual function is common and patients may progress to blindness if untreated. Diagnosi...
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... obscuring the retina adjacent to the optic disc. The temporal border of the optic disc is spared, presumably because of the fine caliber of these axons (Fig. 3). The C-shaped halo becomes circumferential with grade 2 papilledema (Fig. 4). In grade 3 papilledema, there is complete obscuration of at least 1 major vessel as it leaves the optic disc (Fig. 5). With the increased optic disc edema of grade 4 there is complete obscuration of at least 1 major vessel on the optic disc (Fig. 6). Grade 5 is characterized by total obscuration of at least 1 vessel on the disc and leaving the disc and at least partial obscuration of all major vessels leaving or on the disc (Fig. ...
Similar publications
Pseudotumor cerebri syndrome (PTCS) is a condition characterized by raised cerebrospinal fluid pressure in the setting of evidently normal brain parenchyma and stable composition of the cerebrospinal fluid. The pathophysiology of this syndrome is complex and not yet fully understood. Idiopathic intracranial hypertension (IIH), which is the primary...
Introduction:
Pseudotumor cerebri causes symptoms of intracranial hypertension and visual alterations secondary to damage to the optic nerve and the retina.
Case Report:
Hispanic 11-year-old male, obese. Presents to ER with recurrent visual alterations and vomit. Besides arterial hypertension and fundoscopy with hemorrhages, exudate, papilledema a...
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...
Purpose
To quantify the effects of CSF pressure alterations on intracranial venous morphology and hemodynamics in idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension (SIH) and assess reversibility when the underlying cause is resolved.
Methods
We prospectively examined venous volume, intracranial venous blood flow a...
Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype or an atypical disease course should alert the physician...
Citations
... The etiology of CSF leaks can affect ITF accuracy during localization, which may be due to intrinsic differences in the patho-etiological mechanisms of the leak. For example, in idiopathic intracranial hypertension, there is a dysfunction in the CSF absorptive mechanism of the arachnoid granulations or extracranial lymphatics [40]. In patients with trauma, CSF depletion and subsequent intracranial hypotension can limit the complete dissemination and visualization of fluorescein at the skull base. ...
... Another potential explanation for traumatic leaks is the formation of leptomeningeal fibrosis due to inflammatory reactions to blood products when there is hemorrhage in the subarachnoid space. This could restrict [20,40,41]. The ITF process may also be affected by insufficient time from intrathecal injection to endoscopic intervention or uneven fluorescein distribution within the subarachnoid space [17]. ...
Purpose
The intraoperative detection of cerebrospinal fluid (CSF) leaks during endoscopic skull base surgery is critical to ensure watertight sealed defects. Intrathecal fluorescein (ITF) is a valuable adjunct to intraoperative investigation. Hence, our aim is to summarize the evidence of the efficacy of ITF as an accurate diagnostic modality and reconstruction guide for non-congenital skull base defects.
Methods
Using the Cochrane Central, MEDLINE, and Embase databases, we identified studies involving the use of ITF in non-congenital CSF leaks which were published until November 2023. The STATA 18 software was used for meta-analysis.
Results
Fourteen studies met the inclusion criteria, in which seven studies were included in the meta-analysis. ITF was used in 1898 (90.3%) of patients, with a detection rate of 88.1%. The overall detection rate of non-congenital CSF leaks among ITF concentrations of 5% and 10% had a statistically significant pooled effect size of 2.6 (95% CI = 2.25, 2.95), while when comparing the ITF to other alternative radiological tests, it was not statistically significant with a mean difference of 0.88 (95% CI = − 0.4, 2.16). Moreover, the pooled prevalence was statistically significant in regards of the complications associated with ITF with an effect size of 0.6 (95% CI = 0.39, 0.82), indicating that 60% of patients who underwent ITF would experience at least one of the measured complications.
Conclusion
ITF is considered as an efficient tool in localizing skull base defects. However, there was no significant results when comparing the ITF to other alternative radiological tests. Accordingly, if the ITF intervention is indicated, patients should be carefully selected based on their clinical need.
... 0.5-2 per 100,000 and is in a rising trend. [14][15][16] It affects women unjustifiably more than men, and with elevated BMI. In children, however, both genders are equally affected. ...
Key Clinical Message
Pseudotumor cerebri (PTC) encompasses a constellation of symptoms caused by elevated intracranial pressure of unclear etiology. Various associations have been described, rarely hypovitaminosis D. Vitamin D deficiency should be considered as a potential etiology of neurological manifestations like PTC and seizures in children. Early diagnosis and correction of vitamin D deficiency is key to preventing morbidity and achieving good outcomes.
... Papilledema, characterized by optic disc swelling resulting from increased ICP, is a hallmark sign of IIH and can lead to visual impairment if left untreated. Diagnosis of IIH typically involves a comprehensive approach, including a thorough patient history, detailed physical examination, and diagnostic imaging tests such as CT or MRI scans, along with a lumbar puncture to measure CSF pressure [5,22,23]. ...
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.
... Patients usually experience symptoms like headaches, blurred vision, sensitivity to light, ringing in the ears, or double vision, and are sometimes asymptomatic [27,28]. Diagnosis involves measuring cerebrospinal fluid pressure, using imaging techniques, and checking cerebrospinal fluid chemistry, in addition to finding optic disc swelling [29]. However, only 10% of our patients with high ICP had IIH, and this is because our study focused on cancer patients. ...
... Typical optic neuritis is often associated with multiple sclerosis (MS), an inflammatory demyelinating disease. Atypical optic neuritis can be due to infections, inflammation, or autoimmune causes [29,30]. The incidence of optic neuritis is around 1-2 per 100,000 people and is more common in young white women [29]. ...
... Atypical optic neuritis can be due to infections, inflammation, or autoimmune causes [29,30]. The incidence of optic neuritis is around 1-2 per 100,000 people and is more common in young white women [29]. However, it was less common in our study because we focused on cancer patients, who have different characteristics than the general population. ...
Purpose: To analyze the etiology and implications of optic disc swelling in cancer patients treated at a specialized tertiary cancer center in Jordan. Methods: This was a retrospective study of all cancer patients who had optic disc swelling between January 2019 and December 2020 at King Hussein Cancer Center (KHCC). Patients’ data included age, sex, laterality, visual acuity, and the underlying cause and management for the optic disc swelling. Results: Optic disc swelling was present in 58 cancer patients (96 eyes), with 38 (65%) having bilateral involvement. Among these, 33 (57%) were female, and 43 (74%) were ≤40 years old. At diagnosis, 58 (63%) eyes had a best-corrected visual acuity (BCVA) better than 0.5, improving to 73 (76%) eyes at the last follow-up. High intracranial pressure (ICP) was the most common primary cause (30 patients/52%), followed by tumor infiltration of the optic nerve (10 patients/17%), optic nerve compression (7 patients/12%), and optic nerve inflammation (5 patients/9%). Four patients had pseudopapilledema. Among the 30 patients with high ICP, CNS tumors were predominant (21 patients/70%), with only 3 having idiopathic intracranial hypertension. Medications, including ATRA (All-Trans Retinoic Acid) and systemic steroids, contributed to increased ICP in six patients (20%). BCVA was less than 0.5 in all eyes (100%) affected by tumor infiltration, optic nerve inflammation, and ischemic optic neuropathy, while only eight eyes (14%) with optic disc swelling due to elevated ICP had a BCVA less than 0.5 (p < 0.0001). Management included steroids (53 patients/91%), acetazolamide (30 patients/52%), chemotherapy (20 patients/34%), radiation therapy (13 patients/22%), frequent lumbar punctures (12 patients/21%), and surgery (28 patients/48%). Visual acuity improved in 40 eyes (42%), with only 4 eyes (4%) experiencing deterioration. At a 12-month median follow-up period, 11 (19%) patients were dead, 10 (10%) eyes had poor vision (BCVA less than 0.1), and 21 (22%) eyes had BCVA 0.5 or better. Conclusions: Various underlying pathologies can induce optic disc swelling in cancer patients, a grave condition capable of causing vision loss. Notably, tumor infiltration of the optic nerve tends to result in more profound visual impairment compared to papilledema due to elevated ICP. Timely detection is crucial, and immediate symptomatic treatment followed by addressing the underlying cause is essential to prevent irreversible optic nerve damage and vision loss in cancer patients.
... It is a rare disorder that affects one to three people in every 100,000 [8]. It may be a primary disorder, predominantly seen in obese female patients of childbearing age, or it may be secondary to various medical conditions (Addison's disease, hypoparathyroidism, obstructive sleep apnea), obstruction of the venous system (cerebral venous sinus thrombosis, jugular vein thrombosis), medications (steroids, lithium, amiodarone), or infections (HIV, borreliosis) [9]. ...
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.
... Idiopathic intracranial hypertension (IIH) is a rare headache syndrome mainly observed in overweight women of childbearing age [1][2][3][4]. The defining feature is an elevated intracranial pressure (ICP), without an identifiable source of hydrocephalus or cerebral mass lesions. ...
Background
Neurofilament light chain (NfL) reflects axonal damage in neurological disorders. It has recently been evaluated in idiopathic intracranial hypertension (IIH). A biomarker indicating the severity of optic nerve damage in IIH could support diagnostic accuracy and therapeutic decisions.
Methods
We retrospectively reviewed NfL concentrations in the cerebrospinal fluid (CSF) of 35 IIH patients and 12 healthy controls, who had received diagnostic workup for IIH in our clinic. The diagnosis of IIH was made according to the modified Friedman criteria for IIH and for IIH without papilledema Friedman DI et al Neurol 81:1159–1165 (2013) [1]. NfL in the CSF (CSF-NfL) was correlated with the severity of papilledema and with CSF opening pressure.
Results
CSF-NfL correlated with CSF opening pressure at the time of collection. In patients with IIH and moderate or severe papilledema, CSF-NfL was significantly increased compared to patients with mild or no papilledema. Healthy controls with raised intracranial pressure showed no relevant elevation of CSF-NfL.
Conclusion
CSF-NfL appears to correlate with the severity of papilledema in IIH and with CSF opening pressure and may therefore be a predictor of optic nerve damage in IIH patients.
... Because 10% of IIH patients present with headaches, and papilledema that can result in permanent vision loss, treatment is mainly symptomatic and preventive [18]. Acetazolamide is the mainstream treatment [17]. ...
... As it allows a decrease of the volume of CSF by promoting diuresis and due to the limited side effect spectrum being limited to oral or limb paresthesias, and weight gain beyond the dose of 500 mg per day [18], acetazolamide was ideal for our patient and improved her headache after 24 hours of initiation. Papilledema was improved three days after treatment with acetazolamide. ...
... Papilledema was improved three days after treatment with acetazolamide. The duration of treatment is not standardized and in most cases is guided by the symptomatology of the patients [18,19]. In this case, the patient stopped the medication after three weeks on her own. ...
Idiopathic intracranial hypertension (IIH), called pseudotumor cerebri, could cause postpartum headaches. Generally, this diagnosis is idiopathic and treatment is mainly medical to avoid serious complications of possible vision loss. In this paper, we report the case of a 24-year-old lady who developed a similar constellation of symptoms and was diagnosed with this condition. Postpartum, the patient demonstrated symptoms of headache and vision disturbances. Workup ruled out infectious processes and intracranial pathologies. Normal cranial magnetic resonance imaging (MRI) and high cerebrospinal fluid (CSF) pressure during lumbar puncture led to a diagnosis of IIH. Initiation of medication allowed rapid improvement of symptoms and evaded imminent morbidity. Further discussion in light of the latest findings of the literature is held after the presentation of the case. This case sheds light on the importance on importance of fundoscopy in patients demonstrating new-onset headaches especially postpartum with the absence of intracranial pathologies.
... This generally has less effect on visual acuity and pupillary reflexes, unlike optic neuritis due to vasculitis, which exhibits relatively more afferent pupillary disturbance and significant vision loss. 88,89 Thus, unlike optic nerve oedema due to vasculitis, patients with optic papilloedema due to increased intracranial pressure can be more effectively treated. ...
Ocular lesions due to Brucella infection are uncommon and easily overlooked in clinical management, but must be differentiated from non-infectious eye diseases and treated promptly to protect the patient’s vision. We reviewed the relevant literature and identified 47 patients with ocular complications of Brucella infection. Among them, 28 showed ocular neuropathy, 15 presented with uveitis, and four patients displayed other ocular symptoms. Ocular symptoms accompanying Brucella infection require prompt diagnosis and treatment. The main methods of diagnosis are intraocular fluid tests and blood tests. Early diagnosis and treatment with suitable antibiotics are central to protecting the patient’s vision. Notably, in terms of mechanism of injury, Brucella infection is chronic and cannot be eliminated by phagocytes, and can cause damage to the eye by inducing autoimmune reactions, antigen-antibody complex production, release of endogenous and exogenous toxins, and bacterial production of septic thrombi in the tissues. In this review, we summarize the ocular symptoms, diagnosis, treatment and prognosis of Brucella infection, and discuss the mechanisms of Brucella in ocular lesions, providing a reference for the diagnosis and treatment of Brucella ocular lesions.
... Previous literature supports these findings as it has shown that approximately 70-80% of IIH patients are obese and over 90% are overweight [26][27][28]. Another study suggests that as many as 90% of patients with IIH are obese [29]. Study done by Daniels et al. concluded that those with (BMI <30) are also at higher risk of IIH [18]. ...
Background. Idiopathic intracranial hypertension (IIH) also termed pseudotumor cerebri syndrome (PTCS). It mainly affects young overweight women of the reproductive age group. There is a scarcity of literature on IIH among the Indian population. Objective. To evaluate the clinical, demographic profile, laboratory parameters and outcomes of Idiopathic Intracranial hypertension (IIH) patients. Materials and method. The current study was a prospective observational study on 43 patients who were diagnosed as primary PTCS presenting to the neurology department of Sri Ramachandra Medical College and Research Institute from December 2019 to August 2021. Patients were assessed for BMI, papilledema, CSF manometry, MRI brain with MR venography was done. Co-guide software, V.1.03, was used for statistical analysis. Results. The mean age was 33.12 ± 12.29 years (ranged 14 to 55) in the study population. There were 5(11.63%) males and 38(88.37%) females. The mean BMI was 26.72 ± 3.56 kg/m2 (ranged from 17.40 to 34.20). The mean duration of headache was 28.41 ± 26.23 days (ranged 3 to 120) in the study population, and 39(90.69%) had papilledema. The mean CSF manometry was 29.6 ± 5.88(ranged from 16 to 42) cmH2O. Conclusion. In the Indian subcontinent, obesity may not be considered as a major risk factor in the causation of IIH.
... IIH is a disorder characterized by abnormally increased CSFP for unknown reasons. The age-and sex-adjusted annual incidence of IIH was reported to be 0.9 per 100,000 persons and 3.5 per 100,000 in females 15-44 years of age, predominantly affecting obese women of childbearing age (18,19). Common symptoms of IIH include headache (76-94%), transient visual obscurations (68-72%), pulsatile tinnitus (PT) (52-61%), and neck and back pains (42-53%) (20)(21)(22). ...
The clinical and hemodynamic characteristics of venous pulsatile tinnitus (PT) patients with normal or elevated cerebrospinal fluid pressure (CSFP) have not been clearly differentiated. This study aimed to explore CSFP among patients with PT as the solitary symptom, as well as quantitatively and qualitatively assess the role of the degree of transverse sinus (TS) stenosis and jugular hemodynamics in venous PT patients. A total of 50 subjects with venous PT with or without sigmoid sinus wall anomalies (SSWAs) were enrolled in this study. In addition to radiologic assessments for TS stenosis and invagination of arachnoid granulation (AG) in TS, CSFP and jugular hemodynamics were measured via cerebrospinal fluid (CSF) manometry and Doppler ultrasound. Apart from group comparisons and correlation analyses, multivariate linear regression, and receiver operating characteristic (ROC) models were used to identify the sensitivity and specificity of the index of transverse sinus stenosis (ITSS) and hemodynamic variables with inferential significance. The mean CSFP of all cases was 199.5 ± 52.7 mmH2O, with no statistical difference in CSFP between the diverticulum and dehiscence groups. Multivariate linear regression analysis demonstrated that CSFP was linearly correlated with ITSS and pulsatility index (PI). ROC analysis showed that the area under the ROC curve of PI was 0.693 at 200 mmH2O threshold, and the best PI cut-off value was 0.467, with a sensitivity of 65.7% and specificity of 81.8%. For 250 mmH2O threshold, the area under the ROC curve of PI was 0.718, and the best PI cut-off value was 0.467 with a sensitivity of 68.4% and specificity of 75.0%. Additionally, the area under the ROC curve of ITSS was 0.757, and the best ITSS cutoff value was 8.5 (p = 0.002, 95% CI = 0.616–0.898) with a sensitivity of 72.4% and specificity of 75.0% at 200 mmH2O threshold. In conclusion, patients with venous PT as the only presenting symptom should be suspected of having borderline or increased CSFP when they present with high ITSS, BMI and low PI. Further, AG in TS without encephalocele and empty sellae are not limiting findings for differentiating the level of CSFP in patients with venous PT.
