Abstract
Autoimmune encephalitis (AE) is a rapidly progressive inflammatory neurological disease. Underlying autoantibodies can bind to neuronal surfaces and synaptic proteins resulting in psychiatric symptoms, focal neurological signs, autonomic dysfunction and cognitive decline. Early and effective treatment is mandatory to reduce clinical symptoms and to achieve remission. Therapeutic apheresis, involving both plasma exchange (PE) and immunoadsorption (IA), can rapidly remove pathogenic antibodies from the circulation, thus representing an important first-line treatment in AE patients. We here review the most relevant studies regarding therapeutic apheresis in AE, summarizing the outcome for patients and the expanding clinical spectrum of treatment-responsive clinical conditions. For example, patients with slowly progressing cognitive impairment suggesting a neurodegenerative dementia can have underlying autoantibodies and improve with therapeutic apheresis. Findings are encouraging and have led to the first ongoing clinical studies assessing the therapeutic effect of IA in patients with anti-neuronal autoantibodies and the clinical presentation of dementia. Therapeutic apheresis is an established and well tolerated option for first-line therapy in AE and, potentially, other antibody-mediated central nervous system diseases.
Highlights
Autoimmune encephalitis (AE) is a rapidly progressive inflammatory neurological disease with subacute onset
We included all articles about patients with autoimmune encephalitis—antibody-mediated as well as paraneoplastic—treated with plasma exchange or immunoadsorption
It has been proven to be beneficial in primary demyelinating disease as well as in encephalitis caused by antibodies targeting neuronal proteins [14] (Table 2)
Summary
Autoimmune encephalitis (AE) is a rapidly progressive inflammatory neurological disease with subacute onset. Patients may present with behavioral changes and altered mental status as well as reduced levels of consciousness and new focal neurological signs or epileptic seizures [1]. AE comprises both, antibody-mediated and paraneoplastic, i.e., cytotoxic T-cell-mediated, encephalitides. Clinical presentation is diverse and depends on the specific underlying antibody (Table 1). As more and more novel antibodies and new clinical phenotypes are being identified, the incidence is rising and currently estimated at 5–10 per 100,000 inhabitants per year [1]. Age and gender preferences are often specific for a given antibody. The exact target of novel antibodies is not known yet. Even if the underlying antigen is known, the pathogenic relevance still awaits scientific clarification
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