Epidemiology of epilepsy in Tasmania : a thesis presented in partial fulfilment of the requirements for the degree of Doctor of Philosophy in Epidemiology at Massey University, Wellington, New Zealand

Background Better understanding of the demographic distribution of epilepsy and the prevalence of 'more specific forms of epilepsy' in community-based settings would improve our understanding of this disorder at the population level . Although we now have good estimates of epilepsy prevalence for most countries, we still lack knowledge on its demographic distribution by age, ethnicity, region, and socioeconomic status. In addition, no studies to date have reported the prevalence of epilepsy syndromes using patient interview outside a hospital setting. This thesis provides the first community-based estimates of the prevalence of the most common clinical group of epilepsies presumed to have a genetic basis - The Idiopathic Generalised Epilepsies (IGE) - by patient and witness interview. Methods This thesis has involved conducting five pieces of new research: (i) a series of reviews and analyses of descriptive data on epilepsy prevalence, particularly focusing on the critical methodological issues of ascertainment, diagnosis and classification of epilepsy for epidemiological purposes; (ii) the validation of a modified diagnostic epilepsy questionnaire adapted for administration in population studies; (iii) recruitment of a community-based cohort - The Tasmanian Epilepsy Register (TER) - through the Australian national prescription database; (iv) estimation of the overall prevalence and distribution of self-reported treated epilepsy in Tasmania by imputation methods; (v) estimation of the prevalence and distribution of IGE in Tasmania by telephone interviewing. Results My modified diagnostic questionnaire, administered by telephone interviewing and interpreted with standardized guidelines, demonstrated excellent agreement with an epilepsy specialist's clinical assessment in diagnosing the presence of epilepsy (K = 0.94), seizure-onset types (K = 0.84), simple or complex partial seizures (K=0. 87), any generalized non-convulsive seizure (K=0.82), and IGE (K = 0.82). A lthough stil l substantial, agreement was not as close for secondarily general ized seizures (K = 0.74), and generalized tonic-clonic seizures (K = 0.79). 7541 patients treated with antiepileptic drugs (AEDs) in the preceding year in Tasman ia were eligible for recruitment through the Australian national prescription database. After three mail contacts, 54.0% responded, with 43.6% who indicated treatment for epilepsy representing 86.0% of total possible epilepsy cases by imputation (n=2063) in Tasmania. 1180 agreed to participate in the TER, 90.0% of participants received their AEDs either exclusively from their general practitioner (70.9%) or in combination with a medical specialist (19.1%) in the preceding twelve months. The adjusted treated epilepsy prevalence was 4.36 per 1000 (95% CI 4.34, 4.39); this was: lower in women (prevalence ratio 0.92 (95% CI 0.84, 1.00); greater with increasing age (p< 0.001 ); similar in the three main geographical regions; and similar by categories of socioeconomic status based on postcode of residence. Following enrolment, 959/1083 (88.6%) eligible TER participants completed the diagnostic telephone interviewing, with partial epilepsy classified in two thirds, and generalised epilepsy in slightly more than one-fifth. IGE was observed in 20.3%, with tonic-clonic seizures (17.03%) and the absence epilepsies combined (11.01 %) being the most common IGE seizure types and syndromes respectively. The estimated prevalence of IGE was 0.89 per 1000; is highest between the ages of 20-39 years and in females, but was similar between Tasmanian regions and socio-economic groups. IGE prevalence beyond childhood related to refractory childhood or adolescent disease rather than olderonset cases, and was characterised by the presence of myoclonic and tonic-clonic seizures. Generalised seizures, but not IGE, were less prevalent in southern Tasmania. Conclusions Utilising the design approach described in this thesis may provide an alternative to neurological assessment, and when coupled with case ascertainment through prescription data, can provide a valid estimate of the prevalence of 'more specific forms of epilepsy' in countries with high access to health services. The observed pattern of high elderly epilepsy prevalence, is similar to patterns in recent studies in other developed countries, and has important implications for future planning of health services in these countries. IGE represents a considerable proportion of community-treated disease with important aetiological and prognostic determinants occurring at the seizure rather than syndrome level of classification