ENCEFALITE LIMBICA PDF

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. 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. None of them presented with tumor and both showed a good response after immunotherapy.

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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. 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.

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.

Keywords: Creutzfeldt-Jakob disease; anti-LGI1 encephalitis; autoimmune encephalitis; faciobrachial dystonic seizures; rapidly progressive dementias. Disclosure: The authors report no conflicts of interest.

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Conflict of interest statement Disclosure: The authors report no conflicts of interest. The ipsilateral face grimacing and arm posturing are visible in both cases. Videos are available in the online supplementary data, with the patients' consent. Figure 2 5 Brain MRI case 1 showing….

See this image and copyright information in PMC. Similar articles Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Irani SR, et al. Epub Sep 6. Clinical features of limbic encephalitis with LGI1 antibody.

Wang M, et al. Neuropsychiatr Dis Treat. Yang J, et al. Front Neurol. Three cases of antibody-LGI1 limbic encephalitis and review of literature. Bing-Lei W, et al. Int J Neurosci. Epub Mar 1. PMID: Review. Antibody-LGI 1 autoimmune encephalitis manifesting as rapidly progressive dementia and hyponatremia: a case report and literature review. Li X, et al.

BMC Neurol. Show more similar articles See all similar articles. Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol. Clinical trials for prion disease: difficult challenges, but hope for the future. Lancet Neurol. Rapidly progressive dementia: causes found in a Greek tertiary referral center in Athens.

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The Importance of Recognizing Faciobrachial Dystonic Seizures in Rapidly Progressive Dementias

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Autoimmune encephalitis , also known as autoimmune limbic encephalitis , is an antibody-mediated brain inflammatory process, typically involving the limbic system , although all parts of the brain can be involved. Autoimmune encephalitis can be divided broadly into two groups, based on whether or not antibodies are the result of an underlying tumor:. Unfortunately, there is considerable heterogeneity in how the term limbic encephalitis is used. Most authors limit the term to autoimmune limbic encephalitis, including both paraneoplastic and non-paraneoplastic causes. Some, however, include viral encephalitides especially HSV encephalitis under the broad term limbic encephalitis.

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. A rare limbic encephalitis characterized by the presence of autoantibodies against NMDA receptors in serum and cerebrospinal fluid. It may be of paraneoplastic most commonly associated with ovarian teratoma or non-paraneoplastic origin and is life-threatening but potentially treatable. Patients present with acute behavioral change, psychosis, and catatonia, rapidly progressing to seizures, memory deficit, dyskinesias, speech problems, and autonomic and breathing dysregulation.

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.

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