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Soon-tae Lee - One of the best experts on this subject based on the ideXlab platform.
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Tofacitinib treatment for refractory Autoimmune Encephalitis.
Epilepsia, 2021Co-Authors: Yoonhyuk Jang, Sang Kun Lee, Woo Jin Lee, Han Sang Lee, Kon Chu, Soon-tae LeeAbstract:To treat intractable cases of Autoimmune Encephalitis, the need for novel immunotherapy that penetrates the blood-brain barrier (BBB) is increasing. Tofacitinib is a Janus kinase (JAK) inhibitor used to treat refractory immune-mediated diseases that effectively penetrates the BBB. Accordingly, tofacitinib could be a new option for patients with refractory Autoimmune Encephalitis. Patients treated with tofacitinib were selected from Seoul National University Hospital cohort for Autoimmune Encephalitis from April 2019 until July 2020. We retrospectively analyzed the efficacy of tofacitinib in patients with Autoimmune Encephalitis who showed insufficient responses to multimodal conventional immunotherapies. Tofacitinib was administered orally at a dose of 5 mg twice daily. A total of eight patients were treated with tofacitinib; two had good responses (clinical global impression-improvement score [CGI-I] = 1 or 2), three had partial responses (CGI-I = 3), and three showed no significant improvements (CGI-I = 4) in response to tofacitinib. The two good responders showed the improvement of chronic Autoimmune meningoEncephalitis and the cessation of the new-onset refractory status epilepticus in anti-myelin oligodendrocyte glycoprotein (MOG)-associated disorder, which was previously intractable to anesthetics and the other immunotherapies. No patients had serious side effects. Our findings suggest the potential of tofacitinib as a therapeutic option for central nervous system Autoimmune diseases.
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Ca V α2δ Autoimmune Encephalitis: A Novel Antibody and its Characteristics.
Annals of neurology, 2021Co-Authors: Soon-tae Lee, Byoung Joo Lee, Ji-yeon Bae, Young Sook Kim, Dohyun Han, Hyun‐sook Shin, Soyun Kim, Dong-kyu Park, Sang Won Seo, Kon ChuAbstract:OBJECTIVE Discovery of a novel antibody would enable diagnosis and early treatment of Autoimmune Encephalitis. The aim was to discover a novel antibody targeting a synaptic receptor and characterize the pathogenic mechanism. METHOD We screened for unknown antibodies in serum and cerebrospinal fluid samples from Autoimmune Encephalitis patients. Samples with reactivity to rat brain sections and no reactivity to conventional antibody tests underwent further processing for antibody discovery, using immunoprecipitation to primary neuronal cells, mass-spectrometry analysis, an antigen-binding assay on an antigen-overexpressing cell line, and an electrophysiological assay with cultured hippocampal neurons. RESULTS Two patients had a novel antibody against CaV α2δ (voltage-gated calcium channel alpha-2/delta subunit). The patient samples stained neuropils of the hippocampus, basal ganglia, and cortex in rat brain sections and bound to a CaV α2δ-overexpressing cell line. Knockdown of CaV α2δ expression in cultured neurons turned off the immunoreactivity of the antibody from the patients to the neurons. The patients were associated with preceding meningitis or neuroendocrine carcinoma and responded to immunotherapy. In cultured neurons, the antibody reduced neurotransmitter release from presynaptic nerve terminals by interfering with tight coupling of calcium channels and exocytosis. INTERPRETATION Here, we discovered a novel Autoimmune Encephalitis associated with anti-CaV α2δ antibody. Further analysis of the antibody in Autoimmune Encephalitis might promote early diagnosis and treatment. ANN NEUROL 2021;89:740-752.
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HLA‐B27 association of Autoimmune Encephalitis induced by PD‐L1 inhibitor
Annals of clinical and translational neurology, 2020Co-Authors: Hyeyeon Chang, Yong Won Shin, Bhumsuk Keam, Miso Kim, Soon-tae LeeAbstract:OBJECTIVE While immune checkpoint inhibitors are increasingly used for various cancers, unpredictable immune-related adverse events (irAEs) such as Autoimmune Encephalitis is life-threatening. Here, we report an association between human leukocyte antigen (HLA) and atezolizumab-induced Encephalitis. METHODS From an institutional prospective cohort for Encephalitis, we identified patients with Autoimmune Encephalitis after the use of atezolizumab, a PD-L1 (programmed death-ligand 1) inhibitor, from August 2016 to September 2019 and analyzed their HLA genotypes. RESULTS A total of 290 patients received atezolizumab, and seven patients developed Autoimmune Encephalitis, and five of whom were enrolled for the analysis. The patients presented altered mentality, seizures, or myelitis. Three patients had the HLA-B*27:05 genotype in common (60%), which is significantly frequent given its low frequency in the general population (2.5%). After Bonferroni correction, HLA-B*27:05 was significantly associated with Autoimmune Encephalitis by atezolizumab (corrected P
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Posterior reversible encephalopathy syndrome as initial manifestation of Autoimmune Encephalitis.
Neurology. Clinical practice, 2019Co-Authors: Jaeho Kim, Soon-tae Lee, Silvia Park, Eun Yeon Joo, Chin-sang Chung, Mi Ji LeeAbstract:Autoimmune Encephalitis should be considered when patients with posterior reversible encephalopathy syndrome show atypical clinical courses.
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High albumin level is a predictor of favorable response to immunotherapy in Autoimmune Encephalitis
Scientific reports, 2018Co-Authors: Yoonhyuk Jang, Soon-tae Lee, Kon Chu, Kyung Il Park, Tae Joon Kim, Jin Sun Jun, Jangsup Moon, Keun-hwa Jung, Sang Kun LeeAbstract:There is no known biomarker that predicts the response to immune therapy in Autoimmune synaptic Encephalitis. Thus, we investigated serum albumin as a prognostic biomarker of early immune therapies in patients with Autoimmune Encephalitis. We enrolled patients who were diagnosed with definite Autoimmune Encephalitis and underwent IVIg treatment at Seoul National University Hospital from 2012 to 2017. Patients were dichotomized according to serum albumin prior to IVIg administration with a cut-off level of 4.0 g/dL, which was the median value of 50% of patients. Seventeen (53.1%) of the 32 patients with definite Autoimmune Encephalitis who received IVIg treatment in our hospital had low serum albumin (
Eoin P. Flanagan - One of the best experts on this subject based on the ideXlab platform.
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Autoimmune Encephalitis proposed best practice recommendations for diagnosis and acute management
Journal of Neurology Neurosurgery and Psychiatry, 2021Co-Authors: Hesham Abboud, Russell C Dale, John C. Probasco, Sarosh R. Irani, Beau M. Ances, David R. Benavides, Michael J. Bradshaw, Paulo Pereira Christo, Mireya Fernandezfournier, Eoin P. FlanaganAbstract:The objective of this paper is to evaluate available evidence for each step in Autoimmune Encephalitis management and provide expert opinion when evidence is lacking. The paper approaches Autoimmune Encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG Encephalitis and 48.5% for classical paraneoplastic Encephalitis. Half the responders indicated they would add a second-line agent only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in all patients. As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.
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Autoimmune Encephalitis: proposed recommendations for symptomatic and long-term management.
Journal of neurology neurosurgery and psychiatry, 2021Co-Authors: Hesham Abboud, Russell C Dale, John C. Probasco, Sarosh R. Irani, Beau M. Ances, David R. Benavides, Michael J. Bradshaw, Paulo Pereira Christo, Mireya Fernández-fournier, Eoin P. FlanaganAbstract:The objective of this paper is to evaluate available evidence for each step in Autoimmune Encephalitis management and provide expert opinion when evidence is lacking. The paper approaches Autoimmune Encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. The most popular bridging therapy was oral prednisone taper chosen by 38% of responders while rituximab was the most popular maintenance therapy chosen by 46%. Most responders considered maintenance immunosuppression after a second relapse in patients with neuronal surface antibodies (70%) or seronegative Autoimmune Encephalitis (61%) as opposed to those with onconeuronal antibodies (29%). Most responders opted to cancer screening for 4 years in patients with neuronal surface antibodies (49%) or limbic Encephalitis (46%) as opposed to non-limbic seronegative Autoimmune Encephalitis (36%). Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.
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Post-Transplant Autoimmune Encephalitis (P5.382)
Neurology, 2018Co-Authors: Devon A. Cohen, Sebastian Lopez, Andrew Mckeon, Sean J. Pittock, Anastasia Zekeridou, Barry A. Boilson, William J. Hogan, John J. Poterucha, Eoin P. FlanaganAbstract:Objective: To report a case series of post-transplant Autoimmune Encephalitis. Background: Autoimmune Encephalitis is not well-recognized as a cause of Encephalitis in the post-transplant setting. Design/Methods: We retrospectively identified Mayo Clinic patients through our electronic medical record who developed Autoimmune Encephalitis after transplant (solid organ or hematopoietic cell). All patients met diagnostic criteria for Autoimmune Encephalitis (AE). Neural autoantibody (Ab) testing was undertaken in the Mayo Clinic Neuroimmunology laboratory. Results: Six patients were included. The median age was 58 years (range, 40–68) and 4 were women. AE occurred post-organ transplant in 3 (cardiac, 1; liver, 1; pancreatic, 1) and post-allogeneic hematopoietic cell transplantation in 3. The median time from transplant to AE symptom onset was 22 months (range, 1–143). All received immunotherapy prior to symptom onset: tacrolimus, 4; mycophenolate, 3; prednisone, 3; dexamethasone, 2; cyclosporine, 1; cyclophosphamide, 1. Four patients were receiving immunosuppressants at the time of AE symptom onset including: tacrolimus, 4; mycophenolate, 3; and prednisone, 3. All 6 patients had a subacute presentation. Patients met criteria for antibody positive AE, specific disease (cerebrospinal fluid [CSF] NMDA-R Ab, 1; serum & CSF AMPA-R Ab, 1; serum myelin-oligodendrocyte-glycoprotein (MOG) ab, 1) or definite Autoimmune limbic Encephalitis (Ab negative), 3. CSF findings included: pleocytosis, 3; protein elevation, 4; positive IgG index, 1; oligoclonal bands, 1. MRI Brain revealed bilateral mesial temporal lobe T2-signal abnormalities in 5 (83%). EEG revealed epileptiform activity, 2; and slowing, 2. All patients had clinical improvement with additional immunosuppressive therapy including one or more of: intravenous methylprednisolone, 4; rituximab, 3; cyclosporine, 3; mycophenolate, 3; plasma exchange, 2; intravenous immune globulin, 1. Median modified-Rankin-Scale was 2.5 (range, 1–6) at last follow-up (median of 10 months from onset, range 5–47). Conclusions: Autoimmune Encephalitis may follow organ or hematopoietic cell transplant and responds well to additional immunotherapy. Disclosure: Dr. Cohen has nothing to disclose. Dr. Lopez has nothing to disclose. Dr. McKeon has received research support from Medimmune, Euroimmun, Grifols and Alexion. Dr. Pittock has nothing to disclose. Dr. Zekeridou has nothing to disclose. Dr. Boilson has nothing to disclose. Dr. Hogan has nothing to disclose. Dr. Poterucha has nothing to disclose. Dr Flanagan has nothing to disclose.
David R. Benavides - One of the best experts on this subject based on the ideXlab platform.
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Autoimmune Encephalitis: proposed recommendations for symptomatic and long-term management.
Journal of neurology neurosurgery and psychiatry, 2021Co-Authors: Hesham Abboud, Russell C Dale, John C. Probasco, Sarosh R. Irani, Beau M. Ances, David R. Benavides, Michael J. Bradshaw, Paulo Pereira Christo, Mireya Fernández-fournier, Eoin P. FlanaganAbstract:The objective of this paper is to evaluate available evidence for each step in Autoimmune Encephalitis management and provide expert opinion when evidence is lacking. The paper approaches Autoimmune Encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. The most popular bridging therapy was oral prednisone taper chosen by 38% of responders while rituximab was the most popular maintenance therapy chosen by 46%. Most responders considered maintenance immunosuppression after a second relapse in patients with neuronal surface antibodies (70%) or seronegative Autoimmune Encephalitis (61%) as opposed to those with onconeuronal antibodies (29%). Most responders opted to cancer screening for 4 years in patients with neuronal surface antibodies (49%) or limbic Encephalitis (46%) as opposed to non-limbic seronegative Autoimmune Encephalitis (36%). Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.
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Autoimmune Encephalitis proposed best practice recommendations for diagnosis and acute management
Journal of Neurology Neurosurgery and Psychiatry, 2021Co-Authors: Hesham Abboud, Russell C Dale, John C. Probasco, Sarosh R. Irani, Beau M. Ances, David R. Benavides, Michael J. Bradshaw, Paulo Pereira Christo, Mireya Fernandezfournier, Eoin P. FlanaganAbstract:The objective of this paper is to evaluate available evidence for each step in Autoimmune Encephalitis management and provide expert opinion when evidence is lacking. The paper approaches Autoimmune Encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG Encephalitis and 48.5% for classical paraneoplastic Encephalitis. Half the responders indicated they would add a second-line agent only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in all patients. As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.
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association of Autoimmune Encephalitis with combined immune checkpoint inhibitor treatment for metastatic cancer
JAMA Neurology, 2016Co-Authors: Tanya J Williams, Josep Dalmau, John C. Probasco, David R. Benavides, Kellyann Patrice, Alexandre Avila, Evan J Lipson, Ellen M MowryAbstract:Importance Paraneoplastic encephalitides usually precede a diagnosis of cancer and are often refractory to immunosuppressive therapy. Conversely, Autoimmune encephalitides are reversible conditions that can occur in the presence or absence of cancer. Objective To report the induction of Autoimmune Encephalitis in 2 patients after treatment of metastatic cancer with a combination of the immune checkpoint inhibitors nivolumab and ipilimumab. Design, Setting, and Participants A retrospective case study was conducted of the clinical and management course of 2 patients with progressive, treatment-refractory metastatic cancer who were treated with a single dose each (concomitantly) of the immune checkpoint inhibitors nivolumab, 1 mg/kg, and ipilimumab, 3 mg/kg. Exposures Nivolumab and ipilimumab. Main Outcomes and Measures The clinical response to immunosuppressive therapy in suspected Autoimmune Encephalitis in the setting of immune checkpoint inhibitor use. Results Autoantibody testing confirmed identification of anti– N -methyl-D-aspartate receptor antibodies in the cerebrospinal fluid of 1 patient. Withdrawal of immune checkpoint inhibitors and initiation of immunosuppressive therapy, consisting of intravenous methylprednisolone sodium succinate equivalent to 1000 mg of methylprednisolone for 5 days, 0.4 mg/kg/d of intravenous immunoglobulin for 5 days, and 2 doses of rituximab, 1000 mg, in 1 patient and oral prednisone, 60 mg/d, in the other patient, resulted in improved neurologic symptoms. Conclusions and Relevance Immune checkpoint inhibition may favor the development of immune responses against neuronal antigens, leading to Autoimmune Encephalitis. Early recognition and treatment of Autoimmune Encephalitis in patients receiving immune checkpoint blockade therapy will likely be essential for maximizing clinical recovery and minimizing the effect of drug-related toxic effects. The mechanisms by which immune checkpoint inhibition may contribute to Autoimmune Encephalitis require further study.
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Autoimmune Encephalitis and Its Relation to Infection
Current Neurology and Neuroscience Reports, 2015Co-Authors: Arun Venkatesan, David R. BenavidesAbstract:Encephalitis, an inflammatory condition of the brain that results in substantial morbidity and mortality, has numerous causes. Over the past decade, it has become increasingly recognized that Autoimmune conditions contribute significantly to the spectrum of Encephalitis causes. Clinical suspicion and early diagnosis of Autoimmune etiologies are of particular importance due to the need for early institution of immune suppressive therapies to improve outcome. Emerging clinical observations suggest that the most commonly recognized cause of antibody-mediated Autoimmune Encephalitis, anti- N -methyl- d -aspartate (NMDA) receptor Encephalitis, may in some cases be triggered by herpes virus infection. Other conditions such as Rasmussen’s Encephalitis (RE) and febrile infection-related epilepsy syndrome (FIRES) have also been posited to be Autoimmune conditions triggered by infectious agents. This review focuses on emerging concepts in central nervous system autoimmunity and addresses clinical and mechanistic findings linking Autoimmune Encephalitis and infections. Particular consideration will be given to anti-NMDA receptor Encephalitis and its relation to herpes simplex Encephalitis.
Josep Dalmau - One of the best experts on this subject based on the ideXlab platform.
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Sleep disorders in Autoimmune Encephalitis
The Lancet. Neurology, 2020Co-Authors: Amaia Muñoz-lopetegi, Josep Dalmau, Francesc Graus, Joan SantamariaAbstract:Sleep disorders in people with Autoimmune Encephalitis have received little attention, probably overshadowed by the presence of other neurological and psychiatric symptoms in this group of conditions. However, sleep disorders are frequent, often severe, and usually persist beyond the acute disease stage, interfering with patients' recovery and quality of life. Because Autoimmune Encephalitis can affect any brain network involved in sleep initiation and regulation, all types of sleep disorders can occur, with varying distinct associations, frequency, and intensity. Anti-IgLON5 and anti-NMDA receptor Encephalitis exemplify two diseases in which sleep disorders are prominent. In anti-IgLON5 disease, sleep disorders were the core symptoms that led to the description of this disease, whereas in anti-NMDA receptor Encephalitis, sleep disorders vary according to the disease stage along with other neuropsychiatric symptoms. Comprehensive, systematic, multicentre studies are needed to characterise sleep disorders and their mechanisms in Autoimmune Encephalitis.
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association of Autoimmune Encephalitis with combined immune checkpoint inhibitor treatment for metastatic cancer
JAMA Neurology, 2016Co-Authors: Tanya J Williams, Josep Dalmau, John C. Probasco, David R. Benavides, Kellyann Patrice, Alexandre Avila, Evan J Lipson, Ellen M MowryAbstract:Importance Paraneoplastic encephalitides usually precede a diagnosis of cancer and are often refractory to immunosuppressive therapy. Conversely, Autoimmune encephalitides are reversible conditions that can occur in the presence or absence of cancer. Objective To report the induction of Autoimmune Encephalitis in 2 patients after treatment of metastatic cancer with a combination of the immune checkpoint inhibitors nivolumab and ipilimumab. Design, Setting, and Participants A retrospective case study was conducted of the clinical and management course of 2 patients with progressive, treatment-refractory metastatic cancer who were treated with a single dose each (concomitantly) of the immune checkpoint inhibitors nivolumab, 1 mg/kg, and ipilimumab, 3 mg/kg. Exposures Nivolumab and ipilimumab. Main Outcomes and Measures The clinical response to immunosuppressive therapy in suspected Autoimmune Encephalitis in the setting of immune checkpoint inhibitor use. Results Autoantibody testing confirmed identification of anti– N -methyl-D-aspartate receptor antibodies in the cerebrospinal fluid of 1 patient. Withdrawal of immune checkpoint inhibitors and initiation of immunosuppressive therapy, consisting of intravenous methylprednisolone sodium succinate equivalent to 1000 mg of methylprednisolone for 5 days, 0.4 mg/kg/d of intravenous immunoglobulin for 5 days, and 2 doses of rituximab, 1000 mg, in 1 patient and oral prednisone, 60 mg/d, in the other patient, resulted in improved neurologic symptoms. Conclusions and Relevance Immune checkpoint inhibition may favor the development of immune responses against neuronal antigens, leading to Autoimmune Encephalitis. Early recognition and treatment of Autoimmune Encephalitis in patients receiving immune checkpoint blockade therapy will likely be essential for maximizing clinical recovery and minimizing the effect of drug-related toxic effects. The mechanisms by which immune checkpoint inhibition may contribute to Autoimmune Encephalitis require further study.
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Autoimmune Encephalitis as differential diagnosis of infectious Encephalitis
Current Opinion in Neurology, 2014Co-Authors: Thaís Armangue, Frank Leypoldt, Josep DalmauAbstract:Purpose of reviewThis review describes the main types of Autoimmune Encephalitis with special emphasis on those associated with antibodies against neuronal cell surface or synaptic proteins, and the differential diagnosis with infectious Encephalitis.Recent findingsThere is a continuous expansion of
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Autoimmune Encephalitis update
Neuro-oncology, 2014Co-Authors: Josep Dalmau, Myrna R. RosenfeldAbstract:Cancer-associated immune-mediated disorders of the central nervous system are a heterogeneous group. These disorders include the classic paraneoplastic neurologic disorders and the more recently described Autoimmune Encephalitis associated with antibodies to neuronal cell-surface or synaptic receptors that occur with and without a cancer association. Autoimmune Encephalitis is increasingly recognized as the cause of a variety of neuropsychiatric syndromes that can be severe and prolonged. In contrast to the classic paraneoplastic disorders that are poorly responsive to tumor treatment and immunotherapy, Autoimmune Encephalitis often responds to these treatments, and patients can have full or marked recoveries. As early treatment speeds recovery, reduces disability, and decreases relapses that can occur in about 20% of cases, it is important that the immune pathogenesis of these disorders is recognized.
Gregory S. Day - One of the best experts on this subject based on the ideXlab platform.
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Sleep Disturbances in Patients with Autoimmune Encephalitis.
Current neurology and neuroscience reports, 2020Co-Authors: Margaret Blattner, Gregory S. DayAbstract:To review sleep complaints reported in patients with Autoimmune Encephalitis, explore the relationship between sleep disturbances and subtypes of Autoimmune Encephalitis, and leverage knowledge concerning antibody-antigen specificity to inform the receptors, structures, and disseminated neural networks that contribute to sleep function in health and disease. Autoimmune Encephalitis is an inflammatory brain disorder characterized by the subacute onset of psychiatric symptoms, cognitive impairment, and focal neurologic deficits or seizures. Sleep disturbances are detected in a majority of patients systematically screened for sleep complaints, may be the presenting symptom in patients with Autoimmune Encephalitis, and may compromise recovery in patients with Autoimmune Encephalitis. Early recognition of specific sleep disturbances in patients with subacute changes in behavior or cognition may support the diagnosis of Autoimmune Encephalitis. Similarly, recognition and treatment of sleep dysfunction in patients with known Autoimmune Encephalitis may speed recovery and improve long-term outcomes.
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Long-Term Cognitive Outcomes in Patients with Autoimmune Encephalitis.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques, 2018Co-Authors: Julien Hébert, Gregory S. Day, Claude Steriade, Richard Wennberg, David F. Tang-waiAbstract:Background: A need exists to characterise the long-term cognitive outcomes in patients who recovered from Autoimmune Encephalitis and to identify the modifiable factors associated with improved outcomes. Methods: We retrospectively analysed data from patients diagnosed with Autoimmune Encephalitis in our outpatient Autoimmune Encephalitis clinic over a 5-year period, where the Montreal Cognitive Assessment (MoCA) is routinely administered. Results: In total, 21 patients met the inclusion criteria, of whom 52% had persistent cognitive impairment at their latest follow-up (median delay to testing=20 months, range 13-182). Visuospatial and executive abilities, language, attention, and delayed recall were predominantly affected. Patients with status epilepticus at presentation had lower total MoCA scores at their last follow-up (median total score 21, range 15-29) compared with patients without status epilepticus at presentation (median total score 27.5, range 21-30; r 2 =0.366, p =0.004). Patients who experienced delays of more than 60 days from symptom onset to initiation of treatment (either immunosuppression or tumour removal) were more likely to have a MoCA score compatible with cognitive impairment at their last follow-up ( r 2 =0.253, p =0.0239; z -score=−2.01, p =0.044). Conclusions: Our study suggests that the MoCA may be used to evaluate cognition in recovering patients with Autoimmune Encephalitis. Delays to treatment shorter than 60 days and absence of status epilepticus at onset were associated with better performance on the MoCA obtained more than 1 year after symptom onset, and may predict better long-term cognitive outcomes.
