The Clinical Features And Treatment Of Landau-Kleffner Syndrome

Objective: to analyze the clinical characteristics of LKS in children,to follow up the prognosis and to analyze the prognostic factors.Methods: 40 cases of LKS July 2010-2016 year in February the hospital and follow-up treatment,a retrospective analysis of the clinical features of LKS cases,gene detection and EEG data;give the adrenocortical hormone(June treatment)and antiepileptic drugs(according to seizure type drug treatment),prospectively follow-up LKS case effect;establish the evaluation standard,were recorded in various clinical aphasia(onset,effective,complete recovery time),cognitive effect(invalid,improve and restore normal EEG)and follow-up(background activity,paroxysmal abnormality,the disappearance time of ESES);the Fisher exact probability of prognostic factors of single factor analysis.Results:(1)characteristics of aphasia: the aphasia: 20% of the expression barrier and 80% of hearing loss;Degree of aphasia: 52.5 percent complete aphasia,47.5% of partial aphasia;(2).Epileptic seizures occurred in 27 cases(n = 67.5%),of which,there were 18 cases(n = 66.7%)before aphasia,and in the other group(n = 59.3%),aphasia occurred in all of the patients(n = 40.7).The classification of epileptic seizures was as follows: a single episode occurred in all the patients(n = 66.7%),and multiple seizures occurred in all the patients(33.3%)(n = 5)(n = 10);(3)cognitive assessment: moderate abnormal,accounting for 64%;(4)EEG characteristics:(1)background activity: normal in 28 cases(70%),(2)mild slowing down in 32 cases(80%);(3)paroxysmal abnormality: in(ESES)in 10 cases(25%).2.prognostic analysis: a follow-up period of 3 weeks(n = 29),March(n = 28),in June(n = 26),in the year of December(n = 18),for a period of 2 years(n = 9),for a period of 3years(n = 6),over a period of 3 years;(1)the assessment of aphasia efficacy of aphasia: complete recovery in 22cases(55%),partially recovered in 16cases(40%),2 cases were lost;the time of onset time: the efficacy of aphasia in March accounted for 89.3% of the time;in June 77.3%;complete recovery within 1 years accounted for 80% of the time;3 cases of LKS repeated cases;(2)the curative effect of epilepsy: in LKS,there were epileptic seizures in 40 cases(control group)in 33 cases(n = 86.7%),and the improvement was found in 5 cases(13.3%);(3)cognitive follow-up: 2/3 children with cognitive impairment;(4)EEG follow-up: 10 cases showed slow background activity,can be returned to normal;the abnormal discharge in 37 cases,the abnormal discharge disappeared in 33 cases;the ESES disappear within 3 weeks,3 cases were repeated;3.analysis of prognostic factors: multiple factors have no statistical significance,but clinical data show that long duration age and ESES release and aphasia have poor prognosis.Conclusion: LKS clinical rare,its clinical features for further identification;hormone treatment of LKS cognitive outcome differences,in some cases fully recovered.In some cases the language improved,but there was other cognitive problems,a few cases had recurrent phenomenon;the long course of disease,age of onset,ESES release may influence prognosis;KD can be used as treatment choice for refractory epilepsy and can improve cognition.