Abstracts

Rationale: To describe a rare case of limbic encephalitis presenting with facio-brachio-crural dystonic seizures with basal ganglia involvement. Methods: We present the case of a 73 year old man with past medical history of hypertension and hypercholesterolemia who initially presented with jerking movements of the right upper extremity. The patient s wife described the events, as he would start making an involuntary grunting sound followed by flexion of the right upper extremity and turning his body towards the left side. The movements were very brief; lasting 10 to 15 seconds, sometimes involving the ipsilateral lower extremity causing recurrent falls. The patient finally had a tonic seizure after some weeks that required intubation. At that time, he was evaluated with a Brain MRI that showed a small enhancing lesion in the left thalamus.Results: PET scan showed decrease uptake in left basal ganglia as well. The patient was started on levetiracetam and valproic acid, which failed to control the events even on high doses. The patient was admitted to the Epilepsy Monitoring Unit where multiple seizures lasting 20 to 60 seconds were recorded. Within two months of the start of the symptoms, he became confused, disoriented and had short memory problems. Repeat brain MRI showed hyperintensities in the temporal lobes suggestive of limbic encephalitis. The patient was treated with IV-IG and Methylprednisolone 1 gram IV for 5 days followed by oral prednisone with clinical improvement and control of the seizures. CT chest, abdomen and pelvis were negative as well as inflammatory markers including ANA, ANCA, dsDNA, rheumatoid factor, anti-SSA and anti-SSB. Lumbar puncture showed WBC 1, protein 47, glucose 51. High levels of Tau protein and positive 14-3-3 protein in CSF were suggestive of CJD, however, given the clinical improvement of the patient to the treatment this diagnosis was excluded. Finally, voltage gated potassium channel antibody was positive as part of the work up for autoimmune limbic encephalitis. Conclusions: We present a case of limbic encephalitis that initially presented with facio-brachial dystonic seizures involving the lower extremity with crural dystonic seizures presenting with recurrent falls. This patient not only had the typical clinical features associated with limbic encephalitis, but also there was evidence of basal ganglia involvement proven by imaging studies. Our case highlights the importance of the rapid diagnosis and the proper treatment with IV-IG and/or IV steroids for patients presenting with facio-brachio-crural dystonic seizures prior to the development of cognitive manifestations and behavioral changes associated to limbic encephalitis. In conclusion, this case demonstrates the importance of recognizing the association between facio-brachio-crural dystonic seizures and VGK channel antibody limbic encephalitis.