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Ophthalmic Plastic and Reconstructive...To present four female-to-male (FTM) transgender patients on testosterone therapy diagnosed with idiopathic intracranial hypertension (IIH).
PURPOSE
To present four female-to-male (FTM) transgender patients on testosterone therapy diagnosed with idiopathic intracranial hypertension (IIH).
METHODS
The authors report 4 consecutive FTM transgender patients on exogenous testosterone diagnosed with IIH at a single institution.
RESULTS
Patient 1 presented with progressive blurred vision and a central scotoma 10 weeks after starting testosterone cypionate injections for hormonal gender transition. Bilateral grade 5 papilledema was present; the patient underwent bilateral optic nerve sheath fenestration with improved vision and resolution of edema. Patient 2 presented with transient vision loss, pulsatile tinnitus, and blurred vision 13 months after starting testosterone cypionate injections. The patient had grade 4 and 3 disc edema of the right and left eyes, respectively. Patient 3 presented with headaches and pulsatile tinnitus and was on testosterone injections at an unknown dose. The examination revealed grade 1 and 2 disc edema of the right and left eyes, respectively. Patient 4 presented with decreased vision, transient visual obscurations, and daily migraines while using topical testosterone gel every other day. Color vision was reduced, and lumbar puncture revealed elevated intracranial pressure. All patients had neuroimaging findings consistent with increased intracranial pressure.
CONCLUSIONS
Testosterone therapy plays an essential role in FTM hormonal transitioning and may play a role in IIH. Patients undergoing testosterone therapy for gender transition should be informed of the possibility of developing IIH while on treatment, with obesity possibly increasing this risk. Comprehensive eye examinations should be considered in these patients before initiating hormone therapy.
Topics: Humans; Male; Female; Pseudotumor Cerebri; Transgender Persons; Tinnitus; Papilledema; Vision Disorders; Testosterone; Edema
PubMed: 36804335
DOI: 10.1097/IOP.0000000000002344 -
Journal of Neuro-ophthalmology : the... Dec 2023Venous sinus stenosis, typically at the junction of the transverse and sigmoid sinus, is increasingly recognized as a contributor to the pathophysiology of idiopathic...
BACKGROUND
Venous sinus stenosis, typically at the junction of the transverse and sigmoid sinus, is increasingly recognized as a contributor to the pathophysiology of idiopathic intracranial hypertension (IIH), whether it be the intrinsic type that does not reverse with normalization of intracranial pressure or the extrinsic type, which does. Efforts to treat the stenosis and reduce the associated transstenotic gradient through placement of a stent at the site of stenosis have been studied over the past 2 decades, primarily through retrospective studies, with variable emphasis on formal visual testing and direct assessment of poststent opening pressure. Most studies have presented evidence for utilization of stenting as an alternative to cerebrospinal fluid shunting or optic nerve sheath fenestration in patients with IIH who harbor the stenosis and are refractory to or intolerant of intracranial pressure-lowering medications, but an assessment of the current data is needed to better understand the role of stenting for this patient population.
EVIDENCE ACQUISITION
A search in PubMed was made for "IIH," "papilledema," and "venous stenting." Data pre and post stenting, including symptoms attributable to IIH, intracranial pressure, papilledema, retinal nerve fiber layer thickening on optical coherence tomography, and visual field assessment (mean deviation), were collected. Need for retreatment and complications were assessed among all studies. Studies using stenting for special circumstances, such as cerebrospinal leaks or for stenosis along anomalous vessels, were reviewed.
RESULTS
In total, 49 studies (45 retrospective and 4 prospective) and 18 case reports (with 3 or less patients) were found and included in the analysis, for a total of 1,626 patients. In 250 patients in whom poststent intracranial pressure was measured, the mean value was 19.7 cm H 2 O, reduced from a mean of 33 cm H 2 O. Transient visual obscurations resolved in 79.6% of 201 patients who complained of it, pulsatile tinnitus resolved in 84.7% of 515, diplopia resolved in 93% of 86 patients, and nonspecific visual symptoms such as "blurry vision" improved in 76.2% of 537 patients. Headaches resolved in 36% and improved in a further 40.7% of 1,105 patients in whom they were documented before stenting. Of 1,116 with papilledema, 40.8% demonstrated resolution and 38.2% improvement. The mean retinal nerve fiber layer thickness improved from 170.2 µm to 89.2 µm among 402 eyes in which optical coherence tomography was used to measure it. Among 135 eyes in which formal visual fields were performed pre and post stenting, the prestent average mean deviation of -7.35 dB improved to -4.72 dB after stenting. Complications associated with stenting included in-stent stenosis or thrombosis, subdural hematoma, intracerebral hematoma, cerebral edema, stent migration, and death. A recurrence of symptoms requiring a follow-up surgical intervention occurred in 9%.
CONCLUSIONS
A growing body of evidence supports the use of venous sinus stenting as a viable option for medically refractory IIH, especially when papilledema threatens visual function. Complication and failure rates seem to be similar to alternative surgical approaches, although serious neurological sequalae can rarely occur. Emerging studies evaluating stent type, including novel stents designed for use in the venous system, may help improve ease of the procedure and long-term success rates. Prospective head-to-head studies are needed to better understand the performance of stenting compared with other interventions.
Topics: Humans; Pseudotumor Cerebri; Papilledema; Retrospective Studies; Prospective Studies; Constriction, Pathologic; Treatment Outcome; Stents; Vision Disorders; Hypertension; Intracranial Hypertension
PubMed: 37410913
DOI: 10.1097/WNO.0000000000001898 -
Journal of Neuro-ophthalmology : the... Dec 2023
Topics: Humans; Pseudotumor Cerebri; Feeding and Eating Disorders; Retrospective Studies
PubMed: 35086124
DOI: 10.1097/WNO.0000000000001528 -
Journal of Neuro-ophthalmology : the... Dec 2023
Topics: Humans; Pseudotumor Cerebri; Intracranial Pressure; Intracranial Hypertension
PubMed: 37974367
DOI: 10.1097/WNO.0000000000001388 -
CNS Neuroscience & Therapeutics Jan 2024Previous studies have suggested that cerebral dural sinus stenosis could be a possible underlying cause of idiopathic intracranial hypertension (IIH). Venous sinus...
BACKGROUNDS
Previous studies have suggested that cerebral dural sinus stenosis could be a possible underlying cause of idiopathic intracranial hypertension (IIH). Venous sinus stenting (VSS) has emerged as a potential alternative for treating IIH related to dural sinus stenosis. However, most of the documented studies have been conducted in Western countries. In this study, we present the results of 16 Chinese IIH patients who underwent VSS treatment in our single center.
METHODS
We prospectively collected angiographic and manometric data from IIH patients who underwent angioplasty/stenting. All patients had confirmed dural sinus stenosis and had failed maximal medical therapy (MMT). Demographic, clinical, and radiological presentation, as well as long-term follow-up outcomes were collected retrospectively.
RESULTS
A total of 16 patients who underwent VSS were enrolled in the present study. Demographic data revealed a mean age of 40 (range 20-55), with 69% (11/16) being female, and a mean body mass index (BMI) of 27.05 (range 19.18-38.04) kg/m . All patients presented with papilledema and visual disturbances. During a median follow-up period of 47.5 months, 93.75% (15/16) of patients reported improvement in symptoms, although only 37.5% (6/16) experienced complete resolution. Headaches, blurred vision, and amaurosis related to increased pressure improved in 100% (8/8), 81.25% (13/16), and 75% (3/4) of patients, respectively. However, one patient suffered cerebral infarction and secondary epilepsy soon after VSS, and another patient had recurrence of symptoms due to stent wall thrombosis 2 years later.
CONCLUSIONS
The significance of venous sinus stenosis in the development of IIH may be undervalued. Our study, based on a Chinese case series, affirms the long-term safety and effectiveness of VSS in treating IIH patients with relatively lower BMI than those from Western countries.
Topics: Humans; Female; Adult; Male; Pseudotumor Cerebri; Retrospective Studies; Constriction, Pathologic; Treatment Outcome; Stents; Vision Disorders; Intracranial Hypertension
PubMed: 37469247
DOI: 10.1111/cns.14356 -
European Journal of Pediatrics Nov 2023This study aimed to identify predictors for unfavorable disease course and clinical and visual outcomes in pediatric patients with idiopathic intracranial hypertension...
UNLABELLED
This study aimed to identify predictors for unfavorable disease course and clinical and visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH). Employing a multi-tiered approach, we retrospectively analyzed clinical, ophthalmic, and neuroimaging data from patients diagnosed with IIH between 2003 and 2021. Of the 97 patients included, 56 (58%) were females. The median age was 12 years [Interquartile range (IQR) 9, 14], and the median follow-up time was 39.0 months (IQR 14.8, 90.9). Forty-two (43%) patients had an unfavorable disease course, 28 (29%) had persistence of headache at last follow-up, and 16 (18%) had a poor visual outcome, most of them with mild visual disturbances. Poor visual outcome was more common in females compared to males [16/47 (34%) vs. 0/39, p < 0.001)]. On multivariate regression analysis, female sex and disease recurrence were significantly associated with poor visual outcomes (OR: 18.5, CI:1.3-270, P = 0.03, and OR: 5.1, CI: 1.2-22.5, P = 0.03, respectively). Patients with persistent headaches exhibited lower incidence of papilledema, lower opening pressure, and fewer neuroimaging markers indicating elevated intracranial pressure.
CONCLUSIONS
This study provides insights into predictive factors for an unfavorable disease course, persistent headaches, and poor visual outcomes in patients with childhood IIH. Patients with persistent headaches may have a variant of a chronic pain syndrome warranting a different therapeutic approach.
WHAT IS KNOWN
• Childhood-onset Idiopathic Intracranial hypertension (IIH) is a heterogenous disease. The knowledge on disease trajectory and long-term outcomes and its predictors is limited.
WHAT IS NEW
• A higher opening pressure and factors suggestive of the metabolic syndrome predict an unfavorable disease course whereas female sex and disease recurrence are significantly associated with poor visual outcomes • A third of the patients diagnosed with IIH experience ongoing headaches despite achieving favorable visual outcomes. This subset, characterized by lower disease-severity indicators at onset may represent a distinct subgroup warranting a different therapeutic approach.
Topics: Male; Humans; Child; Female; Adolescent; Pseudotumor Cerebri; Retrospective Studies; Papilledema; Headache; Disease Progression
PubMed: 37691042
DOI: 10.1007/s00431-023-05173-z -
Expert Review of Neurotherapeutics Jul 2024Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema, leading to chronic headaches and visual... (Review)
Review
INTRODUCTION
Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema, leading to chronic headaches and visual disturbances. By recognizing modifiable risk factors and deploying evidence-based interventions, healthcare providers have the potential to mitigate the burden of the disease and improve patient outcomes.
AREAS COVERED
It is well known that the condition occurs in predominantly pre-menopausal females who live with obesity particularly in the context of recent weight gain. This review discusses what risk factors may contribute to the metabolic underpinnings of cerebrospinal fluid dysregulation. There are a number of disease associations that are important to screen for as they can alter management.
EXPERT OPINION
There is emerging evidence to suggest that idiopathic intracranial hypertension is a systemic metabolic disease and it is unknown what are all the risk factors are that precipitate the condition. Targeting certain hardwired risk factors is unachievable. However, as recent weight gain has been identified as a predominant risk factor for the development of the disease and relapse, modification of body weight should be the primary aim of management. Insightful research into the involvement of the neuroendocrine axis driving cerebrospinal fluid dysregulation now has the potential for the development of therapeutic targets.
Topics: Humans; Risk Factors; Pseudotumor Cerebri; Obesity; Weight Gain; Female
PubMed: 38803205
DOI: 10.1080/14737175.2024.2359420 -
European Journal of Obstetrics,... Jan 2024A few publications have examined the frequency and medical implications of individuals with idiopathic intracranial hypertension (IIH) and polycystic ovarian syndrome... (Review)
Review
BACKGROUND
A few publications have examined the frequency and medical implications of individuals with idiopathic intracranial hypertension (IIH) and polycystic ovarian syndrome (PCOS), but the findings have been inconclusive. IIH and PCOS both mainly affect obese women of reproductive age and have an impact on women's health at various levels. The aim of this systematic review was to compare the prevalence and association between comorbid IIH and PCOS, and their effect on such aspects as metabolism, abnormalities in hormone levels, and reproduction.
METHODS
The criterion for inclusion was a research study of patients suffering from both syndromes. We excluded review articles, case reports, and papers with an inappropriate study design, patient population or outcomes. Electronic databases PubMed, Scopus, Web of Science and gray literature were searched to retrieve studies published from inception to June 10, 2023. The risk of bias assessment was conducted utilizing Covidence software and by discussion between co-authors.
RESULTS
After applying our inclusion/exclusion criteria, we consolidated the initial pool to a final selection of 9 articles, and 2185 patients with comorbidity of these two conditions. The prevalence of PCOS among patients with IIH was observed, with incidence rates ranging from 15.5% to 57%, which is up to 8 times greater, than the 4-10% prevalence of PCOS in the general population. These data may be valuable in clinical practice for both neurologists and gynecologists.
DISCUSSION
PCOS associated with obesity facilitates concurrence of IIH. The diagnosis of concurrence of IIH and PCOS may have significant clinical implications for patients due to the accompanying hormonal disorders, obesity-related consequences, and fertility issues. Other No systematic review was found. We have registered the study in PROSPERO (International prospective register of systematic reviews), and the registration number is CRD42023437485.
Topics: Female; Humans; Comorbidity; Obesity; Polycystic Ovary Syndrome; Pseudotumor Cerebri; Systematic Reviews as Topic
PubMed: 37948928
DOI: 10.1016/j.ejogrb.2023.11.005 -
Eye (London, England) Oct 2023We reviewed the medical case report literature to determine the proportion of cases of idiopathic intracranial hypertension (IIH) that were either inappropriately... (Review)
Review
BACKGROUND
We reviewed the medical case report literature to determine the proportion of cases of idiopathic intracranial hypertension (IIH) that were either inappropriately labelled as IIH or prematurely given this diagnosis.
METHODS
We searched OVID MEDLINE from 2012 to 2022 to identify case reports that diagnosed patients with IIH. Case reports were assessed for diagnostic accuracy using Friedman et al.'s revised diagnostic criteria for primary pseudotumor cerebri syndrome. Our primary outcome was the crude prevalence of inappropriate or premature IIH diagnoses. Our secondary outcome was determining if inaccurate IIH diagnoses were associated with variables such as journal subscription model and impact factor, author affiliation, country of origin, and year of publication.
RESULTS
A total of 33/185 case reports (17.8%) either incorrectly labelled a patient as having IIH or did not perform all of the investigations necessary to make a diagnosis of IIH. Some of these studies (4.8%) were believed to still represent 'probable' IIH given the clinical presentation, whereas 13.0% of studies were determined to have mislabelled their patients as having IIH. The most common reason that case reports did not meet diagnostic criteria included: a lack of MRV in atypical patient cases (42.4%, n = 14), no papilledema in addition to a lack of characteristic neuroimaging features (33.3%, n = 11), intracranial hypertension being secondary to another documented cause (12.1%, n = 4), normal LP opening pressure in addition to other factors (12.1%,n = 4), no description of neuroimaging (6.1%, n = 2), and abnormal CSF composition (6.1%, n = 2). Case reports that used the term 'IIH' incorrectly had a significantly lower journal impact factor (2.0 vs. 2.6, p = 0.01).
CONCLUSIONS
There is a high prevalence of premature or inappropriate diagnoses of IIH in the peer-reviewed case report literature. Adherence to published diagnostic criteria is needed when publishing IIH case reports, and authors are expected to report all relevant data in their report to ensure that an accurate diagnosis is made.
Topics: Humans; Pseudotumor Cerebri; Papilledema; Neuroimaging
PubMed: 36928224
DOI: 10.1038/s41433-023-02499-8 -
Surgical and Radiologic Anatomy : SRA Aug 2023Dilatation of the trigeminal cavum, or Meckel's cave (MC), is usually considered a radiological sign of idiopathic intracranial hypertension. However, the normal size of...
PURPOSE
Dilatation of the trigeminal cavum, or Meckel's cave (MC), is usually considered a radiological sign of idiopathic intracranial hypertension. However, the normal size of the trigeminal cavum is poorly characterized. In this study, we describe the anatomy of this meningeal structure.
METHODS
We dissected 18 MCs and measured the length and width of the arachnoid web and its extension along the trigeminal nerve.
RESULTS
Arachnoid cysts were clearly attached to the ophthalmic (V1) and maxillary (V2) branches until they entered the cavernous sinus and foramen rotundum, respectively, without extension to the skull base. Arachnoid cysts were close to the mandibular branch toward the foramen ovale, with a median anteromedial extension of 2.5 [2.0-3.0] mm, lateral extension of 4.5 [3.0-6.0] mm, and posterior extension of 4.0 [3.2-6.0] mm. The trigeminal cavum arachnoid had a total width of 20.0 [17.5-25.0] mm and length of 24.5 [22.5-29.0] mm.
CONCLUSION
Our anatomical study revealed variable arachnoid extension, which may explain the variability in size of the trigeminal cavum in images and calls into question the value of this structure as a sign of idiopathic intracranial hypertension. The arachnoid web extends beyond the limits described previously, reaching almost double the radiological size of the cavum, particularly at the level of V3 afference of the trigeminal nerve. It is possible that strong adhesion of the arachnoid to the nerve elements prevents the formation of a true subarachnoid space that can be visualized by magnetic resonance imaging.
Topics: Humans; Pseudotumor Cerebri; Arachnoid Cysts; Trigeminal Nerve; Skull Base; Radiography
PubMed: 37318563
DOI: 10.1007/s00276-023-03177-w