FLAIR MRI biomarkers of the normal appearing brain matter are related to cognition.

A novel biomarker panel was proposed to quantify macro and microstructural biomarkers from the normal-appearing brain matter (NABM) in multicentre fluid-attenuation inversion recovery (FLAIR) MRI. The NABM is composed of the white and gray matter regions of the brain, with the lesions and cerebrospinal fluid removed. The primary hypothesis was that NABM biomarkers from FLAIR MRI are related to cognitive outcome as determined by MoCA score. There were three groups of features designed for this task based on 1) texture: microstructural integrity (MII), macrostructural damage (MAD), microstructural damage (MID), 2) intensity: median, skewness, kurtosis and 3) volume: NABM to ICV volume ratio. Biomarkers were extracted from over 1400 imaging volumes from more than 87 centres and unadjusted ANOVA analysis revealed significant differences in means of the MII, MAD, and NABM volume biomarkers across all cognitive groups. In an adjusted ANCOVA model, a significant relationship between MoCA categories was found that was dependent on subject age for MII, MAD, intensity, kurtosis and NABM volume biomarkers. These results demonstrate that structural brain changes in the NABM are related to cognitive outcome (with different relationships depending on the age of the subjects). Therefore these biomarkers have high potential for clinical translation. As a secondary hypothesis, we investigated whether texture features from FLAIR MRI can quantify microstructural changes related to how "structured" or "damaged" the tissue is. Based on correlation analysis with diffusion weighted MRI (dMRI), it was shown that FLAIR MRI texture biomarkers (MII and MAD) had strong correlations to mean diffusivity (MD) which is related to tissue degeneration in the GM and WM regions. As FLAIR MRI is routinely collected for clinical neurological examinations, novel biomarkers from FLAIR MRI could be used to supplement current clinical biomarkers and for monitoring disease progression. Biomarkers could also be used to stratify patients into homogeneous disease subgroups for clinical trials, or to learn more about mechanistic development of dementia disease.

Radiographic morphology of normal ring apophyses in the immature cervical spine.

BACKGROUND: The ring apophyses of the cervical spine have a variable appearance that changes with age. The times at which they appear and fuse at each level are not fixed. In this study, we aim to detail normal ranges of appearance of these ossification centers for each age group. MATERIALS AND METHODS: One hundred and eighty patients under the age of 21 attending the Royal Stoke University Hospital for cervical spine radiographs were retrospectively identified. The presence or absence of ring apophyses at each cervical level and whether these had undergone fusion was reported, as were the thickness, length, and craniocaudal and anteroposterior distance of the apophysis from the vertebral body. The angulation of the apophysis relative to the endplate was also noted. RESULTS: The youngest patient in which apophyses were seen was aged 3, but apophyses were otherwise rarely seen before the age of 6. All apophyses were present from age 14, and the superior apophyses fused by the age of 18, although unfused inferior apophyses were still seen in the 20-year age group. It was observed that apophyses were rarely separated from the vertebral body by greater than 1 mm in craniocaudal distance (1%) or 2.5 mm in anteroposterior distance (2.6%) and the anterior apophysis was angulated towards the endplate in only 1% of cases. CONCLUSIONS: We have detailed the range of normal appearances of the ring apophyses of the developing cervical spine. Cervical spine apophyseal injury is thought to be rare, but knowledge of normative morphological features should help in this diagnosis.

Surveillance of intramedullary cartilage tumours in long bones.

AIMS: The purpose of this study was to determine if clinical and radiological surveillance of cartilage tumours with low biological activity is appropriate. PATIENTS AND METHODS: A total of 98 patients with an intramedullary cartilage neoplasm in a long bone met our inclusion criteria and were included in the study. These patients had undergone a total of 384 scans. Patients with radiological follow-up of more than three years (46 patients) were divided into two groups: an active group (11 patients) and a latent group (35 patients). RESULTS: Active lesions had a total growth in all three planes that was > 6 mm, whilst latent lesions had < 6 mm of growth. Most latent lesions were heavily calcified: active lesions were calcified less than 50% (p = 0.025). CONCLUSION: Clinico-radiological surveillance can identify growing cartilage lesions: MRI is the surveillance modality of choice. A CT scan is recommended, in addition, at presentation to assess the amount of calcification within the lesion. A first follow-up MRI is suggested one year from diagnosis. If the total growth in the cartilage lesion is > 6 mm, surgical treatment should be considered. Otherwise, a second surveillance scan can be performed at three years to determine further management. Cite this article: Bone Joint J 2016;98-B:1542-7.

A new method for measurement of subcoracoid outlet and its relationship to rotator cuff pathology at MR arthrography.

OBJECTIVE: Orthopaedic surgical studies have shown that variations in the vertical distance between the tip of the coracoid process and the supra-glenoid tubercle alter the shape of the subcoracoid outlet. Our objective was to measure the vertical distance between the coracoid tip and the supra-glenoid tubercle (CTGT) on MR and to assess whether this showed better correlation with rotator cuff pathology compared with the axial coraco-humeral distance. MATERIALS AND METHODS: A retrospective review was performed of 100 consecutive shoulder MR arthrograms. Vertical distance between the coracoid tip and the supraglenoid tubercle was measured in the sagittal oblique plane. Separate assessment was then made of tendon pathology of the subscapularis, supraspinatus and long head of biceps tendons. Axial coraco-humeral distance was then measured. Correlation between tendon abnormalities and the two measurements was then made. RESULTS: Of the 100 cases, 42 had subscapularis tendon lesions, 21 had lesions of the long head of biceps and 53 had supraspinatus tendon lesions. Mean vertical distance from the coracoid tip to supraglenoid tubercle was greater in those with lesions of any of these tendons and was statistically significant for the supraspinatus group (P = 0.005). Reduced axial coraco-humeral distance was also seen in patients with tendinopathy, although with less statistically significant difference (p = 0.059). CONCLUSION: Our results support orthopaedic studies that have shown that the vertical distance between the coracoid tip and the supraglenoid tubercle increases the incidence and risk of rotator cuff disease by altering the shape of the subcoracoid outlet.

MRI of inflammatory spondyloarthropathy following traumatic cauda equina syndrome.

BACKGROUND CONTEXT: Spondyloarthropathy has been described radiographically in patients following paralysis from spinal cord trauma. Onset of these findings after cauda equina syndrome have not been reported previously. Furthermore, the magnetic resonance documentation of its early evolution has not been recorded. PURPOSE: We report a case of early-onset spondyloarthropathy shown by magnetic resonance imaging (MRI) in a patient with cauda equina syndrome due to bilateral sacral insufficiency fractures. STUDY DESIGN: Unique case study review, one case. METHODS: Review of the clinical case notes and imaging including initial and subsequent MR imaging. RESULTS: The initial MRI of the lumbosacral spine showed bilateral sacral insufficiency fractures with a kyphotic deformity. The vertebral bodies were normal on the initial computed tomography and MRI studies, which did not reveal pre-existing features of sacroiliitis. The second MRI performed 5 months later clearly showed spondylitis at multiple vertebral levels with partial resolution 18 months post injury. CONCLUSION: Spondyloarthropathy in patients with paralysis due to spinal cord injury is well documented in the English language literature, but until now this has not been demonstrated by MRI. It is a rare complication of traumatic cauda equina syndrome that commences soon after the traumatic event and can resolve spontaneously.

Tumours and tumour mimics in the olecranon.

Lesions in the olecranon are rare and may be identified during the investigation of a clinically suspected abnormality or as an incidental finding. This review describes the spectrum of tumours and tumour-like lesions that can involve the olecranon and illustrates the radiographic, CT, and MRI appearances that may facilitate diagnosis. A variety of pathological processes affecting the olecranon are presented and discussed including the epidemiology and imaging features.

Intra-epiphyseal stress injury of the proximal tibial epiphysis: preliminary experience of magnetic resonance imaging findings.

Stress induced injuries affecting the physeal plate or cortical bone in children and adolescents, especially young athletes, have been well described. However, there are no reports in the current English language literature of stress injury affecting the incompletely ossified epiphyseal cartilage. We present four cases of stress related change to the proximal tibial epiphysis (PTE) along with their respective magnetic resonance imaging (MRI) appearances ranging from subtle oedema signal to a pseudo-tumour like appearance within the epiphyseal cartilage. The site and pattern of intra-epiphyseal injury is determined by the type of tissue that is affected, the maturity of the skeleton and the type of forces that are transmitted through the tissue. We demonstrate how an awareness of the morphological spectrum of MRI appearances in intra-epiphyseal stress injury and the ability to identify concomitant signs of stress in other nearby structures can help reduce misdiagnosis, avoid invasive diagnostic procedures like bone biopsy and reassure patients and their families.

Adiposis dolorosa (Dercum's disease): MRI and ultrasound appearances.

AIM: To describe ultrasound and magnetic resonance imaging (MRI) features of adiposis dolorosa, Dercum's disease, and to evaluate the MRI features prospectively against a large number of MRI examinations. MATERIALS AND METHODS: Institutional review board approval for this study was obtained. The imaging features at MRI and ultrasound of 13 cases of adiposis dolorosa (nine female, four male; age range 32-72 years) were reviewed. MRI findings typical for adiposis dolorosa were proposed and prospectively evaluated on 6247 MRI examinations performed over a period of 8 months. RESULTS: Adiposis dolorosa demonstrates multiple, oblong, fatty lesions in the superficial subcutaneous fatty tissue. They are mostly <2 cm in long axis diameter. They demonstrate nodular ("blush-like") increased fluid signal at unenhanced MRI and are markedly hyperechoic at ultrasound. There is no contrast medium enhancement at MRI and no increased Doppler signal at ultrasound. Most lesions were clinically asymptomatic, some were painful/tender. There was no imaging evidence of oedema or inflammation. During prospective validation of these MRI features on 6247 MRI examinations, two cases with typical imaging features were encountered; both were diagnosed as adiposis dolorosa on clinical review. All cases of adiposis dolorosa showed these imaging findings. This results in a very low likelihood that a nodular, blush-like appearance of subcutaneous fat on MRI is not due to adiposis dolorosa. DISCUSSION: Adiposis dolorosa, Dercum's disease, should be suggested in the presence of multiple (many) small, oblong, fatty lesions in the subcutaneous fatty tissue in adult patients if they are hyperechoic on ultrasound imaging or blush-like at unenhanced MRI; typically a small number of these lesions are tender/painful. Imaging does not demonstrate inflammation or oedema in relation to these lesions. These MRI features should suggest the diagnosis and are likely to be pathognomonic. The radiologist is often the first to suggest the diagnosis based on the imaging features.

Destructive discovertebral degenerative disease of the lumbar spine.

The uncommon variant of degenerative hip joint disease, termed rapidly progressive osteoarthritis, and highlighted by severe joint space loss and osteochondral disintegration, is well established. We present a similar unusual subset in the lumbar spine termed destructive discovertebral degenerative disease (DDDD) with radiological features of vertebral malalignment, severe disc resorption, and "bone sand" formation secondary to vertebral fragmentation. Co-existing metabolic bone disease is likely to promote the development of DDDD of the lumbar spine, which presents with back pain and sciatica due to nerve root compression by the "bone sand" in the epidural space. MRI and CT play a complimentary role in making the diagnosis.

Role of MRI in hip fractures, including stress fractures, occult fractures, avulsion fractures.

MR imaging plays a vital role in the diagnosis and management of hip fractures in all age groups, in a large spectrum of patient groups spanning the elderly and sporting population. It allows a confident exclusion of fracture, differentiation of bony from soft tissue injury and an early confident detection of fractures. There is a spectrum of MR findings which in part is dictated by the type and cause of the fracture which the radiologist needs to be familiar with. Judicious but prompt utilisation of MR in patients with suspected hip fractures has a positive therapeutic impact with healthcare cost benefits as well as social care benefits.

SAPHO: What radiologists should know.

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is an umbrella acronym for inflammatory clinical conditions whose common denominator is aseptic osteoarticular involvement with characteristic skin lesions. It involves all ages, can involve any skeletal site, and has variable imaging appearances depending on the stage/age of the lesion and imaging method. It mimics important differentials including infection and neoplasia. Awareness of the imaging features, especially in the spine, facilitates early diagnosis, prevents repeated biopsies, and avoids unnecessary surgery, while initiating appropriate treatment.

Predictors of delayed referral to a pediatric rheumatology center.

OBJECTIVE: To report on referral patterns of primary physicians for children subsequently diagnosed with juvenile idiopathic arthritis (JIA) and to identify predictors of delayed referral to a pediatric rheumatology center. METHODS: A retrospective cohort study of consecutive patients with JIA referred to a pediatric rheumatology center over a 15-year period was performed. Variables included age, sex, JIA subtype, the physician's subspecialty, and distance to the pediatric rheumatology center. Outcome parameters were the time to first presentation to a primary physician, the time to the first rheumatology visit, and the total time to referral. Putative predictors were evaluated by analysis of variance, resulting in regression models. RESULTS: A total of 132 patients with JIA were included; 83 (63%) were female. The median age at the onset of symptoms was 4.5 years (range 1.0-15.8 years). Most frequently, children were referred by pediatricians (49.4%) or orthopedic surgeons (34.1%). The median time to first presentation was short at 10 days (range 0-1,610 days). In contrast, the median time to first rheumatology visit was 60 days (range 0.0-2,100.0 days), resulting in a long median total time to referral of 90 days (range 0.0-2,160.0 days). Statistically significant predictors for delayed referral were the primary physician's subspecialty (P = 0.016) and the distance to the pediatric rheumatology center (P = 0.001). Children living in remote areas or referred by orthopedic surgeons had the longest referral times. CONCLUSION: Despite free access to health care in Germany, children with JIA are referred to pediatric rheumatology centers with significant delay. Educational interventions targeting primary physicians and orthopedic surgeons may contribute to earlier referral to pediatric rheumatology centers and improve outcome in patients with JIA.

Acute renal failure in paediatric systemic lupus erythematosus: treatment and outcome.

OBJECTIVE: To determine the outcome of paediatric SLE (pSLE) patients with nephritis who developed acute renal failure (ARF). Efficacy and safety of treatment regimens were compared. METHODS: A total of 249 pSLE patients were diagnosed and prospectively followed at a single centre between July 1973 and July 2003; 127 children (51%) had lupus nephritis. ARF was defined as serum creatinine of > 250 micromol/l or > 75% above baseline. Standardized assessments included clinical data and medications, laboratory testing, disease activity and damage scores were obtained. Subsequent renal flares were documented. PRIMARY OUTCOME: renal function at last follow-up. SECONDARY OUTCOMES: treatment efficacy and safety. AZA- and cyclophosphamide (CYCLO)-treated patients were compared. Propensity score methods were applied to balance covariates. An intention to treat approach was chosen. RESULTS: The ARF study cohort included 50 patients; 13 boys and 37 girls with a median age of 13.2 yrs at diagnosis and a mean follow-up of 45 months. Renal histology: Class III nephritis in 16; Class IV in 34. Dialysis requirement and disease activity were similar in both groups. TREATMENT: AZA in 33 patients, CYCLO in 9 and corticosteroids only in 8. OUTCOME: no statistically significant or clinically relevant differences were found for any of the outcome measures including last serum creatinine, time to renal flare, overall renal survival, disease activity over time, disease damage, mean annual corticosteroid dose and rate of infection. CONCLUSION: The treatment of renal failure in this pSLE cohort was associated with an excellent outcome. AZA and CYCLO were equally efficacious.

Assessment of an infectious disease history preceding juvenile dermatomyositis symptom onset.

OBJECTIVES: A number of studies have looked at the role of infectious diseases in triggering juvenile dermatomyositis (JDM). Previous studies have found a moderately high frequency of infectious symptoms prior to disease onset; however, no specific pathogens could be identified. We sought to correlate preceding infectious symptoms with onset and outcomes of JDM. METHODS: We studied an inception cohort of all JDM cases diagnosed at The Hospital for Sick Children (SickKids) between 1988 and 2006. Data pertaining to symptoms at onset, diagnosis and disease outcomes were abstracted. Two independent paediatric infectious disease specialists reviewed all records of patients with symptoms or tests suggestive of infection. RESULTS: A total of 110 patients were reviewed; of these, 78 had sufficient information about disease onset for inclusion. Potential indications of an infectious process prior to JDM onset were found in 55/78 (71%) patients and were further evaluated for evidence of infection temporally associated with symptom onset. Features suggestive of infection prior to JDM symptom onset were found in 40/55 [probable (30/40) or possible (10/40)]. Most children with probable infections had respiratory illnesses [24/30 (80%)]. Fewer patients than expected had disease onset during summer months. The presence of an infection at onset was not found to be associated with differences in characteristics at diagnosis or disease outcomes. CONCLUSIONS: A substantial number of JDM patients have a clinical history consistent with an infection prior to onset. Newly diagnosed patients should undergo a full infectious disease assessment as part of their initial work-up; specific attention should be given to respiratory infections.

The spine in Paget's disease.

Paget's disease (PD) is a chronic metabolically active bone disease, characterized by a disturbance in bone modelling and remodelling due to an increase in osteoblastic and osteoclastic activity. The vertebra is the second most commonly affected site. This article reviews the various spinal pathomechanisms and osseous dynamics involved in producing the varied imaging appearances and their clinical relevance. Advanced imaging of osseous, articular and bone marrow manifestations of PD in all the vertebral components are presented. Pagetic changes often result in clinical symptoms including back pain, spinal stenosis and neural dysfunction. Various pathological complications due to PD involvement result in these clinical symptoms. Recognition of the imaging manifestations of spinal PD and the potential complications that cause the clinical symptoms enables accurate assessment of patients prior to appropriate management.

Angiography of primary central nervous system angiitis of childhood: conventional angiography versus magnetic resonance angiography at presentation.

BACKGROUND AND PURPOSE: To systematically analyze conventional angiographic (CA) features of children with primary central nervous system angiitis (cPACNS), to compare and correlate CA and MR angiography (MRA) lesion characteristics, and to define the sensitivity and specificity of MRA with CA as a reference standard. METHODS: A retrospective, single-center cohort study of consecutive patients with cPACNS was performed. Patients with CA and MRA studies at diagnosis were included. Imaging studies were blindly reviewed by 2 neuroradiologists using a standard analysis protocol. CA and MRA studies were compared using nonparametric analysis. RESULTS: Of 45 patients with MRA at diagnosis, there were 25 for whom CA and MRA studies were performed within 1 month of each other. These comprised the study group. The CA distribution of lesions was multifocal (76%) and proximal (86%) (P < .05) with a trend toward unilaterality (P = .06) with anterior circulation involvement (P = .08). The sensitivity and specificity of MRA for CA abnormality was 70% and 98%, respectively. There was no significant difference between MRA and CA for lesion detection or characterization (P = .87), and the modalities showed a fair correlation (kappa = 0.4). CONCLUSION: Angiographic lesions are multifocal and occur proximally and unilaterally within the anterior circulation. There is no significant difference in the ability of MRA to detect and characterize lesions when compared with CA.

Tumour necrosis factor alpha inhibitors in the treatment of childhood uveitis.

OBJECTIVE: To describe the efficacy of anti-TNF-alpha agents in the treatment of childhood uveitis. METHODS: We performed a retrospective chart review of all children with uveitis treated with TNF-alpha blockers at The Hospital for Sick Children, Toronto. RESULTS: Twenty-one children with uveitis were treated with the anti-TNF-alpha agents etanercept (11 patients) and infliximab (13 patients), resulting in 24 treatment courses. All patients had persistently active uveitis despite treatment with at least one standard immunosuppressive drug before the start of anti-TNF-alpha therapy. Six of 21 patients (29%) had idiopathic uveitis. In the other 15 patients, the underlying disease was juvenile idiopathic arthritis in 12 (57%), Behçet disease in two (9%) and sarcoidosis in one (5%). Response to etanercept treatment was good in 27%, moderate in 27% and poor in 45% of patients. Response to infliximab treatment was good in 38%, moderate in 54% and poor in 8% of patients. The difference in the percentage of patients with a moderate or good response was statistically significant (P = 0.0481). We also observed a lower rate of complications, such as new-onset or worsening glaucoma or cataract in the infliximab-treated group. CONCLUSION: Anti-TNF-alpha treatment was beneficial in a high percentage of patients with childhood uveitis refractory to standard immunosuppressive treatment. Infliximab resulted in better clinical responses with less ocular complications than etanercept.

MR imaging and angiography of primary CNS vasculitis of childhood.

BACKGROUND AND PURPOSE: Primary angiitis of the central nervous system of childhood (cPACNS) is a rare and ill-defined disease. In the absence of a brain biopsy, the diagnosis is based on typical clinical and imaging abnormalities. The aim of this study was to analyze systematically the MR imaging and MR angiographic (MRA) abnormalities in a large cohort of children with cPACNS. METHODS: We analyzed the MR imaging features of a single pediatric center cohort of 45 cPACNS patients. MR imaging studies were performed for all patients, and both MR imaging and MRA were performed for 42 patients, who formed the cohort for review of the presence and correlation of lesions. Proportions were calculated by using the Fisher exact test, and agreement between MR imaging and MRA was calculated by using the McNemar test. The sensitivity of each diagnostic technique was established. RESULTS: The most-common pattern of parenchymal abnormality was multifocal, unilateral involvement, each in 42/45 patients (93%). The lateral lenticulostriate artery terrritory was affected in 56% of cases, with involvement of a supratentorial deep gray matter structure in 91%. No infratentorial lesion occurred in the absence of supratentorial abnormality. MRA was normal in 12/42 patients (28.6%). Among the abnormal studies, stenosis was detected on MRA in 83% and was "benign" in appearance in 73% of patients and "aggressive" in 16.7%. Involvement was proximal in 83% and distal in 27% of patients. Multiple ipsilateral lesions were seen in 63%. MR imaging was abnormal in every patient where MRA was abnormal. With the assumption of MR imaging as the gold standard, the sensitivity of MRA was 72%. The agreement between MR imaging and MRA for abnormality was significant (P = .04). CONCLUSION: We have illustrated the MR imaging and MRA appearances of cPACNS in the largest cohort to date. Both parenchymal and vascular lesions were predominantly proximal, unilateral, and multifocal within the anterior circulation. There was good agreement between MR imaging and MRA for lesion location. MR imaging findings were abnormal in all cases at diagnosis, and this remains the most sensitive technique to the detection of vasculitis.

The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids.

OBJECTIVE: Childhood dermatomyositis (DM) is often a chronic disease, lasting many years. It has traditionally been treated with long-term corticosteroid therapy; side effects are often seen. For more than a decade, methotrexate (MTX) has been safely used for the treatment of juvenile arthritis. Here, we report use of MTX as first-line therapy for DM, along with aggressively tapered corticosteroids, in an attempt to reduce treatment-related side effects. METHODS: We studied an inception cohort of 31 children with DM who were rigorously followed up in our myositis clinic, and compared them with a control group of 22 patients with incident cases of juvenile DM who received treatment just before we instituted a policy of first-line therapy with MTX. The mean starting dosage of MTX in the study group was 15 mg/m(2)/week. RESULTS: Both groups had similar improvement in strength and physical function; however, the median time during which patients in the study group received corticosteroids was 10 months, compared with 27 months for controls (P < 0.0001). As a result, the cumulative prednisone dose in the study group was approximately half that in the control group (7,574 mg versus 15,152 mg; P = 0.0006). The study group had greater height velocity during the first year of treatment and a smaller increase in the body mass index over the first 2 years. In the control group, the relative risk of cataracts developing was 1.95 (95% confidence interval 1.05-4.17). Side effects of MTX were rarely observed. CONCLUSION: Use of MTX in conjunction with an aggressively tapered course of prednisone may be as effective as traditional long-term corticosteroid therapy for children with DM, while decreasing the cumulative dose of corticosteroids.

Magnetic resonance imaging assessment for unicompartmental knee replacement: a limited role.

The purpose of this study was to see if MRI has a role in pre-operative assessment of patients for unicompartmental knee replacement. Until now, surgeons have been unable to predict whether a patient is suitable until the operation itself when the anterior cruciate ligament is inspected. We found that 33% of patients with anteromedial osteoarthritis had a degenerate anterior cruciate ligament according to magnetic resonance imaging, compared to only 13% on surgical inspection. We conclude that MRI is too sensitive to changes of the anterior cruciate ligament to be of much practical value.