ABSTRACT
Objective:To analyze the characteristics of stereoelectroencephalography (SEEG) in children with drug-resistant epileptic spasms (ES), and to explore the surgical strategy of children with spastic seizure under the guidance of SEEG.Methods:The clinical data of 156 children with ES who were preoperatively evaluated in the Department of Neurosurgery Ward 3, Tsinghua University Yuquan Hospital from January 2014 to December 2021 were retrospectively reviewed.All children were evaluated in the second stage of stereotactic electrode placement after a non-invasive preoperative evaluation.The characteristics of intracranial EEG, surgical strategy and prognosis were analyzed.Results:A total of 19 eligible children were included, involving 13 boys and 6 girls.The age of first onset and surgical age of them ranged 1 month to 4 years, and 2 years to 13 years, respectively.The SEEG was divided into 3 types in children with ES at the onset.Five children were SEEG type A, presenting with the focal seizure discharges at the beginning and a gradual propagation to widespread fast-wave bursts.Ten children were SEEG type B, presenting a focal leading spike followed by diffused fast-wave bursts.Four children were SEEG type C, presenting a diffuse fast wave rhythm onset.Although some electrode discharges appeared slightly " leading", they covered more than one brain region.After focal resection or thermocoagulation, 13/19 patients did not have the onset of seizures, and 5/19 and 8/19 were graded as SEEG type A, and B, respectively.During the intermittent period of SEEG attacks in children with SEEG type A and B, a significant phenomenon of focal epileptic discharge consistent with the onset of the attack was observed, and surgical removal of these areas effectively controlled spastic seizures.Conclusions:Epileptic spasms may be triggered by a focal neocortical discharge.Intracranial EEG showed that the focal seizure onset evolves into spasm or a focal " leading spike" is a good indicator of surgical prognosis.
ABSTRACT
Background: This study was undertaken to find out the prevalence and clinical features of intractable epilepsy (IE) in a tertiary referral center.Methods: Study was conducted in a tertiary care hospital on 60 children with intractable epilepsy. Cases includes' intractable epilepsy is when seizures continue to occur despite maximally tolerated doses of more than two antiepileptics, occurrence of an average of one seizure per month for 18 months with no more than a 3 month seizure free period during these 18 months. Controls: epileptic children who had good control of seizures for the previous 18 months.Results: The prevalence of intractable seizures was 10% with maximum number of children 25 (41.6%) belonged to the 5-12 years. 15 (50%) children had daily seizures. Myoclonic seizures proved to be an important predictor of intractability. 4 children among the cases had history of family seizures, 6 children in cases had history febrile seizure, whereas, 6 children among the controls had history of family seizures, 8 children in controls had history febrile seizure.' 23.3% of children presented with Status epilepticus in the cases and 16.6% of the children in the controls. Remote symptomatic etiology 12(40%) is the commonest cause of seizure. 13 (43.3%) children in cases and 3 (10%) children among the controls had a history suggestive of birth asphyxia. EEG was abnormal in 17 (56.6%) cases when compared to 11 (36.6%) children in the controls. CT scan was abnormal in 14 (46.6%) cases and 10 (33.3%) controls. MRI was abnormal in 16 (53.3%) children of the cases and 8 (26.6%) children of the controls.Conclusions: The commonest cause of intractable epilepsy was perinatal asphyxia. Perinatal asphyxia can be prevented by good nutrition during pregnancy, regular antenatal check ups with detection of high risk pregnancy, promoting hospital deliveries and prompt resuscitation of newborn when required.
ABSTRACT
@#Ring chromosome 20 [r(20)] syndrome is typically characterized by intractable epilepsy, variable degrees of behavior problems and cognitive deficits, and an absence of or minimal dysmorphic features. Here we report a case diagnosed with r(20) syndrome exhibiting rare clinical manifestations of intractable epilepsy, growth failure, hypothyroidism, and cataract. This 17-year-old female patient who showed growth failure and no dysmorphic features had the first seizure at the age of 8 years. The seizure pattern was described as periods of non-convulsive status epilepticus with prolonged confusional state, motionless stare, mutism, and subtle motor seizures, lasting for minutes to hours. The interictal EEG showed bilateral synchronous, rhythmic high voltage delta waves intermixed with occasional spikes over the bilateral frontal areas. The seizures were refractory to medical treatments. Hypothyroidism and cataract were subsequently diagnosed at the age of 16 years and 17 years, respectively. Chromosome study showed a female genome with r(20) mosaicism. In conclusion, r(20) syndrome might cause multisystemic involvement, and therefore, comprehensive surveys of the central nervous system, ophthalmologic system, and endocrine system, among others, are crucial.
ABSTRACT
Background: Intractable epilepsy is the pragmatic problem during the treatment of active epilepsy in children. Several risk factors are associated with incidence of intractable/recurrent epilepsy. The current study was done to identify the risk and prognostic factors associated with recurrent epilepsy (RE).Methods: This descriptive study was conducted on 152 children with idiopathic or symptomatic epilepsy who are on two or more AEDs and who were in follow up in Neurology OPD and inpatients in medical ward at ICH&HC, Chennai. All patients underwent relevant investigations to identify the possible risk factors for incidence of RE in study population. Karyotyping was done for idiopathic cases.Results: Male preponderance was seen in the study (M:F-2:1). Risk factors such as male sex, age onset of seizures, type of seizures, developmental delay, CNS congenital anomalies, h/o perinatal injury, neuroabnormality, abnormal MRI and EEG was found to have statistically significant association with incidence of RE. No significant association was observed for the factors microcephaly, behavioural abnormalities, h/o febrile seizures and h/o status epilepticus with incidence of RE. No chromosomal abnormalities were detected in idiopathic cases.Conclusions: Early identification, risk factor analysis and understanding in the dynamics of the disease helps the physician in initiating the appropriate treatment, thereby avoiding the wrong therapy, low dose therapy and infrequent therapy. Above all identification of the risk factors helps in parental counseling and prepare them for expected outcome.
ABSTRACT
Introduction: Epilepsy is a common and diverse disorder with many different causes.Outcomes are varied with 60—70% of newly diagnosed people rapidly entering remission after starting treatment, and 20—30% developing a drug-resistant epilepsy with consequent clinical and psychosocial distress. Methods: It is a Descriptive Cross-sectional study which was conducted in Assam Medical College and Hospital, Dibrugarh from April 2014 to April 2016. A total of 42 patients of IE attending the neurology, paediatrics and medicine department were included in the study. Results: Forty-two patients (males 24, females 18) with intractable epilepsy were included for the study. Maximum patients were in between 20-40 years of age (42.9%) and their mean duration of epilepsy was13.2 ± 7.13 years. The seizure frequency varied from once every month to more than 200 per (mean 26.2 ± 24.17) month. Twenty-six patients (61.9%) had partial seizures, 8 (19.1%) patients had generalized seizures and 8(19.1%) had multiple seizure semiology. Thirty-six patients had risk factors of intractable epilepsy. Seven (16.6%) patients were having family history of epilepsy and 4(9.5%) patients had history of febrile seizures. Mesial temporal lobe sclerosis (MTLS) and birth hypoxia are the two major risk factors for intractable epilepsy. EEG was abnormal in 66.7% cases, with generalized background slowing in 19.1%, focal slowing in 14.2%, generalized epileptiform discharges in 9.5% and focal epileptiform discharges in 23.8%. CT brain was abnormal in 18(42.9%) patients. MRI brains were abnormal in 25 out-off 36 patients (69.4%). Carbamazepine was the most commonly used drug (57.1%) followed by clobazam (47.6%). Phenytoin, levetiracetum, Phenobarbitone, oxcarbazepine, zonisemide, lacosemide are the other AEDs used in combination. Conclusion: This study showed, patients with partial seizures, birth hypoxia, history of febrile seizures, family history of seizures, structural brain abnormalities and background EEG abnormalities were the most common risk factors for development of intractable epilepsy.
ABSTRACT
Deep brain disease stimulation (DBS) is commonly used to treat movement disorders such as Parkinson disease,and current evidence suggests that DBS may also be useful for refractory epilepsy and is affected by a variety of factors.Studies show that stimulation of the anterior nucleus of the thalamus and hippocampus may decrease the frequency of refractory seizures.The efficacy of stimulating other targets remains inconclusive.An absence of structural abnormality on imaging and electrode position are associated with stimulation efficacy.Certain seizure types may respond more favorably to specific targets.There are several factors that potentially predict seizure outcome following DBS,but more large-scale clinical trials are needed.
ABSTRACT
Objective:To observe the effect of Chaibei Zhixian decoction and peimine on Carbamazepine (CBZ) concentration, P-glycoprotein (P-gp) and multi drug resistance 1(MDR1) expression in the brain tissues of rats with refractory epilepsy, and to understand the contribution of Peimine in the compound prescription to treat the refractory epilepsy. Method:Epilepsy rat models were established by injecting kainic acid (KA) in the lateral ventricle. The successfully modeled rats were randomly divided into model group, CBZ group(0.12 g·kg-1),Chaibei Zhixian decoction+CBZ group(8.39 g·kg-1+0.12 g·kg-1), peimine+CBZ group(0.01 g·kg-1+0.12 g·kg-1) and sham operation group. After 60 days of intervention, the expression levels of P-glycoprotein (P-gp) and MDR1b mRNA in the brain cortex were detected by Western blot and quantitative real\|time fluorescence polymerase chain reaction(Real-time PCR),the contents of CBZ and 10,11-epoxidation of carbamazepine (CBZE) were measured by liquid chromatography-mass spectrometry (LC-MS). Result:Compared with sham group, the expression of P-gp/MDR1 in the cortex of model group was significantly increased (PPPPPPPConclusion:Chaibei Zhixian decoction and peimine may increase the content of CBZ and CBZE in the brain tissues in rats with intractable epilepsy by reducing the expression of MDR1/P-gp in the cortex.
ABSTRACT
Objective@#To assess the association of single nucleotide polymorphisms of multidrug resistance gene 1 (MDR1) with refractory epilepsy in children.@*Methods@#Peripheral blood samples were collected from 200 children with epilepsy and 100 healthy controls. Genomic DNA was extracted and subjected to PCR amplification, agarose gel electrophoresis and target site sequencing. Genotypes of rs1922242, rs2235048, rs10808072, rs868755 and rs1202184 loci of the MDR1 gene were analyzed.@*Results@#No significant difference was found in genotypic distribution and allelic frequencies of the rs1922242, rs2235048, rs10808072 and rs868755 loci between the drug-resistant and drug-sensitive groups. For the rs1202184 locus, a significant difference in genotypic distribution was found (P = 0.008). No significant difference was found in the frequencies of various haplotypes between the two groups.@*Conclusion@#Genotypes of the rs1202184 locus of the MDR1 gene are associated with refractory epilepsy in children, for which the AA genotype plays a dominant role.
ABSTRACT
Objective@#To study the effect of ketogenic diet (KD) on vascular endothelial function in children with intractable epilepsy.@*Methods@#Clinical informations of 14 children with intractable epilepsy in Fujian Medical University Affiliated Union Hospital from May 2014 to March 2018 were collected.Their blood index values were tested and retested before and after 3 months of KD treatment, including triglycerides (TG), total cholesterol (TC), high-density lipoprotein (HDL), low density lipoprotein (LDL), nitric oxide (NO), endothelin-1 (ET-1), and von Willebrand factor (vWF). These data were statistically analyzed by using repeated measurement analysis of variance.@*Results@#(1)Changes in blood lipid levels: the levels of TG and TC increased slightly [(1.08±0.14) mmol/L, (5.19±0.64) mmol/L vs.(0.97±0.10) mmol/L, (4.57±0.29) mmol/L]and the level of HDL decreased slightly [(1.19±0.08) mmol/L vs.(1.31±0.08) mmol/L]after 3 months of KD treatment, but the differences were not statistically significant in the above indexes (all P>0.05). The level of LDL before KD was significantly lower than that after 3 months of KD [(2.93±0.25) mmol/L vs.(3.73±0.40) mmol/L ], and the difference was statistically signi-ficant(P=0.034). (2)Assessment of vascular endothelial function: compared with before KD, all the levels of NO [(60.84±5.29) μmol/L vs.(66.45±5.39) μmol/L ], ET-1 [(1.24±0.30) ng/L vs.(2.13±0.78)ng/L] and vWF [(150.53±12.97) μg/L vs.(137.57±13.10) μg/L]had no statistically significant changes after 3 months of KD(all P>0.05).@*Conclusions@#KD treatment lasting 3 months in children with intractable epilepsy can raise the level of LDL, but can′t change the vascular endothelial function.
ABSTRACT
Objective To study the effect of ketogenic diet(KD)on vascular endothelial function in children with intractable epilepsy. Methods Clinical informations of 14 children with intractable epilepsy in Fujian Medical University Affiliated Union Hospital from May 2014 to March 2018 were collected. Their blood index values were tested and retested before and after 3 months of KD treatment,including triglycerides( TG),total cholesterol( TC),high－density lipoprotein( HDL),low density lipoprotein( LDL),nitric oxide( NO),endothelin －1( ET －1),and von Willebrand factor(vWF). These data were statistically analyzed by using repeated measurement analysis of variance. Results (1)Changes in blood lipid levels:the levels of TG and TC increased slightly[(1. 08 ± 0. 14)mmol/L, (5. 19 ± 0. 64)mmol/L νs.(0. 97 ± 0. 10)mmol/L,(4. 57 ± 0. 29)mmol/L]and the level of HDL decreased slightly [(1. 19 ± 0. 08)mmol/L νs.(1. 31 ± 0. 08)mmol/L]after 3 months of KD treatment,but the differences were not sta﹣tistically significant in the above indexes(all P＞0. 05). The level of LDL before KD was significantly lower than that after 3 months of KD[(2. 93 ± 0. 25)mmol/L νs.(3. 73 ± 0. 40)mmol/L ],and the difference was statistically signi﹣ficant(P﹦0. 034).(2)Assessment of vascular endothelial function:compared with before KD,all the levels of NO [(60. 84 ± 5. 29)μmol/L νs.(66. 45 ± 5. 39)μmol/L ],ET－1[(1. 24 ± 0. 30)ng/L νs.(2. 13 ± 0. 78)ng/L]and vWF[(150. 53 ± 12. 97)μg/L νs.(137. 57 ± 13. 10)μg/L]had no statistically significant changes after 3 months of KD(all P＞0. 05). Conclusions KD treatment lasting 3 months in children with intractable epilepsy can raise the level of LDL,but can′t change the vascular endothelial function.
ABSTRACT
Objective Ketogenic diet (KD) is one of the most important treatments of intractable childhood epilepsy.Its antiepileptic mechanism has been confirmed.With its wide application and dietary requirements,more selectable types have emerged.During the diet treatment,the combined use of antiepileptic drugs and vagus nerve stimulation,dietary adjustment and reduction of adverse reactions have gained certain clinical experience.In addition,the economic burden and quality of life for the treatment of ketogenic diet still need to be considered.Therefore,this article reviews the recent literature about ketogenic diet in the treatment of intractable childhood epilepsy and summarizes its clinical progress.
ABSTRACT
Objective To study the predictive value of magnetic resonance imaging (MRI),positron emission computed tomography (PET)and PET/MRI coregistration in intractable epilepsy of children.Methods A retrospec-tively analysis was performed based on the surgery data at the Department of Children Epilepsy Center of Peking Univer-sity First Hospital from September 2015 to March 2016.The clinical data,surgery and follow－up study data,interictal and ictal electroencephalogram,MRI,PET and PET/MRI coregistration data were collected.By comparison with the epi-leptogenic zone designed by pre－surgical workup,the accuracy of MRI,PET and PET/MRI coregistration in detecting lesion was assessed.In the patients who had no seizure during≥1 year follow－up,their sensitivity,specificity,positive predictive value,negative predictive value of MRI,PET and PET/MRI coregistration were calculated.Results (1)A total of 62 patients underwent surgery,30 boys and 32 girls. The average age on epilepsy onset was 2. 50 years (2 days－11.70 years),and average age on surgery was 5.10 years old(0.75－15.60 years old).(2)Surgical treat-ment of 62 cases included the resection of the focal or lobar (32 cases,51.6%),and the multilobar (16 cases, 25. 8%).Hemispherotomy was done in 14 cases (22.6%).During ≥1 year follow－up,seizure outcome was Engel class Ⅰ in 57 cases (91.9%)out of the 62 patients,Engel classⅡto Engel classⅣin 1 case,3 cases,and 1 case, respectively.(3)Referred to epileptogenic zone designed by presurgical workup,MRI represented 64.5%(40/62 ca-ses)results with accordance,PET and PET/MRI coregistration was 72.5%(45/62 cases)and 85.5%(53/62 cases), respectively,and the difference was significant(χ2＝7.25,P＝0.03).(4)Based on the patients of Engel class Ⅰ, their sensitivity and specificity were 66.7%,60.0% in MRI,75.4%,60.0% in PET %,and 85.9%,80.0% in PET/MRI coregistration,respectively.(5)There were 11 ＂non－lesion＂ cases of all focal cortical dysplasia in patholo-gy,and subtle structural abnormalities were de tected in 9 cases by reviewing MRI.Conclusions PET/MRI coregistra-tion can improve lesion detection of intractable epilepsy in children.
ABSTRACT
An uncommon female-limited intractable epilepsy, protocadherin (PCDH) 19-related epilepsy, is characterized by mutations in the PCDH 19 gene, located on chromosome X. Clinical symptoms include early onset, fever sensitivity, focal seizures and psychomotor retardation. PCDH 19-related epilepsy is unresponsive to conventional antiepileptic drugs (AEDs), but corticosteroid is reported to be effective in a few cases. We report a case of a 25-month-old girl who was admitted to our hospital due to developmental regression, accompanied by aggravated seizures with fever. Although several conventional AEDs were administered, the frequency and severity of seizures increased with mild fever, and the symptoms did not improve. Considering possible immune, and inflammatory involvement in seizure generation, the patient was administered corticosteroid treatment during the acute phase. Corticosteroid dramatically improved seizures and her development gradually. The patient was finally diagnosed with PCDH 19-related epilepsy in genomic evaluation. We observed the effect of corticosteroid on intractable epilepsy in patient with PCDH 19 mutation. If a female patient whose seizures are resistant to conventional AEDs or easily provoked by mild fever, has developmental delay or developmental regression, this may be an important clinical clue to the early diagnosis of PCDH 19-related epilepsy.
Subject(s)
Child, Preschool , Female , Humans , Adrenal Cortex Hormones , Anticonvulsants , Drug Resistant Epilepsy , Early Diagnosis , Epilepsy , Fever , SeizuresABSTRACT
Objective To investigate the clinical features,karyotype,and the prenatal diagnosis for his sibling of a Chinese patient with rare ring chromosome 20 syndrome induced intractable epilepsy.Methods The clinical data of the patient diagnosed in Peking University People's Hospital were collected.The clinical manifestations,chromosome karyotype were summarized.Results The proband,a boy,started to show intermittent tonic seizures or atypical absence seizures and psychomotor retardation from the age of 11 months.Several anti-epilepsy drugs and globulin had been tried without effect.Common karyotype analysis and epilepsy-related genes analysis revealed no abnormality.However,abnormal karyotype 46,XY,r(20)(p13q13.3) in his peripheral blood lymphocytes was found by high resolution chromosome karyotype analysis with 550 G-banding,and the diagnosis of ring chromosome 20 syndrome,type Ⅱ was confirmed.The mother of the patient underwent amniocentesis at the midterm of the second pregnancy.The cultured amniocytes karyotypes were normal.The second child(a boy) of the family was 1 year old without epilepsy and the psychomotor development was normal.Conclusions Ring chromosome 20 syndrome is a rare human chromosome abnormality.The syndrome is associated with epileptic seizures,behavior disorders and mental retardation.Since karyotype testing is not a routine investigation for the patient with epilepsy,the diagnosis of ring chromosome 20 syndrome is usually delayed or misdiagnosed.The karyotype analysis should be considered for the etiological study of the patients with intractable epilepsy with unknown origin.
ABSTRACT
Objective To explore the changes of multidrug resistant-associated protein (MRP) 1 expression and MRP2 expression in the peripheral blood mononuclear cells in intractable epileptic children after accepting the Levetiracetam (LEV) add-on therapy.Methods From June 2012 to September 2015,68 intractable epileptic children who had been diagnosed at the Outpatient Department of the Xuzhou Children's Hospital were randomly selected and given the LEV add-on therapy.The expressions of MRP1 and MRP2 in their peripheral blood mononuclear cells were detected by real-time fluorescence quantitative polymerase chain reaction and Western blot before the LEV add-on therapy and 6 months after the LEV add-on therapy.Results (1) Compared to the expressions of MRP1 mRNA and MRP2 mRNA in the peripheral blood mononuclear of intractable epileptic children before therapy,the expressions of MRP1 mRNA (0.23 ± 0.02) and MRP2 mRNA (0.17 ±0.03) after therapy were obviously down-regulated with significant differences(tMRP1 =32.77,P ＜ 0.01;tMRP2 =26.73,P ＜ 0.01).(2) The relative expressions of protein of MRP1 and MRP2 in the peripheral blood mononuclear of intractable epileptic children before therapy were 0.59 ± 0.03,0.53 ± 0.02 respectively,and they were 0.39 ± 0.03,0.38 ± 0.02 after therapy.Compared with those before therapy,the protein expressions of MRP1 and MRP2 were down-regulated,and the differences were significant (tMRP1 =11.55,P＜0.01;tMRP2 =9.87,P＜0.01).Conclusions The expressions of MRP1 and MRP2 mRNA and protein in the peripheral blood mononuclear cells in some intractable epileptic children can be down-regulated if patients receive the LEV add-on therapy,and LEV may control the seizures of intractable epileptic children by down-regulating the expressions of MRP1 and MRP2,then reduce the occurrence and development of drug-resistant epilepsy.
ABSTRACT
Objective To investigate the effect of topiramate in the treatment of intractable epilepsy in children. Methods 120 cases with intractable epilepsy from May 2012 to May 2017 were randomly divided into 2 groups, the control group used antiepileptic drug treatment, study group combined application of anti epileptic drugs targeted nursing application. Results The clinical effect of the treatment group was better than that of the control group (P< 0.05). The frequency of epileptic seizure in the treatment group was significantly higher than that in the control group (P<0.05). Conclusion In the treatment of intractable epilepsy in children, the application of topiramate in the treatment of basic care is helpful to improve the effect of treatment, the effect is ideal, and should be further popularized in clinical practice.
ABSTRACT
Objective To investigate the results of MRI and electroencephalogram examination and pathological examination of drug refractory epilepsy. Methods 70 cases with drug refractory epilepsy were treated with systemic antiepileptic drugs, but they still could not control their condition, and they were given MRI, EEG and pathological examination respectively. MRI, EEG and pathological findings of 70 cases were recorded, and the conclusion was drawn after statistical analysis. Results Compared with pathological examination, the localization accuracy of MRI for epileptogenic foci and related lesions was 88.57%, the accuracy of localization of EEG for epileptogenic foci and related disease was 51.43%, the difference was statistically significant (P<0.05); MRI examination of the epileptogenic lesions and qualitative accuracy the rate of up to 75.71%. Conclusion According to the patients with drug intractable epilepsy, the coincidence rate of MRI examination and pathological examination is higher. Compared with EEG, it can provide clinicians with more reliable basis for the diagnosis and treatment of drug refractory epilepsy. It is helpful to protect the curative effect of these patients.
ABSTRACT
Objective To investigate the effect of topiramate in the treatment of intractable epilepsy in children. Methods 120 cases with intractable epilepsy from May 2012 to May 2017 were randomly divided into 2 groups, the control group used antiepileptic drug treatment, study group combined application of anti epileptic drugs targeted nursing application. Results The clinical effect of the treatment group was better than that of the control group (P< 0.05). The frequency of epileptic seizure in the treatment group was significantly higher than that in the control group (P<0.05). Conclusion In the treatment of intractable epilepsy in children, the application of topiramate in the treatment of basic care is helpful to improve the effect of treatment, the effect is ideal, and should be further popularized in clinical practice.
ABSTRACT
Objective To investigate the results of MRI and electroencephalogram examination and pathological examination of drug refractory epilepsy. Methods 70 cases with drug refractory epilepsy were treated with systemic antiepileptic drugs, but they still could not control their condition, and they were given MRI, EEG and pathological examination respectively. MRI, EEG and pathological findings of 70 cases were recorded, and the conclusion was drawn after statistical analysis. Results Compared with pathological examination, the localization accuracy of MRI for epileptogenic foci and related lesions was 88.57%, the accuracy of localization of EEG for epileptogenic foci and related disease was 51.43%, the difference was statistically significant (P<0.05); MRI examination of the epileptogenic lesions and qualitative accuracy the rate of up to 75.71%. Conclusion According to the patients with drug intractable epilepsy, the coincidence rate of MRI examination and pathological examination is higher. Compared with EEG, it can provide clinicians with more reliable basis for the diagnosis and treatment of drug refractory epilepsy. It is helpful to protect the curative effect of these patients.
ABSTRACT
BACKGROUND AND PURPOSE: Antiepileptic drug (AED)-associated cutaneous adverse drug reactions can lead to the discontinuation of medications. The aim of this study was to determine the long-term efficacy and safety of performing desensitization to oxcarbazepine. METHODS: This study involved 20 patients who exhibited cutaneous adverse drug reactions associated with oxcarbazepine use between July 2009 and March 2016 at Samsung Medical Center. All of the participants had to discontinue oxcarbazepine despite presenting initially positive responses. Human leukocyte antigen genotyping was performed to detect the genetic predisposition to Stevens-Johnson syndrome. The desensitization to oxcarbazepine was performed with a starting dosage of 0.1 mg/day. Efficacy was evaluated by comparing the frequency of seizures before and at 1 and 3 years after desensitization. Adverse events occurring during desensitization and the retention rate after desensitization were also investigated. RESULTS: Nineteen patients (95%) safely completed the desensitization protocol. One withdrew owing to emotional problems that appeared to be associated with oxcarbazepine. The follow-up period was 4.6±1.2 years (mean±SD), and oxcarbazepine was maintained for more than 3 years after desensitization in 15 patients (83.3%). The response rates were 84.2% and 77.8% at 1 and 3 years after desensitization, respectively. Eight patients remained seizure-free for 3 years, and two discontinued all AEDs. Transient adverse reactions such as mild rash and itching were reported by five patients during desensitization. CONCLUSIONS: This study has demonstrated the long-term efficacy and safety of desensitization to oxcarbazepine in patients exhibiting cutaneous adverse drug reactions. This favorable outcome should encourage the implementation of desensitization in patients presenting with hypersensitivity to oxcarbazepine as an alternative strategy in clinical practice.