Epilepsy in Canadian households: The financial pinch.

BACKGROUND: It has long been recognized that epilepsy has a substantial economic impact on populations including health care costs and employment impact. There is a paucity of data on the individual and household impact of epilepsy on financial income. METHODS: Canadian data on households containing persons with epilepsy were derived from the National Population Health Survey of Neurological Conditions within the Canadian Community Health Survey (CCHS), a Canadian population-based cross-sectional survey that collects information related to health status (2010-2011). We determined the number of households in Canada reporting at least one member with epilepsy. We examined the difference in reported household income between households reporting at least one member with epilepsy with those not reporting any members with epilepsy. We then developed a model of reported household income within Canada in order to adjust the estimated impact on household income of having at least one member with epilepsy. RESULTS: In 2010-2011, 122,911 respondents to the CCHS identified 1,254 households containing individuals with epilepsy, representing âˆ¼ 594,200 households in Canada (95 % CI: 534,900-650,500). These respondents reported that household income for households with members with epilepsy was reduced by CDN$ 14,000 when compared to the mean reported household income of CDN$ 83,000, (p < 0.001). In a model of income, the adjusted impact of a household member with epilepsy was CDN$ 14,700 (95 % CI: 10,000-19,500). CONCLUSION: The financial impact on Canadian households of persons with epilepsy is profound. While we can only speculate on the rationale driving this, the differential in average household income in households living with an individual with epilepsy is clear. This places an additional financial burden on persons with epilepsy (PWE) who may already face substantial medication, health care and caregiver costs.

Are YouTube seizure videos misleading? Neurologists do not always agree.

The internet has become the first stop for the public and patients to seek health-related information. Video-sharing websites are particularly important sources of information for those seeking answers about seizures and epilepsy. Because of the widespread popularity of YouTube, we sought to explore whether a seizure diagnosis and classification could reliably be applied. All videos related to "seizures" were reviewed, and irrelevant videos were excluded. The remaining 162 nonduplicate videos were analyzed by 4 independent pediatric neurologists who classified the events as epilepsy seizures, nonepileptic seizures, or indeterminate. Videos designated as epilepsy seizures were then classified into focal, generalized, or unclassified. At least 3 of the 4 reviewers agreed that 35% of the videos showed that the events were "epilepsy seizures", at least 3 of the 4 reviewers agreed that 28% of the videos demonstrated that the events were "nonepileptic seizures", and there was good agreement that 7% of the videos showed that the event was "indeterminate". Overall, interrater agreement was moderate at k=0.57 for epilepsy seizures and k=0.43 for nonepileptic seizures. For seizure classification, reviewer agreement was greatest for "generalized seizures" (k=0.45) and intermediate for "focal seizures" (k=0.27), and there was no agreement for unclassified events (k=0.026, p=0.2). Overall, neurology reviewer agreement suggests that only approximately one-third of the videos designated as "seizures" on the most popular video-sharing website, YouTube, definitely depict a seizure. Caution should be exercised in the use of such online video media for accessing educational or self-diagnosis aids for seizures.

Epilepsy in the Twitter era: a need to re-tweet the way we think about seizures.

Seizures have long been associated with misconceptions and stigma. Exponential growth in Internet use has seen the rapid expansion of social media, such as Twitter, for health promotion. In view of the popularity of Twitter, we sought to explore how seizures are being portrayed on this social networking website and to consider its potential for information dissemination. A 48-hour Twitter search was used as a preliminary data set to determine an appropriate classification scheme of "seizure"-related posts ("tweets"). Analysis was then conducted using "seizure" tweets from a 7-consecutive day sample period. Tweets were analyzed and coded by two independent reviewers. Predominant categories were Metaphorical (32%), Personal Accounts (31%), Informative (12%), and Ridicule/Joke (9%). This study supports the notion that stigmatization associated with seizures continues to flourish, as 41% of "seizure" tweets were derogatory in nature. Although Twitter could be used to disseminate accurate information on seizures and epilepsy, this study suggests that it is currently propagating negative attitudes toward seizures with potential for fueling stigma. In recent years there have been significant advancements in technology offering many new methods of sharing information. Social networking sites allow real-time communication while providing the opportunity for exchange of information and opinions. Twitter, a website launched in 2006, allows users to communicate through "tweets" limited to 140 characters. Twitter's popularity has drastically increased since its inception, with approximately 110 million tweets per day from 200 million users worldwide, as of January 2011 (http://blogs.forbes.com/oliverchiang/2011/01/19/twitter-hits-nearly-200m-users-110m-tweets-per-day-focuses-on-global-expansion/). Such social media facilitate communication about an array of health-related topics including seizures and epilepsy.

Metabolic cost of lateral stabilization during walking in people with incomplete spinal cord injury.

People with incomplete spinal cord injury (iSCI) expend considerable energy to walk, which can lead to rapid fatigue and limit community ambulation. Selecting locomotor patterns that enhance lateral stability may contribute to this population's elevated cost of transport. The goal of the current study was to quantify the metabolic energy demands of maintaining lateral stability during gait in people with iSCI. To quantify this metabolic cost, we observed ten individuals with iSCI walking with and without external lateral stabilization. We hypothesized that with external lateral stabilization, people with iSCI would adapt their gait by decreasing step width, which would correspond with a substantial decrease in cost of transport. Our findings support this hypothesis. Subjects significantly (p<0.05) decreased step width by 22%, step width variability by 18%, and minimum lateral margin of stability by 25% when they walked with external lateral stabilization compared to unassisted walking. Metabolic cost of transport also decreased significantly (p<0.05) by 10% with external lateral stabilization. These findings suggest that this population is capable of adapting their gait to meet changing demands placed on balance. The percent reduction in cost of transport when walking with external lateral stabilization was strongly correlated with functional impairment level as assessed by subjects' scores on the Berg Balance Scale (r=0.778) and lower extremity motor score (r=0.728). These relationships suggest that as functional balance and strength decrease, the amount of metabolic energy used to maintain lateral stability during gait will increase.

Accidental injury is a serious risk in children with typical absence epilepsy.

OBJECTIVES: To determine if young adults with a history of typical absence epilepsy (AE) in childhood have a greater risk of accidental injury than controls with juvenile rheumatoid arthritis (JRA). To assess the nature and severity of these injuries. METHODS: All patients with AE or JRA diagnosed between 1977 and 1985, who were 18 years or older at the onset of the study, were identified from review of pediatric electroencephalographic records for the province of Nova Scotia (AE) or review of the medical records database at the only tertiary care pediatric center for the province (JRA). Fifty-nine (86%) of 69 patients with AE and 61 (80%) of 76 patients with JRA participated in an interview in 1994 or 1995, assessing nature, severity, and treatment of prior accidental injuries. Patients with AE were further questioned about injuries sustained during an absence seizure. RESULTS: Sixteen (27%) of 59 patients with AE reported accidental injury during an absence seizure, with risk of injury being 9% per person-year of AE. Most injuries (81%) occurred during anti-epileptic drug therapy. Although the majority of injuries did not require treatment, 2 (13%) of 16 patients required minor treatment and 2 (13%) of 16 were admitted to hospital. The risk of accidental injury resulting from an absence seizure in person-years at risk was highest in juvenile myoclonic epilepsy (45%), moderate in juvenile AE (14%), and lowest in childhood AE (3%). Patients with AE had a greater number of overall accidental injuries than those with JRA (P<.04), but these differences were particularly marked for bicycle (P<.003) and car accidents (P<.05) and for mild head injuries (P=.05). CONCLUSIONS: Accidental injury is common in AE and usually occurs after anti-epileptic drug treatment is started. Injury prevention counseling is indicated both at diagnosis and follow-up. Bicycle accidents pose a special risk and helmet use should be mandatory.

Long-term prognosis of typical childhood absence epilepsy: remission or progression to juvenile myoclonic epilepsy.

OBJECTIVE: To determine the proportion and characteristics of children presenting with childhood absence epilepsy (CAE) who were not taking anti-epileptic drugs (AEDs) and were seizure-free over the last year of long-term follow-up. METHODS: For case finding, centralized EEG records for the province of Nova Scotia allowed identification of all children with typical CAE diagnosed between 1977 and 1985. Follow-up was done in 1994 to 1995. RESULTS: Of 81 children with CAE, 72 (89%) were contacted for follow-up. Mean age at seizure onset was 5.7 years (range, 1 to 14 years) and at follow-up was 20.4 years (range, 12 to 31 years). Forty-seven (65%) were in remission. Twelve others (17%) were not taking AEDs but continued to have seizures. Thirteen (18%) were taking AEDs; five were seizure-free over the last year (in four of these a trial without AEDs had previously failed). Fifteen percent of the total cohort had progressed to juvenile myoclonic epilepsy (JME). Multiple clinical and EEG factors were examined as predictors of outcome. Factors predicting no remission (p < 0.05) included cognitive difficulties at diagnosis, absence status prior to or during AED treatment, development of generalized tonic clonic or myoclonic seizures after onset of AEDs, abnormal background on initial EEG, and family history of generalized seizures in first-degree relatives. CONCLUSIONS: Only 65% of children presenting with CAE had remission of their epilepsy. Forty-four percent of those without remission had developed JME. At the time of diagnosis, remission is difficult to predict accurately in most patients. However, development of generalized tonic-clonic seizures or myoclonic seizures during AED treatment is ominous, predicting both lack of remission of CAE and progression to JME.

Children with febrile seizures do not consume excess health care resources.

BACKGROUND: Febrile seizures are benign but so terrifying for parents that they may subsequently view their affected children as "vulnerable". Children viewed as vulnerable may be brought to medical attention more frequently. We examined subsequent hospitalizations and physician visits during a 6- to 7 1/2-year period for a group of children who had participated in a case-control study of initial febrile seizures. METHODS: Individual data from a regional cohort of 75 children with a first febrile seizure and 150 febrile and 150 afebrile controls were linked to 2 comprehensive provincial health services databases-a hospital admissions/ separations database and a physician services database. RESULTS: Linkage was achieved for 98% of the study cohort, with heath care utilization data for 6 to 7 1/2 years available for 96%. Children with febrile seizures had nearly identical rates of subsequent hospitalization compared with age-matched controls (chi2 test, P = .88). An excess of day-surgery visits for primarily otolaryngologic procedures was seen for the febrile seizure patients 0 to 12 months after their initial febrile seizure (chi2 test, P < .001). During the next 6 to 7 1/2 years, the febrile seizure patients had nearly identical rates of physician visits (chi2 test, P = .15); however, they had more visits to otolaryngologists in the first 3 to 9 months after the febrile seizure (chi2 test, P < .001), but fewer visits to pediatricians during the next 1 to 4 years (chi2 test, P < .001). CONCLUSIONS: Children with febrile seizures have nearly identical rates of hospital and physician services utilization compared with controls. This supports the hypothesis that febrile seizures are benign, and that parents recover from their initial anxiety and do not consider their children vulnerable to additional illness in the years that follow.

Long-term psychosocial outcome in typical absence epilepsy. Sometimes a wolf in sheeps' clothing.

OBJECTIVES: To determine whether young adults in whom typical absence epilepsy has been diagnosed in childhood have greater psychosocial difficulties than those with a non-neurologic chronic disease and to decide which seizure-related factors predict poor psychosocial outcome. DESIGN: Population-based, inception cohort study. SETTING: The only tertiary care pediatric hospital in the province of Nova Scotia. PATIENTS: All children in whom typical absence epilepsy or juvenile rheumatoid arthritis (JRA) was diagnosed between January 1, 1997, and December 31, 1985, who were aged 18 years or older at follow-up in March 1994 to April 1995. Patients with typical absence epilepsy were identified from centralized electroencephalographic records for Nova Scotia, and those with JRA were identified from discharge diagnoses from the only children's hospital in Nova Scotia. MAIN OUTCOME MEASURE: Patients participated in a structured interview that assessed psychosocial function. RESULTS: Fifty-six (86%) of the 65 patients with absence epilepsy and 61 (80%) of the 76 patients with JRA participated in the interview. The mean age of the patients at the interview was 23 years. Terminal remission occurred in 32 (57%) of the patients with typical absence epilepsy but in only 17 (28%) of the patients with JRA. Factor analysis identified 5 categories of outcome: academic-personal, behavioral, employment-financial, family relations, and social-personal relations. Patients with typical absence epilepsy had greater difficulties in the academic-personal and in the behavioral categories (P < .001) than those with JRA. Those with ongoing seizures had the least favorable outcome. Most seizure-related factors showed minimal correlation with psychosocial functioning. CONCLUSION: Young adults with a history of typical absence epilepsy, particularly those without remission of their seizures, often have poor psychosocial outcomes, considerably worse than those with JRA.

Parents of children with epilepsy are optimistic for their children's health, but relatively pessimistic when compared with other parents.

We examined parents' perceived risk of their children encountering 10 general health conditions and 10 epilepsy-specific health problems using a standard optimistic bias question with standard responses. "Compared to other children of similar age, my child's chance of getting [problem, e.g., kidney disease] in the future is" (on a 7-point response scale) "much below average em leader average em leader much above average." "Pessimistic" parents were defined as those whose mean answers exceeded average risk. Parents demonstrated an optimistic bias for most health risks. For all the general health risks, the parents of children with epilepsy showed less optimistic bias (or pessimism) (P=0.001). Parents of children with epilepsy were much more likely to be "pessimistic" about future health risks (odds ratio 3.0, 95% CI: 1.1, 8.4) but showed an optimistic bias for the epilepsy-specific health risks. Parents of children with epilepsy appear to judge their children as more vulnerable to additional health problems when compared with parents of healthy children.

Pseudo-tics in Tourette syndrome.

We describe two patients with Tourette syndrome who also had pseudo-tics. The psychogenic movements resolved in both children when the stressful issues in their lives were addressed. It is important to differentiate psychogenic symptoms from tics and compulsions in children with Tourette syndrome in order to avoid unnecessary medication and allow appropriate therapy.

Benign rolandic epilepsy: atypical features are very common.

The objective of this study was to determine the frequency of atypical clinical and electrographic features in children with benign rolandic epilepsy. A retrospective case series design was employed in the setting of a tertiary care pediatric hospital. Forty-two children with benign rolandic epilepsy were seen through our neurology department between January 1, 1991, and December 31, 1993. Their charts were reviewed for atypical clinical features, imaging studies and results, total number of seizures at initial presentation and last follow-up, and use of anticonvulsants. Atypical clinical features included status epilepticus, developmental delay, daytime-only seizures, screaming as a seizure component, and postictal Todd's paresis. All children had at least one electroencephalogram, and these records were reviewed for atypical electrographic features such as unusual location, atypical spike morphology, and abnormal background. Atypical clinical features were seen in 50% of patients and atypical electrographic features in 31%. Computed tomographic scans were performed in 15 patients and were consistently normal. Treatment with anticonvulsant medication was initiated in 40%. Although patients with atypical features did not have an increased seizure frequency, they were more likely to undergo imaging studies (P < .01) and to be commenced on anticonvulsant medication (P < .02). Our experience suggests that atypical clinical and electrographic features are the rule rather than the exception in benign rolandic epilepsy. Further work must be done to develop a reliable definition of this common entity.

Brain activity and cognitive status in pediatric patients: development of a clinical assessment protocol.

The purpose of this study was to test the validity of a new computerized task to assess children's cognitive problem-solving skills using the brain event-related potentials. This event-related potential-computerized cognitive problem-solving task does not require a child to give a verbal or motor (ie, pointing) response. The event-related potential waveforms were recorded from 20 typically developing children. Two nonverbal, problem-solving tasks (tasks 1 and 2) were developed for each of two age groups (5 and 6 years). For each task, single pictures, taken from an existing standardized test of nonverbal problem solving, were individually and sequentially presented on a computer screen. One of the seven pictures was classified as incongruent or outside category; it did not belong with the other pictures. As predicted, the event-related potential amplitudes were significantly larger to the outside- versus within-category pictures. This effect was found for tasks 1 and 2 for the 5- and 6-year-old children. Children as young as 5 years of age reliably exhibit brain activity, which can be used to infer cognitive problem-solving skill. This assessment paradigm may eventually serve as a clinically useful adjunct to a thorough neurologic and neurodevelopmental assessment of selected pediatric populations, such as those presenting with moderate-severe cerebral palsy whose expressive language and motor skills are notably impaired.

Tremorgenic encephalopathy: a role of mycotoxins in the production of CNS disease in humans?

We describe a young man who, shortly after exposure to moldy silage, developed a neurological syndrome consisting of dementia and a remarkable tremor which was precipitated by movement. All symptoms resolved within one week. Despite investigation, no definitive diagnosis was reached. We propose that this patient's illness may have resulted from inhalation exposure to a tremorgenic mycotoxin.

Parent perceptions of the value of Pediatric Neurology Clinic visits.

BACKGROUND: There is little data available on the acceptability to parents, of alternate methods of delivering care to their children. This pilot study explored parents' perceptions of conventional clinic visits and their attitudes to potential alternative methods of delivering care. METHODS: Questionnaires were completed by the families of 200 consecutive children before and after a visit to a tertiary-care Pediatric Neurology Clinic in Nova Scotia, Canada. RESULTS: Responses were received from 172 (86%) for the first questionnaire and 138 (69%) for the second. There were 59 new referrals, 76 follow-up visits and 39 were seen because of new concerns. Visiting the clinic resulted in school absenteeism for 85% of the children and lost pay for 19% of the parents. Telephone, video conference or replacement of the physician by a nurse practitioner were acceptable alternate methods of assessment for only 10%. The only factor which was associated with willingness to accept telephone as an assessment modality was an initial opinion that the visit was unnecessary. CONCLUSION: Escalating health care costs and limited specialist availability demand consideration of alternate methods of care delivery. Although adults seem comfortable with care outside the usual clinic setting, there is little data for the pediatric setting. The responses from the parents attending our clinic, indicate that families were happy with services offered in the conventional clinic. In contrast, only 10% found potential alternate methods acceptable. These opinions must be considered in the design of new methods of caring for children.

Prevalence of reported migraine headaches in Canadian adolescents.

OBJECTIVE: To examine self-reported prevalence data for migraine among adolescent Canadians and to explore how reported migraine treatment varies by age. METHODS: We analyzed the microdata files of the Canadian National Population Health Survey (1996-1997). Respondents reported whether they had "migraine headaches diagnosed by a health professional". They also reported whether they received "any treatment or medication for migraine headaches", with treatments subdivided into drug, diet or "other". RESULTS: 99.9% of 173,216 eligible respondents reported whether they had migraine headaches. Migraine was reported by 2.4% of Canadian youth aged 12-14 years and by 5.0% of 15-19 year-olds compared to 7.2% of adults aged > or = 20 years of age (p< 0.0001, chi-square). Active treatment was used by 51.0% - higher by females (53.1%) than males (44.7%) (p<0.0001 chi-square). Treatment was used by 45.1% of 12-14 year-olds, by 45.7% of 15-19 year-olds and by 51.5% of those > or = 20 years (p=0.0027). The nature of the active treatment choice (drug, diet or other) did not significantly vary within the age groups studied. CONCLUSIONS: We present robust estimates of self-report diagnosed migraine prevalence, derived from a large nationally representative population survey. Estimates of the prevalence of active treatment for migraine provide insight into the burden of migraine within this population.

The use of antiepileptic drug levels in children: a survey of Canadian pediatric neurologists.

There are 60 pediatric neurologists in Canada. Replies were received from 56 in response to a survey regarding the use and perceived value of antiepileptic drug (AED) levels. AED levels are frequently ordered and influence clinical care. There were, however, discrepancies among pediatric neurologists regarding the upper and lower limits of the "therapeutic ranges" and the clinical application of levels. We suggest that both the value and use of AED levels needs further study.

The epidemiology of infantile spasms.

OBJECTIVE: The aim of this study was to estimate population based incidence rates for infantile spasms (IS) and to study our clinical impression that the incidence of IS has recently decreased in the Canadian Provinces of Nova Scotia and Prince Edward Island. METHODS: Birth cohorts from 1978 to 1998, identified through the hospital health records, EEG records and physician computerized databases, were followed for two years for the development of IS. Disease incidence rates were calculated using denominators derived from Statistics Canada's reported annual live birth rates. RESULTS: The inclusion criteria for IS were fulfilled by 75 patients. The overall incidence of IS was 30.7/100,000 live births (95% Cl 24.3, 38.8). Etiologic classification was symptomatic for 51 cases (68%), cryptogenic for 18 (24%), and idiopathic in six children (8%). Although there were more males (N=44) than females (N=31), the incidence rates were similar. There was a marked variability in annual and five-year incidence rates. CONCLUSIONS: Although the clinical characteristics of our patients were similar to other reported IS populations, the instability in IS incidence rates indicates a need for caution in interpreting smaller IS epidemiologic studies.

Successful treatment of painful crises of Fabry disease with low dose morphine.

Fabry disease is an X-linked disorder characterized in childhood by angiokeratoma, corneal opacities, and pain. At age 7 years our patient began experiencing an intermittent intense "burning" sensation within his feet and hands (acroparesthesias). Treatment with aspirin, acetaminophen, acetominophen with codeine, and phenytoin was unsuccessful. Carbamazepine and phenytoin reduced the frequency and duration of painful crises to 3-4 times annually. A treatment plan was developed consisting of a low-dose morphine infusion with increasing dosage until pain was relieved. Over the subsequent 28 months, we have had experienced treating 7 crises with morphine given as 0.06 mg/kg IV push, followed by a continuous infusion of 0.02 mg/kg/hr with amitriptyline 0.25 mg/kg at bedtime. Pain control is immediate, with the infusion gradually tapered after 24 hours.

Male Rett syndrome variant: application of diagnostic criteria.

Classic Rett syndrome (RS) has been described in females only. Although an X chromosome origin is probable, it has not been substantiated. It is possible, therefore, that RS could occur in males. The authors describe a male with RS and review all the reported cases involving male patients. The authors compare their patient to the other patients and examine the applicability of the classic RS diagnostic criteria to this variant. To date, nine male patients with RS have been reported. The authors describe an additional male who met seven of nine necessary criteria and six of eight supportive criteria as defined by the RS Diagnostic Criteria Work Group. When the authors applied these criteria to the other nine reported patients, many necessary inclusion criteria were not met despite the absence of exclusion criteria. The supportive criteria were even more variable and limited in many patients. In conclusion, males with RS appear to represent a heterogeneous phenotype, with clinical features that may meet many but not all of the necessary diagnostic criteria of classic RS. Less restrictive criteria are needed to include this variant, which should be considered when evaluating males with idiopathic developmental regression, autistic features, and loss of hand function.

Irritability in Krabbe's disease: dramatic response to low-dose morphine.

Krabbe's disease is characterized by normal neonatal development with subsequent regression and profound, medically intractable irritability. Two female infants presented at 5 months of age with increasing irritability, abnormal motor control, and developmental regression. Investigations confirmed the diagnosis of Krabbe's disease. Maximal treatment of gastroesophageal reflux and nitrazepam 0.1 mg/kg by mouth three times daily were unsuccessful in controlling irritability. Morphine was initiated and titrated to 0.06 mg/kg by mouth every 6 hours in Patient 1 and 0.1 mg/kg by mouth every 8 hours in Patient 2, resulting in remarkably successful control of irritability. The diagnosis of Krabbe's disease is devastating for families and is compounded by the marked irritability. Management is difficult, but in these two infants, irritability was successfully controlled with low-dose morphine.