• Title/Summary/Keyword: autoimmune encephalitis

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Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum

  • Seo, Joo Hee;Lee, Yun-Jin;Lee, Ki Hyeong;Gireesh, Elakkat;Skinner, Holly;Westerveld, Michael
    • Clinical and Experimental Pediatrics
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    • v.63 no.8
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    • pp.291-300
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    • 2020
  • Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neuron-specific autoantibodies targeting intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis are quite dependent on antibody testing and responses to immunotherapy, which might delay the diagnosis. This form of encephalitis can involve the multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than that in adults, and the incorporation of clinical, immunological, electrophysiological, and neuroradiological results is critical to the diagnostic approach. In this review, we document the clinical and immunologic characteristics of autoimmune encephalitis known to date, with the goal of helping clinicians in differential diagnosis and to provide prompt and effective treatment.

Anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma in Korea: three case reports

  • Lee, Jisun;Kang, Seongwoo;Chang, Hye Jin;Lee, Yong Hee;Son, Joo-Hyuk;Kong, Tae Wook;Chang, Suk-Joon;Hwang, Kyung Joo;Kim, Miran
    • Journal of Yeungnam Medical Science
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    • v.38 no.4
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    • pp.350-355
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    • 2021
  • Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune paraneoplastic syndrome associated with ovarian teratomas. Most patients develop neurologic symptoms, including psychosis, memory deficits, seizures, or abnormal movements, and experience abdominal pain related to ovarian neoplasm. We present a case report of three patients diagnosed with anti-NMDAR encephalitis accompanied by ovarian teratomas at Ajou University Hospital in Korea. The patients demonstrated a different clinical course of the disease. However, upon diagnosis, all patients underwent surgical removal of the ovarian teratoma followed by intensive immunotherapy. The symptoms progressively improved following treatment. This is a case report of a rare autoimmune anti-NMDAR encephalitis associated with ovarian neoplasms, including immature teratoma.

Anti-LGI1 Antibody Encephalitis (양극성장애로 오인된 LGI1 자가면역성뇌염)

  • Kim, Eun Soo;Lee, HeeJun;Jeon, Sang Won;Cho, Sung Joon
    • Korean Journal of Biological Psychiatry
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    • v.27 no.2
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    • pp.112-116
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    • 2020
  • Leucine rich glioma inactivated (LGI1) encephalitis is an uncommon neurological disorder rarely encountered in clinical practice. However, it is a potentially fatal autoimmune syndrome that can decrease the level of consciousness, possibly progressing to coma. Additionally, unless diagnosed and promptly treated, it can cause permanent cognitive impairment. Since LGI1 encephalitis can initially present with psychiatric symptoms, there can be delays in reaching a proper diagnosis. This report describes a case of a 47-year-old woman with LGI1 antibodies-associated limbic encephalitis who initially presented with psychosis. Her blood tests were normal and no MRI and EEG abnormalities were found. Cerebrospinal fluid analysis was negative for other possible infectious causes. Three months after admission, she was found to be LGI1 antibody positive. LGI1 encephalitis should be suspected in patients with symptoms such as memory loss, confusion, seizures, and psychiatric symptoms. Prompt diagnosis and treatment of LGI1 encephalitis are warranted because prognosis becomes worse when such actions are delayed.

A Case Report of Anti-NMDA Receptor Encephalitis with Ovarian Teratoma Improved by Korean Medicine (난소기형종을 동반한 항 NMDA 수용체 뇌염 환자 1례에 대한 한방치료 증례 보고)

  • Irang Nam;Min-hwa Kim;Mariah Kim;Ki-beom Ku;Se-yeon Lee;So-yeon Kim;So-jung Park;Jun-yong Choi;Chang-woo Han;Jin-woo Hong;Ji-won Kang;In Lee
    • The Journal of Internal Korean Medicine
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    • v.44 no.6
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    • pp.1337-1345
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    • 2023
  • Objectives: This study reports on the improvement of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis with ovarian teratoma after Korean medicine treatment. Methods: A patient was treated with Korean medicine treatments, such as acupuncture and herbal medications (Gami-ondam-tang and Samulanshin-tang-gamibang). The patient's improvement was evaluated using manual muscle testing (MMT), the Mini-Mental State Exam-Korea (MMSE-K), the modified Barthel index (MBI), and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). Results: After using Korean medicine treatments, the patient's MMT, MMSE-K, and MBI scores increased, and the CASE score decreased. Conclusion: This case suggests that Korean medicine can be effective in treating the symptoms of anti-NMDA receptor encephalitis.

Childhood Onset of Anti-N-Methyl-D-Aspartate Receptor Encephalitis Without Teratoma Masquerading as a Psychotic Disorder

  • Yeum, Tae-Sung;Lee, Jung;Park, Sung-Yeol;Joen, Yaelim;Kim, Bung-Nyun
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.30 no.3
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    • pp.127-131
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    • 2019
  • Many neurologic disorders manifest as psychiatric symptoms. Anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis is an autoimmune disease of the brain characterized by numerous neurological and psychiatric features. Despite being rare, its prevalence is rapidly increasing and early management is critical in ensuring successful and sustainable recovery. Therefore, the illness should be considered as a differential diagnosis when clinically assessing patients. This report presents a case of a female child who was hospitalized for acute psychiatric manifestations, which was later confirmed as anti-NMDA receptor encephalitis. She recovered relatively successfully after combined neurological and psychiatric treatment. This report provides information on the clinical course of early onset anti-NMDA receptor encephalitis, including treatment strategy and prognosis.

Bickerstaff's Brainstem Encephalitis with Guillain-Barr$\acute{e}$ Syndrome Presenting Severe Paralytic Ileus (길랑-바레증후군이 동반된 Bickerstaff 뇌줄기뇌염에서 나타난 심한 마비성 장 폐색)

  • Park, Min-Su
    • Annals of Clinical Neurophysiology
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    • v.11 no.1
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    • pp.33-36
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    • 2009
  • Bickerstaff's brainstem encephalitis (BBE) is an autoimmune central nervous system disorder. It can occur in more limited forms and may overlap with Guillain-Barr$\acute{e}$ syndrome (GBS). A 49-year-old female presented with rapidly progressive paralytic ileus, urinary retention, deep drowsiness, ophthalmoplegia, dysarthria, ataxia, quadriparesis and hyporeflexia after viral meningitis. She was diagnosed as BBE with GBS and treated with immunoglobulin. She was completely recovered after 1 month. It is a rare case of BBE overlapping with GBS presenting with severe paralytic ileus.

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Isolated Leptomeningeal Enhancement in Anti-N-Methyl D-Aspartate Receptor Encephalitis: The Diagnostic Value of Contrast-Enhanced Fluid-Attenuated Inversion Recovery Imaging (항-NMDA 수용체 항체와 관련된 뇌염에서 단독 연수막 조영증강: 조영증강 유체감쇠반전회복기법 영상의 진단적 가치)

  • Jun Kyeong Park;Eun Ja Lee;Kwang Ki Kim
    • Journal of the Korean Society of Radiology
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    • v.83 no.4
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    • pp.945-950
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    • 2022
  • Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a common autoimmune encephalitis that is noted to be a severe but treatable disease entity. Patients with anti-NMDAR encephalitis often develop psychotic symptoms, including delusions, hallucinations, and paranoia, as well as memory impairment and persistent loss of attention. However, MRI findings in such patients show no abnormalities in most cases. Although typical brain abnormality features, known as T2 hyperintensities, involve the brain parenchyma and contrast enhancement at the cerebral cortex or overlying meninges, isolated leptomeningeal enhancement has been rarely reported in anti-NMDAR encephalitis. Herein, we report a patient with anti-NMDAR encephalitis who presented with isolated leptomeningeal enhancement, additionally showing the diagnostic value of contrast-enhanced fluid-attenuated inversion recovery imaging.

A Case of Paraneoplastic Limbic Encephalitis Associated with Primary Adenocarcinoma of Lung (비소세포 폐암과 동반된 부수종양성 변연계뇌염 1예)

  • Shin, Hyun Jong;Kim, Hyun Soo;Lim, Keum Nam;Noh, U Seok;Choi, Jung Hye;Kim, In Soon;Lee, Young Yeul;Park, Byeong Bae;Park, Dong Woo
    • Tuberculosis and Respiratory Diseases
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    • v.63 no.4
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    • pp.382-386
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    • 2007
  • Paraneoplastic limbic encephalitis is a rare disorder that is characterized by personality changes, irritability, depression, seizures, memory loss and dementia, and is commonly associated with small cell lung cancer. The cause is unknown but it is believed to be an autoimmune disorder that develops secondary to a carcinomatous process. We report a patient with the clinical feature consistent with limbic encephalitis. A 64-year-old women developed disorientation, memory loss and general weakness. She was diagnosed with NSCLC (adenocarcinoma) with a brain metastasis 1 year earlier and was treated with radiation and chemotherapy. Although the lung mass and brain metastatic lesions had improved, the brain T2-weighted MRI showed high signal intensity in the right temporal region. This lesion consisted of with limbic encephalitis and was negative to the other viral and immune markers. The patient's symptoms did not improve after steroid treatment. Our case demonstrated that a NSCLC (adenocarcinoma) also can be associated with paraneoplastic limbic encephalitis.

Overlapping Guillain-Barr$\acute{e}$ syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus

  • Rho, Young Il
    • Clinical and Experimental Pediatrics
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    • v.57 no.10
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    • pp.457-460
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    • 2014
  • A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barr$\acute{e}$ syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.