MRI Findings in Autoimmune Voltage-Gated Potassium Channel Complex Encephalitis with Seizures: One Potential Etiology for Mesial Temporal Sclerosis
MRI Findings in Autoimmune Voltage-Gated Potassium Channel Complex Encephalitis with Seizures: One Potential Etiology for Mesial Temporal Sclerosis
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DOI：
10.3174/ajnr.a3633
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发表时间：
2014-01-01
影响因子：
3.5
通讯作者：
Klein, C. J.
中科院分区：
文献类型：
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作者：
Kotsenas, A. L.;Watson, R. E.;Klein, C. J.
电压门控钾通道复合体脑炎是一种常见的自身免疫性脑炎，表现为顽固性癫痫发作，可能对免疫治疗有反应。这些作者回顾了 42 名此类患者的影像学表现，发现最常见的异常是内侧颞叶增大、T2 高信号、对比增强和弥散受限，随后出现海马萎缩。由于该疾病可以通过电压门控钾通道复合物抗体的存在轻松确诊，因此这些影像学检查结果应提示进行血清学检测。背景和目的：自身免疫性电压门控钾通道复合物脑炎是自身免疫性脑炎的常见形式。因这种脑炎引起癫痫发作的患者通常患有难治性癫痫，可能需要免疫治疗来控制癫痫发作。放射科医生必须认识到此类自身免疫性脑炎的影像学特征，并在鉴别诊断中提出建议，因为这种癫痫发作的病因可能尚未得到充分认识。我们的目的是表征该患者群体的 MR 成像结果。 材料和方法：对 42 名患有癫痫和电压门控钾通道复合物自身抗体血清阳性的患者（22 名男性；中位年龄，56 岁；年龄范围，8-79 岁）进行回顾性鉴定，评估 MR 成像的内侧和颞外肿胀和/或萎缩、T2 高信号、弥散受限和增强情况。进行统计分析。结果：42例患者中的33例（78.6%）在某个时间点表现出内侧颞叶结构增大和T2高信号。随访影像学检查时常发现内侧颞叶硬化（16/33，48.5%）。 9 名患者中的 6 名 (66.7%, P = .11) 最初表现出海马增强，8/13 (61.5%, P = .013) 表现出海马受限扩散进展为内侧颞叶硬化。相反，33 名患者中有 6 名异常影像学结果得到解决。结论：自身免疫性电压门控钾通道复合体脑炎在急性期常表现为内侧颞叶结构增大、T2 高信号、强化和弥散受限。识别这些典型的影像学表现可能有助于促进血清学诊断，防止不必要的侵入性操作并促进早期开始免疫治疗。连续 MR 成像可以证明放射学变化的消退或进展，包括涉及对侧的变化的发展和内侧颞叶硬化的频繁发展。
Voltage-gated potassium channel complex encephalitis is a common form of autoimmune encephalitis presenting with intractable seizures that may respond to immunotherapy. These authors reviewed the imaging findings in 42 such patients and the most common abnormalities noted were enlargement, T2 hyperintensity, contrast enhancement, and restricted diffusion in the mesial temporal lobes with subsequent development of hippocampal atrophy. Because the disease can be confirmed easily by the presence of voltage-gated potassium channel complex antibodies, these imaging findings should prompt serologic testing.BACKGROUND AND PURPOSE: Autoimmune voltage-gated potassium channel complex encephalitis is a common form of autoimmune encephalitis. Patients with seizures due to this form of encephalitis commonly have medically intractable epilepsy and may require immunotherapy to control seizures. It is important that radiologists recognize imaging characteristics of this type of autoimmune encephalitis and suggest it in the differential diagnosis because this seizure etiology is likely under-recognized. Our purpose was to characterize MR imaging findings in this patient population.MATERIALS AND METHODS: MR imaging in 42 retrospectively identified patients (22 males; median age, 56 years; age range, 8-79 years) with seizures and voltage-gated potassium channel complex autoantibody seropositivity was evaluated for mesial and extratemporal swelling and/or atrophy, T2 hyperintensity, restricted diffusion, and enhancement. Statistical analysis was performed.RESULTS: Thirty-three of 42 patients (78.6%) demonstrated enlargement and T2 hyperintensity of mesial temporal lobe structures at some time point. Mesial temporal sclerosis was commonly identified (16/33, 48.5%) at follow-up imaging. Six of 9 patients (66.7%, P = .11) initially demonstrating hippocampal enhancement and 8/13 (61.5%, P = .013) showing hippocampal restricted diffusion progressed to mesial temporal sclerosis. Conversely, in 6 of 33 patients, abnormal imaging findings resolved.CONCLUSIONS: Autoimmune voltage-gated potassium channel complex encephalitis is frequently manifested as enlargement, T2 hyperintensity, enhancement, and restricted diffusion of the mesial temporal lobe structures in the acute phase. Recognition of these typical imaging findings may help prompt serologic diagnosis, preventing unnecessary invasive procedures and facilitating early institution of immunotherapy. Serial MR imaging may demonstrate resolution or progression of radiologic changes, including development of changes involving the contralateral side and frequent development of mesial temporal sclerosis.