ObjectiveBone marrow lesions (BML) are an MRI feature of osteoarthritis (OA) offering a potential target for therapy. We developed the Knee Inflammation MRI Scoring System (KIMRISS) to semiquantitatively score BML with high sensitivity to small changes, and compared feasibility, reliability and responsiveness versus the established MRI Osteoarthritis Knee Score (MOAKS).MethodsKIMRISS incorporates a web-based graphic overlay to facilitate detailed regional BML scoring. Observers scored BML by MOAKS and KIMRISS on sagittal fluid-sensitive sequences. Exercise 1 focused on interobserver reliability in Osteoarthritis Initiative observational data, with 4 readers (two experienced/two new to KIMRISS) scoring BML in 80 patients (baseline/1 year). Exercise 2 focused on responsiveness in an open-label trial of adalimumab, with 2 experienced readers scoring BML in 16 patients (baseline/12 weeks).ResultsScoring time was similar for KIMRISS and MOAKS. Interobserver reliability of KIMRISS was equivalent to MOAKS for BML status (ICC=0.84 vs 0.79), but consistently better than MOAKS for change in BML: Exercise 1 (ICC 0.82 vs 0.53), Exercise 2 (ICC 0.90 vs 0.32), and in new readers (0.87–0.92 vs 0.32–0.51). KIMRISS BML was more responsive than MOAKS BML: post-treatment BML improvement in Exercise 2 reached statistical significance for KIMRISS (SRM −0.69, p=0.015), but not MOAKS (SRM −0.12, p=0.625). KIMRISS BML also more strongly correlated to WOMAC scores than MOAKS BML (r=0.80 vs 0.58, p<0.05).ConclusionsKIMRISS BML scoring was highly feasible, and was more reliable for assessment of change and more responsive to change than MOAKS BML for expert and new readers.
SUMMARY:Use of advanced imaging in the emergency department has been increasing in the United States during the past 2 decades. This trend has been most notable in CT, which has increased concern over the effects of increasing levels of medical ionizing radiation. MR imaging offers a safe, nonionizing alternative to CT and is diagnostically superior in many neurologic conditions encountered in the emergency department. Herein, we describe the process of developing and installing a dedicated MR imaging scanner in the Neuroscience Emergency Department at the Barrow Neurological Institute and its effects on neuroradiology and the emergency department in general.
49,XXXXY is a rare aneuploidy with neuroanatomic findings scarcely reported in the literature. Given the fact that many of its phenotypic characteristics are similar to Klinefelter patients, 49,XXXXY has been treated as a variant of Klinefelter syndrome in the past. Newer studies have shown that intellectual disabilities and cardiac sequelae are more common in 49,XXXXY making the need for more precise characterization of the disorder essential. Prior case studies have demonstrated focal (and to a lesser extent confluent) white abnormalities as well as enlarged perivascular cysts (often in clustered arrangements) in the brains of these patients, but high resolution magnetic resonance images of severe myelinopathy are infrequently documented. Presented here is an exceptional manifestation of this rare disease with substantial findings in the brain exhibiting both confluent white matter changes and diffuse perivascular cysts. Cases such as this one serve to expand the differential considerations for confluent dysmyelinating disease and improve diagnostic efficacy.
Here we report the case of a newborn with glycogenosis type IV (Andersen disease), who died shortly after birth. The diagnosis was established in the first instance by light microscopy and histochemistry, and subsequently ultrastructurally. DNA could be extracted from a fibroblast cell culture by sequencing the causative GBE1 gene (glycogen branching enzyme 1). Two compound heterozygous mutations in the gene were identified. The differential diagnosis should include Lafora disease as well as polyglucosan body disease. Since there is no effective therapy for glycogenosis type IV to date, prenatal diagnosis is mandatory.
W e appreciate the interest of Yousem et al in our recent article, "Introduction of a Dedicated MR Imaging Scanner at the Barrow Neurological Institute." 1 The transition to accessible emergency department (ED) MR imaging at the Barrow Neurological Institute is relatively recent, and our article focused on some important factors to consider when implementing a new ED MR imaging program and workflow, as well as associated changes that might be expected in scan volumes and distribution. We thank Yousem et al for bringing to our attention the important works on outcomes following ED MR imaging installation that have been completed at Johns Hopkins. It is encouraging to know that accessible ED MR imaging can have a positive impact on patient admission rates and admission lengths. 2-5 We also agree with the importance of open communication with clinical services; consultation with emergency, neurology, and neurosurgery colleagues before, during, and after installation of an ED MR scanner is crucial to program success and plays an important role in shaping new ED ordering guidelines and protocols. We hope that our experience, taken in conjunction with work from other centers, may provide support and insight to colleagues considering the transition to accessible ED MR imaging in the future.
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