Clinical features and outcomes in children with seronegative autoimmune encephalitis.

AIM: To characterize the presenting features and outcomes in children with seronegative autoimmune encephalitis, and to evaluate whether scores at nadir for the Modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE) or its paediatric-specific modification (ped-CASE) are predictive of outcomes. METHOD: This observational study included children younger than 18 years of age with seronegative autoimmune encephalitis. Demographics and clinical data were collected. The mRS and CASE/ped-CASE scores were used to evaluate disease severity. Descriptive statistics and logistic regression were used for data analysis and to evaluate associations between scale scores and outcomes. RESULTS: Sixty-three children were included (39 [62%] females, median age 7 years, interquartile range [IQR] 4 years 1 months-11 years 6 months), with follow-up available for 56 out of 63 patients (median follow-up 12.2 months, IQR 13.4-17.8). The most frequent presenting neurological manifestation was encephalopathy (81%). Median CASE/ped-CASE and mRS scores at nadir were 12.0 (IQR 7.0-17.0) and 1.0 (IQR 0-2.0) respectively. Thirty-three patients (59%) had persistent neurological deficits at follow-up. Both scoring systems suggested good functional recovery (mRS score ≤2, 95%; CASE/ped-CASE score <5, 91%). CASE/ped-CASE score was more likely than mRS to distinguish children with worse outcomes. INTERPRETATION: Children with seronegative autoimmune encephalitis are likely to have neurological deficits at follow-up. CASE/ped-CASE is more likely to distinguish children with worse outcomes than MRS.

Position Statement on the Use of Medical Cannabis for the Treatment of Epilepsy in Canada.

In Canada, recreational use of cannabis was legalized in October 2018. This policy change along with recent publications evaluating the efficacy of cannabis for the medical treatment of epilepsy and media awareness about its use have increased the public interest about this agent. The Canadian League Against Epilepsy Medical Therapeutics Committee, along with a multidisciplinary group of experts and Canadian Epilepsy Alliance representatives, has developed a position statement about the use of medical cannabis for epilepsy. This article addresses the current Canadian legal framework, recent publications about its efficacy and safety profile, and our understanding of the clinical issues that should be considered when contemplating cannabis use for medical purposes.

Énoncé de position quant à l'utilisation du cannabis médical dans le traitement de l'épilepsie. L'utilisation du cannabis à des fins récréatives a été légalisée au Canada en octobre 2018. Parallèlement à ce changement de politique, de récentes publication visant à évaluer l'efficacité du cannabis dans le traitement de l'épilepsie, de même qu'une sensibilisation médiatique accrue en ce qui concerne son utilisation, ont eu pour effet d'augmenter l'intérêt du grand public à son égard. Le Comité médical thérapeutique de la Ligue canadienne contre l'épilepsie (LCCE), de concert avec un groupe multidisciplinaire d'experts et des représentants de l'Alliance canadienne de l'épilepsie, a ainsi élaboré un énoncé de position en ce qui regarde l'utilisation du cannabis médical dans le traitement de l'épilepsie. Cet article entend donc aborder le cadre légal qui prévaut actuellement au Canada et examiner de récentes publications s'étant penchées sur le profil sécuritaire et sur l'efficacité du cannabis. De plus, nous voulons apporter un éclairage au sujet des aspects cliniques dont il faudrait tenir compte au moment d'envisager l'utilisation du cannabis à des fins médicales.

Risk of seizures after immunization in children with epilepsy: a risk interval analysis.

BACKGROUND: In children with epilepsy, fever and infection can trigger seizures. Immunization can also induce inflammation and fever, which could theoretically trigger a seizure. The risk of seizure after immunization in children with pre-existing epilepsy is not known. The study objective was to determine the risk of medically attended seizure after immunization in children with epilepsy < 7 years of age. METHODS: We conducted a retrospective study of children < 7 years of age with epilepsy in Nova Scotia, Canada from 2010 to 2014. Hospitalizations, emergency visits, unscheduled clinic visits, and telephone calls for seizures were extracted from medical records. Immunization records were obtained from family physicians and Public Health with informed consent. We conducted a risk interval analysis to estimate the relative risk (RR) of seizure during risk periods 0-14, 0-2, and 5-14 days post-immunization versus a control period 21-83 days post-immunization. RESULTS: There were 302 children with epilepsy who were eligible for the study. Immunization records were retrieved on 147 patients (49%), of whom 80 (54%) had one or more immunizations between the epilepsy diagnosis date and age 7 years. These 80 children had 161 immunization visits and 197 medically attended seizures. Children with immunizations had more seizures than either those with no immunizations or those with no records (mean 2.5 versus 0.7 versus 0.9, p < 0.001). The risk of medically attended seizure was not increased 0-14 days after any vaccine (RR = 1.1, 95% confidence interval (CI): 0.5-2.8) or 0-2 days after inactivated vaccines (RR = 0.9, 95% CI: 0.1-7.1) versus 21-83 days post-immunization. No seizure events occurred 5-14 days after live vaccines. CONCLUSIONS: Children with epilepsy do not appear to be at increased risk of medically attended seizure following immunization. These findings suggest that immunization is safe in children with epilepsy, with benefits outweighing risks.

Perceived need for restrictions on activity for children with epilepsy.

BACKGROUND: Children and youth with epilepsy have long been subjected to excessive restrictions on extracurricular activities due to concerns over risk of injury. Over time physicians and medical regulatory associations have liberalized the advice given for people with epilepsy to promote independence, self-esteem and general health benefits of physical activity. Current evidence suggests that few restrictions are needed for children with epilepsy beyond water-related precautions and avoidance of very high-risk activities. However, more stringent restrictions on daily activities may be imposed by caregivers. This study was aimed at exploring current perceptions of parents regarding restrictions on activity for children with epilepsy and the child's perspective on restrictions related to the diagnosis. METHODS: A self-administered questionnaire was offered to a sample of parent-child dyads of children/youth with epilepsy attending summer camp for children with epilepsy age 8-18years. A 10-item validated HARCES Parent Scale of Childhood Epilepsy was completed by the parent/guardian and a modified-HARCES completed by the child. The primary objective was to assess the degree of restrictions placed on children with epilepsy from the perspective of child and parent assessed independently. Agreement of perceived restrictions between parent-child dyads was also determined. RESULTS: 21 parent/guardian-child pairs were recruited with mean age of children/youth 12.7years (range 9-16years). Total HARCES scores for parents and guardians ranged from 11-26 (x=16.5; SD 4.9) while total scores for children with epilepsy similarly ranged from 10-25 (x=15.2; SD 4.9). There were no differences in total parent scores when analyzed by child's age (<13 or >13years), gender, age of seizure onset, seizure frequency or seizure type. Total HARCES scores showed no agreement between parent and child pairs with correlation of 0.2798 (95% CI -0.173-0.635). CONCLUSIONS: Children and youth with epilepsy often face activity restrictions based on fear of perceived risk of injury. This small sample shows evidence that even more permissive parents and his/her children still feel limited by such restrictions. Parents and children do not perceive these restrictions in the same way despite similar education by physicians highlighting an important secondary role of epilepsy camps in targeting misperceptions and educating families on appropriate precautions.

Canadian Paediatric Neurology Workforce Survey and Consensus Statement.

BACKGROUND: Little knowledge exists on the availability of academic and community paediatric neurology positions. This knowledge is crucial for making workforce decisions. Our study aimed to: 1) obtain information regarding the availability of positions for paediatric neurologists in academic centres; 2) survey paediatric neurology trainees regarding their perceptions of employment issues and career plans; 3) survey practicing community paediatric neurologists 4) convene a group of paediatric neurologists to develop consensus regarding how to address these workforce issues. METHODS: Surveys addressing workforce issues regarding paediatric neurology in Canada were sent to: 1) all paediatric neurology program directors in Canada (n=9) who then solicited information from division heads and from paediatric neurologists in surrounding areas; 2) paediatric neurology trainees in Canada (n=57) and; 3) community paediatric neurologists (n=27). A meeting was held with relevant stakeholders to develop a consensus on how to approach employment issues. RESULTS: The response rate was 100% from program directors, 57.9% from residents and 44% from community paediatric neurologists. We found that the number of projected positions in academic paediatric neurology is fewer than the number of paediatric neurologists that are being trained over the next five to ten years, despite a clinical need for paediatric neurologists. Paediatric neurology residents are concerned about job availability and desire more career counselling. CONCLUSIONS: There is a current and projected clinical demand for paediatric neurologists despite a lack of academic positions. Training programs should focus on community neurology as a viable career option.

The diagnostic utility of intracranial EEG monitoring for epilepsy surgery in children.

OBJECTIVE: There are limited data on the indications for the use of chronic invasive electroencephalography (EEG) monitoring (IEM) for pediatric epilepsy surgery. METHODS: We retrospectively studied 102 children who underwent intracranial monitoring to map critical cortex, localize the epileptogenic region, or resolve divergent findings. We assessed IEM utility based on changes to the resection plan following analysis of noninvasive data. RESULTS: IEM was judged useful in 87% of cases and had greatest utility for resolving discordant data and localizing extratemporal and multilobar epileptogenic zones. IEM data were least useful for seizure onset in the temporal lobe and had little utility for direct cortical stimulation mapping unless functional magnetic resonance imaging (fMRI) revealed atypical language representation or the epileptogenic zone was in proximity to critical cortex. SIGNIFICANCE: IEM utility was demonstrated for a majority of cases with well-defined indications. The method of assessing utility will facilitate multicentric studies toward developing future consensus and practice guidelines.

Vaccine coverage among children with epilepsy in two Canadian provinces: A Canadian immunization research network study.

OBJECTIVES: Children with epilepsy are at increased risk of complications from vaccine-preventable infections, yet information on vaccine coverage in these children is scarce. We aimed to compare vaccine coverage among children with epilepsy to children without epilepsy. STUDY DESIGN: We conducted a retrospective cohort study including all 2005-2013 births in Manitoba and Ontario, Canada, creating two cohorts: 2-year-olds and 7-year-olds (followed to age 2 and 7 years). We split each cohort into epilepsy and non-epilepsy subcohorts. We assessed vaccination coverage based on provincial schedules and determined timeliness of MMR (measles, mumps, rubella) dose 1 (recommended at 12 months) and DTaP (diphtheria, tetanus, pertussis) dose 4 (recommended at 18 months). We used logistic regression to calculate adjusted odds ratios (aORs) of the association between epilepsy and vaccination, combining both provincial estimates using random effects meta-analysis. RESULTS: We included 16,558 2-year-olds (Manitoba, 653; Ontario, 15,905) and 13,004 7-year-olds (Manitoba, 483; Ontario, 12,521) with epilepsy. At age 2 years, the aOR for up-to-date vaccination among children with versus without epilepsy was 0.9 (95% confidence interval 0.8-1.1); at age 7 years it was 1.0 (0.9-1.1). Infants diagnosed with epilepsy before age 6 months were less likely to be up-to-date at age 2 years (0.9; 0.8-0.9), although this difference disappeared by age 7 years. Vaccine timeliness was similar between children with and without epilepsy for MMR dose 1 and DTaP dose 4. CONCLUSIONS: Overall, this study suggests that children with epilepsy are not significantly under-vaccinated compared to their peers without epilepsy. As children with epilepsy are at a higher risk of complications from vaccine-preventable diseases, vaccination in children with epilepsy should be optimized, especially early in life, as these children may not be able to rely on herd protection.

Epilepsy and activity--a population-based study.

PURPOSE: To compare the activity profiles of a nationally representative sample of individuals with epilepsy compared to the general population. METHODS: The Canadian Community Health Survey is a cross-sectional survey that uses a stratified cluster sample design to obtain information on Canadians 12 years of age or older. Data on activity and energy expenditure, among those aged 12-39 years, were compared for those who reported having epilepsy and the remainder of the population. RESULTS: Of the 53,552 respondents, 341 reported having epilepsy. There was no difference in the monthly frequency of leisure physical activity of >15 min duration between those who did and did not have epilepsy. The daily energy expenditure related to leisure physical activity was also similar between the two groups. The choice of leisure activity was similar, but those with epilepsy were more likely to use walking as a leisure physical activity and were less likely to be involved in ice hockey, weight training, and home exercise. DISCUSSION: These results suggest that the negative attitudes toward restricting access to physical activity do not appear to be adversely affecting the leisure activity of Canadian youth and young adults with epilepsy.

Adverse Events Following Immunization Among Children With Epilepsy: A Self-Controlled Case Series.

BACKGROUND: In children with epilepsy, fever and illness are known triggers for seizure; therefore, clinicians and parents could be concerned that immunization-induced inflammation and fever could also trigger seizures. We sought to estimate the risk of emergency department (ED) visit or hospitalization for epilepsy/seizure and all causes after immunization in children younger than 7 years of age with epilepsy. METHODS: We conducted a self-controlled case series of children diagnosed with epilepsy before their 7th birthday and immunized from 2005 to 2015 in Ontario (population 14.2 million) and Manitoba (population 1.3 million), Canada, using administrative healthcare data. We estimated the age- and season-adjusted relative incidence (aRI) of epilepsy/seizure-related and all-cause ED visits/hospitalizations during various risk periods 0-28 days after inactivated and live immunizations versus a control period 35-83 days postimmunization. Estimates from each province were analyzed separately and then combined in a random-effects meta-analysis. RESULTS: The combined risk of epilepsy/seizure-related hospitalization/ED visit was increased 0-2 days after inactivated vaccines (aRI = 1.5, 95% confidence interval: 1.1-1.9) and 7-10 days after live vaccines (aRI = 1.9, 1.4-2.7). For all-cause ED visit/hospitalization, the combined aRI estimate was 0.9 (0.8-1.2) 0-2 days after inactivated vaccines and 1.3 (1.1-1.5) 7-10 days after live vaccines. CONCLUSIONS: The risk of epilepsy/seizure-related ED visit/hospitalization was modestly increased among children with epilepsy during peak periods of fever and inflammation following inactivated and live vaccines. These risks must be balanced against the risk of complications from vaccine-preventable diseases.

Canadian adolescents with migraine: impaired health-related quality of life.

The objective of this study was to determine the impact of migraine headaches on health-related quality of life among Canadian adolescents. The Canadian Community Health Survey (CCHS) collects information related to health status, health care utilization, and health determinants for the Canadian population. Analysis was limited to those adolescents 12-19 years of age residing in Manitoba. Respondents reported whether they had migraine and mood/anxiety disorders. Health-related quality of life was measured using the Short Form-36 survey, which covers 8 health concepts related to functional status, well-being, and overall health. Multivariate linear regression analysis was used to model each Short Form-36 scale against age (12-14 years vs 15-19 years), gender, migraine, and the presence of a mood or anxiety disorder. The CCHS was completed by 994 respondents; 9.3% (95% confidence interval, 7.3%-11.5%) reported a diagnosis of migraine. Reported migraine predicted both statistically (P < .0001) and clinically significantly lower health-related quality of life in all Short Form-36 domains except vitality and general health perceptions. Adolescents reporting a mood disorder (2.1%) scored significantly lower in 6 of 8 health-related quality of life domains, most pronounced for emotional role, general mental health, and social functioning. Canadian adolescents with migraine report clinically and statistically significant impairment in health-related quality of life compared to their peers, independent of psychiatric comorbidities.

Caffeine as an adjuvant to ibuprofen in treating childhood headaches.

In adults, caffeine has been shown to enhance the effectiveness of most analgesics, including ibuprofen. This double-blind cross-over pilot study evaluated the effect of ibuprofen and caffeine compared with ibuprofen and placebo in 12 children with headaches. Patients completed diaries for both headaches. Outcome measures included a five-faces severity scale, a measure of clinical disability, and a scale of pain severity. Comparison of the cumulative response scores revealed a trend toward a greater response to ibuprofen-caffeine treatment of headaches (P = 0.14, P = 0.09, and P = 0.07 for the three measures, respectively). Further larger studies are needed to confirm this effect and to identify potential responders.

Hemispheric polymicrogyria and neonatal seizures: a potentially life-threatening combination.

Polymicrogyria (PMG) is a heterogeneous malformation of cortical development characterized by excessive gyration and abnormal cortical lamination. Typically, bilateral forms have more severe developmental delay and early-onset epilepsy, but the full spectrum of severity remains ill-defined. We report two cases of right hemispheric PMG and neonatal-onset, drug-resistant seizures culminating in early death. Case 1 began having seizures on Day 1 of life that intensified in severity and proved resistant to numerous antiepileptic drugs. He underwent right functional hemispherectomy but died three weeks post-operatively due to ongoing seizures. Case 2 presented with seizures on Day 3 of life and required respiratory support for prolonged ictal apnoeas. Seizures were resistant to antiepileptic drugs and eventually led to respiratory arrest, once aggressive resuscitative measures were withdrawn. In both cases, seizures seemingly originated independently in both hemispheres. These cases represent a severe phenotype of unilateral hemispheric PMG with bilateral seizures.

Headaches in the pediatric population.

Headaches are an extremely common complaint encountered in the pediatric population. The headache history establishes the diagnosis in the vast majority and most importantly identifies features suggesting a secondary cause. The headache history outlined will aid in headache classification and screen for ominous causes. A comprehensive headache examination is aimed at excluding secondary causes. Headaches resulting from serious organic causes are virtually always associated with neurologic signs at the time of presentation. Investigations are not routinely required for pediatric headache, but neuroimaging should be strongly considered in children with an abnormal neurologic examination or history worrisome for intracranial pathology. The management approach for children with primary headaches should focus on reassurance and education. Developing an individualized therapeutic strategy requires knowledge of the child's headache-related disability and impact on quality of life. Treatment should begin with a nonpharmacologic approach, which influences lifelong prevention and management of headaches. Pharmacologic interventions target both acute symptomatic treatment and prophylactic medications. Preventative treatment may be beneficial when headaches result in significant disability and impaired quality of life. The limited available evidence for prophylactic treatment options is reviewed. Research into pediatric headache prevention and management remains a priority given the potential lifelong morbidity associated with headache.

Activity levels among adolescents with migraine.

Data was analyzed from the nationally representative Canadian Community Health Survey. A total of 17,549 adolescents reported whether they had "migraine headaches" (response rate 99.9%) and in what exercise activities they participated. Those with migraine reported more daily activity than migraine-free peers when corrected for age and sex. They were as likely to play contact sports but were more involved in other noncompetitive activities, such as walking (P<0.001), gardening and yard work (P=0.008), jogging and running (P=0.002), and fishing (P=0.03). Canadian adolescents with migraine are therefore overall more active than their peers without migraine.

When are episodes of loss of consciousness life-threatening?

Episodic loss of consciousness is common in the paediatric population. While the majority of cases are due to benign conditions, life-threatening causes must be promptly identified.The present paper presents a case of recurrent loss of consciousness associated with convulsive activity and subsequent cardiac arrest due to a rare inherited ion channel disease known as Brugada syndrome. The case highlights the challenges of distinguishing epilepsy from convulsive syncope and the potentially disastrous consequences of an undiagnosed cardiac etiology. The most important paediatric causes of paroxysmal loss of consciousness and their salient features are reviewed. Clinical features suggesting a cardiac cause are emphasized because early recognition of these cases is critical.

The prognosis of childhood headache: a 20-year follow-up.

BACKGROUND: Headaches affect most children and rank third among illness-related causes of school absenteeism. Although the short-term outcome for most children appears favorable, few studies have reported long-term outcome. OBJECTIVE: To evaluate the long-term prognosis of childhood headaches 20 years after initial diagnosis in a cohort of Atlantic Canadian children who had headaches diagnosed in 1983. METHODS: Ninety-five patients with headaches who consulted 1 of the authors in 1983 were previously studied in 1993. The 77 patients contacted in 1993 were followed up in 2003. A standardized interview protocol was used. RESULTS: Sixty (78%) of 77 patients responded (60 of the 95 of the original cohort). At 20-year follow-up, 16 (27%) were headache free, 20 (33%) had tension-type headaches, 10 (17%) had migraine, and 14 (23%) had migraine and tension-type headaches. Having more than 1 headache type was more prevalent than at diagnosis or initial follow-up (P<.001), and headache type varied across time. Of those with headaches at follow-up, 80% (35/44) described their headaches as moderate or severe, although an improvement in headaches was reported by 29 (66%). Tension-type headaches were more likely than migraine to remit (P<.04). Headache severity at diagnosis was predictive of headache outcome at 20 years. During the month before follow-up, nonprescription medications were used by 31 (70%) of those with ongoing headaches, and prescription medications were used by 6 (14%). However, 20 (45%) believed that nonpharmacological methods were most effective. Medication use increased during the 10 years since last follow-up. No patient used selective serotonin receptor agonists (triptans). CONCLUSIONS: Twenty years after diagnosis of pediatric headache, most patients continue to have headache, although the headache classification often changes across time. Most patients report moderate or severe headache and increasingly choose to care for their headaches pharmacologically.

Sublingual lorazepam in the treatment of familial paroxysmal nonkinesigenic dyskinesia.

Familial paroxysmal nonkinesigenic dyskinesia (Mount and Reback syndrome) is characterized by episodes of dystonia and chorea, which are precipitated by fatigue, emotional stress, alcohol, or foods. We report two children from a large kindred with this condition who responded to sublingual lorazepam.

Alice in wonderland and other migraine associated phenomena-evolution over 30 years after headache diagnosis.

BACKGROUND: The International Classification of Headache Disorders-III beta includes a number of episodic syndromes associated with migraine. Those who treat pediatric headaches are aware of a number of other phenomena (such as the Alice in Wonderland syndrome) which are thought to occur as precursors of migraine. There is no available data on the course of these phenomena over the decades following childhood headache diagnosis. METHODS: Patients who were observed by one of the authors in 1983 were contacted by telephone in 1993, 2003, and 2013. Details were gathered regarding the presence and characteristics of ongoing headaches and about the presence of sleepwalking, motion sickness, and distortions of either time or space perceptions. RESULTS: Twenty-eight patients were monitored in 1993, 2003, and 2013. Ongoing headaches were reported by 71%. Sleepwalking was only present in one patient in 2013. More than a third still complained of motion sickness, and more than one quarter still experienced distortions of time. Distortions of space were still reported by nearly 20%. Reporting any of these phenomena was not consistent over time, with some patients reporting distortions for the first time in adulthood. There was no clear correlation with migraine, and patients with tension-type headaches also reported the phenomena. CONCLUSIONS: Motion sickness and distortions of both space and time persist into the fifth decade for many patients initially observed with headaches in childhood. The correlation with migraine is less clear than previously thought.

The prognosis of pediatric headaches--a 30-year follow-up study.

BACKGROUND: Although headaches in childhood are common, there are few data available on their long-term prognosis. We have monitored a group of patients since diagnosis in 1983. METHODS: Patients who were part of the 20-year follow-up study in 2003 were contacted, and data were collected using a standardized telephone interview. Details of headache characteristics and identified precipitants and alleviating factors were gathered. The most effective means of controlling the headaches were also recorded. RESULTS: Follow-up was achieved for 28 of 60 patients (47%). Over the 30 years since diagnosis, eight patients (29%) reported a complete resolution of headaches, including three whose headaches resolved between the 20- and 30-year follow-up studies. The type of headache varied over the 30-year time interval with only three patients maintaining the same headache type at all four time periods of 1983, 1993, 2003, and 2013. Only one patient used prescription medication as the primary method for controlling headaches. The most commonly used intervention was nonprescription analgesia, self-relaxation and/or hypnosis, and precipitant avoidance. CONCLUSIONS: Headaches persist in approximately 70% of children 30 years after diagnosis. Encouraging children to manage their headaches with simple analgesia and precipitant avoidance appears to have long-term benefits.

What do patients and families want from a child neurology consultation?

Understanding what patients and their parents want is essential to plan appropriate patient-centered care. Questionnaires were distributed to 500 consecutive children and parents seen for their first pediatric neurology consultation. Both patients and their families answered questions about their expectations of the consultation, their level of worry, and the Penn State Worry Questionnaire. The 5 most important issues for the parents were to get information, to work with the doctor to manage the problem, to have questions answered, to find out what was wrong, and to discuss the impact on the child's life. The children had very similar priorities. The 5 least important concerns for parents were to get a prescription, blood tests, to talk to others with similar problems, to get a radiograph/computed tomography/magnetic resonance imaging (MRI) and to be told nothing is wrong. The pediatric neurologists did well in anticipating these priorities but had more difficulty appreciating parent and patient level of worry.