An Update to Approach to the Childhood Electroclinical Syndromes
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REVIEW ARTICLE
An Update to Approach to the Childhood Electroclinical Syndromes Pratibha Singhi 1 & Jitendra Kumar Sahu 2 Received: 17 December 2019 / Accepted: 20 October 2020 # Dr. K C Chaudhuri Foundation 2020
Abstract Childhood Electroclinical Syndrome (CES) is a term which refers to distinct epilepsies of childhood which have peculiar similarities such as the age of onset, seizure semiology, EEG and prognosis. With advancements in the diagnostics and genetics, pediatric epilepsy is entering in the age of precision medicine. The present paper provides an update of CES in light of recent advances in the terminologies and classifications by International League Against Epilepsy and genetic underpinnings of epilepsy. The core features of CES for diagnosing and managing some common CES is presented here. Keywords Childhood Electroclinical Syndrome . Pediatric epilepsy . Approach
Epilepsy constitutes one of the most common causes of chronic neurological disorders of childhood. With advancement in diagnostics and genetics, pediatric epilepsy is entering the age of precision medicine. The International League Against Epilepsy (ILAE) has updated the various terminologies and classification of seizures and epilepsies [1]. The present paper provides an update of previous publication in light of recent advances by ILAE and genetic underpinnings of epilepsy [2]. Childhood Electroclinical Syndrome (CES) is a term which refers to distinct epilepsies of childhood which have peculiar similarities such as the age of onset, seizure semiology, electroencephalogram (EEG) and prognosis [2]. Basic knowledge of CES helps the treating physician holistically managing the child, as well as addressing prognostic concerns of parents. It is important to note that the concept of focal and generalized does not apply to CES. The core features of CES for diagnosing and managing some common CES is presented here. Further reading is suggested for indepth details of CES, which is beyond the purview of this article. It is important to understand that many children with epilepsy do not fulfil the precise diagnosis of CES and need
to be described with epilepsy classification based on seizure type and etiology. Furthermore, there are distinctive constellations, based on the lesion and peculiar clinical features, but these do not qualify for ECS e.g., Gelastic seizures with hypothalamic hamartoma, Mesial temporal lobe epilepsy with hippocampal sclerosis, Rasmussen syndrome, and Hemiconvulsion–hemiplegia–epilepsy [3]. The classification of epilepsy and epileptic syndromes provides the basic foundation for such an approach (Table 1). Most CES are characterized by distinct age predilection though there may be some variations. Children who have neurodevelopmental impairments usually have different CES than developmentally normal children. Hence one needs to take note of the following three key factors for presumptively diagnosing a CES: (i) age of the child, (ii) neurodevelopmental status, (iii) seizure semiology, and (iv) routine or sleep deprived EE
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