On February 24, 2022, Russia launched a full-scale invasion of Ukraine that dominated headlines around the world. Millions of Ukrainians would flee the country, and a third of the population would be ...displaced. In the days following the invasion, Swedish migration expert Gregg Bucken-Knapp sent text messages to his Ukrainian colleagues, offering support and assistance. These were their responses. In a series of graphic vignettes, Messages from Ukraine takes the words of Ukrainian migration professionals and transforms them into snapshots of how war affects the lives of everyday people: those who are forced to flee home and seek safety elsewhere, those who choose to stay and volunteer or fight, those who witness events unfolding from afar, and those who find themselves trapped in cities under siege. Messages from Ukraine captures a moment in time to tell a timeless story about war, displacement, determination, and resilience. Proceeds from the sale of Messages from Ukraine will go to the Canada-Ukraine Foundation, a national charitable foundation that provides humanitarian aid to the people of Ukraine.
The war in Ukraine has altered the course of global history. These authors explore how.When Vladimir Putin's forces sought to conquer Ukraine in February 2022, they did more than threaten the ...survival of a vulnerable democracy. The invasion unleashed a crisis that has changed the course of world affairs. This conflict has reshaped alliances, deepened global cleavages, and caused economic disruptions that continue to reverberate around the globe. It has initiated the first great-power nuclear crisis in decades and raised fundamental questions about the sources of national power and military might in the modern age. The outcome of the conflict will profoundly influence the international balance of power, the relationship between democracies and autocracies, and the rules that govern global affairs. In War in Ukraine, Hal Brands brings together an all-star cast of analysts to assess the conflict's origins, course, and implications and to offer their appraisals of one of the most geopolitically consequential crises of the early twenty-first century. Essays cover topics including the twists and turns of the war itself, the successes and failures of US strategy, the impact of sanctions, the future of Russia and its partnership with China, and more.Contributors: Anne Applebaum, Joshua Baker, Alexander Bick, Hal Brands, Daniel Drezner, Peter Feaver, Lawrence Freedman, Francis Gavin, Brian Hart, William Inboden, Andrea Kendall-Taylor, Michael Kimmage, Michael Kofman, Stephen Kotkin, Mark Leonard, Bonny Lin, Thomas Mahnken, Dara Massicot, Michael McFaul, Robert Person, Kori Schake, and Ashley Tellis.
Myotonic Dystrophy Type 1 (DM1) is the most common muscular dystrophy in adults. Structured multi- disciplinary care is required to manage the multi-system involvement, to improve patient safety, and ...to reduce mortality and morbidity. Previous audits based on the 2018 consensus-based care recommenda- tions have been conducted at individual UK centres. In this study a standardised audit tool was created and deployed to 3 tertiary UK neuroscience centres.In total, data regarding 375 patients (197 (53%) female, mean age 47 years (range 1-84)) with DM1 were analysed. 62% had adult-onset disease. Neurology teams reviewed 64% of patients at least once a year. Key findings included: Cardiology review was performed annually for only 30%; of patients with respiratory needs, only 12% were reviewed six-monthly by specialist teams; Swallowing function was assessed in only 44% of those required; Clinical assessment for cognitive impairment was conducted in 12%.In conclusion, this multi-centre UK audit identifies several significant areas of care that need to be optimised in DM1, even within dedicated neuromuscular clinics. This highlights the need for a national initiative to establish minimum care standards. Such initiative can help drive resource allocation resulting in positive change.
Bickerstaff’s Brainstem Encephalitis (BBE) is a condition causing impairment of sensorium in association with ophthalmoplegia and ataxia. Here we present a case of BBE presenting with bilateral vocal ...cord paralysis.A 35-year-old female presented with a sore throat and shortness of breath in the context of a fall and ankle injury the previous day. She became confused and agitated in hospital. Several hours after pres- entation she was found cyanosed with stridulous breathing (with normal saturations). Fibre-optic nasola- ryngoscopy revealed bilateral vocal cord palsy. She was intubated and transferred to ICU. Investigations including a full infection screen, CSF analysis and MRI were normal and non-diagnostic. Examination during a sedation hold revealed a unilateral third, fourth, and bilateral sixth and seventh cranial nerve palsies. She had flaccid quadriparesis and was globally areflexic. Nerve conduction studies showed a motor predominant polyneuropathy. A diagnosis of BBE was made based on the clinical findings with acute confusion. She was treated with plasma exchange and immunoglobulins and was discharged with almost complete recovery.There are no previous case reports of vocal cord paralysis being the presenting feature of BBE. Neurologi- cal disease accounts for only 12% of cases of bilateral cord palsy. These causes may be systemic such as myasthenia gravis or diphtheria, or focal affecting the nucleus ambiguus in the medulla. However, clini- cians must recognise these cases promptly to ensure adequate investigation and treatment.
ObjectiveTo calculate the efficiency index (EI), a recently described metric based on test accuracy and inaccuracy, for dementia diagnosis using the mini-Addenbrooke’s Cognitive Examination (MACE), ...and to compare this with balanced EI (BEI) and unbiased EI (UEI), based on balanced and unbiased accuracy and inaccuracy measures respectively. All efficiency indexes have boundary values of 0 and ∞; inflection point at 1; where value >1 indicates correct diagnostic classification and <1 indicates incorrect classification.ResultsUsing the dataset from a large (N = 755) prospective pragmatic study of MACE, in which 114 patients received criterion diagnosis of dementia, EI was 2.81 at the optimal MACE cut-off (≤20/30, deter- mined by maximal Youden index). BEI was 4.24 and UEI was 0.61 at this cut-off. Plotting values of EI, BEI, and UEI across the range of MACE cut-offs (≤11/30 to ≤29/30), UEI values were lowest, EI values were highest at lower cut-offs and BEI values were highest at higher cut-offs. Adjusting for dementia prevalence (fixed BEI), both EI and UEI values increased with dementia prevalence, with EI>UEI.ConclusionsEfficiency indexes are easily calculatedThese unitary metrics may have greater utility than others in communicating risk of correct diagnosis versus misdiagnosis of test outcomes.
Posterior reversible encephalopathy syndrome (PRES) is a syndrome characterised by reduced GCS/confusion, seizures and visual disturbance along with characteristic MRI findings. Autoimmune disorders ...have been identified in one third of patients with PRES.Here we discuss a 33-year-old female with SLE, who presented with reduced GCS and arm weakness. She had an AKI and metabolic acidosis and was admitted to ICU. Although the patients GCS improved, ongoing upper limb weakness remained. MRI imaging to rule out cerebral lupus, showed high T2 and FLAIR signal within the deep white matter of the occipital, frontal and parietal lobes. This was suggestive of PRES. Whilst in ICU the patient developed hypertension and was started on amlodipine. The patient also had a long history of immunosuppression with mycophenolate, hydroxychloroquine and rituximab. A neurology referral was sent for further advice.Although the name suggests, PRES is often not completely reversible and is not limited to the posterior cerebral cortex, as seen here. It still remains unclear whether it is the autoimmune condition or treatment of these conditions with immunosuppressant’s that is the primary association. Case reports linking PRES with immunosuppressant therapy have shown an early onset of symptoms following treatment, rather than years.
A 31-year old male with a background of occasional Migraine without aura and mechanical lower back pain, presented with recurrent transient episodes of generalised headache, speech impairment, ...confusional state, and hemi-body sensory-motor symptoms. He had 9-10 episodes over a 2-week period. These were sudden, non-stereotypical, and lasted between 30-120 minutes. He was well in between and the very first episode happened just before his travel to the Canaries for holidays. He had 3 separate admissions with these episodes.Routine blood tests were normalContrast CT head and MRI brain showed transient generalised leptome- ningeal enhancement, CSF showed sequential WCC of 420 and 200 before returning to normal. Repeat EEG showed no epileptiform abnormality. Extensive tests for infectious agents including viral serology were all either negative or suggestive of previous exposure. Only HSV-7 PCR was positive. Inflammatory and paraneoplastic tests were negative. He fully recovered from these in 2 months with no sequelae.The clinical Syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lympho- cytosis (HaNDL) is well described, rarely seen and poorly understood. Proposed mechanisms include migraine pathophysiology, post-infectious and immunological phenomenon. This is the first report of an active HSV-7 infection, causing HaNDL.
Retrospective review of referrals to the National-Multidisciplinary-Team (NMDT) in England (& Wales), and of the clinical records of SMAtype1 patients included for Zolgensma® therapy in the UK.Data ...was available for 42 patients: 13, 12, 10, 6, 1 from Evelina-London, Sheffield, Bristol, Manchester and Belfast centres respectively.Patients’ age ranged from 2-months to 46-months and weights from 4.44kg to 13.5kg. Post-Zolgensma-infusion monitoring:Most patients had asymptomatic thrombocytopaenia in week-1, resolving by week-2. No thrombotic microangiopathy was reported. Majority developed transient transaminitis with mild/moderate elevation of AST/ALT. Some had more severe/prolonged transaminitis – Liver ultrasound, coagulation-studies and clinical examination remained normal: 11 (weight>7.5kg) had ALT-peaks of >100 IU/L; 22 (15/22 weighed >7.5kg) had AST-peaks of >100 IU/L – good response seen to doubling Prednisolone, where indicated. Echocardiograms remained normal in patients with elevated Troponin-I levels; 4 had levels >100ng/l, prednisolone doubled in one, with good response.13/42 needed doubling of Prednisolone; 12/13 had weight >7.5kg.CHOP-INTEND scores post gene-therapy were available for 22/42. Scores improved in all patients except one (difficult assessment). Improvement ranged from 2-24 points.ConclusionAll patients tolerated the Zolgensma®-therapy well and have recovered well from any transient issues. No persistent complications from gene-therapy or steroid-cover were reported.
BackgroundWe present baseline data on the first 50 participants recruited to the UK MS Pregnancy Register.MethodsData collected via questionnaires from consenting participants until 20th December ...2021 were included.Results50 participants (all with relapsing remitting MS; mean age at diagnosis 28.0 years; mean age at recruitment 33.0 years) were included. Median EDSS was 2.5 (n=16). Gestation at recruitment ranged from 2 to 40 weeks. 78% had discussed their pregnancy in advance with their MS team. 90% of patients had ever taken DMT. Of the patients that stopped DMT (n=23), 16 reported stopping for pregnancy-related reasons. Of these, 39% stopped before pregnancy and 30% following conception. 15 women are continuing DMT during their current pregnancy, taking the following DMT: Glatiramer acetate (n=5), natalizumab (n=7), Peginterferon beta-1a (n=2), not recorded (n=1). In those with prior pregnancies, 61% (14/23) reported pregnancy loss with 1 case of a rare genetic condition in the baby. None of the prior pregnancy losses happened whilst on DMT. One participant reported previous PPH and foetal macrosomia and another reported previous pre-eclampsia.ConclusionsThese results show that a patient-facing pregnancy MS registry is feasible and can collect previous adverse pregnancy outcomes. Future results will inform clinicians and women about the safety of DMT and adjunctive medication during pregnancy and postpartum.
IntroductionHNPP is an autosomal dominant disorder with estimated prevalence between 0.84- 16/100,000, commonly presenting as mononeuropathies involving pressure-prone areas. Presentation as both a ...brachial plexopathy and spinal accessory neuropathy is rare, and previously unreported.Case ReportA 30-year old female with no known medical co-morbidities presented with a 3-month history of right shoulder pain and weakness, and intermittent numbness of the elbow and hand. Examina- tion showed wasting of right trapezius and spinati muscles, shoulder droop, and weakness involving the right sternocleidomastoid, shoulder, and median and ulnar-supplied hand muscles. Nerve conduction studies and electromyography revealed axonal-loss pattern neuropathy of right suprascapular and spinal accessory nerves. Focal demyelination was seen in bilateral median and ulnar nerves across the wrists and elbows. Needle EMG showed denervation changes in right infraspinatus and trapezius muscles. Sub- sequent genetic testing of patient and her mother revealed deletion of chromosome 17p11.2 including PMP22. She improved clinically following a period of intensive physiotherapy.ConclusionHNPP typically affects pressure-prone sites such as the median, ulnar or peroneal nerves. Brachial plexopathy is uncommon, and involvement of spinal accessory nerve is very rare. Recognition of symptoms, with appropriate targeted electrodiagnostic and genetic investigations is important in achieving an accurate diagnosis and management.
