| Objective: To explore clinical characteristics, therapy and prognosis of myoclonic epilepsy(ME), providing the theoretical evidence for diagnosis, treatment and prognosis judgement.Methods: The database of 47 children with myoclonic epilepsy diagnosed in Children's Hospital of Chongqing Medical University from January, 2003 to October, 2008 was reviewed, regarding the sex, age, age of onset, course of disease, clinical manifestation, etiological factor, EEG and treatment, of which 25 cases were followed up.Results: A total of 47 cases were taken into this study. Male children are 1.35 times of the female children. Within 5 years of age accounted for 78.72%. 42.55% of all had etiological factors, the major underlying causes were perinatal asphyxia, hypoxic-ischemic encephalopathy and brain dysplasia. The clinical seizure patterns of myoclonic epilepsy: Of 47 patients, 12 only with myoclonic jerks(MJ), 6 combined with tonic seizures(TS), 8 combined with generalized tonic clonic seizures(GTCS), 11 combined with general convulsive seizures(GCS), 1 combined with astatic seizures(ASS) and tonic seizures, 1 combined with astatic seizures and atypical absences(ATS), 1 combined with absences(AS) and tonic seizure, 2 combined with absences and generalized tonic clonic seizures, 4 combined with atypical absences and tonic seizures, 1 combined with astatic seizures and absences. As to interictal EEG, 2 normal, 32 had repeatedly paroxysmal sharp or polyspike slow wave, 7 hypsarrhythmia, 3 modified hypsarrhythmia and 3 burst suppression. Valproate sodium, clonazepam and topamax were used in the therapy of this study. Among 45 received treatments, 30 were effective, 15 were ineffective. 14 received monotherapy with valproate sodium, 10(71.42%) were effective. The effective power of cryptogenic group and group with etiological factors were 84.00% and 65.00%. The effective powers in different types of myoclonic epilepsy were prominent differences: WS 77.77%, LGS 68.75%, OS 33.33%, EME 0%, JME 100%. Several influence factors were analyzed in relation to short-term curative effect, including sex, age, age of onset, course of disease, neurodevelopment when taken into hospital, etiological factor, mode of onset, treatment and classification. Only etiological factor had a positive correlation with poor curative effect. 25 cases were followed up, in which 12 free of seizure or obvious mitigation, 21 had mental and motor development retardation. Several influence factors were analyzed in relation to seizure control and neurodevelopment, including sex, age, course of disease, neurodevelopment, etiological factor, time of treatment and classification. Only classification had a positive correlation with poor prognosis.Conclusions:①Identifying the diagnosis and classification of myoclonic epilepsy as soon as possible is very important for treatment and prognosis judgement.②Idiopathetic myoclonic epilepsy usually only have myoclonic seizure, and have normal mental, motor development. Symptomatic myoclonic epilepsy usually combine with other modes of onset, and have mental, motor development retardation.③Video EEG is a useful tool for myoclonic epilepsy discriminating with other diseases.④Valproate sodium should be first line drug, clonazepam, topamax, levetiracetam can olso be considered. Carbamazepine and phenytoin sodium should be avoided. Most of symptomatic myoclonic epilepsy need frequently more than 2 antiepilepsy drugs.⑤Strengthening prenatal and perinatal conservancy are important for reducing and preventing symptomatic myoclonic epilepsy.⑥The seizure control and neurodevelopment of idiopathetic myoclonic epilepsy are better than those of symptomatic myoclonic epilepsy.⑦Different classification of myoclonic epilepsy has different prognosis, the prognosis of WS, LGS, OS and EME is unfavourable. EME has the worst prognosis. The prognosis of BMEI and JME is favourable. BMEI has the best prognosis.
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