| Literature DB >> 24709680 |
Andrea Klang1, Peter Schmidt, Sibylle Kneissl, Zoltán Bagó, Angela Vincent, Bethan Lang, Teresa Moloney, Christian G Bien, Péter Halász, Jan Bauer, Akos Pákozdy.
Abstract
Voltage-gated potassium channel complex (VGKC-complex) antibody (Ab) encephalitis is a well-recognized form of limbic encephalitis in humans, usually occurring in the absence of an underlying tumor. The patients have a subacute onset of seizures, magnetic resonance imaging findings suggestive of hippocampal inflammation, and high serum titers of Abs against proteins of the VGKC-complex, particularly leucine-rich, glioma-inactivated 1 (LGI1). Most patients are diagnosed promptly and recover substantially with immunotherapies; consequently, neuropathological data are limited. We have recently shown that feline complex partial cluster seizures with orofacial involvement (FEPSO) in cats can also be associated with Abs against VGKC-complexes/LGI1. Here we examined the brains of cats with FEPSO and compared the neuropathological findings with those in a human with VGKC-complex-Ab limbic encephalitis. Similar to humans, cats with VGKC-complex-Ab and FEPSO have hippocampal lesions with only moderate T-cell infiltrates but with marked IgG infiltration and complement C9neo deposition on hippocampal neurons, associated with neuronal loss. These findings provide further evidence that FEPSO is a feline form of VGKC-complex-Ab limbic encephalitis and provide a model for increasing understanding of the human disease.Entities:
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Year: 2014 PMID: 24709680 DOI: 10.1097/NEN.0000000000000063
Source DB: PubMed Journal: J Neuropathol Exp Neurol ISSN: 0022-3069 Impact factor: 3.685