Discrete Choice Experiment on a Magnetic Resonance Imaging Scoring System for Temporomandibular Joints in Juvenile Idiopathic Arthritis.

OBJECTIVE: To determine the relative importance weights of items and grades of a newly developed additive outcome measure called the juvenile idiopathic arthritis (JIA) magnetic resonance imaging (MRI) scoring system for the temporomandibular joint (TMJ) (JAMRIS-TMJ). METHODS: An adaptive partial-profile, discrete choice experiment (DCE) survey using the 1000Minds platform was independently completed by members of an expert group consisting of radiologists and non-radiologist clinicians to determine the group-averaged relative weights for the JAMRIS-TMJ. Subsequently, an image-based vignette ranking exercise was done, during which experts individually rank ordered 14 patient vignettes for disease severity while blinded to the weights and unrestricted to JAMRIS-TMJ assessment criteria. Validity of the weighted JAMRIS-TMJ was tested by comparing the consensus-graded, DCE-weighted JAMRIS-TMJ score of the vignettes with their unrestricted image-based ranks provided by the experts. RESULTS: Nineteen experts completed the DCE survey, and 21 completed the vignette ranking exercise. Synovial thickening and joint enhancement showed higher weights per raw score compared to bone marrow items and effusion in the inflammatory domain, while erosions and condylar flattening showed nonlinear and higher weights compared to disk abnormalities in the damage domain. The weighted JAMRIS-TMJ score of the vignettes correlated highly with the ranks from the unrestricted comparison method, with median Spearman's ρ of 0.92 (interquartile range [IQR] 0.87-0.95) for the inflammation and 0.93 (IQR 0.90-0.94) for the damage domain. CONCLUSION: A DCE survey was used to quantify the importance weights of the items and grades of the JAMRIS-TMJ. The weighted score showed high convergent validity with an unrestricted, holistic vignette ranking method.

Assessing the Reliability of the OMERACT Juvenile Idiopathic Arthritis Magnetic Resonance Scoring System for Temporomandibular Joints (JAMRIS-TMJ).

Contrast-enhanced magnetic resonance imaging (MRI) remains the most comprehensive modality to assess juvenile idiopathic arthritis (JIA)-related inflammation and osteochondral damage in the temporomandibular joints (TMJ). This study tested the reliability of a new JIA MRI scoring system for TMJ (JAMRIS-TMJ) and the impact of variations in calibration and reader specialty. Thirty-one MRI exams of bilateral TMJs were scored independently using the JAMRIS-TMJ by 20 readers consisting of radiologists and non-radiologist clinicians in three reading groups, with or without a calibrating atlas and/or tutorial. The inter-reader reliability in the multidisciplinary cohort assessed by the generalizability coefficient was 0.61-0.67 for the inflammatory and 0.66-0.74 for the damage domain. The atlas and tutorial did not improve agreement within radiologists, but improved the agreement between radiologist and non-radiologist groups. Agreements between different calibration levels were 0.02 to 0.08 lower by the generalizability coefficient compared to agreement within calibration levels; agreement between specialty groups was 0.04 to 0.10 lower than within specialty groups. Averaging two radiologists raised the reliability above 0.8 for both domains. Therefore, the reliability of JAMRIS-TMJ was moderate-to-good depending on the presence of specialty and calibration differences. The atlas and tutorial are necessary to improve reliability when the reader cohort consists of multiple specialties.

Imaging of temporomandibular joint abnormalities in juvenile idiopathic arthritis with a focus on developing a magnetic resonance imaging protocol.

Inflammation and damage in the temporomandibular joint (TMJ) often develop without clinical symptoms but can lead to severe facial growth abnormalities and impaired health-related quality of life, making early diagnosis of TMJ changes crucial to identify. Inflammatory and osteochondral changes detectable through magnetic resonance imaging (MRI) occur in TMJs of approximately 40% of children with juvenile idiopathic arthritis (JIA), and no other imaging modality or physical method of examination can reliably detect these changes. Therefore contrast-enhanced MRI is the diagnostic standard for diagnosis and interval monitoring of JIA. However the specific usage of MRI for TMJ arthritis is not standardized at present. There is a recognized need for a consensus effort toward standardization of an imaging protocol with required and optional sequences to improve detection of pathological changes and shorten study time. Such a consensus imaging protocol is important for providing maximum information with minimally necessary sequences in a way that allows inter-site comparison of results of clinical trials and improved clinical management. In this paper we describe the challenges of TMJ imaging and present expert-panel consensus suggestions for a standardized TMJ MRI protocol.

Defining the normal appearance of the temporomandibular joints by magnetic resonance imaging with contrast: a comparative study of children with and without juvenile idiopathic arthritis.

BACKGROUND: Up to 80% of children with juvenile idiopathic arthritis (JIA) develop arthritis involving their temporomandibular joint (TMJ). Recent studies have questioned the sensitivity of an abnormal MRI in the diagnosis of active arthritis. METHODS: 122 children without arthritis undergoing contrast MRI of the head were prospectively consented to undergo a simultaneous contrast MRI of their TMJs. As a comparison point, the initial MRI of the TMJ of 35 newly diagnosed children with JIA were retrospectively scored. The presence and size of effusion and contrast enhancement were measured in the left TMJ in all subjects. RESULTS: 62/122 (51%) controls compared to only 10/35 JIA (29%) patients had an effusion (p = 0.022). Contrast enhancement was present in ≥97% of both groups, although the size of the enhancement was, on average, 0.2 mm larger in controls (1.1 ± 0.24 vs 0.88 ± 0.27 mm, p <  0.001). Among JIA patients, the size of the enhancement correlated inversely with disease duration (r = - 0.475, p = 0.005). Chronic changes were present in none of the controls versus 2/35 (5.5%) of the JIA patients (p = 0.049). CONCLUSION: Findings consistent with minimally active TMJ arthritis appear to be equally likely in children with JIA as compared to non-inflamed controls, while this and other studies confirm that chronic changes are specific to JIA. Thus, small amounts of effusion or contrast enhancement, in the absence of chronic changes, should be interpreted with caution.

Toward Establishing a Standardized Magnetic Resonance Imaging Scoring System for Temporomandibular Joints in Juvenile Idiopathic Arthritis.

OBJECTIVE: The temporomandibular joints (TMJs) are frequently affected in children with juvenile idiopathic arthritis (JIA). Early detection is challenging, as major variation is present in scoring TMJ pathology on magnetic resonance imaging (MRI). Consensus-driven development and validation of an MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis and serving as an outcome measure. We report on a multi-institutional collaboration toward developing a TMJ MRI scoring system for JIA. METHODS: Seven readers independently assessed MRI scans from 21 patients (42 TMJs, from patients ages 6-16 years) using 3 existing MRI scoring systems from American, German, and Swiss institutions. Reliability scores, scoring system definitions, and items were discussed among 10 JIA experts through 2 rounds of Delphi surveys, nominal group voting, and subsequent consensus meetings to create a novel TMJ MRI scoring system. RESULTS: Average-measure absolute agreement intraclass correlation coefficients (avICCs) for the total scores of all 3 scoring systems were highly reliable at 0.96 each. Osteochondral items showed higher reliability than inflammatory items. An additive system was deemed preferable for assessing minor joint changes over time. Eight items were considered sufficiently reliable and/or important for integration into the consensus scoring system: bone marrow edema and enhancement (avICC 0.57-0.61, smallest detectable difference [SDD] ± 45-63% prior to redefining), condylar flattening (avICC 0.95-0.96, SDD ± 23-28%), effusions (avICC 0.85-0.88, SDD ± 25-26%), erosions (avICC 0.94, SDD ± 20%), synovial enhancement and thickening (previously combined, avICC 0.90-0.91, SDD ± 33%), and disk abnormalities (avICC 0.90, SDD ± 19%). CONCLUSION: A novel TMJ MRI scoring system was developed by consensus. Further iterative refinements and reliability testing are warranted in upcoming studies.

Magnetic Resonance Imaging Findings following Intraarticular Infliximab Therapy for Refractory Temporomandibular Joint Arthritis among Children with Juvenile Idiopathic Arthritis.

OBJECTIVE: To evaluate the involvement of intraarticular (IA) infliximab (IFX) in the management of temporomandibular joint (TMJ) arthritis associated with juvenile idiopathic arthritis (JIA) that is refractory to systemic treatment and IA corticosteroid therapy. METHODS: Ours was a retrospective study of children with JIA who received IA IFX into the TMJ. The effectiveness of treatment on the progression of acute and chronic changes was assessed by a quantitative magnetic resonance imaging scoring system. RESULTS: Median acute and chronic scores worsened by 0.25 and 0.75, respectively. In multivariate analysis, worsening acute scores and passage of time predicted worsening of the chronic scores. CONCLUSION: IA IFX allowed for progression of refractory TMJ arthritis in most but not all children with JIA.

Hemispherectomy for Hemimegalencephaly Due to Tuberous Sclerosis and a Review of the Literature.

BACKGROUND: Hemimegalencephaly with tuberous sclerosis complex is an uncommon association, usually associated with intractable seizures that begin in the neonatal period or early infancy. Typically, the seizures are managed with medications until the patient is older when surgical treatment is considered safe. PATIENT DESCRIPTION: We describe a 7-week-old infant with tuberous sclerosis (TSC1 mutation) and hemimegalencephaly who underwent a functional hemispherectomy for status epilepticus. No clinical seizures have occurred since surgery nearly 5 years ago and subsequent weaning of antiepileptic drugs 3 years ago. This is one of the youngest patients with tuberous sclerosis complex treated with a hemispherectomy and one of seven patients described in the literature. CONCLUSIONS: Our patient, along with previously reported cases, suggests that a hemispherectomy is a viable option in the very young. With evolution of this surgical process since its inception nearly 6 decades ago, it may now be performed safely in early infancy, engendering the possibility of seizure freedom in most and thus optimizing neurodevelopmental outcome.

A simplified algorithm for diagnosis of spinal cord lesions.

Neuroimaging is indispensable for evaluation of Myelopathy not only for localization but also for etiologic determination. MRI is the preferred examination for further characterization of the majority of these conditions. These include traumatic, inflammatory, infections, compressive and neoplastic conditions. This article provides an overview of a variety of pathologies that afflict the spinal cord in an easy to understand format. Their respective imaging manifestations on MRI and differential diagnoses are focused in this review. Early diagnosis and treatment of Myelopathy is critical in preventing or arresting neurological morbidity.

Progressive lung calcification after orthotopic heart transplant.

Focal, asymmetrical pulmonary airspace opacities in post-transplant setting are commonly from infection, hemorrhage, edema or infarction. Rarely, stable or mildly progressive dense pulmonary opacities are due to pulmonary calcifications. In the majority of cases, these are asymptomatic and warrant no further intervention.

Myelin, myelination, and corresponding magnetic resonance imaging changes.

Accurate diagnosis of white matter diseases requires a thorough understanding of white matter maturation. These maturational changes are complex and require knowledge of the histologic background and time course of development. This article reviews the in vivo magnetic resonance (MR) appearance of myelination with emphasis on the appearance of different regions of the brain using various pulse sequences at different developmental time points. The appearance of white matter, using the MR pulse sequences that have been shown to be most useful during the stages of myelination, is also discussed.

Retrograde Wallerian degeneration of cranial corticospinal tracts in cervical spinal cord injury patients using diffusion tensor imaging.

Diffusion tensor imaging (DTI) has the potential to reveal disruption of white matter microstructure in chronically injured spinal cords. We quantified fractional anisotropy (FA) and mean diffusivity (MD) to demonstrate retrograde Wallerian degeneration (WD) of cranial corticospinal tract (CST) in cervical spinal cord injury (SCI). Twenty-two patients with complete cervical SCI in the chronic stage were studied with DTI along with 13 healthy controls. Mean FA and MD values were computed for midbrain, pons, medulla, posterior limb of internal capsule, and corona radiata. Significant reduction in the mean FA and increase in MD was observed in the cranial CST in patients with SCI compared with controls, suggesting retrograde WD. Statistically significant inverse FA and MD changes were noted in corona radiata, indicating some restoration of spared white matter tracts. Temporal changes in the DTI metrics suggest progressing degeneration in different regions of CST. These spatiotemporal changes in DTI metrics suggest continued WD in injured fibers along with simultaneous reorganization of spared white matter fibers, which may contribute to changing neurological status in chronic SCI patients.