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Autoimmune Encephalitis

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Emergencies in Neurology

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Abstract

Limbic encephalitis causes a wide spectrum of neurological symptoms ranging from confusion, behavioural disturbance, involuntary movements, psychiatric symptoms and seizures. The original description was largely in patients with a progressive neurological disease and an underlying neoplasia but the antibodies detected were directed towards intracellular proteins, shared by the tumours and the nervous system, and were thus markers of the presence of the tumour and the associated neurological disease, but not pathogenic. Over the last 15 years, by contrast, several pathogenic antibodies against the extracellular domains of neuronal cell surface antigens have been identified; these antibodies cause immunomodulatory therapy-responsive encephalopathic symptoms which are often termed “autoimmune encephalitis”. The target antigens are membrane receptors or ion channel proteins and include glutamate receptor subtypes (N-methyl d-aspartate receptor, alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPAR type 1 and 2)), voltage gated potassium channel associated proteins (leucine-rich glioma inactivated protein 1 (LGI1) and contactin-associated protein 2 (CASPR2)) and gamma-aminobutyric acid receptor type (GABAbR and GABAaR). In addition, patients with progressive encephalomyelitis, rigidity and myoclonus often have antibodies against the alpha-1 subunit of the glycine receptor. The forms of autoimmune encephalitis associated with neuronal surface antibodies often have subacute or even acute onset. Early and appropriate recognition of these syndromes is important since prompt initiation of immunomodulation in the form of plasma exchange, intravenous immunoglobulins or steroids can lead to substantial improvement. This chapter will discuss the common autoimmune encephalitides, their pathophysiological mechanisms, diagnosis and treatment.

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Abbreviations

ACE-R:

Addenrooke’s cognitive examination-revised

ADAM:

Disintegrin and metalloproteinase domain-containing protein

ADEM:

Acute disseminated encephalomyelitis

AMPAR:

α-amino-3-hydroxy-5-methyl-4-isoxazol-propionic acid receptor

AZA:

Azathioprine

BIRDS:

Brief ictal rhythmic discharges

CASPR2:

Contactin-associated protein 2

CJD:

Creutzfield jakob disease

CSF:

Cerebrospinal fluid

FDG-PET:

18-fluorodeoxyglucose positron emission tomography

FLAIR:

Fluid attenuation inversion recovery

GABAaR:

γ-amino-butyric acid A-receptor

GABAbR:

γ-amino-butyric acid B-receptor

GAD:

Glutamic acid decarboxylase

GBS:

Guillain–Barré syndrome

GCS:

Glasgow coma scale

GluR:

Glutamate receptor

GlyR:

Glycine receptor

IST:

Immunosuppressive therapy

IVIg:

Intravenous immunoglobulin

LGI1:

Leucine-rich glioma inactivated protein 1

MMF:

Mycophenolate mofetil

MMSE:

Mini-mental state examination

MOG:

Myelin oligodendrocyte glycoprotein

MRI:

Magnetic resonance imaging

NMDAR:

N-methyl d-aspartate receptor

NMO:

Neuromyelitis optica

PERM:

Progressive encephalomyelitis with rigidity and myoclonus

PLEX:

Plasma exchange

USS:

Ultrasound scan

VGKC:

Voltage gated potassium channel

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Authors and Affiliations

  1. Department of Neurology, University Hospitals Birmingham and Neuroimmunology Clinical Lead, University of Birmingham, Birmingham, UK

    Saiju Jacob

  2. Nuffield Department of Clinical Neurology, University of Oxford, John Radcliffe Hospital, Oxford, UK

    Angela Vincent

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  1. Department of Neurology, All India Institute of Medical Sciences, New Delhi, India

    Mamta Bhushan Singh

  2. Department of Neurology, All India Institute of Medical Sciences, New Delhi, India

    Rohit Bhatia

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Jacob, S., Vincent, A. (2019). Autoimmune Encephalitis. In: Singh, M., Bhatia, R. (eds) Emergencies in Neurology . Springer, Singapore. https://doi.org/10.1007/978-981-13-7381-7_2

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