PMID- 28248701 OWN - NLM STAT- MEDLINE DCOM- 20180111 LR - 20220321 IS - 1473-6551 (Electronic) IS - 1350-7540 (Linking) VI - 30 IP - 3 DP - 2017 Jun TI - Autoimmune encephalitis with anti-leucine-rich glioma-inactivated 1 or anti-contactin-associated protein-like 2 antibodies (formerly called voltage-gated potassium channel-complex antibodies). PG - 302-309 LID - 10.1097/WCO.0000000000000444 [doi] AB - PURPOSE OF REVIEW: Twenty years since the discovery of voltage-gated potassium channel (VGKC)-related autoimmunity; it is currently known that the antibodies are not directed at the VGKC itself but to two closely associated proteins, anti-leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (Caspr2). Antibodies to LGI1 and Caspr2 give well-described clinical phenotypes. Anti-LGI1 encephalitis patients mostly have limbic symptoms, and anti-Caspr2 patients have variable syndromes with both central and peripheral symptoms. A large group of patients with heterogeneous symptoms are VGKC positive but do not have antibodies against LGI1 or Caspr2. The clinical relevance of VGKC positivity in these 'double-negative' patients is questionable. This review focusses on these three essentially different subgroups. RECENT FINDINGS: The clinical phenotypes of anti-LGI1 encephalitis and anti-Caspr2 encephalitis have been described in more detail including data on treatment and long-term follow-up. A specific human leukocyte antigen (HLA) association was found in nontumor anti-LGI1 encephalitis, but not clearly in those with tumors. There has been increasing interest in the VGKC patients without LGI1/Caspr2 antibodies questioning its relevance in clinical practice. SUMMARY: Anti-LGI1 encephalitis and anti-Caspr2 encephalitis are separate clinical entities. Early recognition and treatment is necessary and rewarding. The term VGKC-complex antibodies, lumping patients with anti-LGI1, anti-Caspr2 antibodies or lacking both, should be considered obsolete. FAU - Bastiaansen, Anna E M AU - Bastiaansen AEM AD - aDepartment of Neurology, Erasmus University Medical Center, Rotterdam bDepartment of Neurology, Haga Hospital, The Hague, the Netherlands. FAU - van Sonderen, Agnes AU - van Sonderen A FAU - Titulaer, Maarten J AU - Titulaer MJ LA - eng PT - Journal Article PT - Review PL - England TA - Curr Opin Neurol JT - Current opinion in neurology JID - 9319162 RN - 0 (Autoantibodies) RN - 0 (CNTNAP2 protein, human) RN - 0 (Intracellular Signaling Peptides and Proteins) RN - 0 (LGI1 protein, human) RN - 0 (Membrane Proteins) RN - 0 (Nerve Tissue Proteins) RN - 0 (Potassium Channels, Voltage-Gated) RN - 0 (Proteins) RN - Hashimoto's encephalitis SB - IM MH - Autoantibodies/*analysis MH - Encephalitis/*immunology/physiopathology/therapy MH - Hashimoto Disease/*immunology/physiopathology/therapy MH - Humans MH - Intracellular Signaling Peptides and Proteins MH - Membrane Proteins/*immunology MH - Nerve Tissue Proteins/*immunology MH - Potassium Channels, Voltage-Gated/immunology MH - Proteins/*immunology EDAT- 2017/03/02 06:00 MHDA- 2018/01/13 06:00 CRDT- 2017/03/02 06:00 PHST- 2017/03/02 06:00 [pubmed] PHST- 2018/01/13 06:00 [medline] PHST- 2017/03/02 06:00 [entrez] AID - 10.1097/WCO.0000000000000444 [doi] PST - ppublish SO - Curr Opin Neurol. 2017 Jun;30(3):302-309. doi: 10.1097/WCO.0000000000000444.