Search PubMed⌕ Search

Biomedical subjects

J M Dooley

Publications and source records attributed to J M Dooley.

At least 37 records · Page 2Linked to original sources
PubMed1999

Topiramate in intractable childhood onset epilepsy--a cautionary note.

OBJECTIVES: To study the effectiveness and safety of topiramate in clinical practice, for a group of patients with childhood onset epilepsy. METHODS: All patients treated with topiramate at the three study centers between November 1995 and December 31, 1997 were analyzed retrospectively, using a standardized study protocol. Data were gathered on demographic features, seizures response and medication related adverse events. RESULTS: Eighty-seven patients were treated with topiramate. Over 90% seizure reduction was achieved in 8 (9%) patients, 50%-90% in 21 (24%), < 50% in 54 (62%) patients. Four patients (5%) had a deterioration in seizure control. Adverse events required topiramate discontinuation in 36 (41%). Of these 27 (31%) complained of unacceptable cognitive dulling. The rate of dose escalation and final dose in mg/kg were similar in those who remained on topiramate and those who were intolerant because of cognitive side effects. CONCLUSIONS: Although topiramate resulted in > 50% seizure reduction in 29 (33%) of this group of patients with difficult epilepsy, its usefulness was limited by a high incidence of adverse effects. Adverse events prevented ongoing therapy for 36 (41%) and cognitive dulling resulted in topiramate discontinuation by 27 (31%) of the group.

Adolescent↗
PubMed1999

Lamotrigine-associated rash: risk/benefit considerations in adults and children.

PURPOSE: Lamotrigine (LTG) is an antiepileptic drug (AED) recently released in several countries. It is effective for a variety of seizure types in adults and children both as an add-on agent and in monotherapy, and is generally well tolerated. This report reviews the apparent risk factors for rash associated with LTG to determine whether and how the risk of serious rash can be minimized in practice. METHODS: The panel of experts reviewed all published and unpublished data related to the incidence and risk factors for serious rash with LTG. RESULTS: An allergic skin reaction occurs in approximately 10% of patients, usually in the first 8 weeks. Rashes leading to hospitalization, including Stevens-Johnson syndrome and hypersensitivity syndrome, occurred in approximately one of 300 adults and one of 100 children in clinical trials and appeared to be increased with overrapid titration when starting therapy and with concurrent valproate (VPA). CONCLUSIONS: Recommendations are made for both minimizing the likelihood of serious rash and for management of rash in patients taking LTG. Risk of serious rash may possibly be lessened by strict adherence to manufacturer's dosing guidelines, particularly in patients who are at higher risk: those on concurrent VPA and in the pediatric population.

Adult↗
PubMed1999

Parent perceptions of symptom severity in Tourette's syndrome.

The families of 66 consecutive children with Tourette's syndrome were surveyed for their perception of symptom significance using a questionnaire. Families considered attention deficit and learning difficulties to be most significant, while motor and vocal tics were least important. When present, episodic rage was the most impairing symptom. Physicians must be aware of the significance of these comorbid symptoms for patients with Tourette's syndrome.

Adolescent↗
PubMed1997

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.

Adolescent↗
PubMed1997

If a first antiepileptic drug fails to control a child's epilepsy, what are the chances of success with the next drug?

OBJECTIVE: This study was carried out to determine how often a child's epilepsy is controlled and remits if a first antiepileptic drug (AED) fails to control seizures. STUDY DESIGN: We used the Nova Scotia population-based epilepsy study, which identified children between 1977 and 1985 who had two or more unprovoked seizures without progressive cause and followed them up for at least 4 years. Seizure types were partial, primary, and secondarily generalized (excluding absence seizures). The study documented success or failure of the initial AED in the first year of treatment, as well as long-term seizure control and remission. RESULTS: The number of eligible children was 417, with an average follow-up period of 8 years. The initial prescribed AEDs were phenobarbital (48%), carbamazepine (38%), and phenytoin (11%). Overall, 345 (83%) children received only one AED in the first year of treatment; 61% became free of seizures and no longer required AED treatment at the end of follow-up (remission). Only 4% of those treated with a single AED during the first year later experienced intractable epilepsy. In contrast, 72 of 417 (17%) had inadequate seizure control with their first AED and received a second AED, with only 42% having complete remission of their epilepsy. The 72 children in whom seizures were not controlled with the first AED were more likely to have neurologic deficits (p = 0.01) and complex partial seizures (p = 0.01), and 29% had intractable epilepsy (p < 0.0001). CONCLUSIONS: If the first AED is not efficacious, the outcome is less favorable, although many children will have remission of their epilepsy. Invasive or complex treatments for epilepsy with partial and generalized tonic-clonic seizures should not be used until at least two AEDs have failed to control seizures.

Age of Onset↗
PubMed1996

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.

Accidental Falls↗
PubMed1996

Incidence of epilepsy in childhood and adolescence: a population-based study in Nova Scotia from 1977 to 1985.

Data from a regional EEG laboratory allowed us to identify almost all children in Nova Scotia (population 85,000) with one or more unprovoked, afebrile seizures from 1977 through 1985. We then reviewed hospital and pediatric neurology physician charts to limit cases to those with two or more definite afebrile seizures between the ages of 1 month and 16 years. In all, 693 children developed epilepsy: typical childhood absence seizures (AS) (97), either generalized tonic-clonic (GTCs) or partial seizures either secondarily generalized or not (511), and other generalized seizure types, including infantile spasms (IS) as well as myoclonic, akinetic, tonic, and atypical AS (85). The incidence of epilepsy was 118 in 100,000 for children aged less than 1 year, 48 in 100,000 for those aged 1-5 years, 43 in 100,000 for those aged 6-10 years, and 21 in 100,000 for those aged 11-15 years. The incidence for each year of age between 1 and 10 years was remarkably constant (mean 46 in 100,000 +/- 7 SD). Comparison of the incidence rates showed significant differences for those aged less than one year as compared with all others, and for those aged greater than 10 years as compared with those aged 1-10 years. We conclude that the incidence of epilepsy is highest in the first year of life, plateaus in early childhood, and decreases markedly after age 10 years. The overall incidence of epilepsy in childhood is lower than that reported in previous studies.

Adolescent↗
PubMed1996

Will a critical level of hyperventilation-induced hypocapnia always induce an absence seizure?

We wished to determine if the degree of hypocapnia correlates with increased frequency of absence seizures and if there is a critical pCO2 at which absence seizures are reliably provoked. Twelve untreated children with newly diagnosed absence epilepsy were continuously monitored by EEG and end-expiratory CO2 recording during quiet respiration and hyperventilation (to absence seizure or exhaustion) while breathing four gas mixtures: (a) room air, (b) 100% O2, (c) 4% CO2 in room air, or (d) 4% CO2 + 96% O2). In quiet respiration, a reduction in number of spike and wave bursts and total seconds of spike and wave was noted in children breathing supplemental CO2 (gases c and d vs. gases a and b), p < 0.05. Supplemental O2 had no effect. Eight subjects had absence seizures elicited with each trial of hyperventilation. All subjects had their own critical pCO2, ranging from 19 to 28 mmHg. Three children had no seizures, two despite hypocapnia to pCO2 of 19 and 21 and 1 who achieved a pCO2 of only 25. In 1, absence seizures were provoked in only six of nine hyperventilation trials to pCO2 of 17-23. In 67% of subjects, absence seizures were reliably provoked by hypocapnia. Critical pCO2 varied among children with absence. Determination of whether variation in sensitivity to hypocapnia may be helpful in determining response to antiepileptic drugs (AEDs) or remission of seizures will require further study.

Air↗
PubMed1996

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.

Child, Preschool↗
PubMed1995

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.

Age of Onset↗
PubMed1994

Correlation between electroretinogram findings and molecular analysis in the Duchenne muscular dystrophy phenotype.

Fifteen consecutive patients with the Duchenne muscular dystrophy (DMD) phenotype were studied. Each patient was asked to undergo an ophthalmic examination, an electroretinogram (ERG), and to donate a blood sample for molecular diagnosis. All 15 patients had a normal ophthalmic examination. Electroretinography was successful in 14/15 patients. The ERG tracings were normal in seven patients, abnormal in seven, and unreliable in one. Blood for molecular analysis was obtained in 12/15 patients. In the seven patients with a normal ERG, five underwent molecular analysis, and in these five no deletion was detected in the dystrophin gene. In the seven patients with an abnormal ERG, six had molecular analysis available, and all six were found to have a deletion. These results suggest that patients with a classic DMD phenotype are genetically heterogeneous, and that this heterogeneity is reflected in the ERG.

Adolescent↗
PubMed1994

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.

Adolescent↗
PubMed1994

Duchenne muscular dystrophy: negative scotopic bright-flash electroretinogram but not congenital stationary night blindness.

Patients with Duchenne muscular dystrophy (DMD) have recently been reported to have an abnormal scotopic electroretinogram (ERG) showing weak rod-related responses along with a negative configuration of the bright-flash response, which has been described as being similar to the one in congenital stationary night blindness (CSNB). We compared qualitatively and quantitatively the ERGs of 6 subjects with DMD, 10 subjects with the complete form of CSNB (cCSNB), 13 subjects with the incomplete form of CSNB (iCSNB) and 1 subject with complex glycerol kinase deficiency (CGKD). The rod-related activity and the bright-flash responses were abnormal and similar in all four groups. The cone-related activity, however, was within normal limits only in the DMD group; the b-wave was subnormal in CGKD, truncated in cCSNB and nearly absent in iCSNB. The electrophysiologic signature in DMD clearly distinguishes the retinal function of these patients from any other retinal condition so far described.

Adolescent↗
PubMed1994

Duchenne muscular dystrophy: negative scotopic bright-flash electroretinogram and normal dark adaptation.

Recent reports indicate that most subjects with Duchenne muscular dystrophy (DMD) have a negative scotopic bright-flash electroretinogram (ERG). We performed dark adaptometry in seven patients with the DMD phenotype. Three patients had the negative bright-flash response combined with severely reduced rod-related activity; their mean psychophysical dark-adapted threshold was -5.5 +/- 0.3 (normally -5.4 +/- 0.3) cd.m-2. Two of the three patients were able to sit through the complete test: the profile of their dark adaptation curve was normal. The four other patients had a normal scotopic ERG, and their mean psychophysical dark-adapted threshold was -5.3 +/- 0.3 cd.m-2. One of the four was able to complete the entire procedure, and a normal profile was found. In patients with DMD the dark adaptation measurements are normal despite abnormalities in rod-related ERG activity.

Adolescent↗
PubMed1993

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.

Adolescent↗
PubMed1992

Aspartame exacerbates EEG spike-wave discharge in children with generalized absence epilepsy: a double-blind controlled study.

There are anecdotal reports of increased seizures in humans after ingestion of aspartame. We studied 10 children with newly diagnosed but untreated generalized absence seizures. Ambulatory cassette recording of EEG allowed quantification of numbers and length of spike-wave discharges in a double-blind study on two consecutive days. On one day the children received 40 mg/kg aspartame and on the other day, a sucrose-sweetened drink. Baseline EEG was the same before aspartame and sucrose. Following aspartame compared with sucrose, the number of spike-wave discharges per hour and mean length of spike-wave discharges increased but not to a statistically significant degree. However, the total duration of spike-wave discharge per hour was significantly increased after aspartame (p = 0.028), with a 40% +/- 17% (SEM) increase in the number of seconds per hour of EEG recording that the children spent in spike-wave discharge. Aspartame appears to exacerbate the amount of EEG spike wave in children with absence seizures. Further studies are needed to establish if this effect occurs at lower doses and in other seizure types.

Adolescent↗
PubMed1991

Peer problems in Tourette's disorder.

To explore the social adjustment and peer relationships of children with Tourette's disorder, 29 patients with mild to moderate Tourette's disorder were studied. Children underwent neuropsychological testing. The patients completed self-esteem scales and their parents and teachers completed behavior rating scales. Peer relationships were examined with the Pupil Evaluation Inventory, which is a sociometric questionnaire completed by the child's classmates and provides measures of aggression, withdrawal, and likability. As a group, Tourette's disorder patients were significantly more withdrawn, more aggressive, and less popular than their classmates. Thirty-five percent of the children with Tourette's disorder received the lowest rating in the class on one or more of the Pupil Evaluation Inventory factors. These social problems were not predicted by the frequency or duration of tics. A clinical diagnosis of attention-deficit hyperactivity disorder and teachers' ratings on the summary scale of the Child Behavior Checklist and the Pupil Evaluation Inventory did predict poor adjustment. It is concluded that social adjustment is a major difficulty for many children with Tourette's disorder, irrespective of tic severity.

Child↗