| Objective To explore the etiology, clinical characteristics, treatment and prognosis of myoclonic epilepsy(ME) for to provide theoretical basis for future clinical diagnosis, therapy and prognosis judgement.Methods The clinical data of myoclonic epilepsy patients was collected in Children's Hospital of Chongqing Medical University from November 1995 to March 2010, which were retrospectively analyzed with gender, age of first onset, duration, clinical manifestations, pathogenesis factors, EEG, imaging, treatment and prognosis.Results The total patient number was 20, male: female = 1.1:1, and the average age of onset was (3.03±3.55) years old, 80.0% onset was within 8 years. Patients with potential risk factors accounted for 40.0%, and perinatal brain injury, cerebral dysplasia were the most common risk factors.Among these cases, ten cases were the only attack way of myoclonic seizures, ten were combined with generalized tonic-clonic seizures, absence seizures, and other forms. In these cases, 4 cases were diagnosed with juvenile myoclonic epilepsy, and 5 cases were diagnosed with infant benign myoclonic epilepsy, one was myoclonic absence epilepsy, 2 were epilepsy with myoclonic-atonic seizures, 3 were Lennox-Gastaut syndrome, 2 were infantile spasms, 1 was myoclonic epilepsy with ragged red fibers. Only 2 cases were not classified clearly.There were 4 cases with normal interictal EEG, 16 patients with EEG abnormalities. The abnormalities include 9 cases with the spine - slow or spike-slow wave, 6 cases with spike or sharp, or sharp-slow wave, 1 case with hypsarrhythmia. Six cases showed abnormal head CT/MRI examination results. During hospitalization, these patients were commonly prescribed with sodium valproate and clonazepam. Among these 20 patients, 15 cases were completedly controlled seizures, and 5 patients were incompletedly controlled. The complete control rate was 77.8% in 18 cases with valproic acid alone or combination therapy.The control rates of possible-risk-factor and non-risk-factor group were 37.5% and 100.0% respectively. The efficiency of different categories of myoclonic epilepsy was 88.9% for BIME + JME, 100% for EMAS, 40% for LGS + infantile spasms, 100% for MAE, none for myoclonic epilepsy with ragged red fibers. 100% are not clearly classified.Conclusion The etiology of myoclonic epilepsy is complicated. Myoclonic jerks is the main manifestation of myoclonic epilepsy, easily misdiagnosis. The specialists should pay more attention to this group disease and more awareness of myoclonic epilepsy. EEG is an important basis for the myoclonic epilepsy diagnosis and classification, differential diagnosis. The prognosis of myoclonic epilepsy depends primarily on the classification. BIME and JME have the best prognosis, and LGS and infantile spasms have poorer prognosis. The myoclonic epilepsy with ragged red fibers have the worst prognosis. Most MAE cases were better prognosis, and the prognosis of EMAS is difficult to determine.To make the diagnosis and classification as fast as possible is crucial to the treatment and prognosis of myoclonic epilepsy. Clear etiology and tissue damage of brain also have impact on prognosis. Strengthening prenatal and perinatal care have a great significance for the prevention and reduction of the occurrence of symptomatic myoclonic epilepsy. Valproate acid therapy should be preferred, followed by clonazepam, nitrazepam and lamotrigine, etc. The treatment should avoid the usage of carbamazepine and phenytoin, avoiding to aggravate seizures.
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