{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T03:22:11Z","timestamp":1762917731021},"reference-count":34,"publisher":"FapUNIFESP (SciELO)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dement. neuropsychol."],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:p>ABSTRACT  Background: Creutzfeldt-Jakob Disease (CJD) is the prototypical cause of rapidly progressive dementia (RPD). Nonetheless, efforts to exclude reversible causes of RPD that mimic prion disease are imperative. The recent expanding characterization of neurological syndromes associated with antibodies directed against neuronal cell surface or sympathic antigens, namely autoimmune encephalitis is shifting paradigms in neurology. Such antigens are well known proteins and receptors involved in synaptic transmission. Their dysfunction results in neuropsychiatric symptoms, psychosis, seizures, movement disorders and RPD. Faciobrachial dystonic seizure (FBDS) is a novel characterized type of seizure, specific for anti-LGI1 encephalitis.  Objective: In order to improve clinical recognition we report the cases of two Brazilian patients who presented with characteristic FDBS (illustrated by videos) and anti-LGI1 encephalitis.  Methods: We have included all patients with FBDS and confirmed anti-LGI1 encephalitis and video records of FDBS in two tertiary Brazilian centers: Department of Neurology of Hospital das Cl\u00ednicas, Sao Paulo University, Sao Paulo, Brazil and Hospital Geral de Fortaleza, Fortaleza, Brazil between January 1, 2011 and December 31, 2015.  Results: Both patients presented with clinical features of limbic encephalitis associated with FBDS, hyponatremia and normal CSF. None of them presented with tumor and both showed a good response after immunotherapy.  Conclusion: FBDSs may be confounded with myoclonus and occurs simultaneously with rapid cognitive decline. Unawareness of FDBS may induce to misdiagnosing a treatable cause of RPD as CJD.<\/jats:p>","DOI":"10.1590\/s1980-5764-2016dn1004016","type":"journal-article","created":{"date-parts":[[2016,12,21]],"date-time":"2016-12-21T23:46:15Z","timestamp":1482363975000},"page":"351-357","source":"Crossref","is-referenced-by-count":13,"title":["The importance of recognizing faciobrachial dystonic seizures in rapidly progressive dementias"],"prefix":"10.1590","volume":"10","author":[{"given":"Mateus Mistieri","family":"Simabukuro","sequence":"first","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paulo Ribeiro","family":"N\u00f3brega","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil; Hospital Geral de Fortaleza,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Milena","family":"Pitombeira","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil; Hospital Geral de Fortaleza,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wagner Cid Palmeira","family":"Cavalcante","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronnyson Susano","family":"Grativvol","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L\u00e9cio Figueira","family":"Pinto","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luiz Henrique Martins","family":"Castro","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo","family":"Nitrini","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o 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