This chapter will outline the methodology used to investigate the neuropsychological profile of patients with refractory JME. The methodology was designed to investigate refractory JME patients, and assess whether JME patients with abnormal personalities are more impaired on executive functions and cognition than JME patients with a personality profile within normal limits. In addition, the methodology was designed to assess the impact of the following factors:
i. Seizure type and frequency ii. AEDs
iii. Age of epilepsy onset iv. Family history of epilepsy v. History of febrile seizures vi. Age
vii. Years in education
The design, participants and procedure of the current thesis will be given in this chapter and split into the following sections:
1. General methodology
2. Recruitment and description of participants
3. Neuropsychological tools utilised
4. Procedure
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6.2GENERAL METHODOLOGY
6.2.1R
ECAP OFA
IMS ANDH
YPOTHESISThe main aim of the current thesis is to investigate the relationship between the reported neuropsychological deficits in patients with JME, and the abnormal personality traits these patients are described to have. Additionally, the aim is to assess the impact of the contributory factors listed above and discussed in detail in Chapter four. Finally, the current thesis aims to examine the neuropsychological, social and clinical profile of JME patients who do not respond on AED treatment [13].
6.2.2C
ONTRIBUTION OF THE THESIS AUTHORJ.W.
18 participants were collected by research assistants at the University of Liverpool as part of the MRC funded ReJuMEC study. A further 21 participants were recruited and assessed by Dr Rhys Thomas as part of Wales Epilepsy Study. The author, J.W conducted 21 assessments, and arranged and confirmed each by letter and telephone. The author spent 62 hours conducting testing face to face, 51 hours of scoring and 16 hours of preparing feedback. J.W travelled a total of 2416.8 miles to carry out the assessments, equating to an average of 115 miles per assessment. This constitutes an average of 2.5 hours of travelling per participant and 48 hours spent travelling overall. In addition, J.W. arranged travel and accommodation required for the long distance assessments.
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Fig. 6.1 Number of neuropsychological assessments conducted across the UK by the author J.W
6.2.3E
THICSEthical approval was granted by the North West 1 Research Ethics Committee – Cheshire, in 2009 for the study titled ‘Juvenile Myoclonic Epilepsy Cohort’ (Ref: 09/H1017/55). Several amendments, including inclusion of seizure diaries, choice of length of EEG, and changes to the patient information sheet to the original application were made and granted between 2009-2010. With regards to the current study the author submitted a substantial amendment that was approved in January 2011. The amendment was for the inclusion of the ‘Eysenck Personality Questionnaire-Brief Version’ [129], so that the personalities of participants could be assessed. A research passport was obtained to gain Research and Development (R&D) approval from 11 NHS organisations. An honorary contract was granted by R&D at the University Hospital of Wales. Indemnity was covered by the University of Liverpool.
North West n=8 East Midlands n=3
South East n=1
West Midlands n=1
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6.3 RECRUITMENT
6.3.1P
ARTICIPANTSIn total 78 patients were recruited from five different epilepsy outpatient clinics across England and Wales. Four of these patients were excluded from the study as their seizures were controlled with AED treatment. One patient was excluded due to their inability to complete the assessment due to problems caused by an additional clinical illness. Four patients did not attend their pre-arranged neuropsychological assessment, and either could not be contacted afterwards or did not wish to arrange a follow up appointment. Three patients that were recruited by physicians could not be contacted due to insufficient details or change of address. Finally, six patients no longer wished to take part in the study once contacted following initial recruitment.
Therefore data from 60 (45 female:15 male) patients with refractory JME were used in the analysis. Patients were classified as refractory if they experience ≥1 myoclonic and/or absence and/or tonic-clonic seizure per month despite prior or current exposure to a dose of at least 1000mg of Sodium Valproate (VPA). The definition of drug-refractory epilepsy varies greatly in the literature [130]and has not been defined in JME research. The criteria used were based on a combination of the knowledge of sodium valproate being effective in 85–90% of the patients with most becoming seizure-free [66], the criteria used by others [131, 132] and clinical experience of members of the ReJuMEC group. .Exclusion criteria included abnormal MRI brain scan,
alcoholism, a history of drug abuse, and/or neurological disorder besides epilepsy. In addition none of the patients had experienced a GTCS within the 24 hours prior to the neuropsychological assessment. For full demographic and clinical characteristics of the participants please see Tables 7.1 and 7.2.
Participants were recruited during routine outpatient appointments with epilepsy specialists based in major neurological departments in the UK. Table 6.2 below gives a list of sites and number of patients tested at each site. Written informed consent was obtained for all participants by either the enrolling neurologist or specialist epilepsy nurse, or the author J.W.
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Table 6.2 Hospitals involved in recruitment and number of patients assessed at each site
Hospital n
Walton Centre, Liverpool 23 University Hospital Wales, Cardiff 32 Kings College London 1 Royal Hallamshire Hospital, Sheffield 3 Queen Elizabeth Hospital, Birmingham 1
Total number of participants 60