Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, 812-8582, Japan.
Research Center for Environment and Developmental Medical Sciences, Kyushu University, Fukuoka, Japan.
Eur J Pediatr. 2023 Jul;182(7):3175-3185. doi: 10.1007/s00431-023-04989-z. Epub 2023 Apr 29.
Myelin oligodendrocyte glycoprotein antibody (MOG-Ab) is an autoantibody associated with acquired demyelinating syndrome (ADS) in childhood and adults. The pathogenic roles of MOG-Ab and long-term outcomes of children with MOG-Ab-associated disease (MOGAD) remain elusive. We investigated the clinical features of children with ADS during follow-up in our institute. Clinical data were retrospectively analyzed using medical charts of patients managed in Kyushu University Hospital from January 1st, 2001, to March 31st, 2022. Participants were children of < 18 years of age when they received a diagnosis of ADS in our hospital. Cell-based assays were used to detect MOG-Ab in serum or cerebrospinal fluid at the onset or recurrence of ADS. The clinical and neuroimaging data of MOG-Ab-positive and MOG-Ab-negative patients were statistically analyzed. Among 31 patients enrolled in this study, 22 (13 females, 59%) received tests for MOG antibodies. Thirteen cases (59%) were MOG-Ab-positive and were therefore defined as MOGAD; 9 (41%) were MOG-Ab-negative. There were no differences between MOGAD and MOG-Ab-negative patients in age at onset, sex, diagnostic subcategories, or duration of follow-up. MOGAD patients experienced headache and/or somatosensory symptoms more frequently than MOG-Ab-negative patients (12/13 (92%) vs. 3/9 (22%); p = 0.0066). Somatosensory problems included persistent pain with hyperesthesia in the left toe, perineal dysesthesia, and facial hypesthesia. No specific neuroimaging findings were associated with MOGAD or the presence of somatosensory symptoms.
Long-lasting somatosensory disturbances are prominent comorbidities in children with MOGAD. Prospective cohorts are required to identify molecular and immunogenetic profiles associated with somatosensory problems in MOGAD.
• Recurrence of demyelinating events occurs in a group of children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
• Long-lasting headache and somatosensory problems are frequent comorbidities with pediatric MOGAD. Pain and somatosensory problems may persist for more than 5 years. • Neuroimaging data do not indicate specific findings in children with somatic disturbances.
研究本单位获得性脱髓鞘综合征(ADS)患儿的临床特征。
回顾性分析 2001 年 1 月 1 日至 2022 年 3 月 31 日在九州大学医院就诊的患儿的临床资料。纳入标准为年龄<18 岁,在我院被诊断为 ADS 的患儿。采用细胞检测法检测患儿血清或脑脊液中的 MOGA 抗体。统计分析 MOGA 阳性和 MOGA 阴性患儿的临床和神经影像学资料。
共纳入 31 例患儿,其中 22 例行 MOGA 抗体检测,13 例(59%)MOGA 抗体阳性,诊断为 MOGA 相关疾病(MOGAD);9 例(41%)MOGA 抗体阴性。MOGAD 组和 MOGA 抗体阴性组在发病年龄、性别、诊断亚类和随访时间方面均无差异。MOGAD 组头痛和(或)感觉症状较 MOGA 抗体阴性组更常见(12/13 [92%] vs. 3/9 [22%];p=0.0066)。感觉障碍包括左脚趾持续性疼痛伴感觉过敏、会阴感觉异常和面部感觉减退。MOGAAD 或感觉症状均无特定的神经影像学表现。
MOGAD 患儿存在持久的感觉障碍,这是其常见的共病。需要前瞻性队列研究来确定与 MOGAD 感觉障碍相关的分子和免疫遗传学特征。