Seronegative (or antibody-negative) Autoimmune Encephalitis with Josep Dalmau, MD, PhD

UCB sponsors the 2024 Speaker Series celebrating Autoimmune Encephalitis Awareness Month.
Josep Dalmau, MD, PhD IDIBAPS-Hospital Clinic, University of Barcelona, and Caixa Research Institute, Barcelona (Spain). Adjunct Professor of Neurology, University of Pennsylvania, Philadelphia, USA
Dr. Dalmau is a neurologist specializing in Neuro-oncology, paraneoplastic syndromes, and autoimmune encephalitis. He discovered the first type of Autoimmune Encephalitis, anti-NMDAr, which was documented in a 2007 case study. Thus, identifying a new disease. All of us owe our lives to Dr. Dalmau. Dr. Dalmau has discovered 11 autoimmune diseases, known as autoimmune encephalopathies.
e-mail: [email protected]

Dr. Dalmau begins by describing the types of antibodies found in this group of diseases. Currently, there are 17 different types of identified AE. A case study of a patient who had a differential diagnosis of anti-NMDAr AE illustrates the importance of the patient's clinical presentation not comporting with AE. Differential diagnoses and the process of arriving at a diagnosis, errors in past diagnoses, and antibody testing with an emphasis on tissue-based assays not being reliable, and the proposed steps to refine the diagnosis of AE are reviewed.
How to treat antibody-negative but probable AE. What is the future direction we are taking to advance the understanding of this group of diseases? The presentation is followed by a 50-minute lively and detailed question-and-answer period.
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Autoimmune Encephalitis is a group of inflammatory brain diseases 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.