scholarly journals Acute Disseminated Encephalomyelitis after endophthalmitis in immunosuppresed patient

2021 ◽  
Author(s):  
Clara Kimie Miyahira ◽  
Beatriz Medeiros Correa ◽  
Raphael Palomo Barreira ◽  
Thomas Zurga Markus Torres ◽  
Natália Figueiredo Miranda ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Motor Deficits ◽  
Social Risk ◽  
Blurred Vision ◽  
Mental Confusion ◽  
Surgery Patient ◽  
First Choice ◽  
History Of

Context: Acute Disseminated Encephalomyelitis (ADEM) usually happens after an infectious event, vaccination reaction and with history of immunosuppression. Clinical features can be varied: headache, fever, seizure, blurred vision, ataxia, motor deficits and mental confusion. Case report: A 35-yearsold woman, in a social risk situation, complained about pain and blurred vision, history of cocaine abuse, victim of sexual and physical abuse and recent Measles vaccination. She had corneal ulcer with bilateral endophthalmitis and optic nerve infection. Despite intravitreal injections of Vancomycin + Ceftazidime, patient had to eviscerate the left eye. After surgery, patient evolved with mental confusion and paraparesis, CSF cell 69, lymphomonocyte, proteins 257, MRI showed central bulbar hyperintensity, lesions in the dentate nucleus and periaqueductal of gray substance in T2 / FLAIR, thoracic and lumbar spine with demyelinating pattern, extensive longitudinal myelitis. Patient obtained clinical improvement after treatment with Methylprednisolone 1gr for 5 days. It is important to discard other hypothesis: transverse myelitis, neuromyelitis optica, multiple sclerosis and systemic lupus erythematosus. The diagnosis is clinical and radiological with multifocal and confluent areas of hypersignal on MRI, CSF analysis reveals high protein and lymphocytic pleocytosis. Treatment with Methylprednisolone is the first choice and then Plasmapheresis. Conclusion: there are several risk factors for the development of ADEM correlated with a rapid and aggressive evolution in this patient.

Neuromyelitis optica spectrum disorder in a patient with systemic lupus erythematosus and anti-phospholipid antibody syndrome

2008 ◽  
Vol 14 (3) ◽  
pp. 425-427 ◽  
Author(s):  
Lahar R Mehta ◽  
Melissa K Samuelsson ◽  
Anatole K Kleiner ◽  
Andrew D Goodman ◽  
Jennifer H Anolik ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Specific Marker ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
History Of ◽  
Recurrent Transverse Myelitis

Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system characterized by severe episodes of optic nerve and spinal cord inflammation. NMO-IgG (anti-aquaporin-4) has been recently described as a sensitive and specific marker for NMO. As there have been prior published reports of an association between NMO and systemic autoimmune diseases, the prognostic value of the antibody test in these cases is uncertain. We describe a 47-year old woman with recurrent transverse myelitis and a long-standing history of systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APLS). While she did not have a history of optic neuritis, serological testing for the NMO-IgG was positive when she was admitted for her second episode of transverse myelitis. Testing for the NMO-IgG in cases of isolated or recurrent transverse myelitis attributed to current SLE and APLS may help clarify the diagnosis of a distinct disease process likely to cause recurrent and severe disability, warranting more aggressive immunotherapy. Multiple Sclerosis 2008; 14: 425—427. http://msj.sagepub.com

A-80 Psychosocial Functioning in Pediatric Anti-MOG Associated Disease

2021 ◽  
Vol 36 (6) ◽  
pp. 1125-1125
Author(s):  
Erin E Kennedy ◽  
Ana Hernandez ◽  
Benjamin Greenberg ◽  
Patricia Plumb ◽  
Cynthia Wang ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Academic Performance ◽  
Psychosocial Functioning ◽  
Social Problems ◽  
Transverse Myelitis ◽  
Psychosocial Problems ◽  
Acute Disseminated Encephalomyelitis ◽  
Self Report ◽  
History Of

Abstract Objective Anti-myelin oligodendrocyte glycoprotein associated disease (MOGAD) is a newly recognized demyelinating disorder, most commonly associated with acute disseminated encephalomyelitis (ADEM), optic neuritis and/or transverse myelitis. Given that psychosocial problems have previously been noted in individuals with demyelinating disorders, including ADEM, the primary aims of this study were to examine psychosocial functioning in patients with MOGAD and explore differences between subjects with a history of ADEM and those without. Method Thirty-two subjects with MOGAD (M age = 10.57; ADEM = 59.4%) and their caregivers completed questionnaires (Behavior Assessment Scales for Children, Pediatric Quality of Life, Educational History Form) to assess psychosocial functioning including externalizing (EXT) and internalizing behaviors (INT), quality of life (QOL), and academic performance. Results Overall, caregiver-ratings of internalizing and externalizing behaviors were not significantly different from the normative mean (EXT: p = 0.307; INT: p = 0.775). Caregiver and self-rated QOL was significantly below the normative mean for school and social functioning (p = < 0.05), as was self-rated emotional functioning (p = 0.026). Further analysis did not reveal significant differences between subjects who had ADEM vs. those who did not across caregiver ratings or academic performance. Self-ratings revealed significantly more social problems for subjects with a history of ADEM (p = 0.018). Conclusions MOGAD may be associated with few caregiver-reported behavioral or emotional problems. While pediatric patients with MOGAD are noted to have academic and social problems per caregiver report, self-report further indicates problems with emotional QOL and greater risk of social problems for those with a history of ADEM. Additional research is needed to further understand the psychosocial features associated with MOGAD.

Inflammatory disorders of the central nervous system

2016 ◽  
Author(s):  
Sathiji Nageshwaran ◽  
Heather C Wilson ◽  
Anthony Dickenson ◽  
David Ledingham
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Evidence Based Treatment ◽  
System P ◽  
Primary Angiitis ◽  
Autoimmune Limbic Encephalitis ◽  
The Central Nervous System

Primary neuroinflammatory disorders of the central nervous system (multiple sclerosis, neuromyelitis optica (NMO), transverse myelitis, optic neuritis, acute disseminated encephalomyelitis (ADEM), primary angiitis of the central nervous system, autoimmune limbic encephalitis, and Susac’s syndrome) and multisystem diseases with inflammatory involvement of the central nervous system (sarcoidosis, systemic lupus erythematosus (SLE), giant cell arteritis, Behçet’s disease, Sjögren’s, and other vasculitides) are discussed in depth, covering the aetiology, clinical features and evidence-based treatment.

Anti-Myelin Oligodendrocyte Glycoprotein Encephalomyelitis and Extensive Longitudinal Transverse Myelitis Associated with Compound Heterozygous NLRP3 Missense Mutations in a Young Child

2020 ◽  
Author(s):  
Deirdre O'Sullivan ◽  
Michael Moore ◽  
Susan Byrne ◽  
Andreas O. Reiff ◽  
Susanna Felsenstein
Keyword(s):  
Young Child ◽  
Genetic Basis ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Compound Heterozygous ◽  
Missense Mutations ◽  
Childhood Onset

AbstractAcute disseminated encephalomyelitis in association with extensive longitudinal transverse myelitis is reported in a young child with positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody with heterozygous NLRP3 missense mutations; p.(Arg488Lys) and p.(Ser159Ile). This case may well present an exceptional coincidence, but may describe a yet unrecognized feature of the spectrum of childhood onset cryopyrinopathies that contribute to the understanding of the genetic basis for anti-MOG antibody positive encephalomyelitis. Based on this observation, a larger scale study investigating the role of NLRP3 and other inflammasomes in this entity would provide important pathophysiological insights and potentially novel avenues for treatment.

scholarly journals When systemic lupus erythematosus affects vision: a rare presentation of this condition

2020 ◽  
Vol 13 (1) ◽  
pp. e229382
Author(s):  
Tiago Gama Ramires ◽  
Luísa Vieira ◽  
Nuno Riso ◽  
Maria Francisca Moraes-Fontes
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Blurred Vision ◽  
Rare Presentation ◽  
Complement Components ◽  
Oral Ulcers ◽  
Dsdna Antibody ◽  
Rare Manifestation ◽  
Retinal Vascular Tortuosity

A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy. Steroid therapy alone was highly effective and was also accompanied by a normalisation of haemoglobin and lymphocyte counts, after which azathioprine was added. Hydroxychloroquine was introduced after resolution of retinal changes. Immunosuppressive therapy was progressively tapered over the course of 12 months and then discontinued, and the patient remains in remission 48 months after the initial presentation. Our patient exemplifies a very rare manifestation of systemic lupus erythematosus. We emphasise the importance of its early detection and complexity of treatment in order to reduce visual morbidity.

scholarly journals Role of MRI evaluation in acute secondary inability to walk in children

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
K. H. Sedeek ◽  
K. Aboualfotouh ◽  
S. M. Hassanein ◽  
N. M. Osman ◽  
M. H. Shalaby
Keyword(s):  
Transverse Myelitis ◽  
High Sensitivity ◽  
Acute Disseminated Encephalomyelitis ◽  
Limb Weakness ◽  
Non Invasive ◽  
Highly Sensitive ◽  
Common Causes ◽  
Bilateral Lower Limb ◽  
Severity Of The Disease

Abstract Background Acute bilateral lower limb weakness is a common problem in children which necessitates a rapid method for diagnosis. MRI is a non-invasive imaging technique that produces high-quality images of the internal structure of the brain and spinal cord. Results MRI was very helpful in reaching rapid and prompt diagnosis in children with acute inability to walk. Acute disseminated encephalomyelitis (ADEM), Guillain–Barré syndrome (GBS), and acute transverse myelitis (ATM) were the most common causes in our study. MRI proved to be of high sensitivity in detecting the lesions and reaching the diagnosis in ADEM and GBS; however, there was no significant relation between the lesions’ size, enhancement pattern, and severity of the disease or prognosis, yet in ATM the site of the lesion and number of cord segment affection were significantly related to the severity of the disease and prognosis. Conclusion MRI is a quick tool to reach the diagnosis of children with acute secondary inability to walk, and to eliminate other differential diagnosis which is essential for proper treatment and rapid full recovery. It is highly sensitive in detecting the lesions, their site and size.

scholarly journals AB0420 RISK OF DEVELOPING TYPE 2 DIABETES MELLITUS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1510.2-1510
Author(s):  
L. Kondrateva ◽  
T. Popkova ◽  
E. Nasonov ◽  
A. Lila
Keyword(s):  
Abdominal Obesity ◽  
Lupus Erythematosus ◽  
Antihypertensive Drugs ◽  
Control Group ◽  
Systemic Lupus ◽  
Type 2 Dm ◽  
History Of ◽  
Systemic Rheumatic Diseases ◽  
Very High

Background:Patients with systemic lupus erythematosus (SLE) have higher than in general population prevalence of diabetes mellitus (DM). Hyperinsulinemia is a predictor of developing type 2 DM, however routine measurement of insulin levels for DM risk assessment is uncomfortable in daily clinical practice. International Diabetes Federation recommends the use of patient questionnaires to quickly identify people who may be at a higher risk of DM development.Objectives:To determine the 10-years risk of developing type 2 DM in SLE patients using dedicated questionnaire - Finnish Type 2 Diabetes Risk Assessment Form (FINDRISK) data.Methods:The study included 92 SLE patients without DM (83 women, 9 men, 39 [34; 47] years old). The median disease duration was 6 [2,14] years, SLEDAI-2K was 4[2;8]. SLE pts were treated with glucocorticoids (GC) (89%) and hydroxychloroquine (78%), immunosuppressive drugs (28%) and biological agents (10%). The control group consisted of 88 subjects without systemic rheumatic diseases, inflammatory arthritis or DM, matched by age and sex with SLE patients. Eight items of FINDRISK questionnaire (age, overweight, abdominal obesity, family history of diabetes, physical inactivity, eating habits, history of antihypertensive drugs treatment, history of hyperglycemia) were taken into account to calculate the total risk score (TS). The risk of developing DM within following 10 years is regarded as low (1%) or slightly elevated (4%) with TS ≤11 points, as moderate (17%), high (33%) or very high (50%) with TS ≥12 points.Results:The risk of developing DM was low or slightly elevated in 65 (71%) SLE pts and moderate, high or very high in 27 (29%) pts. The difference was significant compared with the control group, in which 76 (86%) subjects had a low or slightly elevated risk and 12 (14%) had a moderate, high or very high risk (p=0,01). The number of risk factors (4[2;5]) and the median TS of SLE pts (9[5;12] points) were higher than values in control subjects (3[2,4] factors and 6[3;9] points, respectively) (p<0,01 for both). DM risk factors profiles were similar in two groups, except for higher prevalence of abdominal obesity (66% vs 41%, p<0,01) and history of antihypertensive drugs treatment (57% vs 17%, p<0,01) in SLE. There were positive correlations between TS and CRP levels (r=0,25, p=0,02), SLICC (r=0,36, p<0,01), HAQ (r=0,29, p<0,01), and negative correlations between TS and SLEDAI-2K (r= -0,32, p<0,01), glomerular filtration rate by CKD-EPI (r=-0,23, p=0,03). Current GC use had no influence on TS values in SLE.Conclusion:Patients with SLE were more likely than individuals without systemic rheumatic diseases to have a moderate, high and very high risk of developing DM, and therefore, required interventions to prevent the metabolic disease. Increased risk of developing DM was associated with most common traditional factors, especially by abdominal obesity and regular use of antihypertensive drugs that can be considered a kind of equivalent to the presence of hypertension. Curtain contribution of inflammation, lupus activity and irreversible damage index can’t be ignored. Clarification of SLE-specific phenomena in DM pathogenesis requires further research.Disclosure of Interests: :None declared

scholarly journals “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case

Lupus ◽  
2021 ◽  
pp. 096120332098176
Author(s):  
Sarah J van der Lely ◽  
Jeffrey Boorsma ◽  
Marc Hilhorst ◽  
Jesper Kers ◽  
Joris Roelofs ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Gestational Trophoblastic Disease ◽  
Grand Round ◽  
Placental Site Trophoblastic Tumor ◽  
Trophoblastic Tumor ◽  
Anchoring Bias ◽  
Placental Site ◽  
History Of

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.

“Protenuria in SLE: Is it always lupus?”

Lupus ◽  
2021 ◽  
pp. 096120332098345
Author(s):  
Alessandra Ida Celia ◽  
Roberta Priori ◽  
Bruna Cerbelli ◽  
Francesca Diomedi-Camassei ◽  
Vincenzo Leuzzi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Fabry Disease ◽  
Histological Examination ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Kidney Involvement ◽  
History Of ◽  
Genetic Assay

Proteinuria is one of the most typical manifestations of kidney involvement in Systemic Lupus Erythematosus (SLE). We report the case of a 23-year-old woman with a 6-year-long history of SLE presenting with proteinuria after a three-year remission on hydroxychloroquine. Kidney histological examination showed alterations inconsistent with lupus nephritis and suggestive of hydroxychloroquine toxicity or Fabry disease. The latter was confirmed by genetic assay.

scholarly journals AB0283 CHOREOATHETHOSIS AND LONGITUDINALLY EXTENSIVE TRANSVERSE MYELITIS AS RARE AND SEVERE MANIFESTATIONS OF NEUROPSYCHIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS AND PRIMARY SJÖGREN’S SYNDROME – A TWO-CENTER EXPERIENCE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1167.2-1168
Author(s):  
P. Korsten ◽  
M. Plüß ◽  
S. Glaubitz ◽  
A. Jambus ◽  
R. Vasko ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Complete Remission ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Primary Sjögren’S Syndrome ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Systemic Lupus ◽  
Primary Sjogren’S Syndrome ◽  
Life Threatening ◽  
Extensive Transverse Myelitis

Background:Systemic lupus erythematosus (SLE) can affect almost any organ system. Nevertheless, Lupus nephritis and neuropsychiatric manifestations (NPSLE) are associated with increased mortality (1). Therapeutic options include glucocorticoids, often pulse methylprednisolone (MP), and other immunosuppressive therapies. In refractory cases, therapeutic plasma exchange, rituximab, or intravenous immunoglobulins are often used (2). However, an optimal therapeutic strategy has not been established because NPSLE is an exclusion criterion in most clinical trials. In addition, NPSLE can present with a broad spectrum of manifestations ranging from cognitive dysfunction to severe and life-threatening disease with choreoathetosis or transverse myelitis (TM). In primary Sjögren’s syndrome (pSS), neurological manifestations most often include peripheral neuropathies, but TM has also been reported.Objectives:To analyze the clinical presentation and outcomes after treatment in severe, life-threatening NPSLE.Methods:We retrospectively analyzed clinical, laboratory, and imaging features in severe NPSLE manifestations in SLE and pSS patients at two tertiary academic centers (University Medical Center Göttingen, Germany, and ASST Spedali Civili Brescia, Italy) with a high volume of SLE patients. Severe NPSLE was defined as either severe movement disorder or extensive tetra- or paraplegia secondary to (longitudinally extensive) transverse myelitis.Results:Our retrospective chart review resulted in seven patients fulfilling the inclusion criteria (six with SLE and 1 with pSS). Of these, five were females (71.4%). Median age was 26 (16-55) years. Three were of Asian origin, four were of European descent. Median disease duration was 15 (2-228) months. Three patients presented with severe choreoathetosis, all had positive ANA, anti-dsDNA antibodies (abs), and complement consumption. Of note, all three had at least one positive antiphospholipid antibody (APLA). All patients received IV MP 1g x 3 and mycophenolate mofetil and achieved complete remission. Of the four patients with longitudinally extensive TM, all were ANA positive, only two had anti-dsDNA abs. None of them had APLA, and only one tested positive for anti-aquaporine-4 abs. Of all patients, only one had positive ribosomal P-abs. Patients with TM received IV MP 1g x 5 and either RTX (4 cycles with 375 mg/m2 or IVIg 0.4 g/kg/d x 5). All four TM patients improved; two improved markedly, two only moderately with residual deficits as assessed by EDMUS-grading scale and functional independence measure.Conclusion:Severe NPSLE, defined as choreoathetosis or TM require intensive treatment. While the former patients achieved complete remission, two of four patients with TM only achieved partial remission. Our data support the use of early and aggressive immunosuppressive therapy. Nevertheless, therapy for TM in the context remains insufficient and should be assessed in a controlled clinical trial setting.References:[1]Monahan RC, et al. Mortality in patients with systemic lupus erythematosus and neuropsychiatric involvement: A retrospective analysis from a tertiary referral center in the Netherlands. Lupus. 2020 Dec;29(14):1892–901.[2]Papachristos DA, et al. Management of inflammatory neurologic and psychiatric manifestations of systemic lupus erythematosus: A systematic review. Semin Arthritis Rheum. 2020 Dec 17;51(1):49–71.Disclosure of Interests:PETER KORSTEN Consultant of: PK has received honoraria by Abbvie, Bristol-Myers-Squibb, Chugai, Gilead, Glaxo Smith Kline, Janssen-Cilag, Pfizer, and Sanofi-Aventis, all unrelated to this study., Grant/research support from: PK has received research grants from GSK, unrelated to this study., Marlene Plüß: None declared, Stefanie Glaubitz: None declared, Ala Jambus: None declared, Radovan Vasko: None declared, Bettina Meike Göricke: None declared, Silvia Piantoni: None declared

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