PMID- 32699718
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200928
IS  - 2168-8184 (Print)
IS  - 2168-8184 (Electronic)
IS  - 2168-8184 (Linking)
VI  - 12
IP  - 6
DP  - 2020 Jun 20
TI  - A Tale of Two Brothers: Familial Voltage-Gated Potassium Channel Autoimmune 
      Encephalitis.
PG  - e8723
LID - 10.7759/cureus.8723 [doi]
LID - e8723
AB  - This is the first reported case of familial voltage-gated potassium channel 
      (VGKC) autoimmune encephalitis. The symptoms of autoimmune encephalitis can mimic 
      infectious encephalitis with headache, fatigue, and neuropsychiatric symptoms. 
      Autoimmunity is emerging as a distinct cause of encephalitis in the children. 
      Prompt recognition, diagnosis, and treatment are important to prevent brain 
      damage. Two brothers presented two years apart with different symptoms. The 
      explanation for their distinct symptoms lies in the multifactorial development of 
      autoimmunity. The presentation of autoimmune encephalitis can depend on the 
      offending antibodies. The most common are antibodies against the 
      N-methyl-D-aspartic acid (NMDA) receptor and the VGKC complex. Antibodies to the 
      VGKC complex are divided into three different groups depending on their antigenic 
      target: leucine-rich glioma-inactivated protein 1 (LGI1), contactin-associated 
      protein-like 2 (CASPR2), or neither. Anti-VGKC antibodies in children are 
      associated with neuroinflammation and encephalitis. Autoimmunity to LGI1 and 
      CASPR2 antigens is associated with distinct human leukocyte antigen (HLA) 
      alleles. Different HLA isotypes are involved in antigen processing and 
      presentation and can lead to a genetic predisposition to autoimmunity. VGKC 
      autoimmune encephalitis can present with memory changes, psychiatric symptoms, 
      and motor abnormalities. Both brothers presented with these symptoms in their own 
      unique way. Efficient diagnosis and immunosuppression helped improve their 
      outcomes.
CI  - Copyright (c) 2020, Gillespie et al.
FAU - Gillespie, Lauren E
AU  - Gillespie LE
AD  - Pediatrics, Creighton University School of Medicine, Omaha, USA.
FAU - Dave, Amanda
AU  - Dave A
AD  - Pediatrics, University of Nebraska Medical Center, Omaha, USA.
FAU - Goldstein, Amy
AU  - Goldstein A
AD  - Pediatrics, University of Nebraska Medical Center, Omaha, USA.
LA  - eng
PT  - Case Reports
DEP - 20200620
PL  - United States
TA  - Cureus
JT  - Cureus
JID - 101596737
PMC - PMC7372199
OTO - NOTNLM
OT  - autoimmune
OT  - autoimmune encephalitis
OT  - caspr2
OT  - channelopathies
OT  - encephalitis
OT  - lgi1
OT  - neuroimmunology
OT  - vgkc
OT  - vgkc encephalitis
OT  - voltage-gated potassium channel autoimmune encephalitis
COIS- The authors have declared that no competing interests exist.
EDAT- 2020/07/24 06:00
MHDA- 2020/07/24 06:01
PMCR- 2020/06/20
CRDT- 2020/07/24 06:00
PHST- 2020/07/24 06:00 [entrez]
PHST- 2020/07/24 06:00 [pubmed]
PHST- 2020/07/24 06:01 [medline]
PHST- 2020/06/20 00:00 [pmc-release]
AID - 10.7759/cureus.8723 [doi]
PST - epublish
SO  - Cureus. 2020 Jun 20;12(6):e8723. doi: 10.7759/cureus.8723.