The Treatments for Autoimmune Encephalitis with Dr. Grace Gombolay
ฝัง
- เผยแพร่เมื่อ 1 ก.พ. 2024
- This is the first of a 5-part Speaker Series celebrating Autoimmune Encephalitis Awareness Month 2024 hosted by IAES and sponsored by UCB.
Dr. Gombolay begins her presentation with an overview of how Autoimmune Encephalitis is diagnosed, the many disorders that mimic the presentation, and the various antibody tests conducted. She takes us on a deep dive of the treatments. Reviews what each treatment does, the expected response time for treatments and when treatments should be escalated. Supportive medications for symptoms are reviewed. These are used until the treatment for the patient’s AE has reached full effect and then are slowly peeled off.
A question-and-answer session follows the presentation.
Grace Gombolay, MD, is the Director of the Pediatric Neuroimmunology and Multiple Sclerosis Clinic at Emory University School of Medicine/Children's Healthcare of Atlanta. She is a Pediatric Neurologist at Children’s Healthcare of Atlanta and an Assistant Professor at Emory University School of Medicine. She started the Pediatric Neuroimmunology and Multiple Sclerosis Clinic. This multidisciplinary clinic helps manage all aspects of patient care, including medical, psychological, and school-related issues. Her goal is to have the clinic become part of multi-center collaborations for clinical care and research. Dr. Gombolay strives to provide excellent clinical care to patients while studying the mechanisms in the immune system that result in disease and ways to modify disease.
Autoimmune Encephalitis is a group of inflammatory brain diseases that are characterized by prominent neuropsychiatric symptoms. These patients are often believed to have psychiatric presentations, but they do NOT. Common clinical features include a change in behavior, psychosis, seizures, memory, and cognitive deficits, abnormal movements, dysautonomia, and a decreased level of consciousness. Psychiatrists and Emergency Room Physicians are often the first medical professionals who see these patients. Clinicians must consider the possibility of an autoantibody-related etiology and become familiar with the red flags suggestive of synaptic autoimmunity as the underlying cause in all cases of first-onset, out-of-the-blue psychosis. A high level of suspicion is necessary as autoimmune encephalitis is treatable with immunotherapy. Firm evidence shows that earlier recognition and treatment lead to improved outcomes. It is important to note, that the disorder is refractory to antipsychotics; indeed, antipsychotic agents make affected patients much worse, even to the point of developing something akin to neuroleptic malignant syndrome.
The International Autoimmune Encephalitis Society, (IAES), is the only Family/Patient-centered organization for people with a diagnosis of Autoimmune Encephalitis. Leading with integrity, IAES strives to advance services, advocacy, education, awareness, and research for this group of diseases. IAES provides science-based information backed by trusted medical experts in the field of autoimmune neurology and relies on the expertise of our Medical Advisory Board. We are an established non-profit organization with a history of providing all-inclusive services from diagnosis to recovery and the many challenges experienced in one’s AE journey.
Visit our website: www.autoimmune-encephalitis.org
Your donations help us continue our important work.
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Really great presentation. We are lucky to have Dr. Gombolay treating our daugher.
🎯 Key Takeaways for quick navigation:
00:02 🎶 *Introduction to Autoimmune Encephalitis Awareness Month*
- Autoimmune Encephalitis Awareness Month is hosting a series of webinars on key topics.
- The first webinar focuses on treatments for autoimmune encephalitis.
- Dr. Grace Gombolay, Director of Pediatric Neuroimmunology at Emory University School of Medicine, is the honored guest.
03:40 💉 *Objectives and Disclosures*
- Dr. Gombolay discusses her objectives for the webinar, including an introduction to autoimmune encephalitis and discussions on treatment strategies.
- She briefly mentions her disclosures regarding support from the CDC and other activities.
04:36 🔍 *Approaches to Autoimmune Encephalitis Diagnosis*
- Overview of diagnostic approaches for autoimmune encephalitis, including pediatric and adult criteria.
- Discussion on distinguishing autoimmune encephalitis from other conditions with overlapping symptoms.
- Highlighting the importance of history, clinical judgment, and available diagnostic tests.
05:46 📋 *Diagnostic Guidelines and Considerations*
- Explanation of diagnostic criteria for pediatric and adult autoimmune encephalitis.
- Discussion on definite positive antibody AE, probable antibody-negative AE, and possible AE.
- Overview of other conditions mimicking autoimmune encephalitis and considerations for differential diagnosis.
12:42 🧪 *Assays for Autoimmune Encephalitis Antibodies*
- Explanation of immunofluorescence assay, radioimmunoassay, and cell-based assay for detecting autoimmune encephalitis antibodies.
- Mention of common antibodies tested for, such as GFAP, GAT-65, NMDA, LGI1, and CASPR2.
- Importance of understanding assay methods and interpretation for accurate diagnosis.
15:30 ⚙️ *Pathophysiology of Autoimmune Encephalitis*
- Discussion on immune responses targeting surface antigens and intracellular antigens in autoimmune encephalitis.
- Considerations for treatment selection based on the underlying immune mechanism.
- Explanation of management strategies, including evaluation for tumors, immunotherapy, and symptomatic treatment.
18:54 🧬 *Treatment Approaches for Autoimmune Encephalitis*
- Overview of current treatment approaches for autoimmune encephalitis.
- Explanation of the standard disease vs. severe disease treatment escalation.
- Discussion of first-line and second-line treatments based on antibody type and disease severity.
23:44 💉 *Steroids and Other First-Line Treatments*
- Detailed overview of corticosteroids (steroids) as a common first-line treatment.
- Explanation of intravenous immunoglobulin (IVIG) and its side effects.
- Discussion of plasma exchange as another first-line treatment option and its side effects.
29:29 🩸 *Second-Line Treatment Options: Rituximab (Rmab)*
- Introduction to rituximab (Rmab) as a common second-line treatment.
- Overview of how Rmab targets B cells to prevent them from becoming plasma cells.
- Discussion of potential side effects, including infusion reactions and immune suppression.
30:51 🧬 *Second-Line Treatment Options: Cyclophosphamide (CyC)*
- Explanation of cyclophosphamide (CyC) targeting both B and T cells.
- Overview of side effects, including hemorrhagic cystitis and immune suppression.
- Counseling considerations, particularly regarding fertility and pregnancy.
32:02 💊 *Second-Line Treatment Options: Inebilizumab (Inebil)*
- Introduction to inebilizumab targeting CD19 to eliminate B cells.
- Overview of side effects, including infusion reactions and immune suppression.
- Discussion of monitoring and counseling considerations for potential long-term risks.
32:57 💉 *Second-Line Treatment Options: Tocilizumab (Tocil)*
- Explanation of tocilizumab blocking interleukin-6 (IL-6) to reduce inflammation.
- Overview of side effects, including cholesterol elevation and liver enzyme changes.
- Discussion of monitoring and counseling, particularly regarding immune suppression and potential long-term risks.
35:57 🧠 *Long-term treatment considerations for autoimmune encephalitis:*
- Treatment for autoimmune encephalitis focuses on managing symptoms and may not require long-term interventions except for residual psychiatric symptoms or epilepsy in some cases.
- Various medications and interventions are utilized for movement disorders and seizures, including levodopa, benzodiazepines, antiepileptic drugs, and even botulinum toxin (Botox).
- Differentiating between seizures and EEG patterns like Delta waves can be challenging, requiring careful monitoring and assessment.
38:13 🛌 *Managing agitation, insomnia, and autonomic instability:*
- Patients with autoimmune encephalitis commonly experience agitation and insomnia, requiring a range of treatments such as melatonin, clonidine, trazodone, and others.
- Autonomic instability, including heart rate and blood pressure fluctuations, presents challenges and may necessitate careful monitoring and management strategies like minimizing parasympathetic stimulation.
- Hypoventilation and excessive salivation are additional complications that need to be addressed to prevent respiratory issues and infections.
41:26 📊 *Clinical course, recovery, and outcomes:*
- Recovery from autoimmune encephalitis is complex and prolonged, influenced by factors such as the underlying cause, timing of diagnosis, and response to treatment.
- Predicting outcomes is challenging but early treatment initiation and response to treatment within four weeks are associated with better outcomes.
- Neuropsychological impairments, including memory, attention, processing speed, and executive function, often persist even in patients with favorable physical recovery, highlighting the need for comprehensive assessment tools and long-term support.
53:27 🛌 *Sleep and Lifestyle Management*
- Managing sleep disturbances and establishing a consistent sleep schedule.
- Addressing psychiatric symptoms like anxiety and depression to improve concentration and memory.
- Incorporating memory aids, stress management techniques, healthy diet, social interactions, and exercise for overall well-being.
55:06 🧬 *Multicenter AE Registry & Summary*
- Introduction of the US-based multicenter AE registry called CONNECT for data collection and research collaboration.
- Encouragement for patients and clinicians to participate in the registry to advance understanding and treatment of autoimmune encephalitis.
- Summary of key points discussed, including diagnostic criteria, treatment options, and ongoing research efforts.
56:55 💡 *Q&A Session Highlights*
- Discussion on the utility of FDG-PET scans in diagnosis and treatment planning.
- Considerations for sequential versus simultaneous administration of IVIG and plasma exchange.
- Evaluation of symptomatic medications for cognitive symptoms and management of abnormal EEG findings without clinical seizures.
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Thank you for your overview of this webinar via AI.
What of patients who have acute escalations in inflammation and symptoms based on inflammatory triggers? Do we assume this is viral or bacterial?
What of trickier situations, such as a patient being treated for inborn errors such as CVID. What of their titers? How is that handled?
These cases where its an antibody panel or two and the same small pool of drugs is easy. What about the harder stuff, and research for that? (Which endlessly defining easy cases wont at all help...)
See the paper: A clinical Approach to Diagnosis of Autoimmune Encephalitis Graus, Dalmau et al 2016 and see the diagnostic panel for possible autoimmune encephalitis.
Anyone Near Baltimore Maryland I Can Contact Im Getting The Symptoms Of Ataxia Balance And Neurological 😔🙏
Hi, just to be clear, a patient can have all the symptoms in AE and not have AE because it mimics many different disorders. That said, we recommend you go to John Hopkins where they have an Encephalitis Center. Request an appointment with Arun Venkatesan, M.D., Ph.D. www.hopkinsmedicine.org/profiles/details/arun-venkatesan
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