Open AccessClaustrum hyperintensities: A potential clue to autoimmune epilepsy
Author(s) -
Steriade Claude,
TangWai David F.,
Krings Timo,
Wennberg Richard
Publication year - 2017
Publication title -
epilepsia open
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.247
H-Index - 16
ISSN - 2470-9239
DOI - 10.1002/epi4.12077
Subject(s) - hyperintensity , claustrum , fluid attenuated inversion recovery , ictal , epilepsy , neuroscience , medicine , psychology , autoimmune encephalitis , pathology , magnetic resonance imaging , radiology , encephalitis , immunology , virus , nucleus
Summary In a cohort of 34 patients with autoimmune limbic encephalitis and/or epilepsy, we identified 4 patients exhibiting claustrum fluid‐attenuated inversion recovery ( FLAIR ) hyperintensities. All 4 patients presented with explosive onset of seizures and developed medically intractable epilepsy, and 2 exhibited a marked response to immunotherapy. Associated features included cognitive and behavioral disturbances (4/4), cerebrospinal fluid ( CSF ) lymphocytic pleocytosis (3/4), and a neural autoantibody (2/4). Electroencephalogram ( EEG ) features consisted of slow wave activity and epileptiform discharges in frontal and parasagittal regions, where ictal patterns were captured in 1 patient. In 1 patient, magnetoencephalographic source imaging of interictal spikes revealed dipole sources in anterior insular or subinsular localizations, mirroring claustrum FLAIR hyperintensities, which developed after a short lag from presentation and resolved in all but 1 patient. These MRI abnormalities were isolated (2/4) or associated with mesial temporal hyperintensities (2/4). Claustrum FLAIR hyperintensities may be a useful MRI marker of autoimmune epilepsy.