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The multifaceted challenges of rare disease research remain, leaving a large proportion of rare disease patients without treatment options. This study aims to identify the challenges of rare diseas...

Background:Concerns regarding potential neurological complications of COVID-19 are being increasingly reported, primarily in small series. Larger studies have been limited by both geography and specialty. Comprehensive characterisation of clinical syndromes is crucial to allow rational selection and evaluation of potential therapies. The aim of this study was to investigate the breadth of complications of COVID-19 across the UK that affected the brain.Methods:During the exponential phase of the pandemic, we developed an online network of secure rapid-response case report notification portals across the spectrum of major UK neuroscience bodies, comprising the Association of British Neurologists (ABN), the British Association of Stroke Physicians (BASP), and the Royal College of Psychiatrists (RCPsych), and representing neurology, stroke, psychiatry, and intensive care. Broad clinical syndromes associated with COVID-19 were classified as a cerebrovascular event (defined as an acute ischaemic, haemorrhagic, or thrombotic vascular event involving the brain parenchyma or subarachnoid space), altered mental status (defined as an acute alteration in personality, behaviour, cognition, or consciousness), peripheral neurology (defined as involving nerve roots, peripheral nerves, neuromuscular junction, or muscle), or other (with free text boxes for those not meeting these syndromic presentations). Physicians were encouraged to report cases prospectively and we permitted recent cases to be notified retrospectively when assigned a confirmed date of admission or initial clinical assessment, allowing identification of cases that occurred before notification portals were available. Data collected were compared with the geographical, demographic, and temporal presentation of overall cases of COVID-19 as reported by UK Government public health bodies.Findings:The ABN portal was launched on April 2, 2020, the BASP portal on April 3, 2020, and the RCPsych portal on April 21, 2020. Data lock for this report was on April 26, 2020. During this period, the platforms received notification of 153 unique cases that met the clinical case definitions by clinicians in the UK, with an exponential growth in reported cases that was similar to overall COVID-19 data from UK Government public health bodies. Median patient age was 71 years (range 23–94; IQR 58–79). Complete clinical datasets were available for 125 (82%) of 153 patients. 77 (62%) of 125 patients presented with a cerebrovascular event, of whom 57 (74%) had an ischaemic stroke, nine (12%) an intracerebral haemorrhage, and one (1%) CNS vasculitis. 39 (31%) of 125 patients presented with altered mental status, comprising nine (23%) patients with unspecified encephalopathy and seven (18%) patients with encephalitis. The remaining 23 (59%) patients with altered mental status fulfilled the clinical case definitions for psychiatric diagnoses as classified by the notifying psychiatrist or neuropsychiatrist, and 21 (92%) of these were new diagnoses. Ten (43%) of 23 patients with neuropsychiatric disorders had new-onset psychosis, six (26%) had a neurocognitive (dementia-like) syndrome, and four (17%) had an affective disorder. 18 (49%) of 37 patients with altered mental status were younger than 60 years and 19 (51%) were older than 60 years, whereas 13 (18%) of 74 patients with cerebrovascular events were younger than 60 years versus 61 (82%) patients older than 60 years. Interpretation:To our knowledge, this is the first nationwide, cross-specialty surveillance study of acute neurological and psychiatric complications of COVID-19. Altered mental status was the second most common presentation, comprising encephalopathy or encephalitis and primary psychiatric diagnoses, often occurring in younger patients. This study provides valuable and timely data that are urgently needed by clinicians, researchers, and funders to inform immediate steps in COVID-19 neuroscience research and health policy. Funding: None.

Reply by the current author to the comments made by Mark A Oldham et al (see record 2020-79526-008) and Dorothy Wade et al (see record 2020-79526-009) on the original article (see record 2020-71085-023) We thank Dorothy Wade and colleagues and Mark Oldham and colleagues for their recognition of the cross-speciality effort of members of the UK's major professional neuroscience bodies who undertook this challenging UK-wide study during the exponential phase of the COVID-19 pandemic We also welcome the involvement of geriatricians and psychologists in future research We agree that delirium is common, especially in severe infections and in the intensive care unit As stated in our Article, we acknowledge that the study might have not recorded all such cases Changes in mental status with clear and recognised risk factors were not the focus of this study We agree that if such commonly observed complications were included, they might have substantially increased the number of patients recruited, mirroring the situation in other critical illnesses In this situation, the burden of CNS complications arising from COVID-19 would be even greater than we found in our study (PsycInfo Database Record (c) 2020 APA, all rights reserved)

Driving restrictions faced by people with epilepsy (PWE) represent a crucial and modifiable factor that predicts their social participation. In the case of refractory epilepsy, a person may lose the ability to drive forever—a handicap associated with significant detrimental effects, including reduced employability and a lower income.1 Encouragingly, progress in self-driving car technology provides renewed hope for people restricted from driving. A self-driving car is any car in which steering or acceleration/deceleration are controlled by the vehicle, but relevant to PWE are those that require no human input to drive: fully autonomous vehicles . The UK government aims to have self-driving cars on the road by 2021.2 Promisingly, Department of Transport guidelines stipulate: ‘ It would seem reasonable to allow ownership or use of a fully automated vehicle without the need to hold a driving license ’.3 This could mean that all PWE will be able to ‘drive’ in the imminent future, and although this innovation is exciting, it is important to consider the views of PWE. Eight PWE (or carers of) were recruited (four men, four women) between the ages of 31 years and 68 years. Three detailed semistructured focus groups were conducted. Recruitment and group interviews were continued until no new themes were identified. To ensure that all parties were …

Background: Increasingly neurological complications of COVID-19 are identified, mostly in small series. Larger studies have been limited by both geography and specialty.Consequently, the breadth of complications is not represented. Comprehensive characterization of clinical syndromes is critical to rationally select and evaluate potential therapies.Methods: During the exponential pandemic phase, we developed coordinated online portals for rapid notification across the spectrum of major UK neuroscience bodies, representing neurology, stroke, psychiatry, and intensive care. Evidence of infection and clinical case definitions were applied prospectively. Cases were compared to overall Government Public Health COVID-19 reporting.Findings: Within three weeks, 153 cases were notified, both geographically and temporally representative of overall COVID-19 Public Health reports. Median (range) age was 71 (23-94) years. 77 (62%) had a cerebrovascular event: 57 (74%) ischemic strokes, nine (12%) intracerebral hemorrhages, and one CNS vasculitis.The second most common group were 39 (31%) who had altered mental status, including 16 (41%) with encephalopathy of whom seven (44%) had encephalitis. The remaining 23 (59%) had a psychiatric diagnosis of whom 21 (92%) were new diagnoses; including ten (43%) with psychosis, six (26%) neurocognitive (dementia-like) syndrome, and 4 (17%) an affective disorder. Cerebrovascular events predominated in older patients. Conversely, altered mental status, whilst present in all ages, had disproportionate representation in the young.Interpretation: This is the first nationwide, cross-specialty surveillance study of acute complications of COVID-19 in the nervous system. Alteration in mental status was common, reflecting encephalopathy/encephalitis and primary psychiatric diagnoses, often in young patients.These data provide valuable and timely information urgently needed by clinicians, researchers, and funders to inform immediate steps in COVID-19 neuroscience research and health policy throughout the areas of neurology and neuropsychiatry.

Ventricular noncompaction is a kind of cardiomyopathy which is called Noncompaction Cardiomyopathy (NCC). In this condition in which the muscular wall of the main pumping chamber of the heart -the left ventricle (LV) appears to be spongy and “non-compacted”, consisting of a meshwork of numerous muscle bands called trabeculations. Here, anatomically LV wall has deep trabeculations. This condition is associated major clinical problems like systolic and diastolic dysfunction, arrhythmia and even systemic embolism. Sudden cardiac death may occur in this group. Early detection of this condition may help to plan the management. DOI: http://dx.doi.org/10.3329/uhj.v7i1.10209 UHJ 2011; 7(1): 39-41