Psychogenic nonepileptic seizures in patients with learning disability: comparison with patients with no learning disability

Among a consecutive series of patients with psychogenic nonepileptic seizures (PNES), we compared patients with learning disability (LD) (n=25) with patients with no LD (n=263), with respect to demographic and clinical variables. A higher proportion of the LD group had epilepsy as well as PNES (P<0.001) (uncorrected P values are quoted), and a higher proportion were taking antiepileptic drugs at the time of diagnosis of PNES (P=0.007). Fewer patients with LD had a history of antecedent sexual abuse (P=0.036). A higher proportion of the LD group had previous pseudostatus (P<0.001), and a higher proportion had immediate situational or emotional triggers for their attacks (P<0.001). There were trends toward a higher proportion of men in the LD group (P=0.056) and a longer delay between onset of PNES and diagnosis (P=0.072). Our data suggest potentially important clinical differences between PNES populations with and without LD, as well as possible differences in mechanism.     

Levetiracetam in adult patients with and without learning disability: focus on behavioral adverse effects

Optimal antiepileptic drug treatment in patients with learning disability (LD) represents a particular challenge. These patients are often unable to report toxicity, and side effects may manifest as behavioral problems. The aim of this open study was to compare efficacy and tolerability of levetiracetam (LEV) in patients with LD and those without LD. One hundred eighty-four consecutive adult patients who received LEV were followed for an average of 8.1 months. Fifty-six patients (30%) had LD. Thirty-nine percent of patients with refractory epilepsy (37% with and 40% without LD) had > 50% seizure reduction. Significantly more behavioral side effects (23% vs 10%) and a tendency toward less reported somatic central nervous side effects were found in the LD group. We conclude that LEV is equally effective and well tolerated in both patients with LD and patients without LD. However, behavioral problems are more frequent in patients with LD, whereas the tendency toward seizure increase is not enhanced.     

Autism spectrum disorders in children with active epilepsy and learning disability: comorbidity, pre- and perinatal background, and seizure characteristics

The aim of this study was to examine the comorbidity pattern, seizure characteristics, and aetiology in a representative group of children with a combination of autism spectrum disorder (ASD), active epilepsy, and learning disability. Ninety children (47 males, 43 females; mean age 11 years 2 months, range 8 to 16 years at the time of psychiatric examination) with active epilepsy and learning disability, identified in a population‐based study in Göteborg, Sweden, were subdivided into those with and those without ASD and compared with respect to aetiology, additional neuroimpairments, and seizure characteristics. In addition, the cohorts were examined for trends of prevalence over a period of time. Results indicated that established aetiology was much more often present in the prenatal period than in the peri‐ or postnatal periods in the ASD group. Cerebral palsy and visual impairment were under‐represented in the ASD group. Partial seizures tended to be more common and generalized seizures less common in the ASD group compared with the non‐ASD group. Seizure onset was later in the ASD group. Many of the significant differences were accounted for by a large group of psychiatrically unclassifiable participants in the non‐ASD group. There was no trend towards an increase of affected children over the 12‐year period. There was no increase in the prevalence of active epilepsy and learning disability nor in the rate of autism with active epilepsy and learning disability in children born between 1981 and 1986 compared with those born from 1976 to 1980, indicating no statistical association with the general measles‐mumps‐rubella vaccination introduced in the early 1980s.     

Antiepileptic efficacy of vigabatrin in people with severe epilepsy and intellectual disability

The short- and long-term clinical efficacy of add-on vigabatrin treatment was evaluated in a group of 36 patients with intellectual disability and drug-refractory epilepsy. The results were compared to the efficacy of vigabatrin in 75 non-retarded patients with drug-resistant complex partial and secondarily generalized seizures. After 3 months, 42% of the patients with intellectual disability had experienced a reduction in seizure frequency of more than 50% (responders). The percentage of responders was still 22% after 6 years. No impairment in psychological function was observed during vigabatrin treatment compared with baseline values. However, one patient was excluded from long-term treatment because of psychotic depression and two patients because of psychomotor slowing after 1-2 years of treatment The need for extra supervision appeared to diminish and three patients were able to be discharged from institutional care during the follow-up. In the group of non-retarded patients, the percentages of the responders were 55% and 27% after 3 months and 6 years of treatment, respectively. The results from these studies suggest that vigabatrin is effective and relatively well tolerated, and that the successful treatment of epilepsy also has socio-economic consequences in patients with intellectual disability and severe epilepsy.     

Examining a community model of epilepsy care for people with learning disabilities.

PURPOSE: To assess the use of specialised medical epilepsy services by people with learning disabilities (LD) and epilepsy in a community healthcare setting, to compare medical epilepsy care in this group to current management guidelines, and to contrast important outcomes with those achieved in different healthcare settings. METHODS: Postal survey with a carer completed questionnaire addressed to all adults with epilepsy registered on an LD register in Sheffield, UK (n=442). RESULTS: An analysis based on 225 returned questionnaires revealed that 22.7% of individuals with LD and epilepsy had been free of seizures for over 1 year. 95.1% were taking antiepileptic drugs (AEDs), 46.2% had had an EEG, and 41.3% a brain scan. 53.3% of diagnoses had been made by epilepsy experts, 38.7% of individuals with LD and epilepsy were under specialist review. Although patients with more severe epilepsy were more likely to be under specialist care, 60.6% of patients with ongoing seizures, 57.9% with major seizures and 68.7% of individuals taken to hospital with prolonged had no access to specialist advice. CONCLUSION: The proportion of people with LD who achieved seizure-control in the described population was lower than in all previously reported studies of LD patient groups. The poor outcome in terms of seizure-control, the lack of access to the epilepsy specialist service, and the apparent under-utilisation of investigations indicate that there are grounds for serious concern about this community model of medical epilepsy care for people with LD.     

Long-term retention rates of new antiepileptic drugs in adults with chronic epilepsy and learning disability

We compared the long-term retention rates of several newly licensed antiepileptic drugs (AEDs) in a residential community of adults with chronic epilepsy and learning disability. Data relating to duration of therapy, maximum dose, and tolerability of six new AEDs-gabapentin (GBP), lamotrigine (LTG), levetiracetam (LEV), oxcarbazepine (OXC), tiagabine (TIA), and topiramate (TPM)-were collected. Drug retention at 2 years was 85% (OXC), 57% (LTG), 56% (LEV), 45% (TPM), 24% (TIA) and 15% (GBP). OXC was used mainly as a substitute for carbamazepine. LTG, LEV, and TPM were all associated with retention rates higher than those of GBP or TIA. TPM had the highest rate of adverse event development at the maximum tried dose (60%), whereas LEV had the lowest (16%). Experience from this single epilepsy community study indicated limited impact for GBP or TIA but higher retention of OXC, LEV, LTG, and TPM in patients with chronic epilepsy and learning disability.     

Efficacy and tolerability of perampanel in ten patients with Lafora disease

Lafora disease (LD) is a fatal intractable adolescence-onset progressive myoclonus epilepsy. Recently, two single-case studies reported drastic reductions in seizures and myoclonus with the AMPA antagonist perampanel and improved activities of daily living. We proceeded to study the effect of perampanel on 10 patients with genetically confirmed LD with disease duration ranging from 2 to 27 years. Open-label perampanel was added to ongoing medications to a mean dose of 6.7 mg/day. Seizures, myoclonus, functional disability, and cognition scores were measured for the third and ninth months following initiation and compared with those of the month prior to the start of therapy. Three patients withdrew because of inefficacy or side effects. Four had significant reduction in seizures of greater than 74% from baseline. Seven had major improvement in myoclonus with group-adjusted sum score of myoclonus intensity reduced from 7.01 at baseline to 5.67 and 5.18 at 3 and 9 months, respectively. There was no significant improvement in disability and cognition. While not universal, perampanel adjunctive therapy appears to confer particular benefit not commonly seen with other antiepileptic drugs on seizures and myoclonus in LD. Improvement in the extremely disabling myoclonus of LD is a major benefit in this devastating disease.     

Seizure control and mortality in epilepsy.

Mortality rates are increased among people with epilepsy, and may be highest in those with uncontrolled seizures. Because epilepsy surgery eliminates seizures in some people, we used an epilepsy surgery population to examine how seizure control influences mortality. We tested the hypothesis that patients with complete seizure relief after surgery would have a lower mortality rate than those who had persistent seizures. Three hundred ninety-three patients who had epilepsy surgery between January 1986 and January 1996 were followed after surgery to assess long-term survival; 347 had focal resection or transection, and 46 had anterior or complete corpus callosotomy. A multivariate survival analysis was performed, contrasting survival in those who had seizure recurrence with survival of those who remained seizure free. Standardized mortality ratios and 95% confidence intervals were calculated. Overall, seizure-free patients had a lower mortality rate than those with persistent seizures. This was true for the subset of patients with localized resection or multiple subpial transection. No patients died among 199 with no seizure recurrence, whereas of 194 patients with seizure recurrence, 11 died. Six of the deaths were sudden and unexplained. Most patients who died had a substantial reduction in postoperative seizure frequency. The standardized mortality ratio for patients with recurrent seizures was 4.69, and the risk of death in these patients was 1.37 in 100 person-years, whereas among patients who became seizure free, there was no difference in mortality rate compared with the age- and sex-matched population of the United States. Elimination of seizures after surgery reduces mortality rates in people with epilepsy to a level indistinguishable from that of the general population, whereas patients with recurrent seizures continue to suffer from high mortality rates. This suggests that uncontrolled seizures are a major risk factor for excess mortality in epilepsy. Achieving complete seizure control with epilepsy surgery in refractory patients reduces the risk of death, so the long-term risk of continuing medical treatment appears to be higher than the risk of epilepsy surgery in suitable candidates.     

A general practice-based prevalence study of epilepsy among adults with intellectual disabilities and of its association with psychiatric disorder, behaviour disturbance and carer stress

Background Although the elevated occurrence of epilepsy in people with intellectual disabilities (ID) is well recognized, the nature of seizures and their association with psychopathology and carer strain are less clearly understood. The aims were to determine the prevalence and features of epilepsy in a community‐based population of adults with ID, and to explore whether the presence of epilepsy was associated with greater psychopathology or carer strain. Methods Data were collected on the age, gender, place of residence, adaptive and challenging behaviour, social abilities and psychiatric status of 318 adults from 40 general practices, together with the degree of malaise and strain of family carers. For participants with epilepsy, a nurse collected information on seizures, investigations, treatment and carer concerns by interview. Association between epilepsy and psychiatric morbidity, challenging behaviour and caregiver malaise or strain, was explored by comparing those with epilepsy with a comparison group matched on adaptive behaviour. Results Fifty‐eight participants (18%) had epilepsy: 26% were seizure free, but 34% had extremely poorly controlled seizures. Earlier onset and seizure frequency were associated with adaptive behaviour. Carer concerns were related to seizure frequency and a history of injury. There were no significant differences in psychopathology, carer malaise or caregiver strain between the matched epilepsy and non‐epilepsy groups. Conclusions This study supports the high occurrence and chronicity of epilepsy among people with ID. While psychopathology and carer strain is common within this population, underlying disability‐related factors appear to be more important than the presence of epilepsy per se.     

Depression and altered quality of life in women with epilepsy of childbearing age

PURPOSE: To calculate the prevalence of depression in a referral population of women of childbearing age, to define the factors associated with depression, and to assess health-related quality of life (HRQOL) in the same population. METHODS: The 642 consecutive women with epilepsy aged 18-55 years were enrolled by 40 neurologists over an 8-month period and asked to give details on selected demographic and clinical features regarding the disease, any associated clinical condition, and any drug treatment. Depression was diagnosed by using the Hamilton depression scale and HRQOL was measured through the SF-36 form. Demographic, clinical, and therapeutic risk factors for depression were searched for within the study population. RESULTS: Depression (any severity) was present at interview in 242 women, giving a prevalence rate of 37.7%[95% confidence interval (CI), 33.9-41.6]. Mild depression was reported by 18.5% of women, moderate depression by 8.6%, major depression by 10.3%, and severe depression by 0.3%. Factors found to be independently associated with depression (any severity) included treatment of associated conditions [relative risk (RR), 1.5; 95% CI, 1.2-1.8), concurrent disability (RR, 1.3; 95% CI, 1.0-1.6), seizures in the preceding 6 months (RR, 1.4; 95% CI, 1.1-1.7), and being unemployed or a housewife (RR, 1.3; 95% CI, 1.0-1.5). Factors associated with moderate to severe depression included treatment for associated conditions (RR, 2.0; 95% CI, 1.4-2.7), seizures in the preceding 6 months (RR, 1.7; 95% CI, 1.2-2.5), and being unemployed or a housewife (RR, 1.6; 95% CI, 1.1-2.2). Compared with normal women of similar age, patients with epilepsy tended to present lower scores for each HRQOL domain (mostly Role Physical, General Health, Social Functioning, and Role Emotional). However, when the analysis was limited to nondepressed women with epilepsy, any difference disappeared. CONCLUSIONS: Women with epilepsy of childbearing age are at high risk of depression. Factors associated with depression include lack of occupation, the presence of an underlying disabling condition (with treatment), and the severity of epilepsy. Compared with the general population, depressed women have greater impairment of HRQOL with epilepsy, which reflects the physical, social, and emotional implications of the disease.     

Sleep and Behavioral Problems in Rolandic Epilepsy

Although patients with benign childhood epilepsy with centrotemporal spikes exhibit a benign course of the disease, some of them display sleep and behavioral problems. Sixty-one patients with rolandic epilepsy, aged 6-11 years, were included in this study. The patients were divided into two subgroups according to the presence of seizures over the preceding 6 months. The control group comprised 25 patients without epilepsy and with similar characteristics in terms of age and sex. All patients underwent evaluation of sleep (Sleep Disturbance Scale for Children) and behavior (Lithuanian version of the Child Behaviour Checklist). Only patients who had had seizures over the preceding 6 months displayed significantly higher scores for sleep problems (disorders of excessive daytime sleepiness, disorders of sleep-wake transition, and scores for total sleep problems), worse sleep quality (longer sleep-onset latency), and behavioral problems (anxiety/depression, social problems, thought problems, attention problems, and aggressive behavior) than the patients of the control group. Our data add to evidence that active epilepsy has an impact on sleep and behavior. Clinically significant sleep problems were related to the higher risk of behavioral problems. Parents' ratings for existing sleep problems were sensitive to Sleep Disturbance Scale for Children scores above normal values.     

Epilepsy in patients with severe motor and intellectual disabilities: a long-term follow-up

Long-term prognosis of epilepsy was investigated on 117 institutionalized patients with severe motor and intellectual disabilities, who were above 15 years of age in 1977, for a 20-year-period from 1977 to 1997. The incidence of epilepsy was 64.1% (75 patients), which was active in 28 patients (37.3%). The patients with the most severe psychomotor disabilities (bedridden and DQ < 20) showed the highest incidence of epilepsy (85.0%). Patients who died during the follow-up period showed higher incidence of active epilepsy (p < 0.01). During the follow-up of 94 surviving patients, persistence, relapse, and onset of seizures were frequent in patients with most severe intellectual disability, whereas those with less severe intellectual disability (20 < DQ < 35) were all seizure-free. Twenty-one patients had active epilepsy; symptomatic partial epilepsy in 17 (81.0%) and generalized epilepsy in 4 (19.0%). Notably, 5 of the 6 patients with persistent frequent seizures had age-dependent epileptic encephalopathy; persistent Lennox-Gastaut syndrome (LGS) (2 patients), severe epilepsy with multiple independent spike foci evolved from West syndrome (WS) and LGS (2 patients), and partial epilepsy with the history of LGS (1 patient).     

Physically traumatic events in patients with epilepsy compared with non-epileptic subjects

To determine the incidence of traumatic events among epileptic patients compared with non-epileptic individuals, we distributed a questionnaire on physically traumatic events occurring during the preceding three months to consecutive epileptic patients and to age- and sex-matched controls. There were 145 epileptic patients, 121 with seizures (age 36 +/- 15 y, 60 males) and 24 who were seizure free during this period (age 39 +/- 17 y, 13 males), and 145 controls (age 36 +/- 15 y, 73 males). There was no significant difference in the duration of epilepsy between the two groups of patients with epilepsy. Traumatic events (n= 27, three of them unrelated to seizure) were most common in patients with seizures, followed by controls (n= 20), and absent in seizure-free patients (P< 0.001). Patients with epilepsy most commonly injured the head while the extremities were more usually involved in controls. Patients with epilepsy had significantly more traumatic events at home, whereas controls underwent most traumatic events at work and in public areas. There was no significant difference in the type and severity of trauma between the two groups. We conclude that physically traumatic events not related to seizures are fewer among patients with epilepsy with and without seizures compared with controls, probably because of increased cautiousness.     

A randomized open-label study of gabapentin and lamotrigine in adults with learning disability and resistant epilepsy.

The aim of this study was to evaluate the efficacy and safety of gabapentin in patients with learning disabilities and resistant epilepsy, comparing the effects of gabapentin with lamotrigine on efficacy, behaviour and mood. An open-label, randomized, parallel group, multicentre add-on study comparing gabapentin with lamotrigine in 109 patients with drug-resistant localization-related epilepsy and learning disabilities was conducted: 39 patients were randomized to gabapentin and 44 to lamotrigine. The study population had a range of learning disabilities and severe partial epilepsy. The percentage of patients achieving a greater than or equal to 50% reduction in seizure frequency on gabapentin was 50%, (mean reduction in seizures was 51%). Compared to 48.6% of lamotrigine patients, no statistically significant treatment differences could be identified. The safety profile of both drugs was consistent with that seen in previous clinical trials. Carer-rated visual analogue scales detected significant improvements (P< 0.05) for the gabapentin-treated patients in seizure severity, attention, general health and sleeping pattern, while for lamotrigine seizure severity improved significantly. For learning disabled patients with resistant epilepsy, gabapentin and lamotrigine provide safe and effective treatment, with positive benefits on behaviour.     

Epilepsy in a sample of children with intellectual disability in Jordan

This study assessed the prevalence of epileptic seizures in 378 children attending special education centres for students with intellectual disability in the city of Amman in Jordan. The findings indicated that 20% of the sample had a history of epilepsy. Significant association was found between epilepsy and severity of intellectual disability. Epilepsy was more prevalent among males than females and among younger than older children, but differences lacked statistical significance.     

Psychiatric morbidity, quality of life, and disability in mesial temporal lobe epilepsy patients before and after anterior temporal lobectomy

Considerable interest has been focused on the psychiatric complications of medically refractory temporal lobe epilepsy (TLE) before and after epilepsy surgery. The aim of the present study was to evaluate the psychiatric status, quality of life, and level of disability in medically refractory mesial temporal lobe epilepsy (MTLE) patients, a homogenous subgroup of patients with TLE, before and after anterior temporal lobectomy (ATL). The study population consisted of 22 patients with medically refractory MTLE who were candidates for ATL. Patients were examined before surgery as well as in the third and sixth months of the postoperative period. Psychiatric diagnosis was determined by using SCID-I. To rate the severity of psychiatric disorders, BPRS, HDRS, and HARS were employed on each visit. WHO-DAS-II and WHOQOL-BREF were used to determine the level of disability and quality of life. Preoperatively, six patients had a psychiatric diagnosis. Three months after surgery, six of the patients had psychiatric diagnoses. Five of these six patients had not been previously diagnosed. There was no significant difference between preoperative and postoperative follow-up evaluations in terms of HDRS, HARS, and BPRS ratings. With respect to the total scores and domains of WHO-DAS-II, the change in pre- and postoperative evaluations was statistically significant only for the social life attendance domain. There was no significant difference in the mean scores on the WHOQOL-BREF domains or on the first question about general evaluation of quality of life. For the second question on the level of satisfaction with health, the difference between the three ratings was statistically significant. Preoperative and postoperative rates of psychiatric disorders in our sample were low. While social phobia was frequently seen preoperatively, the postoperative period was spearheaded by major depressive disorder. The decrease in disability in attendance to social life and improvement in the quality of health were in concordance with the literature, indicating the positive results of surgical treatment of epilepsy on quality of life. This study suggests that surgical intervention might be one of the causes of postoperative psychiatric disorders in patients with MTLE.     

Relationship between depression and intractability of seizures

Patients with epilepsy have a higher prevalence of depressive disorders than the general population, but the relationship between seizure rates and depression has not been adequately studied. We used the Beck Depression Inventory to evaluate depressive symptoms in 143 consecutive epilepsy patients from outpatient clinics. Patients who were seizure free more than 6 months were considered not intractable. Thirty-six percent were neither intractable nor depressed, 43% had intractable epilepsy and were not depressed, 10% had intractable epilepsy and were depressed, and 11% did not have intractable epilepsy and were depressed. Patients with epilepsy have a higher prevalence of depression than the general population, but the intractability of the seizure disorder does not seem to be an independent risk factor for the occurrence of depression. There is no relationship between the severity of depression and monthly seizure rate.     

Clinical predictors of late-onset seizures and epilepsy in patients with cerebrovascular disease

BACKGROUND: Seizures and epilepsy are harmful and worsen the disability of stroke patients. There are currently no good clinical predictors of late-onset seizures and epilepsy in patients with cerebrovascular disease (CVD). PATIENTS AND METHODS: 110 patients with delayed seizures after an ischaemic or a haemorrhagic stroke, a transient ischaemic attack or a subarachnoid haemorrhage (60 with a single seizure and 50 with epilepsy) and 366 without seizures were included in this retrospective study. The clinical syndrome, the stroke aetiology and the vascular risk factors were compared. The groups with a single seizure and with epilepsy were also analysed separately. RESULTS: There were no differences in age, gender, aetiology and vascular risk factors between the groups with and without seizures. When comparing the incidence of the clinical syndromes, ischaemic partial anterior circulation syndrome (PACS) was significantly more and transient ischaemic attack less frequent in the group with seizures compared to the control group. The severity of the neurological impairment on admission and the degree of disability on discharge after a PACS was similar in those who developed late-onset seizures compared with those who did not. Also on the Cox proportional hazards analysis, PACS appeared to be the only clinical risk factor for development of seizures and epilepsy in patients with CVD. No differences were observed in clinical predictors between patients with a single seizure and those with epilepsy. CONCLUSION: PACS is the only independent predictor for the occurrence of late-onset seizures in patients with CVD.