The burden of normality: A framework for rehabilitation after epilepsy surgery

Summary   This paper describes a conceptual framework that generates a modus operandi for rehabilitation after epilepsy surgery derived from regular longitudinal and prospective follow-up of patients and families. The framework focuses on patient experiences of undergoing surgery placed within the context of the family and broader community. It adopts a holistic view of patient care to understand the complex interactions between neurobiological and psychosocial factors that determine surgical outcome in the eyes of the patient, family, and clinical team. It emphasises the importance of anticipating postoperative adjustment issues using a preventive treatment approach.     

Paradoxical results in the cure of chronic illness: the "burden of normality" as exemplified following seizure surgery

Little has been written about processes of recovery following life-changing medical interventions for chronic illness. This article reviews our research with chronic epilepsy patients undergoing neurosurgery for the relief of intractable partial seizures. This research has given rise to a new conceptualization of adjustment and outcome following effective treatment of chronic illness, representing the first, detailed characterization of this process from a psychological and psychosocial perspective. Crucial to outcome are patient and family expectations prior to treatment, and learning to discard roles associated with chronic illness after treatment. These and the posttreatment affective functioning of the patient temper the view of medical outcome, and can account for paradoxical clinical effects, such as worsening patient psychosocial functioning in the context of medical treatment success. Our results have clear implications for the clinical management of chronically ill patients and their families to optimize treatment outcome.     

The longitudinal course of adjustment after seizure surgery.

Surgical alleviation of chronic epilepsy can give rise to a process of adjustment as the chronically ill patient learns to become well. This process can manifest clinically as an array of symptoms which we have previously described as the 'burden of normality'. The aim of this study was to explore the longitudinal course of post-operative adjustment by mapping the incidence of symptoms of the burden of normality over a period of 2 years, and examining symptom occurrence relative to seizure outcome. A series of 90 anterior temporal lobectomy (ATL) patients was drawn from our Seizure Surgery Follow-up and Rehabilitation Program. All patients were prospectively assessed using the Austin CEP Interview, which covers symptoms of the burden of normality. In total, 66% of patients reported symptoms at some time within the first 2 years of surgery. Symptoms often emerged by the 3 month review, but were still seen frequently in the second year. At the 24 month review, patients who had been seizure free or experienced auras only within the previous 18 months were significantly more likely to report symptoms compared to patients who had experienced complex partial and/or generalized tonic-clonic seizures (P = 0.03). Surgical alleviation of seizures in chronic epilepsy brings with it the burden of normality. Recognition of this syndrome is essential in maximizing patient outcome.     

Nonepileptic seizures after resective epilepsy surgery

PURPOSE: To identify factors that are associated with the emergence of nonepileptic seizures (NES) after resective epilepsy surgery. METHODS: Twenty-two patients with medically refractory epilepsy in whom NESs were documented by EEG after resective surgery were compared with a larger series of epilepsy surgery patients on demographic, neurologic, and psychiatric variables. RESULTS: NES tended to become apparent in the first few months after surgery. Patients who developed NESs did not differ from other epilepsy surgery patients in terms of age, IQ, or preoperative psychiatric diagnoses. However, surgical NES patients' neurologic problems and seizures began later in life, the NES group included a larger proportion of female subjects and patients with right hemisphere surgery, and NES patients were more likely to develop non-NES psychiatric problems after surgery. CONCLUSIONS: The heterogeneous collection of behaviors subsumed under the label NESs are determined by multiple factors. Several variables were found to be specifically associated with the development of NES after resective epilepsy surgery: A disproportionate number of postsurgical NES patients are female, they have primary neurologic dysfunction in the right hemisphere, and their epileptic seizures often began after adolescence. We propose that at least one group of patients with somatoform tendencies develop NESs as part of the psychiatric instability that occurs often in the few months after resective surgery.     

Predictive coding: A contemporary view on the burden of normality and forced normalization in individuals undergoing epilepsy surgery

Following epilepsy surgery, a good psychosocial outcome is not necessarily contingent on a good seizure outcome. Increasingly, it is believed that “successful” surgery is a combination of both an acceptable and expected seizure status as well as the individual's perception of improvements in quality of life (QOL). The factors that create this optimal outcome remain an ongoing area of research in the epilepsy community. That being said, there have been some major breakthroughs in observing and understanding poor outcomes seen in a subset of postoperative patients with epilepsy. Characteristics of burden of normality and forced normalization are two phenomena that have been evident in cases of poor postoperative outcomes. In this review, we provide a summary of research and concepts used to explain these poor QOL outcomes for a seemingly successful surgery and suggest a contemporary view in understanding the mechanism of forced normalization through understanding the brain as a predictive organ. Using such a predictive coding model together with recommendations of other studies, we suggest the crucial need for a preoperative intervention addressing patient predictions and expectations to optimize on the benefits achievable through epilepsy surgery.     

Electrographic neonatal seizures after infant heart surgery

PURPOSE: Neonatal seizures are relatively common and an important early sign of acute encephalopathy in those who survive infant heart surgery. The contemporary occurrence of seizures in this setting is not fully known, and their electrographic characteristics are incompletely described. This study describes the characteristics of electrographic neonatal seizures (ENSs) in contemporary infants with congenital heart disease (CHD) surgically repaired by using cardiopulmonary bypass, with or without deep hypothermic circulatory arrest. METHODS: Consecutive infants undergoing heart surgery were monitored by video-EEG for 48 h postoperatively to establish the time of first seizure, total number of ENSs, site(s) of ENS(s) origin and other characteristics. RESULTS: ENSs occurred in 21 (11.5%) of 183 infants. None had clinically visible seizures. The mean time to the first ENS was 21 h (range, 10-36 h). The total number of ENSs among the entire cohort was 1,429. Mean total number of ENSs per patient over a 48-h period was 72 (range, 1-217). Phenobarbital administration was associated with a > or =50% reduction in seizure counts in five (41.7%) of 12 subjects. CONCLUSIONS: ENSs were relatively common in a large, contemporary cohort of infants after infant heart surgery. A wide variation was noted in seizure burden, but many experienced numerous seizures. Electrographic neonatal seizures are a candidate outcome end point in future neuroprotection trials in this patient population.     

Natural history of recurrent seizures after resective surgery for epilepsy.

Seizures persist or recur in 20-60% of patients after resective surgery for intractable partial epilepsy. Further information about the natural course of these seizures is lacking in the literature. During one decade of epilepsy surgery at a single institution, we identified 72 patients with recurrent postoperative seizures after resective procedures for epilepsy. Prospectively compiled seizure diaries, hospital records, and outpatient office records were reviewed and supplemented by telephone communications to assess subsequent seizure frequency. Follow-up data was available ranging from 6 months to 7 years 5 months (mean 3 years 5 months). The likelihood of persistent seizures and recurrent intractability was examined with life-tables. Seizures recurred within the first postoperative year in 86% of patients and were similar to preoperative events in 74% of patients. After the first seizure recurrence, there was 80% likelihood of persistent seizures in the next 6 years and 40% likelihood of intractability (more than one seizure a month despite optimal medical therapy). The interval until recurrence within the first postoperative year did not affect the likelihood of subsequent seizures or intractability. Late seizure recurrence (after the first year) was not associated with any instances of subsequent intractability. Recurrent seizures after extratemporal resections were more likely to become persistent and intractable than seizures recurring after temporal resections. This information provides rational prognostication and assists in counseling patients with recurrent seizures after resective surgery for intractable epilepsy.     

Clinical and economic burden of breakthrough seizures

PurposeThe purpose of this study was to measure health-care resource utilization and costs in treatment-adherent, previously seizure-free patients with epilepsy who were treated in the inpatient/emergency room (ER) setting for new-onset seizures, compared with matched controls.MethodsThe study used a retrospective case/control study design using administrative claims from the IMS PharMetrics™ database. We identified adult patients with epilepsy with 1 + ER visit/hospitalization with primary diagnosis of epilepsy between 1/1/2006 and 3/31/2011, preceded by 6 months of seizure-free activity and antiepileptic drug (AED) treatment adherence (≥ 80% of days covered by any AED); the first observed seizure defined the “breakthrough” seizure/index event. Treatment-adherent patients with epilepsy without any ER/hospital admission for seizures served as controls: an outpatient epilepsy-related medical claim within the selection window was chosen at random as the index date. The following were continuous enrollment requirements for all patients: ≥ 12-month pre- and ≥ 6-month postindex. Each case matched 1:1 to a control using propensity score matching. All-cause and epilepsy-related (epilepsy/convulsion diagnosis, AED pharmacy) resource utilization and unadjusted and adjusted direct health-care costs (per person, 2012 US dollars (USD)) were assessed in a 6-month follow-up period.Principal resultsThere were 5729 cases and 14,437 controls eligible. The final sample comprised 5279 matched case/control pairs. In unadjusted analyses, matched cases had significantly higher rates of all-cause hospitalization and ER visits compared to controls and significantly higher total all-cause direct health-care costs (median $12,714 vs. $5095, p < 0.001) and total epilepsy-related costs among cases vs. controls (median $7293 vs. $1712, p < 0.001), driven by higher inpatient costs. Among cases, costs increased with each subsequent seizure (driven by inpatient costs). Cases had 2.3 times higher adjusted all-cause costs and 8.1 times higher adjusted epilepsy-related costs than controls (both p < 0.001).ConclusionInpatient/ER-treated breakthrough seizures occurred among 28.4% of our treatment-adherent study sample and were associated with significant incremental health-care utilization and costs, primarily driven by hospitalizations. Our findings suggest the need for better seizure control via optimal patient management and the use of effective AED therapy, which can potentially lower health-care costs.     

Psychosocial adjustment before and after coronary artery bypass surgery

Twenty-eight consecutive male patients were examined a few days before, and again twelve months after, coronary artery bypass surgery. The evaluation included the patients' psychological distress, psychosocial adjustment, cardiac state, personality and family relations. Before surgery the patients were relatively well adjusted, despite being severely disabled physically. On the follow-up evaluation the cardiac state improved significantly on every index examined. On the other hand no change occurred in the psychological distress, personality and family relations. Significant post-operative improvement was found in some of the domains of the psychosocial adjustment, but not in others.     

"Illusions of normality": a methodological critique of category-specific naming

Category-specific disorders are perhaps the archetypal example of domain-specificity--being typically defined by the presence of dissociations between living and nonliving naming ability in people following neurological damage. The methods adopted to quantify naming across categories are therefore pivotal since they provide the criterion for defining whether patients have a category effect and necessarily influence the subsequent direction and the interpretation of testing. This paper highlights a series of methodological concerns relating to how we measure and define category (or any) dissociations. These include the common failure to include control data or the use of control data that is inappropriate e.g. at ceiling, unmatched. A review of past cases shows that the overwhelming majority suffers from these problems and therefore challenges conclusions about the purported empirical demonstrations of dissociations and double dissociations in the category specific literature. This is not a refutation of category deficits, but skepticism about the current existence of any convincing empirical demonstrations of category specific double dissociations. As a potential solution, certain minimal criteria are proposed that might aid with the attempt to document category effects that are more methodologically convincing.     

Management of febrile seizures: current concepts and recommendations for phenobarbital and the electroencephalogram

Recent emphasis in the medical and lay press on phenobarbital-induced cognitive deficits and failure to obtain satisfactory compliance and benefit from long-term anticonvulsant therapy has fueled the controversy concerning current concepts of management of febrile seizures and prompted alternative recommendations. A survey of pediatricians in Central and Southern Illinois showed the mean number of febrile seizures treated in an office practice in a 12-month period was 7.56 +/- 7.4, of which 20% were of the complex type. Complex febrile seizures, affecting an estimated 5000 children in Illinois, were treated with phenobarbital by 90% of respondents, and therapy was continued for an average of 2 years. The electroencephalogram (EEG) was used in determining the need for phenobarbital prophylactic therapy by 52% of respondents. An alternative approach to treatment is outlined in light of the questionable benefits and potential side effects of long-term phenobarbital. Parental education and counseling in the management of fever and convulsions and intermittent methods of prophylaxis are emphasized, and the more limited use and careful monitoring of phenobarbital therapy in selected high-risk patients is suggested. The value of the EEG in prognosis and prediction of epilepsy in children with febrile seizures deserves further study.     

Late seizures in patients initially seizure free after epilepsy surgery

PURPOSE: Surgery for medically intractable epilepsy is currently the most effective means of achieving seizure control. Although relatively few long-term outcome studies have been performed, evidence is mounting that the possibility of late seizure recurrence exists, even after an early seizure-free period. No published reports document the rate and predictors of late recurrence in a large series of patients undergoing surgery in the magnetic resonance imaging (MRI) era. METHODS: We retrospectively queried the databases of two epilepsy surgery centers. Patients eligible for study had preoperative MRI scans, were seizure free for 1 year after surgery, and had a minimal follow-up period of 3 years. Patients with tumors or vascular lesions were excluded. We performed log-rank comparison of Kaplan-Meier product limit estimates for categoric variables and used a Cox proportional hazards model for continuous variables. Variables that were significant (p<0.05) on a univariate screen were entered into a multivariate forward step-wise Cox regression. RESULTS: The study included 285 patients, 254 with medial temporal lobe (MTLE) and 31 with neocortical epilepsy. The probability of having a single seizure after being seizure free for 1 year was 18.3% at 5 years and 32.7% at 10 years. However, only 13% were not seizure free at the last follow-up. Predictors of late recurrences on both uni- and multivariate analysis were the presence of preoperative generalized tonic-clonic (GTC) seizures in patients with neocortical epilepsy and late age at surgery in patients with MTLE. MRI results and location of surgery were not predictive. CONCLUSIONS: Although the risk of at least one recurrent seizure after initially successful epilepsy surgery is relatively high, the rate of recurrent intractability is low. The finding that late age at surgery and presence of preoperative GTC seizures are predictors of late recurrence indicates the importance of patient selection and early surgery for persistent seizure control.     

Absence seizures: A review of recent reports with new concepts

Absence seizures with bilateral spike–wave (SW) complexes at 3 Hz are divided into the childhood form, with onset at around 6 years of age, and the juvenile form, with onset usually at 12 years of age. These seizures typically last 9–12 s and, at times, are activated by hyperventilation and occasionally by photic stimulation. Generalized tonic–clonic (GTC) seizures may also occur, especially in the juvenile form. There may be cognitive changes, in addition to linguistic and behavioral problems. Possible mechanisms for epileptogenesis may involve GABAergic systems, but especially T-calcium channels. The thalamus, especially the reticular nucleus, plays a major role, as does the frontal cortex, mainly the dorsolateral and orbital frontal areas, to the extent that some investigators have concluded that absence seizures are not truly generalized, but rather have selective cortical networks, mainly ventromesial frontal areas and the somatosensory cortex. The latter network is a departure from the more popular concept of a generalized epilepsy. Between the “centrencephalic” and “corticoreticular” theories, a “unified” theory is presented. Proposed genes include T-calcium channel gene CACNA1H, likely a susceptible gene in the Chinese Han population and a contributory gene in Caucasians. Electroencephalography has revealed an interictal increase in prefrontal activity, essential for the buildup of the ictal SW complexes maximal in that region. Infraslow activity can also be seen during ictal SW complexes. For treatment, counter to common belief, ethosuximide may not increase GTC seizures, as it reduces low-threshold T-calcium currents in thalamic neurons. Valproic acid and lamotrigine are also first-line medications. In addition, zonisamide and levetiracetam can be very helpful in absence epilepsy.     

Outcome assessment in seizure surgery: The role of postoperative adjustment

The usual criteria employed in assessing the outcome of seizure surgery is almost always that of success in abolition of seizures. Whilst this is a logical method of assessment, experience with large series of such patients shows that this is by no means the only significant criterion. Here we examine the process of postoperative adjustment and the role it plays in determining the outcome of temporal lobectomy for refractory epilepsy. The specific domains involved in this process are examined and it is shown that in a number of cases what is thought to have been surgical success has in fact been significantly diminished by difficulties encountered in postoperative adjustment — to such an extent that total seizure abolition has not resulted in clinical advantage.