The value of LGI1, Caspr2 and voltage-gated potassium channel antibodies in encephalitis.
Nat Rev Neurol. 2017-04-18; 13(5): 290-301
DOI: 10.1038/nrneurol.2017.43
Lire sur PubMed
van Sonderen A(1)(2), Petit-Pedrol M(3)(4), Dalmau J(3)(4)(5)(6), Titulaer MJ(1).
Author information:
(1)Department of Neurology, Erasmus University Medical Center, ‘s-Gravendijkwal 230, PO Box 2040, 3000CA Rotterdam, Netherlands.
(2)Department of Neurology, Haga Hospital, Leyweg 275, 2545CH The Hague, Netherlands.
(3)Neuroimmunology Program, Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), University of Barcelona, Carrer Casanova 143, Cellex P3A, 08036 Barcelona, Spain.
(4)Centro de Investigación Biomédica en Red (CIBER), Carrer Casanova 143, Cellex P3A, 08036 Barcelona, Spain.
(5)Department of Neurology, Hospital Clinic, Carrer Casanova 143, Cellex P3A, 08036 Barcelona, Spain.
(6)Institució Catalana de Recerca i Estudis Avançats (ICREA), Carrer Casanova 143, Cellex P3A, 08036 Barcelona, Spain and University of Pennsylvania, 3400 Spruce Street, 3 W. Gates, Philadelphia, Pennsylvania 19104, USA.
The discovery, in 2010, of autoantibodies against the extracellular proteins LGI1
and Caspr2 facilitated a change of view regarding the clinical importance of
voltage-gated potassium channel (VGKC) antibodies. Currently, these antibodies
are all classified as VGKC-complex antibodies, and are commonly considered to
have a similar clinical value. However, studies from the past few years show that
the immune responses mediated by these antibodies have differing clinical
relevance. Here, we review the clinical importance of these immune responses in
three settings: patients with anti-LGI1 antibodies, patients with anti-Caspr2
antibodies, and patients with antibodies against the VGKC complex that lack LGI1
and Caspr2 specificity. Antibodies against LGI1 and Caspr2 are associated with
different but well-defined syndromes, whereas the clinical importance of
VGKC-complex antibodies without LGI1 and Caspr2 specificity is questionable. We
describe each of these syndromes, discuss the function of the target antigens and
review the limited paediatric literature on the topic. The findings emphasize the
importance of defining these disorders according to the molecular identity of the
targets (LGI1 or Caspr2), and caution against the use of VGKC-complex antibodies
for the diagnosis and treatment of patients without further definition of the
antigen.