Hippocampal Dentate Gyrus Atrophy Predicts Pattern Separation Impairment in Patients with LGI1 Encephalitis

Hanert, Annika; Rave, Julius; Granert, Oliver; Ziegler, Martin; Pedersen, Anya; Born, Jan; Finke, Carsten; Bartsch, Thorsten

doi:10.1016/j.neuroscience.2018.12.046

Cited by 30 publications

(33 citation statements)

References 70 publications

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“…At present, most patients with anti-LGI1 encephalitis have a relatively good prognosis after immunotherapy. FBDS can be quickly relieved, and most symptoms can be improved; however, cognitive status is slowly improved, and some patients ( 23 ) may have permanent memory impairment. The better understanding will be of great significance for early diagnosis, essentially immunotherapy, and even better prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…The better understanding will be of great significance for early diagnosis, essentially immunotherapy, and even better prognosis. Some studies suggest ( 9 – 11 , 23 ) that effective and long-term immunotherapy should be given to prevent long-term complications, including hippocampal atrophy ( 23 ) and sustained memory impairment ( 13 ). Second-line drugs can be added to the therapy of patients ( 23 , 24 ) who did not respond well to the first-line drugs or had a recurrence, including rituximab, MMF, or cyclophosphamide.…”

Section: Discussionmentioning

confidence: 99%

“…Some studies suggest ( 9 – 11 , 23 ) that effective and long-term immunotherapy should be given to prevent long-term complications, including hippocampal atrophy ( 23 ) and sustained memory impairment ( 13 ). Second-line drugs can be added to the therapy of patients ( 23 , 24 ) who did not respond well to the first-line drugs or had a recurrence, including rituximab, MMF, or cyclophosphamide. Once cognitive impairment is confirmed, patients should receive immunotherapy ( 9 – 11 ) and long-term maintenance therapy to relieve their symptoms ( 23 , 24 ), improve prognosis, and avoid intractable epilepsy and hippocampal atrophy.…”

Section: Discussionmentioning

confidence: 99%

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Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

et al. 2020

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Introduction: Anti-leucine-rich glioma-inactivated 1 antibody (anti-LGI1) encephalitis is one of the most common autoimmune encephalitis. Anti-LGI1 encephalitis presented with subacute or acute onset of cognitive impairment, psychiatric disturbances, faciobrachial dystonic seizures (FBDSs), convulsions, and hyponatremia. The common sequela of anti-LGI1 encephalitis is cognitive disorder, but there are few studies on the recovery of cognitive function after immunotherapy. This study aimed to explore clinical characteristics of cognitive impairment and 1-year outcome in patients with anti-LGI1 encephalitis. Methods: The clinical data and characteristics of cognitive impairment of 21 patients with anti-LGI1 encephalitis from 2016 to 2019 in Nanjing Brain Hospital were analyzed retrospectively. At the time of onset of hospitalization and 1 year after discharge, the cognitive functions in these patients were assessed using two cognitive screening scales—Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment-Basic (MoCA-B). Results: Among the 21 patients, 13 were male and 8 were female, aged 51.10 ± 14.69 (age range 20–72) years. Nineteen patients, comprising 90.48%, had recent memory deterioration. Routine electroencephalography (EEG) results of 13 cases were abnormal. EEG results were epileptic or slow-wave activity involving the temporal lobes. Eleven cases of brain MRI were abnormal, and the focus involved the hippocampus and mediotemporal lobe. The decrease of short-term memory [recall scores: 0.57 ± 0.81 (MMSE), 0.76 ± 1.34 (MoCA-B)] is the most obvious at the time of admission. After intravenous (IV) injection of methylprednisolone and/or immunoglobulin, the clinical symptoms of the patients improved obviously. Total MMSE and MoCA-B scores of patients were significant increased after 1 year (21.19 ± 3.54 vs. 26.10 ± 3.02, P < 0.001; and 19.00 ± 4.38 vs. 25.19 ± 4.25, P < 0.001, respectively). Recall scores and orientation scores of MoCA-B were significantly improved after 1 year (0.76 ± 1.34 vs. 3.24 ± 1.48, P < 0.001; and 3.10 ± 1.26 vs. 5.00 ± 1.22, P < 0.001, respectively). However, 3/21 (14.29%) patients still have obvious short-term memory impairment (recall scores ≤ 1). Conclusion: Cognitive impairment is one of the most common manifestations of anti-LGI1 encephalitis, with the main prominent being acute or subacute short-term memory loss. Although most patients with anti-LGI1 encephalitis respond well to immunotherapy, a small number of patients still have cognitive disorders, mainly recent memory impairment, after 1 year.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

et al. 2020

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“…Hippocampal atrophy with further reduced mediodorsal thalamic and posteromedial cortical volumes were reported in the limbic encephalitis associated with antibodies to components of the voltage-gated potassium channel complex (VGKCC-Ab-LE), where LGI1 was the prominent autoantibody (Loane et al, 2019). Another recent study showed that hippocampal dentate gyrus atrophy predicted pattern separation impairment in patients with LGI1 encephalitis (Hanert et al, 2019). Besides, diffusion tensor imaging (DTI) has been extensively applied to unveil white matter abnormalities in diverse neurological diseases (Gatto et al, 2018;Gatto and Weissmann, 2019).…”

Section: Introductionmentioning

confidence: 99%

Functional and Structural Brain Alterations in Encephalitis With LGI1 Antibodies

Qiao

Wang

et al. 2020

Front. Neurosci.

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The purpose of this study was to examine the neural substrates and mechanisms that generate memory deficits, seizures and neuropsychiatric abnormalities in encephalitis with LGI1 antibodies using a data-driven, multimodal magnetic resonance imaging (MRI) approach. Methods: Functional MRI data were acquired from 14 anti-LGI1 encephalitis patients and 14 age and gender matched normal controls. Independent component analysis with hierarchical partner matching (HPM-ICA) was used to assess the whole-brain intrinsic functional connectivity. Granger causality (GC) was applied to investigate the effective connectivity among the brain regions that identified by HPM-ICA. Diffusion tensor imaging (DTI) was utilized to investigate white matter microstructural changes of the patients. Results: Participants with LGI1 antibodies encephalitis presented reduced functional connectivity in the brain areas associated with memory, cognition and motion circuits, while increased functional connectivity in putamen and caudate in comparison to the normal controls. Moreover, the effective connectivity in patients was decreased from the frontal cortex to supplementary motor area. Finally, patients had significant reductions in fractional anisotropy (FA) for the corpus callosum, internal capsule, corona radiata and superior longitudinal fasciculus, accompanied by increases in mean diffusivity (MD) for these regions as compared to controls. Conclusion: Our findings suggest that the neural disorder and behavioral deficits of anti-LGI1 encephalitis may be associated with extensive changes in brain connectivity and microstructure. These pathological alterations affect the basal ganglia and limbic system besides the temporal and frontal lobe.

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“…In this report, the patient showed no obvious abnormalities in MRI scans. Which lacks the characteristic imaging presentations in hippocampus [20], striatum [21] and mesial temporal lobes [22]. However, EEG based studies showed hippocampal functional dynamics changes beyond structural damage [19,23].…”

Section: Case Presentationmentioning

confidence: 99%

Neuro-psycho disorders and Hyponatremiain a Renal Carcinoma patient revealed as Anti-LGI1 Encephalitis and Renal Salt Wasting: a Case Report

Lin

Maesaka

et al. 2019

Preprint

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Backgroud: LGI1(leucine-richanti-glioma1protein) is one of the latest identified voltage-gated potassium channels related antigens. Anti-LGI1 encephalitis affected the limbic systems presenting with subacute onset of progressive neurological, cognitive, psychiatric disturbance and life threatening obstinate hyponatremia. However, Anti-LGI1 encephalitis was mostly considered to be non-malignancy related. The hyponatremia mechanism remains to be elusive. Case presentation:Here we firstly report a 71-year-old female diagnosed of anti-LGI1 encephalitis after renal clear cell carcinoma resection. The old female discovered a renal mass which was identified as renal clear cell carcinoma. Two months after the renal carcinoma resection, the patient fell down frequently and showed discontinuous neuro-psychiatric disorders manifested as memory decline, disorientation, and unintentional upper limb movements as typical faciobrachial dystonic seizure episode. She also had hyponatremia. The combination of her clinical presentations and laboratory assessments supported a diagnosis ofanti-LGI1 encephalitis and renal salt wasting. The Immunohistochemistry studies of the kidney resection indicated circulating LGI1 antibodies in sera might be binding to extracellular LGI1 predominantly in the proximal tubule where the major defect in solute transport exists in renal salt wasting. Conclusion: Anti-LGI1 encephalities with renal carcinoma indicated its paralimbic pathology origin. The early diagnosis and immune-modulation therapy could lead to good outcome. The hyponatremia in anti-LGI1 encephalitis was renal salt wasting instead of syndrome of inappropriate secretion of antidiuretic hormone.

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Hippocampal Dentate Gyrus Atrophy Predicts Pattern Separation Impairment in Patients with LGI1 Encephalitis

Cited by 30 publications

References 70 publications

Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

Clinical Characteristics of Cognitive Impairment and 1-Year Outcome in Patients With Anti-LGI1 Antibody Encephalitis

Functional and Structural Brain Alterations in Encephalitis With LGI1 Antibodies

Neuro-psycho disorders and Hyponatremiain a Renal Carcinoma patient revealed as Anti-LGI1 Encephalitis and Renal Salt Wasting: a Case Report

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