Awareness of social care needs in people with epilepsy and intellectual disability.

BACKGROUND: Nearly a quarter of people with intellectual disability (ID) have epilepsy with large numbers experiencing drug-resistant epilepsy, and premature mortality. To mitigate epilepsy risks the environment and social care needs, particularly in professional care settings, need to be met. PURPOSE: To compare professional care groups as regards their subjective confidence and perceived responsibility when managing the need of people with ID and epilepsy. METHOD: A multi-agency expert panel developed a questionnaire with embedded case vignettes with quantitative and qualitative elements to understand training and confidence in the health and social determinants of people with ID and epilepsy. The cross-sectional survey was disseminated amongst health and social care professionals working with people with ID in the UK using an exponential non-discriminative snow-balling methodology. Group comparisons were undertaken using suitable statistical tests including Fisher's exact, Kruskal-Wallis, and Mann-Whitney. Bonferroni correction was applied to significant (p < 0.05) results. Content analysis was conducted and relevant categories and themes were identified. RESULTS: Social and health professionals (n = 54) rated their confidence to manage the needs of people with ID and epilepsy equally. Health professionals showed better awareness (p < 0.001) of the findings/recommendations of the latest evidence on premature deaths and identifying and managing epilepsy-related risks, including the relevance of nocturnal monitoring. The content analysis highlighted the need for clearer roles, improved care pathways, better epilepsy-specific knowledge, increased resources, and better multi-disciplinary work. CONCLUSIONS: A gap exists between health and social care professionals in awareness of epilepsy needs for people with ID, requiring essential training and national pathways.

Tackling increased risks in older adults with intellectual disability and epilepsy: Data from a national multicentre cohort study.

PURPOSE: People with intellectual disabilities (ID) suffer multimorbidity, polypharmacy and excess mortality at a younger age than general population. Those with ID and epilepsy are at higher risk of worse clinical outcomes than their peers without epilepsy. In the ID population the health profile of those aged ≥40 years can be compared to those aged over 65 in the general population. To date there is limited data available to identify clinical characteristics and risk factors in older adults (≥40 years) with ID and epilepsy. METHODS: The Epilepsy in ID National Audit (Epi-IDNA) identified 904 patients with ID and epilepsy from 10 sites in England and Wales. This subsequent analysis of the Epi-IDNA cohort compared the 405 adults over 40 years with 499 adults ≥18 years aged under 40 years. Comparison was made between clinical characteristics and established risk factors using the Sudden Unexpected Death in Epilepsy (SUDEP) and Seizure Safety Checklist. RESULTS: The older adults' cohort had significantly higher levels of co-morbid physical health conditions, mental health conditions, anti-seizure medications (median 5), and antipsychotics compared to the younger cohort. The older group were significantly less likely to be diagnosed with a co-morbid neurodevelopmental disorder, and to have an epilepsy care plan. CONCLUSION: This is the largest study to date focused on adults with ID and epilepsy over 40 years. The ≥40 years cohort compared to the younger group has higher levels of clinical risk factors associated with multi-morbidity, potential iatrogenic harm and premature mortality with worse clinical oversight mechanisms.

Epilepsy, anti-seizure medication, intellectual disability and challenging behaviour - Everyone's business, no one's priority.

PURPOSE: People with Intellectual Disability (ID) and epilepsy are more likely to experience psychiatric conditions, challenging behaviour (CB), treatment resistance and adverse effects of anti-seizure medications (ASM) than those without. This population receives care from various professionals, depending on local care pathways. This study evaluates the training status, confidence, reported assessment and management practices of different professional groups involved in caring for people with ID, epilepsy and CB. METHODS: A cross sectional survey using a questionnaire developed by expert consensus which measured self-reported training status, confidence, and approaches to assessment and management of CB in people with ID and epilepsy was distributed to practitioners involved in epilepsy and/or ID. RESULTS: Of the 83 respondents, the majority had either a psychiatry/ID (n = 39), or Neurology/epileptology background (n = 31). Psychiatry/ID and Neurology/epileptology had similar confidence in assessing CB in ID-epilepsy cases, but Psychiatry/ID exhibited higher self-rated confidence in the management of these cases. While assessing and managing CB, Psychiatry/ID appeared more likely to consider mental health aspects, while Neurology/epileptology typically focused on ASM. CONCLUSION: Psychiatry/ID and Neurology/epileptology professionals had varying training levels in epilepsy, ID and CB, had differing confidence levels in managing this patient population, and considered different factors when approaching assessment and management. As such, training opportunities in ID should be offered to neurology professionals, and vice versa. Based on the findings, a best practice checklist is presented, which aims to provide clinicians with a structured framework to consider causal explanations for CB in this population.

Dealer's Dytonia (Croupier's Cramp) - An Unusual Hazard Of Gambling.

Dystonia takes many forms and often presents acutely to emergency care. The diagnosis is often delayed because it mimics other more common conditions. This report describes a patient with a rare occupational dystonia, the typical clinical features of dystonia in general, and differentials to consider.

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.

Discussing sudden unexpected death in epilepsy: Are we empowering our patients? A questionnaire survey.

OBJECTIVE: To examine patient knowledge about sudden unexpected death in epilepsy (SUDEP) compared to other risks in epilepsy. To explore patients' experiences surrounding SUDEP disclosure and opinions on how information should be delivered. DESIGN: A cross-sectional questionnaire. SETTING: Royal Free Hospital, London outpatient epilepsy clinics. PARTICIPANTS: New and follow-up patients attending epilepsy clinics at a London teaching hospital over six months. Patients identified as being at risk of suffering negative emotional or psychological consequences of SUDEP discussions were excluded. MAIN OUTCOME MEASURES: Patient knowledge about epilepsy risks; patient opinion regarding source, timing and delivery of SUDEP information; impact on health seeking behaviour. RESULTS: Ninety-eight per cent of patients were aware of medication adherence, 84% of factors influencing seizure frequency, 78% of driving regulations, 50% of SUDEP and 38% of status epilepticus; 72% of patients felt that SUDEP information should be given to all patients. Preferences for timing of SUDEP discussions varied between those wanting information at diagnosis (40%) and those preferring to receive it after three clinic appointments (18%) to avoid information overload at the first consultation. Emotional responses (48% positive, 38% negative) predominated over measurable behavioural change following SUDEP discussions. CONCLUSIONS: Less than half the patients knew about SUDEP and status epilepticus. Although the majority of patients with epilepsy wish to be informed about SUDEP early on in their diagnosis, information must be delivered in a way that promotes patient knowledge and empowerment.

Convergence of occipital nerve and superior sagittal sinus input in the cervical spinal cord of the cat.

Co-existence of facial and occipital pain may occur in occipital neuralgia, migraine and cluster headache; suggesting convergence of trigeminal and cervical afferents. Such convergence has been shown in humans and other animals, but the site and extent of this are uncertain. In anaesthetized adult cats, the superior sagittal sinus and occipital nerve were stimulated electrically, and extracellular recordings made in the dorsolateral area of the upper cervical cord using glass-coated tungsten electrodes. Of 49 units in 10 cats, 33 (67%) had input from the superior sagittal sinus and the occipital nerve. Thirteen (27%) had superior sagittal sinus input and 3 (6%) had occipital nerve input. Convergent receptive fields were identified mechanically in 7 units. These experiments in cats show convergent input from occipital nerve and superior sagittal sinus on dorsolateral area units in two-thirds of cases studied. This experimental site of trigeminocervical convergence may relate to referral of pain in occipital neuralgia and other headaches.

Convergence of afferents from superior sagittal sinus and tooth pulp on cells in the thalamus of the cat.

We have previously shown convergence of craniovascular and tooth pulp afferents in the cervical spinal cord of cats. This study looked for similar convergence in the thalamus. Fifty-four thalamic cells with input from tooth pulp, superior sagittal sinus, or both, were identified. Twenty-nine cells with tooth pulp and superior sagittal sinus input were located in the ventrobasal complex of the intralaminar nuclei. Most of these 29 cells were also excited by cooling the contralateral tooth pulp, and 21 had receptive fields on the contralateral face or forelimb. Twenty cells excited by stimulation of superior sagittal sinus, and not tooth pulp, were found in several nuclei. The 5 cells excited by stimulation of tooth pulp, but not sagittal sinus, were restricted to the ventrobasal complex. The data confirm convergence from sagittal sinus, tooth pulp, and skin in the thalamus of anaesthetized cats.

Convergence of afferents from superior sagittal sinus and tooth pulp on cells in the upper cervical spinal cord of the cat.

Units in the dorsolateral area of the upper cervical cord respond to craniovascular stimulation. This study examined tooth pulp responses in this area in cats. Eleven of 21 units tested in the dorsolateral area had convergent inputs from superior sagittal sinus and tooth pulp; while 10 units had sagittal sinus, but not tooth pulp, input. Mean response latency to tooth pulp stimulation (25.8 ms) was significantly longer than to superior sagittal sinus stimulation (9.8 ms). Half of the units had cutaneous receptive fields; and in five units, action potentials could be evoked by electrical stimulation in the posterior complex of the thalamus.

The spinal cord processing of input from the superior sagittal sinus: pathway and modulation by ergot alkaloids.

The effects of ergot alkaloids on field potentials and unit responses produced in the upper cervical spinal cord by stimulation of the superior sagittal sinus (SSS) were examined in 57 anesthetized cats. Electrical stimulation of the SSS produced field potentials and single-unit responses at latencies of 5-20 ms. Field potentials were abolished by section of the first division of the trigeminal nerve but were unaffected or increased by section of the upper cervical nerves. Field potentials were reduced or abolished by intravenous injection of ergotamine or dihydroergotamine (DHE). The evoked response of 41 units (34.4%) were suppressed by either i.v. or iontophoretic administration of ergotamine, DHE or ergometrine. The results suggest that ergot alkaloids exert an effect at a spinal cord relay centre which receives trigeminally mediated input from cranial blood vessels.

The function of large and small nerve fibers in renal failure.

Previous clinical and neurophysiological studies of uremic neuropathy have focused almost exclusively on the function of large sensory and motor axons. The sensations of heat and cold depend on the function of unmyelinated afferents and small myelinated afferents, respectively, and these sensations can be quantified using a standardized psychophysical technique. Thermal thresholds were measured in 20 patients with end-stage renal failure to determine the extent of small afferent fiber involvement and to compare this with the clinical and electrophysiological evidence of large fiber involvement. Whereas abnormalities of standard nerve conduction studies were found in 16 patients, abnormal thermal thresholds were found in only 6 patients. In the nerve conduction studies, the amplitudes of nerve potentials were reduced more than their conduction velocities, consistent with an axonopathy. This study found little evidence of significant dysfunction of small afferent fibers in end-stage renal failure and, when such changes occurred, they did not correlate with the clinical evidence of polyneuropathy. The functional sparing of axons of small diameter is consistent with the relative sparing of these axons in pathological studies.

Craniovascular nociceptive pathways relay in the upper cervical spinal cord in the cat.

Units in the dorsolateral area of the upper cervical cord and the ventroposteromedial nucleus of the thalamus respond to stimulation of cranial vessels. To study the physiological role of the upper cervical cord in craniovascular transmission, we used a cryoprobe to interrupt reversibly neural transmission through the cord while recording in the thalamus. Twenty-one of 47 thalamic units tested showed reversible diminution in their response to superior sagittal sinus stimulation during cervical cord cooling. In contrast, receptive field responses and spontaneous thalamic activity were unaffected. These data suggest offt the cervical cord relays craniovascular nociceptive afferents.