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Journal of Neuro-ophthalmology : the... Dec 2023Optic neuropathy in childhood leukemia occurs through multiple direct and indirect mechanisms, including leukemic infiltration of the optic nerve, infection, blood...
BACKGROUND
Optic neuropathy in childhood leukemia occurs through multiple direct and indirect mechanisms, including leukemic infiltration of the optic nerve, infection, blood dyscrasias, or adverse effects of treatment. We aimed to characterize visual outcomes in pediatric patients with leukemia-associated neuro-ophthalmic manifestations.
METHODS
We retrospectively identified patients with leukemia and optic nerve pathology over 13 years by diagnostic billing codes. We collected information on demographics, presentation, treatment course, and visual outcomes directly from medical records.
RESULTS
Of the 19 patients who met inclusion criteria, 17 (89.5%) had pseudotumor cerebri and 2 had direct optic nerve infiltration. Causes of increased intracranial pressure included central nervous system infiltration (6 of 17), hyperviscosity/leukemia (2 of 17), venous sinus thrombosis (3 of 17), medication induced (5 of 17), and bacterial meningitis (1 of 17). 47.1% (8 of 17) had papilledema at the time of leukemia diagnosis, and 94.1% (16 of 17) of patients with pseudotumor cerebri were treated with acetazolamide. At presentation, 3 patients had decreased vision secondary to macular ischemia, subhyaloid vitreous hemorrhage, or steroid induced glaucoma. Following treatment of pseudotumor cerebri, binocular visual acuity was ≥20/25 in all patients. One patient with optic nerve infiltration had a final visual acuity of count fingers in the affected eye.
CONCLUSIONS
In our chart review, the most common mechanism of neuro-ophthalmic involvement in pediatric leukemia was elevated intracranial pressure from a myriad of causes. Visual outcomes from patients with elevated intracranial pressure were excellent. Understanding the mechanisms by which leukemia can cause optic nerve disease in pediatric patients can facilitate earlier diagnosis and treatment and potentially improve visual outcomes.
Topics: Humans; Child; Pseudotumor Cerebri; Retrospective Studies; Papilledema; Eye; Optic Nerve Diseases; Leukemia
PubMed: 37247249
DOI: 10.1097/WNO.0000000000001879 -
Journal of Child Neurology Apr 2021Pseudotumor cerebri also known as idiopathic intracranial hypertension is a relatively uncommon disorder of unknown pathophysiology. Although pseudotumor cerebri occurs...
Pseudotumor cerebri also known as idiopathic intracranial hypertension is a relatively uncommon disorder of unknown pathophysiology. Although pseudotumor cerebri occurs in both children and adults, the pseudotumor cerebri literature is heavily dominated by adult studies. The aim of this study is to retrospectively describe the clinical presentation, imaging, treatment, and outcomes of a large pediatric pseudotumor cerebri population over a 23-year period. We also discuss secondary pseudotumor cerebri (44%) as well as the increasingly recognized patient subgroups without headache (13.3%) and without papilledema (7.3%). Female sex, obesity, and initial symptoms were consistent with the literature; however radiographic findings were surprisingly low in this cohort. Headache outcomes at 1 week, 1 month, and 3 months following initial lumbar puncture/treatment and visual function outcomes are reported.
Topics: Adolescent; Age Factors; Brain; Child; Female; Humans; Magnetic Resonance Imaging; Male; Pediatric Obesity; Pseudotumor Cerebri; Retrospective Studies; Sex Factors; Spinal Puncture; Treatment Outcome; Vision Disorders
PubMed: 33148096
DOI: 10.1177/0883073820972231 -
Expert Review of Neurotherapeutics May 2023Idiopathic intracranial hypertension is characterized by raised intracranial pressure that triggers disabling headaches and can cause permanent visual loss. There is an... (Review)
Review
INTRODUCTION
Idiopathic intracranial hypertension is characterized by raised intracranial pressure that triggers disabling headaches and can cause permanent visual loss. There is an increased incidence and prevalence of the condition linked to location-specific obesity rates. There are no licensed treatments for the condition. The majority of approaches to managing the disease prioritize resolution of papilledema. However, evidence is emerging that idiopathic intracranial hypertension is a systemic metabolic disease.
AREAS COVERED
The aim of this review is to present the emerging pathophysiology evidence which is leading to novel targeted therapeutics. The diagnostic pathway is outlined. The current and potential management approaches for idiopathic intracranial hypertension are also discussed.
EXPERT OPINION
Idiopathic intracranial hypertension is a condition with metabolic dysregulation with systemic manifestations that are present over and above what can be expl.ained by obesity alone. While most of the current management of this condition focuses on the eyes, future management needs to address the disabling headaches and the systemic risks of preeclampsia, gestational diabetes, and major cardiovascular events.
Topics: Female; Pregnancy; Humans; Pseudotumor Cerebri; Intracranial Hypertension; Papilledema; Obesity; Headache
PubMed: 37114519
DOI: 10.1080/14737175.2023.2206567 -
Seminars in Pediatric Neurology May 2017Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS... (Review)
Review
Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure in the setting of normal brain parenchyma and cerebrospinal fluid. PTCS can occur in the pediatric and adult populations and, if untreated, may lead to permanent visual loss. In this review, discussion will focus on PTCS in the pediatric population and will outline its distinct epidemiology and key elements of diagnosis, evaluation and management. Finally, although the precise mechanisms are unclear, the underlying pathophysiology will be considered.
Topics: Brain; Child; Disease Management; Humans; Pseudotumor Cerebri
PubMed: 28941525
DOI: 10.1016/j.spen.2017.04.002 -
Current Opinion in Neurology Feb 2016Idiopathic intracranial hypertension (IIH) is a potentially blinding disorder of unknown cause, characterized by elevated intracranial pressure in the absence of a mass... (Review)
Review
PURPOSE OF REVIEW
Idiopathic intracranial hypertension (IIH) is a potentially blinding disorder of unknown cause, characterized by elevated intracranial pressure in the absence of a mass lesion, venous sinus thrombosis, or meningitis. This review summarizes recent developments and insights from leading treatment trials, emerging treatment options, and evolving ways to evaluate IIH.
RECENT FINDINGS
The Idiopathic Intracranial Hypertension Treatment Trial is the first large-scale, randomized, prospective study to evaluate medical treatment of patients with mild vision loss. These data have facilitated our understanding of baseline clinical manifestations, including impact on quality of life and treatment outcomes. Recent hypotheses and studies evaluating the role of cerebral venous sinus stenosis and stenting are discussed. Technological advances in optical coherence tomography are emerging to provide novel ways of evaluating and tracking optic disc swelling in IIH.
SUMMARY
Recent changes in defining IIH, understanding the impact and treatment of mild visual loss, and the roles that cerebral venous stenting and optical coherence tomography might have in clinical practice provide the framework to better treat patients with IIH.
Topics: Constriction, Pathologic; Humans; Intracranial Hypertension; Pseudotumor Cerebri; Quality of Life; Stents; Treatment Outcome
PubMed: 26641809
DOI: 10.1097/WCO.0000000000000273 -
Journal of Obstetrics and Gynaecology... Nov 2021Idiopathic intracranial hypertension (IIH) is a syndrome characterized by elevated intracranial pressure without an identifiable underlying cause. Pregnancy has unique...
Idiopathic intracranial hypertension (IIH) is a syndrome characterized by elevated intracranial pressure without an identifiable underlying cause. Pregnancy has unique and important diagnostic and therapeutic implications for patients with IIH. Despite these implications, there are no guidelines to assist clinicians in managing IIH during pregnancy. Our review aims to summarize the key considerations related to the diagnosis and management of IIH during pregnancy, to optimize the care of these patients and mitigate the risk of disease-related complications. The optimal management of IIH in pregnancy should include a multidisciplinary team, including an obstetrician (or maternal-fetal medicine specialist), a neurologist, and an ophthalmologist (or neuro-ophthalmologist).
Topics: Female; Humans; Pregnancy; Pseudotumor Cerebri
PubMed: 33412303
DOI: 10.1016/j.jogc.2020.12.019 -
Current Neurology and Neuroscience... May 2018Idiopathic intracranial hypertension (IIH), pseudotumor cerebri syndrome (PTCS), and benign intracranial hypertension are all terms that have been used for a neurologic... (Review)
Review
PURPOSE OF REVIEW
Idiopathic intracranial hypertension (IIH), pseudotumor cerebri syndrome (PTCS), and benign intracranial hypertension are all terms that have been used for a neurologic syndrome consisting of elevated intracranial pressure (ICP), headache and vision loss without mass lesion or underlying infection or malignancy. In this review article, categorization, diagnostic criteria, symptom management strategies, and disease treatment options for pseudotumor cerebri syndrome will be discussed.
RECENT FINDINGS
The Idiopathic Intracranial Hypertension Treatment Trial has now proven that acetazolamide should be the first line therapy in primary PTCS, but other treatment options exist in patients who cannot tolerate acetazolamide or in selected cases, which requires surgical intervention for PTCS which acutely threatens vision. Headache has also been shown to require focused treatment beyond therapies that lower ICP, specifically targeting coexistent primary headache disorders and medication overuse. Advances in treatment and diagnostic modalities have improved understanding of PTCS types and their treatment. The pathophysiology of primary PTCS, however, remains incompletely understood, but continued evaluation of cerebrospinal fluid flow dynamics, aquaporins, hormones, natriuretic peptides, and the link with female gender and obesity may lead to future answers.
Topics: Animals; Humans; Intracranial Hypertension; Pseudotumor Cerebri; Syndrome
PubMed: 29721718
DOI: 10.1007/s11910-018-0839-1 -
Otolaryngologic Clinics of North America Jun 2022Idiopathic intracranial hypertension (IIH) is a triad of headaches, visual changes, and papilledema in the absence of a secondary cause for elevated intracranial... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a triad of headaches, visual changes, and papilledema in the absence of a secondary cause for elevated intracranial pressure. There is an association with obesity, and the incidence is rising in parallel with the obesity epidemic. Sometimes these patients present to an otolaryngologist with complaints like tinnitus, dizziness, hearing loss, and otorrhea or rhinorrhea from cerebrospinal fluid leak. IIH diagnosis in conjunction with neurology and ophthalmology, including neuroimaging and lumbar puncture with opening pressure, is key to managing of this condition. Otolaryngologists should recognize IIH as a possible diagnosis and initiate appropriate referrals and treatment.
Topics: Humans; Intracranial Hypertension; Obesity; Otolaryngologists; Papilledema; Pseudotumor Cerebri
PubMed: 35490040
DOI: 10.1016/j.otc.2022.02.005 -
Indian Journal of Ophthalmology Oct 2014The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy. (Review)
Review
AIMS
The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy.
SETTING AND DESIGN
Review article.
MATERIALS AND METHODS
Existing literature and the authors' experience were reviewed.
RESULTS
Treatment options range from observation to surgical intervention. Weight loss and medical treatment may be utilized in cases without vision loss or in combination with surgical treatment. Cerebrospinal fluid shunting procedures and/or optic nerve sheath decompression is indicated for severe vision loss or headache unresponsive to medical management. The recent use of endovascular stenting of transverse sinus stenoses has also demonstrated benefit in patients with pseudotumor cerebri.
CONCLUSION
While each treatment form may be successful individually, a multimodal approach is typically utilized with treatments selected on a case-by-case basis.
Topics: Disease Management; Humans; Pseudotumor Cerebri
PubMed: 25449933
DOI: 10.4103/0301-4738.145991 -
Ugeskrift For Laeger Aug 2021Idiopathic intracranial hypertension (IIH) is a challenging disease characterized by an increase in intracranial pressure which occurs without any known cause. The...
Idiopathic intracranial hypertension (IIH) is a challenging disease characterized by an increase in intracranial pressure which occurs without any known cause. The disease is mainly seen in obese females of child-bearing age. While originally described as rare, the incidence is increasing in parallel with pandemic obesity, and clinicians in all fields are increasingly likely to meet patients with IIH. The condition causes major morbidity due to chronic headache and visual loss if untreated. In the past ten years new diagnostic criteria and guidelines for management have been published.
Topics: Female; Humans; Incidence; Intracranial Hypertension; Intracranial Pressure; Obesity; Pseudotumor Cerebri; Vision Disorders
PubMed: 34378523
DOI: No ID Found