Treatment of Movement Disorder Emergencies in Autoimmune Encephalitis in the Neurosciences ICU

Neurocrit Care. 2020 Feb;32(1):286-294. doi: 10.1007/s12028-019-00875-5.

Abstract

Immune response against neuronal and glial cell surface and cytosolic antigens is an important cause of encephalitis. It may be triggered by activation of the immune system in response to an infection (para-infectious), cancer (paraneoplastic), or due to a patient's tendency toward autoimmunity. Antibodies directed toward neuronal cell surface antigens are directly pathogenic, whereas antibodies with intracellular targets may become pathogenic if the antigen is transiently exposed to the cell surface or via activation of cytotoxic T cells. Immune-mediated encephalitis is well recognized and may require intensive care due to status epilepticus, need for invasive ventilation, or dysautonomia. Patients with immune-mediated encephalitis may become critically ill and display clinically complex and challenging to treat movement disorders in over 80% of the cases (Zhang et al. in Neurocrit Care 29(2):264-272, 2018). Treatment options include immunotherapy and symptomatic agents affecting dopamine or acetylcholine neurotransmission. There has been no prior published guidance for management of these movement disorders for the intensivist. Herein, we discuss the immune-mediated encephalitis most likely to cause critical illness, clinical features and mechanisms of movement disorders and propose a management algorithm.

Keywords: Autoimmune; Chorea; Dyskinesia; Encephalitis; Movement disorder.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Adrenergic alpha-Antagonists / therapeutic use
  • Adrenergic beta-Antagonists / therapeutic use
  • Analgesics, Opioid / therapeutic use
  • Anticonvulsants / therapeutic use
  • Antiparkinson Agents / therapeutic use
  • Autoantibodies / immunology
  • Autoimmune Diseases of the Nervous System / complications
  • Autoimmune Diseases of the Nervous System / drug therapy*
  • Autoimmune Diseases of the Nervous System / immunology
  • Autoimmune Diseases of the Nervous System / physiopathology
  • Benzodiazepines / therapeutic use
  • Catatonia / drug therapy
  • Catatonia / etiology
  • Catatonia / physiopathology
  • Cholinergic Antagonists / therapeutic use*
  • Chorea / drug therapy
  • Chorea / etiology
  • Chorea / physiopathology
  • Critical Illness
  • Dopamine Agents / therapeutic use*
  • Dopamine Antagonists / therapeutic use
  • Dyskinesias / drug therapy
  • Dyskinesias / etiology
  • Dyskinesias / physiopathology
  • Dystonia / drug therapy
  • Dystonia / etiology
  • Dystonia / physiopathology
  • Emergencies
  • Encephalitis / complications
  • Encephalitis / drug therapy*
  • Encephalitis / immunology
  • Encephalitis / physiopathology
  • Humans
  • Hypnotics and Sedatives / therapeutic use
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / therapeutic use*
  • Intensive Care Units
  • Movement Disorders / drug therapy*
  • Movement Disorders / etiology
  • Movement Disorders / physiopathology
  • Myoclonus / drug therapy
  • Myoclonus / etiology
  • Myoclonus / physiopathology
  • Neuromuscular Blocking Agents / therapeutic use*
  • Paraneoplastic Syndromes, Nervous System / complications
  • Paraneoplastic Syndromes, Nervous System / drug therapy
  • Paraneoplastic Syndromes, Nervous System / immunology
  • Paraneoplastic Syndromes, Nervous System / physiopathology
  • Plasmapheresis

Substances

  • Adrenal Cortex Hormones
  • Adrenergic alpha-Antagonists
  • Adrenergic beta-Antagonists
  • Analgesics, Opioid
  • Anticonvulsants
  • Antiparkinson Agents
  • Autoantibodies
  • Cholinergic Antagonists
  • Dopamine Agents
  • Dopamine Antagonists
  • Hypnotics and Sedatives
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Immunosuppressive Agents
  • Neuromuscular Blocking Agents
  • Benzodiazepines