To explore the efficacy of spinal MRI study with subtraction analysis as a rapid, reliable, and noninvasive procedure to detect epidural CSF collection in spontaneous intracranial hypotension (SIH) ...syndrome.
Seventeen patients (mean age 42 years, age range 17-65 years; 11 female) with SIH diagnosed using the International Classification of Headache Disorders criteria and 13 age-matched control subjects underwent standard sagittal spinal MRI. Postprocessing image analysis with subtraction of T1-weighted from T2-weighted MRI scans was performed and tested for the detection of the CSF leak.
The CSF epidural collection was visible in all patients with SIH and was detected at the dorsal (16 of 17), cervical (13 of 17), lumbar (13 of 17), and sacral (12 of 17) levels. None of the control subjects showed a CSF leak. Diverticula were present in 23% of patients, whereas the actual site of the CSF leak was recognized in only one patient. Eight patients were treated conservatively, whereas 9 patients required an epidural blood patch, performed at a fixed L2-L3 or L3-L4 spinal level, with complete recovery.
Spinal MRI with dedicated subtraction analysis could represent the first-line diagnostic tool in the management of patients with SIH, thus leaving invasive investigation for selected patients, such those requiring dural surgery.
To determine the effect of an intensive care management protocol on the intensive care unit (ICU) and hospital mortality of severely head-injured patients, we designed a longitudinal observational ...study of all patients admitted with a head injury between 1992 and 2000.
A computerized patient database was used to identify all patients with severe head injury admitted to the ICU at Frenchay Hospital, Bristol, UK: a tertiary referral centre for the clinical neurosciences. We compared the ICU and hospital mortality and length of stay in patients before and after implementation of a protocol for their ICU management in 1997.
Implementation of the protocol was associated with a significant reduction in ICU mortality from 19.95% to 13.5% (odds ratio 0.47; 95% CI 0.29–0.75), and in hospital mortality from 24.55% to 20.8% (odds ratio 0.48; 95% CI 0.31–0.74). This was achieved despite a significant increase in the median APACHE II score (14 vs 18) of patients admitted after implementation of the protocol. The median ICU and hospital length of stay remained constant over the study period.
The introduction of an evidence-based protocol to guide the ICU management of patients with severe head injury has been associated with a significant reduction in both ICU and hospital mortality.
Background
Medial Unicompartmental Knee Replacement (UKR) has well-documented benefits over Total Knee Replacement in the treatment of anteromedial osteoarthritis of the knee. There has been an ...increasing move from cemented to cementless UKR over the last decade. This non-design centre study assesses the initial experience using the cementless Oxford medial partial knee replacement and provides medium term revision data, as well as Patient Reported Outcome Measures (PROMs).
Methods
A cohort of 200 consecutive patients undergoing medial UKR using the cementless Oxford were identified from our knee groups prospectively collected database. Cases were performed in a single centre under the care of one of four surgeons. All patients were beyond the 5-year minimum timepoint following UKR surgery in order to produce medium term results, at a mean of 7.9 years. Eligible patients completed a postal questionnaire to collect PROMs: Oxford Knee Score, WOMAC and modified American Knee Society Score questionnaires in January 2020 and had their clinical records reviewed.
Results
The survivorship in our cohort was 94.5% at a mean follow up of 7.9 years following surgery. There were 11 re-operations in total with a three percent risk of re-operation within the first 18 months following surgery. There was a sustained improvement in Oxford Knee Score with a near 20 points improvement on pre-operative scores.
Conclusions
Our results provide further evidence that partial knee replacements using the cementless Oxford produce good clinical outcomes. Revision rates are similar to those published in the National Joint Registry.
Level of Evidence
: III.
This is the second part of a retrospective and review MR imaging study aiming to define the frequency rate, timing, imaging features, and evolution of gray matter changes in Menkes disease, a rare ...multisystem X-linked disorder of copper metabolism characterized by early, severe, and progressive neurologic involvement. According to our analysis, neurodegenerative changes and focal basal ganglia lesions already appear in the early phases of the disease. Subdural collections are less common than generally thought; however, their presence remains important because they might challenge the differential diagnosis with child abuse and might precipitate the clinical deterioration. Anecdotal findings in our large sample seem to provide interesting clues about the protean mechanisms of brain injury in this rare disease and further highlight the broad spectrum of MR imaging findings that might be expected while imaging a child with the suspicion of or a known diagnosis of Menkes disease.
The objective of this study was to determine the degree of brain involvement in a cohort of myotonic dystrophy type 1 and type 2 (DM1, DM2) patients by brain studies and functional tests and to ...compare the results of the two groups. DM1, DM2 are multisystemic disorders due to polynucleotide expansions. Previous studies on brain involvement by neuroimaging and functional methods have led to contradictory results. Fifty molecularly defined DM1 patients and 14 DM2 patients, were recruited for the study. Age at recruitment, age at disease onset, disease duration and educational level were recorded. Neuromuscular assessment was done by MIRS. An extensive neuropsychological battery was performed in 48/50 DM1 and in a control group of 44 healthy matched subjects. Forty six of 50 DM1 and 12/14 DM2 underwent brain MRI; 21/50 DM1 and 9/14 DM2 underwent brain perfusion SPECT, with semiquantitative analysis of the results. MRI images were classified by ARWMC (age-related white matter changes) score, in order to quantify recurrence, localization and patterns of distribution of white matter hyperintense lesions (WMHLs) in our two cohorts. MRI results were matched to SPECT and to neuropsychological results. Thirty-seven of 46 DM1 and 10/12 DM2 had abnormal MRI imaging, showing scattered supratentorial, bilateral, symmetrical focal or diffuse WMHLs. A typical temporo-insular diffuse subcortical pattern was seen in DM1 subjects only, with no correlation with cognitive involvement. Major cognitive involvement was seen in the case of diffuse frontal lesions. A relationship with CTG expansion size was documented for DM1 subjects. SPECT showed minimal hypoperfusion in the posterior cortex planes in DM1 and, to a lesser extent, in DM2. Very mild degrees of involvement in the DM2 cohort were seen. Neuroimaging and functional investigations confirmed a more severe involvement of the brain in DM1 compared to DM2. A temporo-insular diffuse lesional pattern, specific for DM1, was found on MRI. This confirms greater expansion size as a risk factor for more extensive brain involvement in DM1.
Background In the UK demand for organ transplantation continues to outstrip supply and one strategy aimed at reversing this trend is the introduction of non-heart beating donor (NHBD) schemes. In ...this paper we describe our experience after the introduction of the NHBD scheme at a regional neuroscience intensive care unit (ICU) that also provides general intensive care. Methods We describe the steps taken to establish the scheme and present our results from the time of its implementation in July 2002 until March 2007. Results Of the 100 patients whom we referred to the transplant co-ordinators, 71 were identified as potential NHBDs and of these 29 went on to become actual donors (conversion rate of 40.8%). Fifty-six kidneys were retrieved and 53 successfully transplanted. In addition, two livers were retrieved but subsequently found to be unsuitable for transplantation, while eight pancreas were retrieved and used for islet cell research. The serum creatinine at 1 yr demonstrates that there is no significant difference between transplanted kidney function from NHBDs and heart-beating donors (HBDs). Conclusions We believe that by establishing the NHBD organ donation scheme we are able to fulfil the wishes of more patients who have indicated that they would like to donate their organs while increasing the availability of solid organs for transplantation. With careful preparation, audit, and communication our experience demonstrates that the NHBD scheme can be successfully introduced in an ICU and expanded to other ICUs in a region.
Summary
The refusal rate for organ donation in the UK is 42%, among the highest in Europe. We extracted data on every family approach for donation in UK ICUs or Emergency Departments between 1st ...April 2012 and 30th September 2013, and performed multiple logistic regression to identify modifiable factors associated with consent. Complete data were available for 4703 of 4899 approaches during the study period. Consent for donation after brain death was 68.9%, and for donation after circulatory death 56.5% (p < 0.0001). Patient ethnicity, knowledge of a patient's wishes and involvement of a specialist nurse in organ donation in the approach were strongly associated with consent (p < 0.0001). The impact of the specialist nurse was stronger for donation after circulatory death than for donation after brain death, even after accounting for the impact of prior knowledge of patients' wishes. Involvement of the specialist nurse in the approach, encouraging family discussions about donation wishes and promotion of the organ donor register are key strategies to increase UK consent rates, and are supported by this study.
Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A. Central nervous system (CNS) manifestations consist mainly of cerebrovascular ...events. Brain MRI results are often abnormal.
The aim of the study was to describe CNS involvement in a group of Italian patients with AFD.
Clinical and brain MRI data of 43 patients with AFD (25 men, 41.94+/-10.83 years old and 18 women, 52.48+/-17.50 years old) were analysed retrospectively. 17 male patients and 7 female patients were under treatment with enzyme replacement therapy (ERT).
All 43 patients had signs or symptoms of AFD. 16 men (64%) and 13 women (72%) demonstrated CNS involvement, although with varying severity. Overall, 6 men and 5 women had suffered from cerebrovascular accidents with an age at onset of 33.64+/-13.65 years and 53.68+/-11.71 years, respectively. Brain MR images were abnormal in 16/25 men and in 13/16 women. During CNS monitoring, some patients receiving ERT (5/17 men and 2/6 women) demonstrated neurological deterioration, especially those who had presented with cerebrovascular disease already before starting ERT.
The study demonstrated a high frequency of CNS involvement in homozygous and heterozygous AFD patients, often characterised by early age at onset and abnormal brain MRIs. At present, ERT is widely used; however, potential beneficent effects may be disguised by the progression of irreversible pathology in short-term follow-up. Therefore, primary and secondary prophylaxes of cerebrovascular disease are extremely important.
The coronavirus 2019 (COVID-19) pandemic has disrupted health systems worldwide, including solid organ donation and transplantation programs. Guidance on how best to screen patients who are potential ...organ donors to minimize the risks of COVID-19 as well as how best to manage immunosuppression and reduce the risk of COVID-19 and manage infection in solid organ transplant recipients (SOTr) is needed.
Iterative literature searches were conducted, the last being January 2021, by a team of 3 information specialists. Stakeholders representing key groups undertook the systematic reviews and generation of recommendations using a rapid response approach that respected the Appraisal of Guidelines for Research and Evaluation II and Grading of Recommendations, Assessment, Development and Evaluations frameworks.
The systematic reviews addressed multiple questions of interest. In this guidance document, we make 4 strong recommendations, 7 weak recommendations, 3 good practice statements, and 3 statements of "no recommendation."
SOTr and patients on the waitlist are populations of interest in the COVID-19 pandemic. Currently, there is a paucity of high-quality evidence to guide decisions around deceased donation assessments and the management of SOTr and waitlist patients. Inclusion of these populations in clinical trials of therapeutic interventions, including vaccine candidates, is essential to guide best practices.