Efficacy Of Methylprednisolone Therapy Arid Follow-up Study Of19Cases With Electrical Status Epilepticus During Slow Sleep

Background and objective: electrical status epilepticus during slow sleep (ESES)is an electroencephalogram(EEG) phenomenon characterized by continuous (ornear-continuous) spike-wave discharges during non-rapid eye movement (NREM)sleep. ESES is most often associated with an encephalopathy (ESES syndrome)characterized by regression of cognitive function. ESES is not a specific epilepsysyndrome, but some of epilepsy syndromes can be associated with the ESESphenomena, for instance Benign childhood Epilepsy with Centrotemporal Spikes,Continuous Spike wave during slow Wave Sleep and Landau-Kleffner Syndrome andso on. There are anti-epileptic drugs, corticosteroid therapy, surgery and so on that cancure the EEG phenomena, but we believe that the corticosteroid therapy is most safeand effective. Recently, the regression of cognitive function is with more and moreattention, and the pathophysiological mechanisms of ESES phenomenon is still notclear. In order to improve the understanding of the phenomenon and the prognosis ofchildren with ESES, to find the appropriate treatment choice, we selected19cases withESES, who have methylprednisolone therapy. The clinical data were retrospectivelyanalyzed.Method: We have collected all clinical data of19cases with ESES fromDecember2011to June2013in pediatric department of the first hospital of Ji LinUniversity, who have methylprednisolone therapy. The data contain gender, age,personal history, family history, MRI of head, seizure types, seizure frequencies, drugsused before, EEG, age of found seizure, duration of ESES.In one month after thetreatment, examinations of EEG have been collected. Evaluations on cognitivefunctions were conducted in6and12months after the treatment, and with examinations of EEG. The adverse reactions, seizure types and seizure frequencies arecollected. All clinical data were retrospectively analyzed.Results: There were2symptomatic and17cryptogenic cases. Mean ESESduration before corticosteroid treatment was1year and2months.14patients fulfilledcriteria of BECTS,2patients fulfilled criteria of Doose syndrome,1patient fulfilledcriteria of LKS,1patient fulfilled criteria of LGS,1case was symptomatic and didn’tfulfill criteria of anyone. Positive response to steroids was found during the first1month of treatment in10of19patients (52.6%), including9cases of BECTS,1caseof LKS. The response to steroids during the first6months of treatment is same as theformer. Positive response to steroids was found during the first12months of treatmentin9of19patients (47.4%), including9cases of BECTS. One of them relapsed, whofulfilled criteria of LKS.In12cases between6and16years of age, who fulfilled criteria of BECTS,positive response to steroids was highly significantly associated with higher IQ whichwas conducted in6and12months after the treatment, less previous AEDs trials andless seizure types. Shorter ESES duration, but not age, age of found seizure, wasassociated with positive response to steroids.Cases in others, including2cases of BECTS,1case of LKS,1case of LGS,2cases of Doose syndrome,1case of symptomatic ones (unknown-ESES), wereretrospectively analyzed. In6and12months after the treatment, all cases of BECTShave improved cognitive function, the LKS had better cognitive function in6monthsafter the treatment and had worse one in12months after the treatment, the others haveno improvement.Effective rate of all cases of BECT is92.9%in6and12months after thetreatment. Attack control ineffectively in1case of LGS,1case of Doose syndrome and1case of symptomatic ones(unknown-ESES) in6and12months after the treatment.Attack control effectively in1case of Doose syndrome in6and12months after thetreatment. The case of LKS attack control in6months after the treatment, ineffectivein12months after the treatment.The side effects of steroids occurred in all patients, including weight gain, Cushing’s Face, Infection and so on. After discontinuation of the treatment, the sideeffects have disappeared. The usual side effects of steroids, like weight gain andCushing’s Face, occurred in all patients when they were treated with hydrocortisone.Conclude: Not only BECTS, LKS, LGS and CSWS, but also the symptomaticones and Doose syndrome can be associated with epilepsy syndromes. In our study,positive response to steroids was found in some epilepsy syndromes, like the BECTSand the LKS, who has better cognitive functions after treatment. The others are poorresponse, including the LGS, the Doose syndrome and the symptomatic ones, whoreceive lower IQ after treatment. Positive responders are patients with short duration ofESES and receive higher IQ after treatment in all cases of BECTS. The usual sideeffects of steroids, like weight gain and Cushing’s Face, occurred in all patients whenthey were treated with hydrocortisone.