scholarly journals Autoimmune Encephalitis Post-SARS-CoV-2 Infection: Case Frequency, Findings, and Outcomes

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012931
Author(s):  
Cristina Valencia Sanchez ◽  
Elitza Theel ◽  
Matthew Binnicker ◽  
Michel Toledano ◽  
Andrew McKeon
Keyword(s):  
Diagnostic Criteria ◽  
Mayo Clinic ◽  
Immune Therapy ◽  
Case Series ◽  
Autoimmune Encephalitis ◽  
Systemic Infection ◽  
Diagnostic Features ◽  
Metabolic Encephalopathy ◽  
Memory Problems ◽  
Clinical Records

Background and objectives:Autoimmune encephalitis (AE) cases post-SARS-CoV-2 infection have been reported, but the frequency is unknown. We aimed to determine the frequency and diagnostic features of COVID-19 related AE.Methods:Residual sera from 556 consecutive Mayo Clinic Rochester patients (laboratory cohort) who underwent autoimmune encephalopathy neural IgG evaluation were tested for total antibodies against the SARS-CoV-2 spike glycoprotein using an FDA-authorized chemiluminescence assay (October 2019-December 2020). Clinical records from patients with a positive SARS-CoV-2 antibody result and available research consent were reviewed. This laboratory cohort was cross-referenced with the Department of Neurology’s COVID-related consultative experience (encephalopathy cohort, n=31).Results:Eighteen of the laboratory cohort (3%) were SARS-CoV-2 antibody positive (April-December 2020). Diagnoses were: AE, 2; post-acute sequelae of SARS CoV-2 infection [PASC], 3; toxic-metabolic encephalopathy during COVID-19 pneumonia, 2; diverse non-COVID-19 relatable neurological diagnoses, 9; unavailable, 2. Five of the encephalopathy cohort had AE (16%, including the 2 laboratory cohort cases which overlapped) representing 0.05% of 10,384 patients diagnosed and cared for with any COVID-19 illness at Mayo Clinic Rochester in 2020. The 5 patients met definite (n=1), probable (n=1), or possible (n=3) AE diagnostic criteria; median symptom onset age was 61 years (range, 46-63), 3 were women. All 5 were neural IgG negative and 4 tested were SARS-CoV-2 PCR/IgG index negative in CSF. Phenotypes (and accompanying MRI and EEG findings) were diverse (delirium [n=5], seizures [n=2], rhombencephalitis [n=1], aphasia [n=1], and ataxia [n=1]). No ADEM cases were encountered. The 3 patients with possible AE had spontaneously resolving syndromes. One with definite limbic encephalitis was immune therapy responsive but had residual mood and memory problems. One patient with probable autoimmune rhombencephalitis died despite immune therapy. The remaining 26 encephalopathy cohort patients had toxic-metabolic diagnoses.Discussion:We encountered occasional cases of AE in our 2020 COVID-19 experience. Consistent with sporadic reports and small case series during the COVID-19 pandemic, and prior experience of postinfectious AE, our cases had diverse clinical presentations and were neural IgG and CSF viral particle negative. Application of diagnostic criteria assists in differentiation of AE from toxic-metabolic causes arising in the setting of systemic infection.

Criteria positive and criteria negative anaphylaxis, with a focus on undifferentiated somatoform idiopathic anaphylaxis: A review and case series

2020 ◽  
Vol 41 (6) ◽  
pp. 436-441 ◽  
Author(s):  
Daniel A. Rosloff ◽  
Kunal Patel ◽  
Paul J. Feustel ◽  
Jocelyn Celestin
Keyword(s):  
Diagnostic Criteria ◽  
Statistical Significance ◽  
Case Series ◽  
Poor Response ◽  
Response To Treatment ◽  
Fisher Exact Test ◽  
Subjective Symptoms ◽  
Significant Difference ◽  
Physical Findings ◽  
Idiopathic Anaphylaxis

Background: Undifferentiated somatoform (US) idiopathic anaphylaxis (IA) is considered a psychogenic disorder characterized by a lack of observable physical findings and poor response to treatment. Although failure to diagnose true anaphylaxis can have disastrous consequences, identification of US-IA is crucial to limit unnecessary expenses and use of health care resources. Objective: To better define the presentation and understand the potential relationship between US-IA and underlying psychiatric comorbidities. Methods: We retrospectively reviewed 110 visits by 107 patients to our institution for evaluation and management of anaphylaxis over a 1-year period. The patients were classified as having either criteria positive (CP) or criteria negative (CN) anaphylaxis based on whether they met Second National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network Symposium criteria for the clinical diagnosis of anaphylaxis. Patient characteristics, including objective and subjective signs and symptoms, and the presence of psychiatric diagnoses were collected and analyzed. Statistical significance was assessed by using the Fisher exact test. A literature review of US-IA and other psychogenic forms of anaphylaxis was performed. Results: Patients with CP anaphylaxis were more likely to present with hypotension, wheezing, urticaria, and vomiting than were patients with CN anaphylaxis. The patients with CN anaphylaxis were more likely to present with subjective symptoms of sensory throat tightness or swelling compared with patients with CP anaphylaxis. No significant difference was detected in the prevalence of psychiatric conditions between the two groups. Conclusion: Patients who met previously established diagnostic criteria for anaphylaxis were more likely to present with objective physical findings than those who did not meet criteria for true anaphylaxis. CN patients who presented for treatment of anaphylaxis were more likely to present with subjective symptoms. Formal diagnostic criteria should be used by clinicians when evaluating patients with suspected anaphylaxis.

scholarly journals FRI0419 LOW DOSE OF GLUCOCORTICOIDS FOR PAIN CONTROL IN THE ESTROGEN-DEPENDENT PRIMARY POLYARTICULAR OSTEOARTHRITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 807.2-808
Author(s):  
G. Puerta ◽  
M. Bautista ◽  
M. Urbano ◽  
F. Bonilla ◽  
C. Cañas
Keyword(s):  
Bone Scintigraphy ◽  
Pain Control ◽  
Controlled Trial ◽  
Case Series ◽  
Tender Joint Count ◽  
Hand Osteoarthritis ◽  
Average Dose ◽  
Tender Joint ◽  
Double Blind ◽  
Clinical Records

Background:Low doses of glucocorticoids (GCs) can be useful in the management of osteoarthritis when it is related to hypoestrogenic states (estrogen-dependent primary polyarticular osteoarthritis [EDPOA]), that usually can appear after the menopause. Deflazacort is a GC that has similar anti-inflammatory effects than other steroids, but with fewer side effects.Objectives:To describe the average dose of GCs that best controlled articular pain, based on tender joint count in patients with EDPOA.Methods:The diagnosis of EDPOA was made in postmenopausal patients with polyarticular compromised (six or more joints affected), morning stiffness less than 30 minutes, erythrocyte sedimentation rate less than 45mm/hour and imaging studies with changes related to osteoarthritis (radiography, magnetic resonance imaging or bone scintigraphy). Patients with autoimmune diseases such as rheumatoid arthritis, lupus or Sjögren syndrome were excluded.The clinical records of patients diagnosed with EDPOA and treated between January 2015 and June 2019 at the Valle del Lili foundation Hospital were reviewed. The patients treated with deflazacort GC were included. Pain was assessed by the treating rheumatologist using the visual analog scale (VAS, possible score 0-10). Tender joints were those with VAS> 5. The count of compromised joints was compared with inflammatory findings on bone scintigraphy (Figure 1).Figure 1.Comparison between number of joints with inflammatory findings on bone scintigraphy and number of swollen joints in physical evaluationThe number of tender joints was recorded at the start of treatment, which was a dose of 6 mg/day of deflazacort for two months. Subsequently, the dose was reduced depending on the improvement of pain (items: intensity of pain and number of tender joints) until achieving a stabilization along the time with an improvement of 75% of the items evaluated. The number of painful joints was recorded again two months after the stabilization on pain control was achieved.Quantitative variables were described with medians and interquartile ranges because the absence of normal distribution of the sample size. To assess the presence of a significant decrease on the number of tender joints the Wilcoxon range test was used, a value of p<0.001 was considered statistically significant. The data were analyzed with Stata v.15.Results:Twenty-eight patients with EDPOA were included, with a median of age of 50 years (IQR 44-51), 56 years (IQR 52-66) and 61 years (IQR 54-69) at the time of menopause, onset of symptoms and the diagnosis of EDPOA respectively. A median of 18 tender joints (IQR 10-27) was obtained from the physical examination of the records reviewed. The dose of deflazacort that achieved stabilization on the improvement of the pain along the time was 21mg/week (IQR 12-21); after 8 weeks of treatment the number of tender joints was 2 (IQR 1-4), which implies a reduction of 14 (IQR 8-20; p<0.0001) on the tender joint count (Figure 2).Figure 2.Number of tender joints before and after eight weeks of treatment achieving with a stable pain control in patients with EDPOA treated with deflazacort with a media dose of 3mgr/day.Conclusion:In this case series a media dose of deflazacort of 21mg per week (3mg/day) was useful to significantly reduce the number of tender joints in patients with EDPOA.References:[1]Roman-Blas JA, Castañeda S, Largo R,et al. Osteoarthritis associated with estrogen deficiency. Arthritis Research & Therapy 2009;11:241.[2]Cvoro A, Yuan C, Paruthiyil S,et al. Crosstalk between glucocorticoid and estrogen receptors occurs at a subset of proinflammatory genes. The Journal of Immunology 2011;186:4354-4360.[3]Féline K, Marion K, Annelies B,et al. Results of a 6-week treatment with 10 mg prednisolone in patients with hand osteoarthritis (HOPE): a double-blind, randomised, placebo-controlled trial. Lancet 2019;394:1993-2001.Disclosure of Interests:None declared

scholarly journals Hemophagocytic Lymphohistiocytosis in Adults (aHLH): Results from the German HLH Registry

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2523-2523 ◽  
Author(s):  
Sebastian Birndt ◽  
Thomas Schenk ◽  
Frank M. Brunkhorst ◽  
Georg Maschmeyer ◽  
Frank Rothmann ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Research Funding ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Compound Heterozygous ◽  
Cytokine Release ◽  
Laboratory Findings

Abstract Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome caused by excessive immune activation leading to a life-threatening pro-inflammatory cytokine storm. HLH is not an entity of its own, but a clinical syndrome triggered by various conditions like infections, malignancy or autoimmune disorders. HLH pathogenesis is complex and still not fully understood. Contributing factors include immunosuppression (chemotherapy, long-term immunosuppressive therapy), cytokine release from tumor cells, imbalance between infected and immune effector cells as well as genetic predisposition. Despite improved HLH-specific therapy (adapted components from the pediatric HLH-1994 protocol), prognosis is still poor among adult HLH patients. Due to the lack of data on adult HLH in Germany, a national multicenter registry (http://www.hlh-registry.org/) was initiated. Methods: Patients (pts) with proven or suspected HLH were registered by 35 institutions across Germany from August 2010 to July 2016. Both HLH-2004 diagnostic criteria and the HScore (www.saintantoine.aphp.fr/score/) were used to confirm HLH diagnosis. To characterize adult HLH patients, data referring to underlying disease, treatment, outcome, clinical manifestations and laboratory findings were recorded. Where available, patient samples were tested for mutations of the perforin gene PRF1 by standard Sanger sequencing. Results: A total of 125 pts (48 female) were enrolled in our registry, of whom 105 pts either fulfilled diagnostic criteria according to HLH-2004 diagnostic guidelines (n=96) or met at least 4 out of 8 criteria and reached HLH-probability of over 90 % in the HScore (n=9). Among these 105 pts, n=38 (36 %) were female and n=67 (64 %) male. Median age at diagnosis was 49 years (range 17 - 81). Trigger diseases were in line with the literature, with infections (n=34) and malignancy (n=40) being most frequent (Table 1). Patients show a wide spectrum of underlying conditions, i.e. allogeneic stem cell transplantation (alloSCT) or HLH-mimicking diseases due to cytokine release in response to blinatumomab therapy. Late onset hereditary HLH was found in 3 pts (XLP-1 and -2 respectively in EBV-coinfected pts, one pt with perforin mutation - see below). 22 of 105 patients (21 %) were tested for PRF1 mutations. A compound heterozygous PRF1 A91V/Q405X mutation was identified in one pt presenting with NK/T-cell lymphoma. Heterozygous PRF1 A91V mutations were found in 2 pts with B-cell lymphoma and HLH following alloSCT respectively, in one case with available buccal swab DNA to prove germline origin of the mutation. Table 2 summarizes clinical and laboratory findings in the cohort. A median ferritin value of 32,000 µg/L underlines the importance to evaluate pts with highly elevated ferritin with respect to potential HLH diagnosis. Apart from fever and splenomegaly, clinical presentation frequently comprised hepatomegaly, liver failure, hyperbilirubinemia, renal failure, lung involvement like ARDS, or bleeding. Treatment included steroids in the vast majority of pts (n=89), often combined with i.v. immunoglobulins (n=47) and etoposide (n=49). After a median follow up time of 164 days, 48/97 pts (49.5 %) were alive, 8 pts were lost to follow up. Survival analysis revealed median overall survival of 454 days (Figure 1a). Comparing malignancy-associated HLH and HLH after alloSCT with infection- and autoimmune-associated HLH or HLH due to unknown triggers, survival was significantly poorer in the malignancy/alloSCT group (Figure 1b). Conclusions: HLH trigger conditions in adult patients in Germany are in accordance with published case series. Outcome in adult HLH is still poor, with malignancy-associated and HLH after alloSCT showing the worst prognosis. Diagnostic vigilance and early treatment is a prerequisite for improving outcome of adult HLH. In particular, high ferritin values should raise suspicion of HLH. Disclosures Hochhaus: Pfizer: Honoraria, Research Funding; Novartis: Honoraria, Research Funding; BMS: Honoraria, Research Funding; ARIAD: Honoraria, Research Funding.

scholarly journals Autoimmune Encephalitis in Latin America: A Critical Review

2021 ◽  
Vol 11 ◽  
Author(s):  
Gabriel de Albuquerque Vasconcelos ◽  
Rodrigo Montenegro Barreira ◽  
Karmelita Emanuelle Nogueira Torres Antoniollo ◽  
Alina Maria Nuñez Pinheiro ◽  
Cíntia Fernandes Rodrigues Maia ◽  
...  
Keyword(s):  
Latin America ◽  
Latin American ◽  
World Literature ◽  
Case Reports ◽  
Case Series ◽  
Autoimmune Encephalitis ◽  
Developed Countries ◽  
Future Research ◽  
Epidemiologic Factors ◽  
Nmda Receptor Encephalitis

Autoimmune encephalitis is an increasingly recognized cause of encephalitis. The majority of case series report patients residing in developed countries in the northern hemisphere. The epidemiologic features of autoimmune encephalitis in Latin America are still unclear. The aim of the study was to perform a review of the clinical presentation of autoimmune encephalitis in Latin America and compare to world literature. References were identified by an in-depth literature search and selected on the basis of relevance to the topic and authors' judgment. We selected clinical studies and case reports published from 2007 to July, 2020 including patients from Latin American countries. Of the 379 patients included, the majority were cases of anti-NMDA receptor encephalitis (93.14%), followed by anti-VGKC-complex encephalitis (N = 17; 4.48%), anti-GAD encephalitis (N = 9; 2.37%), anti-AMPA receptor encephalitis (N = 1; 0.26%), anti-GABA receptor encephalitis (N = 1; 0. 26%), anti-mGluR5 encephalitis (N = 1; 0. 26%), and anti-mGluR1 encephalitis (N = 1; 0. 26%). Reported cases of Anti-NMDA encephalitis in Latin-America had a very slight female predominance, lower prevalence of associated tumors and a lower incidence of extreme delta brush on electroencephalogram. Autoimmune encephalitis is possibly underdiagnosed in underdeveloped countries. Its outcome after treatment, however, appears to be similarly favorable in Latin American patients as has been reported in developed countries based on available case reports and case series. Regional specificities in the manifestation of autoimmune encephalitis could be related to epidemiologic factors, such as the presence of different triggers and different genetic and immunologic background, that need to be studied by future research.

scholarly journals Case Report: Successful Management of a Refractory Plasmablastic Lymphoma Patient With Tislelizumab and Lenalidomide

2021 ◽  
Vol 12 ◽  
Author(s):  
Lili Cheng ◽  
Qi Song ◽  
Mengke Liu ◽  
Yan Wang ◽  
Hongmei Yi ◽  
...  
Keyword(s):  
Standard Treatment ◽  
Hematological Malignancy ◽  
Checkpoint Inhibitor ◽  
Immune Therapy ◽  
Case Series ◽  
The Elderly ◽  
Plasmablastic Lymphoma ◽  
Successful Management ◽  
Immunodeficiency Virus ◽  
Specific Standard

Plasmablastic lymphoma (PBL) is a rare and aggressive hematological malignancy. PBL commonly occurs in immune incompetent patients, such as those with human immunodeficiency virus (HIV), post-transplant status, or immunosenescence. Given its rarity, there is no specific standard treatment for PBL. However, small case series have shown that intensive chemotherapies combined with anti-myeloma agents such as bortezomib and lenalidomide were effective in treating PBL. Unfortunately, some fragile patients could not tolerate intensive chemotherapeutic regimens, especially the elderly patients. Here we presented a 76-year-old female PBL patient refractory to miniCHOP regimen combined with bortezomib but achieved complete remission when treated with tislelizumab combined with lenalidomide, indicating that immune therapy may be a potential treatment for PBL. To our knowledge, this is the first chemoresistant PBL patient that has been successfully treated with checkpoint inhibitor plus lenalidomide, thus providing new insight towards PBL management.

Autoimmune Antibody in Encephalopathic Patients: A Pilot Study

2021 ◽  
Vol 104 (6) ◽  
pp. 943-950
Keyword(s):  
False Positive ◽  
Laboratory Data ◽  
Autoimmune Encephalitis ◽  
Positive Test ◽  
Thyroid Antibodies ◽  
Acute Encephalopathy ◽  
True Negative ◽  
Metabolic Encephalopathy ◽  
False Positive Test ◽  
Significant Difference

Objective: To investigate the specificity of the antibodies related to autoimmune encephalitis and to identify possible associated factors with the false-positive result. Materials and Methods: The present study was a prospective observational study, conducted at the Ramathibodi Hospital between June and December 2019. All patients, who had acute to subacute encephalopathy from any causes, were recruited to the study. Their serum or cerebrospinal fluid (CSF) were taken to analyze for autoimmune encephalitis assays and anti-thyroid antibodies. The authors did not interfere with the primary physicians on any management of the patients. Clinical and laboratory data were systematically reviewed and collected from medical records. The clinical outcome was evaluated one month after the onset. Results: Fifty-one patients were recruited. Only one patient had autoimmune encephalitis related to anti-CV2/CRMP5 antibody. Seventeen out of the remaining fifty patients had positive tests for anti-thyroid antibodies of which five had Hashimoto’s thyroiditis and one of them did not have the document of thyroid status. Eleven remaining patients appeared to have false-positive test since their medical conditions were all clearly explained by other causes. Comparison of clinical and laboratory data between patients with false-positive test and patients with true negative test did not show any significant difference except the duration of the symptoms, which was significantly shorter in the false-positive group. Conclusion: False-positive anti-thyroid antibodies appear to be common in patients with acute encephalopathy. The occurrence of serum/CSF antibody in acute encephalopathy may be a true association, but it may not be the cause of encephalopathy. Therefore, the diagnosis of autoimmune encephalopathy based on anti-thyroid antibodies should be carefully made and excluded from all other possible causes. Keywords: Autoimmune encephalitis; Metabolic encephalopathy; Hashimoto’s encephalopathy; False positive

Diagnosis of lung cancer: a necropsy-based study of 128 cases (1997–2016)

2020 ◽  
pp. 030089162094966
Author(s):  
Pietro Gino Barbieri ◽  
Dario Mirabelli
Keyword(s):  
Computed Tomography ◽  
Lung Cancer ◽  
Case Series ◽  
The Elderly ◽  
Positive Finding ◽  
Chest Computed Tomography ◽  
Pleural Plaques ◽  
High Prevalence ◽  
Diagnosis Of Lung Cancer ◽  
Clinical Records

Background: The diagnosis of lung cancer (LC) may be difficult to make in the elderly. We report on the diagnostic elements available in life in an LC necropsy case series of asbestos-exposed workers and describe the frequency of non-neoplastic asbestos-related diseases as biological exposure indices. Methods: We reviewed pathologic and clinical records of an unselected series of autopsies (1997–2016) in patients with LC employed in the Monfalcone shipyards. We assessed the consistency with autopsy results of diagnoses based on, respectively, radiologic, cytologic, and histologic findings. Results: Data on 128 autopsy-confirmed LC cases were available; in life, 119 had been diagnosed as LC. Among these, 49 had histologic confirmation of diagnosis (17 with immunophenotyping); histology had been negative in 4. Cytology had been the main positive finding and the basis for diagnosis in 24 cases, but had been negative in 13. Chest computed tomography had been the basis for diagnosis in 45; in 18 cases, it had been negative. Nine patients had received a diagnosis different from LC, among whom 4 had been suspected to have malignant pleural mesothelioma by chest computed tomography. Pleural plaques were found in 124 and histologic asbestosis in 46 cases. Conclusions: Autopsies confirmed all LC diagnoses received in life, including 46 that would have been considered only possible LC based on clinical workup. The overall survival in this case series was poor. The high prevalence of pleural plaques and asbestosis suggest severity of asbestos exposures.

scholarly journals Clozapine prescribing in COVID-19 positive medical inpatients: a case series

2020 ◽  
Vol 10 ◽  
pp. 204512532095956
Author(s):  
Matthew Butler ◽  
Felicity Bano ◽  
Marilia Calcia ◽  
Isabel McMullen ◽  
Chun Chiang Sin Fai Lam ◽  
...  
Keyword(s):  
Respiratory Infections ◽  
Case Series ◽  
Systemic Infection ◽  
Published Data ◽  
Retrospective Case ◽  
Retrospective Case Series ◽  
Medical Inpatients ◽  
Medical Wards ◽  
Hospital Wards

There is both uncertainty regarding the safety of clozapine in COVID-19 patients owing to limited published data and a lack of consensus on continuing clozapine in patients with severe respiratory infections. COVID-19 is known to induce an acute immune response which can affect haematological parameters associated with clozapine monitoring, and systemic infection may reduce clozapine clearance. Clozapine, which has been associated with worse outcomes in some pneumonias, may in theory worsen outcomes in COVID-19. Despite these concerns, there are some data to indicate it is safe to continue clozapine in COVID-19 infection. In this retrospective case series, we describe our experiences of clozapine prescribing and disease progression of eight SARS-CoV-2 positive patients on medical wards in a major London teaching hospital. In four cases clozapine was stopped during the hospital admission. A COVID-19 pneumonia developed in four patients: three of these required intensive care unit admission for an average of 34 days. At the time of writing, three patients had died (two directly from COVID-19 pneumonia), two remained in general hospital wards, two were recovering in the community and one had been transferred to an inpatient psychiatric hospital. Follow-up length varied but in each case was not more than 104 days. Delirium was the most common adverse neuropsychiatric event, and in one case a relapse of psychosis occurred after cessation of clozapine. This retrospective case series illustrates the safe use of clozapine during COVID-19 infection. Our experiences suggest that consideration should be made to continuing clozapine even in those most unwell with COVID-19. We also identify areas which require larger scale hypothesis-testing research.

scholarly journals The association of frontal fibrosing alopecia with skin and hair care products: A survey-based case series of 56 patients seen at the Mayo Clinic

2020 ◽  
Author(s):  
Reese L. Imhof ◽  
Sydney C. Larkin ◽  
Hafsa M. Cantwell ◽  
Rochelle R. Torgerson ◽  
Stanislav N. Tolkachjov
Keyword(s):  
Mayo Clinic ◽  
Case Series ◽  
Hair Care
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