Ismail Ismail Ibrahim, Alnaser Fahad, Al-Hashel Jasem Y
Department of Neurology, Ibn Sina Hospital, Sabah Medical Region, Kuwait.
Department of Radiology, Ibn Sina Hospital, Sabah Medical Region, Kuwait.
J Med Case Rep. 2021 Mar 24;15(1):130. doi: 10.1186/s13256-021-02742-4.
Limbic encephalitis (LE), a variant of autoimmune encephalitis, is inflammation of the limbic system of the brain. The disorder presents with subacute impairment of short-term memory, psychiatric manifestations, confusion and seizures. "Seronegative LE" is a challenging diagnosis in the absence of well-characterized autoantibodies.
A 33-year-old Kuwaiti woman with no relevant past history presented with subacute progressive amnesia of 6-month duration. Magnetic resonance imaging (MRI) showed bilateral hyperintensity of the limbic structures. An extensive workup of the blood and cerebrospinal fluid (CSF) failed to identify paraneoplastic or autoimmune antibodies. The diagnosis of seronegative LE was made, and immunotherapy was initiated, with improvement in cognitive function.
Seronegative LE is a challenging diagnosis. Inability to detect autoantibodies, especially early in the disease course, should not rule out the diagnosis of autoimmune encephalitis. Early diagnosis and treatment with immunotherapy may prevent irreversible brain damage.
边缘叶脑炎（LE）是自身免疫性脑炎的一种变体，是大脑边缘系统的炎症。该病症表现为短期记忆的亚急性损害、精神症状、意识模糊和癫痫发作。在缺乏特征明确的自身抗体的情况下，“血清阴性LE”是一种具有挑战性的诊断。
一名33岁无相关既往病史的科威特女性，出现了持续6个月的亚急性进行性失忆。磁共振成像（MRI）显示边缘叶结构双侧高信号。对血液和脑脊液（CSF）进行的广泛检查未能发现副肿瘤或自身免疫抗体。诊断为血清阴性LE，并开始免疫治疗，认知功能有所改善。
血清阴性LE是一种具有挑战性的诊断。无法检测到自身抗体，尤其是在病程早期，不应排除自身免疫性脑炎的诊断。早期诊断并采用免疫疗法进行治疗可预防不可逆的脑损伤。
