Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis

Irani, Sarosh R.; Michell, Andrew W.; Lang, Bethan; Pettingill, Philippa; Waters, Patrick; Johnson, Michael R.; Schott, Jonathan M.; Armstrong, Richard J. E.; Zagami, Alessandro S; Bleasel, Andrew; Somerville, Ernest; Smith, Shubulade; Vincent, Angela

doi:10.1002/ana.22307

Cited by 799 publications

(914 citation statements)

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“…Two patients had antibodies targeting CASPR2. It is well known that FBDS were reported to precede LGI1 antibody‐associated encephalitis, suggesting that early immunotherapy could prevent the evolution to limbic encephalitis 12, 25. We identified similar FBDS in 6 (28.57%) of 21 LGI1‐seropositive patients in this cohort, often accompanied by other seizure semiologies including SPS and CPS.…”

Section: Discussionsupporting

confidence: 58%

“…Faciobrachial dystonic seizures (FBDS) are the most characteristic seizures seen in VGKC complex antibody encephalitis. These seizures are manifested by sudden onset of flexion contraction of one upper extremity, accompanied by contraction of the ipsilateral face, head, and neck 12. These seizures, which typically last for approximately 1 sec, and occur multiple times per day, are present in approximately one fourth of VGKC complex antibody epilepsy patients 11.…”

Section: Introductionmentioning

confidence: 99%

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Seizure semiology: an important clinical clue to the diagnosis of autoimmune epilepsy

Ren

Guan

et al. 2018

Ann Clin Transl Neurol

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ObjectiveThe purpose of this study is to analyze the seizure semiologic characteristics of patients with autoimmune epilepsy (AE) and describe the investigation characteristics of AE using a larger sample size.MethodsThis observational retrospective case series study was conducted from a tertiary epilepsy center between May 2014 and March 2017. Cases of new‐onset seizures were selected based on laboratory evidence of autoimmunity. At the same time, typical mesial temporal lobe epilepsy (MTLE) patients with hippocampal sclerosis (HS) were recruited as the control group from the subjects who underwent presurgical evaluation during the same period.ResultsA total of 61 patients with AE were identified. Specific autoimmune antibodies were detected in 39 patients (63.93%), including anti‐VGKC in 23 patients (37.70%), anti‐NMDA‐R in 9 patients (14.75%), anti‐GABAB‐R in 6 patients (9.84%), and anti‐amphiphysin in 1 patient (1.64%). Regarding the seizure semiology, no significant differences were noted between AE patients with autoantibody and patients with suspected AE without antibody. Compared to typical MTLE patients with HS, both AE patients with autoantibody and patients with suspected AE without antibody had the same seizure semiologic characteristics, including more frequent SPS or CPS, shorter seizure duration, rare postictal confusion, and common sleeping SGTC seizures.SignificanceThis study highlights important seizure semiologic characteristics of AE. Patients with autoimmune epilepsy had special seizure semiologic characteristics. For patients with autoimmune epilepsy presenting with new‐onset seizures in isolation or with a seizure‐predominant neurological disorder, the special seizure semiologic characteristics may remind us to test neuronal nuclear/cytoplasmic antibodies early and initiate immunomodulatory therapies as soon as possible. Furthermore, the absence of neural‐specific autoantibodies does not rule out AE.

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Section: Discussionsupporting

confidence: 58%

Section: Introductionmentioning

confidence: 99%

Seizure semiology: an important clinical clue to the diagnosis of autoimmune epilepsy

Ren

Guan

et al. 2018

Ann Clin Transl Neurol

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“…Patients with LGI1 antibodies often have a novel seizure type, faciobrachial dystonic seizures, during or even preceding the limbic encephalitis [52,53]. Prompt recognition of these seizures and immunomodulatory treatment may prevent progression to limbic encephalitis [53].…”

Section: Antibodies To Identified Cns Proteinsmentioning

confidence: 99%

Neuroinflammation: Ways in Which the Immune System Affects the Brain

et al. 2015

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Neuroinflammation is the response of the central nervous system (CNS) to disturbed homeostasis and typifies all neurological diseases. The main reactive components of the CNS include microglial cells and infiltrating myeloid cells, astrocytes, oligodendrocytes, and the blood-brain b a r r i e r , c y t o k i n e s , a n d c y t o k i n e s i g n a l i n g . Neuroinflammatory responses may be helpful or harmful, as mechanisms associated with neuroinflammation are involved in normal brain development, as well as in neuropathological processes. This review examines the roles of various cell types that contribute to the immune dysregulation associated with neuroinflammation. Microglia enter the CNS very early in embryonic development and, as such, play an essential role in both the healthy and diseased brain. B-cell diversity contributes to CNS disease through both antibody-dependent and antibody-independent mechanisms. The influences of these B-cell mechanisms on other cell types, including myeloid cells and T cells, are reviewed in relationship to antibody-mediated CNS disorders, paraneoplastic neurological diseases, and multiple sclerosis. New insights into neuroinflammation offer exciting opportunities to investigate potential therapeutic targets for debilitating CNS diseases.

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“…Structural magnetic resonance imaging (MRI) showed extratemporal T2 hyperintensities in the early phase with faciobrachial (dys)tonic seizures (FBDS) and reduced volume of the whole brain one year after FBDS [6][7][8][9][10]. Glucose metabolism is also abnormal in areas outside of the limbic structures [8,[11][12][13]. Although these data point to global structural and metabolic alterations, no study examined metabolic changes several years after LGI1 encephalitis.…”

Section: Introductionmentioning

confidence: 99%

Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

Szőts

Blaabjerg

Orsi

et al. 2017

Journal of the Neurological Sciences

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Background: Chronic cognitive deficits are frequent in leucin-rich glioma-inactivated 1 protein (LGI1) encephalitis. We examined structural and metabolic brain abnormalities following LGI1 encephalitis and correlated findings with acute and follow-up clinical outcomes. Methods:Nine patients underwent prospective multimodal 3 Tesla MRI 33.1±18 months after disease onset, including automated volumetry, diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS). Data were compared to 9 age-and sex-matched healthy controls. Conclusions:Poor clinical outcome following LGI1 encephalitis is associated with global brain atrophy and disintegration of white matter tracts. The pathological changes affect not only temporomesial structures but also frontal lobes and the cerebellum.

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Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis

Abstract: Recognition of FBDS should prompt testing for VGKC-complex/Lgi1 antibodies. AEDs often produce adverse effects; treatment with immunotherapies may prevent the development of LE with its potential for cerebral atrophy and cognitive impairment.

Cited by 799 publications

References 32 publications

Seizure semiology: an important clinical clue to the diagnosis of autoimmune epilepsy

Seizure semiology: an important clinical clue to the diagnosis of autoimmune epilepsy

Neuroinflammation: Ways in Which the Immune System Affects the Brain

Global brain atrophy and metabolic dysfunction in LGI1 encephalitis: A prospective multimodal MRI study

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