Children's Hospital at Westmead, Sydney 2145, Australia.
Neurology. 2011 Apr 5;76(14):1252-5. doi: 10.1212/WNL.0b013e3182143552.
Voltage-gated potassium channel antibodies (VGKC Ab) are associated with limbic encephalitis and neuromyotonia in adults. There have been no systematic investigations in children to date.
We looked for antibodies that are associated with CNS syndromes in adults including antibodies to VGKCs, NMDARs, glutamic acid decarboxylase (GAD), and glycine receptor (GlyR) in the stored acute serum from 10 children with unexplained encephalitis presenting with encephalopathy and status epilepticus. We also looked for antibodies to leucine-rich glioma-inactivated 1 (Lgi1) and contactin-associated protein-like 2 (Caspr2), which are now known to be tightly complexed with VGKCs in vivo. Sixty-nine pediatric controls were used for comparison.
An elevated VGKC Ab (>100 pM) was detected in 4/10 patients with encephalitis compared to only 1/69 controls (p < 0.001). The outcome in the 4 VGKC Ab-positive patients with encephalitis was variable including good recovery (n = 1), cognitive impairment (n = 3), temporal lobe epilepsy (n = 2), and mesial temporal sclerosis (n = 1). No other antibodies were detected, including those to Lgi1 and Caspr2.
Encephalitis associated with VGKC Ab occurs in children and presents with status epilepticus and focal epilepsy. These antibodies are not directed against Lgi1 or Caspr2.
电压门控钾通道抗体（VGKC Ab）与成人的边缘性脑炎和神经肌强直有关。迄今为止，尚未对儿童进行系统调查。
我们在 10 名患有不明原因脑炎的儿童的急性血清中寻找与成人中枢神经系统综合征相关的抗体，包括与 VGKCs、NMDARs、谷氨酸脱羧酶（GAD）和甘氨酸受体（GlyR）相关的抗体。我们还寻找与亮氨酸丰富的胶质瘤失活 1（Lgi1）和接触蛋白相关蛋白 2（Caspr2）相关的抗体，这些抗体现在已知在体内与 VGKCs 紧密结合。69 名儿科对照用于比较。
与 69 名对照组中的 1 名（p < 0.001）相比，在 10 名脑炎患者中有 4 名（40%）检测到 VGKC Ab（>100 pM）升高。4 名脑炎伴 VGKC Ab 阳性患者的预后各不相同，包括完全康复（n = 1）、认知障碍（n = 3）、颞叶癫痫（n = 2）和海马硬化（n = 1）。未检测到其他抗体，包括针对 Lgi1 和 Caspr2 的抗体。
儿童发生与 VGKC Ab 相关的脑炎，表现为癫痫持续状态和局灶性癫痫。这些抗体不是针对 Lgi1 或 Caspr2 的。
