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Cephalalgia : An International Journal... Apr 2021To review and discuss the clinical presentation and treatment of idiopathic intracranial hypertension. (Review)
Review
OBJECTIVE
To review and discuss the clinical presentation and treatment of idiopathic intracranial hypertension.
DISCUSSION
Visual alterations and headache are the two main symptoms of idiopathic intracranial hypertension, although additional features including cranial nerve palsies, cognitive deficits, olfactory deficits and tinnitus are not uncommon. The headache associated with idiopathic intracranial hypertension frequently has a migrainous phenotype. The underlying cause of the disorder has not yet been elucidated. Several hypotheses have been postulated but none of them can explain the full clinical picture. Therapeutic options remain limited, focusing mainly on reduction in body weight and the reduction of CSF production with carbonic anhydrase inhibitors.
CONCLUSION
The accurate diagnosis of idiopathic intracranial hypertension is essential as visual deterioration due to papilledema may be irreversible. Given its phenotypic similarity and frequent overlap with chronic migraine it is essential to consider idiopathic intracranial hypertension in the diagnostic workup of chronic headache; in particular, when considering its increasing prevalence. Understanding in detail the pathophysiological mechanisms behind the associated headache would also allow study of current and future therapeutic options in a structured way.
Topics: Acetazolamide; Analgesics; Headache; Headache Disorders; Humans; Intracranial Hypertension; Migraine Disorders; Papilledema; Pseudotumor Cerebri; Vision Disorders; Weight Reduction Programs
PubMed: 33631966
DOI: 10.1177/0333102421997093 -
Neurologic Clinics Feb 2017Idiopathic intracranial hypertension (IIH) is a disorder of elevated intracranial pressure of unknown cause occurring predominantly in young women of childbearing age.... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a disorder of elevated intracranial pressure of unknown cause occurring predominantly in young women of childbearing age. The typical patient symptom profile is the presence of daily headache, pulse synchronous tinnitus, transient visual obscurations, and papilledema with its associated visual loss. Although surgical procedures are performed for those who fail medical therapy, their relative efficacy remains unclear. The main morbidity of IIH is from visual loss. This visual loss is present in most patients and can usually be reversed if recognized early in the patients' course and treated.
Topics: Acetazolamide; Diuretics; Humans; Papilledema; Pseudotumor Cerebri; Randomized Controlled Trials as Topic; Vision Disorders
PubMed: 27886895
DOI: 10.1016/j.ncl.2016.08.004 -
Deutsches Arzteblatt International Sep 2015Typical optic neuritis is often the presenting manifestation of multiple sclerosis (MS). Its incidence in central Europe is 5 cases per 100 000 persons per year. (Review)
Review
BACKGROUND
Typical optic neuritis is often the presenting manifestation of multiple sclerosis (MS). Its incidence in central Europe is 5 cases per 100 000 persons per year.
METHODS
This review is based on articles retrieved by a selective search of the PubMed database, on the pertinent guidelines, and on the authors' clinical experience.
RESULTS
The diagnosis of optic neuritis is based on a constellation of symptoms and signs. The onset is usually with pain on eye movement in one eye and subacute visual loss. In unilateral optic neuritis, the direct pupillary light reflex is weaker in the affected eye. One-third of patients with optic neuritis have a mildly edematous optic disc. The visual disturbance resolves in 95% of cases. A less favorable course may be evidence of neuromyelitis optica, and macular involvement may be evidence of neuroretinitis. High-dosed intravenous methylprednisolone therapy speeds recovery but does not improve the final outcome. The risk that a patient with optic neuritis will later develop multiple sclerosis can be assessed with an MRI scan of the brain.
CONCLUSION
Optic neuritis is easy to distinguish from otherv diseases affecting the optic nerve. Atypical forms of this disease and other optic nerve diseases require special treatment. For patients judged to be at high risk of developing multiple sclerosis, immune prophylaxis with beta- interferon or glatiramer acetate is recommended.
Topics: Anti-Inflammatory Agents; Diagnosis, Differential; Diagnostic Techniques, Neurological; Evidence-Based Medicine; Humans; Optic Neuritis; Papilledema; Symptom Assessment; Treatment Outcome; Vision Disorders
PubMed: 26396053
DOI: 10.3238/arztebl.2015.0616 -
BMC Neurology Apr 2023Cerebral venous and sinus thrombosis (CVST) can cause increased intracranial pressure, often leading to papilledema. In this study, we investigated the association...
BACKGROUND
Cerebral venous and sinus thrombosis (CVST) can cause increased intracranial pressure, often leading to papilledema. In this study, we investigated the association between papilledema and venous stasis on susceptibility weighted imaging (SWI) in CVST.
METHODS
Patients with CVST between 2008 and 2020 were reviewed. Patients without fundoscopic examination or SWI were excluded in this study. Venous stasis was evaluated and scored for each cerebral hemisphere: each hemisphere was divided into 5 regions according to the venous drainage territories (superior sagittal sinus, Sylvian veins, transverse sinus and vein of Labbé, deep cerebral veins, and medullary veins) and 1 point was added if venous prominence was confirmed in one territory on SWI. The venous stasis score on SWI between cerebral hemispheres with and without papilledema was compared.
RESULTS
Eight of 19 patients with CVST were excluded because of the absence of fundoscopic examination or SWI. Eleven patients (26.5 ± 2.1 years) were included in this study. Papilledema was identified in 6 patients: bilateral papilledema in 4 patients and unilateral papilledema in 2 patients. The venous stasis score on SWI was significantly higher (P = 0.013) in the hemispheres with papilledema (median, 4.0; 95% CI, 3.038-4.562) than in the hemispheres without papilledema (median, 2.5; 95% CI, 0.695-2.805).
CONCLUSIONS
This study shows that higher score of venous stasis on SWI is associated with papilledema. Therefore, the venous stasis on SWI may be an imaging surrogate marker of increased intracranial pressure in patients with CVST.
Topics: Humans; Cerebral Veins; Papilledema; Sinus Thrombosis, Intracranial; Magnetic Resonance Imaging; Intracranial Hypertension
PubMed: 37118674
DOI: 10.1186/s12883-023-03228-0 -
The Pan African Medical Journal 2019Sarcoidosis is a multisystem granulomatous disorders of unknown cause, characterised by the presence of epithelioid granulomas and giant-cell granulomas without caseous...
Sarcoidosis is a multisystem granulomatous disorders of unknown cause, characterised by the presence of epithelioid granulomas and giant-cell granulomas without caseous necrosis. Ocular sarcoidosis mainly manifests as bilateral anterior granulomatous uveitis. The involvement of the posterior segment in patients with ocular sarcoidosis is rare and manifests as periphlebitis or choroidal invasion. Isolated papillary edema is uncommon; hence the peculiarity of our case study. Posterior involvement is a prognostic indicator of poor outcome, threatening visual acuity. Early management, in close cooperation with pneumologists and based on systemic corticosteroid therapy, improves visual outcome and reduces complications.
Topics: Eye Diseases; Female; Granuloma; Humans; Middle Aged; Papilledema; Prognosis; Sarcoidosis; Uveitis; Visual Acuity
PubMed: 31223420
DOI: 10.11604/pamj.2019.32.132.18253 -
Postgraduate Medical Journal Dec 1964
Topics: Diagnosis; Humans; Papilledema; Pathology
PubMed: 14227663
DOI: 10.1136/pgmj.40.470.707 -
Continuum (Minneapolis, Minn.) Aug 2014Papilledema is one of the most concerning physical examination findings in neurology: it has a broad differential diagnosis of intracranial (and occasionally spinal)... (Review)
Review
PURPOSE OF REVIEW
Papilledema is one of the most concerning physical examination findings in neurology: it has a broad differential diagnosis of intracranial (and occasionally spinal) pathology associated with increased intracranial pressure. Papilledema impairs axoplasmic flow within the optic nerves and compresses the optic nerves externally; it may lead to profound visual loss. Thus, detection of papilledema and assessment of visual function are essential to patient management. This article reviews the treatment of papilledema-related visual loss in pseudotumor cerebri syndrome, one of the most common causes of papilledema encountered by neurologists.
RECENT FINDINGS
Results from the Idiopathic Intracranial Hypertension Trial (IIHTT), the first randomized, double-masked, placebo-controlled trial for the treatment of patients with mild visual loss from idiopathic intracranial hypertension, were published in April 2014. The IIHTT provides the first evidence-based treatment recommendations, showing the benefit of acetazolamide and weight loss for improving visual status in patients with mild visual field loss from idiopathic intracranial hypertension.
SUMMARY
A detailed ophthalmic examination, including perimetry, is critical to the evaluation, treatment, and assessment of treatment response in patients with papilledema.
Topics: Adult; Diagnosis, Differential; Double-Blind Method; Female; Humans; Intracranial Hypertension; Ophthalmoscopes; Optic Disk; Papilledema; Randomized Controlled Trials as Topic
PubMed: 25099098
DOI: 10.1212/01.CON.0000453314.75261.66 -
Acta Ophthalmologica Scandinavica Sep 2007Hypotony maculopathy, first described in 1954 by Dellaporta, usually occurs after antiglaucomatous surgery or after perforating eye injuries; it is characterized by... (Review)
Review
Hypotony maculopathy, first described in 1954 by Dellaporta, usually occurs after antiglaucomatous surgery or after perforating eye injuries; it is characterized by hypotony associated with fundus abnormalities, including papilloedema, vascular tortuosity and chorioretinal folds. In hypotony maculopathy, the scleral wall collapses inward, resulting in redundancy of the choroid and retina, leading to chorioretinal wrinkling. As the antero-posterior diameter of the vitreous cavity decreases, the very thick perivofeal retina surrounding the very thin foveal retina is thrown into radial folds around the fovea. It has been reported that hypotony maculopathy occurs in up to 20% of cases of glaucoma filtering surgery and has become more common after the introduction of antimetabolites. Young age, myopia, primary filtering surgery, systemic illnesses and elevated preoperative intraocular pressure (IOP) have been found to be associated with hypotony maculopathy. Hypotony maculopathy is treated with procedures designed to elevate IOP, which may reverse the inward scleral bowing and improve visual acuity. The successful treatment of hypotony maculopathy depends on the correct identification of its cause. Once the cause is detected, treatment should be employed as soon as possible because delayed normalization of the IOP may result in permanent macular chorioretinal changes and poor vision. This review will explore the definition, mechanisms, clinical findings and treatment of hypotony maculopathy.
Topics: Choroid Diseases; Humans; Incidence; Intraocular Pressure; Microscopy, Acoustic; Ocular Hypotension; Papilledema; Retinal Diseases; Risk Factors; Tomography, Optical Coherence
PubMed: 17542978
DOI: 10.1111/j.1600-0420.2007.00910.x -
Journal of Neuro-ophthalmology : the... Dec 2021To review the literature and provide a summary of COVID-19-related neurologic and neuro-ophthalmic complications. (Review)
Review
BACKGROUND
To review the literature and provide a summary of COVID-19-related neurologic and neuro-ophthalmic complications.
METHODS
The currently available literature was reviewed on PubMed and Google Scholar using the following keywords for searches: CNS, Neuro-Ophthalmology, COVID-19, SARS-CoV-2, coronavirus, optic neuritis, pseudotumor cerebri, Acute Disseminated Encephalomyelitis, posterior reversible encephalopathy syndrome (PRES), meningitis, encephalitis, acute necrotizing hemorrhagic encephalopathy, and Guillain-Barré and Miller Fisher syndromes.
RESULTS
Neuroradiologic findings of neurologic and neuro-ophthalmologic complications in relationship to COVID-19 infection were reviewed. Afferent visual pathway-related disorders with relevant imaging manifestations included fundus nodules on MRI, papilledema and pseudotumor cerebri syndrome, optic neuritis, Acute Disseminated Encephalomyelitis, vascular injury with thromboembolism and infarct, leukoencephalopathy, gray matter hypoxic injury, hemorrhage, infectious meningitis/encephalitis, acute necrotizing hemorrhagic encephalopathy, and PRES. Efferent visual pathway-related complications with relevant imaging manifestations were also reviewed, including orbital abnormalities, cranial neuropathy, Guillain-Barré and Miller Fisher syndromes, and nystagmus and other eye movement abnormalities related to rhombencephalitis.
CONCLUSION
COVID-19 can cause central and peripheral nervous system disease, including along both the afferent and efferent components of visual axis. Manifestations of disease and long-term sequela continue to be studied and described. Familiarity with the wide variety of neurologic, ophthalmic, and neuroradiologic presentations can promote prompt and appropriate treatment and continue building a framework to understand the underlying mechanism of disease.
Topics: Brain; COVID-19; Eye; Humans; Magnetic Resonance Imaging; Neuroimaging; Optic Neuritis; Papilledema
PubMed: 34788237
DOI: 10.1097/WNO.0000000000001454 -
American Journal of Ophthalmology Mar 2022This study is the first to evaluate the prevalence of retinal thinning and the correlation with papilledema and visual acuity (VA) in a large population with...
PURPOSE
This study is the first to evaluate the prevalence of retinal thinning and the correlation with papilledema and visual acuity (VA) in a large population with craniosynostosis.
DESIGN
Prospective clinical cohort study.
METHODS
All and complex patients syndromic and complex with craniosynostosis who visited the only national referral center between 2018 and 2020 were included. Retinal layers were segmented using optical coherence tomography. Patients were seen by an ophthalmologist for VA assessment and fundoscopy. Multivariate regression models were developed to evaluate correlations between retinal thickness, papilledema and VA.
RESULTS
We included 127 patients. Retinal thinning was most prevalent in the peripapillary retinal nerve fiber layer (≤38%). A longer duration of papilledema in early childhood correlated with an increased peripapillary retinal nerve fiber layer and total retinal thickness optic nerve head later in life (+0.3 ± 0.2, P = .04 and +1.0 ± 1.0, P = .003); however, its thickness was not correlated with the VA (P = .20 and P = .53). Macular retinal thinning was associated with a worse VA (P = .01); however, it was not correlated with the duration of papilledema (P = .95).
CONCLUSIONS
Following a preventative treatment strategy for syndromic and complex craniosynostosis, the prevalence of retinal ONH thinning is low. Although the prevalence of peripapillary retinal nerve fiber layer thinning is considerable, its thickness is not correlated with VA. In contrast, macular thinning is correlated with worse VA scores and should, therefore, be evaluated during follow-up. Future studies should evaluate the (1) causative mechanism for macular thinning, (2) correlation between the time to surgery and macular thinning, and (3) results of reactive treatment strategies and compare those results to the current study.
Topics: Child, Preschool; Cohort Studies; Craniosynostoses; Humans; Nerve Fibers; Papilledema; Prospective Studies; Retinal Ganglion Cells; Tomography, Optical Coherence; Visual Acuity
PubMed: 34487703
DOI: 10.1016/j.ajo.2021.08.014