No abstract available.
Acidosis, Lactic ; Brain Diseases ; Humans ; Myoclonic Epilepsy, Juvenile ; Wolff-Parkinson-White Syndrome

Adult ; Anticonvulsants ; therapeutic use ; Brain ; drug effects ; pathology ; physiopathology ; Carbamazepine ; analogs & derivatives ; therapeutic use ; Electroencephalography ; Epilepsy, Frontal Lobe ; diagnosis ; drug therapy ; Female ; Humans ; Myoclonic Epilepsy, Juvenile ; diagnosis ; drug therapy ; Myoclonus ; diagnosis ; drug therapy

PURPOSE: The aim of the this study was to verify the efficacy of the occipital nerve block (ONB) for occipital neuralgia pediatric patients who has tender point around suboccipital area. METHODS: In a retrospective way, 47 pediatric patients with occipital neuralgia were enrolled during July 2005 to July 2013. Diagnosis of occipital neuralgia is made by the International headache society criteria (2004, 2nd edition). We conducted a chart review of all the ONB performed in our clinic over 9 years. Patients were analyzed according to gender, headache type, headache duration and frequency, family history, medication history before ONB, symptoms with headache, recurrence, complications, magnetic resonance imaging scan and electroencephalogram results. Headache was measured before and after the procedure using the visual analog pain indexes. RESULTS: Forty seven (100%) patients were treated with occipital nerve block. Pain indexes were declined in 41 (87.2%) patients, but 6 (12.8%) were not response. Mean visual analog pain index scores declined by 4.7 units. Twenty five (53.2%) patients experienced recurrence of the disorder, but 14 (56.0%) patients were relived without the need for any further treatment. No complications were reported. CONCLUSION: Our data suggests that occipital nerve block was safe and effective treatment for the children with occipital neuralgia.
Child ; Diagnosis ; Electroencephalography ; Headache ; Humans ; Magnetic Resonance Imaging ; Myoclonic Epilepsy, Juvenile* ; Nerve Block ; Neuralgia ; Recurrence ; Retrospective Studies ; Valproic Acid*

PURPOSE: The purpose of this study was to evaluate the efficacy and tolerability of extended-release valproic acid once daily dosing in juvenile myoclonic epilepsy (JME). METHODS: Medical records of patients who received valproic acid monotherapy for the treatment of JME were retrospectively reviewed. Their clinical information regarding age, gender, seizure types, underlying neurologic status, dosing regimen, response to treatment, and adverse events related to valproic acid, were analyzed. Seizure control, compliance, and adverse events rates were compared between the group of once daily dosing and the group of twice daily dosing. RESULTS: Twenty one patients (11 boys and 10 girls) were included in the study. Twelve patients were taking valproic acid extended-release once daily and nine patients were taking twice a day. More than 50% decrease in myoclonic seizure was achieved in all the patients in both groups. Generalized tonic-clonic seizure was controlled in all the patients who were taking once daily while 3 patients (36%, 3/8 patients) in twice daily group had breakthrough generalized tonic clonic seizures during the 2 year period of treatment. However, there were no statistically significant differences in seizure control, compliance, and adverse event rates between the two groups. CONCLUSION: This study demonstrated that valproic acid extended-release once daily dosing was as effective and tolerable as twice daily in the treatment of JME. Once daily dosing of valproic acid would be convenient which improve patient compliance and consequently bring better outcome in treatment of JME.
Compliance ; Humans ; Medical Records ; Myoclonic Epilepsy, Juvenile* ; Patient Compliance ; Retrospective Studies ; Seizures ; Valproic Acid*

PURPOSE: To verify the efficacy and safety of lamotrigine (LTG) monotherapy in newly diagnosed children with epilepsy. METHODS: We prospectively enrolled 148 children who had undergone LTG monotherapy at our institution between September 2002 and June 2009. Twenty-nine patients were excluded: 19 due to incomplete data and 10 were lost to follow up. The data of the remaining 119 patients was analyzed. RESULTS: We enrolled 119 pediatric epilepsy patients (aged 2.8-19.3 years; 66 males and 53 females) in this study. Out of 119 patients, 29 (25.2%) had generalized epilepsy and 90 (74.8%) had partial epilepsy. The responses of seizure reduction were as follows: Seizure freedom (no seizure attack for at least 6 months) in 87/111 (78.4%, n=111) patients; partial response (reduced seizure frequency compared to baseline) in 13 (11.7%) patients; and persistent seizure in 11 (9.9%) patients. The seizure freedom rate was in 81.6% in patients with partial seizure (75.9% for complex partial seizure and 90.9% for benign rolandic epilepsy) and 44.8% in patients with generalized epilepsy (30.0% for absence seizure, 35.7% for juvenile myoclonic epilepsy patients, and 100.0% for idiopathic generalized epilepsy patients). Adverse reactions were reported in 17 (14.3%) patients, and 8 patients (6.7%) discontinued LTG because of rash and tic. No patient experienced severe adverse reaction such as Stevens-Johnson syndrome. CONCLUSION: LTG showed excellent therapeutic response and had few significant adverse effects. Our findings report may contribute in promoting the use of LTG monotherapy in epileptic children.
Child ; Epilepsies, Partial ; Epilepsy ; Epilepsy, Absence ; Epilepsy, Generalized ; Exanthema ; Freedom ; Humans ; Lost to Follow-Up ; Male ; Myoclonic Epilepsy, Juvenile ; Prospective Studies ; Seizures ; Stevens-Johnson Syndrome ; Tics ; Triazines

BACKGROUND: Juvenile myoclonic epilepsy (JME) is characterized by myoclonic seizures on awakening and giant somatosensory evoked potentials. Those characteristics suggest that JME is related with dysfunction of the brainstem reticular formation. Cardiovascular reflex is also closely related with the brainstem reticular formation, and head-up tilt-table test (HUT) is frequently employed to evaluate the cardiovascular reflex. We performed HUT to assess the cardiovascular reflex in patients with JME and syncope/presyncope. METHODS: We included 52 patients with alleged JME. HUT was performed in 20 of them with a history of presyncope or syncope. The protocol of HUT included 70 degree table tilting test and isoproterenol infusion test (1~5 microgram/min). We determined the positive responses according to the change of blood pressure and heart rate. The positive responses were classified as vasodepressive, cardioinhibitory, or mixed types. We retrospectively analyzed the clinical findings and responses of HUT. RESULTS: Syncope or presyncope occurred during the seizure-prone situation in 3 (15%), and during the syncope-prone situation in 17 (85%) patients. Fifteen (75%) patients had positive responses in HUT. The positive responses were during the baseline test in 1 (5%), during the low dose ((< or = 3 microgram/min) of isoproterenol infusion in 10 (50%), and during the high dose (> or = 4 microgram/min) of isoproterenol infusion in the remaining 4 (20%) patients. All the positive responses were vasodepressive type. CONCLUSIONS: In patients with JME, vasomotor reflex may be impaired. Our results suggest dysfunction of the brainstem reticular formation in JME.
Blood Pressure ; Brain Stem ; Evoked Potentials, Somatosensory ; Heart Rate ; Humans ; Isoproterenol ; Myoclonic Epilepsy, Juvenile ; Reflex ; Reticular Formation ; Retrospective Studies ; Seizures ; Syncope ; Tilt-Table Test

BACKGROUND AND PURPOSE: We investigated the cognitive change of patients with juvenile myoclonic epilepsy (JME) after a long-term antiepileptic drug(s) (AED) administration to clarify the cause of cognitive impairment. METHODS: Thirty-three patients with JME who were newly diagnosed or did not take any AED for at least 6 months prior to the beginning of the study were included. We conducted neuropsychological tests at baseline and after at least 12 months of AEDs trial. Forty healthy controls were acquired according to age- and education-match to patients with JME. We compared the differences of neuropsychological outcomes among them. We tried to identify the determinants for cognitive performances after AEDs trial. RESULTS: Twenty-seven patients completed the second neuropsychological tests. Seizure frequency and EEG abnormality were significantly decreased after AEDs intake. The Number of epileptiform discharges (EDs) on EEG tended to be decreased at last visit. However, cognitive performances between baseline and follow-up period were not different. Cognitive measures of baseline and follow-up period were worse than those of controls in list learning, forward digit span, backward digit span, Trail Making Test, and verbal fluency. Cognitive performances of follow-up period in the JME group were not correlated with age at seizure onset, duration of epilepsy, seizure recurrence, EEG abnormality, and type of AEDs. CONCLUSIONS: Cognitive performances of JME were not recovered to the level of healthy controls despite the control of seizures and EDs by AEDs. Therefore, cognitive impairment of JME may be due to irreversible, disease-related characteristics.
Cognition ; Electroencephalography ; Epilepsy ; Follow-Up Studies ; Humans ; Learning ; Myoclonic Epilepsy, Juvenile ; Neuropsychological Tests ; Recurrence ; Seizures ; Trail Making Test

BACKGROUND AND PURPOSE: Cognitive impairments are frequent consequences of epilepsy, with intellectual ability reportedly being lower in patients with idiopathic generalized epilepsies than in the general population. However, neuropsychological investigations have been rarely performed in patients with juvenile myoclonic epilepsy (JME). We aimed to quantify the cognitive function in JME patients using various neuropsychological tests. METHODS: We compared cognitive function in 27 JME patients with that in 27 healthy volunteers using tests examining cognitive performance, such as the verbal and visual memory, frontal function, attention, IQ score, and mood. In the JME group, we examined risk factors for cognitive function such as age, sex, family history, education level, age at seizure onset, seizure frequency, EEG abnormality, disease duration, and previous intake of antiepileptic drugs. RESULTS: Verbal learning was significantly lower in JME patients than in controls, and attention and verbal fluency were impaired in JME patients compared with controls. However, general intellectual ability and mood did not differ between the groups. Early onset of seizure and long duration of disease were closely related to impaired cognitive function. CONCLUSIONS: JME patients may exhibit impaired cognitive function, in terms of memory and execution, despite having normal intelligence and mood.
Anticonvulsants ; Cognition ; Education ; Electroencephalography ; Epilepsy ; Epilepsy, Generalized ; Healthy Volunteers ; Humans ; Intelligence ; Memory ; Myoclonic Epilepsy, Juvenile* ; Neuropsychological Tests ; Risk Factors ; Seizures ; Verbal Learning

Using simultaneous EEG-correlated functional MRI (EEG-fMRI), we studied the blood oxygenation level-dependent (BOLD) signals in a juvenile myoclonic epilepsy (JME) patient with interictal epileptiform discharges. Extensive and symmetric activation and deactivation areas were assessed in bilateral hemispheric regions respectively, and these signals decreased gradually from the parieto-occipital region to the frontal region. Activations were found in cuneus, insulae, mesial midfrontal region, midline and bilateral cerebellum and thalamus, while the deactivities were in the bilateral anterior frontal region and parietal region as well as the posterior cingulate gyri. These findings suggest that the synchronized neuronal activities represented by spike and wave complex in EEG be reflected in the activation of BLOD signals in thalamocortical regions. However, those regions of deactivation reflect the suspension of the default state of brain function resulting indirectly from this discharge. There is good correspondence between neuronal activity (EEG) and fMRI. The combination of EEG and fMRI is a powerful tool in studying brain function.
Adolescent ; Brain ; blood supply ; physiopathology ; Electroencephalography ; methods ; Female ; Humans ; Image Processing, Computer-Assisted ; Magnetic Resonance Imaging ; methods ; Myoclonic Epilepsy, Juvenile ; physiopathology ; Oxygen ; blood

OBJECTIVE: We aimed to find structural brain abnormalities in juvenile myoclonic epilepsy (JME) patients. MATERIALS AND METHODS: The volumes of the cerebrum, hippocampus and frontal lobe and the area of the corpus callosum's subdivisions were all semi-automatically measured, and then optimized voxel-based morphometry (VBM) was performed in 19 JME patients and 19 age/gender matched normal controls. RESULTS: The rostrum and rostral body of the corpus callosum and the left hippocampus were significantly smaller than those of the normal controls, whereas the volume of the JME's left frontal lobe was significantly larger than that of the controls. The area of the rostral body had a significant positive correlation with the age of seizure onset (r = 0.56, p = 0.012), and the volume of the right frontal lobe had a significant negative correlation with the duration of disease (r = -0.51, p = 0.025). On the VBM, the gray matter concentration of the prefrontal lobe (bilateral gyri rectus, anterior orbital gyri, left anterior middle frontal gyrus and right anterior superior frontal gyrus) was decreased in the JME group (corrected p < 0.05). CONCLUSION: The JME patients showed complex structural abnormalities in the corpus callosum, frontal lobe and hippocampus, and also a decreased gray matter concentration of the prefrontal region, which all suggests there is an abnormal neural network in the JME brain.
*Signal Processing, Computer-Assisted ; Organ Size ; Myoclonic Epilepsy, Juvenile/*pathology ; Male ; Magnetic Resonance Imaging/*methods ; Imaging, Three-Dimensional/*methods ; Image Interpretation, Computer-Assisted/*methods ; Humans ; Female ; Brain/*pathology ; Adult