van Iterson Loretta, Vrij Suzanne, Sie Lilian T L, Augustijn Paul B, Rooze Anne C S, Jansen Floor E
SEIN Stichting Epilepsie Instellingen Nederland, Department of Psychology, The Netherlands.
Department of Neurology, Heemstede, The Netherlands.
Epilepsy Behav Rep. 2021 Jun 11;16:100465. doi: 10.1016/j.ebr.2021.100465. eCollection 2021.
Acquired visual agnosia in the context of continuous spikes and waves during slow sleep (CSWS) is rarely described. We present a case of an almost 7-year-old boy who lost his ability to name pictures and recognize familiar faces. Initial encephalography (EEG) revealed sleep induced epileptiform activity with a spike-wave index (SWI) of 100%, predominanting in the left posterior head region.
Serial neuropsychological testing with concomitant EEG was done during the first 18 months of treatment with intravenous methylprednisolone. We administered intelligence scales, verbal tasks (memory, fluency), visual tasks (drawings, search, face recognition), and tasks requiring visual-verbal integration (picture naming, visual closure).
Neuropsychological recovery studied with reliable cognitive change cut-offs and 95% confidence intervals.
With treatment, there was an improvement of the EEG pattern (SWI reduction to 45%), followed by a relapse (SWI 82%). Neuropsychological measures in part synchronized with improvement, stability, and fluctuating values. Significant increases were seen on Verbal Comprehension Index and semantic memory. Visual Spatial Index remained unchanged (67 to 73). Naming pictures showed only limited change. Interpreting degraded pictures remained extremely difficult.
Acquired visual agnosia may be seen in children with CSWS. Early recognition, prompt accurate treatment and tailored neuropsychological assessment remain crucial.
在慢波睡眠期持续棘慢波（CSWS）背景下获得性视觉失认症鲜有报道。我们报告一例近7岁男孩，他丧失了命名图片和识别熟悉面孔的能力。最初的脑电图（EEG）显示睡眠诱发的癫痫样活动，棘慢波指数（SWI）为100%，主要集中在左后头部区域。
在静脉注射甲泼尼龙治疗的前18个月期间，进行了连续的神经心理学测试并同步记录脑电图。我们使用了智力量表、言语任务（记忆、流畅性）、视觉任务（绘画、搜索、人脸识别）以及需要视觉-言语整合的任务（图片命名、视觉闭合）。
采用可靠的认知变化临界值和95%置信区间研究神经心理学恢复情况。
随着治疗，脑电图模式有所改善（SWI降至45%），随后复发（SWI 82%）。神经心理学测量结果部分与改善、稳定和波动值同步。言语理解指数和语义记忆显著增加。视觉空间指数保持不变（67至73）。图片命名仅显示有限变化。解读退化图片仍然极其困难。
CSWS患儿可能出现获得性视觉失认症。早期识别、及时准确的治疗以及量身定制的神经心理学评估仍然至关重要。
