BACKGROUND: Juvenile myoclonic epilepsy(JME) is the most common idiopathic generalized epileptic syndrome which occurs mostly in the second decade of life. It is still frequently unrecognized and misdiagnosed. JME responds well to valproate, but is hardly controlled by other commonly used antiepileptic drug such as carbamazepine or phenytoin. We intended to Investigate the clinical symptoms and EEG findings of JME to support the diagnosis and treatment of JME. SUBJECTS AND METHODS: We retrospectively reviewed the medical record of 18 childrens with JME, from March 1991 to February 1997 We have analysed the clinical symptoms, seizure type, EEG findings including photosensitivity, and the effect of antiepileptic drug. RESULTS: 1) Seven patients were boys and eleven patients were girls(M : F=1 : 1.6). The seizure onset between 5 and 7 years of age were noted in 5 cases, that between 8 and 10 years in 5 cases, and in 8 cases seizures began after 11 years of age 2) Generalized convulsive seizures were noted in 16 cases(GTC 13, GC 2, GCTC 1), myoclonic seizures in 18 cases, absence seizures in 5 cases, and photoconvulsive seizures during video game were associated in 3 cases. 3) Epileptiform discharges at the diagnosis of JME were noted 12 cases(66.7%), and no epileptiform discharges were seen in 6 cases(33.3%). The background activities were normal in all cases. In all 12 cases that showed abnormal epileptiform discharge, generalized spike and wave discharges were obtained, and photosensitivies noted in 4 cases. 4) Follow up EEG obtained after treatment, showed normal EEG findings in 16 cases, and abnormal EEG findings were obtained in 2 cases. 5) Sixteen cases responded well to valproate monotherapy, and 2 other cases responded to valproate and ethosuximide combined therapy. In one case who received vigabatrin, the seizure was aggravated. 6) In 5 cases who discontinued antiepicoptic drug medication after 3 year seizure free duration, 3(60%) cases relapsed within 1 year, and 2(40%) cases remained seizure free for over 1 year. CONCLUSION: Juvenile myoclonic epilepsy is an epileptic syndrome with generalized convulsive seizure, myoclonic seizure, and absence seizure, and EEG findings showed generalized spike and wave discharge in 66.7%, and photosensitivity in 22.2%. JME responded well to valproate monotherapy or valproate and ethosuximide combined the rape, but not responded to other antiepileptic drugs.
Anticonvulsants ; Carbamazepine ; Child ; Diagnosis ; Electroencephalography ; Epilepsy, Absence ; Ethosuximide ; Follow-Up Studies ; Humans ; Medical Records ; Myoclonic Epilepsy, Juvenile* ; Phenytoin ; Rape ; Retrospective Studies ; Seizures ; Valproic Acid ; Video Games ; Vigabatrin

No abstract available.
Epilepsy* ; Vigabatrin*

No abstract available.
Cardiomyopathies* ; Congenital Hypothyroidism* ; Echocardiography*

No abstract available.
Prognosis* ; Reye Syndrome*

No abstract available.
Child* ; Humans ; Testosterone*

No abstract available.
Humans ; Hypocalcemia* ; Infant, Newborn ; Prognosis*

No abstract available.
Chlamydia* ; Fluorescent Antibody Technique, Indirect*

PURPOSE: Abnormalities in calcium(Ca), vitamin D and bone mineral density (BMD) associated with antiepileptic drug(AED) are reported, but the results are inconsistent. In case of intractable epilepsy, poor growth and altered bone mineral metabolism may be prominent, possibly related to previous long-term use of multiple AED and poor activity. The aim of this study was to assess growth status, concentrations of calcium regulating hormones and BMD in children with intractable epilepsy. METHODS: Sixty-six intractable epilepsy patients aged 0.8 to 14.7 years(mean+/-D:4.6+/-.6 years) were included in the study. Height and weight were measured and then height SDS and weight SDS were calculated. Serum Ca, i-Ca, P, Mg, Zinc, osteocalcin, intact-PTH, 25-OHD, 1,25(OH)2D were measured. BMD of the lumbar spine was measured by dual energy X-ray absorption. RESULTS: Most of the patients showed normal height SDS and weight SDS. Percentage of severe short stature(height SDS <-2) was 1.5% and tall stature(height SDS >2) was 4.5%. Percentage of severe thin(weight SDS <-2) was 1.5% and obesity(weight SDS >2) was 6%. Duration of AED was not related to height SDS or weight SDS. Etiology of epilepsy and physical activity were not related to height SDS and weight SDS. Most of them had normal Ca, iCa, P, Mg, Zinc, intact-PTH, osteocalcin, 25-OHD and 1,25(OH)2D concentrations. BMD was not related to the levels of Ca, i-Ca, P, Mg, intact-PTH, osteocalcin, 25-OHD, 1,25(OH)2D. BMD was not related to the duration of AED. BMD positively correlated with age(r=0.75, P>0.01) and body weight(r=0.72, P<0.01). CONCLUSION: Most of the children with intractable epilepsy, who regularly visits epilepsy clinic, showed normal growth and normal bone mineral metabolism, but careful monitoring about growth and bone mineral metabolism is needed.
Absorption ; Bone Density* ; Calcium* ; Child* ; Epilepsy* ; Humans ; Metabolism ; Motor Activity ; Osteocalcin ; Spine ; Vitamin D ; Zinc

Based on our experience with 23 frontotemporal reconstructions performed, this paper attempts to provide guidelines for appropriate selection among the four most commonly employed materials (e.g., autogenous parietal bone, silicone rubber, methylmethacrylate, and porous polyethylene) so that optimal results can be achieved. Clinical follow-up ranged from 6 to 91 months (mean 32 months). Every patient was analyzed clinically by ordinary scale method. The mean defect size was 134.8 cm2 in the silicone rubber, 36.5 cm2 in the methylmethacrylate, 17.4 cm2 in the autogenous bone graft and 7.3 cm2 in the porous polyethylene. The clinical assessment was excellent (mean, 29.3 points) in silicone rubber, excellent (mean, 28.6 points) in autogenous bone, excellent (mean, 26.8 points) in methylmethacrylate, and good (mean, 24.8 points) in polyethylene. To sum up, a large bony defect of congenital calvarial anomaly produced an excellent result using custom-made silicone implant. A relatively small bony defect with a scarred bed produced an excellent result using autogenous parietal bone grafting. Unexpected and medium-sized defect was reconstructed successfully through a simple procedure using methylmethacrylate. Porous Polyethylene was used at an incidental small defect because of its expensiveness.
Cicatrix ; Follow-Up Studies ; Humans ; Methylmethacrylate ; Parietal Bone ; Polyethylene ; Silicone Elastomers ; Transplants

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