Epileptic spasms in older children: persistence beyond infancy

Area of Science:

• Epilepsy and Neurological Disorders
• Pediatric Neurology
• Clinical Neurophysiology

Background:

• Infantile spasms (IS) are a recognized epileptic syndrome.
• Recent proposals suggest classifying spasms as a distinct seizure type characterized by axial jerks in clusters.
• The persistence of spasms beyond infancy in older children with intractable epilepsy is not well-documented.

Purpose of the Study:

• To investigate the occurrence and characteristics of spasms in older children with intractable mixed seizure disorders.
• To describe the clinical and electrophysiological features of spasms persisting beyond infancy.
• To evaluate treatment outcomes in children with persistent spasms.

Main Methods:

• Retrospective analysis of video-electroencephalography (EEG) monitoring data from five older children (4.5-14.2 years) with intractable mixed seizure disorder.
• Detailed review of seizure semiology, EEG findings (ictal and interictal), and treatment responses.
• Seizure onset and persistence from infancy were documented.

Main Results:

• All five children experienced spasms in infancy that continued into older childhood.
• Spasms were characterized by axial flexion/extension jerks in clusters, with specific ictal EEG patterns (delta burst followed by background attenuation/decrease with superimposed rhythmic activity).
• Multiple other seizure types were present, and interictal EEGs were markedly abnormal.
• Two children showed no response to ACTH treatment; three underwent corpus callosotomy.

Conclusions:

• Spasms can occur in older children with intractable mixed seizure disorders.
• Spasms may persist beyond infancy, presenting as a distinct seizure type.
• These findings highlight the importance of recognizing persistent spasms and considering appropriate management strategies.