희귀 신경계질환 극복을 위한 전문가 단체와 환자 단체와의 협력

Ki Hoon Kim,Young Nam Kwon,Jun-Soon Kim,Sooyoung Kim,Seung Woo Kim,Kee Hong Park,Jin-Sung Park,Jeong Bin Bong,Jin Myoung Seok,Hung Youl Seok,Hye Lim Lee,Eunbin Cho,Byeoung-Jun Jeon,Seong-il Oh,Public 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.1

Rare neurological disorders present significant challenges in diagnosis, treatment, and patient care. Despite their low prevalence, these conditions affect millions of people, necessitating collaboration between medical association and patient organizations or patient advocacy groups. This review explores the partnership between the Korean Society for Neuroimmunology and the Korean Multiple Sclerosis Society as a model of effective cooperation. Patient organizations play a vital role by providing support that includes education, emotional assistance, and policy advocacy. On the other hand, medical associations contribute through research, development of treatment guidelines, and medical education. Their collaborative efforts have led to notable advancements such as expanded financial support programs, public awareness campaigns, and improved healthcare policies. However, challenges such as funding limitations and regulatory hurdles persist. Therefore, strengthening domestic and international partnerships and establishing long-term frameworks for cooperation are crucial in the future. Effective collaboration among stakeholders is essential to advance research, improve healthcare access, and improve the quality of life for patients with rare neurological diseases.

다발경화증의 인지 장애와 평가

Eun Oh,Seong-il Oh 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.2

Cognitive impairment is a common and disabling feature of multiple sclerosis (MS), affecting up to 65% of patients. It primarily manifests as deficits in processing speed, memory, executive function, and verbal fluency, significantly influencing daily activities and work performance. Despite its clinical relevance, cognitive assessment in MS remains underutilized. Several neuropsychological tools, including the multiple sclerosis neuropsychological questionnaire, the symbol digit modalities test, and the brief international cognitive assessment for MS, facilitate early detection of cognitive impairment. Moreover, depression, anxiety, and fatigue often worsen cognitive decline. Recent research has identified distinct cognitive phenotypes in MS, aiding in personalized management strategies. Integrating cognitive assessments with neuroimaging biomarkers and cognitive rehabilitation is essential for improving patient outcomes. Future studies should focus on refining diagnostic tools and exploring targeted interventions to better support MS patients.

신경학적 면역관문억제제 연관 부작용의 진단과 치료

Jae-Won Hyun 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.1

As the use of immune checkpoint inhibitors (ICIs) continues to rise, particularly in combination therapies, neurological immune-related adverse events (irAEs) are expected to become more prevalent in clinical practice. These neurological irAEs are rare but often present with atypical and multifocal manifestations, potentially leading to severe neurological impairments or even mortality. Treatment generally involves discontinuation of ICIs, along with corticosteroids, intravenous immunoglobulin, and/or plasma exchange. Effective management requires early diagnosis and a multidisciplinary approach. To this end, a comprehensive understanding of neurological irAEs by clinicians is essential, and this review describes key considerations for the accurate diagnosis and treatment of neurological irAEs.

Concanavalin A가 細胞性免疫反應과 體液性免疫反應에 미치는 影響 : 마우스의 T依存性 體液免疫反應에 대한 Con A의 抑制作用 Suppression of a thymus Dependent Humoral Response by Con A in Mice

張友鉉,金翼詳,李明洙,崔明植 大韓免疫學會 1981 大韓免疫學會誌 Vol.3 No.1

Mice treated with Con A 1 or 2 days prior to primary immunization with SRBC exhibited a significai.F suppression of direct PFC response. This immunosuppressive effect could be reversed by using higher doses of antigen designad to by pass T-cell function. Normal syngeneic recipient mice transferred with Con A-activated spleen cells showed a suppression of primary direct PFC response on day 6. Recipients transferred with Con A-activated thymocytes showed no effect on direct PFC responses. It is suggested that Con A induced immunosuppression of thymus-dependent humoral immune response in mice is at least partly due to the activation of a subpopulation of thymus derived cells, of which tissue source is not thymus but spleen, and that the effect is short-lived and the suppressive effect of HIR is marked in late stage.

대상포진-유발성 항아쿠아포린-4 항체 양성 시신경척수염범주질환: 증례 보고 및 문헌 고찰

Daecheol Moon,Dong Kyu Kim,Seong-il Oh 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.2

We report the case of a 70-year-old female who developed acute transverse myelitis 1 month after herpes zoster infection and was diagnosed with aquaporin-4 (AQP4) antibody-positive neuromyelitis optica spectrum disorder. The patient was treated with high-dose corticosteroid and intravenous immunoglobulin therapy and her serum AQP4 antibody seroconverted to negative 3 months later. This case suggests that varicella-zoster virus reactivation may trigger transient AQP4 autoimmunity and highlights the clinical significance of post-treatment seroreversion in distinguishing infection-triggered from idiopathic neuromyelitis optica spectrum disorder.

국내 다발경화증 역학 조사: 2025년 전국 다기관 병원 기반 연구

Sooyoung Kim,Ho Jin Kim,Jae-Won Hyun,Eun-Jae Lee,Young-Min Lim,Hyunjin Kim,Ju-Hong Min,Byung Joon Kim,Seung Ho Choo,Sung-Min Kim,Dong Seok Ohn,Ha Young Shin,Young Nam Kwon,Seung Woo Kim,Ki Hoon Kim,Ju 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.2

Background: A hospital-based survey conducted in 2023 provided initial insight into the realworld landscape of multiple sclerosis (MS) care. Based on 2023 survey, the present study aims to offer an updated nationwide assessment of MS in Korea. Methods: In January 2025, we collected data on the number of patients with MS, MS subtypes, newly diagnosed cases in the past year, onset age, sex ratio, current disease modifying therapies (DMTs), and disability status (expanded disability status scale, EDSS) from 47 major MS centers in Korea. Results: A total of 1,799 patients had clinically confirmed MS and 365 patients were under follow-up for suspected MS. 193 patients were newly diagnosed in 2024. MS onset occurred most frequently at 20-29 years, whereas very early or late onset was uncommon. DMTs were administered to 95% of patients, with 62% receiving moderate-efficacy agents and 28% receiving high-efficacy agents. A majority of patients (78.1%) were followed at centers in the Seoul Capital Area, which also accounted for 79.9% of those receiving high-efficacy therapies. Regarding neurological disability, 307 patients (17%) had an EDSS score greater than 3.0, and 115 patients (6%) had a score above 5.5, indicating moderate to severe disability. Conclusion: This updated nationwide hospital-based survey reveals a gradual increase in the number of patients with MS under clinical follow-up compared to a 2023 survey conducted in April 2022. The vast majority of patients are receiving DMTs, and while the proportion receiving high-efficacy agents remains modest, it demonstrates a clear upward trend.

다발성경화증에서의 다차원적 장애 평가 척도

You-Ri Kang,Tai-Seung Nam 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.2

Multiple sclerosis (MS) is a chronic inflammatory disorder of the central nervous system in which relapses and neurodegeneration lead to cumulative neurological decline. Historically, therapeutic development in MS has focused on relapse prevention, while disability progression has been evaluated using the Expanded Disability Status Scale (EDSS). However, EDSS is heavily weighted toward ambulation, and thus lacks sensitivity to less noticeable motor changes such as upperlimb dysfunction, and cognitive changes. Recently, the growing recognition that MS can progress independent of relapse activity has underscored the need for more precise, multidimensional assessment tools capable of capturing the full spectrum of MS-related impairment. Recent advances have introduced performance-based measures and composite functional outcomes integrating domains of ambulation, manual dexterity, cognition, and vision. These quantitative assessments provide objective and reproducible metrics of function and can detect subclinical progression earlier than conventional scales. As the focus of MS management shifts from relapse control to prevention of long-term disability, multidimensional functional assessment offers a practical and comprehensive approach for both clinical and research settings, with the potential to refine therapeutic decisions and improve patient outcomes.

GM1, GD1a 항체 양성인 횡단성척수염과 병발한 급성운동축삭신경병의 임상적 증상과 예후

A Young Kim,Hong Keun Yoo,Jong Hyun Jeon,Hyuk Sung Kwon 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.1

Antibodies against GM1 and GD1a are closely associated with acute motor axonal neuropathy (AMAN). Patients simultaneously positive for both anti-GM1 and anti-GD1a antibodies are rare, and their clinical manifestations and prognosis remain unclear. Herein we report a 41-year-old male diagnosed with AMAN and acute transverse myelitis who was positive for immunoglobulin M anti-GM1, immunoglobulin G (IgG) anti-GM1, and IgG anti-GD1a antibodies. He experienced rapid progression of motor weakness to near quadriplegia within a few days, followed by substantial improvement over 6 months after initiation of steroid treatment, resulting in return of most activities of daily living.

낮은 역가 MOG항체 양성 소견의 신중한 해석과 감별 진단의 중요성

Jae-Won Hyun,Ho Jin Kim 대한신경면역학회 2025 대한신경면역학회지 Vol.16 No.1

바이러스 감염에 대한 면역반응

황응수,박정규,차창용 대한면역학회 2004 Immune Network Vol.4 No.2

Viruses are obligate intracellular parasites which cause infection by invading and replicating within cells. The immune system has mechanisms which can attack the virus in extracellular and intracellular phase of life cycle, and which involve both non-specific and specific effectors. The survival of viruses depends on the survival of their hosts, and therefore the immune system and viruses have evolved together. Immune responses to viral infection may be variable depending on the site of infection, the mechanism of cell-to-cell spread of virus, physiology of the host, host genetic variation, and environmental condition. Viral infection of cells directly stimulates the production of interferons and they induce antiviral state in the surrounding cells. Complement system is also involved in the elimination of viruses and establishes the first line of defence with other non-specific immunity. During the course of viral infection, antibody is most effective at an early stage, especially before the virus enters its target cells. The virus- specific cytotoxic T lymphocytes are the principal effector cells in clearing established viral infections. But many viruses have resistant mechanism to host immune responses in every step of viral infection to cells. Some viruses have immune evasion mechanism and establish latency or persistency indefinitely. Furthermore antibodies to some viruses can enhance the disease by the second infection. Immune responses to viral infection are very different from those to bacterial infection. (Immune Network 2004;4(2):73-80) 바이러스는 세포 내 절대 기생체로서 세포에 침투하여 복제하여 증식한다. 면역계는 바이러스가 세포 밖에 존재하는 시기와 세포 내에 있는 시기 모두 공격을 할 수 있으며, 비특이적으로나 특이적인 반응을 보인다. 바이러스의 궁극적인 생존은 숙주의 생존 여부에 달려 있으므로 숙주의 면역체계와 바이러스는 상호 진화하여 왔다. 바이러스 감염에 대한 면역반응은 감염부위, 세포 간 바이러스의 전파기전, 숙주의 생리학적 상태, 유전적 소인과 환경요인에 따라 매우 다양하게 나타난다. 바이러스 감염은 인터페론의 생산을 유도해서 항바이러스 상태를 유발하고, 보체의 작용 등 선천면역에 의해 일차적으로 방어된다. 항체는 감염 초기 단계에 바이러스가 표적세포에 침투하기 전에는 매우 효과적으로 항바이러스 기능을 발휘한다. 확립된 바이러스 감염을 제거하고 완결시키는 데는 세포독성 T 림프구가 결정적인 역할을 한다. 그러나 바이러스는 이와 같은 단계별 숙주의 방어기전에 대항하는 기전을 갖고 있어서 바이러스에 따라서는 평생 숙주의 몸에서 잠복 또는 지속 감염을 이루게 된다. 한편 면역반응이 형성된 경우에 재감염이 되면 오히려 증상을 악화시키는 경우도 있는 등 바이러스 감염에 대한 면역반응의 특성은 다른 세균 등의 면역반응과 상당히 다른 점이 있다.