Search

Purpose Epilepsy is a chronic neurological disorder that is often diagnosed in childhood and may negatively impact physical, social and psychological abilities. Most tools measuring quality of life (QoL) rely on parent/caregiver feedback rather than the child’s perspective. CHEQOL-25 is a QoL tool that documents both child and caregiver perspectives across five domains. The primary objective was to determine the QoL of children living with epilepsy (CWE) using the CHEQOL-25 tool in a Kenyan paediatric population. Other objectives were to describe the correlation between the caregivers’ and children’s’ perspectives and describe factors affecting QoL. Method We conducted a cross-sectional study across four sites in Nairobi. Quantitative data was collected using a self-administered CHEQOL-25 questionnaire. Caregivers and their children aged 7–15 years attending neurology clinics participated in the study. We used Kappa statistics to compare child and caregiver responses. Results A total of 354 participants were interviewed (177 children and 177 caregivers). A good QoL was reported by 60.5 % of children with a similar caregiver perception of 56.5 %. Caregivers with little education and male caregivers were associated with a poor QoL (p = 0.01); other socio-demographic factors had little impact on the measured QoL of CWE. Parent and child questionnaires correlated well in terms of response in terms of interpersonal (p = 0.001) and intrapersonal (p = 0.004) domains. Conclusion This study demonstrated that a good quality of life was reported by the majority of CWE and their caregivers, although some factors such as a male caregiver gender and lower level of education were associated with poor QoL.

Background Neuromyelitis optica spectrum disorder (NMOSD) is an auto-immune disease of the central nervous system (CNS) associated with the IgG-antibody against aquaporin-4 (AQP4-IgG). There is little published epidemiology of NMOSD from sub-Saharan Africa (SSA). Methods We retrospectively collated NMOSD cases admitted to our tertiary regional neurology centre. Results We identified 11 cases (10 female, average age 30 years). 64% (7/11) were seropositive for AQP4-IgG, measured using indirect immunofluorescence. The remaining cases could either not afford tests, or had pathognomonic radiological features. 57% (4/7) of seropositive cases had concurrent/recent CNS infection. All patients were treated with high-dose intravenous methylprednisolone (IVMP), and 36% (4/11) also had plasma exchange. Only 55% (6/11) of the patients were seen by a neurologist at presentation: they had less relapses (1.3 vs 2.4), less diagnostic delay (2.3 vs 7.4 months), and were less disabled at the end of our review period. 10 cases were immunosuppressed long-term: 60% on mycophenolate, 30% azathioprine, and one on rituximab. Conclusion Our study is the largest case series of NMOSD from the East Africa region. Patients faced challenges of access to appropriate and affordable testing, and timely availability of a neurologist at onset, which had impacts on their functional outcomes. The majority of the seropositive cases had recent/concurrent CNS infections, suggesting triggered auto-immunity., Highlights • This is the largest case series of NMOSD from the East Africa region. • Early involvement of neurologists translated to early diagnoses and better outcomes. • There remain challenges in accessing appropriate diagnostics for NMOSD. • Half the seropositive cases had recent infections, suggesting triggered auto-immunity.

Question design during history-taking has clear implications for patients' ability to share their concerns in general and their seizure experiences in particular. Studies have shown that unusually open questions at the start of the consultation enable patients to display interactional and linguistic markers which may help with the otherwise challenging differentiation of epileptic from nonepileptic seizures (NES). In this study, we compared the problem presentation approach taken by trainee neurologists in outpatient encounters with new patients before and after a one-day conversation analytic training intervention in which doctors were taught to adopt an open format of question design and recognize diagnostically relevant linguistic features. We audio/video-recorded clinical encounters between ten doctors, their patients, and accompanying persons; transcribed the interactions; and carried out quantitative and qualitative analyses. We studied 39 encounters before and 55 after the intervention. Following the intervention, doctors were significantly more likely to use nondirective approaches to soliciting patient accounts of their presenting complaints that invited the patient to describe their problems from their own point of view and gave them better opportunity to determine the initial agenda of the encounter. The time to first interruption by the doctor increased (from 52 to 116 s, p

The diagnostic distinction between epilepsy and psychogenic nonepileptic seizures (PNES) can be challenging. Previous studies have demonstrated that experts in conversation analysis can identify linguistic and interactional features in transcripts and recordings of interviews with patients that reliably distinguish between epilepsy and PNES. In this study, ten senior neurology trainees took part in a one-day intervention workshop about linguistic and interactional differences in the conversation behavior of patients with epilepsy and those with PNES. Participants were familiarized with a 12-item questionnaire designed to capture their conversational observations immediately after talking to a patient with seizures. After the intervention, 55 initial outpatient visits of patients referred to seizure clinics were video and audio recorded. All medical diagnoses were confirmed two years after initial presentation on the basis of a chart review (including MRI and EEG findings) by a fully trained epilepsy expert. Postvisit questionnaires relating to patients confirmed to have epilepsy (n=20) or PNES (n=13) were analyzed. Doctors' mean responses to 6 of the 12 questions about linguistic and interactional observations differed significantly between the groups with epilepsy and PNES. Receiver operating curve analysis showed that a summation scale based on items demonstrating significant between-group differences correctly classified 81.8% of patients as having epilepsy or PNES. This study shows that a brief Conversation Analytic teaching intervention can enable neurologists to identify linguistic and interactional features supporting the differentiation of epilepsy and PNES as they take their patients' history in routine seizure clinic consultations, potentially improving diagnostic accuracy.

In schizophrenia, auditory verbal hallucinations (AVHs) are likely to be perceived as gender-specific. Given that functional neuro-imaging correlates of AVHs involve multiple brain regions principally including auditory cortex, it is likely that those brain regions responsible for attribution of gender to speech are invoked during AVHs. We used functional magnetic resonance imaging (fMRI) and a paradigm utilising 'gender-apparent' (unaltered) and 'gender-ambiguous' (pitch-scaled) male and female voice stimuli to test the hypothesis that male and female voices activate distinct brain areas during gender attribution. The perception of female voices, when compared with male voices, affected greater activation of the right anterior superior temporal gyrus, near the superior temporal sulcus. Similarly, male voice perception activated the mesio-parietal precuneus area. These different gender associations could not be explained by either simple pitch perception or behavioural response because the activations that we observed were conjointly activated by both 'gender-apparent' and 'gender-ambiguous' voices. The results of this study demonstrate that, in the male brain, the perception of male and female voices activates distinct brain regions.

Navigating the online galaxy for up-to-date evidence-based knowledge in clinical neurology can be a challenge. Practical Neurology is a noted and popular online clinical resource,1 which according to its website is "…practical in the sense of being useful for everyone who sees neurological patients and who want to keep up to date, and safe, in managing them." As UK-based neurology trainees, we have been fortunate to have automatic access to Practical Neurology during our training. We found that the articles adequately covered most elements in our syllabus and were ideal reference material for our department. This accords with the recent readership survey: for almost 80% of readers, the journal impacts positively on their clinical work, contains reliable information and reflects real-life clinical neurology practice.2 Therein lay an opportunity to address the challenge of how to navigate online resources for high-standard reference material: that is, by using Practical Neurology as a standard knowledge base. We set about arranging all the articles from the …

Multiple sclerosis (MS) may run a rapidly progressive monophasic course (the Marburg, or malignant form), that carries a poor prognosis [1]. Autologous hematopoietic stem cell transplant (AHSCT), initially utilised in chronic MS [2], is now occasionally administered in acute aggressive relapsing–remitting MS (RRMS) [3]. We describe a case of malignant-type MS treated with pulsed cyclophosphamide (CY) and non-myeloablative AHSCT. A 21-year-old woman, presented with sensorimotor disturbance to all four limbs in April 2011. Neuro-imaging demonstrated demyelination with enhancement (Fig. 1a), cerebrospinal fluid (CSF) demonstrated 47 lymphocytes and was positive for oligoclonal bands. Serum was negative for aquporin4 (Aq4) antibodies. Three days of 1 g IV methylprednisolone (IVMP) failed to slow progression. A second course of IVMP 5 weeks later was unsuccessful, as were seven sessions of plasma exchange undertaken over a 2-week period and the patient became tetraplegic. A 5-day course of alemtuzumab with pulsed IVMP was completed (week 9) but deterioration continued with decreased visual acuity (right eye: counting fingers, left eye: 6/36), vertigo and respiratory compromise requiring ventilatory support. MRI demonstrated new lesions (Fig. 1b). Repeat CSF revealed 10 lymphocytes and Aq4 was negative in serum and CSF. Alemtuzumab can take up to 3 months to take effect, however, in July 2011 (13 weeks after presentation) treatment was intensified due to continued decline and stem cells mobilised with CY 2 g/m plus G-CSF (10 lg/kg/day). Stem cell harvest was undertaken 2 weeks later. A second dose of CY (2 g/m) 4 weeks poststem cell harvest was followed by improvement—return of sensorimotor function to the left arm, cessation of vertigo and weaning from ventilatory support. Two months after first treatment with CY the patient underwent AHSCT using CY 200 mg/kg ? rabbit antithymocyte globulin (ATG, total dose 6 mg/kg). At this point she had an Expanded Disability Status Score (EDSS) of 8.5. Routine toxicity, including neutropenic sepsis, was managed and engraftment prompt. Four months from AHSCT, she could walk a few steps using parallel bars and had visual acuities of 6/24 (right) and 6/9 (left); EDSS 7.5. Repeat MRI demonstrated a stable lesion load (Fig. 1c). One year from the initiation of treatment with CY (8 months from AHSCT) she was able to walk 10 M with bilateral support, EDSS 6.5. Further imaging demonstrated no change. EBMT guidance [4] advises that patients with an EDSS score higher than 6.5 be excluded from AHSCT, except in instances of ‘malignant’ MS. Our patient had a rapid-onset demyelinating illness that we have termed malignant MS. We cannot exclude neuromyelitis optica (NMO), although Aq4 antibodies were consistently negative, including in CSF. In such cases, the distinction between MS, NMO and J. J. P. Alix (&) D. J. Blackburn B. Sharrack Sheffield Institute for Translational Neuroscience, University of Sheffield, 385A Glossop Road, Sheffield S10 2HQ, UK e-mail: j.alix@sheffield.ac.uk

Discerning a speaker's gender from their voice is a basic and crucial aspect of human communication. Voice pitch height, the perceptual correlate of fundamental frequency, is higher in females and provides a cue for gender discrimination. However, male and female voices are also differentiated by multiple other spectral and temporal characteristics, including mean formant frequency and spectral flux. The robust perceptual segregation of male and female voices is thought to result from processing the combination of discriminating features, which in neural terms may correspond to early sound object analysis occurring in non-primary auditory cortex. However, the specific mechanism for gender perception has been unclear. Here, using functional magnetic resonance imaging, we show that discrete sites in non-primary auditory cortex are differentially activated by male and female voices, with female voices consistently evoking greater activation in the upper bank of the superior temporal sulcus and posterior superior temporal plane. This finding was observed at the individual subject-level in all 24 subjects. The neural response was highly specific: no auditory regions were more activated by male than female voices. Further, the activation associated with female voices was 1) larger than can be accounted for by a sole effect of fundamental frequency, 2) not due to psychological attribution of female gender and 3) unaffected by listener gender. These results demonstrate that male and female voices are represented as distinct auditory objects in the human brain, with the mechanism for gender discrimination being a gender-dependent activation-level cue in non-primary auditory cortex.

BACKGROUND: UK National Guidelines (UKNG) advise HIV testing in clinically indicated neurological presentations. We audited the impact of our practical strategies to increase uptake of HIV testing at a regional acute neurology admissions unit. METHODS: We audited HIV testing in 4 periods over 2 years: before we designed a UKNG-based "HIV testing in Neurology" protocol ("pre-protocol"); after dissemination of the protocol alone ("post-protocol"); post-protocol dissemination combined with both a tailored departmental admissions clerking proforma to prompt for HIV testing & consenting, and regular focussed tutorials to doctors on HIV testing in neurological patients ("post-proforma"); and finally one year after the post-proforma period ("+1 year"). We also looked at the total number of HIV tests sent from the unit during the two-year period. We assessed significance using Fisher's exact test. RESULTS: 47.8% of all acute neurology non-stroke admissions were eligible for HIV testing during all the audit periods. Testing rates were as follows: pre-protocol 21.9%; post-protocol 36.6%; post-proforma 83.3%; and at +1 year 65.4% (p