Following a very successful Lady Captain’s Charity Evening on 11th March, a cheque for £4,858 was presented to Dr. Graeme Sills, Chairman of Epilepsy Research UK last weekend at the West Lancashire Golf Club.
Last year’s Ladies’ Captain Jackie Bickerstaffe introduced Dr. Sills to some of the main helpers at the fundraising event and presented the cheque totalling £4,858. She thanked everyone who had given so generously and Dr. Sills for coming to accept the Club’s donation.
Dr. Sills was delighted to attend and accept the cheque on behalf of Epilepsy Research UK and explained the work he does at Liverpool University into the understanding and treatment of epilepsy, a condition which affects a huge number of the population.
We in the Fundraising Office add our thanks to that of Dr. Sills to all at West Lancashire Golf Club, especially Jackie Bickerstaffe for nominating us as a beneficiary for such fantastic support of Epilepsy Research UK.
Pictured L to R are:
Paula Morris, Kiran Sharma, Linda Foy, Dr. Sills, Immediate Past Ladies’ Captain Jackie Bickerstaffe, Wayne Arands Asst. Catering Manager, Margaret McDowell and Immediate Past Captain Dr. Anil Sharma.
Patients affected by protocadherin 19 (PCDH19)–female limited epilepsy (PCDH19-FE) present a remarkable reduction in allopregnanolone blood levels. However, no information is available on other neuroactive steroids and the steroidogenic response to hormonal stimulation. For this reason, we evaluated allopregnanolone, pregnanolone, and pregnenolone sulfate by liquid chromatographic procedures coupled with electrospray tandem mass spectrometry in 12 unrelated patients and 15 age-matched controls. We also tested cortisol, estradiol, progesterone, and 17OH-progesterone using standard immunoassays. Apart from estradiol and progesterone, all the considered hormones were evaluated in basal condition and after stimulation with adrenocorticotropic hormone (ACTH). A generalized decrease in blood levels of almost all measured neuroactive steroids was found. When considering sexual development, cortisol and pregnenolone sulfate basal levels were significantly reduced in postpubertal girls affected by PCDH19-FE. Of interest, ACTH administration did not recover pregnenolone sulfate serum levels but restored cortisol to control levels. In prepubertal girls with PCDH19-FE, by challenging adrenal function with ACTH we disclosed defects in the production of cortisol, pregnenolone sulfate, and 17OH-progesterone, which were not apparent in basal condition. These findings point to multiple defects in peripheral steroidogenesis associated with and potentially relevant to PCDH19-FE. Some of these defects could be addressed by stimulating adrenocortical activity.
Comorbid anxiety and depressive disorders in people with epilepsy (PWE) are highly prevalent and associated with various adverse outcomes. However, the prevalence of anxiety disorders in PWE across studies is highly variable. Our aim was to estimate the prevalence and moderating factors of anxiety and depressive disorders in PWE.Methods
Following prospective registration (PROSPERO; CRD42015027101), electronic databases were searched for studies that reported the prevalence of both anxiety and depressive disorders in samples of PWE up until July 2016. Data extracted included the prevalence of anxiety and depressive disorders, and moderators of interest (e.g., method of diagnosis, prevalence of drug-resistant epilepsy). Meta-analysis of the overall pooled prevalence of anxiety and depressive disorders was conducted.Results
The search yielded 8,636 unique articles, with 27 studies meeting final inclusion criteria (3,221 PWE). The pooled prevalence of anxiety and depressive disorders was 20.2% (95% confidence interval [CI] 15.3–26.0%) and 22.9% (95% CI 18.2–28.4%), respectively. Method of diagnosis significantly moderated anxiety disorder prevalence (Q statistic with one degree of freedom [Q1] = 36.29, p < 0.0001); the prevalence of anxiety disorders based on unstructured clinician assessment was 8.1% (95% CI 5.7–11.4%), compared to a prevalence of 27.3% (95% CI 22.1–33.3%) based on a structured clinical interview. There were no significant moderators of depressive disorder diagnosis.Significance
Findings suggest the prevalence of anxiety and depressive disorders in PWE are equivalent, and variability in prevalence of anxiety disorders across studies can be attributed partly to the method of diagnosis. These findings also challenge widely held assumptions that psychiatric comorbidity is more common in people with drug-resistant epilepsy. Future research should aim to improve the detection and management of these comorbidities in PWE, particularly anxiety disorders, which have remained relatively neglected.
Until now, it has been unclear if the three subsyndromes of adolescent-onset generalized genetic epilepsy (GGE) differ in long-term prognosis. Therefore, this study aimed to compare long-term seizure outcome in juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and epilepsy with generalized tonic–clonic seizures alone (EGTCS).Methods
This retrospective study is based on the archive of an institutional tertiary care outpatient clinic for adult patients with epilepsy. Charts of 870 epilepsy outpatients were reviewed among whom 176 had adolescent-onset GGE (53 JAE, 66 JME, 57 EGTCS). Median patient age at investigation was 60 years; median follow-up time was 42.5 years. If possible, GGE patients were additionally interviewed on psychosocial and clinical variables.Results
Age at first seizure was significantly higher in EGTCS patients (median 18 years) than in patients with JAE or JME (14 years each; p ≤ 0.001). Long-term seizure outcome hardly differed between the three subsyndromes. At the end of follow-up, 60% of all patients were in 5-year terminal seizure remission, and in 14%, epilepsy even had resolved (>10 years without seizures, >5 years without pharmacotherapy). Twenty percent of patients had persistent seizures during the last year of follow-up. Across all patients, 23% reported a psychiatric comorbidity, 87% had married, and 57% had achieved university entrance qualification.Significance
Long-term outcome was shown to be highly similar across all subsyndromes of adolescent-onset GGE. Even in a selection of difficult-to-treat epilepsy patients still attending an adult epilepsy clinic, most become seizure-free. To confirm these findings, prospective studies are needed.
It is well known that sleep-related motor seizures can originate from the temporal lobe. However, little is known about the clinical features of minor motor manifestations during sleep in patients with temporal lobe epilepsy. The main objective of our study was to verify the existence of minor motor events during sleep in patients with mesial temporal lobe epilepsy (MTLE) and to define their clinical features and electroencephalography (EEG) correlations.Methods
We enrolled in the study patients with diagnosis of symptomatic MTLE and a group of healthy controls. All patients and controls underwent long-term video -EEG monitoring, including at least one night of nocturnal sleep. We analyzed all the movements recorded during nocturnal sleep of patients and controls and their electroencephalographic correlations.Results
We analyzed the nocturnal sleep of 15 patients with symptomatic MTLE (8 males and 7 females; mean age ± standard deviation [SD]31.8 ± 14.9 years) and of 15 healthy controls (6 males and 9 females; mean age ± SD 32.8 ± 11.2 years). The analysis of movements during sleep revealed significant differences between groups, with the patients presenting significantly more movements in sleep than healthy controls (56.7 ± 39.2 vs. 15 ± 6.1; p < 0.001) with significant differences regarding oroalimentary automatisms, limb dystonia, straightening movements and gestural automatisms. EEG analysis showed that the proportion of movements preceded by EEG abnormalities was significantly higher in patients than in controls (57.8 ± 35.9 movements vs. 16.6 ± 13.4 movements; p < 0.001).Significance
The results of our study demonstrated the presence of minor motor events during sleep in patients with MTLE, suggesting an epileptic origin of these episodes. The study of nocturnal sleep in MTLE patients is useful in helping the clinicians in the diagnostic and therapeutic workup of these patients.
Psychiatric and behavioral disorders are important aspects of epilepsy and have received increasing attention in the last several years. The literature upon which most of the field relies contains some biases that must be carefully examined and resolved in future studies. First, in the pediatric epilepsy literature, many reports find that children with epilepsy have high levels of behavioral and psychiatric disorders when compared to appropriate controls. Most of these studies rely on parent-proxy completed instruments to assess these behavioral endpoints. Parents’ reports are not objective but reflect parents’ reactions and emotions. Increasing evidence suggests inherent biases in proxy reports and highlights the need to assess children directly. Second, periictal phenomena may be mischaracterized as underlying mood disorders. Third, many studies report elevated levels of psychiatric morbidity before and after the diagnosis of epilepsy, suggesting an inherent relation between the two types of disorders. Psychogenic nonepileptic seizures, while widely recognized as posing a diagnostic dilemma in the clinic, may account for some of these research findings. Diagnostic errors between epilepsy and psychogenic nonepileptic seizures need careful consideration when evaluating studies demonstrating associations between psychiatric disorders and epilepsy or poorer seizure control in association with psychiatric disorders in people who have epilepsy. Mental health concerns are important for everyone. An accurate, undistorted understanding of the relation between mental health disorders and epilepsy is essential to ensure appropriate therapy and to avoid unnecessary and potentially harmful treatments and common misconceptions.
Obstructive apnea due to laryngospasm links ictal to postictal events in SUDEP cases and offers practical biomarkers for review of past cases and prevention of new ones
Seizure spread into autonomic and respiratory brainstem regions is thought to play an important role in sudden unexpected death in epilepsy (SUDEP). As the clinical dataset of cases of definite SUDEP available for study grows, evidence points to a sequence of events that includes postictal apnea, bradycardia, and asystole as critical events that can lead to death. One possible link between the precipitating seizure and the critical postictal sequence is seizure-driven laryngospasm sufficient to completely obstruct the airway for an extended period, but ictal laryngospasm is difficult to fully assess. Herein, we demonstrate in a rat model how the electrical artifacts of attempts to inspire during airway obstruction and features of the cardiac rhythm establish this link between ictal and postictal activity and can be used as practical biomarkers of obstructive apnea due to laryngospasm or other causes of airway obstruction.