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Lupus Jun 2024Idiopathic intracranial hypertension (IIH) is a diagnosis of exclusion characterized by features of raised intracranial pressure (ICP) in the absence of brain...
Idiopathic intracranial hypertension (IIH) is a diagnosis of exclusion characterized by features of raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. Commonly used other terms for this entity include benign intracranial hypertension (BIH) or pseudotumor cerebri. Few case reports of systemic lupus erythematosus (SLE) presenting as IIH are available in the literature. We report a 12-year-old girl presented with chronic holocranial headache and occasional episodes of projectile vomiting for the last 6 months and then developed blurring of vision for the last month. She fulfilled the criteria for IIH. Subsequent evaluation revealed a diagnosis of SLE. The occurrence of IIH in SLE is not coincidental and is reported in 1%-5.4% of patients with SLE. Though corticosteroids have not been widely used in IIH, underlying SLE warranted administering corticosteroids with subsequent complete resolution of IIH. Pediatricians, neurologists, intensivists, and ophthalmologists should consider SLE as a differential diagnosis in children presenting with IIH.
Topics: Humans; Female; Lupus Erythematosus, Systemic; Child; Pseudotumor Cerebri; Diagnosis, Differential; Headache; Intracranial Hypertension
PubMed: 38607271
DOI: 10.1177/09612033241247358 -
World Neurosurgery Apr 2024This review explores the future role of venous sinus stenting (VSS) in the management of idiopathic intracranial hypertension and pulsatile tinnitus. Despite its... (Review)
Review
This review explores the future role of venous sinus stenting (VSS) in the management of idiopathic intracranial hypertension and pulsatile tinnitus. Despite its favorable safety profile and clinical outcomes compared with traditional treatments, VSS is not yet the standard of care for these conditions, lacking high-level evidence data and guidelines for patient selection and indications. Current and recently completed clinical trials are expected to provide data to support the adoption of VSS as a primary treatment option. Additionally, VSS shows potential in treating other conditions, such as dural arteriovenous fistula and cerebral venous sinus thrombosis, and it is likely that the procedure will continue to see an expansion of its approved indications. The current lack of dedicated venous stenting technology is being addressed with promising advancements, which may improve procedural ease and patient outcomes. VSS also offers potential for expansion into modulation of brain electrophysiology via endovascular routes, offering exciting possibilities for neurodiagnostics and treatment of neurodegenerative disorders.
Topics: Humans; Treatment Outcome; Pseudotumor Cerebri; Stents; Cranial Sinuses; Endovascular Procedures; Intracranial Hypertension; Retrospective Studies
PubMed: 38590072
DOI: 10.1016/j.wneu.2023.12.128 -
World Neurosurgery Apr 2024Although numerous case series and meta-analyses have shown the efficacy of venous sinus stenting (VSS) in the treatment of idiopathic intracranial hypertension and...
Although numerous case series and meta-analyses have shown the efficacy of venous sinus stenting (VSS) in the treatment of idiopathic intracranial hypertension and idiopathic intracranial hypertension-associated pulsatile tinnitus, there remain numerous challenges to be resolved. There is no widespread agreement on candidacy; pressure gradient and failed medical treatment are common indications, but not all clinicians require medical refractoriness as a criterion. Venous manometry, venography, and cerebral angiography are essential tools for patient assessment, but again disagreements exist regarding the best, or most appropriate, diagnostic imaging choice. Challenges with the VSS technique also exist, such as stent choice and deployment. There are considerations regarding postprocedural balloon angioplasty and pharmacologic treatment, but there is insufficient evidence to formalize postoperative decision making. Although complications of VSS are relatively rare, they include in-stent stenosis, hemorrhage, and subdural hematoma, and the learning curve for VSS presents specific challenges in navigating venous anatomy, emphasizing the need for wider availability of high-quality training. Recurrence of symptoms, particularly stent-adjacent stenosis, poses challenges, and although restenting and cerebrospinal fluid-diverting procedures are options, there is a need for clearer criteria for retreatment strategies. Despite these challenges, when comparing VSS with traditional cerebrospinal fluid-diverting procedures, VSS emerges as a favorable option, with strong clinical outcomes, lower complication rates, and cost-effectiveness. Further research is necessary to refine techniques and indications and address specific aspects of VSS to overcome these challenges.
Topics: Humans; Pseudotumor Cerebri; Tinnitus; Constriction, Pathologic; Cranial Sinuses; Stents; Intracranial Hypertension; Treatment Outcome; Retrospective Studies
PubMed: 38590071
DOI: 10.1016/j.wneu.2023.12.164 -
World Neurosurgery Apr 2024Venous sinus stenosis has garnered increasing academic attention as a potential etiology of idiopathic intracranial hypertension (IIH) and pulsatile tinnitus (PT). The...
Venous sinus stenosis has garnered increasing academic attention as a potential etiology of idiopathic intracranial hypertension (IIH) and pulsatile tinnitus (PT). The complex anatomy of the cerebral venous sinuses and veins plays a crucial role in the pathophysiology of these conditions. Venous sinus stenosis, often found in the superior sagittal or transverse sinus, can lead to elevated intracranial pressure (ICP) and characteristic IIH symptoms. Stenosis, variations in dural venous anatomy, and flow dominance patterns contribute to aberrant flow and subsequent PT. Accurate imaging plays a vital role in diagnosis, and magnetic resonance (MR) venography is particularly useful for detecting stenosis. Management strategies for IIH and PT focus on treating the underlying disease, weight management, medical interventions, and, in severe cases, surgical or endovascular procedures. Recently, venous sinus stenting has gained interest as a minimally invasive treatment option for IIH and PT. Stenting addresses venous sinus stenosis, breaking the feedback loop between elevated ICP and stenosis, thus reducing ICP and promoting cerebrospinal fluid outflow. The correction and resolution of flow aberrances can also mitigate or resolve PT symptoms. While venous sinus stenting remains an emerging field, initial results are promising. Further research is needed to refine patient selection criteria and evaluate the long-term efficacy of stenting as compared to traditional treatments.
Topics: Humans; Pseudotumor Cerebri; Tinnitus; Constriction, Pathologic; Intracranial Hypertension; Cranial Sinuses; Stents
PubMed: 38590070
DOI: 10.1016/j.wneu.2023.12.125 -
Cureus Mar 2024Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a newly discovered autoimmune demyelinating disorder. The clinical manifestations of MOGAD are...
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.
PubMed: 38586776
DOI: 10.7759/cureus.55652 -
Neurologic Clinics May 2024Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either... (Review)
Review
Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.
Topics: Humans; Pseudotumor Cerebri; Intracranial Hypertension; Headache; Vision Disorders; Syndrome; Papilledema
PubMed: 38575259
DOI: 10.1016/j.ncl.2024.02.001 -
Current Pain and Headache Reports Jun 2024The purpose of this review is two-fold: (1) to discuss a case report of idiopathic intracranial hypertension (IIH) after abrupt cessation of a glucagon-like peptide-1... (Review)
Review
Idiopathic Intracranial Hypertension After Abrupt Cessation of Medication: A Case Report of Abrupt Glucagon-Like Peptide-1 (GLP-1) Receptor Agonist Cessation and Review of the Literature.
PURPOSE OF REVIEW
The purpose of this review is two-fold: (1) to discuss a case report of idiopathic intracranial hypertension (IIH) after abrupt cessation of a glucagon-like peptide-1 (GLP-1) receptor agonist with resultant rapid weight gain and (2) to review the literature regarding the potential role of GLP-1 receptor agonists in the treatment of IIH as well as potential pitfalls.
RECENT FINDINGS
GLP-1 receptor agonists have become widely used to treat obesity. Obesity is a known risk factor for the development of IIH, though the precise pathophysiology is unclear. GLP-1 receptor agonists may help treat IIH by promoting weight loss, lipolysis of adipose tissue, and potentially decreasing the secretion of CSF, as was seen in rat models. Abrupt cessation of GLP-1 receptor agonists can result in regaining lost weight rapidly. In the case that we present, the patient stopped duraglutide abruptly due to lack of insurance coverage and regained the weight she had lost within a month. She subsequently developed IIH. GLP-1 receptor agonists have the potential to help treat IIH; however, this class of medication needs to be used carefully, as cessation of the medication and resultant rapid weight gain can result in IIH.
Topics: Humans; Female; Glucagon-Like Peptide-1 Receptor; Pseudotumor Cerebri; Adult; Weight Gain; Obesity
PubMed: 38573570
DOI: 10.1007/s11916-024-01215-9 -
Pseudotumor cerebri with status epilepticus in a child: A rare presentation of vitamin D deficiency.Clinical Case Reports Apr 2024Pseudotumor cerebri (PTC) encompasses a constellation of symptoms caused by elevated intracranial pressure of unclear etiology. Various associations have been described,...
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.
PubMed: 38550743
DOI: 10.1002/ccr3.8695 -
World Neurosurgery Jun 2024Idiopathic intracranial hypertension (IIH) is a cerebrospinal fluid (CSF) disorder defect that is frequently treated with CSF shunts. Shunts utilizing the cisterna magna...
Cerebrospinal Fluid Diversion from the Cisterna Magna in Patients with Idiopathic Intracranial Hypertension and Slit Ventricles: Long-Term Effectiveness, Revision Rates, and Clinical Outcomes.
OBJECTIVE
Idiopathic intracranial hypertension (IIH) is a cerebrospinal fluid (CSF) disorder defect that is frequently treated with CSF shunts. Shunts utilizing the cisterna magna as a proximal reservoir have been described in literature; however, long-term outcomes are unknown. The present study aims to describe the long-term effectiveness, revision rates, and clinical outcomes of this shunt in 14 patients with IIH and slit ventricles.
METHODS
A single-center retrospective review of 14 IIH patients treated by cisterna magna shunts was performed. Shunt histories, including revision rate and time until first shunt failure for ventricular, lumbar, and cisterna magna shunts were recorded. "Revision rate" was calculated as the total number of shunt revisions over years of total shunt placement. The average follow-up time was 12.08 years.
RESULTS
The mean age at first cisterna magna shunt placement was 18.1 years (6.6-43.3 years) and all patients had radiological evidence of slit ventricles. Cisterna magna shunts improved or resolved clinical symptoms for all 14 patients and had a lower rate of revisions (0.42 revision/year) compared to ventricular (0.72 revision/year) and lumbar (1.30 revision/year) shunts. Of the 11 patients still requiring CSF diversion at the end of the study, eight had functioning shunts that utilized the cisterna magna.
CONCLUSIONS
The cisterna magna shunt may be a suitable option for patients with IIH and slit ventricles. Further study is needed to understand the clinical utility of this shunt for the population in which it is indicated.
Topics: Humans; Adult; Female; Male; Retrospective Studies; Pseudotumor Cerebri; Cerebrospinal Fluid Shunts; Cisterna Magna; Reoperation; Young Adult; Adolescent; Treatment Outcome; Slit Ventricle Syndrome; Child; Cerebral Ventricles; Follow-Up Studies
PubMed: 38548048
DOI: 10.1016/j.wneu.2024.03.130 -
The British Journal of Dermatology May 2024
Evaluation of the association between tetracycline-class antibiotic use in patients with acne and idiopathic intracranial hypertension risk: a population-level cohort study.
Topics: Humans; Acne Vulgaris; Anti-Bacterial Agents; Female; Male; Pseudotumor Cerebri; Adult; Young Adult; Adolescent; Cohort Studies; Risk Factors; Tetracyclines
PubMed: 38530996
DOI: 10.1093/bjd/ljae099