1.Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children
Ji Yeon HAN ; Soo Yeon KIM ; Woojoong KIM ; Hunmin KIM ; Anna CHO ; Jieun CHOI ; Jong-Hee CHAE ; Ki Joong KIM ; Young Se KWON ; Il Han YOO ; Byung Chan LIM
Journal of Clinical Neurology 2025;21(1):65-73
Background:
and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Methods:
We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse.
Results:
The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates.
Conclusions
Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.
2.Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children
Ji Yeon HAN ; Soo Yeon KIM ; Woojoong KIM ; Hunmin KIM ; Anna CHO ; Jieun CHOI ; Jong-Hee CHAE ; Ki Joong KIM ; Young Se KWON ; Il Han YOO ; Byung Chan LIM
Journal of Clinical Neurology 2025;21(1):65-73
Background:
and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Methods:
We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse.
Results:
The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates.
Conclusions
Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.
3.Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children
Ji Yeon HAN ; Soo Yeon KIM ; Woojoong KIM ; Hunmin KIM ; Anna CHO ; Jieun CHOI ; Jong-Hee CHAE ; Ki Joong KIM ; Young Se KWON ; Il Han YOO ; Byung Chan LIM
Journal of Clinical Neurology 2025;21(1):65-73
Background:
and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Methods:
We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse.
Results:
The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates.
Conclusions
Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.
4.Full arch rehabilitation using a digital diagnostic model in a patient with skeletal class III malocclusion
Se-Hyoun KIM ; Ho-Beom KWON ; Myung-Joo KIM ; Young-Jun LIM
The Journal of Korean Academy of Prosthodontics 2024;62(4):357-365
By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.
5.Full arch rehabilitation using a digital diagnostic model in a patient with skeletal class III malocclusion
Se-Hyoun KIM ; Ho-Beom KWON ; Myung-Joo KIM ; Young-Jun LIM
The Journal of Korean Academy of Prosthodontics 2024;62(4):357-365
By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.
6.Full arch rehabilitation using a digital diagnostic model in a patient with skeletal class III malocclusion
Se-Hyoun KIM ; Ho-Beom KWON ; Myung-Joo KIM ; Young-Jun LIM
The Journal of Korean Academy of Prosthodontics 2024;62(4):357-365
By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.
7.Full arch rehabilitation using a digital diagnostic model in a patient with skeletal class III malocclusion
Se-Hyoun KIM ; Ho-Beom KWON ; Myung-Joo KIM ; Young-Jun LIM
The Journal of Korean Academy of Prosthodontics 2024;62(4):357-365
By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.
8.Full arch rehabilitation using a digital diagnostic model in a patient with skeletal class III malocclusion
Se-Hyoun KIM ; Ho-Beom KWON ; Myung-Joo KIM ; Young-Jun LIM
The Journal of Korean Academy of Prosthodontics 2024;62(4):357-365
By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.
10.Neoadjuvant Cisplatin-Based Chemotherapy Followed by Selective Bladder Preservation Chemoradiotherapy in Muscle-Invasive Urothelial Carcinoma of the Bladder: Post Hoc Analysis of Two Prospective Studies
Sung Wook CHO ; Sung Hee LIM ; Ghee Young KWON ; Chan Kyo KIM ; Won PARK ; Hongryull PYO ; Jae Hoon CHUNG ; Wan SONG ; Hyun Hwan SUNG ; Byong Chang JEONG ; Se Hoon PARK
Cancer Research and Treatment 2024;56(3):893-897
Purpose:
Bladder preservation chemoradiotherapy (CRT) in patients with a clinical complete response (cCR) following cisplatin-based neoadjuvant chemotherapy (NAC) is a promising treatment strategy for muscle-invasive bladder urothelial carcinoma (MIBC). A combined analysis of raw data from two prospective phase II studies was performed to better evaluate the feasibility of selective bladder preservation CRT.
Materials and Methods:
The analysis was based on primary efficacy data from two independent studies, including 76 MIBC patients receiving NAC followed by bladder preservation CRT. The efficacy data included metastasis-free survival (MFS) and disease-free survival (DFS). For the present analysis, starting point of survival was defined as the date of commencing CRT.
Results:
Among 76 patients, 66 had a cCR following NAC. Sixty-four patients received gemcitabine and cisplatin (GC) combination chemotherapy in neoadjuvant setting, and 12 received nivolumab plus GC. Bladder preservation CRT following NAC was generally well-tolerated, with low urinary tract symptoms being the most common late complication. With a median follow-up of 64 months, recurrence was recorded in 43 patients (57%): intravesical only (n=20), metastatic only (n=16), and both (n=7). In 27 patients with intravesical recurrence, transurethral resection, and Bacillus Calmette-Guerin treatment was given to 17 patients. Salvage cystectomy was performed in 10 patients. Median DFS was 46.3 (95% confidence interval [CI], 25.1 to 67.5) months, and the median MFS was not reached. Neither DFS nor MFS appeared to be affected by any of the baseline characteristics. However, DFS was significantly longer in patients with a cCR than in those without (hazard ratio, 0.465; 95% CI, 0.222 to 0.976).
Conclusion
The strategy of NAC followed by selective bladder preservation CRT based on the cCR is feasible in the treatment of MIBC. A standardized definition of cCR is needed to better assess disease status post-NAC.

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