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Sickle cell disease (SCD) is the most common type of hereditary anaemia and genetic disorder worldwide. Cerebrovascular disease is one of its most devastating complications, with consequent increased morbidity and mortality. Current guidelines suggest that children and adults with SCD who develop acute ischaemic stroke should be transfused without delay. Those with acute ischaemic stroke aged over 18 years who present within 4.5 hours of symptom onset should be considered for intravenous thrombolysis; older patients with conventional vascular risk factors are the most likely to benefit. Endovascular thrombectomy should be considered carefully in adults with SCD as there are few data to guide how the prevalence of cerebral vasculopathy may confound the expected benefits or risks of intervention. We present a practical approach to cerebrovascular disease in sickle cell patients based on the available evidence and our experience.

Spontaneous spinal cord infarction is significantly less common than cerebrovascular disease. Because of the tight anatomical distribution of pathways in the cord, small spinal cord infarcts usually give more obvious symptoms and signs than similar lesions in the brain. Large epidemiological stroke studies have generally not included spinal cord stroke and so the incidence of vascular syndromes in the spinal cord is unknown. Management and prevention strategies for spontaneous spinal cord infarcts stem from small case series and case reports. Patient outcomes from spinal cord infarction are better with prompt recognition, timely management and prevention of associated medical complications arising from paraplegia, tetraplegia, neurogenic bladder and bowel dysfunction. The process of rehabilitation following spinal cord infarction is an evolving area.

Patent foramen ovale (PFO) is the most common anatomical cause of an interatrial shunt. It is usually asymptomatic but may cause paradoxical embolism, manifesting as stroke, myocardial infarction or visceral/peripheral ischaemia. PFO is a risk factor for stroke and may be associated with migraine with aura. New evidence suggests PFO closure reduces the risk of recurrent ischaemic stroke in a highly selected population of stroke survivors: those aged 60 years or younger with a cryptogenic stroke syndrome, a large right-to-left shunt, an atrial septal aneurysm and no evidence of atrial fibrillation. They benefit from percutaneous PFO closure in addition to antiplatelet therapy, rather than antiplatelet therapy alone. Current evidence does not support PFO closure in the treatment of migraine.

There is no consensus on how to structure and deliver neurology training. The General Medical Council’s annual National Training Survey indicates that the quality of UK neurology training is very variable, but does not explain this variation. We used the survey data to identify the four highest and lowest performing sites for neurology training across the UK. We conducted semistructured interviews with groups of local trainees and, separately, local trainers in an exploratory qualitative study, and identified common themes across a range of aspects of neurology training. Here we present our findings, share case studies from top-performing sites and make recommendations on how best to train a neurologist.

Vijyarangam Senthilkumar Shanmugam (“Kumar”) was an incredible, brave, and sincere doctor. He completed his medical training in 2000, graduating from Madurai Medical College in India. On the eve of his final practical MBBS examination, he was riding his “bullet” motorbike to pay a visit to the temple in Madurai. On the way, he was forced to swerve to avoid some boys playing a ball game, one of whom ran out into the road. He crashed, breaking his forearm. He abandoned the motorbike and returned to his digs, where he attempted to reset his arm by himself. Eventually, his friends persuaded him …

Introduction Inter-professional simulation immerses participants in realistic scenarios in a safe and reproducible environment. Its use in neurology education is limited. Learning Objectives: (i) Technical skills and knowledge in neurological emergencies (ii) Non-technical skills including communication and leadership. Methods Three acute neurology scenarios (refractory status epilepticus, coma and neuromuscular respiratory failure) with high fidelity mannequin. 30 participants (7 nursing; 8 physician9s associates; 15 medical). Mixed-methods evaluation before and after training and statistical analysis with Mann–Whitney U test. Results Analysis of pre and post course questionnaires. 56%(14/25) had previous simulation experience. On scale of 1=poor to 7 good: scored 5.66 (SD±1.14) for enjoyment and 6.28 (SD±1.21) for relevance to clinical practice. Improvement in technical skills with increased confidence in managing emergency neurology situations (pre-course: 3.5 [SD±1.45] post-course:4.63 [SD±0.956] p=0.00736). No improvement in non-technical skills:communication skills (pre-course: 4.31 [SD±1.12] post-course: 4.75 [SD±0.737] p=0.187 NS) and leadership skills (pre-course: 3.92 [SD±1.13] post-course: 4.33 [SD±0.868] p=0.271 NS). Discussion Simulation improved technical but not non-technical skills. Participants started with a higher opinion of their non-technical skill than their knowledge. Knowledge is learnt during a scenario but improvement in non-technical skills are only realised later so maybe underestimated. In future sessions we will add an introduction about non-technical skills and use the diamond debrief approach.

Cerebral fat embolism can complicate long bone fractures and orthopaedic surgery. We present two patients with this syndrome who developed coma following long bone fracture or hip replacement surgery. Patient 1 was a 71 year old previously independent lady who was admitted following a fall and underwent total hip replacement surgery for a left neck of femur fracture. Immediately after cementing the shaft, she developed marked hypoxia, tachycardia, hypotension and pyrexia and remained intubated and sedated after surgery. Following withdrawal of sedation, she remained unconscious. Neurological examination revealed a GCS of 3/15, roving eye movements, intact brainstem reflexes, increased tone on the left side and up–going plantars. Over the next few days her GCS remained low at 5/15, with flexor motor response to pain. EEG was diffusely slow and compatible with an encephalopathic process. MRI showed innumerable small emboli on DWI (“star–field pattern”), and multiple, hyper–intense lesions on T2 and FLAIR. Bubble echo was positive and suggestive of a patent foramen ovale. Over the next four weeks, she made a dramatic recovery and one month after surgery was obeying commands, verbally communicating, oriented in time but disoriented in place. Patient 2 was a 66 year old, previously independent lady who was admitted with a femoral shaft fracture. The day following her admission, her GCS dropped to 7/15 and she developed hypoxia, tachycardia and pyrexia. Neurological examination initially did not reveal a focal deficit. During a transient improvement in GCS to 14/15, she was noted to have a right sided face and arm weakness. MRI DWI again showed the characteristic “star–field pattern”. Clinically she deteriorated again with a drop in her GCS, development of thrombocytopaenia, pulmonary emboli and nephrogenic diabetes insipidus and remained on ITU at the time of writing this abstract. Cerebral fat embolism syndrome may be under–diagnosed in clinical practice. It should be considered in the differential of coma following orthopaedic surgery and fractures. Neurological outcome has been reported to be generally good.

A 24-year-old female Punjabi student came to the UK in 2010. A right hilar mass on routine CXR and respiratory physicians performed mediastinoscopy. Right paratracheal lymph node histology demonstrated non-caseating granulomatous lymphadenitis with central necrosis. TB culture was negative. She was well and followed up with presumed quiescent sarcoidosis or TB. In September 2011, she reported 2 months of headache, 5 weeks of right eye swelling and a persistent cough. She had a right exopthalmos with left-sided weakness especially the arm. MRI brain demonstrated six enhancing intra-axial masses within the right hemisphere, six in the cerebellum and one lesion extending into the medulla. Abnormal tissue expanded into the right orbit causing exopthalmos. HIV status was negative and she was not diabetic. She was commenced on steroids and empirical quadruple therapy for TB. The presumed differentials were sarcoidosis, TB or possible malignancy Bone marrow trephine was normal (no evidence of lymphoma or organisms). Biopsy of the orbital lesion contained numerous, well preserved, fungal hyphae within the granulomas. IgE was 2000 kU/L (0–81), aspergillus precipitins >200 (0–40). Culture was positive for aspergillus flavus. Histological re-examination of the original chest lesion also confirmed aspergillus hyphae. This is a case of disseminated aspergillus infection. The patient responded well to antifungal treatment with ambisome, andulifungin IV, voroconizole and dexamethsone with resolution clinically and radiologically.

A 23-year-old left-handed female developed progressive right arm weakness and expressive dysphasia. She had papilloedema and right-sided UMN facial palsy, arm weakness and positive Babinski reflex. MRI brain demonstrated a 7.7 cm diameter heterogeneously enhancing mass lesion with surrounding vasogenic oedema lying within the left posterior frontal lobe with associated midline shift. CSF contained unmatched oligoclonal bands, consistent with a diagnosis of tumefactive multiple sclerosis. She was treated with IV methylprednisolone with complete recovery. Three years later she presented with dizziness, visual disturbance and cognitive deficits. On examination, she had a left homonymous hemianopia and a Gerstmann-like syndrome. She rapidly deteriorated with left-sided weakness, neglect, hemisensory loss, aphasia and dysphagia. MRI demonstrated an acute, demyelinating plaque of 7.2 cm involving the right parieto-occipital white matter and posterior frontal white matter with mass effect. Repeat CSF examination showed unmatched oligoclonal bands. She was treated with intravenous methylprednisolone and plasma exchange and dramatically improved. She is continuing her inpatient rehabilitation. Tumefactive lesions in MS usually convert to typical relapsing-remitting disease on recurrence. Here we present a rare case of relapsing-remitting tumefactive disease which has only previously been reported once in the literature. We have demonstrated a good response to immunosuppressive and immunomodulatory therapy.

Stroke has become a major NHS priority. Daniel Davies and colleagues look at a career in the area

We enjoyed A practical approach to acute vertigo by Seemungal and Bronstein in the August issue. However, we disagree that the head impulse test “will only be positive in patients with vestibular neuritis”1 and so …