Case Report Faciobrachial dystonic seizures result from fronto–temporo–basalganglial network involvement Rajesh Shankar Iyer a, ⁎, T.C.R. Ramakrishnan a , Karunakaran b , Ajit Shinto c , Koramadai Karuppuswamy Kamaleshwaran c a Department of Neurology, KG Hospital & Post Graduate Medical Institute, Coimbatore, Tamil Nadu, India b Department of Radiology, KG Hospital & Post Graduate Medical Institute, Coimbatore, Tamil Nadu, India c Department of Nuclear Medicine, Kovai Medical Centre and Hospital, Coimbatore, Tamil Nadu, India article info Article history: Received 25 December 2016 Received in revised form 19 February 2017 Accepted 1 June 2017 Available online 8 June 2017 Keywords: Faciobrachial dystonic seizures LGI1antibody VGKC antibody encephalitis Fronto–temporo–basalganglial network Basalganglial hypermetabolism Network hypothesis 1. Introduction In 2008, Faciobrachial dystonic seizures (FBDS), characterized by unilateral, short-lived dystonic posturing of the upper limb and face were recognized as an immunotherapy-responsive disorder [1]. They are accompanied by autoantibodies to leucine-rich glioma-inactivated 1 (LGI1), a component of the voltage-gated potassium channel complex (VGKC-complex) [2,3]. Since their initial description, the epileptic origin of FBDS has been extensively debated with no definite conclusions drawn so far [4]. We discuss the clinical, electrophysiological and imag- ing features of two patients with LGI1 antibody mediated encephalitis who presented with FBDS. We also analyze the existing literature on FBDS and propose the network hypothesis to explain the various fea- tures of FBDS described so far. 2. Case vignette 1 A 45-year-old lady presented with a two-month history of intermit- tent generalized seizures, behavior disturbances and hallucinations along with memory disturbances. On examination she had very frequent events associated with facio-brachial dystonic jerks occurring on either side followed by brief loss of awareness associated with ictal Epilepsy & Behavior Case Reports 8 (2017) 47–50 ⁎ Corresponding author. E-mail address: dr_rsh@hotmail.com (R.S. Iyer). speech and hand automatisms. The events were of 30–45 seconds in du- ration. The jerks did not have any electrical correlate. They were associ- ated with movement artifacts followed by 2 seconds of attenuation followed further by right temporal progression of sharp waves coincid- ing with ictal speech and automatisms (Fig. 1F&G). MRI Brain showed bilateral medial temporal hyperintensities whereas basal ganglia (BG) structures were spared (Fig. 1A&B). Her serum VGKC-antibody (LGI1 antibodies) titer was very high at 3069 pmol/L. 18F-fluoro-2-deoxy-D- glucose positron emission tomography/computerized tomography (FDG PET/CT) showed bilateral BG and amygdalar hypermetabolism (Fig. 1D), and hypometabolism of bilateral frontal and temporal lobes. A thorough work-up for neoplasms was negative. There was no clinical response to antiseizures drugs. She was given a course of intravenous methylprednisolone (IVMP) followed by weekly pulses of IVMP. Two months later on follow-up she showed good clinical improvement in behavior and psychiatric disturbances. FBDS of shorter duration (4– 6 seconds) persisted though less in number with occasional loss of bal- ance and fall. Emotional disturbances would trigger the attacks. Howev- er loss of awareness and automatisms following the jerks disappeared after treatment. Electrical changes were restricted to movement artifacts without the ensuing temporal lobe changes. This time the serum VGKC-antibody titer was at 560 pmols/L. Repeat MRI showed generalized atrophy and some persistence of the medial temporal hyperintensities (Fig. 1C). A repeat FDG PET/CT showed persistence of the bilateral BG hypermetabolism and disappearance of the amygdalar changes (Fig. 1E). She was started on intravenous immunoglobulins following which her FBDS resolved completely. 3. Case vignette 2 A 53-year-old lady presented with a one-month history of one episode of nocturnal GTCS followed by frequent jerkiness of the left upper limb and occasional falls. The events were of 5–7 seconds in du- ration. Interestingly her memory and behavior were relatively intact. Clinically she hadleft-sided FBDS occurring multiple times a day. A few of them were facio-brachio-crural events responsible for her falls. MRI showed T1, T2 and FLAIR hyperintensities involving the bilateral cau- date nuclei and putamen (Fig. 1H,I&J) and the substantia nigra on the left side (Fig. 1K). The left hippocampus was edematous. Her interictal EEG showed bilateral temporal slow waves. Her serum LGI1 antibodies assay by immunofluorescence was positive (low VGKC complex IgG Contents lists available at ScienceDirect Epilepsy & Behavior Case Reports journal homepage: www.elsevier.com/locate/ebcr http://dx.doi.org/10.1016/j.ebcr.2017.06.001 2213-3232/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).