Immunotherapeutics for autoimmune encephalopathies and dementias

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

The timely implementation of immunotherapy is key to successful treatment of autoimmune encephalopathies or dementias (from here on will be referred to as autoimmune encephalopathies). There are different levels of diagnostic certainty which should guide the immunological treatment of autoimmune encephalopathies. There is a high level of diagnostic certainty for patients who have classic limbic encephalitis and have a neural antibody detected in serum or CSF (such as potassium channel complex antibody). For these patients, initiating high-dose corticosteroids or IVIg is indicated, with plasma exchange, rituximab or cyclophosphamide used as second-line therapy if first-line therapy proves only partially beneficial. There is a lower level of diagnostic certainty in patients with non-limbic atypical phenotypes (though rapidly progressive) when no neural antibody is detected in serum and CSF. A trial of cortico-steroids or IVIg (or both sequentially) may be undertaken in these patients, but if no objective improvements occur, further immunotherapy is unlikely to be beneficial. Antiepileptic treatment also plays a critical role in those who have seizures as well as cognitive symptoms. Evaluation for and treatment of any underlying cancer is another component for those patients with a paraneoplastic cause of encephalitis. An individualized maintenance regimen needs to be designed for patients who do improve with immunotherapy. Individual factors that need to be considered when formulating a program of maintenance treatment include disease severity, antibody specificity and proclivity for disease relapse. Azathioprine and mycophenolate mofetil are frequently used for the purpose of remission maintenance, and should permit gradual withdrawal of steroids, IVIg or more toxic immunosuppressants. The duration of maintenance therapy is uncertain, but this author typically recommends 3-5 years of relapse-free maintenance treatment before discontinuing immunotherapy altogether.

Original languageEnglish (US)
Pages (from-to)723-737
Number of pages15
JournalCurrent Treatment Options in Neurology
Volume15
Issue number6
DOIs
StatePublished - 2013

Fingerprint

Brain Diseases
Dementia
Immunotherapy
Therapeutics
Antibodies
Limbic Encephalitis
Steroids
Maintenance
Mycophenolic Acid
Recurrence
Neurobehavioral Manifestations
Plasma Exchange
Antibody Specificity
Poisons
Potassium Channels
Azathioprine
Encephalitis
Immunosuppressive Agents
Serum
Anticonvulsants

Keywords

  • Autoimmune
  • Autoimmune encephalopathies
  • Azathioprine
  • Cyclophosphamide
  • Dementia
  • Encephalopathy
  • Immunotherapeutics
  • Immunotherapy
  • Intravenous immune globulin
  • IVIg
  • Mycophenolate mofetil
  • Paraneoplastic
  • Plasma exchange
  • Rituximab
  • Steroids

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Immunotherapeutics for autoimmune encephalopathies and dementias. / McKeon, Andrew B.

In: Current Treatment Options in Neurology, Vol. 15, No. 6, 2013, p. 723-737.

Research output: Contribution to journalArticle

@article{8bc79290232f4d3a9edeecc0fbaa86ff,
title = "Immunotherapeutics for autoimmune encephalopathies and dementias",
abstract = "The timely implementation of immunotherapy is key to successful treatment of autoimmune encephalopathies or dementias (from here on will be referred to as autoimmune encephalopathies). There are different levels of diagnostic certainty which should guide the immunological treatment of autoimmune encephalopathies. There is a high level of diagnostic certainty for patients who have classic limbic encephalitis and have a neural antibody detected in serum or CSF (such as potassium channel complex antibody). For these patients, initiating high-dose corticosteroids or IVIg is indicated, with plasma exchange, rituximab or cyclophosphamide used as second-line therapy if first-line therapy proves only partially beneficial. There is a lower level of diagnostic certainty in patients with non-limbic atypical phenotypes (though rapidly progressive) when no neural antibody is detected in serum and CSF. A trial of cortico-steroids or IVIg (or both sequentially) may be undertaken in these patients, but if no objective improvements occur, further immunotherapy is unlikely to be beneficial. Antiepileptic treatment also plays a critical role in those who have seizures as well as cognitive symptoms. Evaluation for and treatment of any underlying cancer is another component for those patients with a paraneoplastic cause of encephalitis. An individualized maintenance regimen needs to be designed for patients who do improve with immunotherapy. Individual factors that need to be considered when formulating a program of maintenance treatment include disease severity, antibody specificity and proclivity for disease relapse. Azathioprine and mycophenolate mofetil are frequently used for the purpose of remission maintenance, and should permit gradual withdrawal of steroids, IVIg or more toxic immunosuppressants. The duration of maintenance therapy is uncertain, but this author typically recommends 3-5 years of relapse-free maintenance treatment before discontinuing immunotherapy altogether.",
keywords = "Autoimmune, Autoimmune encephalopathies, Azathioprine, Cyclophosphamide, Dementia, Encephalopathy, Immunotherapeutics, Immunotherapy, Intravenous immune globulin, IVIg, Mycophenolate mofetil, Paraneoplastic, Plasma exchange, Rituximab, Steroids",
author = "McKeon, {Andrew B}",
year = "2013",
doi = "10.1007/s11940-013-0251-8",
language = "English (US)",
volume = "15",
pages = "723--737",
journal = "Current Treatment Options in Neurology",
issn = "1092-8480",
publisher = "Current Science, Inc.",
number = "6",

}

TY - JOUR

T1 - Immunotherapeutics for autoimmune encephalopathies and dementias

AU - McKeon, Andrew B

PY - 2013

Y1 - 2013

N2 - The timely implementation of immunotherapy is key to successful treatment of autoimmune encephalopathies or dementias (from here on will be referred to as autoimmune encephalopathies). There are different levels of diagnostic certainty which should guide the immunological treatment of autoimmune encephalopathies. There is a high level of diagnostic certainty for patients who have classic limbic encephalitis and have a neural antibody detected in serum or CSF (such as potassium channel complex antibody). For these patients, initiating high-dose corticosteroids or IVIg is indicated, with plasma exchange, rituximab or cyclophosphamide used as second-line therapy if first-line therapy proves only partially beneficial. There is a lower level of diagnostic certainty in patients with non-limbic atypical phenotypes (though rapidly progressive) when no neural antibody is detected in serum and CSF. A trial of cortico-steroids or IVIg (or both sequentially) may be undertaken in these patients, but if no objective improvements occur, further immunotherapy is unlikely to be beneficial. Antiepileptic treatment also plays a critical role in those who have seizures as well as cognitive symptoms. Evaluation for and treatment of any underlying cancer is another component for those patients with a paraneoplastic cause of encephalitis. An individualized maintenance regimen needs to be designed for patients who do improve with immunotherapy. Individual factors that need to be considered when formulating a program of maintenance treatment include disease severity, antibody specificity and proclivity for disease relapse. Azathioprine and mycophenolate mofetil are frequently used for the purpose of remission maintenance, and should permit gradual withdrawal of steroids, IVIg or more toxic immunosuppressants. The duration of maintenance therapy is uncertain, but this author typically recommends 3-5 years of relapse-free maintenance treatment before discontinuing immunotherapy altogether.

AB - The timely implementation of immunotherapy is key to successful treatment of autoimmune encephalopathies or dementias (from here on will be referred to as autoimmune encephalopathies). There are different levels of diagnostic certainty which should guide the immunological treatment of autoimmune encephalopathies. There is a high level of diagnostic certainty for patients who have classic limbic encephalitis and have a neural antibody detected in serum or CSF (such as potassium channel complex antibody). For these patients, initiating high-dose corticosteroids or IVIg is indicated, with plasma exchange, rituximab or cyclophosphamide used as second-line therapy if first-line therapy proves only partially beneficial. There is a lower level of diagnostic certainty in patients with non-limbic atypical phenotypes (though rapidly progressive) when no neural antibody is detected in serum and CSF. A trial of cortico-steroids or IVIg (or both sequentially) may be undertaken in these patients, but if no objective improvements occur, further immunotherapy is unlikely to be beneficial. Antiepileptic treatment also plays a critical role in those who have seizures as well as cognitive symptoms. Evaluation for and treatment of any underlying cancer is another component for those patients with a paraneoplastic cause of encephalitis. An individualized maintenance regimen needs to be designed for patients who do improve with immunotherapy. Individual factors that need to be considered when formulating a program of maintenance treatment include disease severity, antibody specificity and proclivity for disease relapse. Azathioprine and mycophenolate mofetil are frequently used for the purpose of remission maintenance, and should permit gradual withdrawal of steroids, IVIg or more toxic immunosuppressants. The duration of maintenance therapy is uncertain, but this author typically recommends 3-5 years of relapse-free maintenance treatment before discontinuing immunotherapy altogether.

KW - Autoimmune

KW - Autoimmune encephalopathies

KW - Azathioprine

KW - Cyclophosphamide

KW - Dementia

KW - Encephalopathy

KW - Immunotherapeutics

KW - Immunotherapy

KW - Intravenous immune globulin

KW - IVIg

KW - Mycophenolate mofetil

KW - Paraneoplastic

KW - Plasma exchange

KW - Rituximab

KW - Steroids

UR - http://www.scopus.com/inward/record.url?scp=84892364013&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84892364013&partnerID=8YFLogxK

U2 - 10.1007/s11940-013-0251-8

DO - 10.1007/s11940-013-0251-8

M3 - Article

AN - SCOPUS:84892364013

VL - 15

SP - 723

EP - 737

JO - Current Treatment Options in Neurology

JF - Current Treatment Options in Neurology

SN - 1092-8480

IS - 6

ER -