The Fault in their stars - Accumulating astrocytic inclusions associated with clusters of epileptic spasms in children with global developmental delay

Publication date: Available online 18 April 2017
Source:Pediatric Neurology
Author(s): Robyn Whitney, Sameer AlMehmadi, Bláthnaid McCoy, Ivanna Yau, Ayako Ochi, Hiroshi Otsubo, Shelly K. Weiss, James Rutka, Lili-Naz Hazrati, O. Carter Snead, Cristina Go
BackgroundThe presence of cerebral astrocytic inclusions recently has been described in a subset of children with early-onset refractory epilepsy, with or without structural brain malformations and varying degrees of developmental delay.MethodsWe report two new cases of epilepsy with astrocytic inclusions that suggests that in some children this disorder may represent a unique hemispheric epilepsy. We make reference to previously reported cases of epilepsy with astrocytic inclusions.ResultsTwo children with early onset epilepsy with astrocytic inclusions had refractory clusters of epileptic spasms, developmental delay, abnormal neuroimaging, and an electroencephalogram that revealed hemispheric or diffuse interictal epileptiform discharges. In both cases, the initial focal resection of the putative epileptogenic zone was unsuccessful and pathology failed to show astrocytic inclusions. Subsequently, functional hemispherectomy was performed in both children due to ongoing clusters of epileptic spasms and the presence of multilobar astrocytic inclusions was demonstrated. Post-operatively, both children have remained seizure free in the short-term with improved development.ConclusionsWe importantly highlight that functional hemispherectomy may be required for seizure control in a select subset of children with clusters of epileptic spasms, astrocytic inclusions and global developmental delay. Given the small number of cases published however, ongoing collaboration is needed to better understand the pathophysiology of this condition and determine the optimal way to diagnose and manage these children.



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