The Walton Centre neurology network - an equitable, sustainable and deliverable model for a large-scale neurology service.

UK neurology has many different models of provision and a shortage of essential clinical staff. Services are sometimes unsatisfactory and there is much variation and inequality, especially in areas outside London where there are far fewer consultants. Some hospitals have much better staffed and resourced neurological services than others which may have far less provision or even no neurology service at all. There is no national strategy or agreed model of service delivery - local areas have evolved individual arrangements, often dictated by consultant availability. We describe, with clear operational details, a neurology network model in a large population, with outcomes. In many areas with limited resources it could, by re-organisation of current services, be considered instead of existing separate, variable and potentially inequitable local arrangements.

The long-term outcomes of epilepsy surgery.

OBJECTIVE: Despite modern anti-epileptic drug treatment, approximately 30% of epilepsies remain medically refractory and for these patients, epilepsy surgery may be a treatment option. There have been numerous studies demonstrating good outcome of epilepsy surgery in the short to median term however, there are a limited number of studies looking at the long-term outcomes. The aim of this study was to ascertain the long-term outcome of resective epilepsy surgery in a large neurosurgery hospital in the U.K. METHODS: This a retrospective analysis of prospectively collected data. We used the 2001 International League Against Epilepsy (ILAE) classification system to classify seizure freedom and Kaplan-Meier survival analysis to estimate the probability of seizure freedom. RESULTS: We included 284 patients who underwent epilepsy surgery (178 anterior temporal lobe resections, 37 selective amygdalohippocampectomies, 33 temporal lesionectomies, 36 extratemporal lesionectomies), and had a prospective median follow-up of 5 years (range 1-27). Kaplan-Meier estimates showed that 47% (95% CI 40-58) remained seizure free (apart from simple partial seizures) at 5 years and 38% (95% CI 31-45) at 10 years after surgery. 74% (95% CI 69-80) had a greater than 50% seizure reduction at 5 years and 70% (95% CI 64-77) at 10 years. Patients who had an amygdalohippocampectomy were more likely to have seizure recurrence than patients who had an anterior temporal lobe resection (p = 0.006) and temporal lesionectomy (p = 0.029). There was no significant difference between extra temporal and temporal lesionectomies. Hippocampal sclerosis was associated with a good outcome but declined in relative frequency over the years. CONCLUSION: The vast majority of patients who were not seizure free experienced at least a substantial and long-lasting reduction in seizure frequency. A positive long-term outcome after epilepsy surgery is possible for many patients and especially those with hippocampal sclerosis or those who had anterior temporal lobe resections.

A novel method testing the ability to imitate composite emotional expressions reveals an association with empathy.

Social communication relies on intentional control of emotional expression. Its variability across cultures suggests important roles for imitation in developing control over enactment of subtly different facial expressions and therefore skills in emotional communication. Both empathy and the imitation of an emotionally communicative expression may rely on a capacity to share both the experience of an emotion and the intention or motor plan associated with its expression. Therefore, we predicted that facial imitation ability would correlate with empathic traits. We built arrays of visual stimuli by systematically blending three basic emotional expressions in controlled proportions. Raters then assessed accuracy of imitation by reconstructing the same arrays using photographs of participants' attempts at imitations of the stimuli. Accuracy was measured as the mean proximity of the participant photographs to the target stimuli in the array. Levels of performance were high, and rating was highly reliable. More empathic participants, as measured by the empathy quotient (EQ), were better facial imitators and, in particular, performed better on the more complex, blended stimuli. This preliminary study offers a simple method for the measurement of facial imitation accuracy and supports the hypothesis that empathic functioning may utilise motor control mechanisms which are also used for emotional expression.

Hyperosmolar non-ketotic hyperglycaemia: an important and reversible cause of acute bilateral ballismus.

An 83-year-old lady with type 2 diabetes mellitus was admitted to hospital with pneumonia. After 3 days of oral amoxicillin she developed ballism-choreiform movements of all four limbs. Her serum glucose and osmolality were raised. She had no factors suggestive of genetic or iatrogenic causes. A CT scan of the brain revealed bilateral putamen hyperintensities. She was started on tetrabenazine and subcutaneous insulin, which led to complete resolution of her symptoms.

When the first antiepileptic drug fails in a patient with juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy is one of the most common types of epilepsy seen in an adult epilepsy clinic. Most patients have a good prognosis, particularly when treated with valproate, but a significant minority are more difficult to treat. In this article, we will focus on a management strategy when the initial antiepileptic drug is unsuccessful and outline an approach that translates directly to the clinic.

Human immunodeficiency virus seroconversion presenting with acute inflammatory demyelinating polyneuropathy: a case report.

INTRODUCTION: Acute Human Immunodeficiency Virus infection is associated with a range of neurological conditions. Guillain-Barré syndrome is a rare presentation; acute inflammatory demyelinating polyneuropathy is the commonest form of Guillain-Barré syndrome. Acute inflammatory demyelinating polyneuropathy has occasionally been reported in acute Immunodeficiency Virus infection but little data exists on frequency, management and outcome. CASE PRESENTATION: We describe an episode of Guillain-Barré syndrome presenting as acute inflammatory demyelinating polyneuropathy in a 30-year-old man testing positive for Immunodeficiency Virus, probably during acute seroconversion. Clinical suspicion was confirmed by cerebrospinal fluid analysis and nerve conduction studies. Rapid clinical deterioration prompted intravenous immunoglobulin therapy and early commencement of highly active anti-retroviral therapy. All symptoms resolved within nine weeks. CONCLUSION: Unusual neurological presentations in previously fit patients are an appropriate indication for Immunodeficiency-Virus testing. Highly active anti-retroviral therapy with adequate penetration of the central nervous system should be considered as an early intervention, alongside conventional therapies such as intravenous immunoglobulin.

A prospective analysis of the outcome of levetiracetam in clinical practice.

In this postlicensing surveillance study in a large unselected population, data were collected prospectively on all patients prescribed levetiracetam (LEV) at a regional epilepsy clinic over a 2-year period. Two hundred forty-five (69.2%) patients remained on LEV, with 8.8% achieving remission and some improvement in seizure control in 49.3%. Sedation was the most common adverse effect (10.7%), but mood disturbance was more likely to lead to discontinuation (4.8%). Cumulative probability of remaining on LEV at 12 months was 0.74 (95% CI 0.69 to 0.79). Factors predictive of a poorer retention were a greater number of previous antiepileptic drugs and a faster initial titration regimen.

The coexistence of idiopathic generalized epilepsy and partial epilepsy.

PURPOSE: To describe the clinical, EEG, and imaging data of a series of patients with features of both idiopathic generalized epilepsy (IGE) and partial epilepsy. METHODS: A computerized database of all patients attending the regional epilepsy clinic was used to identify all patients with IGE. Case notes were reviewed, and cases with clinical evidence of co-existing partial epilepsy identified. RESULTS: Nine cases with clinical features of a partial and generalized-onset epilepsy and with electrophysiological evidence of IGE are presented. This represented <1% of the overall IGE population in the clinic. Five (55.6%) patients are currently in remission, with successful epilepsy surgery in one patient. CONCLUSIONS: The coexistence of IGE and partial epilepsy is uncommon, and if appropriate treatment with a broad-spectrum antiepileptic drug is given, then the prognosis can be good. Surgery should be considered for those cases with medically intractable partial-onset seizures as part of this syndrome.