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Isolated left ventricular non-compaction (ILVNC) is a rare congenital cardiomyopathy and is associated with arrhythmias, heart failure and thromboembolism including ischaemic stroke. Pregnancy is a relative contraindication to thrombolysis for acute ischaemic stroke, although case reports suggest the treatment can be given in selected cases. We report a case of recurrent cryptogenic strokes in a 36-year-old female who was thrombolysed with good outcome at 37 weeks’ gestation and was eventually found to have ILVNC as the cause. She had a predilection to recurrent posterior circulatory strokes due to foetal posterior communicating arteries. To our knowledge this is the first case report of safe thrombolysis for acute ischaemic stroke in pregnancy caused by ILVNC.

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare prion disease that causes rapidly progressive fatal neurodegeneration. The rarer Heidenhain variant of sCJD presents with visual symptoms and is rarely reported in the literature from sub-Saharan Africa. We report the case of a 57-year-old male with a three-week history of losing direction when driving home and visual hallucinations described as seeing rainbows. Magnetic resonance imaging (MRI) of the brain revealed unilateral parieto-occipital sulcal hyperintensities with restriction on diffusion-weighted imaging (DWI), and electroencephalography (EEG) showed right para-central slowing leading to an initial diagnosis of non-convulsive status epilepticus. He was treated with anti-epileptic medication but was re-admitted less than a month later with worsening spatial memory, aggression, ataxia, dysarthria, myoclonic jerks and a positive startle response, later developing generalised tonic-clonic seizures. Repeat MRI brain scan showed widespread posterior-predominant sulcal DWI restriction in a cortical ribboning pattern pathognomonic for sCJD. EEG showed diffuse slowing, and cerebrospinal fluid was analyzed for abnormal prion protein using real-time quaking-induced conversion but was inconclusive due to suboptimal sample collection. The patient fulfilled the diagnostic criteria for probable sCJD, Heidenhain variant; the family declined brain biopsy for definitive diagnosis. He was subsequently palliated at a local hospice where he died approximately three months after the onset of symptoms. Our case highlights the presence of a rare form of sCJD, and the diagnostic challenges faced in our resource-limited setting.

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.

Chronic headache can be a presenting manifestation of Takayasu arteritis, although patients usually have other characteristic features. A thorough clinical assessment remains key in the evaluation of chronic headache., Careful history taking and physical examination are invaluable in diagnosis of secondary causes of chronic headache.

In a patient with Collet‐Sicard syndrome and multiple myeloma, both extramedullary plasmacytomas and internal jugular vein‐sigmoid sinus thrombosis should be considered as they can coexist.

Background\ud Dissociative seizures are paroxysmal events resembling epilepsy or syncope with characteristic features that allow them to be distinguished from other medical conditions. We aimed to compare the effectiveness of cognitive behavioural therapy (CBT) plus standardised medical care with standardised medical care alone for the reduction of dissociative seizure frequency.\ud \ud Methods\ud In this pragmatic, parallel-arm, multicentre randomised controlled trial, we initially recruited participants at 27 neurology or epilepsy services in England, Scotland, and Wales. Adults (≥18 years) who had dissociative seizures in the previous 8 weeks and no epileptic seizures in the previous 12 months were subsequently randomly assigned (1:1) from 17 liaison or neuropsychiatry services following psychiatric assessment, to receive standardised medical care or CBT plus standardised medical care, using a web-based system. Randomisation was stratified by neuropsychiatry or liaison psychiatry recruitment site. The trial manager, chief investigator, all treating clinicians, and patients were aware of treatment allocation, but outcome data collectors and trial statisticians were unaware of treatment allocation. Patients were followed up 6 months and 12 months after randomisation. The primary outcome was monthly dissociative seizure frequency (ie, frequency in the previous 4 weeks) assessed at 12 months. Secondary outcomes assessed at 12 months were: seizure severity (intensity) and bothersomeness; longest period of seizure freedom in the previous 6 months; complete seizure freedom in the previous 3 months; a greater than 50% reduction in seizure frequency relative to baseline; changes in dissociative seizures (rated by others); health-related quality of life; psychosocial functioning; psychiatric symptoms, psychological distress, and somatic symptom burden; and clinical impression of improvement and satisfaction. p values and statistical significance for outcomes were reported without correction for multiple comparisons as per our protocol. Primary and secondary outcomes were assessed in the intention-to-treat population with multiple imputation for missing observations. This trial is registered with the International Standard Randomised Controlled Trial registry, ISRCTN05681227, and ClinicalTrials.gov, NCT02325544.\ud \ud Findings\ud Between Jan 16, 2015, and May 31, 2017, we randomly assigned 368 patients to receive CBT plus standardised medical care (n=186) or standardised medical care alone (n=182); of whom 313 had primary outcome data at 12 months (156 [84%] of 186 patients in the CBT plus standardised medical care group and 157 [86%] of 182 patients in the standardised medical care group). At 12 months, no significant difference in monthly dissociative seizure frequency was identified between the groups (median 4 seizures [IQR 0–20] in the CBT plus standardised medical care group vs 7 seizures [1–35] in the standardised medical care group; estimated incidence rate ratio [IRR] 0·78 [95% CI 0·56–1·09]; p=0·144). Dissociative seizures were rated as less bothersome in the CBT plus standardised medical care group than the standardised medical care group (estimated mean difference −0·53 [95% CI −0·97 to −0·08]; p=0·020). The CBT plus standardised medical care group had a longer period of dissociative seizure freedom in the previous 6 months (estimated IRR 1·64 [95% CI 1·22 to 2·20]; p=0·001), reported better health-related quality of life on the EuroQoL-5 Dimensions-5 Level Health Today visual analogue scale (estimated mean difference 6·16 [95% CI 1·48 to 10·84]; p=0·010), less impairment in psychosocial functioning on the Work and Social Adjustment Scale (estimated mean difference −4·12 [95% CI −6·35 to −1·89]; p

Phenytoin is one of the most commonly used anticonvulsants in the developing world, but lack of monitoring and concurrent medications can easily lead to toxicity. We report the case of a 35-year-old female on phenytoin for symptomatic epilepsy due to previously treated glioblastoma multiforme, who presented with status epilepticus 1 week after being treated for a urinary tract infection. She was loaded with phenytoin and levetiracetam as per emergency protocol but had a persistently low level of consciousness, and her preloading phenytoin level result came back in the toxic range. She was managed conservatively, but after 4 days with no change she was dialyzed and her level of consciousness improved within 24 h, allowing for safe discharge home shortly after. Our case illustrates the option of haemodialysis in phenytoin-toxic patients who do not improve with conservative measures or who may need urgent reduction due to potentially fatal complications of phenytoin toxicity.

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.

When an unidentified patient who cannot communicate presents with symptoms and signs suggesting an acute stroke, the decision to thrombolyse is a particular challenge. In a time-pressured environment, clinicians need clear thought processes for diagnosis and treatment. Ethical considerations, diagnosis, identity and previous history, contraindications, time of symptom onset (EDICT) can help decision-making in this situation.

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

We introduce the ABN Sustainability SIG. We present our aims & objectives, and practical ways of implementing sustainability strategies in Neurology.At the 2016 ABN annual meeting, a keynote speech by Dr. David Pencheon, then director of the National Health Service Sustainability Development Unit, highlighted the relevance and importance of Sustainability in Neurology. This planted the seed for our SIG’s formation. Initial interest was gathered from an ABN newsletter notice, via word-of-mouth and informal discussions at ABN annual meetings (2017, 2018). A series of teleconferences & email discussions enabled the formation of SIG byelaws and application to the ABN council.Our aims and objectivesTo be a positive force within the ABN to highlight issues surrounding global environmental sustainability.To provide a forum to consider the impact of choices made in neurology practice on global environmental sustainability. This will cover all aspects of neurology including, but not limited to, clinical practice, service provision, technological and digital developments, meetings and ABN resources and investments.To identify areas where the choice made could impact positively on global environmental sustainability and disseminate this information to the ABN membership to inform their decisions.

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.

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

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