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Childhood and Adolescent Epileptic Syndromes

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Clinical Child Neurology

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Abstract

Classification of epilepsies and their organization into epilepsy syndromes are primarily for clinical purposes since it influences every clinical consultation. It also forms the foundation of choosing the appropriate anti-seizure medication(s) and is vital for clinical and basic epilepsy research, as well as the development of novel therapies [1]. A specific epilepsy syndrome can usually be recognized by taking together the typical age of seizure onset, specific seizure types, and electroencephalography (EEG) characteristics. Appropriate early diagnosis of the epilepsy syndrome also helps in avoiding the use of antiepileptic drugs (AED) known to aggravate seizures in a specific epilepsy syndrome.

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Authors and Affiliations

  1. Medeor 24×7 Hospital, Abu Dhabi, UAE

    Imad Y. Saadeldin

  2. Division of Clinical Neurophysiology, Department of Neuroscience, Prince Sultan Medical City, Riyadh, Saudi Arabia

    Mohammad M. Kabiraj

  3. Division of Pediatric Neurology, Department of Pediatrics, College of Medicine and King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia

    Mustafa A. M. Salih

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  1. Imad Y. Saadeldin
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  2. Mohammad M. Kabiraj
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  3. Mustafa A. M. Salih
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Correspondence to Mustafa A. M. Salih .

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Editors and Affiliations

  1. College of Medicine Division of Pediatric Neurology, King Saud University, Riyadh, Saudi Arabia

    Mustafa A.M. Salih

29.1 Electronic Supplementary Material

Video EEG of a child who had epilepsy with myoclonic-atonic seizures. Interictal EEG shows generalized continuous slow (3–4 Hz) with frontal predominance and generalized spike and wave discharges at 2–3 Hz. Morphology of the discharge is first positive wave, then negative, and then deep positive followed by long slow wave without any clinical manifestation. Ictal EEG shows generalized slow waves with mild voltage attenuation and movement artifact, without prominent muscle artifact. The child had a sudden head drop and the seizure lasted for 3–4 s without loss of consciousness (MPG 7192 kb)

One of twin girls who have epilepsy with eyelid myoclonias. The video shows eyelid myoclonia which characterizes this syndrome and consists of marked jerking of the eyelids which are brief, often repetitive, and rhythmic (MP4 1024 kb)

The twin of the girl shown in Video 29.2, who has epilepsy with eyelid myoclonias, making grimaces to avoid eye closure phenomena. This can be mistaken for facial tics (MP4 1776 kb)

Five-year-4-month-old boy with Lennox-Gastaut syndrome with tonic seizure precipitated by an external stimulus (sudden noise). The child has severe cognitive delay. Notice the head scars from repetitive “drop attacks” (MP4 49127 kb)

Four-year-old girl with Lennox-Gastaut syndrome having a tonic seizure. The child maintained normal cognitive function at that time, and she was aware of the event, telling her father what happened to her (MP4 854 kb)

Four-year-old girl with Lennox-Gastaut syndrome who has frequent prolonged atypical absences (same patient as in Video 29.5) (MP4 32987 kb)

Five-year-4-month-old boy with Lennox-Gastaut syndrome (same patient as in Video 29.4), with atypical absence seizure (MP4 65067 kb)

Four-year-old girl with Lennox-Gastaut syndrome (same patient shown in Videos 29.5 and 29.6): Fragmented myoclonia of hands and body occurred after adding valproate to the patient which subsided after stopping it (MP4 1777 kb)

Four-year-old girl with Lennox-Gastaut syndrome (same patient shown in Videos 29.5 and 29.6): Fragmented myoclonia of hands and body occurred after adding valproate to the patient which subsided after stopping it (MP4 1179 kb)

Video EEG of a boy with absence seizures recorded while eating. The first seizure was preceded by chewing artifact (5 s); then generalized spike-slow wave discharges started. Then for a second, the boy continued chewing during the generalized discharges (automatism) without obvious muscle artifact. Onset frequency was 3.5–4 Hz and then smoothly slowed down to 3 Hz, the boy resumed his chewing, and EEG showed muscle artifacts. During the second seizure, epileptiform discharges started at little faster frequency, manifesting clinically a second later, and lasted for 3 s with smooth termination (MPG 3617 kb)

Eight-year-old boy with recurrent focal seizures, mostly during sleep but also during wake state with twitching of the angle of the mouth and salivation (MPG 10072 kb)

Nine-year-old boy with speech arrest, fear, and guttural noises (MP4 4788 kb)

Eight-year-7-month-old boy with focal, tonic-clonic seizure, sialorrhea, laryngeal/guttural noises, and vomiting (MP4 6995 kb)

Six-year-5-month-old boy with focal seizure evolving to a bilateral convulsion associated with guttural noises and sialorrhea (MP4 3179 kb)

Negative myoclonus in a boy with atypical childhood epilepsy with centrotemporal spikes leading to instability during standing (a) and sitting (b) and resulting in falling backward with consciousness entirely preserved (Courtesy of Dr. Ahlam A. Hamed, Faculty of Medicine, University of Khartoum) (MP4 8750 kb)

Negative myoclonus in a boy with atypical childhood epilepsy with centrotemporal spikes leading to instability during standing (a) and sitting (b) and resulting in falling backward with consciousness entirely preserved (Courtesy of Dr. Ahlam A. Hamed, Faculty of Medicine, University of Khartoum) (MP4 1595 kb)

Eleven-year-4-month-old girl with frequent hyperkinetic, bizarre seizures of short duration characteristic of autosomal-dominant nocturnal frontal lobe epilepsy (AVI 5685 kb)

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Saadeldin, I.Y., Kabiraj, M.M., Salih, M.A.M. (2020). Childhood and Adolescent Epileptic Syndromes. In: Salih, M.A. (eds) Clinical Child Neurology. Springer, Cham. https://doi.org/10.1007/978-3-319-43153-6_29

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  • DOI: https://doi.org/10.1007/978-3-319-43153-6_29

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