No abstract available.
Neuromuscular Diseases*

PURPOSE: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. METHODS: The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital. We grouped the patients according to the MRI findings as follows. Group I (14 cases): Multi- or unifocal lesions only in the cerebral white matter. Group II (7 cases): lesions in the gray matter with or without white matter involvement. RESULTS: 1.Preceding events were as follows: no defined prodrome (38.1%), upper respiratory tract infection (28.6%), nonspecific febrile illness (19.0%), gastointestinal disturbance and vaccination. 2.Presenting symptoms were as follows: seizures (76.2%), headache/vomiting (47.6%), altered consciousness (38.1%), hemiparesis, cerebellar ataxia, visual disturbance and facial nerve palsy. 3.Laboratory findings were as follows: CSF pleocytosis (76.2%), leucocytosis (38.1%) and elevated CSF protein (28.6%). 4.Fifteen patients were recovered completely without neurological sequelae. Three patients in group I and 1 patient in group II had intractable seizures. Two patients in group I and 2 patients in group II had motor disturbance. CONCLUSION: There were no statistically significant differences in preceding events, presenting symptoms, and neurological outcomes according to the distribution of the lesions on brain MRI. However, the ADEM have quite diverse clinical manifestations and neuroimage findings. MRI plays an important role in making diagnosis of the patients who are suspected of ADEM.
Brain ; Central Nervous System ; Cerebellar Ataxia ; Consciousness ; Demyelinating Diseases ; Diagnosis ; Encephalomyelitis ; Encephalomyelitis, Acute Disseminated* ; Facial Nerve ; Humans ; Leukocytosis ; Magnetic Resonance Imaging* ; Paralysis ; Paresis ; Prognosis* ; Respiratory Tract Infections ; Seizures ; Vaccination

Many new antiepileptic drugs (AEDs) have been developed in the last two decades, contributing to the optimal treatment for childhood epilepsy. The goal of the treatment is to achieve seizure-free without any side effects, that deteriorates the quality of life by causing negative consequences. The new AEDs have not shown better efficacy, but generally seem to be better tolerated, having fewer systemic reactions and better pharmacokinetics than the established AEDs. The new AEDs have a broad spectrum of activities, which offer new opportunities to patients who have not shown any favorable responses to the established ones. There are more choices when trying to select AEDs for epileptic seizures and syndromes. Majority of the new AEDs have more than one action mechanism. AEDs acting selectively through the GABAergic system are tiagabine and vigabatrin; acting by inhibition of voltagedependent Na+ and Ca2+ channels are lamotirigine, oxcabarbazepine and topiramate; and acting by inhibition of glutamate-mediated excitation are felbamate, topiramate. The pharmacokinetic parameters of the new AEDs compared to the established AEDs, new AEDs have improved in terms of longer half-lives, permitting less frequent daily dosing, reduced potential for drug interactions. Considerations in selecting an AEDs are not only dependent on seizure types or syndromes, side effect profile, action mechanism, drug interaction, pharmacokinetic profile, facility of drug initiation, but also on age and sex of patients. Patients with worsened seizurefrequency or development of new types of seizure after the introduction of AEDs, should be questioned on the previously diagnosed seizure types or syndromes.
Anticonvulsants ; Drug Interactions ; Epilepsy ; Fructose ; Humans ; Nipecotic Acids ; Phenylcarbamates ; Propylene Glycols ; Quality of Life ; Seizures

PURPOSE: This study aims to examine and compare the features of rolandic epilepsy. METHODS: Of 158 patients selected retrospectively, 116 had typical (group A) and 42 had atypical (group B) rolandic epilepsy, as defined by Worrall's criteria. RESULTS: The age at onset of the seizures in group Awas 8.6+/-2.0 y and 6.2+/-1.7 y in group B (P>0.05). Among the 40 patients who underwent neuroimaging studies (25 patients in group Aand 15 patients in group B), abnormal findings in group B included ventricular dilatation, mild cortical atrophy, and partial agenesis of corpus callosum. group A had no abnormal findings. The frequency of seizures was 2.0+/-1.0 and 2.3+/-1.2 per month in groups A and B respectively. Seizure control from the initial anticonvulsant treatment was achieved within 3 months in group A, and 3 to 12 months in group B. A 2-year remission rate was noted in 105 patients in group A and in 38 patients in group B. Of these, the recurrence rate after 2 y was 13 in group A and 12 in group B. CONCLUSION: Age of onset of seizures, gender, frequency of seizures before therapy, and 2-y remission rate were not significantly different in the 2 groups. However, neuroimaging abnormalities, the time to achieving seizure control from the initial anticonvulsant treatment, and the recurrence rate after being seizure-free for 2 y were significantly different in the 2 groups.
Age of Onset ; Agenesis of Corpus Callosum ; Atrophy ; Dilatation ; Epilepsy, Rolandic ; Humans ; Neuroimaging ; Recurrence ; Retrospective Studies ; Seizures

Febrile seizure (FS) is the most common seizure disorder of childhood, and occurs in an age-related manner. FS are classified into simple and complex. FS has a multifactorial inheritance, suggesting that both genetic and environmental factors are causative. Various animal models have elucidated the pathophysiological mechanisms of FS. Risk factors for a first FS are a family history of the disorder and a developmental delay. Risk factors for recurrent FS are a family history, age below 18 months at seizure onset, maximum temperature, and duration of fever. Risk factors for subsequent development of epilepsy are neurodevelopmental abnormality and complex FS. Clinicians evaluating children after a simple FS should concentrate on identifying the cause of the child's fever. Meningitis should be considered in the differential diagnosis for any febrile child. A simple FS does not usually require further evaluation such as ordering electroencephalography, neuroimaging, or other studies. Treatment is acute rescue therapy for prolonged FS. Antipyretics are not proven to reduce the recurrence risk for FS. Some evidence shows that both intermittent therapy with oral/rectal diazepam and continuous prophylaxis with oral phenobarbital or valproate are effective in reducing the risk of recurrence, but there is no evidence that these medications reduce the risk of subsequent epilepsy. Vaccine-induced FS is a rare event that does not lead to deleterious outcomes, but could affect patient and physician attitudes toward the safety of vaccination.
Antipyretics ; Child ; Classification ; Diagnosis, Differential ; Diazepam ; Electroencephalography ; Epilepsy ; Fever ; Humans ; Meningitis ; Models, Animal ; Multifactorial Inheritance ; Neuroimaging ; Phenobarbital ; Recurrence ; Risk Factors ; Seizures ; Seizures, Febrile* ; Vaccination ; Valproic Acid

PURPOSE: To study the spectrum of epilepsy in children with cerebral palsy. METHODS: A total of 93 consecutive patients with cerebral palsy(CP) were retrospectively suited. Criteria for inclusion were a follow-up period of at least 2 years. The study examined the correlation between the incidence of epilepsy and seizure types in the different forms of CP. Other factors associated with epilepsy, such as age of first seizure, occurrence of abnormalities on brain imaging, and electroencephalogram were also analyzed. RESULTS: The overall prevalence of epilepsy in children with CP was 46.2 percent. The incidence of epilepsy was predominant in patients with mixed, diplegic, and quadriplegic palsies:55.5 percent, 51.6 percent, and 50.0 percent in frequency. The first seizure occurred during the first year of life in 48.8 percent of patients with epilepsy. Generalized tonic-clonic seizures were the most common seizure type(44.2 percent), predominant in diplegic patients(64.3 percent). On the other hand, infantile spasms and myoclonic seizures were the main cause of seizures among quadriplegic children(60 percent and 40 percent, respectively). The occurrence of epilepsy was more popular in the group with abnormal brain imagings; especially encephalomalacia and cortical atrophy. All children with epilepsy in this study showed abnormal electroencephalogram(EEG) findings: Generalized abnormalities were observed in 55.8 percent of children with epilepsy; more dominantly in quadriplegic children(80.0 percent); and 40 percent of children with diplegia showed focal abnormalities. CONCLUSION: Cerebral palsy is associated with a higher incidence of seizure disorders, which, in the majority, has its onset in the first year of life; brain imaging and EEG are most effective in spotting epilepsy in children with CP.
Atrophy ; Brain ; Cerebral Palsy* ; Child* ; Electroencephalography ; Encephalomalacia ; Epilepsy* ; Female ; Follow-Up Studies ; Hand ; Humans ; Incidence ; Infant ; Infant, Newborn ; Metrorrhagia ; Neuroimaging ; Prevalence ; Retrospective Studies ; Seizures ; Spasms, Infantile

PURPOSE: To investigate the efficacy of topiramate monotherapy in West syndrome prospectively. METHODS: The study population included 28 patients (15 male and 13 female children aged 2 to 18 months) diagnosed with West syndrome. After a 2-week baseline period for documentation of the frequency of spasms, topiramate was initiated at 2 mg/kg/day. The dose was increased by 2 mg/kg every week to a maximum of 12 mg/kg/day. Clinical assessment was based on the parents' report and a neurological examination every 2 weeks for the first 2 months of treatment. The baseline electroencephalograms (EEGs) were compared with the post-treatment EEGs at 2 weeks and 1 month. RESULTS: West syndrome was considered to be cryptogenic in 7 of the 28 patients and symptomatic in 21 patients. After treatment, 11 patients (39%) became spasm-free, 6 (21%) had more than 50% spasmsreduction, 3 (11%) showed less than 50% reduction, and 8 (29%) did not respond. The effective daily dose for achieving more than 50% reduction in spasm frequency, including becoming spasm-free, was found to be 5.8+/-1.1 mg/kg/day. Nine patients (32%) showed complete disappearance of spasms and hypsarrhythmia, and 11 (39%) showed improved EEG results. Despite adverse events (4 instances of irritability, 3 of drowsiness, and 1 of decreased feeding), no patients discontinued the medication. CONCLUSION: Topiramate monotherapy seems to be effective and well tolerated as a first line therapy for West syndrome and is not associated with serious adverse effects.
Aged ; Child ; Electroencephalography ; Female ; Fructose ; Humans ; Infant ; Infant, Newborn ; Male ; Neurologic Examination ; Sleep Stages ; Spasm ; Spasms, Infantile

PURPOSE: Electroencephalography (EEG) is frequently ordered for patients with febrile seizures despite its unclear diagnostic value. We evaluated the prevalence of abnormal EEGs, the association between clinical findings and abnormal EEGs, and the predictive value of EEG for the recurrence of febrile seizures. METHODS: Data were collected on 230 children who were treated for febrile seizures at Kyung Hee University Medical Center from 2005 to 2009. EEGs were recorded after 1-2 days of hospitalization when children became afebrile. EEG patterns were categorized as normal, epileptiform, or nonspecific relative to abnormalities. The patients' medical records were reviewed, and telephone interviews with the families of the children were conducted to inquire about seizure recurrence. The relationships between clinical variables, including seizure recurrence, and EEG abnormalities were evaluated. RESULTS: Of the 131 children included, 103 had simple and 28 had complex febrile seizures. EEG abnormalities were found in 41 children (31%). EEG abnormalities were more common in children with complex than simple febrile seizures (43% vs. 28%), but the difference was not statistically significant. Logistical regression analysis showed that having multiple seizures in a 24-hour period was significantly predictive of abnormal EEG (odds ratio, 2.98; 95% confidence interval, 1.0 to 88; P=0.048). The frequency of recurrence did not differ significantly in the normal (31%) and abnormal (23%) EEG groups. CONCLUSION: Multiple seizures within 24 hours were predictive of abnormal EEG in children with febrile seizures. Abnormal EEG was not predictive of febrile seizure recurrence.
Academic Medical Centers ; Child* ; Electroencephalography* ; Hospitalization ; Humans ; Interviews as Topic ; Medical Records ; Prevalence ; Recurrence ; Seizures ; Seizures, Febrile*

PURPOSE: This study was conducted for evaluation of the effects of new antiepileptic drugs on bone mineral density in children with epilepsy. METHODS: The study group consisted of 35 age and gender matched controls and 35 epileptic children taking new antiepileptic drugs: 14 on topiramate, 10 on lamotrigine, and 11 on oxcarbazepine in monotherapy. All patients were treated for more than one year and all were normally ambulatory children. We measured serum levels of calcium, alkaline phosphatase, 25-hydroxyvitamin D, and 1,25-dihydroxyvitamin D. BMD was measured by dual-energy X-ray absorptiometry at lumbar spine regions L1-L4. RESULTS: Vitamin D levels of the oxcarbazepine group (25-hydroxyvitamin D: 40.3+/-10.5 ng/mL and 1,25-dihydroxyvitamin D: 57.9+/-15.2 pg/mL) were significantly lower than in controls (44.6+/-11.5 ng/mL, 66.2+/-10.5 pg/mL, P<0.05); however, they did not differ significantly in the topiramate and lamotrigine groups. The bone mineral density value was significantly lower in the oxcarbazepine (L1-L4: 0.73+/-0.11 g/cm2) group, compared with the controls (0.84+/-0.06 g/cm2, P<0.05) or patients taking topiramate or lamotrigine. CONCLUSION: Monitoring of bone metabolism is recommended for patients treated with new antiepileptic drugs, particularly oxcarbazepine.
Absorptiometry, Photon ; Alkaline Phosphatase ; Anticonvulsants ; Bone Density ; Calcium ; Carbamazepine ; Child ; Fructose ; Humans ; Spine ; Triazines ; Vitamin D