Acute Disseminated Encephalomyelitis followed by Optic Neuritis: A Rare Syndrome of Uncertain Treatment and Prognosis

2020 ◽  
Vol 51 (04) ◽  
pp. 286-291 ◽  
Author(s):  
Maria Serra ◽  
Anna Presicci ◽  
Martina Fucci ◽  
Mariella Margari ◽  
Roberto Palumbi ◽  
...  

Abstract Aim Acute Disseminated Encephalomyelitis followed by optic neuritis (ADEM-ON), first described in 2013, is a rare demyelinating syndrome, typical of the pediatric age. We conducted a mini review of the existing literature, focusing on clinical, laboratory, radiological, therapeutic, and prognostic aspects in order to improve the identification of new cases. Methods We searched PubMed and Cochrane Library for studies on ADEM-ON between 2013 and 2018. Results Examination of the reported cases (three case reports and eight observational studies) established the following features. Time between ADEM and ON is highly variable. Almost all patients show antimyelin oligodendrocyte glycoprotein antibody (MOG-abs) seropositivity. High-dose intravenous steroid and plasmapheresis efficacy is reported for the acute phase; oral prednisone and other maintenance drugs may be useful in avoiding relapses. The clinical history may lead to a complete recovery but also to residual deficits. Conclusion MOG-abs detection strongly supports ADEM-ON diagnosis, confirming this entity as part of MOG-abs spectrum disorder. Owing to the very small number of cases so far reported, predicting clinical evolution is very difficult.

2020 ◽  
pp. 24-28
Author(s):  
Silvina ILUŢ ◽  
Vitalie VACARAS ◽  
M. Roxana RADU ◽  
I. Simina BARAC ◽  
F. Dafin MURESANU

Acute disseminated encephalomyelitis (ADEM) is a disease mainly affecting children, however, adult cases have been also reported. The disease represents a demyelinating disorder of the central nervous system, with a monophasic evolution and mostly full recovery. Mortality is documented at only 2%, but there are risks of complications in the acute phase, mostly due to the vast number of lesions and their distribution in the cerebrum. We present the case of a 40 year-old female patient who presented with visual impairment, coordination issues with walking difficulties, hypoesthesia of the entire body, back and upper limbs paresthesia, upper limbs and torso tremor as well as speech impairment. Symptoms appeared on the same day after discharge from the Infectious Disease Hospital where she was treated for viral meningitis. MRI findings on admission described multiple demyelinating lesions located bilaterally in the white matter and in the cervical spine. The patient was started on high dose parenteral methylprednisolone 1g/day for 5 days and afterwards was switched to oral corticoids with dose tapering over a period of 40 days. Rehabilitation treatment was started during hospitalization and continued after discharge. Evolution was favorable, with almost complete recovery, the patient presenting with only minor hypoesthesia of the torso at discharge. Key words: acute disseminated encephalomyelitis, ADEM, meningitis, rehabilitation,


2020 ◽  
Author(s):  
Ritwick Mondal ◽  
Shramana Deb ◽  
Gourav Shome ◽  
Upasana Ganguly ◽  
Durjoy Lahiri ◽  
...  

Background and aims- Recent reports reveal incidences of spinal cord involvement in form of para-infectious or post-infectious myelitis raising potential concerns about the possibilities of SARS-CoV-2 behind the pathogenesis of spinal cord demyelination. In this study, we intend to summarise so far available pieces of evidence documenting SARS-CoV-2 mediated spinal demyelination in terms of clinical, laboratory parameters and imaging characteristics. Methodology- This review was carried out based on the existing PRISMA (Preferred Report for Systemic Review and Meta-analyses) consensus statement. Data was collected from four databases: Pubmed/Medline, NIH Litcovid, Embase and Cochrane library and Preprint servers up till 10th September, 2020. Search strategy comprised of a range of keywords from relevant medical subject headings which includes "SARS-COV-2", "COVID-19", "demyelination" etc. Results- A total of 21 cases were included from 21 case reports after screening from various databases and preprint servers. Biochemical analysis reveals that the majority of cases showed elevated CSF protein as well as lymphocytic pleocytosis. Interestingly, a majority of cases were found to be associated with long extensive transverse myelitis (LETM), and remaining cases were found to be associated with isolated patchy involvement or isolated short segment involvement or combined LETM and patchy involvement. Few cases were also found with significant co-involvement of the brain and spine based on the imaging data. Conclusion- It can be interpreted that SARS-CoV-2 may play a potential role in spinal demyelinating disorders in both para-infectious and post-infectious forms.


2020 ◽  
Vol 5 (3) ◽  
pp. 575-587 ◽  
Author(s):  
Danni Li ◽  
Angela Ferguson ◽  
Mark A Cervinski ◽  
Kara L Lynch ◽  
Patrick B Kyle

Abstract Background Laboratory tests that use streptavidin–biotin binding mechanisms have the potential to be affected by high circulating biotin concentrations, which would produce positive and negative interference in biotinylated competitive and noncompetitive (sandwich) immunoassays, respectively. Consumption of high-dose biotin supplements for cosmetic or health-related reasons has drawn attention to biotin interference in clinical laboratory tests. Case reports and in vivo studies show that ingestion of supplemental biotin can cause clinically significant errors in select biotinylated immunoassays. Content This AACC Academy document is intended to provide guidance to laboratorians and clinicians for preventing, identifying, and dealing with biotin interference. In vivo and in vitro spiking studies have demonstrated that biotin concentrations required to cause interference vary by test and by manufacturer. This document includes discussion of biotin’s mechanisms for interference in immunoassays, pharmacokinetics, and results of in vitro and in vivo studies and cites examples of assays known to be affected by high biotin concentrations. This document also provides guidance recommendations intended to assist laboratories and clinicians in identifying and addressing biotin interference in laboratory testing. Summary The recent increase in the use of high-dose biotin supplements requires laboratorians and clinicians to be mindful of the potential for biotin interference in biotinylated immunoassay-based laboratory tests. Laboratories, clinicians, regulators, and patients should work together to ensure accurate laboratory results. Laboratories have several options for identifying suspected biotin interference in specimens. Alternatively, the relatively fast elimination of biotin allows the potential for rapid follow-up specimen analysis if necessary.


Biomedicines ◽  
2021 ◽  
Vol 9 (3) ◽  
pp. 291
Author(s):  
Eva Ausó ◽  
Violeta Gómez-Vicente ◽  
Gema Esquiva

Phosphodiesterase type 5 (PDE5) inhibitors such as Viagra® (sildenafil citrate) have demonstrated efficacy in the treatment of erectile dysfunction (ED) by inducing cyclic guanosine monophosphate (cGMP) elevation followed by vasodilation and increased blood flow. It also exerts minor inhibitory action against PDE6, which is present exclusively in rod and cone photoreceptors. The effects of sildenafil on the visual system have been investigated in a wide variety of clinical and preclinical studies due to the fact that a high dose of sildenafil may cause mild and transient visual symptoms in some patients. A literature review was performed using PubMed, Cochrane Library and Clinical Trials databases from 1990 up to 2020, focusing on the pathophysiology of visual disorders induced by sildenafil. The aim of this review was not only to gather and summarize the information available on sildenafil clinical trials (CTs), but also to spot subpopulations with increased risk of developing undesirable visual side effects. This PDE inhibitor has been associated with transient and reversible ocular side effects, including changes in color vision and light perception, blurred vision, photophobia, conjunctival hyperemia and keratitis, and alterations in the electroretinogram (ERG). Sildenafil may induce a reversible increase in intraocular pressure (IOP) and a few case reports suggest it is involved in the development of nonarteritic ischemic optic neuropathy (NAION). Reversible idiopathic serous macular detachment, central serous retinopathy and ERG disturbances have been related to the significant impact of sildenafil on retinal perfusion. So far, sildenafil does not seem to cause permanent toxic effects on chorioretinal tissue and photoreceptors as long as the therapeutic dose is not exceeded and is taken under a physician’s direction to treat a medical condition. However, the recreational use of sildenafil can lead to harmful side effects, including vision changes.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 573.2-574
Author(s):  
N. Citeroni ◽  
S. Rampes ◽  
P. Kotecha ◽  
J. Schoones ◽  
E. Nikiphorou ◽  
...  

Background:Hydroxychloroquine (HCQ) has attracted much attention especially during the COVID-19 pandemic. HCQ and closely related chloroquine (CQ) have known ocular and cardiac toxicity. However, although screening guidance now exists from the Royal College of Ophthalmologists for the former (RCOphth)1, little is known regarding predictors of both forms of toxicity.Objectives:To systematically explore the literature on predictors of toxicity limited to cardiac and ocular toxicity.Methods:A detailed search of the following databases was conducted: PubMed, Medline, Embase, Web of Science, The Cochrane library, EMCare and Academic Search Premier. Studies addressing predictors of HCQ toxicity with relevant search terms used were included. Exclusion criteria were: non-English articles, pre-clinical and paediatric studies. Three authors (SR, NC, PK) independently screened titles and abstracts for inclusion, ensuring each article was screened twice. Disagreement over inclusion was adjudicated by senior reviewers (EN, JG). Data extraction (SR, NC, PK) focused on predictors of toxicity.Results:The search strategy retrieved 3103 studies. 147 studies were included for data extraction, of which 92 were eventually excluded due to: not identifying predictors (n=17), reviews (n=19), not ocular or cardiovascular toxicity (n=18), case reports (n=3), paediatrics (n=1), screening articles that focused on detecting retinopathy (n=30), article unobtainable at time of abstract submission (n=4), leaving 55 studies for full review.Studies addressing cardiac toxicity (n=16) included: cohort, retrospective observational, a comparative pharmacovigilance, systematic monitoring protocol and a randomised control trial (RCT). The majority of these involved high-dose (>5mg/kg/day) use for acute COVID-19 infection. The main significant predictors identified were: Hydroxychloroquine (HCQ) use in combination with azithromycin (6/7 studies), cumulative dose (2 studies), pre-existing cardiovascular morbidity (2/3 studies) and prolonged baseline QTc (2 studies). Individual associations were also identified in the following: longer treatment duration, daily dosage, increased age, male gender, severe COVID-19 infection, abnormal liver function tests (LFTs) and concurrent use of loop-diuretics.Regarding predictors of ocular toxicity (n=39), only one study was a RCT. The remainder were observational studies: case-control, cohort, retrospective chart reviews and letters to the editor which included original patient data.Several predictors of retinopathy and maculopathy were examined in two or more studies and included: duration of use (16/18 studies), daily dosage (7/13 studies), cumulative dose (11/14 studies), increased age (10/11 studies), body weight or BMI (3/5 studies), renal impairment (5 studies), HCQ blood levels (2 studies), keratopathy (2 studies) and tamoxifen use (2 studies). Sex (2 studies) and history of cataract surgery (2 studies) were not found to be predictors of toxicity.Only few studies performed regression analysis presenting odds and/or hazard ratios with confidence intervals.Conclusion:The most recognised predictor of cardiac toxicity was co-administration with azithromycin. In ocular toxicity, commonly cited predictors included those already recognised by the RCOphth1, as well as cumulative dose, increased age, weight considerations, HCQ blood levels and keratopathy. Further research is warranted on better characterising predictors of cardiac and ocular toxicity in patients on HCQ and CQ therapy.References:[1]The Royal College of Ophthalmologists. Hydroxychloroquine and Chloroquine Retinopathy: Recommendations on Monitoring [Internet]. The Royal College of Ophthalmologists; 2020 [cited 2021 Jan 25].Disclosure of Interests:Natasha Citeroni: None declared, Sanketh Rampes: None declared, Pinky Kotecha: None declared, Jan Schoones: None declared, Elena Nikiphorou: None declared, James Galloway Speakers bureau: JG has received honoraria / speaker fees from Abbvie, BMS, Celgene, Chugai, Gilead, Janssen, Lilly, Novartis, Pfizer, Roche, Sanofi, Sobi and UCB.


2019 ◽  
Vol 6 (4) ◽  
pp. e567 ◽  
Author(s):  
Jocelyne Beelen ◽  
Susanne M. Benseler ◽  
Anastasia Dropol ◽  
Brianna Ghali ◽  
Marinka Twilt

ObjectiveChildhood primary angiitis of the CNS (cPACNS) is a devastating neurologic disease. No standardized treatment protocols exist, and evidence is limited to open-label cohort studies and case reports. The aim of this review is to summarize the literature and provide informed treatment recommendations.MethodsA scoping review of cPACNS literature from January 2000 to December 2018 was conducted using Ovid, MEDLINE, PubMed, Embase, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, ClinicalTrials.gov, Vasculitis Foundation, European Vasculitis Society, CanVasc, Google Scholar, and Web of Science. Potentially relevant articles were selected for full-text review using the STROBE checklist if they met the following inclusion criteria: (1) reported treatment, (2) addressed pediatrics, (3) focused on the disease of interest, (4) included ≥5 patients, (5) original research, and (6) full-length articles. Reviews, expert opinions, editorials, case reports with <5 patients, articles lacking treatment information, or non-English articles were excluded. A standardized assessment tool measured study quality. Treatment and outcomes were summarized.ResultsOf 2,597 articles screened, 7 studies were deemed high quality. No trials were available so no meta-analysis was possible. Overall, treatment strategies recommended are induction with acute antithrombotic therapy subsequently followed by high-dose oral prednisone taper over 3–12 months and long-term platelet therapy. In angiography-positive progressive–cPACNS and angiography-negative–cPACNS, we also recommend 6 months of IV cyclophosphamide therapy, with trimethoprim/sulfamethoxazole as part of induction, and maintenance therapy with mycophenolate mofetil/mycophenolic acid.ConclusionNo grade-A evidence exists; however, this review provides recommendations for treatment of cPACNS.


2021 ◽  
Author(s):  
Juliana Oliveira de Almeida ◽  
Alice Cavalcante de Almeida Lins ◽  
Bruna Acioly Leão ◽  
Patrícia Pereira Nunes ◽  
Nayra Roberta Sales Salvador

Context: In Brazil, schistosomal myelitis is the third most common cause of myelopathy. The diagnosis of neuroschistosomiasis is not easy to make. In a study published in 2001, the author proposed a classification for the diagnosis of schistosomal myelorradiculopathy: Proven Diagnosis; Probable Diagnosis and Possible Diagnosis. Case Reports: Case 1, admitted in 2021 with 40 days of paraparesis and ascending paraesthesia, urinary retention and severe low back pain. He had proximal and distal grade 1 paraparesis, eosinophilia at blood count, CSF with hyperproteinorrhachia, normal cellularity and indirect immunofluorescence (IFI) for reactive schistosomiasis, as well as stool parasitology (EPF) and spine MRI with hypersignal in the spinal cord. Case 2, admitted in 2020 with 20 days of constipation and urinary retention followed by paraparesis and ascending paresthesia and sexual impotence. He presented proximal and distal grade 3 paraparesis and CSF with protein-cytological dissociation. Both had abolished deep reflexes and hypoaesthesia with levels in L2 and T10, respectively. As a result of the other exams and MRI with hypersignal of D6 to medullary cone with contrast impregnation and cauda equina roots, despite case 2 having EPF and IFI for schistosomiasis in the CSF negative, for presenting positive epidemiology for schistosomiasis, both were treated with solumedrol for 5 days, praziquantel and oral prednisone, with improvement afterwards. Conclusion: The outcome of schistosomal myelitis is directly related to early diagnosis and treatment. It is necessary to value all clinical, laboratory and epidemiological findings in patients living in an endemic area.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 863.1-864
Author(s):  
C. A. Pereda Testa ◽  
M. B. Nishishinya ◽  
P. Díaz del Campo Fontecha ◽  
N. Brito-García

Background:The incidence of Pneumocistis jirovencii pneumonia (PCP) has increased substantially during the past years in patients with systemic autoimmune diseases (SAD). Mortality associated to PCP was reported to be up to 20 to 58%, particularly in those receiving immunosuppressive therapy, such as tumoral necrosis antagonist factors or glucocorticoid therapy. Though, there is clear evidence of the effectiveness of Cotrimoxazole against PCP, the risk of adverse effects is important, increasing morbidity and mortality. Up to date, there is no consensus about the need of PCP prophylaxis in SAD patients with immunosuppressed therapies.Objectives:To analyse the efficacy and safety of Cotrimoxazole prophylaxis against PCP in SAD adult patients receiving immunosuppressive therapies.Methods:We performed a comprehensive literature search, screening different databases, MEDLINE, EMBASE and Cochrane Library up to April 2019. Outcomes covered prevention of PCP or other infections, morbidity, mortality and safety. All categories of studies were included. Two reviewers selected and extracted data from studies. The information obtained was summarized through a narrative review and results tabulated.Results:From the initial 340 identified references, 12 were finally included. Two were randomized controlled trials, six observational studies, and four case reports. The quality in the majority of studies resulted moderate or low, with limited level of evidence. Besides, all Cotrimoxazole prophylaxis regimens described in each study were distinct. Results were consistent to exhibit the efficacy of Cotrimoxazole prophylaxis, compared to non-prophylaxis in the prevention of PCP in patients receiving immunosupresor therapy, particularly, those taking high glucocorticoid dose above 20mg/day. In terms of efficacy, Cotrimoxazole 400mg/80mg/day, given three times per week, or 200mg/40mg/day or in dose escale exhibited a similar performance. In contrast, Cotrimoxazole 400mg/80mg/day displayed a higher incidence of adverse effects.Conclusion:Cotrimoxazole prophylaxis against PCP exhibited efficacy compared to non-prophylaxis, mainly in patients treated with high dose of glucocorticoids (≥20mg/day), causing a significant reduction in mortality. Positive efficacy results did not differ despite the diverse Cotrimoxazol regimens exposed. However, Cotrimoxazole adverse effects were observed after two months from initiation; particularly with daily dose of 400mg/80mg. In contrast, escalate dose or 200mg/40mg/day dose regimens appeared better tolerated.**This review is part of the Spanish Rheumatology Society –SER- recommendations on Systemic Autoimmune Diseases.References:[1] Utsunomiya, M., et al. (2017). “Optimal regimens of sulfamethoxazole-trimethoprim for chemoprophylaxis of Pneumocystis pneumonia in patients with systemic rheumatic diseases: results from a non-blinded, randomized controlled trial.” Arthritis Res Ther 19(1): 7.[2] Yamamoto, T., et al. (2014). “A feasibility study assessing tolerability of daily versus twice weekly trimethoprim-sulfamethoxazole regimen for prophylaxis against pneumocystis pneumonia in patients with systemic autoimmune diseases on glucocorticoid therapy.” Japanese Journal of Clinical Pharmacology and Therapeutics 45(3): 89-92.Disclosure of Interests:None declared


2020 ◽  
pp. 089033442097049
Author(s):  
Johnson Jeslin ◽  
C. Seetharaman ◽  
Virna M. Shah

Background Optic neuritis is a rare condition that can lead to sudden blindness and also could be a precursor to multiple sclerosis. When it occurs postpartum during lactation, it is called lactation optic neuritis. Main issue We present four cases of optic neuritis in lactating mothers, two of which had additional features of demyelinating disease upon neurological imaging. Management All participants were treated with high dose intravenous steroids followed by 11 days of oral steroids, per the optic neuritis treatment trial, which led to complete recovery of vision. Two participants with demyelinating disease on magnetic resonance imaging scans were advised to wean, because of a need for immunosuppressive therapy later. Conclusion Optic neuritis during lactation should be suspected following acute loss of vision. Prompt referral to an ophthalmologist is mandated for early diagnosis and treatment to prevent long-term co-morbidities.


Author(s):  
Christian Zanza ◽  
Yaroslava Longhitano ◽  
Marco Artico ◽  
Gianmaria Cammarota ◽  
Andrea Barbanera ◽  
...  

Background: in the last years, ultrasound technology has entered in clinical practice as a tank and today, it has also allowed to no-cardiologists to extend and to deep their medical examination without the needing to call the consultant and having a good profile of diagnostic accuracy. The ultrasound bedside does not replace the consultant but it allows not to perform inappropriate consultations with more savings for hospitals. Objective: The aim was to review recently published literature to inform the clinician about the most up to date management of use bedside echography in emergency setting. In this short review we focused on two types of syndromes, no traumatichypotension and dyspnea, common to the three holistic disciplines of medicine, showing the main and basic questions and answers that ultrasound can give us for rapid identification of the problem Methods: We conducted a systematic review using Pubmed/Medline, Ovid/Willey and Cochrane Library, combining key terms such as “cardiac ultrasound, “cardiac diseases”,“emergency medicine”,“pocus”, “dyspnea”,“ hypotension”. We selected the most relevant clinical trials and review articles (excluding case reports) published in the last 19 years and in our opinion 59 publications appeared the best choice according to the PRISMA statement. In additional papers identified from individual article reference lists were also included. Conclusion: Recent studies have shown promise in establishing best practices for evaluation of heart, lung abdomen and deep vessels At the moment bedside US is widely used in an integrated ultrasound vision just like the holistic view have internal medicine, intensive care and emergency medicine and many medical schools in Europe and the USA are inserting ultrasonography into the core curriculum but we still have to find a standard method for the training program for minimum competence acquisition.


Sign in / Sign up

Export Citation Format

Share Document