Pathognomonic seizures in limbic encephalitis associated with anti-LGI1 antibodies

Sen, Arjune; Wang, Justine; Laue-Gizzi, Hanka; Lee, Teresa; Ghougassian, Daniel F.; Somerville, Ernest

doi:10.1016/s0140-6736(14)60684-x

Cited by 26 publications

(16 citation statements)

References 4 publications

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“…We confirmed several pathognomonic features of tonicdystonic seizures previously associated with LGI1-antibody encephalitis (Sen et al, 2014). Motor events are usually of short duration, often triggered by an external stimulus.…”

Section: Discussionsupporting

confidence: 85%

“…This latter possibility differs from classical cortical ictal spread where firing propagates to adjacent areas continuously. EEG abnormalities specifically linked to TDS have not been reported previously (Boesebeck et al, 2013;Sen et al, 2014) although variable EEG activities related to cortical seizures have been described (Irani et al, , 2013Shin et al, 2013). We thus identified a novel EEG marker for isolated TDS.…”

Section: Discussionmentioning

confidence: 69%

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Motor cortex and hippocampus are the two main cortical targets in LGI1-antibody encephalitis

Navarro¹,

Kas²,

Apartis³

et al. 2016

Brain

169

161

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Ã These authors contributed equally to this work. Encephalitis associated with antibodies against leucine-rich glioma-inactivated 1 (LGI1) protein is increasingly recognized as an auto-immune disorder associated with characteristic tonic-dystonic seizures. The cortical or subcortical origin of these motor events is not clear. Some patients also present with different epileptic seizures and with cognitive impairment. The frequency of these features and their timing during the natural history of this encephalitis have not been fully described. We therefore reviewed data from 34 patients harbouring antibodies against LGI1 protein (21-81 years, median age 64) referred to the French Reference Centre for Neurological Paraneoplastic Syndrome. Three types of evidence suggested tonic-dystonic seizures were of cortical origin: (i) a slow, unilateral, frontal electroencephalographic wave, of duration $580 ms and amplitude $71 mV, preceded the contralateral tonic-dystonic seizures in simultaneous electroencephalographic and myographic records from seven of seven patients tested; (ii) 18-Fluorodeoxyglucose imaging revealed a strong hypermetabolism in primary motor cortex, controlateral to the affected limb, during encephalitis for five patients tested, as compared with data from the same patients after remission or from 16 control subjects; and (iii) features of polymyographic records of tonic-dystonic seizure events pointed to a cortical origin. Myoclonic patterns with brief, rhythmic bursts were present in three of five patients tested and a premyoclonic potential was identified in the cortex of one patient. Initially during encephalitis, 11 of 34 patients exhibited tonic-dystonic seizures (32%). Distinct epileptic syndromes were evident in 13 patients (38%). They were typically simple, focal seizures from the temporal lobe, consisting of vegetative symptoms or fear. At later stages, 22 of 32 patients displayed tonic-dystonic seizures (68%) and 29 patients presented frequent seizures (91%) including status epilepticus. Cognitive impairment, either anterograde amnesia or confusion was evident in 30 of 34 patients (88%). Brain imaging was normal in patients with isolated tonic-dystonic seizures; in patients with limbic symptoms it revealed initially a hippocampal hyperintensity in 8 of 19 patients (42%) and 17 of 24 patients (70%) at later stages. Our data suggest that the major signs of LGI1-antibody encephalitis can be linked to involvement of motor cortex and hippocampus. They occur in parallel with striatum involvement. One of these cortical targets is involved, often unilaterally at disease onset. As the encephalitis progresses, in the absence of immunomodulatory treatment, the second cortical target is affected and effects become bilateral. Progression to the second cortical target occurs with a variable delay of days to several months.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 69%

Motor cortex and hippocampus are the two main cortical targets in LGI1-antibody encephalitis

Navarro¹,

Kas²,

Apartis³

et al. 2016

Brain

169

161

View full text Add to dashboard Cite

show abstract

“…Treatment delays can result in ongoing functional memory problems and other lingering neurological deficits (12). FBDSs may be the earliest manifestation of LE associated with the LGI1 antibody, so recognizing this unique type of seizure at once is important and allows treatment to start quickly, when it is most effective (13).…”

Section: Discussionmentioning

confidence: 99%

Anti-leucine-rich glioma-inactivated 1 limbic encephalitis: A case report and literature review

Liu

Li³

et al. 2015

Experimental and Therapeutic Medicine

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Abstract. This study describes the case of a 41-year-old woman admitted for anterograde memory loss, right facial grimacing and right arm posturing that had begun 1 month previously. Cranial magnetic resonance-diffusion weighted imaging and -fluid-attenuated inversion recovery imaging revealed a hyperintense signal in the left hippocampus and right basal ganglia, but no contrast enhancement. An electroencephalogram revealed rhythmic sharp and slow waves and rhythmic θ build-ups in the left temporal area. Single-photon emission computed tomography showed increased regional blood flow perfusion in the left cerebral frontal lobe and the right basal ganglia. The cerebrospinal fluid was normal, with the exception of the presence of leucine-rich glioma-inactivated 1 (LGI1) antibodies, and LGI1 antibodies were also found in the blood serum. The presence of the antibodies, the faciobrachial dystonic seizures (FBDSs) and the memory loss indicated limbic encephalitis. After 3 months of immunotherapy, the patient was free from epileptic seizures and had undergone a partial memory restoration. FBDSs alone justify the immediate initiation of immunotherapy, even prior to laboratory confirmation of the disease, as early treatment limits the duration of the illness.

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“…Les spasmes brachio-faciaux sont quasi pathognomoniques de ce type d'encéphalites [1,[6][7][8][9][10][11]. Ils peuvent précéder l'apparition des troubles cognitifs, être à bascule et résistants aux antiépileptiques [9].…”

Section: Discussionunclassified

Troubles neuropsychiatriques inexpliqués : penser aux encéphalites dysimmunitaires. À propos d’une observation d’encéphalite à anticorps anti-leucine rich glioma inactivated 1 (LGI-1)

Dault

Lagarde

Guedj

et al. 2016

La Revue de Médecine Interne

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In the presence of acute neuropsychiatric disorders with a negative etiologic research, physician should think about dysimmune encephalitis. Facio-brachial dystonic seizures and syndrome of inappropriate antidiuretic hormone secretion are highly evocative of anti-leucine rich glioma inactivated 1 encephalitis. The diagnosis needs specific diagnostic tests (cerebral (18)FDG position emission tomography and antibodies research in cerebro-spinal fluid and in serum), after the exclusion of alternative diagnoses. Extensive and repeated diagnostic work-up for neoplasia is required. Immunosupressive therapies are effective in most cases.

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Pathognomonic seizures in limbic encephalitis associated with anti-LGI1 antibodies

Cited by 26 publications

References 4 publications

Motor cortex and hippocampus are the two main cortical targets in LGI1-antibody encephalitis

Motor cortex and hippocampus are the two main cortical targets in LGI1-antibody encephalitis

Anti-leucine-rich glioma-inactivated 1 limbic encephalitis: A case report and literature review

Troubles neuropsychiatriques inexpliqués : penser aux encéphalites dysimmunitaires. À propos d’une observation d’encéphalite à anticorps anti-leucine rich glioma inactivated 1 (LGI-1)

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