A comprehensive assessment of cognitive function in the common genetic generalized epilepsy syndromes.

BACKGROUND AND PURPOSE: Considered to be benign conditions, the common genetic generalized epilepsy (GGE) syndromes are now known to be frequently accompanied by cognitive dysfunction. However, unresolved issues impede clinical management of this common comorbidity, including which cognitive abilities are most affected, whether there are differences between syndromes and how seizure type and mood symptoms affect cognitive dysfunction. We provide a detailed description of cognitive ability and evaluate factors contributing to cognitive dysfunction. METHODS: A total of 76 adults with GGE were assessed with the Woodcock Johnson III Tests of Cognitive Abilities. RESULTS: Scores on tests of overall cognitive ability, acquired knowledge, long-term retrieval and speed of information processing were significantly below the normative mean. Long-term retrieval was a pronounced weakness with a large reduction in scores (d = 0.84). GGE syndrome, seizure type and the presence of recent psychopathology symptoms were not significantly associated with cognitive function. CONCLUSIONS: This study confirms previous meta-analytic findings with a prospective study, offers new insights into the cognitive comorbidity of these common epilepsy syndromes and reinforces the need for cognitive interventions in people with GGE.

A Systematic Review of Psychiatric and Psychosocial Comorbidities of Genetic Generalised Epilepsies (GGE).

Psychiatric disorders and associated poor psychosocial outcomes are recognised to be a common sequelae of epilepsy. The extent to which this is true of genetic generalised epilepsies (GGE), particularly syndromes other than juvenile myoclonic epilepsy (JME) is unclear. This systematic review synthesises findings regarding psychiatric and associated comorbidities in adults and children with GGE. Systematic review yielded 34 peer-reviewed studies of psychiatric and psychosocial outcomes in adults and children with GGE. Clinically significant psychiatric comorbidity was reported in over half of all children and up to a third of all adults with GGE. There was no evidence to support the presence of personality traits specific to JME or other syndromes; rather rates mirrored community samples. A small number of studies report poor psychosocial outcomes in GGE, however the interpretation of these findings is limited by paucity of healthy comparison groups. Some evidence suggests that anti-epileptic drug polytherapy in children and seizure burden at all ages may constitute risk factors for psychopathology. Findings highlight the importance of early screening so as not to overlook early or developing symptoms of psychopathology.

Clinical experience with using lacosamide for the treatment of epilepsy in a tertiary centre.

OBJECTIVE: Lacosamide is approved for the adjunctive treatment of partial-onset seizures in adults. Phase II/III clinical trials suggest that it is a safe, effective and well-tolerated medication. However, there is little post-marketing information available about this medication. METHODS: We report our clinical experience from a tertiary referral epilepsy centre, which has been using lacosamide for the past 18 months, with 128 patients treated during this time. RESULTS: Fifty-three patients (41%) achieved at least a 50% reduction in seizure frequency, with 14 patients (11%) achieving seizure freedom for a mean time of 35 weeks. This 50% responder rate matches, and the seizure free rate outperforms that seen in previous pooled trials. The efficacy of lacosamide did not vary with concurrent sodium channel blocking agent (SCB) use, and a statistically significant dose-dependent response was not shown, which is in contrast to previous trials. Treatment emergent adverse effects (TEAEs) were noted in 52 patients (41%), with 24 patients (19%) discontinuing the medication. TEAEs were more frequent in patients on concurrent SCBs, affecting 51% vs. 28% of patients not on other SCBs. This increased risk of TEAEs from concurrent SCB use was of statistical significance (P = 0.01). The most frequently noted TEAEs from lacosamide were dizziness, sedation and diplopia, which all appeared to be dose-related. CONCLUSION: This post-marketing analysis suggests that lacosamide in clinical practice at least mirrors, and possibly outperforms the results seen in previous phase II/III trials.

The prevalence and demographic distribution of treated epilepsy: a community-based study in Tasmania, Australia.

OBJECTIVES: To estimate the prevalence and demographic distribution of treated epilepsy in a community-based population. MATERIALS & METHODS: We surveyed all residents in Tasmania, Australia, who were supplied at least one antiepileptic drug prescription between July 1, 2001 and June 30, 2002, recorded on the national prescription database. We adjusted for the effect of disease-related non-response bias by imputation methods. RESULTS: After three mail contacts, 54.0% (4072/7541) responded, with 1774 (43.6%) indicating treatment for epilepsy, representing 86.0% of the estimated total possible cases in Tasmania. The adjusted treated epilepsy prevalence was 4.36 per 1000 (95% CI 4.34, 4.39); lower in women (prevalence ratio 0.92 (95% CI 0.84, 1.00)); greater with increasing age (P < 0.001); similar in the three main geographic regions; and similar with socioeconomic status of postcode of residence. CONCLUSIONS: Although our estimates are likely to be affected by access to health services, overall treated epilepsy prevalence of 4.4 per 1000 is similar to previous studies. Our finding of high elderly prevalence has been reported in a few recent studies in developed countries and has important clinical and public health implications in populations with similar aging demographics.

Ring and peg electrodes for minimally-Invasive and long-term sub-scalp EEG recordings.

OBJECTIVE: Minimally-invasive approaches are needed for long-term reliable Electroencephalography (EEG) recordings to assist with epilepsy diagnosis, investigation and more naturalistic monitoring. This study compared three methods for long-term implantation of sub-scalp EEG electrodes. METHODS: Three types of electrodes (disk, ring, and peg) were fabricated from biocompatible materials and implanted under the scalp in five ambulatory ewes for 3months. Disk electrodes were inserted into sub-pericranial pockets. Ring electrodes were tunneled under the scalp. Peg electrodes were inserted into the skull, close to the dura. EEG was continuously monitored wirelessly. High resolution CT imaging, histopathology, and impedance measurements were used to assess the status of the electrodes at the end of the study. RESULTS: EEG amplitude was larger in the peg compared with the disk and ring electrodes (p<0.05). Similarly, chewing artifacts were lower in the peg electrodes (p<0.05). Electrode impedance increased after long-term implantation particularly for those within the bone (p<0.01). Micro-CT scans indicated that all electrodes stayed within the sub-scalp layers. All pegs remained within the burr holes as implanted with no evidence of extrusion. Eight of 10 disks partially eroded into the bone by 1.0mm from the surface of the skull. The ring arrays remained within the sub-scalp layers close to implantation site. Histology revealed that the electrodes were encapsulated in a thin fibrous tissue adjacent to the pericranium. Overlying this was a loose connective layer and scalp. Erosion into the bone occurred under the rim of the sub-pericranial disk electrodes. CONCLUSIONS: The results indicate that the peg electrodes provided high quality EEG, mechanical stability, and lower chewing artifact. Whereas, ring electrode arrays tunneled under the scalp enable minimal surgical techniques to be used for implantation and removal.

Long-term benefits for Mäori of an asthma self-management program in a Mäori community which takes a partnership approach.

BACKGROUND: In 1991, an intervention trial of the efficacy of an asthma self-management plan was carried out in partnership with a rural Mäori community. The program relied on Mäori community health workers and other health professionals working in partnership, was delivered through clinics in traditional Mäori community centres and Mäori processes were followed throughout. The plan was shown to be effective in reducing asthma morbidity. OBJECTIVE: To assess whether the long-term benefits of the program extent beyond reduced asthma morbidity and the extent to which any additional benefits may be related to the partnership approach employed by the program. METHOD: Forty-seven (68%) of the original program participants were surveyed in August 1997. Participants were questioned on the program's impact in areas such as cultural development, health service access and lifestyle. RESULTS: In addition to the improvements in asthma morbidity, the program was found to have four key benefits: cultural affirmation; improved access to other health services; a greater sense of control for participants; and positive impacts on the extended family. CONCLUSIONS: The program's benefits extended beyond reduced asthma morbidity and were not due simply to the introduction of the asthma self-management plan but also to the partnership approach employed by the program. IMPLICATIONS: The study provides support for providing public health services for indigenous communities that take a partnership approach, utilise community expertise and are delivered in a way that is consistent with each community's cultural processes.

Toothbrushing-induced epilepsy with structural lesions in the primary somatosensory area.

We report three patients with reflex toothbrushing-induced epilepsy associated with small circumscribed structural lesions in the primary somatosensory cortex in close proximity to the hand and speech motor areas. Sensory symptoms were observed at clinical onset with localizing focal ictal and interictal epileptiform discharges on EEG. These cases refine the localization, possible mechanisms of epileptogenesis, and classification of this reflex epilepsy.

Visual disturbances representing occipital lobe epilepsy in patients with cerebral calcifications and coeliac disease: a case series.

Paroxysmal visual manifestations may represent epileptic seizures arising from the occipital lobe. In coeliac disease (CD) bilateral occipital calcifications and seizure semiology consistent with an occipital origin have been described, primarily in Mediterranean countries. By reporting three adult patients from an Australian outpatient clinic with visual disturbances, occipital cerebral calcifications, and CD, this study seeks to emphasise that CD should be considered even when patients of non-Mediterranean origin present with these symptoms. Seizure types included simple partial, complex-partial, and secondarily generalised seizures. The seizure semiology consisted of visual disturbances such as: blurred vision, loss of focus, seeing coloured dots, and brief stereotyped complex visual hallucinations like seeing unfamiliar faces or scenes. Symptoms of malabsorption were not always present. Neurological examination was unremarkable in two patients, impaired dexterity and mild hemiatrophy on the left was noted in one. Routine electroencephalography was unremarkable. In all cases, computed tomography demonstrated bilateral cortical calcification of the occipital-parietal regions. Magnetic resonance imaging showed no additional lesion. All patients had biopsy confirmed CD. Seizure control improved after treatment with gluten free diet and anticonvulsants. This report illustrates the association between seizures of occipital origin, cerebral calcifications, and CD even in patients not of Mediterranean origin.

Asthma morbidity 6 yrs after an effective asthma self-management programme in a Maori community.

A 6-month Maori community-based asthma self-management programme, involving a "credit card" asthma self-management plan, has previously been shown to be an effective and acceptable system for reducing asthma morbidity. The effectiveness of the asthma self-management programme and participants' self-management behaviour was assessed 6 yrs after the formal end of the programme. Participants were surveyed at the time of enrollment, and 1, 2, and 6 yrs after completing the programme. In each survey, participants were questioned on markers of asthma morbidity and use of medical services during the previous 12 months. Self-management behaviour was assessed using a questionnaire at 2 years and 6 yrs. Of the 69 original participants, 47 (68%) were surveyed after 6 yrs. They generally had reduced severe asthma morbidity and emergency use of health services from baseline. In particular, the proportion who had an emergency visit to a general practitioner had decreased from 41% to 18% (p=0.02). However, the percentage of nights woken due to asthma had returned to preintervention levels, and the proportion of participants taking prescribed regular inhaled steroid had decreased from 91% to 53% (p<0.001). Compared with 2 yrs after completion of the asthma programme, self-management behaviour had also deteriorated, with 29% versus 73% (p<0.001) using their peak flow meter daily when their asthma was "getting bad" and 41% versus 86% (p<0.001) using the "credit card" plan to increase the amount of inhaled steroids in the last year. Although the programme participants were still experiencing reduced morbidity from their asthma 6 yrs after the end of the self-management programme, the benefits were less than those observed at 2 yrs. These findings suggest that under-recognition and under-treatment of asthma with appropriate amounts of inhaled steroids is a major factor contributing to asthma morbidity in this indigenous rural community. To obtain enduring benefits from a self-management system of care continued reinforcement of self-management skills seems to be an essential component of any follow-up.

Long-term reduction in asthma morbidity following an asthma self-management programme.

The adult "credit card" asthma self-management plan has been shown to be an effective and acceptable system for reducing asthma morbidity when introduced as part of a 6 month community-based asthma programme. The aim of the present study was to assess the effectiveness of the credit card plan 2 yrs after the end of the programme. Markers of asthma morbidity and use of medical services were compared during the 12 months before enrolment, and 2 yrs after completing the 6 month asthma programme. Of the 69 participants who originally enroled in the 6 month asthma programme, 58 were surveyed 2 yrs after completion of the programme. These participants showed a significant improvement in all but one of the asthma morbidity measures. The proportion waking most nights with asthma in the previous 12 months decreased from 29 to 9% (p=0.02), emergency visits to a general practitioner decreased from 43 to 16% (p=0.001), hospital emergency department visits with asthma decreased from 19 to 5% (p=0.02) and hospital admissions decreased from 17 to 5% (p=0.04). Only 24% of patients reported that they usually monitored their peak flow rate daily, but this increased to 73% during a "bad" attack of asthma. A long-term reduction in asthma morbidity and requirement for acute medical services can result following the introduction of the adult credit card asthma self-management plan. Adult patients with asthma are most likely to undertake peak flow monitoring preferentially during periods of unstable asthma, rather than routinely during periods of good control.

Family size, infections, and asthma prevalence in New Zealand children.

We conducted a prevalence case-control study to investigate the relation between family composition, infection, and development of asthma at age 7-9 years. Potential cases (399) and controls (398) were selected from the Wellington, NZ, arm of the International Study of Asthma and Allergies in Childhood, a population-based prevalence study. Further screening questions restricted cases to children with a diagnosis of asthma and current medication use (N = 233) and restricted controls to children without a history of wheezing and no diagnosis of asthma (N = 241). After controlling for confounders (including infections, atopy, and socioeconomic status), family size was strongly related to asthma. Having no siblings [prevalence odds ratio (POR) = 2.51; 95% confidence interval (CI) = 1.05-6.01] or one sibling (POR = 1.86; 95% CI = 1.14-3.03) was associated with an increased risk of asthma compared with having more than one sibling. Parent-reported rubeola infection (and possibly other similar viral exanthems) was independently associated with a decreased risk of asthma (POR = 0.48; 95% CI = 0.27-0.83), but reported pertussis infection (POR = 1.57; 95% CI = 0.58-4.24) and day care attendance in the first year of life (POR = 1.81; 95% CI = 0.93-3.51) were not strongly associated with increased risks of asthma.