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Questions and Answers
What symptoms were HLA DRB1*10:01-positive patients more likely to experience?
What symptoms were HLA DRB1*10:01-positive patients more likely to experience?
Which phenotype does NOT associate with HLA DRB1*11:01 in patients with CASPR2 antibodies?
Which phenotype does NOT associate with HLA DRB1*11:01 in patients with CASPR2 antibodies?
What was the first step in confirming the pathogenicity of antibodies in patients?
What was the first step in confirming the pathogenicity of antibodies in patients?
What type of neurons were used for functional assessment of antibodies in vitro?
What type of neurons were used for functional assessment of antibodies in vitro?
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How old were the cultured neurons typically during the experimental assessments?
How old were the cultured neurons typically during the experimental assessments?
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What method was used to introduce patients’ antibodies to the cultured neurons?
What method was used to introduce patients’ antibodies to the cultured neurons?
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Which of the following is a condition associated with HLA DRB1*10:01?
Which of the following is a condition associated with HLA DRB1*10:01?
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What type of studies were used to assess functional alterations caused by antibodies?
What type of studies were used to assess functional alterations caused by antibodies?
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Which immune-related neurologic disease is NOT commonly induced by immune checkpoint inhibitors (ICIs)?
Which immune-related neurologic disease is NOT commonly induced by immune checkpoint inhibitors (ICIs)?
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In cases of autoimmune encephalitis triggered by ICIs, which type of antibodies is most commonly found?
In cases of autoimmune encephalitis triggered by ICIs, which type of antibodies is most commonly found?
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Which bacterium is associated with triggering autoimmune responses leading to Guillain–Barré syndrome?
Which bacterium is associated with triggering autoimmune responses leading to Guillain–Barré syndrome?
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What is the reported percentage of patients with herpes simplex encephalitis (HSE) found to have IgG NMDAR antibodies?
What is the reported percentage of patients with herpes simplex encephalitis (HSE) found to have IgG NMDAR antibodies?
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What is suggested to trigger neuronal autoimmunity in patients with autoimmune encephalitis post-HSE?
What is suggested to trigger neuronal autoimmunity in patients with autoimmune encephalitis post-HSE?
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What term describes the phenomenon where infections lead to autoimmune reactions by resembling nervous system molecules?
What term describes the phenomenon where infections lead to autoimmune reactions by resembling nervous system molecules?
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Where is it believed that the activation process of neuronal autoimmunity primarily occurs?
Where is it believed that the activation process of neuronal autoimmunity primarily occurs?
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Which autoimmune condition is typically linked to infections by group A streptococcus?
Which autoimmune condition is typically linked to infections by group A streptococcus?
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What role does CD4+ T cells play in the differentiation of naive B cells?
What role does CD4+ T cells play in the differentiation of naive B cells?
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What is unclear regarding antibodies present in the brain of patients with autoimmune encephalitis?
What is unclear regarding antibodies present in the brain of patients with autoimmune encephalitis?
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Which of the following commonly occurs in patients with acute disseminated encephalomyelitis (ADEM)?
Which of the following commonly occurs in patients with acute disseminated encephalomyelitis (ADEM)?
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Which neural antibodies were identified in a minority of encephalitis cases triggered by ICIs?
Which neural antibodies were identified in a minority of encephalitis cases triggered by ICIs?
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In studies of patients post-HSE, which antibodies are first detected in the CSF?
In studies of patients post-HSE, which antibodies are first detected in the CSF?
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What does the process of antibody response in autoimmune encephalitis typically involve after initial synthesis?
What does the process of antibody response in autoimmune encephalitis typically involve after initial synthesis?
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What effect did the intranasal administration of HSV-1 have in the mouse model?
What effect did the intranasal administration of HSV-1 have in the mouse model?
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Which aspect of the blood-brain barrier may influence the presence of antibodies in the brain?
Which aspect of the blood-brain barrier may influence the presence of antibodies in the brain?
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What is the role of immune checkpoint inhibitors targeting CTLA-4 and PD-1/PD-L1 molecules?
What is the role of immune checkpoint inhibitors targeting CTLA-4 and PD-1/PD-L1 molecules?
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What can occur when immune checkpoint inhibitors are directed against antigens in the central or peripheral nervous system?
What can occur when immune checkpoint inhibitors are directed against antigens in the central or peripheral nervous system?
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Why do tumors in patients with paraneoplastic syndromes appear to have a better outcome?
Why do tumors in patients with paraneoplastic syndromes appear to have a better outcome?
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What is a potential negative consequence of immune checkpoint inhibitors?
What is a potential negative consequence of immune checkpoint inhibitors?
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Which type of cancer has been noted to have subclinical immune responses against onconeural antigens?
Which type of cancer has been noted to have subclinical immune responses against onconeural antigens?
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What has been associated with the introduction of immune checkpoint inhibitors in clinical practice?
What has been associated with the introduction of immune checkpoint inhibitors in clinical practice?
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What is the common clinical scenario for autoimmune encephalitis (AE) following herpes simplex encephalitis (HSE)?
What is the common clinical scenario for autoimmune encephalitis (AE) following herpes simplex encephalitis (HSE)?
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What percentage of patients with herpes simplex encephalitis developed autoimmune encephalitis in the study presented?
What percentage of patients with herpes simplex encephalitis developed autoimmune encephalitis in the study presented?
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What is suggested regarding the use of immune checkpoint inhibitors in certain cancer patients?
What is suggested regarding the use of immune checkpoint inhibitors in certain cancer patients?
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Which type of encephalitis is noted as increasing with the use of immune checkpoint inhibitors?
Which type of encephalitis is noted as increasing with the use of immune checkpoint inhibitors?
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When were neuronal antibodies predominantly detected in patients after the discontinuation of acyclovir?
When were neuronal antibodies predominantly detected in patients after the discontinuation of acyclovir?
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How does the outcome of AE post-HSE compare to classical anti-NMDAR encephalitis?
How does the outcome of AE post-HSE compare to classical anti-NMDAR encephalitis?
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Which type of infection has NOT been reported as a precursor to anti-neural immune responses?
Which type of infection has NOT been reported as a precursor to anti-neural immune responses?
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What characterizes the clinical course of autoimmune encephalitis after Japanese B encephalitis?
What characterizes the clinical course of autoimmune encephalitis after Japanese B encephalitis?
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What is the median time frame for the development of AE after the presentation of HSE?
What is the median time frame for the development of AE after the presentation of HSE?
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What is the significance of detecting neuronal antibodies in the cerebrospinal fluid (CSF) of patients with AE?
What is the significance of detecting neuronal antibodies in the cerebrospinal fluid (CSF) of patients with AE?
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What is a primary function of IgG4 autoantibodies?
What is a primary function of IgG4 autoantibodies?
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Which autoimmune disease is associated with IgG4 antibodies?
Which autoimmune disease is associated with IgG4 antibodies?
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Which of the following is true regarding IgG4-associated autoimmune diseases?
Which of the following is true regarding IgG4-associated autoimmune diseases?
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What kind of proteins do IgG4-associated autoimmune diseases primarily target?
What kind of proteins do IgG4-associated autoimmune diseases primarily target?
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Which antibodies have shown a strong HLA association in antibody-mediated encephalitis?
Which antibodies have shown a strong HLA association in antibody-mediated encephalitis?
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What is suggested to increase susceptibility to IgG4-mediated autoimmunity?
What is suggested to increase susceptibility to IgG4-mediated autoimmunity?
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Which autoimmune condition is primarily targeted by IgG4 antibodies as stated in the content?
Which autoimmune condition is primarily targeted by IgG4 antibodies as stated in the content?
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Which property is NOT associated with IgG4 autoantibodies?
Which property is NOT associated with IgG4 autoantibodies?
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Study Notes
Antibody-Mediated Encephalitis
- Some central nervous system (CNS) diseases are mediated by neuronal antibodies.
- Paraneoplastic syndromes of the CNS are remote effects of cancer often associated with immune responses against intracellular neuronal proteins.
- Myasthenic syndromes (myasthenia gravis or Lambert-Eaton syndrome) can access their cognate cell surface antigens (AChR and VGCC), altering their structure and function via crosslinking and internalization.
- There are 19 CNS disorders where antibodies target neuronal cell surface proteins, including receptors, and proteins for processes like signal integration, clustering, and synaptogenesis.
- Antibodies contribute to disease by altering neuronal cell surface proteins' structure and function leading to alterations in synaptic function.
Pathogenesis and Disease Mechanisms
- Historical overview of the concept that some CNS diseases are mediated by neuronal antibodies is described in chapter 1.
- The study of paraneoplastic syndromes and myasthenic syndromes contributed to the understanding of how neuronal antibodies can alter synaptic function.
- Ectopic expression of neuronal proteins by tumors can trigger an enhanced immune response.
- Cytotoxic T-cell responses against the nervous system can also be involved in the neurological syndrome.
- Mechanisms of antibody-mediated disease vary depending on the target antigens (intracellular or on cell surface).
Key Factors Influencing the Development of Antibodies
- Systemic tumors, viral encephalitis (like herpes simplex and Japanese B), and HLA susceptibility are key factors influencing antibody development.
- The exact cause of the disease is unknown in many patients, but there's evidence linking tumors and viral infections to some antibody-mediated forms of encephalitis.
Summary of Clinical Findings
- Clinical phenotypes of autoimmune encephalitis show similarities to conditions where the same antigens are altered genetically or pharmacologically.
- Treatments removing antibodies or antibody-producing cells can reverse symptoms.
- Neuropathological findings (e.g., presence of B or plasma cells, absence of neuronophagic clusters) differ in some autoimmune encephalitis forms compared to those mediated by cytotoxic T cells.
- Antibodies' access to surface epitopes on neuronal cells may play a role.
Immunoglobulin (IgG) Subclasses
- IgG1 and IgG3 and sometimes IgG2 are main IgG subclasses associated with autoimmune encephalitis.
- IgG4 antibodies may be associated with different mechanisms compared to IgG1 and IgG3.
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Description
Test your knowledge on autoimmune encephalitis and the role of specific HLA antibodies. This quiz covers various symptoms, associated conditions, and methods used in research. Dive deep into the complexities of immune-related neurologic diseases and their triggers.
