ABSTRACT
Importance: Autoimmune encephalitis (AE) refers to a group of neuropsychiatric conditions associated with specific circulating autoantibodies directed against synaptic receptors, neuronal cell surface proteins, and intracellular targets. Increased recognition of these disorders is of value, as affected patients prominently display cognitive impairment, behavioral disturbances, and seizures requiring multidisciplinary teams, with early recognition often impacting prognosis.
Observations: This case series is based on a retrospective record review of adult patients diagnosed with AE between January 1, 2010– January 1, 2020. Cases 1 and 2, demonstrating anti– N-methyl-d-aspartate receptor (NMDAR) encephalitis with initial manifestations of neurologic and psychiatric symptoms, correlate with the literature describing a higher prevalence of this condition in young women. Case 3, despite being seronegative, exhibited classic features of anti-NMDAR encephalitis. Case 4 demonstrates a classic presentation of anti–leucine-rich glioma-inactivated 1 (LGI1) encephalitis: a middle-aged male with psychosis, altered mentation, and epilepsy. Case 5 had a more indolent neuropsychiatric presentation with mild elevation of N-type voltage-gated potassium channel (VGKC) antibody. Case 6, with glutamic acid decarboxylase 65 (GAD65) antibody, was an elderly female with speech dysfunction and altered mentation, and case 7 was an elderly male with GAD65 antibody who had stiff-person syndrome, ataxia, cognitive decline, and thymoma.
Conclusions: This retrospective case series describes the clinical details of 7 individuals with AE and overlapping neuropsychiatric symptoms. This series is limited in scope, with a small number of cases and observational findings, which prevents specific conclusions from being drawn. Despite this limitation, the present article explores the nuances of variable presentations of this disease to inform better interdisciplinary management and emphasize the gap areas that need rigorous research.
Continue Reading...
Members enjoy unlimited free PDF downloads as part of their subscription! Subscribe today for instant access to this article and our entire library in your preferred format. Alternatively, you can purchase the PDF of this article individually.
References (27)
- Brierley JB, Corsellis JAN, Hierons R, et al. Subacute encephalitis of later adult life mainly affecting the limbic areas. Brain. 1960;83(3):357–368. CrossRef
- Corsellis JA, Goldberg GJ, Norton AR. “Limbic encephalitis” and its association with carcinoma. Brain. 1968;91(3):481–496. PubMed CrossRef
- Vincent A, Buckley C, Schott JM, et al. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain. 2004;127(Pt 3):701–712. PubMed CrossRef
- Dalmau J, Tüzün E, Wu HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36. PubMed CrossRef
- Herken J, Prüss H. Red flags: clinical signs for identifying autoimmune encephalitis in psychiatric patients. Front Psychiatry. 2017;8:25. PubMed CrossRef
- Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391–404. PubMed CrossRef
- Al-Diwani A, Handel A, Townsend L, et al. The psychopathology of NMDAR-antibody encephalitis in adults: a systematic review and phenotypic analysis of individual patient data. Lancet Psychiatry. 2019;6(3):235–246. PubMed CrossRef
- Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7(12):1091–1098. PubMed CrossRef
- Venkatesan A, Adatia K. Anti-NMDA-receptor encephalitis: from bench to clinic. ACS Chem Neurosci. 2017;8(12):2586–2595. PubMed CrossRef
- Iizuka T, Sakai F, Ide T, et al. Anti-NMDA receptor encephalitis in Japan: long-term outcome without tumor removal. Neurology. 2008;70(7):504–511. PubMed CrossRef
- Samanta D, Lui F. Anti-NMDA Receptor Encephalitis. StatPearls. Updated August 10, 2020. https://www.ncbi.nlm.nih.gov/books/NBK551672/
- Kuppuswamy PS, Takala CR, Sola CL. Management of psychiatric symptoms in anti-NMDAR encephalitis: a case series, literature review and future directions. Gen Hosp Psychiatry. 2014;36(4):388–391. PubMed CrossRef
- Dalmau J, Armangué T, Planagumà J, et al. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. Lancet Neurol. 2019;18(11):1045–1057. PubMed CrossRef
- Chapman MR, Vause HE. Anti-NMDA receptor encephalitis: diagnosis, psychiatric presentation, and treatment. Am J Psychiatry. 2011;168(3):245–251. PubMed CrossRef
- Pollak TA, Lennox BR, Müller S, et al. Autoimmune psychosis: an international consensus on an approach to the diagnosis and management of psychosis of suspected autoimmune origin. Lancet Psychiatry. 2020;7(1):93–108. PubMed CrossRef
- Sen A, Wang J, Laue-Gizzi H, et al. Pathognomonic seizures in limbic encephalitis associated with anti-LGI1 antibodies. Lancet. 2014;383(9933):2018. PubMed CrossRef
- Lai M, Huijbers MG, Lancaster E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol. 2010;9(8):776–785. PubMed CrossRef
- Lancaster E, Huijbers MG, Bar V, et al. Investigations of caspr2, an autoantigen of encephalitis and neuromyotonia. Ann Neurol. 2011;69(2):303–311. PubMed CrossRef
- Li JM, Ma XR, Peng T, et al. Clinical features and outcomes in relapsing and monophasic patients with anti-leucine-rich glioma-inactivated 1 encephalitis [in Chinese]. Zhonghua Yi Xue Za Zhi. 2020;100(25):1947–1951. PubMed
- van Sonderen A, Schreurs MW, de Bruijn MA, et al. The relevance of VGKC positivity in the absence of LGI1 and Caspr2 antibodies. Neurology. 2016;86(18):1692–1699. PubMed CrossRef
- Michael S, Waters P, Irani SR. Stop testing for autoantibodies to the VGKC-complex: only request LGI1 and CASPR2. Pract Neurol. 2020;20(5):377–384. PubMed CrossRef
- Gresa-Arribas N, Ariño H, Martínez-Hernández E, et al. Antibodies to inhibitory synaptic proteins in neurological syndromes associated with glutamic acid decarboxylase autoimmunity. PLoS One. 2015;10(3):e0121364. PubMed CrossRef
- Lancaster E, Dalmau J. Neuronal autoantigens–pathogenesis, associated disorders and antibody testing. Nat Rev Neurol. 2012;8(7):380–390. PubMed CrossRef
- Ariño H, Höftberger R, Gresa-Arribas N, et al. Paraneoplastic neurological syndromes and glutamic acid decarboxylase antibodies. JAMA Neurol. 2015;72(8):874–881. PubMed CrossRef
- Manto M, Honnorat J, Hampe CS, et al. Disease-specific monoclonal antibodies targeting glutamate decarboxylase impair GABAergic neurotransmission and affect motor learning and behavioral functions. Front Behav Neurosci. 2015;9:78. PubMed CrossRef
- Tsiortou P, Alexopoulos H, Dalakas MC. GAD antibody-spectrum disorders: progress in clinical phenotypes, immunopathogenesis and therapeutic interventions. Ther Adv Neurol Disord. 2021;14:17562864211003486. PubMed CrossRef
- Saiz A, Blanco Y, Sabater L, et al. Spectrum of neurological syndromes associated with glutamic acid decarboxylase antibodies: diagnostic clues for this association. Brain. 2008;131(Pt 10):2553–2563. PubMed CrossRef
Please sign in or purchase this PDF for $40.
Save
Share
Cite
Already a member? Login
