ArticleDementia & neuropsychologia
The importance of recognizing faciobrachial dystonic seizures in rapidly progressive dementias.
Article in Dementia & neuropsychologia. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 16 citations in OpenAlex.
- Electroencephalographic biomarkers of antibody-mediated autoimmune encephalitis.Frontiers in neurology · 2025Article
- Cortical metabolic characteristics of anti-leucine-rich glioma-inactivated 1 antibody encephalitis based onFrontiers in neurology · 2023Article
- Confusion and Hallucination in a Geriatric Patient. Pitfalls of a Rare Differential: Case Report of an Anti-LGI1-Encephalitis.Clinical interventions in aging · 2022Article
- Faciobrachial Myoclonus as the Presenting Manifestation of Diabetic Keto-Acidosis.Tremor and other hyperkinetic movements (New York, N.Y.) · 2021Article
- Faciobrachial motor seizures: A more apt description?Epilepsy & behavior reports · 2021Article
- Anti-leucine-rich Glioma-inactivated-1 Encephalitis with Faciobrachial Dystonic Seizures and Behavioral Problems: A Case Report.JNMA; journal of the Nepal Medical Association · 2020Article
- The Clinical Value ofFrontiers in neurology · 2020Article
- Autoimmune Encephalitis in Latin America: A Critical Review.Frontiers in neurology · 2020Review
- Brain Magnetic Resonance Imaging Characteristics of Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis and Their Clinical Relevance: A Single-Center Study in China.Frontiers in neurology · 2020Article
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundCreutzfeldt-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.
objectiveIn 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.
methodsWe 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 Clínicas, Sao Paulo University, Sao Paulo, Brazil and Hospital Geral de Fortaleza, Fortaleza, Brazil between January 1, 2011 and December 31, 2015.
resultsBoth 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.
conclusionFBDSs 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.
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