Syndromic Classification in the Management of Childhood Epilepsy

1994 ◽  
Vol 9 (2_suppl) ◽  
pp. 2S14-2S18 ◽  
Author(s):  
Jean Aicardi

Epilepsy syndromes, defmed as clusters of symptoms or signs occurring consistently together, form the basis of the currently accepted classification of the epilepsies. The concept of epilepsy syndrome is practical for the diagnosis, prognosis, orientation of treatment, and selection of appropriate investigations, but it is of variable specificity and usually does not give information on causes and mechanisms of an epilepsy. Some syndromes, such as childhood absences, partial epilepsy with centrotemporal spikes, or juvenile myoclonic epilepsy, are precisely characterized while other syndromes such as grand mal on awakening or the multiple syndromes with myoclonic seizures are poorly delineated. The usefulness of the concept is limited to well-defined and generally accepted syndromes and many cases of epilepsy do not fit easily in recognizable syndromes. (J Child Neurol 1994; 9(Suppl):2S14-2S18).

Author(s):  
Mohammad Abu-Hegazy ◽  
Azza Elmoungi ◽  
Eman Eltantawi ◽  
Ahmed Esmael

Abstract Background Electrophysiological techniques have been used for discriminating myoclonus from other hyperkinetic movement disorders and for classifying the myoclonus subtype. This study was carried out on patients with different subtypes of myoclonus to determine the electrophysiological characteristics and the anatomical classification of myoclonus of different etiologies. This study included 20 patients with different subtypes of myoclonus compared with 30 control participants. Electrophysiological study was carried out for all patients by somatosensory evoked potential (SSEP) and electroencephalography (EEG) while the control group underwent SSEP. SSEP was evaluated in patients and control groups by stimulation of right and left median nerves. Results This study included 50 cases with myoclonus of different causes with mean age of 39.3 ± 15.7 and consisted of 23 males and 27 females. Twenty-nine (58%) of the patients were epileptics, while 21 (42%) were non-epileptics. Cases were classified anatomically into ten cases with cortical myoclonus (20%), 12 cases with subcortical myoclonus (24%), and 28 cases with cortical–subcortical myoclonus (56%). There was a significant difference regarding the presence of EEG findings in epileptic myoclonic and non-epileptic myoclonic groups (P = 0.005). Also, there were significant differences regarding P24 amplitude, N33 amplitude, P24–N33 peak-to-peak complex amplitude regarding all types of myoclonus. Primary myoclonic epilepsy (PME) demonstrated significant giant response, juvenile myoclonic epilepsy (JME) demonstrated no enhancement compared to controls, while secondary myoclonus demonstrated lower giant response compared to PME. Conclusion Somatosensory evoked potential and electroencephalography are important for the diagnosis and anatomical sub-classification of myoclonus and so may help in decision-making regarding to the subsequent management.


2017 ◽  
Vol 66 ◽  
pp. 34-38 ◽  
Author(s):  
Nashi Saraswati ◽  
Chetan Nayak ◽  
Sanjib Sinha ◽  
Madhu Nagappa ◽  
Kandavel Thennarasu ◽  
...  

2016 ◽  
Vol 53 (3) ◽  
pp. 272-275 ◽  
Author(s):  
Zeynep Aydin Ozemir ◽  
Emel Oguz Akarsu ◽  
Zeliha Matur ◽  
Ali Emre Oge ◽  
Betul Baykan

2011 ◽  
Vol 36 (5) ◽  
pp. 2705-2711 ◽  
Author(s):  
Imran Goker ◽  
Onur Osman ◽  
Serhat Ozekes ◽  
M. Baris Baslo ◽  
Mustafa Ertas ◽  
...  

2021 ◽  
Vol 13 (3) ◽  
pp. 237-248
Author(s):  
K. V. Petrov ◽  
E. Yu. Mozheyko ◽  
N. A. Shnayder ◽  
M. M. Petrova ◽  
E. A. Narodova

The International Classification of Functioning, Disability and Health (ICF) is a globally recognized classification for health components and health-related factors, which has been recommended by the World Health Organization for use in medical rehabilitation. In the practice of a rehabilitation doctor dealing with patients suffering from juvenile myoclonic epilepsy (JME), the ICF is used to establish a rehabilitation diagnosis and formulate the goal of rehabilitation, as well as to implement its basic principles. It provides better insight into a range of difficulties patients with JME might face at the level of personal health and in everyday life.


Author(s):  
Sami F. Abdalla ◽  
Hanan B. Eltahir ◽  
Salah M. El-Magzoub

Introduction: Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epilepsy syndrome (IGE) with a strong genetic contribution. The main characters of JME are generalized convulsive or an absences seizures proceeded by myoclonic jerks. Gene variant of gamma-aminobutyric acid type A inhibitory receptor speculated to underlay JME etiology. Objective: This study aimed to screen for JME based on the International League against Epilepsy Commission on Classification and Terminology diagnostic criteria and to assess the link of polymorphism in the GABAA receptor gene, GABRA1 to the development of JME in Sudanese patients. Methods: Our epidemiological study enrolled 44 JME patients, only 23 participated in the genetic part and 35 matched healthy controls were also included. Blood genomic DNA was isolated and PCR based- restriction fragment length polymorphism (RFLP) analysis was done. The data obtained were analyzed using computer software SPSS 21rt edition. Results: The frequency of the mutant G allele was found to be 41.5% in patients and 45.5% in the controls. The genotype distribution of A and G alleles among patients were found to be (AA= 39%), (AG=39%) and (GG= 22%) and that of the controls were (AA=40%), (AG=30%) and (GG= 30%). Conclusion: The mutant G allele of the GABAA1 receptor does not affect the development of JME in Sudanese patients but the AG genotype may be a risk factor.


Sign in / Sign up

Export Citation Format

Share Document