• 제목/요약/키워드: Autoimmune encephalitis

검색결과 16건 처리시간 0.017초

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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    • 제63권8호
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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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    • 제38권4호
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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.

양극성장애로 오인된 LGI1 자가면역성뇌염 (Anti-LGI1 Antibody Encephalitis)

  • 김은수;이희준;전상원;조성준
    • 생물정신의학
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    • 제27권2호
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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.

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

  • 남이랑;김민화;김마리아;구기범;이세연;김소연;박소정;최준용;한창우;홍진우;강지원;이인
    • 대한한방내과학회지
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    • 제44권6호
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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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    • 제30권3호
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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.

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

  • 박민수
    • Annals of Clinical Neurophysiology
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    • 제11권1호
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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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항-NMDA 수용체 항체와 관련된 뇌염에서 단독 연수막 조영증강: 조영증강 유체감쇠반전회복기법 영상의 진단적 가치 (Isolated Leptomeningeal Enhancement in Anti-N-Methyl D-Aspartate Receptor Encephalitis: The Diagnostic Value of Contrast-Enhanced Fluid-Attenuated Inversion Recovery Imaging)

  • 박준경;이은자;김광기
    • 대한영상의학회지
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    • 제83권4호
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    • pp.945-950
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    • 2022
  • 항-N-메틸 D-아스파르테이트 수용체(anti-N-methyl-D-aspartate receptor; 이하 항-NMDAR) 뇌염은 중증이지만 치료가 가능한 흔한 자가면역뇌염이다. 항-NMDAR 뇌염 환자는 종종 망상, 환각 및 편집증과 같은 정신병적 증상뿐만 아니라 기억력 손상 및 지속적인 주의력 상실과 같은 증상을 호소한다. 항-NMDAR 뇌염 환자의 자기공명영상 소견은 대부분의 경우에서 이상 소견을 보이지 않으나, 이상 소견이 보이는 경우에는 뇌 실질의 T2 고강도 병변과 이와 인접한 연수막에 조영증강이 있는 것으로 알려져 있다. 그러나 항-NMDAR 뇌염에서 단독 연수막 조영증강은 드물게 보고되어 있다. 우리는 항-NMDAR 뇌염에서 단독 연수막 조영증강을 보인 환자의 증례 보고와 함께 조영증강 유체감쇠반전회복기법 영상의 진단 가치를 보여주고자 한다.

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

  • 신현종;김현수;임금남;노유석;최정혜;김인순;이영열;박병배;박동우
    • Tuberculosis and Respiratory Diseases
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    • 제63권4호
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    • pp.382-386
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    • 2007
  • 저자들은 이미 비소세포 폐암(선암)을 진단받고 Gefitinib으로 치료중인 여자 환자에서 기억장애, 행동 및 인지기능 장애 등의 증상으로 내원하여 뇌자기공명 소견과 혈청학적 검사로 부수종양성 변연계 뇌염을 진단하였으며 스테로이드 치료에 호전이 없었던 증례를 처음 경험하였기에 보고하는 바이다.

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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    • 제57권10호
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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.