scholarly journals Apheresis in Autoimmune Encephalitis and Autoimmune Dementia

2020 ◽  
Vol 9 (9) ◽  
pp. 2683 ◽  
Author(s):  
Rosa Rössling ◽  
Harald Prüss
Keyword(s):  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Synaptic Proteins ◽  
First Line ◽  
First Line Therapy ◽  
Neuronal Autoantibodies ◽  
Neurodegenerative Dementia ◽  
Pathogenic Antibodies ◽  
Clinical Conditions

Autoimmune encephalitis (AE) is a rapidly progressive inflammatory neurological disease. Underlying autoantibodies can bind to neuronal surfaces and synaptic proteins resulting in psychiatric symptoms, focal neurological signs, autonomic dysfunction and cognitive decline. Early and effective treatment is mandatory to reduce clinical symptoms and to achieve remission. Therapeutic apheresis, involving both plasma exchange (PE) and immunoadsorption (IA), can rapidly remove pathogenic antibodies from the circulation, thus representing an important first-line treatment in AE patients. We here review the most relevant studies regarding therapeutic apheresis in AE, summarizing the outcome for patients and the expanding clinical spectrum of treatment-responsive clinical conditions. For example, patients with slowly progressing cognitive impairment suggesting a neurodegenerative dementia can have underlying autoantibodies and improve with therapeutic apheresis. Findings are encouraging and have led to the first ongoing clinical studies assessing the therapeutic effect of IA in patients with anti-neuronal autoantibodies and the clinical presentation of dementia. Therapeutic apheresis is an established and well tolerated option for first-line therapy in AE and, potentially, other antibody-mediated central nervous system diseases.

scholarly journals Th17 lymphocytes drive vascular and neuronal deficits in a mouse model of postinfectious autoimmune encephalitis

2020 ◽  
Vol 117 (12) ◽  
pp. 6708-6716 ◽  
Author(s):  
Maryann P. Platt ◽  
Kevin A. Bolding ◽  
Charlotte R. Wayne ◽  
Sarah Chaudhry ◽  
Tyler Cutforth ◽  
...  
Keyword(s):  
Microglial Activation ◽  
Neural Circuit ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Abrupt Onset ◽  
Synaptic Proteins ◽  
Excitatory Synapses ◽  
Odor Processing ◽  
Th17 Lymphocytes

Antibodies against neuronal receptors and synaptic proteins are associated with a group of ill-defined central nervous system (CNS) autoimmune diseases termed autoimmune encephalitides (AE), which are characterized by abrupt onset of seizures and/or movement and psychiatric symptoms. Basal ganglia encephalitis (BGE), representing a subset of AE syndromes, is triggered in children by repeated group AStreptococcus(GAS) infections that lead to neuropsychiatric symptoms. We have previously shown that multiple GAS infections of mice induce migration of Th17 lymphocytes from the nose into the brain, causing blood–brain barrier (BBB) breakdown, extravasation of autoantibodies into the CNS, and loss of excitatory synapses within the olfactory bulb (OB). Whether these pathologies induce functional olfactory deficits, and the mechanistic role of Th17 lymphocytes, is unknown. Here, we demonstrate that, whereas loss of excitatory synapses in the OB is transient after multiple GAS infections, functional deficits in odor processing persist. Moreover, mice lacking Th17 lymphocytes have reduced BBB leakage, microglial activation, and antibody infiltration into the CNS, and have their olfactory function partially restored. Th17 lymphocytes are therefore critical for selective CNS entry of autoantibodies, microglial activation, and neural circuit impairment during postinfectious BGE.

Prevalence of serum anti-neuronal autoantibodies in patients admitted to acute psychiatric care

2016 ◽  
Vol 46 (16) ◽  
pp. 3303-3313 ◽  
Author(s):  
M. Schou ◽  
S. G. Sæther ◽  
K. Borowski ◽  
B. Teegen ◽  
D. Kondziella ◽  
...  
Keyword(s):  
Psychiatric Care ◽  
Psychiatric Symptoms ◽  
Psychiatric Patients ◽  
Autoimmune Encephalitis ◽  
International Classification Of Diseases ◽  
Acid Decarboxylase ◽  
Neuronal Autoantibodies ◽  
Acute Psychiatric Care ◽  
Receptor Antibodies ◽  
Gad65 Antibodies

BackgroundAutoimmune encephalitis associated with anti-neuronal antibodies may be challenging to distinguish from primary psychiatric disorders. The significance of anti-neuronal antibodies in psychiatric patients without clear evidence of autoimmune encephalitis is unknown. We investigated the serum prevalence of six anti-neuronal autoantibodies in a cohort of unselected patients admitted to acute psychiatric care.MethodSerum was drawn from 925 patients admitted to acute psychiatric in-patient care. Psychiatric diagnoses were set according to International Classification of Diseases (ICD)-10 criteria. Antibody analysis was performed with an indirect immunofluorescence test for N-methyl d-aspartate receptor (NMDAR) antibodies and five other anti-neuronal autoantibodies of the immunoglobulin (Ig) classes IgA, IgG and IgM isotype.ResultsAnti-neuronal autoantibodies were found in 11.6% of patients: NMDAR antibodies in 7.6%, contactin-associated protein-like 2 (CASPR2) antibodies in 2.5%, glutamic acid decarboxylase-65 (GAD65) antibodies in 1.9%, and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antibodies in 0.1%. Leucine-rich glioma-inactivated protein-1 (LGI1) and γ-aminobutyric acid B (GABAB) receptor antibodies were not detected. NMDAR antibodies of class IgG were present in five patients only (0.5%). NMDAR antibodies of all Ig classes were equally prevalent in patients with and without psychosis. There were no significant differences in antibody prevalence in the different diagnostic categories, except for a higher odds ratio of being NMDAR antibody positive for patients without a specific psychiatric diagnosis.ConclusionsNMDAR IgG autoantibodies, which are known to be strongly associated with anti-NMDAR encephalitis, were rarely found. CASPR2 and GAD65 antibodies were more frequently encountered in the present study than previously reported. Further research on the clinical significance of anti-neuronal autoantibodies in patients with acute psychiatric symptoms is needed.

Chronic mandible inflammation as the first symptom of chronic recurrent multifocal osteomyelitis (CRMO) – a case report

2018 ◽  
Vol 23 (2) ◽  
Author(s):  
Ewa Krasuska-Sławińska ◽  
Paulina Piekarska ◽  
Piotr Gietka ◽  
Anna Wieteska-Klimczak ◽  
Mirela Wadecka ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Chronic Recurrent Multifocal Osteomyelitis ◽  
Unknown Aetiology ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Soft Tissue Swelling ◽  
Multifocal Osteomyelitis ◽  
Inflammatory Drugs ◽  
Mild Fever

Chronic recurrent multifocal osteitis (CRMO) is a rare disease of an unknown aetiology, occurring mainly in children aged 4-14 years. It is characterised by recurring episodes of osteitis, with no detectable cause, lasting from several months up to a few years. It usually affects the metaphysis of long bones. Primary lesions in the form of isolated focuses rarely occur in the mandible. The clinical symptoms of CRMO include ostealgia, soft tissue swelling (oedema), skin reddening, and mild fever. The diagnosis is difficult. It involves numerous laboratory and radiological investigations. In order to exclude infectious and neoplastic aetiology, it is advisable to perform a tissue biopsy. The disease is long-lasting with exacerbations and remissions. The prognosis is uncertain. Non-steroidal anti-inflammatory drugs and empirical antibiotic therapy are a recommended first-line therapy; if no improvement is observed, corticosteroids should be used. The analysed case concerns a 10-year-old boy with mandible inflammation as the first symptom of chronic recurrent multifocal osteitis (CRMO). Mandibular lesions may be the first symptom of chronic recurrent multifocal osteitis. The non-specific onset and variable clinical picture delay the diagnosis. Early diagnosis enables early treatment, which prevents complications.

Az autoimmun encephalitisek laboratóriumi vizsgálati lehetőségei

2018 ◽  
Vol 159 (3) ◽  
pp. 107-112
Author(s):  
Katalin Böröcz ◽  
Zsófia Hayden ◽  
Viktória Mészáros ◽  
Zsuzsanna Csizmadia ◽  
Kornélia Farkas ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Early Stage ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Immunosuppressive Drugs ◽  
Synaptic Proteins ◽  
Receptor Proteins ◽  
Surface Receptors ◽  
The Past ◽  
Paraneoplastic Limbic Encephalitis

Abstract: Introduction: The role of autoimmune responses against central nervous system (CNS) antigens in encephalitis presenting with non-classified neurologic or psychiatric symptoms has been appreciated in the past decade. Paraneoplastic limbic encephalitis has a poor prognosis and is most commonly associated with lung, ovarium, and testicular neoplasms, leading to immune reactions against intracellular antigens (anti-Hu/ANNA1, anti-Ri/ANNA2, anti-CV2/CRMP5 and anti-Ma2/Ta). In contrast, the recently described autoimmune encephalitis subtypes present with a broad spectrum of symptoms, respond to autoimmune therapies well and usually associate with autoantibodies against neuronal cell surface receptors (NMDAR, GABABR, AMPAR) or synaptic proteins (LGI1, CASPR2). Aim: Our aim is to bring to awareness the increasing number of autoimmune encephalitis patients requiring neurologic, psychiatric and intensive care and to emphasize the significance of detecting various autoantibodies in diagnosing patients. Method: In the past 6 years, our laboratory received 836 autoimmune encephalitis diagnostic test requests from a total of 717 patients. Serum and cerebrospinal fluid (CSF) samples were analysed with indirect immunofluorescence using a BIOCHIP consisting of cell lines transfected with 6 different receptor proteins. Results: IgG autoantibodies against receptor proteins were present in 7.5% of patients. The frequency of positive samples was the following: NMDAR > LGI1 > GABABR > CASPR2. Conclusion: Detecting autoantibodies facilitates the diagnosis of autoimmune encephalitis in an early stage. Patients diagnosed early can be effectively treated with plasmapheresis and immunosuppressive drugs. The efficiency of therapies can be monitored by autoantibody detection. Therefore, the diagnostic immune laboratory plays an important role in proper diagnosis and in the prevention of rapidly progressing symptoms. Orv Hetil. 2018; 159(3): 107–112.

scholarly journals Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management

2021 ◽  
pp. jnnp-2020-325300
Author(s):  
Hesham Abboud ◽  
John C Probasco ◽  
Sarosh Irani ◽  
Beau Ances ◽  
David R Benavides ◽  
...  
Keyword(s):  
Best Practice ◽  
Autoimmune Encephalitis ◽  
Second Line ◽  
First Line ◽  
Paraneoplastic Encephalitis ◽  
Electronic Survey ◽  
Practice Recommendations ◽  
First Line Therapy ◽  
Survey Results ◽  
Therapeutic Recommendations

The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. Half the responders indicated they would add a second-line agent only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in all patients. As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.

113 Rituximab and maintenance mycophenolate mofetil for treatment of refractory ANTI-N-METHYL-D-ASPARTATE-receptor (NMDAR) encephalitis

2018 ◽  
Vol 89 (6) ◽  
pp. A44.3-A45 ◽  
Author(s):  
Leon S Edwards ◽  
Michael H Barnett ◽  
Matthew C Kiernan
Keyword(s):  
Mycophenolate Mofetil ◽  
B Cells ◽  
Psychiatric Symptoms ◽  
Whole Body ◽  
Ovarian Teratoma ◽  
Second Line ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Nmdar Encephalitis

IntroductionNMDAR encephalitis is an autoimmune condition with antibodies to the NR1 subunit of the NMDAR. It has a variable clinical presentation with behavioural and psychiatric symptoms. There is frequently a relapsing course. A known association exists with tumours, particularly ovarian teratoma. Cases which are not associated with tumour often fail first line therapy. As such, therapy for this disease can be challenging and involves a spectrum of immunotherapy. Rituximab is widely considered to be second line treatment for this illness. We present two cases of refractory anti-NMDAR encephalitis.CasesBoth patients were confirmed antibody positive in the serum and cerebral spinal fluid. There was no tumour identified on whole body positron emission tomography and transvaginal pelvic ultrasound. Each patient was initially treated with first line therapy of intravenous immunoglobulin, plasmapheresis and methylprednisolone with limited symptomatic improvement. Second line therapy of two doses of rituximab was administered intravenously two weeks apart at a dose of 1000 mg. Mycophenolate mofetil was used at a dose of 1000 mg twice daily to minimise B-cell reconstitution. Response to treatment was monitored with measurement of CD 19+ Pan B cells in peripheral blood and clinical assessment by the consultant liaison psychiatry service. Both patients demonstrated significant response with undetectable CD 19+ Pan B cells on serial testing and sustained clinical improvement in initial behavioural symptoms.ConclusionIn patients with anti-NMDAR encephalitis, rituximab combined with maintenance mycophenolate mofetil may be an effective treatment.

scholarly journals Clinical Characteristics and Outcome of Neuronal Surface Antibody-Mediated Autoimmune Encephalitis Patients in a National Cohort

2021 ◽  
Vol 12 ◽  
Author(s):  
Zsófia Hayden ◽  
Beáta Bóné ◽  
Gergely Orsi ◽  
Monika Szots ◽  
Ferenc Nagy ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Brain Mri ◽  
Disease Onset ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Test Results ◽  
Nmdar Encephalitis

Background: In our previous single-center study of autoimmune encephalitis (AE) related autoantibody test results we found positivity in 60 patients out of 1,034 with suspected AE from 2012 through 2018 as part of a Hungarian nationwide program. In our current multicenter retrospective study, we analyzed the clinical characteristics and outcome of AE patients with positive neuronal cell surface autoantibody test results.Methods: A standard online questionnaire was used to collect demographic and clinical characteristics, laboratory and imaging data, therapy and prognosis of 30 definitive AE patients in four major clinical centers of the region.Results: In our study, 19 patients were positive for anti-NMDAR (63%), 6 patients (20%) for anti-LGI1, 3 patients for anti-GABABR (10%) and 3 patients for anti-Caspr2 (10%) autoantibodies. Most common prodromal symptoms were fever or flu-like symptoms (10/30, 33%). Main clinical features included psychiatric symptoms (83%), epileptic seizures (73%) and memory loss (50%). 19 patients (63%) presented with signs of central nervous system (CNS) inflammation, which occurred more frequently in elder individuals (p = 0.024), although no significant differences were observed in sex, tumor association, time to diagnosis, prognosis and immunotherapy compared to AE patients without CNS inflammatory markers. Anti-NMDAR encephalitis patients were in more severe condition at the disease onset (p = 0.028), although no significant correlation between mRS score, age, sex and immunotherapy was found. 27% of patients (n = 8) with associated tumors had worse outcome (p = 0.045) than patients without tumor. In most cases, immunotherapy led to clinical improvement of AE patients (80%) who achieved a good outcome (mRS ≤ 2; median follow-up 33 months).Conclusion: Our study confirms previous publications describing characteristics of AE patients, however, differences were observed in anti-NMDAR encephalitis that showed no association with ovarian teratoma and occurred more frequently among young males. One-third of AE patients lacked signs of inflammation in both CSF and brain MRI, which emphasizes the importance of clinical symptoms and autoantibody testing in diagnostic workflow for early introduction of immunotherapy, which can lead to favorable outcome in AE patients.

scholarly journals EEG Contribution to the Diagnosis of Antibody-Negative Autoimmune Encephalitis: A Case Report

2021 ◽  
pp. 739-743
Author(s):  
Mayusa Mito ◽  
Kotaro Sakurai ◽  
Yuichi Nakamura ◽  
Azusa Nagai ◽  
Sho Seo ◽  
...  
Keyword(s):  
Movement Disorders ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Brain Diseases ◽  
Test Results ◽  
Temporal Area ◽  
Antiepileptic Medications ◽  
The Right

Autoimmune encephalitis (AE) is a group of inflammatory brain diseases that are characterized by prominent neuropsychiatric symptoms. Early therapeutic intervention is important for AE. Therefore, without waiting for autoantibody test results, clinicians must consider the possibility of AE based solely on clinical symptoms and conventional test results. The case described herein is of antibody-negative encephalitis with abnormalities shown only by EEG, which contributed to the diagnosis and treatment. The patient, a 20-year-old woman, showed autonomic seizures in addition to movement disorders, psychiatric symptoms, and cognitive dysfunction, which worsened subacutely. Her seizures and movement disorders were not responsive to antiepileptic medications. Results obtained from MRI and cerebrospinal fluid (CSF) were normal; EEG findings showed repeated spikes in the right temporal area, with changes over time. Based on the clinical course and EEG, along with administered immunotherapy, which resolved seizures, movement disorders, and psychiatric symptoms, we suspected AE. For diagnosis of AE and for evaluating treatment responsiveness, EEG was useful. Results indicate that EEG can assist clinicians even with AE cases for which MRI and CSF findings are normal.

1904: Outcomes of Large Renal Calculi (>2-Cm) Treated with ESWL as First Line Therapy

2004 ◽  
Vol 171 (4S) ◽  
pp. 503-503
Author(s):  
Richard Vanlangendock ◽  
Ramakrishna Venkatesh ◽  
Jamil Rehman ◽  
Chandra P. Sundaram ◽  
Jaime Landman
Keyword(s):  
Renal Calculi ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy
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