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Biomedical subjects

Elaine Wirrell

Publications and source records attributed to Elaine Wirrell.

At least 19 recordsLinked to original sources
PubMed2006

Sulthiame therapy for continuous spike and wave in slow-wave sleep.

Corticosteroids are often considered as the first therapeutic choice in children with continuous spike and wave in slow-wave sleep on electroencephalogram; however, they are associated with significant adverse effects. "Idiopathic" forms of continuous spike and wave in slow-wave sleep may represent the severe end of the spectrum of benign rolandic epilepsy of childhood. This report describes a 5-year-old male with language delay who presented with a single focal-onset, nocturnal seizure and had continuous spike and wave in slow-wave sleep on electroencephalography. After 1 month of sulthiame therapy, his electroencephalographic abnormality had resolved, and his language development improved.

Child, Preschool↗
PubMed2006

A pediatric case of reversible segmental cerebral vasoconstriction.

BACKGROUND: Reversible segmental cerebral vasoconstriction (RSCV) is a recognizable clinical and radiographic syndrome consisting of thunderclap headache with or without focal neurological symptoms combined with reversible segmental vasoconstriction of proximal cerebral blood vessels. METHODS: We report a case of reversible segmental cerebral vasoconstriction in a child. RESULTS: A healthy 13-year-old boy experienced the sudden onset of a severe, diffuse headache upon surfacing from a deep dive in a swimming pool. Severity was maximal at the onset and improved over several hours. The same headache recurred three times over the next four days and a low baseline headache persisted throughout. Vomiting occurred once and mild photo/osmophobia were reported but throbbing, aura, or autonomic symptoms were absent. Focal neurological signs or symptoms were absent and he denied previous history of headaches, medications, drugs, or trauma. Two normal CT scans were performed within hours of separate headaches. Cerebrospinal fluid study on day 5 was bloody with no xanthochromia. MRI/MRA/MRV of the brain and vasculitic work-up were normal. Cerebral angiography on day 6 demonstrated smooth narrowing of multiple proximal cerebral vessels including supraclinoid internal carotid artery (ICA), M1, and A1 on the right and M1 on the left. By ten days, the patient's headaches had resolved and repeat angiography was normal. CONCLUSION: RSCV should be considered in a child with thunderclap headache.

Adolescent↗
PubMed2006

How do teens view the physical and social impact of asthma compared to other chronic diseases?

We surveyed cognitively normal teens with and without chronic illness regarding the perceived physical and social impact of various chronic diseases including asthma. The overall physical impact of asthma was perceived equivalently to diabetes and arthritis, but less than epilepsy, Down's syndrome, leukemia, and human immunodeficiency virus infection. However, asthma was rated to more commonly cause physical disability (p < 0.001) and restrict activities (p < 0.0005). The social impact of asthma was perceived equivalently to diabetes, but more favorably than the other chronic diseases surveyed. Specifically, teens with asthma were perceived as having fewer behavior problems, being more honest, popular, and fun to be around, but less adept at sports. Only 6 of 149 (4%) teens surveyed expressed any degree of reluctance to befriend peers with asthma.

Adolescent↗
PubMed2006

Physical activity in children/teens with epilepsy compared with that in their siblings without epilepsy.

PURPOSE: To determine (a) whether children and teens with epilepsy participate in less physical activity and have higher body mass index (BMI) percentiles for age than do their siblings without epilepsy; and (b) what epilepsy-specific factors limit their participation. METHODS: Patients 5-17 years, with a >or=3 month history of epilepsy, a development quotient >or=80, no major motor or sensory impairments, and at least one sibling without epilepsy in a similar age range, were identified from the Neurology Clinic database or at the time of clinic visit. Parents completed a questionnaire regarding sedentary activities and group, individual, and total sports activities. Children aged 11-15 years also completed the physical activity portion of the Health Behavior in School Aged Children questionnaire. Clinic charts were reviewed for seizure type, etiology, frequency, duration of epilepsy, and number of antiepileptic drugs (AEDs) ever taken. RESULTS: Teens with epilepsy participated in fewer group and total sports activities than did controls and were more likely to be potentially overweight or overweight. Receiving three or more AEDs in the past showed a significant negative correlation with sports participation. Although a trend was noted for those with higher seizure frequency to be less active, no other epilepsy-specific factors or prior seizures or seizure-related injury during a sports event correlated with participation in physical activity. CONCLUSIONS: Programs that promote exercise in adolescents with epilepsy should be encouraged to improve their physical, psychological, and social well-being.

Adolescent↗
PubMed2006

Sleep disturbance in children with migraine.

The objectives of this study were to determine if (1) children with migraine experience greater sleep disturbances than their siblings, (2) those with more severe migraine have greater levels of sleep disruption, and (3) these sleep disturbances lead to greater behavioral problems and more missed school. Children aged 6 to 18 years with a diagnosis of migraine for > 6 months, who had at least one sibling without migraine in the same age range, were identified through our neurology clinic database or at the time of the clinic visit. Parents completed the (1) demographic, general health, and migraine information questionnaire; (2) Child Sleep Habits Questionnaire; and (3) Behavior Assessment System for Children: Second Edition (BASC-2) Parent Rating Scales for each child. Cases with migraine had higher total sleep (P < .02), sleep delay (P < .03), and daytime sleepiness scores (P < .001) than controls. Cases with more severe migraines had higher total sleep (P < .01) and sleep duration scores (P < .03) than those with milder headaches. In cases, higher total sleep scores predicted greater behavior problems on all four composite scales on the Behavior Assessment System for Children: Second Edition (Externalizing Problems, P < .05; Internalizing Problems, P < .005; Behavior Systems Index, P < .003; and Adaptability Skills, P < .006). We conclude that children with migraine are prone to greater sleep and behavioral disturbances than children without headache. Sleep disorders should be routinely queried and appropriate advice on sleep hygiene provided.

Activities of Daily Living↗
PubMed2006

Adolescents' perception of epilepsy compared with other chronic diseases: "through a teenager's eyes".

Adolescent perception of physical and social impact of chronic illness was assessed to determine (1) if there is greater prejudice toward epilepsy than other chronic disease and (2) if adolescents with chronic disease have less prejudice toward similarly affected peers with all types of chronic disease or just their specific chronic disease. Cognitively normal teens aged 13 to 18 years without chronic disease (n = 41) and with epilepsy (n = 32), asthma (n = 38), diabetes (n = 21), and migraine (n = 17) were interviewed in the outpatient clinics of a tertiary care pediatric center regarding their perceptions of the physical and social impact of eight chronic diseases (epilepsy, asthma, diabetes, Down syndrome, arthritis, migraine, leukemia, human immunodeficiency virus [HIV] infection). Epilepsy was perceived to have a more adverse physical impact than all chronic illnesses except Down syndrome. The perception was that it more frequently caused mental handicap, injured the afflicted individual and bystanders, and led to death. Epilepsy was also perceived to have a more negative social impact, particularly on behavior, honesty, popularity, adeptness at sports, and fun. Significantly more adolescents expressed reluctance to befriend peers with epilepsy, both from their own and their perceived parental perspectives. Having a chronic disease did not generally alter the adolescents' perceptions of peers with chronic disease. However, cases with epilepsy ranked this disease to have less social impact than teens with other chronic diseases. In conclusion, adolescents consider epilepsy to have a greater physical and social impact than most chronic diseases. Educational efforts should focus on the "normality" of most persons with epilepsy and emphasize the low risk of injury when proper first aid is followed.

Adolescent↗
PubMed2005

Homozygous deletion of the very low density lipoprotein receptor gene causes autosomal recessive cerebellar hypoplasia with cerebral gyral simplification.

An autosomal recessive syndrome of nonprogressive cerebellar ataxia and mental retardation is associated with inferior cerebellar hypoplasia and mild cerebral gyral simplification in the Hutterite population. An identity-by-descent mapping approach using eight patients from three interrelated Hutterite families localized the gene for this syndrome to chromosome region 9p24. Haplotype analysis identified familial and ancestral recombination events and refined the minimal region to a 2-Mb interval between markers D9S129 and D9S1871. A 199-kb homozygous deletion encompassing the entire very low density lipoprotein receptor (VLDLR) gene was present in all affected individuals. VLDLR is part of the reelin signaling pathway, which guides neuroblast migration in the cerebral cortex and cerebellum. To our knowledge, this syndrome represents the first human lipoprotein receptor malformation syndrome and the second human disease associated with a reelin pathway defect.

Base Sequence↗
PubMed2005

Autosomal recessive cerebellar hypoplasia in the Hutterite population.

Cerebellar hypoplasia is a rare malformation caused by a variety of etiologies. It usually manifests clinically as non-progressive cerebellar ataxia with or without mental retardation. We further characterize a syndrome of autosomal recessive cerebellar hypoplasia in the Hutterite population, referred to as dysequilibrium syndrome (DES). We reviewed 12 patients (eight females, four males; age range 4 to 33 y) with this syndrome. Patients were examined and underwent a standard set of investigations to characterize better the clinical features, natural history, and neuroimaging of this syndrome. DES is an autosomal recessive disorder with distinct clinical features including global developmental delay, late ambulation (after age 6 y), truncal ataxia, and a static clinical course. Neuroimaging is characterized by hypoplasia of the inferior portion of the cerebellar hemispheres and vermis, and mild simplification of cortical gyri.

Adolescent↗
PubMed2005

Sleep disturbances in children with epilepsy compared with their nearest-aged siblings.

The aim of the study was to compare sleep patterns in children with epilepsy with those of their non-epileptic siblings and to determine which epilepsy-specific factors predict greater sleep disturbance. We conducted a case-control study of 55 children with epilepsy (mean age 10y, range 4 to 16y; 27 males, 28 females) and their nearest-aged non-epileptic sibling (mean age 10y, range 4 to 18y; 26 males, 29 females). Epilepsy was idiopathic generalized in eight children (15%), symptomatic generalized in seven (13%), and focal in 40 (73%); the mean duration was 5 years 8 months. Parents or caregivers completed the Sleep Behavior Questionnaire (SBQ) and Child Behavior Checklist (CBCL) for patients and controls, and the Quality of Life in Childhood Epilepsy (QOLCE) for patients. Patients had a higher (more adverse) Total Sleep score (p<0.001) and scored worse than controls on nearly all subscales of the SBQ. In patients, higher Total Sleep scores were correlated with higher scores on the Withdrawn, Somatic complaints, Social problems, and Attention subscales of the CBCL, and significantly lower Total Quality of Life Scores. Refractory epilepsy, mental retardation, and remote symptomatic etiology predicted greater sleep problems in those with epilepsy. We conclude that children with epilepsy in this current study had significantly greater sleep problems than their non-epileptic siblings.

Adolescent↗
PubMed2005

The epileptic encephalopathies of infancy and childhood.

The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usually result in intractable epilepsy. While some syndromes are relatively easily diagnosed early in their course, others take time to evolve, hampering an early, confident diagnosis. Epileptic encephalopathies are associated with slowing of cognitive function and evolution of severe behavioral disorders, which are often more distressing to families than the epilepsy. While an underlying etiology may explain some of this co-morbidity, many children have no identifiable etiology found for their seizures. In these "idiopathic" cases, recurrent subtle seizures, frequent epileptiform discharge and non-convulsive status epilepticus probably all play a role in deterioration of cognitive function and evolution of behavior disorders. This paper will review the most common epileptic encephalopathy syndromes, discuss the cognitive and behavioral co-morbidities and review current therapeutic options.

Adrenocorticotropic Hormone↗
PubMed2005

Use of complementary and alternative medical therapies in a pediatric neurology clinic.

BACKGROUND: Complementary and alternative medicine (CAM) is increasingly used in adults and children. Studies on CAM in neurological disorders have focused on the adult population and its use among pediatric neurology patients has not been well characterized. OBJECTIVES: The purpose of this study was: 1) To characterize the prevalence of CAM in pediatric neurology patients; 2) To determine the perceived effectiveness of CAM in these children; 3) To compare the cost of CAM with conventional therapies; and 4) To describe caregiver or patient-related variables associated with the use of CAM. METHODS: This was a cross-sectional survey of patients and families attending the Alberta Children's Hospital neurology clinic between February and May 2004. Patients were considered eligible if they were between two and 18 years of age and had a known history of neurological disorders. Caregivers completed several self-administered questionnaires regarding their socio-demographic profile, their child's neurological illness, and their experience with CAM. Caregivers also rated their child's quality of life using the Pediatric Quality of Life Inventory. RESULTS: One hundred and five of 228 (46%) families completed the survey. The mean age of the neurology patients was 9.8 +/- 4.5 years. Forty-six (44%) out of 105 patients received one or more types of CAM, with the most common types being chiropractic manipulations (15%), dietary therapy (12%), herbal remedies (8%), homeopathy (8%), and prayer/faith healing (8%). Caregivers' sociodemographic variables or pediatric health-related quality of life were not significantly associated with the use of CAM. Fifty-nine percent of CAM users reported benefits, and only one patient experienced side effects. There was no significant difference in the total median cost of CAM compared to conventional therapies (31.70 dollars vs. 50.00 dollars per month). Caregivers' personal experience or success stories from friends and media were common reasons for trying CAM. CONCLUSIONS: The use of CAM was common among pediatric neurology patients. Over half of the families reported benefits with CAM, and side effects were perceived to be few. Physicians should initiate discussion on CAM during clinic visits so that the families and patients can make informed decisions about using CAM. Further studies should address the specific role of CAM in children with neurological disorders, and to determine the potential interactions between CAM and conventional therapies in these patients.

Adolescent↗
PubMed2005

Acute necrotizing encephalopathy in caucasian children: two cases and review of the literature.

Acute necrotizing encephalopathy is a fulminant neurologic disease seen predominantly in Japan and Taiwan. We present two cases diagnosed at a Canadian center within the same year in Caucasian children. Both were previously well, developed an acute viral illness with fever and vomiting, and progressed to brain death within 2 to 4 days. Neuroimaging and postmortem examination demonstrated the unique features of bilateral and severe necrosis of deep gray- and subcortical white-matter structures. The first case was associated with extensive, but transient, hepatic involvement, recent varicella and rotavirus infections, and detailed metabolic studies, including mitochondrial functional analysis, were normal. The second case tested positive for influenza A infection, whereas evidence of liver damage was lacking. Both children demonstrated early lymphopenia and myocardial necrosis, two features not previously associated with acute necrotizing encephalopathy. These cases are unique in their occurrence in non-Japanese children and are among the first published reports in Canada.

Brain↗
PubMed2005

Where do families of children with epilepsy obtain their information?

A structured interview of 84 families of children with epilepsy followed through the neurology clinic of a tertiary care children's hospital was conducted to assess the epilepsy-specific information sources accessed and the perceived accuracy of these sources. Families accessed a mean of 3.5 sources from or specifically recommended by the clinic or family doctor and 4.1 sources outside these areas. Families of children with intractable epilepsy and higher-educated parents, but not those of higher socioeconomic status, consulted more extensively. The perceived accuracy of information rated highest for clinic-recommended Internet sites (100%), the clinic nurse (97%), and the neurologist (93%). Sources external to the clinic had variable ratings; those with the greatest perceived accuracy included other Internet sites or family members within the medical profession (85% for both) and lay organizations (84%). Friends within the medical profession, other families, and complementary health care providers also ranked highly. Recommendation of sites and books by epilepsy clinics is more helpful than general handouts.

Adolescent↗