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What critical observation established a pathogenic link between limbic encephalitis and an autoimmune response?
What critical observation established a pathogenic link between limbic encephalitis and an autoimmune response?
Among patients with SCLC and limbic encephalitis, those with Hu (ANNA1) antibodies tend to have what outcome?
Among patients with SCLC and limbic encephalitis, those with Hu (ANNA1) antibodies tend to have what outcome?
What type of limbic encephalitis was characterized by Vincent and colleagues in 2001?
What type of limbic encephalitis was characterized by Vincent and colleagues in 2001?
Which protein was identified as the antigen recognized by VGKC antibodies?
Which protein was identified as the antigen recognized by VGKC antibodies?
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What neurological condition is often associated with limbic encephalitis and known to involve the nervous system more broadly?
What neurological condition is often associated with limbic encephalitis and known to involve the nervous system more broadly?
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What do patients with limbic encephalitis, previously considered antibody-negative, actually have?
What do patients with limbic encephalitis, previously considered antibody-negative, actually have?
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How is the disorder characterized by antibodies against voltage-gated potassium channels (VGKCs) primarily viewed regarding neuronal damage?
How is the disorder characterized by antibodies against voltage-gated potassium channels (VGKCs) primarily viewed regarding neuronal damage?
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The study regarding limbic encephalitis and associated proteins was significant for developing which concept?
The study regarding limbic encephalitis and associated proteins was significant for developing which concept?
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What percentage of patients with limbic encephalitis were found to be non-paraneoplastic according to recent studies?
What percentage of patients with limbic encephalitis were found to be non-paraneoplastic according to recent studies?
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Which antibodies are primarily associated with limbic encephalitis based on recent research?
Which antibodies are primarily associated with limbic encephalitis based on recent research?
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Who coined the term 'limbic encephalitis' and contributed significantly to its clinical characterization?
Who coined the term 'limbic encephalitis' and contributed significantly to its clinical characterization?
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Which structures are included in the 'limbic lobe' characterized by neuronal loss in limbic encephalitis?
Which structures are included in the 'limbic lobe' characterized by neuronal loss in limbic encephalitis?
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What clinical symptoms were observed in the three male patients published by Corsellis and colleagues in 1960?
What clinical symptoms were observed in the three male patients published by Corsellis and colleagues in 1960?
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What significant transformation did Corsellis achieve at Runwell hospital?
What significant transformation did Corsellis achieve at Runwell hospital?
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What was found in the neuropathological examination of limbic encephalitis patients?
What was found in the neuropathological examination of limbic encephalitis patients?
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What did Corsellis suggest about the link between limbic encephalitis and cancer?
What did Corsellis suggest about the link between limbic encephalitis and cancer?
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What is the typical MRI finding in herpes simplex encephalitis?
What is the typical MRI finding in herpes simplex encephalitis?
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How does glioma typically present in terms of MRI features?
How does glioma typically present in terms of MRI features?
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Which feature is almost always associated with herpes simplex encephalitis?
Which feature is almost always associated with herpes simplex encephalitis?
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What distinguishes HHV-6 encephalitis imaging from herpes simplex encephalitis?
What distinguishes HHV-6 encephalitis imaging from herpes simplex encephalitis?
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How common are bilateral lesions in the context of HHV-6 encephalitis?
How common are bilateral lesions in the context of HHV-6 encephalitis?
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In initial evaluation, what MRI finding can be expected for a patient with glioma?
In initial evaluation, what MRI finding can be expected for a patient with glioma?
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Which of the following statements about CSF analysis in herpes simplex encephalitis is true?
Which of the following statements about CSF analysis in herpes simplex encephalitis is true?
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What is the most common demographic affected by glioma?
What is the most common demographic affected by glioma?
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What is a primary function of neuronal antibodies in treatment estimation?
What is a primary function of neuronal antibodies in treatment estimation?
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Which type of antibodies indicates a poor response to treatment?
Which type of antibodies indicates a poor response to treatment?
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What are Hu (ANNA1) antibodies most commonly associated with?
What are Hu (ANNA1) antibodies most commonly associated with?
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In which area of the nervous system is inflammation primarily seen in patients with Hu (ANNA1) antibodies?
In which area of the nervous system is inflammation primarily seen in patients with Hu (ANNA1) antibodies?
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Which entity is characterized by multiple areas of inflammatory involvement in the nervous system?
Which entity is characterized by multiple areas of inflammatory involvement in the nervous system?
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What symptom pattern is suggested in 10% of patients with Hu (ANNA1) antibodies developing limbic encephalitis?
What symptom pattern is suggested in 10% of patients with Hu (ANNA1) antibodies developing limbic encephalitis?
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Which antibodies are less frequently associated with paraneoplastic encephalomyelitis other than Hu (ANNA1)?
Which antibodies are less frequently associated with paraneoplastic encephalomyelitis other than Hu (ANNA1)?
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What is the relationship between intracellular antibodies and neuronal damage?
What is the relationship between intracellular antibodies and neuronal damage?
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Which infectious disorder is NOT mentioned as a potential mimic of limbic encephalitis?
Which infectious disorder is NOT mentioned as a potential mimic of limbic encephalitis?
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What must be ruled out before attributing limbic encephalitis to systemic autoimmune diseases?
What must be ruled out before attributing limbic encephalitis to systemic autoimmune diseases?
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Which systemic autoimmune disease is NOT listed as potentially associated with limbic encephalitis?
Which systemic autoimmune disease is NOT listed as potentially associated with limbic encephalitis?
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Among the following, which statement accurately reflects the current understanding of α-enolase antibodies?
Among the following, which statement accurately reflects the current understanding of α-enolase antibodies?
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What is a common clinical presentation for patients with neurosyphilis?
What is a common clinical presentation for patients with neurosyphilis?
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Which of the following conditions is indicated as a manifestation of Hashimoto encephalopathy?
Which of the following conditions is indicated as a manifestation of Hashimoto encephalopathy?
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In cases of limbic encephalitis, what is the significance of comprehensive analysis of neuronal antibodies?
In cases of limbic encephalitis, what is the significance of comprehensive analysis of neuronal antibodies?
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What distinctive feature is associated with the differential diagnosis of limbic encephalitis?
What distinctive feature is associated with the differential diagnosis of limbic encephalitis?
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What is associated with worse prognosis in patients with autoimmune encephalitis?
What is associated with worse prognosis in patients with autoimmune encephalitis?
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Which antibodies were found in the majority of patients with limbic encephalitis, SCLC, and GABAbR antibodies?
Which antibodies were found in the majority of patients with limbic encephalitis, SCLC, and GABAbR antibodies?
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What is a common presentation in patients with AMPAR antibodies associated with autoimmune encephalitis?
What is a common presentation in patients with AMPAR antibodies associated with autoimmune encephalitis?
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Which factor is associated with poor response to therapy in autoimmune encephalitis?
Which factor is associated with poor response to therapy in autoimmune encephalitis?
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What characterizes the differences between limbic encephalitis with GABAbR antibodies and AMPAR antibodies?
What characterizes the differences between limbic encephalitis with GABAbR antibodies and AMPAR antibodies?
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What syndrome is associated with Hodgkin lymphoma and presents with autoimmune encephalitis?
What syndrome is associated with Hodgkin lymphoma and presents with autoimmune encephalitis?
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In patients with anti-GABAbR encephalitis without detectable tumors, what may help in determining their follow-up?
In patients with anti-GABAbR encephalitis without detectable tumors, what may help in determining their follow-up?
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What is a rare symptom in limbic encephalitis with AMPAR antibodies?
What is a rare symptom in limbic encephalitis with AMPAR antibodies?
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Study Notes
Limbic Encephalitis
- A clinical-pathological entity with subacute onset (typically less than 3 months), characterized by cognitive decline, behavioral changes, seizures, and impaired short-term memory.
- Brain MRI often shows fluid-attenuated inversion recovery (FLAIR) and T2 signal abnormalities bilaterally, mainly in the hippocampus and amygdala.
- Symptoms primarily result from neuronal dysfunction within the limbic system, which includes amygdala, hippocampus, septal nuclei, cingulate cortex, orbitofrontal cortex, and anterior pole of temporal lobe.
- Autoimmune processes are a leading cause, potentially mediated by T cells or antibodies interacting with synaptic receptors.
- Inflammatory infiltrates are observed in cases linked with cytotoxic T cells.
- Often, the cause of the inflammatory response remains unknown.
Diagnostic Criteria
- Definite autoimmune limbic encephalitis: Requires four criteria:
- Subacute onset (rapid progression <3 months) of working memory loss, seizures, or psychiatric symptoms indicative of limbic system involvement.
- Bilateral MRI FLAIR/T2 abnormalities highly restricted to amygdala and medial temporal lobes.
- At least one of the following:
- CSF pleocytosis (WBC >5/mm³).
- EEG with epileptic or slow activity involving temporal lobes.
- Reasonable exclusion of alternative causes.
- Missing any one of the first three requirements excludes a definite diagnosis.
Neuronal Antibodies and Subtypes
- Neuronal antibody detection is a crucial diagnostic aid, especially when diagnostic criteria are not fully met.
- Antibody subtype guides the search for underlying tumors (e.g., LGI1 antibodies).
- A considerable association exists between certain antibodies and specific tumor types.
- Frequently observed associated tumor types include small cell lung cancer (SCLC), testicular tumors, thymoma, breast cancer, Hodgkin lymphoma, accounting for about 90% of cases.
Treatment and Prognosis
- Treatment approaches vary based on the antibody type and presence of tumors.
- Immunotherapeutic interventions (e.g., steroids, IVIg, plasma exchange) are sometimes ineffective in cases with antibodies directed toward intracellular targets.
- Rituximab, may, enhance treatment outcomes for some cases.
- Prognosis varies depending on the implicated antibodies, specific tumor types, and initial clinical presentation.
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Description
This quiz explores key observations linking limbic encephalitis to autoimmune responses, focusing on antibody associations and outcomes in patients. Questions delve into various studies and findings, shedding light on the neurobiological implications of this condition. Test your knowledge on this crucial area of neurology.
