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持续性顺行性遗忘症伴边缘性脑炎和电压门控钾通道复合物抗体相关。

Persistent anterograde amnesia following limbic encephalitis associated with antibodies to the voltage-gated potassium channel complex.

机构信息

Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, , Oxford, UK.

出版信息

J Neurol Neurosurg Psychiatry. 2014 Apr;85(4):387-91. doi: 10.1136/jnnp-2013-306724. Epub 2014 Jan 8.

Abstract

OBJECTIVE

Limbic encephalitis (LE) associated with antibodies to the voltage-gated potassium channel complex (VGKC) is a potentially reversible cause of cognitive impairment. Despite the prominence of cognitive dysfunction in this syndrome, little is known about patients' neuropsychological profile at presentation or their long-term cognitive outcome.

METHODS

We used a comprehensive neuropsychological test battery to evaluate cognitive function longitudinally in 19 patients with VGKC-LE.

RESULTS

Before immunotherapy, the group had significant impairment of memory, processing speed and executive function, whereas language and perceptual organisation were intact. At follow-up, cognitive impairment was restricted to the memory domain, with processing speed and executive function having returned to the normal range. Residual memory function was predicted by the antibody titre at presentation.

CONCLUSIONS

The results show that, despite broad cognitive dysfunction in the acute phase, patients with VGKC-LE often make a substantial recovery with immunotherapy but may be left with permanent anterograde amnesia.

摘要

目的

与电压门控钾通道复合物(VGKC)抗体相关的边缘性脑炎(LE)是认知障碍的一种潜在可逆转病因。尽管该综合征以认知功能障碍为突出表现,但对患者发病时的神经心理学特征或其长期认知结局知之甚少。

方法

我们使用综合神经心理学测试工具包对 19 例 VGKC-LE 患者进行了纵向认知功能评估。

结果

免疫治疗前,该组患者存在记忆、加工速度和执行功能的显著损害,而语言和知觉组织完整。随访时,认知障碍仅限于记忆域,加工速度和执行功能已恢复至正常范围。发病时的抗体滴度可预测残留的记忆功能。

结论

结果表明,尽管在急性期存在广泛的认知功能障碍,但 VGKC-LE 患者经免疫治疗后通常会有明显的恢复,但可能会遗留永久性顺行性遗忘。

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