From the Guidelines
Autoimmune encephalitis symptoms include confusion, memory problems, seizures, personality changes, and psychiatric symptoms like hallucinations, paranoia, or agitation, as reported in the most recent study 1. The presentation of autoimmune encephalitis varies widely depending on which part of the brain is affected and which antibodies are involved.
- Symptoms typically develop subacutely over days to weeks, rather than suddenly, and may fluctuate in severity.
- Patients may experience sleep disturbances, movement disorders (tremors, abnormal postures, or difficulty walking), speech problems, and decreased consciousness that can progress to coma in severe cases.
- Physical symptoms often include headaches, fever, fatigue, and sensory changes. Early recognition is crucial as prompt treatment with immunotherapy significantly improves outcomes, as suggested by the best practice recommendations for diagnosis and acute management 1. The condition occurs when the immune system mistakenly attacks healthy brain tissue, often triggered by infections, tumors, or genetic predisposition, leading to brain inflammation and neurological dysfunction, as described in the introduction to autoimmune encephalitis 1. The most effective approach to managing autoimmune encephalitis involves a combination of immunotherapy and supportive care, with the goal of reducing inflammation and preventing long-term neurological damage 1.
From the Research
Symptoms of Autoimmune Encephalitis
The symptoms of autoimmune encephalitis can vary widely, but common manifestations include:
- Psychiatric symptoms such as psychosis, aggression, mutism, memory loss, euphoria, or fear 2
- Seizures, cognitive decline, coma, and abnormal movements 2
- Fluctuating symptoms that can change rapidly 2
- Cognitive impairment, fatigue, pain, and mental health issues 3
- Highly distinctive cognitive, seizure, and movement disorder phenotypes, making them clinically recognizable 4
Clinical Spectrum
Autoimmune encephalitis comprises a heterogeneous group of disorders that are at least as common as infectious causes of encephalitis 4. The clinical spectrum of autoimmune encephalitis includes:
- Polysymptomatic neurological and neuropsychiatric diseases 4
- Brain inflammation caused by a misdirected immune response against self-antigens expressed in the central nervous system 4
- Autoantibody-mediated encephalitis syndromes with neuronal cell-surface antigens 4
Diagnosis and Treatment
Early identification and treatment of autoimmune encephalitis can improve patient outcomes 4. The diagnosis and treatment of autoimmune encephalitis involve:
- Detection of pathogenic autoantibodies in serum and/or cerebrospinal fluid 3
- Immunotherapy, including corticosteroids, plasma exchange, and intravenous immunoglobulins as first-line treatments 5, 6
- Second-line immunotherapies, such as rituximab and cyclophosphamide, for unresponsive cases 5, 6
- Alternative therapies, such as tocilizumab and bortezomib, for refractory cases 5, 6