Rinsho Shinkeigaku (Clinical Neurology)

Case Report

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

Yuriko Nakaoku, M.D. 1), Takakuni Maki, M.D., Ph.D. 1), Kyoko Kanazawa, M.D. 1), Riki Matsumoto, M.D., Ph.D. 1), Hidenao Fukuyama, MD., Ph.D. 2), Ryosuke Takahashi, M.D., Ph.D. 1) and Akio Ikeda, M.D., Ph.D. 1)

1)Department of Neurology, Kyoto University Graduate School of Medicine
2)Department of Brain Functional Imaging, Human Brain Research Center, Kyoto University Graduate School of Medicine

We report a 59-year-old right-handed woman with smoldering leucine-rich glioma-inactivated 1 (LGI1) antibodyassociated 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 sample of faciobrachial dystonic seizures (FBDS). The right arm shows brief dystonic posture associated with rather trembling, most likely myoclonic, movements. Test words "mouse and rocket" was given with the interval of 10 sec and the patient remembered.
Video is available with the courtesy of patient's consent.
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(CLINICA NEUROL, 53: 706|711, 2013)
key words: faciobrachial dystonic seizure, limbic encephalitis, leucine-rich glioma-inactivated 1, anti-voltage-gated potassium channel complex antibody

(Received: 20-Nov-12)