Residual symptoms and long-term outcomes after all-cause autoimmune encephalitis in adults

Abboud, Hesham; Briggs, Farren; Buerki, Robin; Elkasaby, Mohamed; Baca-Vaca, Guadalupe Fernandez; Fotedar, Neel; Geiger, Christopher D.; Griggins, Cynthia; Lee, Catherine; Lewis, Alexander; Serra, Alessandro; Shrestha, Rajeet; Winegardner, Jill; Shaikh, Aasef G.

doi:10.1016/j.jns.2021.120124

Cited by 13 publications

(23 citation statements)

References 18 publications

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“…According to the long‐term evaluation, residual neurological symptoms (i.e., mild to moderate cognitive impairment) have been described in the affected women. This data is in line with previous studies focusing on long‐term consequences of AE in general population 57 …”

Section: Discussionsupporting

confidence: 93%

“…This data is in line with previous studies focusing on long-term consequences of AE in general population. 57 We did not identify any risk factor associated with increased fetal or maternal mortality. Neither clinical features (i.e., type of autoantibody, severity of neurological/ neuropsychiatric symptoms, diagnosis of malignancy, status epilepticus) nor specific diagnostic procedures (i.e., CT or MRI scans with or without contrast) and treatments (i.e., ASMs, APT, or immunosuppressant drugs) were associated with worsen fetal or maternal clinical outcomes.…”

Section: Rituximab and Ciclophosphamidementioning

confidence: 73%

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Autoimmune encephalitis during pregnancy: A diagnostic and therapeutic challenge—A systematic review with individual patients' analysis and clinical recommendations

et al. 2023

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SUMMARYSeveral reports have described the autoimmune encephalitis’ (AE) possible onset during pregnancy. In this systematic review, we summarize the available data on the diagnostic and therapeutic approach to AE during pregnancy, highlighting the associated maternal and fetal clinical outcomes. A systematic search of the literature was performed. The following databases were used: Pubmed, Google Scholar, EMBASE, CrossRef. The revision was registered on the PROSPERO platform (CRD42022336357). Forty‐nine patients were included. AE onset was mainly observed during the first and the second trimester of pregnancy with psychiatric manifestations and seizures as main onset symptoms. CSF analysis showed AE‐specific autoantibody positivity in 33 patients (anti‐NMDA receptor as the most frequent). EEG generally showed normal findings. MRI revealed pathological findings in less than half of patients. Tumor screening was positive findings in 14 cases. First line immunotherapy (single or combined) was generally employed while second line was administered in a minority of patients. Levetiracetam was the most used antiseizure medication. Cesarean section was performed in 18 women. Most of the women had an excellent early outcome after delivery but 22 showed persistent neurological deficits in long‐term follow‐up. Fetal outcome was positive in 33 cases whereas 12 cases of fetal death were reported. A logistic regression showed that no variable significantly influenced the odds of good/bad maternal and fetal clinical outcome. Diagnosis and treatment of AE during pregnancy is challenging. The rate of miscarriage in women with AE seems to be higher than the general population. In addition, mothers may show long‐term neurological deficits.

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Section: Discussionsupporting

confidence: 93%

Section: Rituximab and Ciclophosphamidementioning

confidence: 73%

Autoimmune encephalitis during pregnancy: A diagnostic and therapeutic challenge—A systematic review with individual patients' analysis and clinical recommendations

et al. 2023

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“…First, it has become increasingly clear that cognitive deficits represent a key clinical outcome of LGI1-E. 6,9,18,21,[40][41][42][43][44] On the one hand, recent evidence suggests that cognitive impairment in LGI1-E is not limited to memory deficits 9 , which are frequently observed in limbic encephalitis due to affliction of the medial temporal lobe. Instead, patients with LGI1-E commonly show multi-domain deficits beyond memory impairment [18][19][20] , involving executive functions, language skills, psychomotor speed, and attentional capacities -cognitive domains not classically attributed to the limbic system.…”

Section: Cognitive Outcomes Of Anti-lgi1 Encephalitismentioning

confidence: 99%

Persistent cognitive deficits in anti-LGI1 encephalitis are linked to a reorganization of structural brain networks

Krohn,

Müller-Jensen,

Kuchling

et al. 2024

Preprint

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Importance: Despite immunotherapy, most patients with anti-leucine-rich, glioma-inactivated 1 encephalitis (LGI1-E) develop long-term cognitive deficits that persist for years after peak illness. However, the structural brain changes that underlie these deficits remain poorly understood.Objective: To study the relationship between cognitive outcomes and white matter (WM) networks in LGI1-E.Design & Setting: Cross-sectional study. German university center.Participants: 25 patients with LGI1-E (19/25 male [76%], mean age: 63 ± 12 years) and 25 age- and sex-matched healthy controls (HC), recruited between January 2013 and April 2019.Main Outcomes and Measures: Clinical assessments including the modified Rankin Scale (mRS) and Clinical Assessment Scale in Autoimmune Encephalitis (CASE); comprehensive cognitive testing; WM tractography using diffusion-weighted MRI.Results: All patients had received first-line immunotherapy, and two-thirds underwent second-line immunotherapy. Patients showed a significant reduction in mRS scores from peak illness to post-acute follow-up (z = -3.8, p < 0.001, n = 20), with 85% presenting "good" functional outcomes (post-acute mRS ≤ 2), paralleled by a significant reduction in CASE scores (z = -3.5, p < 0.001, n = 20). Despite this overall improvement, however, cognitive symptoms were highly prevalent at peak illness (95% of patients affected) and strongly persisted into the post-acute disease stage (85% affected). Neuroimaging at post-acute follow-up (median: 12 months from onset) revealed that LGI1-E is characterized by (i) significantly reduced whole-brain structural connectivity (t = -2.16, p = 0.036, d = -0.61), (ii) a cortico-subcortical hypoconnectivity cluster that strongly affects the hippocampus but also severely impacts extra-limbic brain systems, (iii) systematic limbic and extra-limbic decreases in node degree — a graph-theoretical measure of overall connectedness, and (iv) a "topological reorganization" of structural brain networks, marked by a bidirectional shift in the relative importance of individual brain regions in the network. Importantly, the extent of this network reorganization was significantly related to persistent cognitive deficits in the domains of verbal memory (r = -0.57, p = 0.007, n = 21), attention (r = -0.47, p = 0.030, n = 21), and executive functions (r = -0.60, p = 0.010, n = 17).Conclusion and Relevance: This study characterizes LGI1-E as a network disease that affects both limbic and extra-limbic brain systems and shows that a reorganization of WM networks is linked to multi-domain cognitive deficits in the post-acute disease stage — despite immunotherapy and good overall recovery. These findings highlight the need for extended treatment strategies to improve long-term cognitive outcomes and propose a sensitive new neuroimaging marker to include in prospective clinical trials.

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“…[8,10] Regarding sequelae in AE, various symptoms persist for many years, including cognitive impairment, mood dysfunction, seizures, sleep disturbances, fatigue, and behavioral impairment. [3,6,9] A study evaluated sequelae in 11 preset categories and revealed a higher frequency of cognitive dysfunction and mood dysfunction than other types of sequelae. [9] Regarding psychiatric symptoms, contrary to the common occurrence of behavioral and personality changes at disease onset, psychosis resolved after the acute stage in most patients; however, many patients had residual mood and behavioral symptoms.…”

Section: Tablementioning

confidence: 99%

“…[2][3][4][5] Contrary to substantial recovery in motor functions after AE, persistent impairments in mental or social aspects over years after disease onset, which potentially hinder patients from returning to their previous social activity, [6][7][8] have been recently highlighted. [1,3,8,9] With these findings, researchers argue that the mRS is insufficient to assess potential negative outcomes of AE, including cognitive, behavioral, phycological, or health-related quality of life (HRQOL) outcomes. [8,10] HRQOL has been commonly used as a primary or secondary endpoint in clinical trials and an important patient-oriented outcome in clinical practice guidelines for various neurological disorders.…”

Section: Introductionmentioning

confidence: 99%

Long-term outcomes and health-related quality of life in patients with autoimmune encephalitis: An observational study

Yokota,

Hirose,

Hara

et al. 2023

Medicine

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Autoimmune encephalitis (AE) subacutely causes severe and multiple symptoms; however, most patients achieve neurologically favorable outcomes. Despite the substantial recovery in motor function, persistent impairments in mental/social aspects lasting for several years have been recognized, and its potential effect on health-related quality of life (HRQOL) has been argued. To urgently evaluate the long-term effects of AE on patients’ HRQOL, we investigated patient-oriented long-term outcomes and assessed the HRQOL of patients with AE. Data of patients who were diagnosed with probable/definite AE, defined by Graus AE criteria 2016, and treated at our hospital between January 2011 and October 2020 were retrospectively retrieved. Their long-term (≥2 years) outcomes, which included various sequelae and handicaps in social activities such as returning to previous work/school life through structured interview forms, were evaluated, and the HRQOL was assessed using Neuro-QOL battery. We identified 32 patients who met the Graus AE criteria 2016 and eventually enrolled 21 patients in the study. The median interval between disease onset and survey period was 63 (25–156) months, and 43% of the patients had persistent neuropsychiatric symptoms, including memory disorders, personality changes, and seizures. No more than 71% returned to their previous work/school life. Although most of the patients had global QOL within normal limits, 48% had social QOL under normal limits. Patients with sequelae were significantly less likely to return to previous work/school and had worse global/social quality of life than patients without sequelae. In conclusion, nearly half of patients with AE had social QOL under normal limits 5 years after onset. The difficulty in returning to work/school and a worse HRQOL were notable in patients with sequelae.

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Residual symptoms and long-term outcomes after all-cause autoimmune encephalitis in adults

Cited by 13 publications

References 18 publications

Autoimmune encephalitis during pregnancy: A diagnostic and therapeutic challenge—A systematic review with individual patients' analysis and clinical recommendations

Autoimmune encephalitis during pregnancy: A diagnostic and therapeutic challenge—A systematic review with individual patients' analysis and clinical recommendations

Persistent cognitive deficits in anti-LGI1 encephalitis are linked to a reorganization of structural brain networks

Long-term outcomes and health-related quality of life in patients with autoimmune encephalitis: An observational study

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