Acquired visual agnosia as an uncommon presentation of epileptic encephalopathy in a 6-year-old boy with CSWS

Loretta van Iterson; Suzanne Vrij; Lilian T L Sie; Paul B Augustijn; Anne C S Rooze; Floor E Jansen

doi:10.1016/j.ebr.2021.100465

Acquired visual agnosia as an uncommon presentation of epileptic encephalopathy in a 6-year-old boy with CSWS

Epilepsy Behav Rep. 2021 Jun 11:16:100465. doi: 10.1016/j.ebr.2021.100465. eCollection 2021.

Authors

Loretta van Iterson¹, Suzanne Vrij², Lilian T L Sie³, Paul B Augustijn², Anne C S Rooze¹, Floor E Jansen^{4

5}

Affiliations

¹ SEIN Stichting Epilepsie Instellingen Nederland, Department of Psychology, The Netherlands.
² Department of Neurology, Heemstede, The Netherlands.
³ Department of Pediatric Neurology, Juliana Children's Hospital, Haga Teaching Hospital, The Hague, The Netherlands.
⁴ Department of Child Neurology. Brain Center, University Medical Center Utrecht, Utrecht, The Netherlands.
⁵ Member of the European Reference Network EpiCARE.

Abstract

Background: 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.

Methods: 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).

Analyses: Neuropsychological recovery studied with reliable cognitive change cut-offs and 95% confidence intervals.

Results: 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.

Discussion: Acquired visual agnosia may be seen in children with CSWS. Early recognition, prompt accurate treatment and tailored neuropsychological assessment remain crucial.

Keywords: Acquired visual agnosia; ESES; Occipito-temporal CSWS; Visual epileptic encephalopathy children.

Publication types

Case Reports