Diagnostic and therapeutic strategies for management of autoimmune encephalopathies

  title={Diagnostic and therapeutic strategies for management of autoimmune encephalopathies},
  author={Divyanshu Dubey and Kyle M. Blackburn and Benjamin M. Greenberg and Olaf Stuve and Steven Vernino},
  journal={Expert Review of Neurotherapeutics},
  pages={937 - 949}
ABSTRACT Introduction: The understanding of the etio-pathogenesis of autoimmune encephalopathy syndromes has grown significantly in recent years. These are potentially reversible conditions, with variable clinical presentation and potential dramatic response to immunotherapy. Areas covered: In this article we review various diagnostic techniques and therapeutic options for management of autoimmune encephalopathy. We also review medication adverse effects and monitoring strategies. Expert… 
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The spectrum of autoimmune encephalopathies
Advances in the diagnosis, pathogenesis and treatment of CIDP
  • M. Dalakas
  • Medicine, Biology
    Nature Reviews Neurology
  • 2011
Advances in the understanding of the underlying immunopathology in CIDP have identified new targets for future therapeutic efforts, including T cells, B cells, and transmigration and transduction molecules, including new target antigens.
The diagnosis of an autoimmune dementia requires the detection of objective improvements in cognitive decline after a course of immunotherapy, which should raise concern for a paraneoplastic etiology and may inform a targeted oncologic evaluation.
Immunotherapeutics for Autoimmune Encephalopathies and Dementias
  • A. McKeon
  • Medicine, Biology
    Current Treatment Options in Neurology
  • 2013
The duration of maintenance therapy is uncertain, but this author typically recommends 3–5 years of relapse-free maintenance treatment before discontinuing immunotherapy altogether.
Autoimmune encephalitis: Recent updates and emerging challenges
Clinical management of multiple sclerosis and neuromyelitis optica with therapeutic monoclonal antibodies: approved therapies and emerging candidates
The history of drug development of therapeutic mAbs, putative mechanisms of action, clinical evidence and safety of approved mAbs and those in different stages of clinical development in multiple sclerosis and neuromyelitis optica are reviewed.
[Therapeutic plasma exchange: applications in neurology].
Plasma exchange has proven to be an effective alternative treatment with high grade scientific evidence in diseases such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy and myasthenia gravis.
Anti-NMDA receptor encephalitis: diagnosis, psychiatric presentation, and treatment.
The clinical characteristics of anti-NMDA receptor encephalitis in an adolescent male are described and other disease processes to be considered in the differential diagnosis are outlined and currently used interventions, including psychotropic medications are described.
Neuropathology of autoimmune encephalitides.
Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease
The data support an off-label use of rituximab in pediatric autoimmune and inflammatory CNS disorders, although the significant risk of infectious complications suggests ritUXimab should be restricted to disorders with significant morbidity and mortality.