단일병원에서 경험한 소아뇌염의 임상증상과 예후

박준성,염미선,김은희,유리나,이윤정,고태성 대한소아신경학회 2014 대한소아신경학회지 Vol.22 No.4

Purpose: This study was performed to investigate the clinical presentations, diagnostic findings and prognosis of pediatric patients with the broad definition of “encephalitis”, that is inflammation of brain, in a single Korean tertiary center. Method: We retrospectively analyzed medical records of patients who had been diagnosed with encephalitis from January 2000 to July 2013 at the Asan Medical Center Children’s Hospital. We classified encephalitis in three categories of etiology which are infectious, para-infectious and primary inflammatory. The initial presentation, radiological and electroencephalographic findings, treatments and outcomes were reviewed. Results: During the 13-year study period, we found 199 pediatric cases of encephalitis.128 (64.3%), 50 (25.1%), and 21 (10.5%) cases were classified as infectious, para infectious and primary inflammatory encephalitis, respectively. They presented with fever (62.3%), altered mentality (51.3%), seizures (52.8%) and motor dysfunction (37.7%). Specific pathogens were proven in only 32 (25%) patients from 128 infectious encephalitis. Among 185 patients who had been followed up, 127 cases (68.6%) recovered without sequela and 50 cases (27%) with sequelae. Eight cases (4.3%), six with infectious encephalitis, one with acute disseminated encephalomyelitis and the other with Reye syndrome, expired. Conclusion: Infectious etiology is suggested in a great part of the pediatric encephalitis and additional efforts are needed to find specific etiologies of encephalitis in many cases. Considering the high mortality and morbidity of the pediatric encephalitis, prompt evaluations and appropriate treatment should be implemented. 목적: 다양한 임상증상을 보이며 심각한 합병증 또는 사망에 이를수 있는 소아뇌염의 원인을 알고 이에 따른 진단, 치료, 그 치료 경과를 알아보고자 하였다. 방법: 2000년 1월부터 2013년 7월까지 서울아산병원에 뇌염으로입원한 소아199명을 대상으로 후향적 검토를 통해 소아뇌염의 원인질환을 확인하고 원인질환에 따라 감염성, 부감염성, 일차 염증성 뇌염으로 분류하였으며 각각의 임상양상, 검사결과, 잠정진단에 따른 치료 및 질병경과를 조사하였다. 결과: 감염성 뇌염이 전체 뇌염의 64.3%로 가장 많았으며 특정 원인균이 동정되지 않은 경우가 75% 이었다. 부감염성 뇌염은 25.1%, 일차 염증성 뇌염은 10.5%로 확인되었다. 초기 증상으로 발열(62.3%), 경련발작(52.8%), 의식변화(51.3%), 운동 이상, 두통, 구토를보였으며, 뇌척수액 내 백혈구 증가는 72.6%에서, MRI 이상은 64%에서, EEG 이상은 76.6%에서 나타났다. 68.1%에서 완치되었으며 27% 에서 후유증을 남기고 4.3%는 사망하였다. 일차 염증성 뇌염에서 완치율이 낮고(P=0.032) 합병률이 높은 경향을 보였다(P=0.039). 경련증상(OR 4.17, P=0.007), 뇌파 이상소견(OR 3.37, P=0.037)이 나쁜 예후와 관련이 있었다. 결론: 발열, 운동기능 이상, 경련, 의식변화 등의 임상증상을 보이는환자에서 뇌염을 의심하고 최근 발달한 영상 및 혈청학적 검사를 포함하는 광범위한 검사와 잠정진단에 따른 즉각적인 치료가 필요하며합병증에 대하여 정신건강의학적 평가를 포함한 지속적인 추적관찰이 필요하다.

중합 효소 연쇄 반응을 이용한 단순 포진 바이러스 뇌염의 진단

최은화,김기중,이환종,황용승,김인원 대한감염학회 1999 감염 Vol.31 No.1

급성 뇌염의 치료 전략과 신경학적인 예후 인자에 대한 연구

이소연(So Yeon Lee) 장용석(Yong Seok Jang) 김선준(Sun Jun Kim) 대한소아신경학회 2004 대한소아신경학회지 Vol.12 No.1

목적 : 뇌염은 다양한 원인에 의한 중추신경계의 감염으로 중증의 임상경과를 취하며 높은 사망률과 심각한 신경학적 후유증을 남기게 된다. 원인균들이 뇌염을 일으키는 기전은 중추신경계를 직접 침범하는 것과 자가면역에 의해 일어나는 것의 두가지의 기전에 의한다. 중추신경계를 직접 침범하는 경우 중 혈행성 경로의 경우 원인 균이 혈액속에서 증식 후에 중추신경계로 침입하여 BBB의 변화를 일으키며 주로 회배질에서 병변을 일으킨다. 자가면역에 의해 일어나는 경우 주로 백질의 소정맥과 그 주위조직을 침범하여 인접 부위 신경의 탈수초화를 일으키나 축삭은 비교적 잘 보존되는 반면 직접적인 감염에서는 신경세포의 파괴 소견이 특징이다. 뇌염의 치료는 보존적인 치료뿐이며 현재 사용되고 있는 면역글로불린이나 acyclovir에 대한 효과도 정확히 알려진 바가 없다. 이에 저자들은 전북대학교 병원 소아과에서 뇌염으로 진단된 환아들의 임상상 및 검사소견을 분석하고 임상상과 뇌 자기공명 영상 소견, 치료 약제 등 예후와 관련된 인자를 조사하여 이를 보고하는 바이다. 방법 : 1993년 7월부터 2003년 7월까지 8년 동안 전북대학교병원 소아과에 뇌염으로 입원한 환아 46명을 대상으로 후향적으로 조사하여 이들의 임상증상과 검사 소견, 사용한 약제 등을 분석하고, 발병 연령, 경련 동반 유무와 경련의 시간, 뇌 자기공명 영상 상 침범 부위 그리고 치료 약제들과 환아들의 임상적인 예후와의 관련성을 조사하였다. 결과 : 성별분포를 보면 뇌염의 남녀 발생빈도는 각각 26명(56.5%)과 20명(43.5%)으로 그 비는 1.3: 1로 남아에 더 많았고, 연령분포를 보면 10-14세 사이가 가장 많았다. 뇌염의 원인은, 알 수 없었던 경우가 50%로 반수를 차지하였고, 원인을 알 수 있었던 경우는 홍역 26.1%, 포진정 바이러스 6.5%, 장 바이러스 4.3%, 아데노 바이러스 4.3%, 예방 접종 후 4.3%가 있었다. 임상증상으로는 발열이 78.3%으로 가장 많았고, 그 다음으로는 의식의 변화가 71.7%이었다. 경련은 63.8%의 환아에서 있었고, 간질중첩증은 13.9%에서 있었다. 뇌 자기공명 영상 상 10명(22.7%)에서 정상 소견을 보였고, 비정상소견 중에신는 미만성 음영변화가 27명(61.4%)으로 가장 많았고, 침범부위는 대뇌 백질부가 가장 많았다. 뇌염의 발병 연령이 1세 미만인 경우 낮은 회복률과 높은 후유증 발생률을 보였다. 경련이 동반되었던 경우 경련이 없었던 환아들에 비하여 예후가 좋지 않았으며 특히 간질중첩증의 경우 더욱 예후가 나빴다. 뇌 자기공명 영상 상 대뇌의 회백질을 침범한 경우가 대뇌의 백질을 침범한 경우보다 예후가 좋지 않았다. ADEM을 제외한 뇌염 환아들 중 IVIG를 사용한 군에서는 사망한 경우가 없었고, 사용하지 않은 군에 비하여 예후가 좋았다. 본 연구에서는 acyclovir를 사용 후 특별한 이점을 발견하지 못했다. 결론 : 발병시 어린 나이(영아), 경련의 동반, 특히 간질중첩증 그리고 MRI상 회백질부의 침범 등은 뇌염 환아에서 나쁜 신경학적인 예후를 예측하는 데 유용한 인자이며, 치료시 면역기전에 의한 뇌염 뿐 아니라 원인 균이 직접 침범한 뇌염의 경우에서도 IVIG를 사용하는 것이 효과가 좋고, 항경련제를 사용하여 경련을 치료 할 뿐만 아니라 적극적으로 간질을 예방해야 한다고 생각된다. Purpose : Encephalitis is a cranial nervous system infection that is caused by various etiologies. Most of the patients with encephalitis undergo severe or fatal clinical course with sequelae. This study was conducted to estimate the clinical outcomes and to evaluate factors which can be used to predict clinical outcomes among pediatric patients with encephalitis Methods : We retrospectively reviewed 46 patients with encephalitis who were admitted to the Department of Pediatrics of Chonbuk National University Hospital, from July 1995 to July 2003. Encephalitis was diagnosed based on the presence of neurologic abnormalities, CSF, Brain CT or MRI findings. Information on sequelae was obtained in the outpatient clinic. Results : In this study, five patients(11.6%) were expired, 17 patients(39.5%) developed neurologic sequelaes and 21 patients(46.5%) were recovered without sequelae. Infants had poorer outcomes compared to older age groups. Twenty nine cases who had seizures showed high morbidity rate(48.3%) compared to the seizure-free group(17.6%). Among six patients with status epilepticus, four(66.7%) developed neurologic sequelae and two(33.3%) expired. The group with normal brain MRI findings had better outcomes (70%) than the group with abnormal MRI findings (55.5%). Gray matter involved patients on MRI had poorer recovery rate(33.3%) than white matter involved patients(69.2%). The group treated with intravenous immunoglobulin(IVIG) had better outcomes(64.7%) than the other group(37.9%). Conclusion : The presence of seizure, younger patients, and presence of abnormal findings of brain MRI, especially gray matter lesions were associated with poor clinical outcomes in children with encephalitis.

In vivo Study on the Japanese Encephalitis

김인범(In-Beom Kim),채수림(Soo-Lim Chae),최우영(Woo-Young Choi),박찬(Chan Park),주영란(Young-Ran Joo),조해월(Hae-Wol Cho),박근용(Keun-Yong Park) 대한해부학회 2003 Anatomy & Cell Biology Vol.36 No.5

일본뇌염은 일본뇌염바이러스의 감염에 의해 야기되는 치명적인 중추신경 질환으로서, 동아시아 지역에서 발생하는 뇌 염 중 가장 흔한 형태이다. 본 연구는 일본뇌염의 in vivo 모델을 확립하고 이의 특성을 밝히고자 생후 4주된 생쥐에 일본뇌염바이러스를 복강 내 로 주사한 후 면역조직화학법으로 생쥐 뇌의 바이러스 분포를 조사하였고, 또한 조직병리양상을 관찰하였다. 일본뇌염바 이러스는 뇌의 특정 부분에 존재하는 신경세포에 위치하였고, 조직병리소견은 뇌실질 내에 염증세포침윤 및 단핵구의 혈 관주위 커프현상과 같은 전형적인 급성 바이러스성 뇌염의 양상을 보였다. 이러한 결과는 이 in vivo 모델이 일본뇌염바이러스의 뇌로의 유입기전, 특정세포친화성 및 일본뇌염의 병태생리를 연구하는데 유용하게 사용될 수 있음을 시사한다. Japanese encephalitis is a potentially lethal disease of the central nervous system caused by infection with Japanese encephalitis virus (JEV). JEV is the most common cause of encephalitis over a large part of eastern Asia. To establish and characterize in vivo model to study the Japanese encephalitis, the immunohistochemical localization of JEV and the histopathological finding were investigated in the brains of young adult mice infected with JEV by intraperitoneal inoculation. JEV was localized to neurons in discrete regions of the brain. Histopathological finding showed typical pattern of acute viral encephalitis, such as inflammatory cell infiltration in brain parenchyme and perivascular cuffs of mononuclear cells. These results suggest that this in vivo system can be used to study the mechanism of virus entry into the brain, cell specific tropism, and pathophysiology in Japanese encephalitis.

Analysis of Risk Factors for a Poor Prognosis in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Construction of a Prognostic Composite Score

Yejia Mo,Li Wang,Libo Zhu,Feng Li,Gang Yu,Yetao Luo,Meng Ni 대한신경과학회 2020 Journal of Clinical Neurology Vol.16 No.3

Background and Purpose Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most-common form of autoimmune encephalitis, but its early diagnosis is challenging. This study aimed to identify the risk factors for a poor prognosis in anti-NMDAR encephalitis and construct a prognostic composite score for obtaining earlier predictions of a poor prognosis. Methods We retrospectively analyzed the clinical data, laboratory indexes, imaging findings, and electroencephalogram (EEG) data of 60 patients with anti-NMDAR encephalitis. The modified Rankin Scale (mRS) scores of patients were collected when they were discharged from the hospital. The mRS scores were used to divide the patients into two groups, with mRS scores of 3–6 defined as a poor prognosis. Logistic regression analysis was used to analyze independent risk factors related to a poor prognosis. Results This study found that 23 (38.3%) and 37 (61.7%) patients had good and poor prognoses, respectively. Logistic regression analysis showed that age, disturbance of consciousness at admission, and ≥50% slow waves on the EEG were significantly associated with patient outcomes. An age, consciousness, and slow waves (ACS) composite score was constructed to predict the prognosis of patients with anti-NMDAR encephalitis at an early stage based on regression coefficients. Conclusions Age, disturbance of consciousness at admission, and ≥50% slow waves on the EEG were independent risk factors for a poor prognosis. The ACS prognostic composite score could play a role in facilitating early predictions of the prognosis of anti-NMDAR encephalitis.

The Roles and Perspectives of Toll-Like Receptors and CD^4+ Helper T Cell Subsets in Acute Viral Encephalitis

한영우,Sunit K. Singh,어성국 대한면역학회 2012 Immune Network Vol.12 No.2

Acute viral encephalitis caused by neurotrophic viruses, such as mosquito-borne flaviviruses, is an emerging and re-emerging disease that represents an immense global health problem. Considerable progression has been made in understanding the pathogenesis of acute viral encephalitis, but the immune-pathological processes occurring during the progression of encephalitis and the roles played by various molecules and cellular components of the innate and adaptive systems still remain undefined. Recent findings reveal the significant contribution of Toll-like receptors (TLRs) and regulatory CD4^+ T cells in the outcomes of infectious diseases caused by neurotrophic viruses. In this review, we discuss the ample evidence focused on the roles of TLRs and CD^4+ helper T cell subsets on the progression of acute viral encephalitis. Finally, we draw attention to the importance of these molecules and cellular components in defining the pathogenesis of acute viral encephalitis, thereby providing new therapeutic avenues for this disease.

The Roles and Perspectives of Toll-Like Receptors and $CD4^+$ Helper T Cell Subsets in Acute Viral Encephalitis

Han, Young-Woo,Singh, Sunit K.,Eo, Seong-Kug The Korean Association of Immunobiologists 2012 Immune Network Vol.12 No.2

Acute viral encephalitis caused by neurotrophic viruses, such as mosquito-borne flaviviruses, is an emerging and re-emerging disease that represents an immense global health problem. Considerable progression has been made in understanding the pathogenesis of acute viral encephalitis, but the immune-pathological processes occurring during the progression of encephalitis and the roles played by various molecules and cellular components of the innate and adaptive systems still remain undefined. Recent findings reveal the significant contribution of Toll-like receptors (TLRs) and regulatory $CD4^+$ T cells in the outcomes of infectious diseases caused by neurotrophic viruses. In this review, we discuss the ample evidence focused on the roles of TLRs and $CD4^+$ helper T cell subsets on the progression of acute viral encephalitis. Finally, we draw attention to the importance of these molecules and cellular components in defining the pathogenesis of acute viral encephalitis, thereby providing new therapeutic avenues for this disease.

The Roles and Perspectives of Toll-Like Receptors and CD4⁺ Helper T Cell Subsets in Acute Viral Encephalitis

Han, Young-Woo,Singh, Sunit K.,Eo, Seong-Kug 대한면역학회 2012 Immune Network Vol.12 No.2

Acute viral encephalitis caused by neurotrophic viruses, such as mosquito-borne flaviviruses, is an emerging and re-emerging disease that represents an immense global health problem. Considerable progression has been made in understanding the pathogenesis of acute viral encephalitis, but the immune-pathological processes occurring during the progression of encephalitis and the roles played by various molecules and cellular components of the innate and adaptive systems still remain undefined. Recent findings reveal the significant contribution of Toll-like receptors (TLRs) and regulatory CD4⁺ T cells in the outcomes of infectious diseases caused by neurotrophic viruses. In this review, we discuss the ample evidence focused on the roles of TLRs and CD4⁺ helper T cell subsets on the progression of acute viral encephalitis. Finally, we draw attention to the importance of these molecules and cellular components in defining the pathogenesis of acute viral encephalitis, thereby providing new therapeutic avenues for this disease.

Case of anti-N-methyl D-aspartate receptor encephalitis associated with ovarian teratoma presenting as suicidal ideation

( Sukyo Lee ),( Sejoong Ahn ),( Jong-hak Park ),( Hanjin Cho ),( Sungjin Kim ) 대한응급의학회 2024 대한응급의학회지 Vol.35 No.2

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a common cause of autoimmune encephalitis. The condition is difficult to diagnose or suspect in the emergency department because it usually presents with nonspecific neurological or psychiatric symptoms. It is often mistaken for viral encephalitis or psychiatric illness. This paper reports a case of anti-NMDAR encephalitis in which the patient experienced mood changes, including suicidal ideation, which led to a delayed diagnosis after three visits to the emergency department. This paper aims to raise awareness among emergency physicians about the possibility of anti-NMDAR encephalitis and to encourage them to consider it in their differential diagnosis in the emergency department.

Comparison of the Demographic and Laboratory Profiles of Patients with Aseptic Meningitis and Encephalitis: Significance of Age and C-reactive Protein

박강민,신경진,하삼열,박진세,박봉수,김성은 대한임상신경생리학회 2014 Annals of Clinical Neurophysiology Vol.16 No.2

Background: Viruses can cause either meningitis or encephalitis. It is unclear why some people suffer from aseptic meningitis,and others acquire aseptic encephalitis when infected with the same viral pathogens. The aim of this study wasto compare demographic and laboratory factors between patients with aseptic meningitis and encephalitis. Methods: Thedemographic and laboratory differences were analyzed according to age, sex, diabetes, hypertension, C-reactive proteinin the blood, white blood cell and protein in the cerebrospinal fluid, and glucose ratio (cerebrospinal fluid/blood). Additionally, we analyzed the nation-wide differencesin age between the patients with aseptic meningitis and those withencephalitis in Korea. Results: The patients with aseptic encephalitis were older, more likely to have hypertension, andhad higher levels of C-reactive protein than did the patients with aseptic meningitis. However, the numbers of whiteblood cells in the cerebrospinal fluid were significantly higher in the patients with meningitis than in the patients withencephalitis. Multivariable analysis revealed that age >49 years, hypertension and a C-reactive protein level >5.81mg/dL were independent and significant variables in the prediction of aseptic encephalitis. Additionally, the patientswith aseptic encephalitis were older than those with aseptic meningitis in the nation-wide Korean database. Conclusions:Older age, hypertension, and higher levels of C-reactive protein are useful factors for the prediction of asepticencephalitis.(Korean J Clin Neurophysiol 2014;16:55-61)