More evidence of a neurocardiac prodrome in anti-LGI1 encephalitis

Nilsson, Anna Christine; Blaabjerg, Morten

doi:10.1016/j.jns.2015.07.015

Cited by 11 publications

(10 citation statements)

References 6 publications

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“…Therefore, caution is needed when making initial diagnoses in middle-aged and elderly patients who present with memory deficit. In addition to the aforementioned typical symptoms, patients with LGI1 antibody encephalitis can also exhibit, among other symptoms, sleep or autonomic disorders and ataxias [ 11 , 14 , 22 , 23 ]. Although less frequent, these symptoms also occurred in the patients in our study.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and short-term prognosis of LGI1 antibody encephalitis: a retrospective case study

Meng

et al. 2018

BMC Neurol

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BackgroundRecently, most reports of Leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis are from Europe and the US, while the short term outcome and clinical characteristics of Chinese patients are rarely reported,we study the clinical manifestations, laboratory results and brain magnetic resonance images (MRI) of eight patients who were recently diagnosed with LGI1 antibody encephalitis in our hospital to improve the awareness and knowledge of this disease.MethodsEight patients (five males and three females; mean age, 63.4) with LGI1 antibody encephalitis who were diagnosed and treated in the Department of Neurology of Shengjing Hospital of China Medical University from September 2016 to June 2017 were recruited for the current study. Their general information, clinical manifestations, treatment regimens, and short-term prognoses were retrospectively analyzed, as were the results from MRI and laboratory findings.ResultsOverall, patient symptoms included cognitive impairment, which manifested primarily as memory deficits (8/8), seizures (including faciobrachial dystonic seizure, (FBDS)) (8/8), psychiatric and behavioral disorders (7/8), sleep disorders (4/8), and autonomic abnormalities (3/8). Five patients also had abnormal findings on brain MRI, mainly involving the hippocampus, basal ganglia and insula. Hyponatremia occurred in six cases. All patients tested positive for LGI1 antibodies in their serum/cerebrospinal fluid (CSF)and patients were negative for tumors. Symptoms rapidly improved after treatment with immunoglobulin and/or steroid therapy. The patients were followed up for 4–13 months after discharge, and two patients relapsed.ConclusionPrimary symptoms of LGI1 antibody encephalitis include memory impairments, seizures, FBDS, and mental and behavioral abnormalities. Increased titers of LGI1 antibodies are also present in the serum/CSF of patients. Patients often have hyponatremia, and MRIs show abnormalities in various brain regions. Finally, immunotherapy shows good efficacy and positive benefits, although patients may relapse in the short-term.

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Section: Discussionmentioning

confidence: 99%

Clinical characteristics and short-term prognosis of LGI1 antibody encephalitis: a retrospective case study

Meng

et al. 2018

BMC Neurol

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“…Moreover, 25% had features of autonomic dysfunction including bradycardia. We hypothesize that this might have been due to inflammatory changes of the insular cortex .…”

Section: Discussionmentioning

confidence: 99%

Autoimmune encephalitis associated with voltage‐gated potassium channels‐complex and leucine‐rich glioma‐inactivated 1 antibodies – a national cohort study

Celicanin

Blaabjerg

Mærsk-Møller

et al. 2017

Euro J of Neurology

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“…Syncopes have been previously described in association with limbic encephalitis, mostly of the anti-NMDA, but also GABAB-receptor type [8–12]. We have found 4 published cases with syncope [13,14] and 2 with bradycardia [3] caused by anti-LGI1 limbic encephalitis. The predominant mechanism has been identified as sinus arrest preceded by sinus dysrhythmia/bradycardia [10,12].…”

Section: Discussionmentioning

confidence: 92%

“…The GABA-B-receptors also have been found in the NTS and vagal nuclei [10]. LGI1 protein is known to be present throughout the neocortex, such as in the temporal lobe, the insular region, the hippocampus, and the hypothalamus [7,13,14,16]. These structures play an important role in central regulation of autonomic function [15].…”

Section: Discussionmentioning

confidence: 99%

Syncope and Twitching at the Emergency Department

et al. 2019

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Patient: Female, 49 Final Diagnosis: Anti-LGI1 limbic encephalitis Symptoms: Seizure • sinus arrest Medication: — Clinical Procedure: Immunosupression Specialty: Neurology Objective: Rare co-existance of disease or pathology Background: Anti-LGI1 encephalitis is a type of autoimmune limbic encephalitis. This case report elucidates features of anti-LGI1 limbic encephalitis, focusing on clinical findings and outcome as well as on rarely reported sinus arrest and its pathophysiology. Case Report: A 49-year-old female patient presented at the Emergency Department (ED) because of twitching and an acute confusional state. Initial neurological examination revealed cognitive disturbance with disorientation, inattention, and amnestic deficits. While in the ED, twitching of the right arm was observed and shortly thereafter she experienced a sinus arrest. A temporary cardiac pacemaker was implanted. Laboratory investigations demonstrated mild hyponatremia and positive LGI1-IgG antibodies in serum. An initial head MRI was unremarkable and electroencephalography showed epileptic seizure activity starting focally in the left hemisphere synchronous with the intermittent twitching of the right arm. A seizure-suppressant therapy with levetiracetam, valproate, and gabapentin was initiated, as well as an immunosuppressive pulse therapy with methylprednisolone followed by a tapering oral regimen of prednisolone. Within a few days, the seizures ceased. One month later, neurocognitive test results were back to normal. At 2 years, mild depressive symptoms and anxiety disorder were the main clinical problems, as well as episodic migraine-like headaches. Conclusions: Repetitive focal dystonic seizures, confusion, amnestic deficits, sinus arrest, and mild-to-moderate hyponatremia are pathognomonic features of anti-LGI1 limbic encephalitis. Sinus arrest may occur because of a direct pathophysiological dysfunction of the structures involved in autonomic cardiac rhythm control or as an ictal or postictal phenomenon. Early diagnosis and initiation of immunosuppressive therapy are both of utmost importance for favorable clinical outcome.

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More evidence of a neurocardiac prodrome in anti-LGI1 encephalitis

Cited by 11 publications

References 6 publications

Clinical characteristics and short-term prognosis of LGI1 antibody encephalitis: a retrospective case study

Clinical characteristics and short-term prognosis of LGI1 antibody encephalitis: a retrospective case study

Autoimmune encephalitis associated with voltage‐gated potassium channels‐complex and leucine‐rich glioma‐inactivated 1 antibodies – a national cohort study

Syncope and Twitching at the Emergency Department

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