Wendyl D'Souza

Evaluating the Utility of Inpatient Video‐EEG Monitoring

Summary:  Purpose: Inpatient video‐EEG monitoring (VEM) is widely used for the diagnosis, seizure classification, and presurgical evaluation of patients with seizure disorders. It is resource intensive and relatively expensive, so its utility continues to be debated. Few studies have specifically evaluated the utility of inpatient VEM in altering diagnosis or management of patients with seizure disorders. We sought to assess the proportion of patients for whom the preadmission diagnosis and management were altered after inpatient VEM of patients admitted for diagnostic and presurgical evaluation of seizure disorders.

Occupational asthma in New Zealanders: a population based study.

OBJECTIVES: To examine the effect of occupation on respiratory symptoms in a randomly selected adult population aged 20-44 years. METHODS: It is based on the phase II sampling of the New Zealand part of the European Community respiratory health survey. 1609 people (63.9% response rate) completed a detailed respiratory questionnaire. Of those responding, 1174 (73%) underwent skin tests and 1126 (70%) attended to undergo methacholine bronchial challenge. Current occupation was recorded and a previous occupation was also recorded if it had led to respiratory problems. 21 occupational groups were used for analysis for the five definitions of asthma wheezing in the previous 12 months; symptoms related to asthma; bronchial hyperresponsiveness (BHR); BHR with wheezing in the previous 12 months; and BHR with symptoms related to asthma. RESULTS: Prevalence odds ratios (ORs) were significantly increased for farmers and farm workers (OR 4.16, 95% confidence interval (95% CI) 1.33 to 13.1 for the combination of wheezing and BHR). Increased risks of prevalence of asthma were also found for laboratory technicians, food processors (other than bakers), chemical workers, and plastic and rubber workers. Workers had also been divided into high and low risk exposure categories according to relevant publications. The prevalence of wheezing was greater in the high risk group (OR 1.57, 95% CI 0.83 to 2.95) than in the low risk group. Atopy was associated with asthma, but the prevalence of atopy did not differ significantly between occupational exposure groups. The attributable risk of wheezing that occurred after the age of 15 years and that was estimated to be due to occupational exposure (based on the defined high risk group) was 1.9%, but this increased to 3.1% when farmers and food processors (other than bakers) were also included in the high risk group. CONCLUSIONS: This population based study has identified certain occupations significantly associated with combinations of asthmatic symptoms and BHR.

Stereotypy of psychogenic nonepileptic seizures: Insights from video‐EEG monitoring

Purpose:  To systematically study the semiology of psychogenic nonepileptic seizures (PNES) captured by video–electroencephalography (EEG) monitoring (VEM) and categorize the typical patterns observed.

Family size, infections, and asthma prevalence in New Zealand children.

We conducted a prevalence case-control study to investigate the relation between family composition, infection, and development of asthma at age 7-9 years. Potential cases (399) and controls (398) were selected from the Wellington, NZ, arm of the International Study of Asthma and Allergies in Childhood, a population-based prevalence study. Further screening questions restricted cases to children with a diagnosis of asthma and current medication use (N = 233) and restricted controls to children without a history of wheezing and no diagnosis of asthma (N = 241). After controlling for confounders (including infections, atopy, and socioeconomic status), family size was strongly related to asthma. Having no siblings [prevalence odds ratio (POR) = 2.51; 95% confidence interval (CI) = 1.05-6.01] or one sibling (POR = 1.86; 95% CI = 1.14-3.03) was associated with an increased risk of asthma compared with having more than one sibling. Parent-reported rubeola infection (and possibly other similar viral exanthems) was independently associated with a decreased risk of asthma (POR = 0.48; 95% CI = 0.27-0.83), but reported pertussis infection (POR = 1.57; 95% CI = 0.58-4.24) and day care attendance in the first year of life (POR = 1.81; 95% CI = 0.93-3.51) were not strongly associated with increased risks of asthma.

The Liverpool Adverse Events Profile: Relation to AED Use and Mood

Summary:  Purpose: The Liverpool Adverse Events Profile (LAEP) is used as a systematic measure of adverse effects from antiepileptic drugs (AEDs). This study evaluated LAEP in newly diagnosed seizure patients, and examined the relation between LAEP, anxiety, and depression.

Small CGG repeat expansion alleles of FMR1 gene are associated with parkinsonism

Fragile X‐associated tremor/ataxia syndrome (FXTAS) affects older males carrying premutation, that is, expansions of the CGG repeat (in the 55–200 range), in the FMR1 gene. The neurological changes are linked to the excessive FMR1 messenger RNA (mRNA), becoming toxic through a ‘gain‐of‐function’. Because elevated levels of this mRNA are also found in carriers of the smaller expansion (grey zone) alleles, ranging from 40 to 54 CGGs, we tested for a possible role of these alleles in the origin of movement disorders associated with tremor.

A case-control study of risk factors for asthma in New Zealand children.

Objective : As in other English‐speaking countries, asthma is a major and increasing health problem in New Zealand. This study examined the risk factors for asthma in children aged 7–9.

Focal abnormalities in idiopathic generalized epilepsy: A critical review of the literature

Conventionally, epilepsy is dichotomized into distinct “focal” and “generalized” categories. However, many studies have reported so‐called focal features among patients with idiopathic generalized epilepsy (IGE) in the domains of semiology, electroencephalography, neuropsychology, neuropathology, and neuroimaging. We sought to review such features and clinical implications. A Web of Science database search was conducted to identify relevant publications. Our search yielded 145 papers describing focal features involving different domains in IGE, with 117 papers analyzed after excluding abstracts and case reports. Focal semiologic features are commonly seen in IGE. There are conflicting data from studies in the domains of electroencephalography, neuroimaging, and neuropathology. Studies on neuropsychology are suggestive of frontal lobe functional deficits in juvenile myoclonic epilepsy. Most advanced neuroimaging studies demonstrate the involvement of both the thalamus and the cortex during generalized spike‐wave discharges (GSWDs). A few electroencephalographic and neuroimaging studies indicate that the cortex precedes the thalamus at the onset of GSWD. Focal features may contribute to misdiagnosis of IGE as focal epilepsy. However there are methodologic limitations in the studies that affect the results.

Cognitive functioning in idiopathic generalised epilepsies: A systematic review and meta-analysis

Cognitive function in idiopathic generalised epilepsies (IGE) is of increasing research attention. Current research seeks to understand phenotypic traits associated with this most common group of inherited epilepsies and evaluate educational and occupational trajectories. A specific deficit in executive function in a subgroup of IGE, juvenile myoclonic epilepsy (JME) has been a particular focus of recent research. This systematic review provides a quantitative synthesis of cognitive function outcomes in 26 peer-reviewed, case-control studies published since 1989. Univariate random-effects meta-analyses were conducted on seven cognitive factor-domains and separately on executive function. Patients with IGE demonstrated significantly lower scores on tests across all cognitive factor-domains except visual-spatial abilities. Effect sizes ranged from 0.42 to 0.88 pooled standard deviation units. The average reduction of scores on tests of executive function in IGE compared to controls was 0.72 standard deviation units. Contrary to current thinking, there was no specific deficit in executive function in JME samples, nor in other IGE syndromes. Of more concern, people with IGE are at risk of pervasive cognitive impairment.

Visual disturbances representing occipital lobe epilepsy in patients with cerebral calcifications and coeliac disease: a case series

Paroxysmal visual manifestations may represent epileptic seizures arising from the occipital lobe. In coeliac disease (CD) bilateral occipital calcifications and seizure semiology consistent with an occipital origin have been described, primarily in Mediterranean countries. By reporting three adult patients from an Australian outpatient clinic with visual disturbances, occipital cerebral calcifications, and CD, this study seeks to emphasise that CD should be considered even when patients of non-Mediterranean origin present with these symptoms. Seizure types included simple partial, complex–partial, and secondarily generalised seizures. The seizure semiology consisted of visual disturbances such as: blurred vision, loss of focus, seeing coloured dots, and brief stereotyped complex visual hallucinations like seeing unfamiliar faces or scenes. Symptoms of malabsorption were not always present. Neurological examination was unremarkable in two patients, impaired dexterity and mild hemiatrophy on the left was noted in one. Routine electroencephalography was unremarkable. In all cases, computed tomography demonstrated bilateral cortical calcification of the occipital-parietal regions. Magnetic resonance imaging showed no additional lesion. All patients had biopsy confirmed CD. Seizure control improved after treatment with gluten free diet and anticonvulsants. This report illustrates the association between seizures of occipital origin, cerebral calcifications, and CD even in patients not of Mediterranean origin.