BACKGROUNDRecently, new anti-epileptic drugs (AEDs) have been more frequently selected to treat epilepsy. In the present study, we evaluated the dynamic changes of efficacy and safety of three newer AEDs for treating partial epilepsy in China.

METHODSPatients were collected sequentially and were divided into three groups which accepted oxcarbazepine (OXC), lamotrigine (LTG) or topiramate (TPM) therapy. Each group included monotherapy and add-on therapy subgroups. We followed all patients for one year and recorded the indexes of efficacy and safety in detail.

RESULTSA total of 909 patients finished the follow-up observation. No significant difference was found in proportion of patients with > or = 50% reduction, > or = 75% reduction and 100% seizure reduction in the LTG and OXC groups between the first and the second six months. In the TPM group there was a statistical difference between the first and the second six months in proportion of patients with > or = 50% reduction (P = 0.002), > or = 75% reduction (P < 0.0001) and 100% seizure reduction (P = 0.009) in the monotherapy subgroup, and about > or = 75% reduction and 100% seizure reduction in the add-on therapy subgroup (P < 0.0001). The efficacy between the add-on and monotherapy subgroups showed a statistical difference. The safety of the three newer AEDs was good.

CONCLUSIONSThe three newer AEDs all showed good efficacy and tolerability for partial epilepsy. And the efficacy can be maintained for at least one year.

Anticonvulsants ; therapeutic use ; Carbamazepine ; analogs & derivatives ; therapeutic use ; China ; Epilepsies, Partial ; drug therapy ; Follow-Up Studies ; Fructose ; analogs & derivatives ; therapeutic use ; Humans ; Treatment Outcome ; Triazines ; therapeutic use

Adult ; Carbamazepine ; adverse effects ; therapeutic use ; Drug Eruptions ; etiology ; Female ; Humans ; Tinnitus ; drug therapy

OBJECTIVETo investigate the clinical and genetic features of a Chinese girl with Schwartz-Jampel syndrome (SJS).

METHODTo analyze the clinical and genetic data of a girl with Schwartz-Jampel syndrome who was sent to neurology outpatient department of Beijing Children's Hospital in Auguest of 2010. Reports on Schwartz-Jampel syndrome published until July of 2015 were searched and the clinical and genetic characteristics of reported cases were summarized.

RESULTAt 8 months after birth, the girl showed myotonia; at 1 year old when she was walking alone she had myotonia of lower limbs, both feet evaginated, walked slowly and was prone to fall. At 2 years of age, she could not climb up stairs, at 3 years she could not jump continuously. At 3 years and 7 months of age when the girl was taken to neurology outpatient department, on examination, she had a dull facial expression, rigid lips and could not fully open her mouth, a micromandible, low-set and prominent ears, systemic muscle rigidity, there were muscular nodes formation on the limbs and gait stiffness. She had high level of creatine kinase and atlanto-axial joint subluxation on cervical CT reconstruction. She also had spontaneous myotonia-like discharges on needle electromyography (NEMG). X-ray of limbs showed metaphyseal dysplasia. The patient was treated with neurologic rehabilitation and carbamazepine. The myotonia at the last follow-up at her 8 years of age was the same as at the onset. On her HSPG2 gene, two novel heterozygous mutations c.10776delT on exon 78 and c.5702-5G>A on intron 45 were found. c.10776delT resulted in the amino acid change on p.Ala3592fsX6 and c.5702-5G>A maybe changed protein splicing. No reports were found among Chinese journals, while 7 reports were found in English literature. The total 34 mutations were known in reviewed reports, which included eleven deletion or insertion, twelve splice site, eight missense, and three nonsense mutations. Four patients had a single mutation. No definite genotype-phenotype correlation was identified.

CONCLUSIONSchwartz-Jampel syndrome is a rare autosomal-recessive hereditary disease appears to be slowly progressive, in which distinctive clinical features were induced by HSPG2 gene mutation. We reported the c.10776delT on exon 78 and c.5702-5G>A on intron 45 which were not reported previously. This is the first report of Schwartz-Jampel syndrome of which genetic mutations was identified in a Chinese child.

Asian Continental Ancestry Group ; Carbamazepine ; therapeutic use ; Child ; Child, Preschool ; Exons ; Female ; Heterozygote ; Humans ; Infant ; Introns ; Mutation ; Osteochondrodysplasias ; diagnosis ; genetics

OBJECTIVETo investigate the clinical efficacy and safety of oxcarbazepine (OXC) suspension in children with focal epilepsy.

METHODSA total of 118 children aged 2-14 years, who were newly diagnosed with focal epilepsy between October 2009 and December 2011, were randomly divided into experimental group (n=60) and control group (n=58). The experimental group was treated with an orally suspension of OXC and the control group was orally administered with carbamazepine (CBZ) tablets. The two treatment regimens were compared in terms of clinical efficacy and safety.

RESULTSAfter 13 and 26 weeks of treatment, the experimental group had response rates of 75% and 72% respectively and seizure-free rates of 53% and 50%, and the control group had response rates of 71% and 66% and seizure-free rates of 50% and 43% respectively. There were no significant differences in the clinical efficacy between the two groups (P>0.05). After 26 weeks of treatment, the adverse event rates of the experimental and control groups were 18% and 40% respectively, with a significant difference between the two groups (P<0.05).

CONCLUSIONSOXC suspension has a comparable clinical efficacy to that of CBZ tablets in children aged 2-14 years who are newly diagnosed with focal epilepsy, but OXC suspension causes fewer adverse events and has higher safety.

Adolescent ; Anticonvulsants ; therapeutic use ; Carbamazepine ; adverse effects ; analogs & derivatives ; therapeutic use ; Child ; Child, Preschool ; Epilepsies, Partial ; drug therapy ; Female ; Humans ; Male ; Suspensions

OBJECTIVE: To determine whether carbamazepine is effective in treating the subjective tinnitus. METHOD: Randomized, prospective,double-blind,controlled trial was used in our study. The study group consisted of 100 adult patients who consulted our outpatient clinic complaining of subjective tinnitus, excluded objective tinnitus and the patients who had tinnitus caused by obvious diseases, such as outer and middle ear diseases, 50 patients were given carbamazepine and Flunarizine Hydrochloride, 50 patients were given Vitamin B6 and Flunarizine Hydrochloride. After a week the effect of the different group of medicines was observed. Tinnitus questionnaire was performed before the treatment, and pure tone audiogram, tinnitus pitch and loudness matching were performed at the end of the treatment. RESULT: Completion of treatment, tinnitus loudness matching assessment showed that the efficacy of the carbamazepine group was similar to that of the control group. The efficacy of treatment was respectively 26% by intend to treat (ITT) and 28.3% by per protocol (PP) in the carbamazepine group and 26% by ITT and 27.7% by PP in the control group. The efficacy of treatment has no statistically significance for tinnitus loudness of the experimental group and the control group. The subjective tinnitus improvement rate showed no difference between two groups. Pure tone thresholds fluctuated within 10 dB in the beginning and at the end of the treatment. There were serious side effects in the carbamazepine group. The side effects rates were respectively 55.3% and 16.7% in the carbamazepine group and the control group, respectively. The difference had statistical significance. CONCLUSION Our results showed that the efficacy of carbamazepine combined with Flunarizine Hydrochloride is similar to that of the control group. There was no improvement in listening. But the side effects of it were more serious than that of the control group. It should not be recommended for the treatment of tinnitus.
Adolescent ; Adult ; Aged ; Carbamazepine ; therapeutic use ; Double-Blind Method ; Drug Therapy, Combination ; Flunarizine ; therapeutic use ; Hearing Tests ; Humans ; Middle Aged ; Prospective Studies ; Tinnitus ; drug therapy ; Young Adult

BACKGROUND: Studies on the relationship between antiepileptic drug (AED) administration and clinical outcomes in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) remain scarce. Levetiracetam (LEV) is an AED that is neuroprotective in various neurologic disorders. This study aimed to determine the impact of LEV on the outcome of MELAS. METHODS: A retrospective, single-center study was performed based on a large cohort of patients with MELAS with a history of seizures (n = 102). Decisions on antiepileptic therapies were made empirically. Patients were followed up for 1 to 8 years (median, 4 years) and divided into 2 groups based on whether LEV was administered (LEV or non-LEV). The modified Rankin scale (mRS) scores and mortality risks were analyzed in all patients. RESULTS: LEV, carbamazepine, benzodiazepines, topiramate, oxcarbazepine, valproate, and lamotrigine were administered in 48, 37, 18, 13, 11, 9, and 9 patients, singly or in combination, respectively. The mean mRS score of the LEV group (n = 48) was lower than that of the non-LEV group (n = 54; mean ± standard deviation, 2.79 ± 1.47 vs. 3.83 ± 1.93, P = 0.006) up to the end of the study. Nevertheless, there was no difference in the proportion of subjects without disability (mRS ranging 0-1) between the groups (P = 0.37). The multivariate regressions revealed that LEV treatment was associated with lower mRS scores (odds ratio 0.32, 95% confidence interval [CI] 0.15-0.68, P = 0.003) and mortality rates (hazard ratio 0.24, 95% CI 0.08-0.74, P = 0.013). There was a significant difference in the Kaplan-Meier survival curves between the groups (χ = 4.29, P = 0.04). CONCLUSIONS The LEV administration is associated with lower mortality in patients with MELAS in this retrospective study. Further laboratory research and prospective cohort studies are needed to confirm whether LEV has neuroprotective effects on patients with mitochondrial diseases.
Acidosis, Lactic ; drug therapy ; mortality ; Adolescent ; Anticonvulsants ; therapeutic use ; Carbamazepine ; therapeutic use ; Child ; Child, Preschool ; Female ; Humans ; Lamotrigine ; therapeutic use ; Levetiracetam ; administration & dosage ; therapeutic use ; Male ; Mitochondrial Encephalomyopathies ; drug therapy ; mortality ; Oxcarbazepine ; therapeutic use ; Prospective Studies ; Retrospective Studies ; Stroke ; drug therapy ; mortality ; Topiramate ; therapeutic use ; Valproic Acid ; therapeutic use

To investigate the effects of carbamazepine (CBZ) on the plasma concentrations of valproic acid (VPA) and its toxic metabolite 2-propyl-4-pentenoic acid (4-ene VPA) in epileptic patients, the plasma concentrations of VPA and 4-ene VPA were determined, and the effect of CBZ on pharmacokinetics of VPA was evaluated. All patients had been divided into two groups (VPA group, n = 87; and VPA+CBZ group, n = 19). As compared to VPA group, the combination of CBZ significantly (P < 0.01) decreased the trough concentration of VPA [VPA group, (69.5 +/- 28.8) microg x mL(-1); VPA+CBZ group, (46.3 +/- 25.6) microg x mL(-1)] and does-adjusted VPA trough concentration [VPA group, (4.89 +/- 2.21) microg x mL(-1) x mg(-1) x kg(-1); VPA+CBZ group, (3.14 +/- 1.74) microg x mL(-1) x mg(-1) x kg(-1)]. However, the addition of CBZ did not influence the concentration of 4-ene VPA. The present study revealed that coadministration of CBZ can reduce VPA plasma concentration and may impact VPA clinical effect, therefore therapeutic drug mornitoring of VPA should be used when combined use of CBZ and VPA.
Adolescent ; Adult ; Anticonvulsants ; blood ; pharmacokinetics ; therapeutic use ; Carbamazepine ; blood ; pharmacokinetics ; therapeutic use ; Drug Interactions ; Drug Therapy, Combination ; Epilepsy ; blood ; drug therapy ; Fatty Acids, Monounsaturated ; blood ; Female ; Humans ; Male ; Valproic Acid ; blood ; pharmacokinetics ; therapeutic use ; Young Adult

This is a long-term, open label, observational study aimed to broaden our clinical experiences in managing infants and toddlers with epilepsy. The long-term retention rate and side effects of topiramate (TPM) in them were evaluated and compared with carbamazepine (CBZ). A total of 146 children were involved in the study (TPM=41, CBZ=105). The retention rates at 24 , 36, and 48 months were 46.3%, 34.1%, 26.8% for TPM and 36.2%, 23.8%, 13.3% for CBZ, respectively. At 6 months after starting antiepileptic drugs (AED), the seizure freedom or clinical efficacy (seizure reduction rate more than 50 percent) were 73.2% for TPM and 62.9% for CBZ. The major side effects led to discontinuation included psychomotor slowing, poor oral intake from TPM and sleepiness and skin rash from CBZ. TPM was discontinued due to side effects in one case (2.4%) and lack of efficacy in five cases (12.2%), whereas CBZ was discontinued due to lack of efficacy (22.9%) and side effects (6.7%). As compared with CBZ, TPM showed the same long-term retention rate in children under the age of 2 yr, and no serious side effects. It is therefore concluded that TPM can be considered as a major AED for treating children with epilepsy under the age of 2 yr.
Anticonvulsants/adverse effects/*therapeutic use ; Carbamazepine/adverse effects/therapeutic use ; Child ; Child, Preschool ; Epilepsy/*drug therapy ; Female ; Follow-Up Studies ; Fructose/adverse effects/*analogs & derivatives/therapeutic use ; Humans ; Infant ; Male ; Treatment Outcome

OBJECTIVE: To study the efficacies of intratympanic prednisolone and dexamethasone injection for the subjective tinnitus. METHOD: A prospective study was designed to compare the efficacies of intratympanic prednisolone injection, intratympanic dexamethasone injection and carbamazepine by oral administration for subjective tinnitus. Seventy-three cases (78 ears) with subjective tinnitus for more than one month and treated by conservative therapy (such as vasodilator agent, Vitamin B, etc. by oral intake. ) were involved. The patients were randomized into 3 groups. Thirty-four cases (35 ears) were included in prednisolone group, 18 cases (18 ears) in dexamethasone group with intratympanic injection of prednisolone or dexamethasone, and 21 cases (25 ears) in carbamazepine group as a control group with oral administration of carbamazepine. All of the cases in intratympanic perfusion group were injected twice in the first week, then once a week consecutively. The patients were acupunctured 4-5 times in the whole course of treatment. All of the cases accepted Betahistine Mesylate, Mecobalamin and Vitamin B1 by oral intake at the same time. Pure tone audiogram and tinnitus matching were tested before the treatment immediately after the course of treatment, and were tested again after half a year's following up. RESULT: All of the cases accepted the whole treatment and were followed up for half a year successfully. The effective rate of the prednisolone group, dexamethasone group and the carbamazepine group was 48.6%, 33.3%, 44.0%, respectively; the control rate half a year after the treatment was 45.7%, 27.8%, 36.0%, respectively. There was no statistically significant difference in the effective rate and control rate between intratympanic perfusion group and carbamazepine group. There is a statistically significant difference both in the effective rate and the control rate between the prednisolone group and the dexamethasone group. Prednisolone may be better than dexamethasone in intratympanic perfusion for subjective tinnitus. CONCLUSION Intratympanic steroid injection has a positive effect on subjective tinnitus and may be considered to be an alternative treatment to subjective tinnitus.
Adolescent ; Adult ; Aged ; Carbamazepine ; administration & dosage ; therapeutic use ; Dexamethasone ; administration & dosage ; therapeutic use ; Drug Administration Routes ; Female ; Humans ; Male ; Middle Aged ; Prednisolone ; administration & dosage ; therapeutic use ; Prospective Studies ; Tinnitus ; drug therapy ; Treatment Outcome ; Young Adult

BACKGROUNDIt is important to choose an appropriate antiepileptic drug (AED) to manage partial epilepsy. Traditional AEDs, such as carbamazepine (CBZ) and valproate (VPA), have been proven to have good therapeutic effects. However, in recent years, a variety of new AEDs have increasingly been used as first-line treatments for partial epilepsy. As the studies regarding the effectiveness of new drugs and comparisons between new AEDs and traditional AEDs are few, it is determined that these are areas in need of further research. Accordingly, this study investigated the long-term effectiveness of six AEDs used as monotherapy in patients with partial epilepsy.

METHODSThis is a retrospective, long-term observational study. Patients with partial epilepsy who received monotherapy with one of six AEDs, namely, CBZ, VPA, topiramate (TPM), oxcarbazepine (OXC), lamotrigine (LTG), or levetiracetam (LEV), were identified and followed up from May 2007 to October 2014, and time to first seizure after treatment, 12-month remission rate, retention rate, reasons for treatment discontinuation, and adverse effects were evaluated.

RESULTSA total of 789 patients were enrolled. The median time of follow-up was 56.95 months. CBZ exhibited the best time to first seizure, with a median time to first seizure of 36.06 months (95% confidential interval: 30.64-44.07). CBZ exhibited the highest 12-month remission rate (85.55%), which was significantly higher than those of TPM (69.38%, P = 0.006), LTG (70.79%, P = 0.001), LEV (72.54%, P = 0.005), and VPA (73.33%, P = 0.002). CBZ, OXC, and LEV had the best retention rate, followed by LTG, TPM, and VPA. Overall, adverse effects occurred in 45.87% of patients, and the most common adverse effects were memory problems (8.09%), rashes (7.76%), abnormal hepatic function (6.24%), and drowsiness (6.24%).

CONCLUSIONThis study demonstrated that CBZ, OXC, and LEV are relatively effective in managing focal epilepsy as measured by time to first seizure, 12-month remission rate, and retention rate.

Adolescent ; Adult ; Anticonvulsants ; therapeutic use ; Carbamazepine ; analogs & derivatives ; therapeutic use ; China ; Epilepsies, Partial ; drug therapy ; Female ; Fructose ; analogs & derivatives ; therapeutic use ; Humans ; Male ; Middle Aged ; Piracetam ; analogs & derivatives ; therapeutic use ; Retrospective Studies ; Treatment Outcome ; Triazines ; therapeutic use ; Valproic Acid ; therapeutic use ; Young Adult