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We report a 59-year-old right-handed woman with smoldering leucine-rich glioma-inactivated 1 (LGI1) antibody-associated limbic encephalitis (LE) following faciobrachial dystonic seizures. During 8 months before her admission, she developed partial seizures manifesting very brief and very frequent dystonia in her right hand sometimes with oral automatism and loss of awareness. In addition, she showed psychiatric disturbances such as emotionally labile condition and personality changes. On admission, neuropsychological examination revealed short-term memory impairment. During electroencephalography (EEG) monitoring, ictal EEG showed rhythmic delta waves and interictal EEG showed intermittent irregular slow waves at the bilateral frontotemporal area. Brain MRI demonstrated high T2/FLAIR signal changes in the left amygdala expanding into the left hippocampus. FDG-PET showed hypermetabolism in the left amygdala, hippocampus and the bilateral basal ganglia. Cerebrospinal fluid analysis was unremarkable. There were no signs of malignant tumor detected on systemic examination. LGI1 antibody was positive in the serum and the cerebrospinal fluid and the clinical diagnosis of LGI1 antibody-associated LE was confirmed. Her symptoms and the abnormalities in the brain MRI/FDG-PET showed immediate improvement after anti-epileptic and steroid therapy.

A case of smoldering anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated limbic encephalitis with faciobrachial dystonic seizure