Electrode location and clinical outcome in hippocampal electrical stimulation for mesial temporal lobe epilepsy

PurposeTo study the clinical outcome in hippocampal deep brain stimulation (DBS) for the treatment of patients with refractory mesial temporal lobe epilepsy (MTLE) according to the electrode location.MethodsEight MTLE patients implanted in the hippocampus and stimulated with high-frequency DBS were included in this study. Five underwent invasive recordings with depth electrodes to localize ictal onset zone prior to chronic DBS. Position of the active contacts of the electrode was calculated on postoperative imaging. The distances to the ictal onset zone were measured as well as atlas-based hippocampus structures impacted by stimulation were identified. Both were correlated with seizure frequency reduction.ResultsThe distances between active electrode location and estimated ictal onset zone were 11 ± 4.3 or 9.1 ± 2.3 mm for patients with a >50% or <50% reduction in seizure frequency. In patients (N = 6) showing a >50% seizure frequency reduction, 100% had the active contacts located <3 mm from the subiculum (p < 0.05). The 2 non-responders patients were stimulated on contacts located >3 mm to the subiculum.ConclusionDecrease of epileptogenic activity induced by hippocampal DBS in refractory MTLE: (1) seems not directly associated with the vicinity of active electrode to the ictal focus determined by invasive recordings; (2) might be obtained through the neuromodulation of the subiculum.     

Long-term seizure outcome in patients with juvenile absence epilepsy; a retrospective study in a tertiary referral center

PurposeThe study aim was to evaluate pharmacotherapy effects and long-term seizure outcomes in patients with juvenile absence epilepsy (JAE) during a five-year follow-up period. The secondary aim was to identify factors from patient history and determine their influence on seizure control.MethodWe retrospectively studied 46 patients with JAE in the period between 2006 and 2011. The age at seizure onset, onset seizure type, family history of epilepsy, status epilepticus in history, medication history, and the rate of seizure control were studied.ResultsThere were 30 females (65.2%) and 16 males (34.8%) in the study. The mean age at seizure onset was 12.9 ± 5.6 years (ranged from 3 to 28 years). In 30 patients (65.2%), seizure onset was with absences, in 15 patients (32.6%) with generalized tonic-clonic seizure (GTCS), and in 1 patient (2.2%) with absence status. In 43 patients (93.5%), GTCS occurred in the course of the disease. Family history for epilepsy was positive in 10 patients (21.7%). In the five-year follow-up period, seizure freedom (Group 1) was achieved in 7 patients (15.2%). In total, 22 patients (47.8%) were classified into the groups involving very poor seizure control and antiepileptic drug resistance (Groups 5 and 6). The mean number of antiepileptic drugs (AEDs) used in the course of the disease in appropriate therapeutic doses was 3.8 ± 2.3 (1–10 AEDs).ConclusionThe study results show that almost half of JAE patients have poor seizure control with a high rate of pharmacoresistance. The outcome of JAE can be very uncertain.     

Incidence of atypical handedness in epilepsy and its association with clinical factors

The incidence of atypical handedness (left-handedness and ambidexterity) in patients with epilepsy, particularly its association with major clinical factors, is not well established. We evaluated a full range of clinical variables in 478 patients with epilepsy from the United States and Korea. With the Edinburgh Handedness Inventory, handedness was established as both a categorical variable (right-handed, left-handed, ambidextrous) and a continuous variable. Seizures were classified as complex or simple partial, primary generalized, or generalized tonic–clonic. The relationship between handedness and a range of clinical findings was explored. The overall incidence of atypical handedness in our patients was higher than in the general population (13.6%) and significantly higher in the U.S. patient group (17.6%) than in the Korean patients (8.8%). Handedness was not associated with sex; age; seizure type; age at onset; type, side, or site of EEG or brain imaging abnormalities; family history of seizures; refractory epilepsy; or history of epilepsy surgery.     

Handedness,eyedness, and hand–eye crossed dominance in patients with different addictions

ObjectiveSome neurologic and psychiatric disorders such as schizophrenia, depression, autism and migraine are referred to as cerebral lateralization abnormalities. In this study the possible relationships among handedness, eye dominance, and crossed hand–eye dominance in patients with different addictions mentioned above are investigated.MethodsThirty three patients with cigarette, 35 patients with alcohol, 133 patients with heroin, 117 patients with hashish, 13 patients with drug addictions and 102 age matched controls were included in the study. Six of 13 patients with drug addictions were addicted to diazepam, 5 to pethidine and 2 to clonazepam. The patient group included 307 men and 24 women who ranged in age from 15 to 70 years. Handedness was ascertained by using the Edinburgh Handedness Inventory. Eye dominance was measured only by the near-far alignment test. Diagnoses were made on the basis of information provided from clinical interviews and Structured Clinical Interview for DSM-IV.ResultsPatients with heroin and hashish had a significantly increased frequency of left-handedness in comparison with the other patients and controls (chi square = 29.36, p < 0.001). Patients with cigarette, alcohol, heroin and hashish addictions had a significantly increased frequency of left-eyedness in comparison with controls (chi square = 25.24, p < 0.01). Also, patients with cigarette, alcohol, heroin and hashish addictions had a significantly increased frequency of the crossed hand–eye dominance in comparison with controls (chi square = 19.11, p < 0.01).ConclusionsDifferent addictions such as cigarette, alcohol, heroin and hashish may be associated with abnormal handedness distribution and accepted as cerebral lateralization abnormalities.     

Can emotional stress trigger the onset of epilepsy?

ObjectiveThe aim of this study was to investigate the potential role of an acute adverse stress as “trigger” for the onset of epilepsy.MethodsAmong 4618 consecutive patients, twenty-two reported a major life event within three months before the onset of epilepsy.ResultsAll patients had focal epilepsy except one with idiopathic generalized epilepsy. The temporal lobe was involved in 90% of patients with focal epilepsy. More precisely, 13 patients (62% of patients with focal epilepsy) had medial temporal lobe epilepsy (MTLE), two had lateral temporal lobe epilepsy, four had temporoparietooccipital junction epilepsy, and two patients had central lobe epilepsy. The mean age and the median age at onset of epilepsy for patients with MTLE were both 38 years (range: 9.5–65 years). Ten patients had right and three had left MTLE. Among patients with focal epilepsy, MRI was abnormal in 7 (33%) with hippocampal sclerosis in four, periventricular nodular heterotopia in two, and complex cortical dysgenesis in one. The mean age at onset of epilepsy for patients with brain lesions was 26 years (range: 9.5–49). Twelve patients (54%) reported a death as a triggering factor for the onset of their epilepsy. Seven patients (32%) reported that a relationship of trust had been broken. Three patients (14%) had been subjects of violence. No patient reported sexual abuse as a triggering factor.ConclusionThis study provides evidence that some patients (5/1000 patients) began their seizures in the wake of significant life events. The average age at onset of epilepsy is quite late, around age 30, even in the presence of brain lesions. These patients are emotionally and affectively more prone to have consequences of a stressful life event. The recognition and management of such situations may bring significant relief with improvement of the control of epilepsy.     

Is ‘burned-out hippocampus’ syndrome a distinct electro‐clinical variant of MTLE-HS syndrome?

AimTo study the clinical, electrophysiological and imaging characteristics of patients with unilateral mesial temporal lobe epilepsy (MTLE) with contralateral ictal onset on scalp EEG, viz. ‘burned-out hippocampus’ syndrome (MTLE-BHS).MethodsMTLE-BHS was defined as TLE with unilateral hippocampal sclerosis (HS) without any dual pathology on MRI and contralateral ictal onset on scalp EEG, unlike in classical hippocampal sclerosis (HS). Consecutive “MTLE-BHS” patients evaluated at our Centre for Comprehensive Epilepsy Care from January 2005 to July 2014 were studied. Twenty-five cases of classic MTLE-HS operated during the same period were also analyzed for comparison.ResultsSeventeen patients were diagnosed to have MTLE-BHS. Mean age of seizure onset was 9.5 ± 7.7 years and the mean duration of epilepsy was18.2 ± 7.3 years. Epigastric aura was more common in MTLE-HS and fear, secondary generalized seizures and temporal polar changes on MRI were more prevalent in the MTLE-BHS subgroup. In the latter group, five (29%) exhibited seizure semiology and 2 (12%) had interictal discharges discordant to the side of MTS. Eight (47%) patients in the MTLE-BHS sub-group had normal medial temporal volume on Scheltens scale. Eight patients among MTLE-BHS underwent surgery (4 following intracranial monitoring that localized to the side of HS) with Engel class I outcome at 1 year follow-up in 6 and Engel class II outcome in 2.ConclusionAttenuation of ipsilateral fast ictal rhythms on scalp EEG as well as neocortical changes are likely to be deterministic factors for MTLE-BHS as opposed to the severity of hippocampal atrophy. Considering good post-operative outcomes, intracranial monitoring for surgical selection is not mandatory in MTLE-BHS despite discordant semiology and ictal onset, in the presence of inter-ictal, functional imaging and neuropsychology data concordant to the side of HS.     

Lennox–Gastaut syndrome in south Iran: Electro-clinical manifestations

PurposeLennox–Gastaut syndrome (LGS) is an uncommon epileptic encephalopathy. In this study, we tried to determine the clinical and EEG characteristics of patients with LGS in south Iran.MethodsIn this retrospective study, all patients with a clinical diagnosis of LGS were recruited at the outpatient epilepsy clinic at Shiraz University of Medical Sciences from 2008 through 2012. Age, gender, age at seizure onset, seizure type(s), epilepsy risk factors, EEG and imaging findings of all patients were registered routinely.ResultsDuring the study period, 2500 patients with epilepsy were registered at our epilepsy clinic. One-hundred and thirty-five patients (5.4%) were diagnosed as having LGS. Age of onset (mean ± standard deviation) was 3.2 ± 3.8 years. In 14 (10.4%) patients, age of onset was above 8 years. Eighty-three patients (61.5%) were male and 52 (38.5%) were female. The most common seizure type was tonic, followed by generalized tonic–clonic and myoclonic seizures. The most common EEG finding was slow spike-wave complexes. The most common abnormal MRI finding was brain atrophy.ConclusionLGS is an uncommon epileptic encephalopathy characterized by multiple seizure types, a specific electroencephalographic pattern and psychomotor retardation, beginning in childhood. However, variants of this classical triad including atypical EEG findings, normal psychomotor function, and late-onset disease could be seen in some patients. These atypical findings in a patient with typical history for LGS should not deter from the correct diagnosis. The mainstay for making a correct syndromic diagnosis is a detailed clinical history.     

Clinical characteristics and prognosis of mah-jong-induced epilepsy: A cohort review of 56 patients

ObjectiveThe objective of this study was to investigate the clinical characteristics of reflex epilepsy elicited by playing mah-jong in Western China.MethodsConsecutive patients with reflex epilepsy triggered by mah-jong from Jan 2011 to April 2014 were recruited at West China Hospital. Each patient underwent EEG recording and brain MRI scan. All clinical data were reviewed. Each patient had at least 12 months of follow-up.ResultsFifty-six patients were included, with majority of them being male (52/56, 92.9%). Mean age was 43.9 ± 10.0 years. Mean age at seizure onset was 35.1 ± 16.3 years. Mean duration of reflex epilepsy was 4.0 ± 2.2 years. The mean follow-up time was 32.0 ± 13.2 months. Seventeen patients (30.4%) took antiepileptic drugs. Regardless of the medication, all 14 patients who avoided playing mah-jong reached seizure freedom, and 22 of the 42 patients (52.4%) who still played mah-jong continued to have seizures (p < 0.05).ConclusionsReflex epilepsy induced by mah-jong is a unique type of epilepsy in the Chinese population and usually occurs in adults after a long time lag of playing mah-jong. Avoiding the triggers is the most effective treatment for epilepsy.     

Initial response to antiepileptic drugs in patients with newly diagnosed epilepsy

This study aimed to identify factors predicting the response to antiepileptic drugs in patients with newly diagnosed epilepsy. We prospectively studied 176 patients with newly diagnosed epilepsy. Patients were included if they had a history of two or more clinically definite unprovoked seizures, or had a definite epileptic focus on MRI or epileptiform discharges on electroencephalography if they had suffered only one seizure. The primary endpoint was seizure freedom during the initial 6 months of antiepileptic drug treatment. The secondary endpoint was the time to the first seizure during the maintenance period of antiepileptic drug treatment. A total of 100 patients were included, and seizure freedom for 6 months was achieved in 73 patients. The response to antiepileptic drugs was significantly lower in patients with early age at seizure onset (⩽16 versus >16 years old, odds ratio = 4; 95% confidence interval [CI] 1.5–12.9; relative risk = 1.4; 95% CI 1.1–1.8). In addition, the time to the first seizure during the maintenance period was significantly earlier in patients with age at seizure onset ⩽16 years compared with those with age at seizure onset >16 years on the Kaplan–Meier survival analysis (p = 0.011). Early age at seizure onset is an important factor influencing the response to antiepileptic drugs in patients with newly diagnosed epilepsy.     

Recognition of facial emotions and identity in patients with mesial temporal lobe and idiopathic generalized epilepsy: An eye-tracking study

PurposeTo describe visual scanning pattern for facial identity recognition (FIR) and emotion recognition (FER) in patients with idiopathic generalized (IGE) and mesial temporal lobe epilepsy (MTLE). Secondary endpoint was to correlate the results with cognitive function.MethodsBenton Facial Recognition Test (BFRT) and Ekman&Friesen series were performed for FIR and FER respectively in 23 controls, 20 IGE and 19 MTLE patients. Eye movements were recorded by a Hi-Speed eye-tracker system. Neuropsychological tools explored cognitive function.ResultsCorrect FIR rate was 78% in controls, 70.7% in IGE and 67.4% (p = 0.009) in MTLE patients. FER hits reached 82.7% in controls, 74.3% in IGE (p = 0.006) and 73.4% in MTLE (p = 0.002) groups. IGE patients failed in disgust (p = 0.005) and MTLE ones in fear (p = 0.009) and disgust (p = 0.03). FER correlated with neuropsychological scores, particularly verbal fluency (r = 0.542, p < 0.001). Eye-tracking revealed that controls scanned faces more diffusely than IGE and MTLE patients for FIR, who tended to top facial areas. A longer scanning of the top facial area was found in the three groups for FER. Gap between top and bottom facial region fixation time decreased in MTLE patients, with more but shorter fixations in bottom facial region. However, none of these findings were statistically significant.ConclusionFIR was impaired in MTLE patients, and FER in both IGE and MTLE, particularly for fear and disgust. Although not statistically significant, those with impaired FER tended to perform more diffuse eye-tracking over the faces and have cognitive dysfunction.     

High spirituality may be associated with right hemispheric lateralization in Korean adults living with epilepsy

PurposeAlthough it is known that epilepsy and spirituality are related, spirituality in epilepsy has received relatively little clinical and scientific attention. Therefore, we investigated which epilepsy-related factors are associated with high spirituality in Korean adults living with epilepsy.MethodsThis cross-sectional study was conducted in two university hospitals in Korea. Spirituality was assessed using the 6-item Spirituality Self-Rating Scale (SSRS). The participants were categorized into high and low spirituality groups according to the median SSRS score. The presumptive seizure onset zone was determined based on the clinical semiology, electroencephalography, and magnetic resonance imaging findings.ResultsOf the 180 participants, 61.7% declared that they had a religious affiliation. The median SSRS score was 15 (interquartile range: 7, 22). The high spirituality subgroup consisted of 92 (51.1%) participants. In the univariate analyses, the high spirituality group was significantly associated with female sex (p < 0.05), older age (p < 0.01), longer epilepsy duration (p < 0.05), polytherapy (p < 0.05), complex partial seizure (p < 0.05), levetiracetam or topiramate usage (p < 0.05), and a right-lateralized seizure onset zone. The multiple logistic regression analysis identified right hemispheric lateralization as the only independent factor associated with high spirituality (odds ratio: 2.410, 95% confidence interval: 1.051–5.528, p < 0.05).ConclusionsHigh spirituality may be associated with right hemispheric lateralization but not with the temporal localization of the seizure onset zone in Korean adults with epilepsy.     

Triggers and techniques in termination of partial seizures

ObjectiveGrowing interest in seizure prediction exists as a means to deliver newer antiepileptic therapies, though patient self-termination of seizures has received little attention.MethodsTwo hundred twenty-three patients able to recognize seizure onset were surveyed in an outpatient epilepsy clinic. A seven-question survey administered prospectively assessed self-reported seizure prediction and clinical techniques used for self-termination. Survey responses targeted percentage predictability of seizures, timing of clinical prediction, likelihood/timing of termination, frequency and effectiveness of methods used, and perspectives of patient and physician belief in self-termination.ResultsTwo hundred twenty-three patients (89 males) with a mean age of 42.7 years, average duration of epilepsy of 20.8 years and monthly mean seizure frequency of 4.1 comprised the study group. Thirty-eight percent completed >75% of the survey. Prior treatment included a mean of 6.0 AEDs (40/192 had surgery); 65% had ongoing seizures. Sixty percent of 223 patients reported a history of an aura, and 39% consistently noted auras for >75% of their current seizures. Of the patients with auras, seizure triggers were reported in 74%, with worry and stress (N = 69), sleep deprivation (N = 60), and missed medication (N = 56) most frequently cited. Seventeen percent were positive/somewhat sure they could predict onset, with approximately 20% noting rapid onset in <15 seconds. Twenty-two of 82 noted that they had some ability to self-terminate their seizures, and 9% were positive that they could do so. Methods to self-terminate were effective (>75% certainty) in 35% (26/75). The primary methods were lying down/resting and taking extra medication.ConclusionThe majority of patients with partial seizures recognize triggers of seizure onset. In addition, more than one-third believe they can effectively self-terminate their partial-onset seizures. Lying down, resting, and taking extra medication were the most common techniques instituted by patients. Correlating clinical symptoms at seizure onset with termination may help improve the sensitivity in seizure prediction.     

Detection of hippocampal atrophy in patients with temporal lobe epilepsy: A 3-Tesla MRI shape

In patients with mesial temporal lobe epilepsy (MTLE), brain MRI often detects hippocampal sclerosis (HS). Almost half of patients with MTLE do not show any hippocampal damage on visual or volumetric assessment. Here, we wished to prospectively assess 65 patients with MTLE (41 women, mean age: 39 ± 10 years, range: 21–69; right (12/65 patients) (MRI-negative) nMTLE; right (14/65 patients) (MRI-positive with HS) pMTLE; left (24/65 patients) nMTLE; and left (15/65 patients) pMTLE) using shape analysis (SA). There were significant differences among pMTLE versus nMTLE for age at seizure onset (20.2 ± 12.8 vs. 31.8 ± 16.7 years; p = .0029), duration of epilepsy (14.6 ± 12.7 vs. 21.3 ± 9.6 years; p = .0227), risk of refractoriness (p = .0067), frequency of antecedent febrile convulsions (FCs) (p < .001), as well as a history of epilepsy or FCs (p = .0104). All the subjects underwent the same 3-Tesla MRI protocol. Shape analysis of hippocampal formation was conducted comparing each group versus 44 matched controls.In all four subgroups, SA detected a significant atrophy in the corresponding hippocampus that coincided with the epileptogenic area. The damage was significantly more severe in patients with pMTLE (F value: 5.00) than in subgroups with nMTLE (F value: 3.50) and mainly corresponded to the CA1 subregion and subiculum. In the patients with MTLE, SA detects hippocampal damage that lateralizes with the epileptogenic area. Such damage is most prominent in the CA1 subregion and subiculum that are crucial in the pathogenesis of MTLE.     

Clinical characteristics and treatment responses in new-onset epilepsy in the elderly

PurposeEpidemiologic studies have shown that the incidence of epilepsy is the highest in the elderly population. Because the elderly constitutes the most rapidly growing population, epilepsy in this group is an important health issue worldwide. To identify the characteristics of epilepsy in the elderly, we reviewed our experience at a tertiary referral center in Japan.MethodsWe searched all electronic medical records of the past 6 years at the epilepsy clinic of the hospital affiliated to our University-affiliated hospital. We defined an elderly person as an individual aged 65 years and above. All patients underwent history and physical examinations, 3 T magnetic resonance imaging and/or computer tomography, and electroencephalogram (EEG). The diagnosis of epilepsy, age of onset, etiology, and antiepileptic medication were recorded.ResultsWe identified 70 patients who developed epilepsy after the age of 65 years. The mean age of seizure onset was 73.1 years and 52.9% patients were males. Complex partial seizures (CPS) without secondarily generalization (n = 33, 47.1%) were most frequent. The most frequent diagnosis was temporal lobe epilepsy (n = 50, 71.4%). Etiological diagnosis was possible in nearly 50% patients, including those with cerebrovascular disease. A clear cause of epilepsy was not found (i.e., non-lesional epilepsy) in 52.8% patients. Interictal EEG revealed focal epileptiform discharges in 72.9% (n = 51) patients. Of the 54 patients who were followed more than 1 year, 42 patients (77.8%) were on antiepileptic monotherapy and 52 patients (96.3%) had been seizure-free for more than 1 year.ConclusionThe most frequent diagnosis in our cohort of elderly persons with new-onset epilepsy was temporal lobe epilepsy. Non-lesional temporal lobe epilepsy was not uncommon. Epileptogenecity was relatively low in elderly patients and they responded well to antiepileptic medication.     

Distribution of regional gray matter abnormalities in a pediatric population with temporal lobe epilepsy and correlation with neuropsychological performance

ObjectiveThe goals of the work described here were to determine if hippocampal and extrahippocampal atrophy in children with temporal lobe epilepsy (TLE) follows a pattern similar to that in adult patients, and to assess the clinical and neuropsychological relevance of regional brain atrophy in pediatric TLE.MethodsChildren with symptomatic TLE (n = 14: 9 with mesial TLE due to hippocampal atrophy and 5 with TLE due to neocortical lesions), healthy children (n = 14), and 9 adults with mesial temporal lobe epilepsy (MTLE) were compared using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI). The children underwent a comprehensive neuropsychological battery.ResultsChildren with MTLE with unilateral hippocampal atrophy (n = 9) exhibited a significant reduction in gray matter in the hippocampus ipsilateral to the seizure origin and significant atrophy in the ipsilateral cingulate gyrus and contralateral middle frontal lobe. Children with TLE (n = 14) exhibited a significant reduction in the gray matter of the ipsilateral hippocampus and parahippocampal gyrus. There was a correlation between gray matter volume in children with TLE and scores on several neuropsychological tests. Atrophy in pediatric patients with MTLE was less extensive than that in adults, and involved the hippocampi and the frontal cortex.ConclusionsSimilar to adult MTLE, pediatric MTLE is associated with hippocampal and extrahippocampal cell loss. However, children display less intense quantifiable gray matter atrophy, which affects predominantly frontal lobe areas. There was a significant association between volume of gray matter in medial temporal and frontal regions and scores on neuropsychological tests. In childhood, TLE and the concomitant cognitive/behavior disturbances are the result of a damaged neural network.     

Focal cortical dysplasias in temporal lobe epilepsy surgery: Challenge in defining unusual variants according to the last ILAE classification

ObjectiveFocal cortical dysplasias (FCDs) represent a common architectural cortical disorder underlying pharmacoresistant focal epilepsy. The recent ILAE classification defines different types of FCDs based on their histopathological features, MRI imaging, and presumed pathogenesis; however, their clinical features and their prognostic significance are still incompletely defined. In addition, the combination of different histopathological abnormalities can represent “unusual” subtypes that can be difficult to classify. The aim of our study was to analyze the incidence and the significance of these “unusual” subtypes of FCDs in drug-resistant mesial temporal lobe epilepsy (MTLE).MethodsWe retrospectively analyzed 133 patients consecutively submitted to tailored anteromesial temporal lobe resection for pharmacoresistant MTLE. Seizure onset, seizure duration, age at surgery, and postoperative seizure outcome were evaluated in relation to the different neuropathological groups defined according to the new ILAE classification.ResultsFocal cortical dysplasias were found in 80 out of 133 patients. Six patients were affected by isolated FCD type I, 12 patients by FCD type II, and 44 patients by FCD type III. Furthermore, we found 18 “atypical” cases (20.5% of all FCD cases and 26.6% of FCDs associated with a principal lesion): 10 cases of associated FCD type II–hippocampal sclerosis (HS) and 8 cases associated with FCD II–epilepsy-associated tumors (EATs).ConclusionOur results indicate that “unusual” subtypes of FCDs, in particular associated FCD type II, are not uncommon findings, suggesting that they deserve a classification recognition. Similarities in seizure outcome and immunohistochemical and molecular evidences, shared by FCD type II + EATs and EATs, suggest a common pathogenic link. The choice to create a specific unifying class or, on the contrary, to also include “associated FCD type II” in the definition of the new unifying class FCD type III should be further discussed.     

A predictive risk model for medical intractability in epilepsy

ObjectiveThis study aimed to investigate early predictors (6 months after diagnosis) of medical intractability in epilepsy.MethodsAll children < 12 years of age having two or more unprovoked seizures 24 h apart at Xinhua Hospital between 1992 and 2006 were included. Medical intractability was defined as failure, due to lack of seizure control, of more than 2 antiepileptic drugs at maximum tolerated doses, with an average of more than 1 seizure per month for 24 months and no more than 3 consecutive months of seizure freedom during this interval. Univariate and multivariate logistic regression models were performed to determine the risk factors for developing medical intractability. Receiver operating characteristic curve was applied to fit the best compounded predictive model.ResultsA total of 649 patients were identified, out of which 119 (18%) met the study definition of intractable epilepsy at 2 years after diagnosis, and the rate of intractable epilepsy in patients with idiopathic syndromes was 12%. Multivariate logistic regression analysis revealed that neurodevelopmental delay, symptomatic etiology, partial seizures, and more than 10 seizures before diagnosis were significant and independent risk factors for intractable epilepsy. The best model to predict medical intractability in epilepsy comprised neurological physical abnormality, age at onset of epilepsy under 1 year, more than 10 seizures before diagnosis, and partial epilepsy, and the area under receiver operating characteristic curve was 0.7797. This model also fitted best in patients with idiopathic syndromes.ConclusionA predictive model of medically intractable epilepsy composed of only four characteristics is established. This model is comparatively accurate and simple to apply clinically.     

The clinical and neurobehavioral course of Down syndrome and dementia with or without new-onset epilepsy

BackgroundAdult patients with Down syndrome (DS) are at higher risk of developing Alzheimer-type dementia and epilepsy. The relationship between developing dementia and the risk of developing seizures in DS is poorly characterized to date. In addition, treatment response and medication tolerability have not been rigorously studied.MethodsWe identified 220 patients with a diagnosis of DS and dementia. Those without a history of developing seizures (DD) were compared to patients with new-onset seizures (DD + S) after the age of 35. Electronic records were reviewed for demographics, seizure characteristics, cognitive status, and psychiatric comorbidities.ResultsOf the patients included for analysis, twenty-six out of 60 patients had new-onset seizures or developed seizures during the follow-up period (the DD + S group) with a median onset of 2.0 years after the dementia diagnosis. Generalized tonic–clonic seizures were the most common seizure type (61.5% of DD + S). Sixteen (61.5%) patients were reported to have myoclonus. Levetiracetam was the most commonly used initial medication, with the majority (73%) of patients treated achieving partial or complete seizure control. The DD + S patients tended to have a similar burden of new-onset neuropsychiatric symptoms compared to the DD group.DiscussionNew-onset epilepsy seems to occur early in the course of dementia in DS patients. Patients generally respond to treatment. A great burden of neuropsychiatric symptoms is seen. Future studies need to explore the relationship between β-amyloid accumulation and epileptiform activity and attend to the care and needs of DS patients with dementia and seizures.