Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management

Abboud, Hesham; Probasco, John C.; Irani, Sarosh R.; Ances, Beau M.; Benavides, David R.; Bradshaw, Michael; Christo, Paulo Pereira; Dale, Russell C.; Fernández-Fournier, Mireya; Flanagan, Eoin P.; Gadoth, Avi; George, Pravin; Grebenciucova, Elena; Jammoul, Adham; Lee, Sang Kun; Li, Yuebing; Matiello, Marcelo; Morse, Anne Marie; Rae-Grant, Alexander; Rojas, Galeno; Rossman, Ian; Schmitt, Sarah; Venkatesan, Arun; Vernino, Steven; Pittock, Sean J.; Titulaer, Maarten J.

doi:10.1136/jnnp-2020-325300

Cited by 243 publications

(300 citation statements)

References 40 publications

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“…Best practice recommendations for the management of autoimmune encephalitis have recently been made (111).…”

Section: Management Of Autoimmune Encephalitismentioning

confidence: 99%

Autoimmune Disorders of the Nervous System: Pathophysiology, Clinical Features, and Therapy

Bhagavati

2021

Front. Neurol.

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Remarkable discoveries over the last two decades have elucidated the autoimmune basis of several, previously poorly understood, neurological disorders. Autoimmune disorders of the nervous system may affect any part of the nervous system, including the brain and spinal cord (central nervous system, CNS) and also the peripheral nerves, neuromuscular junction and skeletal muscle (peripheral nervous system, PNS). This comprehensive overview of this rapidly evolving field presents the factors which may trigger breakdown of self-tolerance and development of autoimmune disease in some individuals. Then the pathophysiological basis and clinical features of autoimmune diseases of the nervous system are outlined, with an emphasis on the features which are important to recognize for accurate clinical diagnosis. Finally the latest therapies for autoimmune CNS and PNS disorders and their mechanisms of action and the most promising research avenues for targeted immunotherapy are discussed.

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“…Best practice recommendations for the management of autoimmune encephalitis have recently been made (111).…”

Section: Management Of Autoimmune Encephalitismentioning

confidence: 99%

Autoimmune Disorders of the Nervous System: Pathophysiology, Clinical Features, and Therapy

Bhagavati

2021

Front. Neurol.

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“…There was no standard management for anti-AMPAR encephalitis. Therapies were usually chosen with reference to other autoimmune encephalitis (47), such as anti-NMDAR encephalitis. About 60-80% of autoimmune encephalitis with antibodies against neuronal surface antigens responded well to immunotherapy (48,49).…”

Section: Treatment and Prognosismentioning

confidence: 99%

Anti-Alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic Acid Receptor Encephalitis: A Review

et al. 2021

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Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis, a rare subtype of autoimmune encephalitis, was first reported by Lai et al. The AMPAR antibodies target against extracellular epitopes of the GluA1 or GluA2 subunits of the receptor. AMPARs are expressed throughout the central nervous system, especially in the hippocampus and other limbic regions. Anti-AMPAR encephalitis was more common in middle-aged women and most patients had an acute or subacute onset. Limbic encephalitis, a classic syndrome of anti-AMPAR encephalitis, was clinically characterized by a subacute disturbance of short-term memory loss, confusion, abnormal behavior and seizure. Magnetic resonance imaging often showed T2/fluid-attenuated inversion-recovery hyperintensities in the bilateral medial temporal lobe. For suspected patients, paired serum and cerebrospinal fluid (CSF) testing with cell-based assay were recommended. CSF specimen was preferred given its higher sensitivity. Most patients with anti-AMPAR encephalitis were complicated with tumors, such as thymoma, small cell lung cancer, breast cancer, and ovarian cancer. First-line treatments included high-dose steroids, intravenous immunoglobulin and plasma exchange. Second-line treatments, including rituximab and cyclophosphamide, can be initiated in patients who were non-reactive to first-line treatment. Most patients with anti-AMPAR encephalitis showed a partial neurologic response to immunotherapy.

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“…In the case of an unknown target antigen, prediction of the effectiveness of either strategy may not be possible ex ante. According to a recent survey, most therapist chose rituximab over cyclophosphamide in a setting with a supposed and unknown antibody [ 138 ].…”

Section: Treatment and Managementmentioning

confidence: 99%

Update on Paraneoplastic Cerebellar Degeneration

2021

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Purpose of review: To provide an update on paraneoplastic cerebellar degeneration (PCD), the involved antibodies and tumors, as well as management strategies. Recent findings: PCD represents the second most common presentation of the recently established class of immune mediated cerebellar ataxias (IMCAs). Although rare in general, PCD is one of the most frequent paraneoplastic presentations and characterized clinically by a rapidly progressive cerebellar syndrome. In recent years, several antibodies have been described in association with the clinical syndrome related to PCD; their clinical significance, however, has yet to be determined. The 2021 updated diagnostic criteria for paraneoplastic neurologic symptoms help to establish the diagnosis of PCD, direct cancer screening, and to evaluate the presence of these newly identified antibodies. Recognition of the clinical syndrome and prompt identification of a specific antibody are essential for early detection of an underlying malignancy and initiation of an appropriate treatment, which represents the best opportunity to modulate the course of the disease. As clinical symptoms can precede tumor diagnosis by years, co-occurrence of specific symptoms and antibodies should prompt continuous surveillance of the patient. Summary: We provide an in-depth overview on PCD, summarize recent findings related to PCD, and highlight the transformed diagnostic approach.

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Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management

Cited by 243 publications

References 40 publications

Autoimmune Disorders of the Nervous System: Pathophysiology, Clinical Features, and Therapy

Autoimmune Disorders of the Nervous System: Pathophysiology, Clinical Features, and Therapy

Anti-Alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic Acid Receptor Encephalitis: A Review

Update on Paraneoplastic Cerebellar Degeneration

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