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Syndromes and Seizures: Case Studies in Cognition and Mental Disorders of Adults with Epilepsy ResearchOnline@JCU This file is part of the following reference: Ballini, Evangelina Evelyn (2013) Syndromes and seizures: case studies in cognition and mental disorders of adults with epilepsy. Masters (Research) thesis, James Cook University. Access to this file is available from: http://researchonline.jcu.edu.au/5497/ The author has certified to JCU that they have made a reasonable effort to gain permission and acknowledge the owner of any third party copyright material included in this document. If you believe that this is not the case, please contact [email protected] and quote http://researchonline.jcu.edu.au/5497/ SYNDROMES AND SEIZURES: CASE STUDIES IN COGNITION AND MENTAL DISORDERS OF ADULTS WITH EPILEPSY Thesis submitted by Evangelina Evelyn BALLINI, M.A. (Hons) June, 2013 For the degree of Master of Philosophy in the School of Arts and Social Sciences James Cook University Australia STATEMENT OF ACCESS I, the undersigned, the author of this thesis, understand that James Cook University will make this thesis available for use within the University Library and allow access to users in other approved libraries. I understand that, as an unpublished work, a thesis has significant protection under the Copyright Act, and thus I do not wish to place any further restriction on access to this work. ____________________________ ____________________________ Signature Date ____________________________ Name (please print) ii STATEMENT OF SOURCES I declare that this thesis is my own work and has not been submitted in any form for another degree or diploma at any university or other institution of tertiary education. Information derived from the published or unpublished work of others has been acknowledged in the text and a list of references is given. I declare that the thesis contains no material which infringes the copyright of any other person(s). ____________________________ ____________________________ Signature Date ____________________________ Name (please print) iii ELECTRONIC COPY I, the undersigned, author of this work, declare that the electronic copy of this thesis provided to James Cook University library is an accurate copy of the print thesis submitted, within the limits of technology available. ____________________________ ____________________________ Signature Date ____________________________ Name (please print) iv COPYRIGHT DECLARATION Every reasonable effort has been made to gain permission and acknowledge the owners of copyright material. I would be pleased to hear from any copyright owner who has been omitted or incorrectly acknowledged. ____________________________ ____________________________ Signature Date ____________________________ Name (please print) v DECLARATION ON ETHICS The research presented and reported in this thesis was conducted within the guidelines for research ethics outlined in the National Statement on Ethical Conduct in Research Involving Humans (1999); the Joint NHMRC/AVCC Statement and Guidelines on Research Practice (1997); the James Cook University Policy on Experimentation Ethics, Standard Practices and Guidelines (2001); and the James Cook University Statement and Guidelines on Research Practice (2001). The proposed research methodology received clearance from the James Cook University Experimentation Ethics Review Committee. Approval Number: H 1949, Category 2. Approval Date: 13 November, 2004. The Cairns Base Hospital Ethics Committee granted permission to conduct the study at CBH and approved the proposed research methodology. Reference: Study Number 331 Approval Dates: 28th June, 2004; 15th December, 2004. Copies of JCU Ethics approval and letters of CBH Ethics approval can be found in Appendix F. ____________________________ ____________________________ Signature Date ____________________________ Name (please print) vi ACKNOWLEDGMENTS My sincere gratitude goes to my two supervisors, Professor Edward Helmes and Dr. John Archer, for their support. Ed, I am most grateful for your guidance in statistical analyses and advice on neuropsychological assessment. John, your expertise in epileptology shared during case discussions enhanced my understanding and interest in this area. I am truly grateful for the generosity of Associate Professor David Andrewes in supplying me not only with the two questionnaires used in Chapter Seven, but also his data from two groups of neurologically healthy adult controls. Participants I must acknowledge the contribution of the volunteers in this study, both the patients with epilepsy and their spouses/family/close friends. This thesis would not have been possible without their participation through many hours of difficult neuropsychological testing and interviews. Respect is due not only for their constancy in attending sessions, but also sharing their stories, often told with calm acceptance. I wish to dedicate this thesis to them, with best wishes for the future. Cairns Base Hospital staff I received a great deal of practical support from employees at the Cairns Base Hospital. In particular, I wish to thank Josie Valese for her emotional support and kindness during my research year at CBH, as well as free access to photo-copiers. Thanks to Mary-Ann Irvin, Head Librarian of CBH Library, for her generosity with inter-library loans and photo-copying services free of charge, and Terry Lallemand of the Neurology Clinic for locating charts. I cannot describe adequately my debt to Maria Curcio for her easy efficiency in finding countless medical charts throughout the year. Many thanks go to Des Letcher and Kate Heath who regularly sent me computer listings of the latest epilepsy admissions to the Emergency Department. JCU Library The JCU Library staff’s competent and ready assistance throughout my candidature is acknowledged, in particular Bronwyn Forster and Brian Kenady who prepared suitable copies of MRI brain scans and EEG recordings. Thanks go to Jackie Wolstenholme from JCU Townsville for her interest and encouragement as well as copyright information. Psychology Department, JCU Cairns I wish to thank John O’Riley who willingly helped with all computer-related problems. Dr. Nerina Caltabiano contributed advice on statistical analyses and support when it was most needed. I wish to gratefully acknowledge Kathy Fowler for the End-Note tutorials, and in particular for professionally formatting the thesis (including its Tables and Figures) in accordance with APA style. Finally, my thanks go to Dr. Marie O’Shea for her editing, feed-back on clinical cases and alternative conceptualizations, and to Professor Bruce Schefft for his useful suggestions on theories and interpretations. vii ABSTRACT The study’s general purpose was to examine cognitive functioning and mental disorders associated with syndromes or seizures of individuals with different epilepsies. More specifically, the aim was to investigate the cognitive and psychosocial profile of individuals with a history of prolonged seizures (status epilepticus). Neuropsychological assessment included cognitive tasks, test batteries and three self-rated questionnaires on psychopathology. Case studies included absence status (ASE), complex partial status (CPSE), generalized convulsive status (GCSE) temporal lobe epilepsy (TLE), and idiopathic generalized epilepsy (IGE). Most of the 24 adults, 18 to 65 years, had epilepsy of a cryptogenic or genetic aetiology. Individuals were compared to an IGE comparison group utilizing computational techniques designed for single case analyses. Overall, the findings from the series of case studies in IGE concurred with the traditional view that IGE does not generally impact on cognitive function. Against this background, two photosensitive cases with perioral myoclonia and eyelid myoclonia with absences had severe impairments of attentional control and memory systems. Those IGE individuals with single or no reflex components had very mild dysfunction (if any at all). At best, the assumption that TLE impacts on localized memory deficits which lateralize according to material-specificity principles was only partially upheld. An organization into three classes of deficits and strengths emerged from the assessment results, based loosely on seizure- types and the presence of structural abnormalities. Adaptability of cognitive skills to alleviate possible memory problems seems to have been possible through an individual’s reserve strengths; less possible when attention responses and fluency errors reflected a quality of rigidity (those cases with secondarily generalized seizures); or when generalized impairment (executive functions, language and verbal memory) was viii associated with compromised brain reserve such as hippocampal sclerosis. It was concluded that these three classes might be interpreted as cognitive phenotypes of TLE. The SE results showed that not all prolonged seizures are associated with long-standing cognitive deficits and a poor prognosis. Overall, the GCSE participants did not demonstrate the most severe cognitive impairments as predicted, most having transient cognitive impairment which resolved over time. More widespread and longer-lasting deficits were reported in the two CPSE cases with secondarily generalized seizures and histories of polysubstance abuse. Attentional impairments were ubiquitous throughout the SE performance scores (particularly divided attention and vigilance) in the SE participants, followed by verbal executive abilities, then language and memory. Four SE cases showed a decline from pre-morbid levels of intelligence which
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