Rajbhandari Lily, Adhikari Prakriti, Nepali Anil
Department of Neurology, Kanti Children Hospital, Maharajgunj, Kathmandu, Nepal.
Department of Neurology, Kathmandu University School of Medical Sciences, Dhulikhel, Nepal.
Ann Med Surg (Lond). 2025 Aug 7;87(9):6148-6152. doi: 10.1097/MS9.0000000000003665. eCollection 2025 Sep.
Seronegative autoimmune encephalitis is a subgroup of encephalitis with suspected immunologic origin but with no identifiable pathogenic autoantibody in serum or cerebrospinal fluid (CSF).
A 14-year-old girl presented with features suggestive of catatonia and altered mental status without any previous medical or psychiatric history and was subsequently diagnosed with seronegative autoimmune encephalitis. The patient showed notable improvement after immunomodulators (methylprednisolone) and lorazepam were initiated.
The absence of typical presentation along with absence of detectable serum or cerebrospinal fluid antibodies highlights the diagnostic and therapeutic challenges of autoimmune encephalitis (AE) in pediatric patients.
The absence of detectable serum or cerebrospinal fluid antibodies does not exclude an autoimmune etiology when the clinical picture and supportive EEG findings are consistent with autoimmune encephalitis. Hence, autoimmune encephalitis should be suspected when presented with a rapid onset of psychological symptoms, and early and aggressive immunotherapy is crucial for favorable outcomes.
血清阴性自身免疫性脑炎是脑炎的一个亚组,其起源疑似免疫性,但血清或脑脊液(CSF)中未发现可识别的致病性自身抗体。
一名14岁女孩,无既往病史或精神病史,出现提示紧张症和精神状态改变的症状,随后被诊断为血清阴性自身免疫性脑炎。在开始使用免疫调节剂(甲泼尼龙)和劳拉西泮后,患者病情显著改善。
缺乏典型表现以及血清或脑脊液抗体检测不到,凸显了儿科患者自身免疫性脑炎(AE)的诊断和治疗挑战。
当临床表现和支持性脑电图结果与自身免疫性脑炎一致时,血清或脑脊液抗体检测不到并不排除自身免疫病因。因此,出现快速发作的心理症状时应怀疑自身免疫性脑炎,早期积极的免疫治疗对良好预后至关重要。