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Autoimmune Encephalitis: Clinical Spectrum and Management

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Autoimmune Encephalitis: Clinical Spectrum and Management Review Pract Neurol: first published as 10.1136/practneurol-2020-002567 on 9 June 2021. Downloaded from Autoimmune encephalitis: clinical spectrum and management Christopher E Uy ,1,2 Sophie Binks ,1,2 Sarosh R Irani 1,2 1Oxford Autoimmune Neurology ABSTRACT This field is of major clinical impor- Group, Nuffield Department of Autoimmune encephalitis defines brain tance to all neurologists because these Clinical Neurosciences, Oxford, UK inflammation caused by a misdirected immune patients present with a wide variety 2Department of Neurology, response against self- antigens expressed in of neurological features and typically Oxford University Hospitals NHS the central nervous system. It comprises a respond to immunotherapies. Therefore, Foundation Trust, Oxford, UK heterogeneous group of disorders that are these conditions are often considered ‘not Correspondence to at least as common as infectious causes of to miss’ diagnoses, with defined patho- Prof Sarosh R Irani, Oxford encephalitis. The rapid and ongoing expansion genic agents that can present to cognitive, Autoimmune Neurology of this field has been driven by the identification movement disorder, epilepsy, psychiatry Group, Nuffield Department of Clinical Neurosciences, Oxford of several pathogenic autoantibodies that and peripheral nerve clinics. University, Oxford, Oxfordshire, cause polysymptomatic neurological and In this pragmatic review, which reflects UK; sarosh. irani@ ndcn. ox. ac. uk neuropsychiatric diseases. These conditions often our experience of managing >200 cases show highly distinctive cognitive, seizure and with surface- directed autoantibodies, we Accepted 14 May 2021 movement disorder phenotypes, making them highlight key clinical features to help clinically recognisable. Their early identification identify these patients, outline immuno- and treatment improve patient outcomes, logical findings that inform laboratory and may aid rapid diagnosis of an underlying testing and describe the clinically relevant associated tumour. Here we summarise the disease biology of relevance to treatment well- known autoantibody- mediated encephalitis decisions. syndromes with neuronal cell- surface antigens. We focus on practical aspects of their diagnosis Autoimmune encephalitis is not rare and treatment, offer our clinical experiences of Until the discovery of neuroglial surface managing such cases and highlight more basic autoantibodies, infections were the most neuroimmunological advances that will inform common known causes of encephalitis. their future diagnosis and treatments. However, over the last 20 years, the http://pn.bmj.com/ description of multiple autoantibodies targeting the extracellular domains of neuroglial proteins in patients with INTRODUCTION encephalitis has shifted this balance. For Autoimmune encephalitis comprises a example, the California Encephalitis group of disorders in which the host immune Project found that among persons under on September 28, 2021 by guest. Protected copyright. system targets self-antigens expressed in the 30 years of age, N- methyl- D- aspartate 1 central nervous system (CNS). Some of the receptor (NMDAR)- antibody encephalitis best- characterised diseases are associated was more common than any individual with autoantibodies that target neuroglial infectious cause of encephalitis.5 Also, antigens (table 1). These autoantibodies autoimmune causes of encephalitis have are considered pathogenic because they been reported to be at least as common are directed against the extracellular—and as viral causes in Olmsted County, USA.6 hence in vivo exposed—domains of their Interestingly, the incidence of autoim- 2–4 target antigens. This fundamental prop- mune encephalitis rose in the second erty has led to much interest and excitement 10- year epoch of this study, likely owing © Author(s) (or their surrounding this rapidly expanding field, to growing awareness of these disorders employer(s)) 2021. Re- use with new autoantibody targets described and more widespread diagnostic capaci- permitted under CC BY. most years. Many established antigens Published by BMJ. ties. Nevertheless, as fever, focal neurolog- are key synaptic proteins, ion channels or ical deficits and cerebrospinal fluid (CSF) To cite: Uy CE, Binks S, receptors, meaning that the extracellular lymphocytosis remain inclusion criteria Irani SR. Pract Neurol Epub ahead of print: [please include domain- targeting autoantibodies are likely for many ‘all cause encephalitis’ studies, Day Month Year]. doi:10.1136/ to directly modulate critical physiological such approaches likely continue to under- practneurol-2020-002567 processes. estimate the prevalence of autoimmune Uy CE, et al. Pract Neurol 2021;0:1–14. doi:10.1136/practneurol-2020-002567 1 2 Review Table 1 Demographic, clinical and paraclinical features of neuronal autoantibody syndromes Neuronal auto- antibody (Ref.) and predominant IgG Median age, years Sex ratio Immunotherapy response and subclass (range) (M:F) Clinical features MR brain scan findings CSF findings EEG findings Other investigations outcomes NMDAR10 25 43 56 21 1:4 Encephalitis 70%–80% normal or non- 80% abnormal 90% abnormal (slowing Ovarian teratoma in 60% of ~50% improve in 4 weeks with IgG1 (2 months–85 years) with prominent specific, with a typical limbic (lymphocytic pleocytosis, most common, 20% adult, female patients. first line immunotherapy (IT). polysymptomatic encephalitis in a minority. unpaired oligoclonal epileptiform abnormalities, After HSV encephalitis, ~70% of non- responders improve neuropsychiatric bands common). rarely extreme delta brush particularly children can develop soon after 2nd line IT. presentation, polymorphic pattern). NMDAR (and other neuronal Improvement up to 24 months, movement disorder, surface) autoantibodies. with 80% reaching mRS 0–2. language disorder, 10%–15% relapse risk—reduced autonomic dysfunction, by IT and tumour removal coma and central apnoea. ~5% mortality. LGI1*33 49 57 64 2:1 Limbic encephalitis with ~75% abnormal. ~25% abnormal (mild ~50% abnormal (~30% >90% with HLA- DRB1*07:01. At 2 years, 1/3 fully recovered, IgG4 (31–84) frequent focal seizures, ~40% increased signal/swelling pleocytosis with elevated epileptiform abnormal, Hyponatraemia common 1/3 functionally independent including characteristic in medial temporal lobes protein). ~20% focal slowing). (~70%). but unable to work, 1/3 severely facio- brachial dystonic (unilateral >bilateral). disabled or dead. seizures. Relapses in 20%–30%; associated with poor outcomes. CASPR2*30 49 55 66 9:1 Main syndromes: ~30% increased signal in medial ~30% abnormal ~70% abnormal (40% HLA- DRB1*11:01. ~50% with good or full response IgG4 (25–77) peripheral nerve temporal lobes. (pleocytosis, elevated epileptiform abnormal). Thymoma in ~20% (often with to tumour therapy/IT. hyperexcitability, limbic protein±oligoclonal LGI1 antibodies in addition) ~45% with partial IT response. encephalitis and Morvan’s bands). Electromyography may ~25% relapse. syndrome. demonstrate hyperexcitability Uy CE, Uy CE, (fasciculations, myokymia). 20 21 GABAAR 40 1:1 Encephalitis with frequent >80% cortical and subcortical 25–50% lymphocytic >80% abnormal Thymoma ~30%. IT- responsive, however, mortality et al IgG1 (2 months–88 years) status epilepticus. FLAIR signal abnormalities pleocytosis±oligoclonal (encephalopathy with ictal due to status epilepticus or related . involving 2+ brain regions. bands and elevated abnormalities). complications ~10–20%. Pract Neurol Pract protein. 22 GABABR 61 1.5:1 Limbic encephalitis with ~70% abnormal (45% increased ~80% lymphocytic ~75% with ictal Tumours in ~50% ~90% show response to IT, those IgG1 (16–77) prominent seizures. signal in medial temporal lobes. pleocytosis. abnormalities. (mostly SCLC). with tumour have poorer prognosis 2021; with recurrent neurological symptoms and higher mortality. 0 58 :1–14. doi:10.1136/practneurol-2020-002567 :1–14. AMPAR Mean 53.1 2:1 Limbic encephalitis with ~85% abnormal (67% with ~70% abnormal. 45% abnormal. Tumour identified in ~70% Most patients showed (14–92) prominent confusion, bilateral mesial temporal (thymus, SCLC, breast, ovary). improvement from peak of disease, amnesia, seizures and involvement). median mRS=1 in survivors. psychiatric/behavioural ~15% of reported patients died symptoms. (commonly due to complications from malignancy). DPPX27 53 1.5:1 Multifocal encephalitis 100% normal or non-specific. ~30% abnormal (mild ~70% abnormal (focal or ~10% with B- cell neoplasm 60%–70% improve with IT. (13–76) with myoclonus, tremors pleocytosis and elevated diffuse slowing). (gastrointestinal follicular and hyperekplexia, protein). lymphoma, ; leukaemia). prominent diarrhoea/ weight loss. Continued Pract Neurol: first published as 10.1136/practneurol-2020-002567 on 9 June 2021. Downloaded from from Downloaded 2021. June 9 on 10.1136/practneurol-2020-002567 as published first Neurol: Pract http://pn.bmj.com/ on September 28, 2021 by guest. Protected by copyright. by Protected guest. by 2021 28, September on Uy CE, Uy CE, et al . Pract Neurol Pract 2021; Table 1 Continued 0 Neuronal auto- :1–14. doi:10.1136/practneurol-2020-002567 :1–14. antibody (Ref.) and predominant IgG Median age, years Sex ratio Immunotherapy response and subclass (range) (M:F) Clinical features MR brain scan findings CSF findings EEG findings Other investigations outcomes GlyR28 50 1:1 3 main syndromes: Brain: temporal lobe ~40% pleocytosis, 20% ~70% abnormal EMG abnormal 60% ~10% mortality in initial case IgG1/3
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