Managing anti-epileptic drug treatment in adult patients with intellectual disability: a serious conundrum.

BACKGROUND AND PURPOSE: About a quarter of people with epilepsy have intellectual disability (ID). This group has communication issues, premature mortality, more treatment resistance, difficulties in making informed choices and greater risks of physical and mental health comorbidities. There is no specific prescribing guidance for this large and vulnerable group. The literature on prescribing for epilepsy in this group was reviewed, in particular examining how antiepileptic drugs (AEDs) work regarding their side effect profiles, effects on specific epilepsy syndromes associated with ID and their individual strengths and weaknesses based on the nature and degree of ID. METHOD: This is a narrative review for which a comprehensive search was conducted to identify evidence for prescribing commonly used AEDs to people with ID including genetic syndromes specifically associated with epilepsy. RESULTS: A detailed analysis of the results has highlighted the urgent requirement for suitable and reliable evidence in AED prescribing amongst adults with epilepsy and ID as no studies taking account of the response to AEDs of the ID populations based on the WHO Diagnostic and Statistical Manual of Mental Disorders criteria of clinical severity of ID were identified. CONCLUSION: There is a significant shortfall in suitably powered studies to provide sufficient evidence for safe prescribing of AEDs to people with ID.

A qualitative study of carers' and professionals' views on the management of people with intellectual disability and epilepsy: a neglected population.

Little attention has been paid to the specific needs of people with intellectual disability and epilepsy despite evidence of increased prevalence of epilepsy, increased risk of complex epilepsy, and heightened use of health services among this group. In an attempt to address this gap, an online international survey was undertaken inviting health professionals and caregivers, both paid and family members, to share their views on the adequacy and quality of available treatment. This paper reports on the responses obtained from 113 individuals from the UK and Ireland. Findings revealed that professionals and carers differ in their expectations of treatment and with regard to maximizing communication during consultations. In addition, findings suggested that the potential of consultations to provide information for carers, as well as to allay concerns, may not be realized. Rescue medications were viewed favorably; however, respondents expressed less satisfaction with routine medications. These findings may reflect a failure by professionals to appropriately transfer knowledge of these treatments.

COVID-19 vaccination uptake in people with epilepsy in wales.

PURPOSE: People with epilepsy (PWE) are at increased risk of severe COVID-19. Assessing COVID-19 vaccine uptake is therefore important. We compared COVID-19 vaccination uptake for PWE in Wales with a matched control cohort. METHODS: We performed a retrospective, population, cohort study using linked, anonymised, Welsh electronic health records within the Secure Anonymised Information Linkage (SAIL) Databank (Welsh population=3.1 million).We identified PWE in Wales between 1st March 2020 and 31st December 2021 and created a control cohort using exact 5:1 matching (sex, age and socioeconomic status). We recorded 1st, 2nd and booster COVID-19 vaccinations. RESULTS: There were 25,404 adults with epilepsy (127,020 controls). 23,454 (92.3%) had a first vaccination, 22,826 (89.9%) a second, and 17,797 (70.1%) a booster. Comparative figures for controls were: 112,334 (87.8%), 109,057 (85.2%) and 79,980 (62.4%).PWE had higher vaccination rates in all age, sex and socioeconomic subgroups apart from booster uptake in older subgroups. Vaccination rates were higher in older subgroups, women and less deprived areas for both cohorts. People with intellectual disability and epilepsy had higher vaccination rates when compared with controls with intellectual disability. CONCLUSIONS: COVID-19 vaccination uptake for PWE in Wales was higher than that for a matched control group.

The provision of care to adults with an intellectual disability in the UK. A Special report from the intellectual disability UK chapter ILAE.

PURPOSE: This article reflects the report by the British Branch of the International League Against Epilepsy (ILAE) Working Group on services for adults with epilepsy and intellectual disability (ID). Its terms of reference was to explore the current status of aspects of the care of people with an ID and epilepsy. METHODS: Survey content was developed from key themes identified by consensus of the working group. An electronic survey was distributed via email. The sample population was the membership of the ILAE UK, Royal College of Psychiatrists (RCPsych) Faculty of ID, Epilepsy Nurses Association (ESNA), and the Association of British Neurologists (ABN). Following a six week response period the data was then collated, anonymised and distributed to the working group in order that opinion statements could be gathered. RESULTS: The time taken for individuals with both new-onset and established epilepsy to undergo routine investigation was commonly at least 1-3 months, far beyond recommendations made by NICE (CG20). A small minority of clinicians would not consider non-pharmacological interventions including epilepsy surgery, vagus nerve stimulation, and ketogenic diet for this population. Almost universally responders are actively involved in the assessment and management of key risk areas including risk of drowning, hospitalization, medication side effects, and sudden unexpected death in epilepsy (SUDEP). CONCLUSION: This investigation identifies key themes and recommendations relating to care delivery and meeting the complex needs of people with ID and epilepsy. Adults with ID and epilepsy appear to exist in a unique, but inadequate, segment of epilepsy care delivery.

Antidepressant prescribing: a comparison between general practitioners and psychiatrists.

BACKGROUND: The 'defeat depression' campaign emphasizes the importance of adequate prescribing of antidepressants in general practice. AIM: A study was undertaken to investigate the prescribing habits of a group of general practitioners and psychiatrists. METHOD: A postal questionnaire was sent to 123 general practitioners and 97 psychiatrists in south Wales. RESULTS: The response rate among general practitioners was 60% and among psychiatrists it was 67%. As a group, the psychiatrists reported using significantly higher daily dosages of antidepressant medication for adult and for elderly patients over a longer period compared with general practitioners. Fifty two per cent of 68 general practitioners and 17% of 60 psychiatrists reported using lower than recommended daily treatment dosages for adult patients and 40% of 68 general practitioners and 7% of 62 psychiatrists used a shorter than recommended period of continuation therapy (less than four months). Both groups showed a wide variation in the use of maintenance therapy. CONCLUSION: Educational efforts should be made to improve the prescribing habits of general practitioners and psychiatrists.

The outcome of prescribing novel anticonvulsants in an outpatient setting: factors affecting response to medication.

A survey of the prescription of the novel anticonvulsants lamotrigine, vigabatrin, gabapentin and topiramate was carried out in an outpatient setting to investigate the outcome of prescription and factors affecting response. One hundred and fifty randomly selected patient notes were analysed retrospectively. Drug continuation and seizure freedom were used as measures of response. Twenty-nine percent of patients had a brain lesion, 14% a psychiatric disorder, 7% neonatal seizures, 21% a family history of epilepsy and 15% a learning disability. On average at the moment of initiation of the novel anticonvulsant the patients had had a diagnosis of epilepsy for 18 years, were 33 years old, had 19 seizures per month and had previously used two drugs which failed to control their epilepsy. The first novel anticonvulsant was in 55% of cases lamotrigine, in 43% vigabatrin and in 1 % gabapentin. The overall percentage of patients who stayed on their first novel anticonvulsant was 55%, and 17% became seizure-free. No factors were found to influence the response in terms of drug continuation. For seizure freedom, the presence of a psychiatric disorder and partial seizures predicted a significantly poorer response. Length of seizure disorder, seizure frequency at initiation, the number of previously used failed drugs and the total number of drugs previously used were all significantly lower in the seizure-free group.

Learning disability and epilepsy. 1, towards common outcome measures.

A major component of the population of people who have epilepsy are people with a learning disability. As a group, such individuals often have complex epilepsy which is refractory to treatment. Current available measures to assess the outcomes of therapeutic interventions in epilepsy are based on seizure frequency, seizure severity and quality-of-life measures, but have not been validated in people with a learning disability. Thus, we do not know if such measures of outcome serve the needs of this group. This review examines how able we are to assess the efficacy of our interventions to control epilepsy in people with learning disability. It is suggested that a standard data set is necessary as the basis of the assessment of any therapeutic intervention. Central components of this data set would encompass a definition of important characteristics of an individual, a description of their epilepsy and an assessment of the impact of their condition on both their own and their carer's health. The approach to obtaining this information should employ a methodology which can allow for environmental influences.

The impact of epilepsy on patients' lives.

The impact of epilepsy is multifaceted and extensive on its effects. The occurrence of seizures is unpredictable and often dangerous, increasing the risk of injury, hospitalization and mortality, and adversely affecting a patient's mental health, often resulting in anxiety, depression or cognitive impairment. Seizures can also result in stigmatization and social exclusion, with detrimental effects on an individual's confidence and self-esteem. However, the burden of epilepsy extends beyond the effects of seizures themselves. In particular, individuals with epilepsy are significantly more likely to have medical or psychiatric comorbidities than those without epilepsy, and comorbidity in patients with epilepsy has been shown to be strongly correlated with negative impacts on subjective health status and quality of life (QoL). In addition, antiepileptic drug (AED) treatment is commonly associated with side effects, which further impair patients' QoL. Patient surveys provide valuable insights into what matters to patients in their daily lives and highlight important discrepancies between the perceptions of patients and their physicians. For example, survey data show that physicians underestimate the number of patients experiencing AED side effects and the impact of these on patients. Screening questionnaires can help physicians to quickly identify problems with treatment side effects; also, to recognize comorbidities such as depression that are otherwise difficult to identify in a time-limited consultation. Ultimately, successful management of epilepsy requires a holistic approach to care, with treatment tailored to the individual patient's needs; this can only be achieved through effective doctor-patient communication and the full involvement of a multidisciplinary care team.

Variability of antiepileptic medication taking behaviour in sudden unexplained death in epilepsy: hair analysis at autopsy.

BACKGROUND: Variable compliance with antiepileptic drugs (AEDs) is a potentially preventable cause of sudden unexplained death in epilepsy (SUDEP). Hair AED concentrations provide a retrospective insight into AED intake variability. METHODS: We compared hair AED concentration variability in patients with SUDEP (n = 16), non-SUDEP epilepsy related deaths (n = 9), epilepsy outpatients (n = 31), and epilepsy inpatients (n = 38). AED concentrations were measured in 1 cm hair segments using high performance liquid chromatography. Individual patient hair AED concentration profiles were corrected for "washout" using linear regression analysis. The coefficient of variation (CV) of the corrected mean hair AED concentration provided an index of variability of an individual's AED taking behaviour. Hair sample numbers varied between subjects, and so weighted regression estimates of the CV were derived for each group. RESULTS: The CV regression estimates for each group were: SUDEP 20.5% (standard error 1.9), non-SUDEP 15.0% (3.9), outpatients 9.6% (1.4), and inpatients 6.2% (2.7). The SUDEP group therefore showed greater hair AED concentration variability than either the outpatient or the inpatient groups (p<0.0001). CONCLUSION: Observed variability of hair AED concentrations, reflecting variable AED ingestion over time, is greater in patients dying from SUDEP than in either epilepsy outpatients or inpatients. SUDEP, at least in a proportion of cases, appears preventable.

Topiramate: uses in people with an intellectual disability who have epilepsy.

The novel anticonvulsant topiramate has been shown to have efficacy across a range of seizure types including both generalized and partial seizures in several well-designed randomized controlled trials. It has also been shown to be effective in atonic seizures associated with Lennox-Gastaut syndrome. Tolerability data show a tendency to neuropsychiatric side-effects, such as confusion and word finding difficulties, when topiramate is used in polytherapy; these side-effects are reduced in monotherapy usage. The efficacy and spectrum of seizures treated by topiramate suggests that it has an important role in managing epilepsy in people with intellectual disability. The predictable side-effects can be monitored in clinical practice and possibly reduced by slow dose increments. The data set of patients with intellectual disability is still too small to rule out idiosyncratic drug reaction.

Primary health care and people with an intellectual disability: the evidence base.

There is growing awareness of the importance of evidence-based medicine in guiding health care delivery. This paper reviews the evidence pertinent to the delivery of primary health care to people with an intellectual disability. Research concerning issues of health status, specialist knowledge of health care, and barriers and solutions to health care delivery for people with an intellectual disability is presented and discussed. Recommendations for future evidence-based research are made, including suggested areas of importance.

A randomized control trial of an opportunistic health screening tool in primary care for people with intellectual disability.

People with intellectual disability have an increased variety of health care problems compared with the general population. The transition of care for such people from institutions into the community places them in a primary care system already facing increasing demands for their services. There is a consensus that health screening is at its most useful in identifying functional disabilities. Therefore, an intervention which helps the general practitioner (GP) towards opportunistically checking those areas of health most often deficient in people with intellectual disability would appear of considerable benefit (especially if information directing the GP towards appropriate secondary care services is also provided). The present study was designed to evaluate the impact of such an intervention.

Health care provision for people with a learning disability. Record-linkage study of epidemiology and factors contributing to hospital care uptake.

BACKGROUND: We know little about how people with a learning disability access secondary health care. AIMS: To describe the epidemiology of learning disability, the influence of deprivation on prevalence and the pattern of secondary care uptake, including the effect of institutionalisation. METHOD: A record-linkage study of secondary care contacts of 434,000 people between 1991 and 1997. A population with learning disability was identified; their secondary care contact was calculated and compared with the general population's. RESULTS: The distribution of people with a learning disability (n = 1595) correlated significantly with deprivation. The presence of a learning disability hospital significantly affected care uptake. Place of residence also affected acute admission to the learning disability hospital. Former institution residents generated 212 admissions per 1000 patients; community patients generated 18 per 1000. The admission rate with any psychiatric diagnosis to any setting was 26.3 per 1000 people with a learning disability; 16.5% of such patients had a dual diagnosis. CONCLUSIONS: Health provision for people with a learning disability is affected by institutional provision.

Social deprivation and prevalence of epilepsy and associated health usage.

OBJECTIVES: To examine the relation between social deprivation and the prevalence of epilepsy and associated morbidity using hospital activity data as a proxy. METHODS: The study was conducted in the health district of South Glamorgan, United Kingdom (population 434 000). Routinely available hospital data (inpatient and outpatient), an epilepsy clinic database, and mortality data underwent a process of record linkage to identify records relating to the same patient and to identify patients with epilepsy. Each patient was allocated a Townsend index deprivation score on the basis of their ward of residence. Age standardised correlations were calculated between deprivation score and prevalence of epilepsy, inpatient admissions, and outpatient appointments. Standardised mortality ratios (SMR) were also calculated. All analyses were performed on two cohorts: (1) all patients with epilepsy and (2) those patients with epilepsy without any underlying psychiatric illness or learning disability. RESULTS: The prevalence of epilepsy ranged between 2.0 and 13.4 per 1000 with a median of 6.7. There were positive correlations between social deprivation and prevalence in both populations: (1) r=0.75 (p<0.001) and (2) r=0.70 (p<0.001). After standardising for underlying prevalence there were also correlations for mean inpatient admissions: (1) r=0.62 (p<0.001), (2) r=0.59, (p<0.001) and for outpatient appointments: (1) r=0.53, (p=0.001) and (2) r=0. 51 (p=0.001). The SMR for those deprived was (1) 1.66 (95% confidence interval (95% CI) 1.27-2.05) and (2) 1.80 (95% CI 0.71-1. 67). For the population as a whole (with and without epilepsy) the SMR was 1.25 (95% CI 1.27-2.32). CONCLUSION: This study shows a strong correlation between the prevalence of epilepsy and social deprivation and weaker correlations between social deprivation and mean hospital activity.

Assessing clinicians' consultation with people with profound learning disability: producing a rating scale.

This paper describes the development of a scale for assessing clinicians' communication with people with profound learning disability. Four psychiatrists and four nurses were assessed completing three simple non-invasive clinical procedures--blood pressure, pulse and axillary temperature--with people with profound learning disability. Videotaped assessment of consultations was performed by three experienced speech and language therapists using a previously designed scale for analysing encounters with people with mild learning disability. This led to the production of a new scale specifically for people with profound learning disability. A significant inter-rater reliability was found between the three speech therapists for total scores (rater a-b, corr = 0.654, P = 0.006; rater a-c, corr = 0.795, P = 0.0001: rater b-c, corr = 0.673, P = 0.004). Significant reliability between raters was also found for the subsections of verbal behaviour and non-verbal behaviour.

Learning disabilities: moving forward--a focus on epilepsy, Birmingham, England, 29 June 1996.

On 29 June 1996 a conference was held in Birmingham to highlight the status of epilepsy in people with learning disabilities. The conference consisted both of seminars and workshops. Dr Tim Betts, Birmingham; Dr Greg O'Brien, Northumberland; and Dr Mike Kerr addressed issues of assessment, diagnosis and drug treatment of epilepsy in this population. This meeting report summarizes the proceedings of the conference.

Functional regulator therapy for cleft palate patients.

Nine cleft lip and palate children exhibiting collapse of maxillary segments and cross-bite were treated orthodontically with the functional regulator (FR) for periods ranging from 6 to 18 months. The purpose of this investigation was to evaluate quantitatively treatment of cleft palate patients with the functional regulator and to evaluate the effect of the appliance on their speech. Cleft palate patients typically have speech, nose, and lip defects which make patient cooperation and appliance acceptance more difficult. However, patient cooperation was considered good with an average mean of 12.7 hours per day of appliance wear and a range of 5.6 to 18.2 hours. Change in interimplant dimension was measured on frontal radiographs, and dental changes were measured of serial dental casts. The resulting data indicated no significant change in maxillary width or cross-bite relationship. The functional regulator was not clinically useful in this sample when the treatment objective was primary expansion of collapsed maxillary segments in the cleft palate patient. Good speech-production skills prior to treatment will minimize the adverse effect that the FR has on speech intelligibility. A significant amount of accommodation to the appliance occurs within 1 week after insertion, but maximum improvement in speech intelligibility occurs with full-time wear of the appliance for as many hours per day as possible.

Reducing antipsychotic medication in people with a learning disability.

BACKGROUND: The use of antipsychotic drugs in people with learning disabilities is currently receiving intensified scrutiny and attempts are being made to reduce it. AIMS: A randomised controlled trial was designed to investigate factors influencing antipsychotic drug reduction among people with learning disabilities prescribed such medication for behavioural problems. METHOD: Thirty-six participants randomly allocated to the experimental group underwent four, monthly 25% drug reduction stages. There were no planned drug changes for the control group (n = 20). RESULTS: Twelve participants (33%) completed full withdrawal; a further seven (19%) achieved and maintained at least a 50% reduction. Drug reduction was associated with increased dyskinesia and higher activity engagement but not increased maladaptive behaviour. Some setting characteristics were associated with drug reinstatement. CONCLUSIONS: A substantial proportion of people with learning disability prescribed antipsychotic medications for behavioural purposes rather than for treating psychotic illness can have their drugs reduced or withdrawn.