Cognitive and psychiatric signs revealing Sneddon syndrome: A case report.

Key Clinical Message: The diagnosis of Sneddon Syndrome should be considered in adults with young-onset dementia accompanied by neuropsychiatric signs and livedo racemosa. Magnetic resonance imaging and cerebral angiography are essential. A cutaneous biopsy may help in the diagnosis. Abstract: Sneddon syndrome (SS) is a clinical entity corresponding to a noninflammatory thrombotic vasculopathy that typically includes livedo racemosa and cerebrovascular ischemia. Psychiatric symptoms with cognitive impairment often occur but are rarely the inaugural symptoms. We present a case of secondary SS in a 45-year-old man in whom dementia and psychosis revealed the disease.

The T4-L1-Hip Axis: Defining a Normal Sagittal Spinal Alignment.

STUDY DESIGN: This is a cross-sectional cohort. OBJECTIVE: The aim was to describe sagittal plane alignment and balance in a multinational cohort of nondegenerated, asymptomatic adults. SUMMARY OF BACKGROUND DATA: Current sagittal alignment targets were developed using correlations between radiographic and quality-of-life measures in spinal deformity patients, rather than disease-free samples leading to relatively poor accounting for variance within a population. MATERIALS AND METHODS: Sagittal balance was defined using vertebral body tilt and spinopelvic alignment was defined as the vertebral pelvic angles from C2 to L5 (vertebral pelvic angle=vertebral tilt+pelvic tilt). Associations with pelvic incidence (PI) were assessed using linear regression. Multivariable linear regression was used to estimate a normal L1-S1 lordosis, adjusting for PI and the L1 pelvic angle (L1PA). Correlation between the L1 and T4 pelvic angles was assessed to define a normal thoracic alignment conditioned on lumbar alignment. RESULTS: Among 320 volunteers from 4 continents, median age was 37% and 60% were female. C2 tilt was independent of PI with minimal variation. PI was inadequate for estimating a normal lumbar lordosis (L1-S1, r2 =0.3), but was strongly associated with the lumbar pelvic angles (L1PA, r2 =0.58). Defining lumbar lordosis as a function of PI and L1PA resulted in high explained variance ( R2 =0.74) and the T4 pelvic angle had near perfect correlation with the L1PA ( r =0.9). CONCLUSIONS: We defined normal sagittal balance and spinopelvic alignment in a disease-free international volunteer cohort. Four parameters are either fixed or directly modifiable in surgery and can define a normal thoracic and lumbar alignment: the L1-S1 lordosis defined as a function of PI and the L1PA; and the T4 pelvic angle is nearly equivalent to the L1PA, aligning the T4-L1-hip axis.

Pelvic thickness, sex, ethnicity, and age affect pelvic incidence in healthy volunteers of Multi-Ethnic Alignment Normative Study (MEANS) database.

BACKGROUND: The purpose of this study was to investigate the affecting factors on pelvic incidence (PI) and to test the hypothesis that PI changes even after skeletal maturity probably due to hypermobility of the sacroiliac joint using a large international multi-center database. METHODS: A prospective and cross-sectional healthy adult volunteers, ages 18-80 years, across 5 countries were used. Radiographic measurements included standard whole body alignment parameters. Bivariate regression analyses between PI versus demographics and spino-pelvic anatomical parameters were performed. An effect of sex on pelvic anatomical parameters was also investigated. Multivariate logistic regression with a forward stepwise procedure was performed to identify the contributing factors to PI, and an appropriate model was obtained. RESULTS: PI showed a significant positive correlation with age in pooled data. Divided by sex, however, there was no correlation in men, but women showed a significant higher correlation coefficient. Pelvic thickness (PTh) had a significant negative correlation with age in pooled data. Divided by sex, no correlation was found in men, but there was a significant correlation in women with higher correlation coefficient. The stepwise multivariate analysis for the factors on PI identified four significant factors: age, sex, ethnicity, and PTh. CONCLUSIONS: PTh, sex, ethnicity, and age affected PI. There was a positive correlation between PI and age. The tendency was more significant in woman than in man. The results support the hypothesis that PI increases with aging, but the change seems to be small and needs to be verified in a longitudinal evaluation.

Brucellar spondylodiscitis: A case series with focus on histopathological features.

Brucellar spondylodiscitis (BS) is the most common form of musculoskeletal brucellosis. The isolation of Brucella spp from blood, other body fluids or tissue cultures is the gold standard for definitive diagnosis of BS. BS shows a large histopathological spectrum of lesions with non-specific and granulomatous forms, and its histopathological features are not widely reported. This case series study reported the histopathological features of a series of 21 BS. Ten cases revealed nonspecific forms of BS. Lymphocytes were the predominant inflammatory cells in this group. In one case neutrophils were predominant. The eleven remaining were granulomatous. The 11 exhibited histiocytic type granulomas. Ten were non-necrotizing. One case, taken from abscess wall, contained minimal deposition of caseous like necrotic material. This case and 2 others corresponding to abscess wall biopsies, showed histiocytic granulomas mixed with small aggregates of epithelioid cells without well-formed epithelioid granuloma nor giant cell. The histopathological diagnosis of BS is challenging. Nonspecific forms of BS, mimiking pyogenic spondylodiscitis, are observed in about half of the cases. Although nonspecific forms of BS are characterized by the predominance of lymphocytes and plasmocytes, BS forms with predominant neutrophil cell infiltration do exist. Histiocytic granuloma is highly suggestive of BS. Purely epithelioid forms of BS were not observed in the present series. Caseous like necrosis suggestive of tuberculosis is a possible feature of BS.

[The value of MRI in the diagnosis of joint involvement in malignant primitive tumors of the knee]. / Valeur de l'IRM dans le diagnostic de l'envahissement articulaire par les sarcomes osseux du genou.

INTRODUCTION: Surgical treatment of malignant primitive tumors of bone needs a precise preoperative assessment of tumor local extension. Joint involvement (JI) represents the most important finding to determine, for the choice of surgical procedure (intra- or extra-articular resection). OBJECTIVE: To determine the value of different MR signs for the diagnosis of joint involvement in malignant primitive tumors of the knee. METHODS: Ten-year period retrospective study of 42 patients. Patients' clinical and imaging data have been studied. Two senior musculoskeletal radiologists have blindly and consensually reviewed imaging data. Histopathological data have been reviewed by an experimented pathologist. The results have been compared using several statistical methods to determine the global and detailed (sign by sign) diagnostic value and accuracy of MRI by reference to histopathology. RESULTS: Some MR signs were performant in the diagnosis of joint involvement. The most sensitive were epiphyseal extension (Se: 100%), osteochondral extension (Se: 87.5%) and extension to the tibial spines (Se: 83.3%). The most specific MR signs were the presence of an intra-articular mass (Sp: 84.62%), extension to the intercondylar notch (Sp: 80.77%) and longitudinal axis≥120mm (Sp: 84.62%). CONCLUSION: A rigorous and reproducible MR technique has to be used, the knee being explored in the transversal, sagittal and coronal planes. MR conclusion has to be clear and simple (intact, doubtful or involved joint). MR interpretation has to consider tumoral extension pathways. In doubtful cases, CT may help in this diagnosis by studying the bone cortex. LEVEL OF EVIDENCE: IV.

Assessment of preclinical atherosclerosis in patients with ankylosing spondylitis.

OBJECTIVE: Epidemiological studies recently confirmed the increased risk of vascular morbidity and mortality during ankylosing spondylitis (AS). Increase of intima-media thickness (IMT) of the common carotid artery is a useful and noninvasive marker of preclinical atherosclerosis. The aim of our study was to compare IMT in patients with AS with matched controls and to determine risk factors of atherosclerosis related to AS. METHODS: We performed a prospective study of 60 consecutive patients meeting modified New York criteria for AS, compared to 60 controls matched for age and sex. Disease-specific measures were determined. Measurement of IMT was performed by the same radiologist using the same machine and probe in right and left common carotid arteries, and the average of the 2 measurements was considered. RESULTS: In total 48 male and 12 female patients were recruited, and 60 corresponding controls; mean age was 36 ± 11 years. We found significantly increased IMT in the AS group (0.51 ± 0.12 mm) compared with controls (0.39 ± 0.09 mm; p = 0.001). After adjustment for confounding factors, increased IMT was still present (p = 0.003). Age at onset of AS (p = 0.001), Bath AS Disease Activity Index (p = 0.002), AS Disease Activity Score (ASDAS) erythrocyte sedimentation rate (ESR; p = 0.047), ASDAS C-reactive protein (CRP; p = 0.012), Bath AS Functional Index (p = 0.008), global spine visual analog scale for pain (p = 0.000), Schober index (p = 0.039), Bath AS Metrology Index (p = 0.028), modified Stoke Ankylosing Spondylitis Spine Score (p = 0.035), and high ESR (p = 0.001) and CRP (p = 0.000) were correlated with high IMT in patients with AS. Otherwise, status of arthritis (p = 0.442), enthesitis (p = 0.482), and HLA-B27 (p = 0.528) seemed to have no effect on IMT. CONCLUSION: AS is associated with an increased risk of atherosclerosis independent of traditional risk factors. Disease activity, functional and mobility limitations, structural damage, and inflammation are the most incriminated risk factors.

Correlations among clinical, radiographic, and sonographic scores for enthesitis in ankylosing spondylitis.

OBJECTIVES: To look for correlations among clinical, radiographic, and sonographic scores for enthesitis in patients with ankylosing spondylitis (AS). METHODS: Prospective study of 60 patients meeting modified New York criteria for AS. The clinical evaluation relied on the BASDAI, BASFI, and ASQoL and on a visual analog scale (VAS) for entheseal pain, as well as on two specific enthesitis indices, the Maastricht Ankylosing Spondylitis Enthesitis Score (MASES) and the Spondyloarthritis Research Consortium of Canada Enthesitis Index (SPARCC). Radiographs and ultrasound scans were taken of five entheses on both sides (patellar insertion of the quadriceps tendon, proximal and distal insertions of the patellar tendon, and calcaneal insertions of the Achilles tendon and superficial plantar fascia). Ultrasound scans were obtained using a Philips HD 11™ machine with a high-frequency linear probe. RESULTS: We studied 48 men and 12 women with a mean age of 36±11 years. The radiographic score correlated with the VAS pain score, BASDAI, and BASFI. The sonographic score for acute enthesitis correlated only with the MASES, and the sonographic score for chronic enthesitis correlated with none of the clinical scores. The Doppler score correlated with the VAS pain score, BASDAI, BASFI, and ASQoL. The overall sonographic score correlated with the MASES and SPARCC. CONCLUSION: Good correlations were found between the clinical and sonographic scores for enthesitis. The radiographic score seemed correlated with the general AS parameters rather than with the clinical scores. Larger studies are needed to better define the role for radiographs and sonography of the entheses in the diagnosis of AS and follow-up of treated AS patients.

Imaging of chest wall infections.

A wide variety of infections can affect the chest wall including pyogenic, tuberculous, fungal, and some other unusual infections. These potentially life-threatening disorders are frequent especially among immunocompromised patients but often misdiagnosed by physical examination and radiographs. The purpose of this article is to describe the clinical and imaging features of these different chest wall infections according to the different imaging modalities with emphasis on ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI). The outcome of chest wall infection depends on early diagnosis, severity of the immunosuppression, offending organism, and extent of infection. Because clinical findings and laboratory tests may be not contributive in immunocompromised patients, imaging plays an important role in the early detection and precise assessment of the disease. US, CT, and MRI are all useful: bone destruction is more accurately detected with CT whereas soft tissue involvement are better visualized with US and MRI. CT and US are also used to guide percutaneous biopsy and drainage procedures. MR images are helpful in pre-operative planning of extensive chest wall infections.

Extralobar pulmonary sequestration revealed by prenatal hydrothorax.

BACKGROUND: Pulmonary sequestration is a rare congenital pulmonary anomaly that can be diagnosed in utero. AIM: Report a New case. CASE REPORT: In this case report of extralobar pulmonary sequestration, the authors report a case revealed by hydrothorax and describe this disease appearance in different imaging technique (Doppler ultrasonography, magnetic resonance imaging and postnatal multislice CT angiography).

Spinal brucellosis: a review.

Brucellosis is a zoonosis of worldwide distribution, relatively frequent in Mediterranean countries and in the Middle East. It is a systemic infection, caused by facultative intra-cellular bacteria of the genus Brucella, that can involve many organs and tissues. The spine is the most common site of musculoskeletal involvement, followed by the sacroiliac joints. The aim of this study was to assess the clinical, biological and imaging features of spinal brucellosis.

Idiopathic tumoral calcinosis.

Idiopathic tumoral calcinosis is an unusual benign condition characterized by the presence of calcified soft tissue masses of varying size around the joints. In this retrospective study,clinical data and radiological features of nine cases of idiopathic tumoral calcinosis are reviewed. Imaging features, particularly magnetic resonance imaging findings are detailed.