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Issue: Issue 1 (2014)


Review Article

Neuropsychiatric symptoms in autoimmune encephalopathies: a clinician’s guide

Author(s):
Fátima Carvalho, João Massano, and Rui Coelho
Abstract:
Background: The spectrum of central nervous system autoimmune disorders has recently expanded with the discovery of disorders associated with antibodies directed against the neuronal membrane surface. Although many of these disorders have an underlying malignancy and present with signs of dysfunction of the limbic system (paraneoplastic limbic encephalitis, PLE), a high proportion of cases is non-paraneoplastic. They may occur with milder symptoms, and present no abnormalities in the exams usually used in the investigation of PLE. A striking number of cases may be misdiagnosed as primary psychiatric or other neurological disorders. This paper aims to review the current knowledge on this topic, and provide physicians with an updated text on the neuropsychiatric presentation, diagnostic approach and current management of autoimmune encephalopathies.

Methods: We searched PubMed for articles published in English until December 2013, using the terms: "Autoimmune limbic encephalitis"; “Limbic encephalitis”; “Psychiatry”; “Psychotic Disorders”; “Anti-N-Methyl-D-Aspartate Receptor Encephalitis”; "Gamma-aminobutyric-acid receptor”; “Leucine-rich-glioma-inactivated 1”; "Voltage-gated-potassium channel”; "?-amino-3-hydroxy-5-methyl-4-isoxazolepropionic-acid receptor”; “Ri”; “Ma2”; and “Hu”. We restricted the search to human studies, and selected articles for further analysis. Article reference lists were also reviewed and relevant articles retrieved for consultation.

Results: 109 articles were reviewed, and data summarized. The authors propose diagnostic and treatment algorithms.

Conclusion: Autoimmune encephalitis is not a rare disorder. It often has a psychiatric presentation, and should be considered whenever a non-psychiatric etiology is contemplated. Diagnosis is often challenging, but certain clinical features should raise suspicion about an underlying autoimmune or paraneoplastic disorder, thus guiding the physician to structured investigations, including tumor screening, and adequate therapeutic interventions, namely immunotherapy.

Keywords: Clinical medicine, Paraneoplastic syndromes, Limbic encephalitis, Psychotic disorders, Autoantibodies,Immunotherapy.

International Journal of Clinical Neurosciences and Mental Health 2014; 1:11

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