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Neurologia I Neurochirurgia Polska 2024Spontaneous CSF leak is a known complication of idiopathic intracranial hypertension (IIH). Patients with CSF rhinorrhea present a unique challenge within the IIH... (Review)
Review
INTRODUCTION
Spontaneous CSF leak is a known complication of idiopathic intracranial hypertension (IIH). Patients with CSF rhinorrhea present a unique challenge within the IIH population, as the occurrence of a leak can mask the typical IIH symptoms and signs, complicating the diagnosis. Treatment of leaks in this population can also be challenging, with the risk of rhinorrhea recurrence if intracranial hypertension is not adequately treated.
OBJECTIVE
The aim of this narrative review was to examine current literature on the association between spontaneous CSF rhinorrhea leaks and IIH, focusing on key clinical features, diagnostic approaches, management strategies, and outcomes.
MATERIAL AND METHODS
A literature search was executed using the PubMed and Scopus databases. The search was confined to articles published between January 1985 and August 2023; extracted data was then analysed to form the foundation of the narrative review.
RESULTS
This search yielded 26 articles, comprising 943 patients. Average age was 46.8 ± 6.5 years, and average body mass index was 35.8 ± 4.8. Most of the patients were female (74.33%). Presenting symptoms were rhinorrhea, headaches and meningitis. The most common imaging findings were empty sella and encephalocele. The standard treatment approach was endoscopic endonasal approach for correction of CSF rhinorrhea leak, and shunt placement was also performed in 128 (13%) patients. Recurrences were observed in 10% of cases.
CONCLUSIONS
The complex relationship between spontaneous CSF leaks and IIH is a challenge that benefits from multidisciplinary evaluation and management for successful treatment. Treatments such as endoscopic repair, acetazolamide, and VP/ /LP shunts reduce complications and recurrence. Personalised plans addressing elevated intracranial pressure are crucial for successful outcomes.
Topics: Humans; Female; Adult; Middle Aged; Male; Pseudotumor Cerebri; Cerebrospinal Fluid Rhinorrhea; Intracranial Hypertension; Acetazolamide; Endoscopy; Cerebrospinal Fluid Leak; Retrospective Studies
PubMed: 38393958
DOI: 10.5603/pjnns.98054 -
Journal of the Neurological Sciences Apr 2024Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure and primarily affects obese women of reproductive age. Venous sinus... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure and primarily affects obese women of reproductive age. Venous sinus stenting (VSS) is a surgical procedure used to treat IIH, but its safety and efficacy are still controversial.
METHODS
A systematic review and meta-analysis were conducted following PRISMA guidelines. Multiple databases were searched for studies evaluating the safety and efficacy of VSS in IIH patients and meta-analysis was performed to pool the data.
RESULTS
A total of 36 studies involving 1066 patients who underwent VSS were included. After VSS, a significant reduction in trans-stenotic gradient pressure was observed. Patients also showed significantly lower cerebrospinal fluid (CSF) opening pressure. Clinical outcomes demonstrated improvement in tinnitus (95%), papilledema (89%), visual disturbances (88%), and headache (79%). However, 13.7% of patients experienced treatment failure or complications. The treatment failure rate was 8.35%, characterized by worsening symptoms and recurrence of IIH. The complications rate was 5.35%, including subdural hemorrhage, urinary tract infection, stent thrombus formation, and others.
CONCLUSION
VSS appears to be a safe and effective treatment option for IIH patients who are unresponsive to medical therapy or have significant visual symptoms. However, long-term outcomes and safety of the procedure require further investigation.
Topics: Humans; Female; Pseudotumor Cerebri; Cranial Sinuses; Treatment Outcome; Headache; Stents; Intracranial Hypertension; Retrospective Studies
PubMed: 38457956
DOI: 10.1016/j.jns.2024.122948 -
Neuro-ophthalmology (Aeolus Press) 2024Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative... (Review)
Review
Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative review of fulminant IIH with the aim of better characterising fulminant IIH presentation and visual outcomes. SCOPUS and PubMed were searched for papers referencing IIH, benign intracranial hypertension, or pseudotumour cerebri. Abstracts were screened for rapid degradation in vision. All studies were required to meet both the modified Dandy and fulminant IIH criteria. Thirty-six studies met the inclusion criteria. Demographics, treatments, and visual outcome data were collected. Case studies made up 69% of the studies and 31% were case series. In total, 72 patients with fulminant IIH were reported, of which 23.6% were paediatric and 96% were female. Surgical intervention occurred in 85% of patients. Anaemia was present in 11% of patients and 85.7% of paediatric patients had a sixth cranial nerve palsy. In conclusion, we propose the following practice guidelines to assist in diagnosing and treating fulminant IIH patients: 1) patients who present with optic disc oedema require urgent visual field testing to evaluate for vision loss; 2) a paediatric patient presenting with a sixth cranial nerve palsy should have a comprehensive eye examination; 3) fulminant IIH can occur in patients with a normal body mass index; and 4) anaemia should be tested for in the setting of fulminant IIH. As little is known about the optimal treatment mechanisms for this presentation, multi-institutional and international collaborations will be a critical step for future research.
PubMed: 38933750
DOI: 10.1080/01658107.2023.2301358 -
The Neuroradiology Journal Dec 2023To investigate the role of diffusion tensor imaging (DTI) of optic pathway in patients with idiopathic intracranial hypertension (IIH).
PURPOSE
To investigate the role of diffusion tensor imaging (DTI) of optic pathway in patients with idiopathic intracranial hypertension (IIH).
PATIENTS AND METHODS
This study included 41 IIH patients and 22 controls. They underwent DTI of the optic nerve (ON) and optic radiation (OR). Their fractional anostrophy (FA) and mean diffusivity (MD) were calculated by two reviewers and correlated with papilledema grade.
RESULTS
The optic nerve mean FA and MD of patients for reviewer-1 were 0.21 ± 0.047 and 2.189 ± 0.52 ×10 mm/s and for reviewer-2 were 0.216 ± 0.047 and 2.17 ± 0.54 ×10 mm/s. The mean ON FA and MD of controls for reviewer-1 were 0.33 ± 0.048 and 1.29 ± 0.26 ×10 mm/s and for reviewer-2 were 0.34 ± 0.05 and 1.3 ± 0.26 ×10 mm/s. There was significant difference in FA and MD between patients and controls ( < 0.00001). The OR mean FA and MD of patients for reviewer-1 were 0.61 ± 0.03 and 2.26 ± 0.55 ×10 mm/s and for reviewer-2 were 0.6 ± 0.03 and 2.24 ± 0.57 ×10 mm/s The mean OR FA and MD of controls for reviewer-1 were 0.6 ± 0.03 and 2.19 ± 0.49 ×10 mm/s and for reviewer-2 were 0.6 ± 0.03 and 2.18 ± 0.49 ×10 mm/s. There was no significant difference in FA and MD obtained in patients and controls. Both the FA and the MD of the ON showed strong correlation with the papilledema grade (r = -0.8 and 0.951, respectively).
CONCLUSION
Our findings suggest that, IIH is associated mainly with pre-chiasmatic or ON involvement rather than post-chiasmatic parenchymal or OR involvement. DTI MD and FA parameters of the ON may be a reliable imaging biomarker for diagnosis of IIH and well correlated with papilledema grades.
Topics: Humans; Diffusion Tensor Imaging; Papilledema; Pseudotumor Cerebri; Optic Nerve; Diffusion Magnetic Resonance Imaging
PubMed: 37401887
DOI: 10.1177/19714009231187346 -
Brain Sciences Nov 2023Idiopathic intracranial hypertension (IIH) has its highest prevalence among women of childbearing age and therefore frequently coincides with pregnancy. This...
Idiopathic intracranial hypertension (IIH) has its highest prevalence among women of childbearing age and therefore frequently coincides with pregnancy. This retrospective cohort study aimed to explore the impact of pregnancy on the clinical course, ophthalmologic findings and on the therapeutic management of IIH patients. Individual patient records were reviewed for neuro-ophthalmologic findings, treatment strategy, adherence to therapy and pregnancy complications. Sixteen patients with 19 documented pregnancies were identified. The visual acuity, visual field defects and the grade of papilledema at baseline and after pregnancy were compared. The visual acuity and visual field mean deviation at baseline and at follow-up after pregnancy did not significantly differ. Papilledema at baseline was more pronounced in patients who had been diagnosed with IIH during pregnancy than in patients with established IIH. In this cohort, the visual acuity and the visual field were not lastingly impacted by pregnancy. The adherence to therapy was low, with 69% discontinuing treatment or medication.
PubMed: 38137064
DOI: 10.3390/brainsci13121616 -
Journal of the Neurological Sciences Sep 2023Fulminant idiopathic intracranial hypertension (FIIH) is characterized by rapid, severe, progressive vision loss and often treated surgically. Cerebral transverse venous...
BACKGROUND
Fulminant idiopathic intracranial hypertension (FIIH) is characterized by rapid, severe, progressive vision loss and often treated surgically. Cerebral transverse venous stenting (CTVS) is efficacious in IIH patients, but emergent CTVS in FIIH is rarely reported. We present our experience with emergent CTVS in patients with FIIH.
METHODS
Since 01/2019, an institutional protocol allowed emergent CTVS in FIIH patients with bilateral transverse sinus stenosis and gradient pressure > 15 on digital subtraction angiography (DSA). We retrospectively analyzed a prospective registry of all IIH patients with details of neurological and neuro-ophthalmological assessments before and after treatment, and subjective assessments of headache and tinnitus were made pre-and post-procedure.
RESULTS
259 IIH patients, including 49 who underwent CTVS, were registered. Among them, five female patients met inclusion criteria for FIIH and underwent emergent CTVS. FIIH patients were younger (18.8 ± 1.64 vs 27.7 ± 4.85, p < 0.01), mean BMI was lower (30.8 ± 10.57 vs 34.6 ± 4.3, p < 0.01), and lumbar puncture opening pressure higher (454 ± vs 361 ± 99.4, p < 0.01) than that of IIH patients. They presented with acute visual loss, severe headache, papilledema, significant bilateral transverse sinus stenosis on CT-venography, and mean dominant side gradient pressure of 26.4 ± 6.2 on DSA. CTVS was performed without significant complications, resulting in remarkable improvement in headache, optical coherence tomography, and visual fields within 1 week. At 1-year follow-up (four patients) and 6-month follow-up (1 patient), there was complete resolution of papilledema and headache, and marked improvement in visual acuity.
CONCLUSIONS
In these patients, emergent-CTVS was a safe and effective treatment option for FIIH. Further evaluation is warranted.
Topics: Humans; Female; Pseudotumor Cerebri; Papilledema; Retrospective Studies; Constriction, Pathologic; Headache; Stents; Vision Disorders; Cranial Sinuses; Intracranial Hypertension
PubMed: 37572407
DOI: 10.1016/j.jns.2023.120761 -
Multiple Sclerosis and Related Disorders Jan 2024Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently defined demyelinating disorder with a rapidly evolving clinical spectrum. Recently,...
BACKGROUND
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently defined demyelinating disorder with a rapidly evolving clinical spectrum. Recently, consensus criteria have been proposed (Banwell et al., 2023) to help with disease diagnosis. However, validation of the proposed criteria in real-life MOGAD patients is lacking. In this study, we applied the proposed criteria to an institutional cohort of MOG antibody-positive patients.
METHODS
A retrospective study was conducted at a tertiary neuroimmunology clinic from 2018 to 2023. Patients who had at least one core clinical feature of MOGAD and positive serum MOG antibody by cell-based assay were included. Demographics and clinical data were recorded and analyzed. Cases were divided into definite MOGAD, questionable MOGAD, and false-positive MOG antibody as determined by the treating neuroimmunology and/or neuro-ophthalmology specialists prior to applying the new MOGAD criteria by an independent investigator. We then calculated the sensitivity, specificity, positive predictive value, and negative predictive value of the new criteria compared to the treating physicians' assessment.
RESULTS
A total of 27 patients were included of which, 19 (70.4%) were female, the average age of the sample was 44 +/- 15 years. High titer MOG antibody (≥ 1:100) was found in 11 patients (40.7%); low titer (< 1: 100) in 13 (48.1%), and unreported titer in 3 patients. As determined by expert opinion; 18 (66.7%) were identified as definitive MOGAD, 6 (22.2%) as false-positive MOG antibody, and 3 (11.1%) as questionable MOGAD. All 18 patients identified by clinicians as definite MOGAD met the new 2023 criteria. Of the 9 patients with questionable MOGAD or false-positive MOG antibody, four patients met the 2023 MOGAD criteria. Those four patients had the following final diagnoses: CNS vasculitis, primary progressive MS with activity and progression, pseudotumor cerebri, and bevacizumab-induced anterior ischemic optic neuropathy in the setting of paraneoplastic retinopathy. Compared to clinician assessment, applying the 2023 MOGAD criteria to our institutional cohort yielded a sensitivity of 100%, a specificity of 55.5%, a positive predictive value of 81.5% and a negative predictive value of 100%.
CONCLUSION
These findings suggest that the 2023 MOGAD criteria are highly sensitive for detection of definite MOGAD but has modest specificity. A number of MOGAD mimickers can resemble the core clinical events of MOGAD and share similar supportive clinical and MRI features. Clinicians should practice caution when evaluating patients with low titer MOG antibody even if they meet the additional supportive features proposed by the 2023 criteria. Further studies are needed to evaluate the 2023 criteria in larger cohorts and in the pediatric population.
Topics: Child; Humans; Female; Adult; Middle Aged; Male; Retrospective Studies; Antibodies; Consensus; Pseudotumor Cerebri; Research Design; Autoantibodies
PubMed: 38043365
DOI: 10.1016/j.msard.2023.105150 -
American Journal of Ophthalmology Sep 2023To describe the racial distribution of patients diagnosed with nonarteritic anterior ischemic optic neuropathy (NAION) in 2 large urban academic centers in the United...
PURPOSE
To describe the racial distribution of patients diagnosed with nonarteritic anterior ischemic optic neuropathy (NAION) in 2 large urban academic centers in the United States.
DESIGN
Retrospective cross-sectional study.
METHODS
All patients with NAION evaluated between 2014 and 2022 in tertiary neuro-ophthalmology services in Atlanta, GA, and New York, NY, respectively, were included. In order to assess the racial accessibility to our services, similar numbers of patients with idiopathic intracranial hypertension seen in the same services were included. Self-reported race at the time of neuro-ophthalmologic examination was collected for all patients. Results were compared with data published by the US Bureau of Census for Decennial Census in 2020 for the states of Georgia and New York, and for the total population of the United States.
RESULTS
In both locations, the vast majority of patients with NAION reported being White (91.06% in Atlanta and 78.9% in New York). Only 7.27% of patients in Atlanta and 3% in New York reported being Black, and 1.24% of patients in Atlanta and 5.9% in New York reported being Asian. There were a much higher proportion of White people among NAION patients than in the group of patients with idiopathic intracranial hypertension and in each respective state and in the United States.
CONCLUSIONS
Our results show that the majority of patients with NAION are White, followed by Black and Asian races, respectively, thus confirming the NAION race distribution reported by past clinical trials.
Topics: Humans; Optic Neuropathy, Ischemic; Retrospective Studies; Cross-Sectional Studies; Pseudotumor Cerebri; New York
PubMed: 36963603
DOI: 10.1016/j.ajo.2023.03.013 -
Neurologia Feb 2024Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the...
Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the diagnosis and treatment of these 2entities in recent years. Therefore, the Spanish Society of Neurology's Headache Study Group (GECSEN) deemed it necessary to prepare this consensus statement, including diagnostic and therapeutic algorithms to facilitate and improve the management of these disorders in clinical practice. This document was created by a committee of experts belonging to GECSEN, and is based on a systematic review of the literature, incorporating the experience of the participants, and establishes practical recommendations with levels of evidence and grades of recommendation.
PubMed: 38431253
DOI: 10.1016/j.nrleng.2024.02.009 -
Neurological Sciences : Official... Dec 2023This systematic review and meta-analysis aimed to evaluate the role of B-mode transorbital ultrasonography (TOS) for the diagnosis of idiopathic intracranial... (Meta-Analysis)
Meta-Analysis Review
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.
Topics: Adult; Humans; Intracranial Hypertension; Intracranial Pressure; Optic Nerve; Papilledema; Pseudotumor Cerebri; Ultrasonography
PubMed: 37599314
DOI: 10.1007/s10072-023-07016-z