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Iranian Journal of Medical Sciences Jan 2022There are reports of ocular tropism due to respiratory viruses such as severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2). Various studies have shown ocular... (Meta-Analysis)
Meta-Analysis
BACKGROUND
There are reports of ocular tropism due to respiratory viruses such as severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2). Various studies have shown ocular manifestation in coronavirus disease-2019 (COVID-19) patients. We aimed to identify ophthalmic manifestations in COVID-19 patients and establish an association between ocular symptoms and SARS-CoV-2 infection.
METHODS
A systematic search of Medline, Scopus, Web of Science, Embase, and Cochrane Library was conducted for publications from December 2019 to April 2021. The search included MeSH terms such as SARS-CoV-2 and ocular manifestations. The pooled prevalence estimate (PPE) with 95% confidence interval (CI) was calculated using binomial distribution and random effects. The meta-regression method was used to examine factors affecting heterogeneity between studies.
RESULTS
Of the 412 retrieved articles, 23 studies with a total of 3,650 COVID-19 patients were analyzed. The PPE for any ocular manifestations was 23.77% (95% CI: 15.73-31.81). The most prevalent symptom was dry eyes with a PPE of 13.66% (95% CI: 5.01-25.51). The PPE with 95% CI for conjunctival hyperemia, conjunctival congestion/conjunctivitis, and ocular pain was 13.41% (4.65-25.51), 9.14% (6.13-12.15), and 10.34% (4.90-15.78), respectively. Only two studies reported ocular discomfort and diplopia. The results of meta-regression analysis showed that age and sample size had no significant effect on the prevalence of any ocular manifestations. There was no significant publication bias in our meta-analysis.
CONCLUSION
There is a high prevalence of ocular manifestations in COVID-19 patients. The most common symptoms are dry eyes, conjunctival hyperemia, conjunctival congestion/conjunctivitis, ocular pain, irritation/itching/burning sensation, and foreign body sensation.
Topics: COVID-19; Eye Diseases; Humans; Prevalence
PubMed: 35017772
DOI: 10.30476/ijms.2021.89475.2026 -
International Journal of Oral and... Jun 2022Computed tomography (CT) is commonly used for the diagnosis, treatment planning, and prognosis of pure orbital fractures of the orbital floor and medial wall. The aim of... (Review)
Review
Computed tomography (CT) is commonly used for the diagnosis, treatment planning, and prognosis of pure orbital fractures of the orbital floor and medial wall. The aim of this study was to systematically review the current literature in order to establish an overview of CT parameters relevant to the choice of treatment and (long-term) clinical outcome for patients treated operatively and conservatively. The PRISMA guidelines were followed. Databases were searched using the terms 'orbital fracture' and 'computed tomography'. Studies evaluating the relationship between CT parameters and the treatment decision or clinical outcome (enophthalmos, diplopia, and/or limitation of ocular movement) were included. The search yielded 4448 results of which 31 were included (except for three, all were retrospective). The systematic use of CT imaging in orbital fractures of the floor and the medial wall can be of great value in the treatment decision and prediction of (long-term) clinical outcomes for both conservatively and surgically treated patients. The following parameters were found to be the most relevant: fracture size, fracture location, orbital volume, soft tissue involvement, and craniocaudal dimension. Although some show great individual potential, it is likely that incorporating all parameters into an algorithm will provide the best predictive power and thus would be the most practically applicable tool.
Topics: Diplopia; Enophthalmos; Humans; Orbital Fractures; Retrospective Studies; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 34696942
DOI: 10.1016/j.ijom.2021.10.001 -
Acta Ophthalmologica Feb 2021The effectiveness and safety of surgery for spheno-orbital meningiomas remains subject of debate, as studies often describe different surgical approaches and... (Meta-Analysis)
Meta-Analysis
PURPOSE
The effectiveness and safety of surgery for spheno-orbital meningiomas remains subject of debate, as studies often describe different surgical approaches and reconstruction techniques with very heterogeneous outcomes. We aimed to systematically summarize and analyse the literature on spheno-orbital meningiomas regarding presenting symptoms, surgical techniques, outcomes and complications.
METHODS
Studies were retrieved from eight databases. Original articles were included if in ≥5 patients presenting symptoms, surgical treatment and outcomes were described. Fixed- and random-effects meta-analysis was performed to estimate weighted percentages with 95%CIs of presenting symptoms, outcomes and complications.
RESULTS
Thirty-eight articles were included describing 1486 patients. Proptosis was the most common presenting symptom (84%; 95%CI 76-91%), followed by unilateral visual acuity deficits (46%; 95%CI 40-51%) and visual field deficits (31%; 95%CI 20-43%). In 35/38 studies (92%), a pterional craniotomy was used. Decompression of the optic canal (82%) and the superior orbital fissure (66%) was most often performed, and usually dural (47%) and bony defects (76%) were reconstructed. In almost all patients, visual acuity (91%; 95%CI 86-96%), visual fields (87%; 95%CI 70-99%) and proptosis (96%; 95%CI 90-100%) improved. Furthermore, surgery showed improvement in 96% (95%CI 78-100%) for both diplopia and ophthalmoplegia. The most common surgical complications were hypesthesia (19%; 95%CI 10-30%), ptosis and diplopia (both 17%; 95%CI, respectively, 10-26% and 5-33%) and ophthalmoplegia (16%; 95%CI 10-24).
CONCLUSION
Patients with spheno-orbital meningioma usually present with proptosis or unilateral decreased visual acuity. Surgery shows to be effective in improving visual acuity and visual field deficits with mostly minor and well-tolerated complications.
Topics: Humans; Meningioma; Neurosurgical Procedures; Orbit; Orbital Neoplasms; Sphenoid Bone; Visual Acuity
PubMed: 32602264
DOI: 10.1111/aos.14517 -
The Cochrane Database of Systematic... Nov 2020Epilepsy is a central nervous system disorder (neurological disorder). Epileptic seizures are the result of excessive and abnormal cortical nerve cell electrical... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Epilepsy is a central nervous system disorder (neurological disorder). Epileptic seizures are the result of excessive and abnormal cortical nerve cell electrical activity in the brain. Despite the development of more than 10 new antiepileptic drugs (AEDs) since the early 2000s, approximately a third of people with epilepsy remain resistant to pharmacotherapy, often requiring treatment with a combination of AEDs. In this review, we summarised the current evidence regarding rufinamide, a novel anticonvulsant medication, which, as a triazole derivative, is structurally unrelated to any other currently used anticonvulsant medication when used as an add-on treatment for drug-resistant epilepsy. In January 2009, rufinamide was approved by the US Food and Drug Administration for the treatment of children four years of age and older with Lennox-Gastaut syndrome. It is also approved as an add-on treatment for adults and adolescents with focal seizures. This is an updated version of the original Cochrane Review published in 2018.
OBJECTIVES
To evaluate the efficacy and tolerability of rufinamide when used as an add-on treatment for people with drug-resistant epilepsy.
SEARCH METHODS
We imposed no language restrictions. We contacted the manufacturers of rufinamide and authors in the field to identify any relevant unpublished studies.
SELECTION CRITERIA
Randomised, double-blind, placebo-controlled, add-on trials of rufinamide, recruiting people (of any age or gender) with drug-resistant epilepsy.
DATA COLLECTION AND ANALYSIS
Two review authors independently selected trials for inclusion and extracted the relevant data. We assessed the following outcomes: 50% or greater reduction in seizure frequency (primary outcome); seizure freedom; treatment withdrawal; and adverse effects (secondary outcomes). Primary analyses were intention-to-treat (ITT) and we presented summary risk ratios (RRs) with 95% confidence intervals (CIs). We evaluated dose response in regression models. We carried out a risk of bias assessment for each included study using the Cochrane 'Risk of bias' tool and assessed the overall certainty of evidence using the GRADE approach.
MAIN RESULTS
The review included six trials, representing 1759 participants. Four trials (1563 participants) included people with uncontrolled focal seizures. Two trials (196 participants) included individuals with established Lennox-Gastaut syndrome. Overall, the age of adults ranged from 18 to 80 years and the age of children ranged from 4 to 16 years. Baseline phases ranged from 28 to 56 days and double-blind phases from 84 to 96 days. Five of the six included trials described adequate methods of concealment of randomisation, and only three described adequate blinding. All analyses were by ITT. Overall, five studies were at low risk of bias and one had unclear risk of bias due to lack of reported information around study design. All trials were sponsored by the manufacturer of rufinamide and therefore were at high risk of funding bias. The overall RR for 50% or greater reduction in seizure frequency was 1.79 (95% CI 1.44 to 2.22; 6 randomised controlled trials (RCTs), 1759 participants; moderate-certainty evidence), indicating that rufinamide (plus conventional AED) was significantly more effective than placebo (plus conventional AED) in reducing seizure frequency by at least 50% when added to conventionally used AEDs in people with drug-resistant focal epilepsy. Data from only one study (73 participants) reported seizure freedom: RR 1.32 (95% CI 0.36 to 4.86; 1 RCT, 73 participants; moderate-certainty evidence). The overall RR for treatment withdrawal (for any reason and due to AED) was 1.83 (95% CI 1.45 to 2.31; 6 RCTs, 1759 participants; moderate-certainty evidence), showing that rufinamide was significantly more likely to be withdrawn than placebo. Most adverse effects were significantly more likely to occur in the rufinamide-treated group. Adverse events significantly associated with rufinamide were headache, dizziness, somnolence, vomiting, nausea, fatigue, and diplopia. The RRs for these adverse effects were as follows: headache 1.36 (95% Cl 1.08 to 1.69; 3 RCTs, 1228 participants; high-certainty evidence); dizziness 2.52 (95% Cl 1.90 to 3.34; 3 RCTs, 1295 participants; moderate-certainty evidence); somnolence 1.94 (95% Cl 1.44 to 2.61; 6 RCTs, 1759 participants; moderate-certainty evidence); vomiting 2.95 (95% Cl 1.80 to 4.82; 4 RCTs, 777 participants; low-certainty evidence); nausea 1.87 (95% Cl 1.33 to 2.64; 3 RCTs, 1295 participants; moderate-certainty evidence); fatigue 1.46 (95% Cl 1.08 to 1.97; 3 RCTs, 1295 participants; moderate-certainty evidence); and diplopia 4.60 (95% Cl 2.53 to 8.38; 3 RCTs, 1295 participants; low-certainty evidence). There was no important heterogeneity between studies for any outcomes. Overall, we assessed the evidence as moderate to low certainty due to wide CIs and potential risk of bias from some studies contributing to the analysis.
AUTHORS' CONCLUSIONS
For people with drug-resistant focal epilepsy, rufinamide when used as an add-on treatment was effective in reducing seizure frequency. However, the trials reviewed were of relatively short duration and provided no evidence for long-term use of rufinamide. In the short term, rufinamide as an add-on was associated with several adverse events. This review focused on the use of rufinamide in drug-resistant focal epilepsy, and the results cannot be generalised to add-on treatment for generalised epilepsies. Likewise, no inference can be made about the effects of rufinamide when used as monotherapy.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anticonvulsants; Bias; Child; Child, Preschool; Drug Resistant Epilepsy; Drug Therapy, Combination; Female; Humans; Male; Middle Aged; Randomized Controlled Trials as Topic; Triazoles
PubMed: 33179247
DOI: 10.1002/14651858.CD011772.pub3 -
International Journal of Ophthalmology 2021To assess all available data to compare the efficacy of glucocorticoids treatment and orbital decompression for dysthyroid optic neuropathy (DON). PubMed, EMBASE, the... (Review)
Review
To assess all available data to compare the efficacy of glucocorticoids treatment and orbital decompression for dysthyroid optic neuropathy (DON). PubMed, EMBASE, the Cochrane Library databases as well as other sources were searched by two independent reviewers followed by extensive hand-searching for the identification of relevant studies. The primary outcomes were the improvement in visual acuity and responder rate. Secondary outcomes were the proptosis reduction, change in diplopia, and clinical activity score (CAS). One randomized controlled trial, three retrospective case series and one prospective case series met the inclusion criteria. They were divided into intravenous high-dose glucocorticoids (ivGC) group and orbital decompression (OD) group. Both groups demonstrated improvement in visual acuity. In addition, the proportion of patients with improved vision in OD group was higher than that in ivGC group (<0.001). Post-treatment proptosis reduction was also reported in both groups. Overall, weighted mean in proptosis reduction estimated at 1.64 and 5.45 mm in patients treated with ivGC and OD respectively. This study also presented results regarding pre-existing and new-onset diplopia. Apart from diplopia, a wide variety of minor and major complications were noted in 5 included studies. The most common complication in ivGC group and OD group was Cushing's syndrome and epistaxis respectively. The present systematic review shows that both glucocorticoids treatment and OD are effective in treating DON and OD may work better in improving visual acuity and reducing proptosis. However, high-quality, large-sample, controlled studies need to be performed in the future.
PubMed: 34282398
DOI: 10.18240/ijo.2021.07.21 -
World Neurosurgery: X Jul 2023Carotid-cavernous fistulas (CCFs) represent a group of rare, abnormal arteriovenous communications between the carotid arterial system and the cavernous sinuses (CS).... (Review)
Review
BACKGROUND AND OBJECTIVES
Carotid-cavernous fistulas (CCFs) represent a group of rare, abnormal arteriovenous communications between the carotid arterial system and the cavernous sinuses (CS). CCFs often produce ophthalmologic symptoms related to increased CS pressures and retrograde venous drainage of the eye. Although endovascular occlusion remains the preferred treatment for symptomatic or high-risk CCFs, most of the data for these lesions is limited to small, single-center series. As such, we performed a systematic review and meta-analysis evaluating endovascular occlusions of CCFs to determine any differences in clinical outcomes based on presentation, fistula type, and treatment paradigm.
METHOD
A retrospective review of all studies discussing the endovascular treatment of CCFs published through March 2023 was conducted using PubMed, Scopus, Web of Science, and Embase databases. A total of 36 studies were included in the meta-analysis. Data from the selected articles were extracted and analyzed using Stata software version 14.
RESULTS
1494 patients were included. 55.08% were female and the mean age of the cohort was 48.10 years. A total number of 1516 fistulas underwent endovascular treatment, 48.05% of which were direct and 51.95% of which were indirect. 87.17% of CCFs were secondary to a known trauma while 10.18% were spontaneous. The most common presenting symptoms were 89% exophthalmos (95% CI: 78.0-100.0; I = 75.7%), 84% chemosis (95% CI: 79.0-88.0; I = 91.6%), 79% proptosis (95% CI: 72.0-86.0; I = 91.8%), 75.0% bruits (95% CI: 67.0-82.0; I = 90.7%), 56% diplopia (95% CI: 42.0-71.0; I = 92.3%), 49% cranial nerve palsy (95% CI: 32.0-66.0; I = 95.1%), 39% visual decline (95% CI: 32.0-45.0; I = 71.4%), 32% tinnitus (95% CI: 6.0-58.0; I = 96.7%), 29% elevated intraocular pain (95% CI: 22.0-36.0; I = 0.0%), 31% orbital or pre-orbital pain (95% CI: 14.0-48.0; I = 89.9%) and 24% headache (95% CI: 13.0-34.0; I = 74.98%). Coils, balloons, and stents were the three most used embolization methods respectively. Immediate complete occlusion of the fistula was seen in 68% of cases and complete remission was seen in 82%. Recurrence of CCF occurred in only 35% of the patients. Cranial nerve paralysis after treatment was observed in 7% of the cases.
CONCLUSIONS
Exophthalmos, Chemosis, proptosis, bruits, cranial nerve palsy, diplopia, orbital and periorbital pain, tinnitus, elevated intraocular pressure, visual decline and headache are the most common clinical manifestations of CCFs. The majority of endovascular treatments involved coiling, balloons and onyx and a high percentage of CCF patients experienced complete remission with the improvement of their clinical symptoms.
PubMed: 37223772
DOI: 10.1016/j.wnsx.2023.100189 -
Frontiers in Oncology 2022To improve the diagnosis and treatment of intracranial chondromas (ICDs) by discussing the clinical manifestations and imaging characteristics of ICDs, as well as...
OBJECTIVE
To improve the diagnosis and treatment of intracranial chondromas (ICDs) by discussing the clinical manifestations and imaging characteristics of ICDs, as well as surgical methods and treatment strategies.
METHODS
We retrospectively analyzed 17 patients diagnosed with ICDs who underwent microsurgery or endoscopic transsphenoidal surgery at the Tangdu Hospital of Air Force Military Medical University and the Mianyang Central Hospital from January 2010 to November 2021. Clinical manifestations, imaging examinations, surgical treatments, and prognosis of these patients were analyzed.
RESULTS
ICDs had often been misdiagnosed as craniopharyngioma, chordoma, schwannoma, cavernous hemangioma, pituitary adenoma, and meningioma before surgery. Of the 17 cases, gross total resection (GTR) was performed in 10 cases, subtotal resection (STR) in 5, and partial resection in 2. GTR of tumor was achieved in eight cases the endoscopic endonasal transsphenoidal approach (EETA) or the extended endoscopic endonasal transsphenoidal approach (EEETA), and the remaining patients underwent craniotomies. Clinical symptoms were assessed 1 week after surgery, 10 cases were relieved at varying degrees, and four cases had no improvement. Postoperative complications included right-limb hemiparesis, diplopia, eyelid ptosis, pulmonary infection, subcutaneous hydrops, cerebrospinal-fluid leakage (CSFL), and intracranial infection (ICI). One patient received gamma knife treatment at 3 months after surgery, two patients died due to tumor progression, and the remaining patients had no tumor recurrence.
CONCLUSIONS
ICDs lack typical imaging features and are often misdiagnosed. The EETA or EEETA helps improve the surgical outcomes and GTR rates of ICDs at different sites.
PubMed: 35692788
DOI: 10.3389/fonc.2022.865865 -
The Cochrane Database of Systematic... Mar 2020This is an updated version of the Cochrane Review previously published in 2016. Epilepsy is a common neurological disorder, affecting 0.5% to 1% of the population. For...
BACKGROUND
This is an updated version of the Cochrane Review previously published in 2016. Epilepsy is a common neurological disorder, affecting 0.5% to 1% of the population. For nearly 30% of these people, their epilepsy is resistant to currently available drugs. Pharmacological treatment remains the first choice to control epilepsy. Lamotrigine is one of the newer antiepileptic drugs. Lamotrigine, in combination with other antiepileptic drugs (add-on), can reduce seizures, but with some adverse effects.
OBJECTIVES
To determine the effects of lamotrigine on (1) seizures, (2) adverse-effect profile, and (3) cognition and quality of life, compared to placebo, when used as an add-on treatment for people with drug-resistant focal epilepsy.
SEARCH METHODS
For the latest update of the review, we searched the following databases on 9 March 2020: Cochrane Register of Studies (CRS Web), MEDLINE (Ovid, 1946 to March 06, 2020). CRS Web includes randomized or quasi-randomized, controlled trials from PubMed, EMBASE, ClinicalTrials.gov, the World Health Organization International Clinical Trials Registry Platform (ICTRP), the Cochrane Central Register of Controlled Trials (CENTRAL), and the Specialized Registers of Cochrane Review Groups including Epilepsy. No language restrictions were imposed.
SELECTION CRITERIA
Randomised placebo-controlled trials of people with drug-resistant focal epilepsy of any age, in which an adequate method of concealment of randomisation was used. The studies were double-, single- or unblinded, placebo-controlled. For cross-over studies, the first treatment period was treated as a parallel trial. Eligible participants were adults or children with drug-resistant focal epilepsy.
DATA COLLECTION AND ANALYSIS
For this update, two review authors independently assessed the trials for inclusion, and extracted data. Outcomes included 50% or greater reduction in seizure frequency, treatment withdrawal (any reason), adverse effects, effects on cognition and quality of life. Primary analyses were by intention-to-treat. Sensitivity best- and worse-case analyses were undertaken to account for missing outcome data. Pooled risk ratios (RRs) with 95% confidence intervals (95% Cls) were estimated for the primary outcomes of seizure frequency and treatment withdrawal. For adverse effects, we calculated pooled RRs and 99% Cls.
MAIN RESULTS
We did not identify any new studies for this update, therefore, the results and conclusions are unchanged. In previous updates of this review, the authors found five parallel add-on studies, eight cross-over studies in adults or children with drug-resistant focal epilepsy, and one parallel add-on study with a responder-enriched design in infants. In total, these 14 studies included 1806 eligible participants (38 infants, 199 children, 1569 adults). Baseline phases ranged from four to 12 weeks; treatment phases from eight to 36 weeks. Overall, 11 studies (1243 participants) were rated as having low risk of bias, and three (697 participants) had unclear risk of bias due to lack of reported information around study design. Effective blinding of studies was reported in four studies (563 participants). The overall risk ratio (RR) for 50% or greater reduction in seizure frequency was 1.80 (95% CI 1.45 to 2.23; 12 trials, 1322 participants (adults and children); moderate-certainty evidence) indicating that lamotrigine was significantly more effective than placebo in reducing seizure frequency. The overall RR for treatment withdrawal (for any reason) was 1.11 (95% CI 0.91 to 1.37; 14 trials; 1806 participants; moderate-certainty evidence). The adverse events significantly associated with lamotrigine were: ataxia, dizziness, diplopia (double vision), and nausea. The RR of these adverse effects were as follows: ataxia 3.34 (99% Cl 2.01 to 5.55; 12 trials; 1525 participants; high-certainty evidence); dizziness 2.00 (99% Cl 1.52 to 2.64;13 trials; 1768 participants; moderate-certainty evidence); diplopia 3.79 (99% Cl 2.15 to 6.68; 3 trials, 944 participants; high-certainty evidence); nausea 1.81 (99% Cl 1.22 to 2.68; 12 studies,1486 participants; moderate-certainty evidence). The limited data available precluded any conclusions about effects on cognition and quality of life. No important heterogeneity between studies was found for any of the outcomes. Overall, we assessed the evidence as high to moderate certainty, due to incomplete data for some outcomes.
AUTHORS' CONCLUSIONS
Lamotrigine as an add-on treatment for drug-resistant focal seizures appears to be effective in reducing seizure frequency, and seems to be fairly well-tolerated. However, the trials were of relatively short duration and provided no evidence for the long term. Further trials are needed to assess the long-term effects of lamotrigine, and to compare lamotrigine with other add-on drugs.
Topics: Adult; Anticonvulsants; Ataxia; Child; Cognition; Cross-Over Studies; Diplopia; Dizziness; Drug Resistance; Drug Resistant Epilepsy; Drug Therapy, Combination; Epilepsies, Partial; Fatigue; Humans; Lamotrigine; Nausea; Patient Dropouts; Quality of Life; Randomized Controlled Trials as Topic; Seizures; Treatment Outcome
PubMed: 32196639
DOI: 10.1002/14651858.CD001909.pub3 -
Journal of Maxillofacial and Oral... Jun 2022Ocular complications during or after dental extraction have been reported in the literature. These complications were either due to infiltration of local anesthetic... (Review)
Review
BACKGROUND
Ocular complications during or after dental extraction have been reported in the literature. These complications were either due to infiltration of local anesthetic solution or due to spread of odontogenic infection extending to the ocular region. The purpose of the present study is to outline the pathophysiological pathway of such ocular complications pertaining to dental procedures due to infiltration/local anesthesia block or spread of odontogenic infection.
METHODOLOGY
An exhaustive literature search was conducted in October 2020 on various online research databases to identify various etiological factors causing ocular complications during dental procedures or followed by odontogenic infections. The findings of all the articles recruited for the review were recorded and analyzed.
RESULTS
The search protocol revealed a total of 897 articles related to the study where only 208 relevant articles were recruited for detailed evaluation, which led to the exclusion of 123 articles and a total of 85 articles were included in the study. Out of the total 113 cases in 85 articles, 92 cases (81.42%) showed complications associated with the use of local anesthetic agent and 21 cases (18.58%) exhibited ocular complications due to spread of infections. Most common ocular complications reported were diplopia, followed by ptosis and ophthalmoplegia.
CONCLUSION
Dental anesthesia can cause a reversible or irreversible ophthalmic complication, while the infectious afflictions of the orbit still remain a challenge to the practicing maxillofacial surgeon.
PubMed: 35712401
DOI: 10.1007/s12663-022-01715-4 -
Frontiers in Neurology 2021IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin, GI...
Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura-An Educative Case and Systematic Review of the Literature.
IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin, GI tract, joints, and kidneys are frequently affected and considered, central nervous system (CNS) involvement of this disease is underestimated. We provide a case report and systematically review the literature on IgAV, collecting data on the spectrum of neurological manifestations. We report on a 7-year-old girl with IgAV who presented with diplopia and afebrile focal seizures, which preceded the onset of purpura. Cranial magnetic resonance imaging was consistent with posterior reversible encephalopathy syndrome (PRES), showing typical focal bilateral parietal swelling and cortical and subcortical high signal intensities on T2-fluid attenuated inversion recovery (FLAIR) images predominantly without diffusion restriction. Cerebrospinal fluid analysis and blood tests excluded systemic inflammation or vasculitis. Interestingly, hypertension was not a hallmark of the developing disease in the initial phase of PRES manifestation. Renal disease and other secondary causes for PRES were also excluded. Supportive- and steroid treatment resulted in restitution . Reviewing the literature, we identified 28 other cases of IgAV with CNS involvement. Severe CNS involvement includes seizures, cerebral edema, or hemorrhage, as well as PRES. Thirteen patients fulfilled all diagnostic criteria of PRES. The mean age was 11.2 years (median 8.0, range 5-42 years), with no reported bias toward gender or ethnic background. Treatment regimens varied from watchful waiting to oral and intravenously steroids up to plasmapheresis. Three cases showed permanent CNS impairment. Collectively, our data demonstrate that (I) severe CNS involvement such as PRES is an underappreciated feature of IgAV, (II) CNS symptoms may precede other features of IgAV, (III) PRES can occur in IgAV, and differentiation from CNS vasculitis is challenging, (IV) pathogenesis of PRES in the context of IgAV remains elusive, which hampers treatment decisions. We, therefore, conclude that clinical awareness and the collection of structured data are necessary to elucidate the pathophysiological connection of IgAV and PRES.
PubMed: 34867743
DOI: 10.3389/fneur.2021.759386