Department of Neurology, the First Affiliated Hospital of Kunming Medical University, Wuhua District, Kunming, China.
Trauma Center, the First Affiliated Hospital of Kunming Medical University, Wuhua District, Kunming, China.
Medicine (Baltimore). 2023 May 19;102(20):e33767. doi: 10.1097/MD.0000000000033767.
Recurrent herpes simplex encephalitis (HSE) can easily induce autoimmune encephalitis (AE). However, there are few reports of anti-contactin-associated protein-2 (CASPR2)-related encephalitis, especially with positive anti-aquaporin 4 (AQP4) antibodies.
A 14-year-old boy was admitted to the Department of Neurology of the First Affiliated Hospital of Kunming Medical University for "headache, dizziness, and fever for four days" with positive anti-CASPR2 and anti-AQP4 antibodies in the cerebrospinal fluid.
Cranial MRI showed lesions in the right hippocampus, amygdala, and insular lobe, with local sulcus enhancement in the right insular, temporal, and frontal lobes. The fluid-attenuated inversion recovery was significantly enhanced. Human herpes virus type I was detected by cerebrospinal fluid metagenomic testing. The patient was diagnosed with AE secondary to HSE, with positive anti-CASPR2 and anti-AQP4 antibodies.
After 2 weeks of immunoglobulin and methylprednisolone immunomodulatory therapy, acyclovir antivirus, mannitol dehydration, reducing intracranial pressure, and other symptomatic support therapy.
The patient's symptoms significantly improved, with no complaints of discomfort, and he was discharged for observation. The patient was followed up a month after discharge and had no complaints of discomfort.
CASPR2 and anti-aquaporin-4 antibody-positive AE have not been reported to be positive. This case will raise awareness of CASPR2 and anti-aquaporin-4 antibody-positive AE secondary to HSE, strengthen diagnostic capacities, and provide advice to treat it.
复发性单纯疱疹脑炎(HSE)易诱发自身免疫性脑炎(AE),但抗接触蛋白相关蛋白-2(CASPR2)相关脑炎少见报道,尤其合并抗水通道蛋白 4(AQP4)抗体阳性者。
14 岁男孩因“头痛、头晕伴发热 4 天”入住昆明医科大学第一附属医院神经内科,脑脊液抗 CASPR2、抗 AQP4 抗体阳性,头颅 MRI 示右侧海马、杏仁核及岛叶异常信号,右侧岛叶、颞叶、额叶局部脑沟强化,液体衰减反转恢复序列明显高信号,脑脊液宏基因组二代测序示人类疱疹病毒 1 型阳性,诊断为 HSE 继发的 AE,伴抗 CASPR2、抗 AQP4 抗体阳性。
予免疫球蛋白及甲泼尼龙免疫调节治疗,阿昔洛韦抗病毒、甘露醇脱水降颅压等对症支持治疗。
患者症状明显好转,无不适主诉,出院观察。出院后 1 个月随访患者无不适主诉。
该病例提示 CASPR2 及抗 AQP4 抗体阳性的 AE 尚未见报道,本例将提高对 HSE 继发 CASPR2 及抗 AQP4 抗体阳性 AE 的认识,增强诊断能力,并为治疗提供建议。