Canavan's spongiform leukodystrophy (Aspartoacylase deficiency) with emphasis on sonographic features in infancy: description of a case report and review of the literature.

Canavan disease (CD; MIM 271,900) or spongy degeneration of the central nervous system (CNS) is a lethal, rare autosomal recessive leukodystrophy, first described in 1931 (Canavan in Arch Neurol Psychiatry 25: 299-308, 1931). The clinical presentation includes severe neurologic impairment and macrocephaly with onset of symptoms at the age of 3-5 months. Biochemical and genetic fundamentals of the disease are elucidated. Imaging diagnosis is principally based on MRI with important role of MR spectroscopy. We report the cerebral sonographic findings in a severely affected infant with CD: Diffuse hyperechogenicity and small multicystic changes of white matter as well as an inverted pattern of echogenicity between cortical gray and subcortical white matter. These findings are compared to to the few cases found in literature and to normal ultrasound examples. Finally, ultrasound and MRI imaging findings are correlated.

Rhizomelia and Impaired Linear Growth in a Girl with Juvenile Paget Disease: The Natural History of the Condition.

In ultra-rare bone diseases, information on growth during childhood is sparse. Juvenile Paget disease (JPD) is an ultra-rare disease, characterized by loss of function of osteoprotegerin (OPG). OPG inhibits osteoclast activation via the receptor activator of nuclear factor-κB (RANK) pathway. In JPD, overactive osteoclasts result in inflammatory-like bone disease due to grossly elevated bone resorption. Knowledge on the natural history of JPD, including final height and growth, is limited. Most affected children receive long-term antiresorptive treatment, mostly with bisphosphonates, to contain bone resorption, which may affect growth. In this study, we report the follow-up of height, growth velocity, and skeletal maturation in a 16-year-old female patient with JPD. The patient was treated with cyclic doses of pamidronate starting at 2.5 years of age and with 2 doses of denosumab at the age of 8 years, when pamidronate was paused. In the following years, a sustainable decline in a height z-score and a stunted pubertal growth spurt; despite appropriate maturation of the epiphyseal plates of the left hand, the proximal right humerus and both femora were observed. Whether this reflects the growth pattern in JPD or might be associated to the antiresorptive treatments is unclear, since there is very limited information available on the effect of bisphosphonates and denosumab on growth and the growth plate in pediatric patients. Studies are needed to understand the natural history of an ultra-rare bone disease and to assess the effects of antiresorptive treatment on the growing skeleton.

Anxiety of patients undergoing CT imaging-an underestimated problem?

RATIONALE AND OBJECTIVES: Prospective evaluation of anxiety in patients undergoing computed tomography (CT) imaging using a standardized state-trait anxiety inventory (STAI-S) and identification of possible risk factors. MATERIAL AND METHODS: During a 9-month interval, patients undergoing CT were questioned using STAI-S. Additionally, 10 questions concerning specific procedure-related features (claustrophobia, radiation, administration of contrast, and so forth) were added. Moreover, sex, age, admitting subspecialty, organ region, reason for imaging, and prior imaging studies were recorded. Statistical analysis was performed using the Student t test and linear regression analysis; significance level was set to 5%. RESULTS: Of 6122 patients, 825 patients undergoing CT (14%) were included (67% men; average age, 54 ± 17 years). Average STAI was 42 ± 10 with women (45 ± 11 vs. 41 ± 10; P < .001) and patients who received intravenous contrast (43 ± 10 vs. 42 ± 11; P = .021) showing significantly higher anxiety levels compared to those without contrast. Patients with investigations of their extremities (41 ± 11 vs. 43 ± 10; P = .020) and trauma patients (41 ± 11 vs. 43 ± 10; P = .006) revealed significantly lower STAI results. Patients who had never received a CT scan before showed significantly greater STAI-S values than those with repeat studies (42 ± 10 vs. 41 ± 11; P = .036). Females had greater fears concerning examination results (P < .001), radiation exposure (P = .032), administration of contrast (P = .014), and claustrophobia (P < .001). Patients with known malignancies had a significantly higher level of anxiety concerning their CT results (P = .002). CONCLUSIONS: Anxiety does not only occur before MRI but also occur before CT. Its sources are manifold and include communication of CT results, administration of contrast agents, radiation exposure, and claustrophobia. In this setting, women seemed to be more receptive than men.

Association between clinical signs assessed by manual segmental examination and findings of the lumbar facet joints on magnetic resonance scans in subjects with and without current low back pain: a prospective, single-blind study.

The relevance of magnetic resonance imaging (MRI) findings such as facet joint (FJ) effusion and edema in low back pain (LBP) is still unknown. Therefore, we prospectively evaluated the presence of these MRI findings in the lumbar spine (Th12-S1) and their association with pain evoked by manual segmental FJ provocation tests (spinal percussion, springing, and segmental rotation tests) in 75 subjects with current LBP (≥30 days in the past 3 months) compared with 75 sex- and age-matched control subjects. FJs were considered painful, if ≥ 1 provocation test triggered LBP. FJs were classified as true positives, if the same FJ was painful and showed effusion and/or edema. FJs with effusion and/or edema and painful FJs were present significantly more frequently in subjects with LBP, but these conditions were also common in control subjects (27% vs 21% and 50% vs 12%, respectively). Effusion and/or edema were present in 65 subjects with LBP (87%) and in 56 control subjects (75%, not significant); painful FJs were present in 68 (91%) and 29 (39%) (P<0.01) LBP and control subjects, respectively. True-positive findings occurred in 16% of LBP FJs and in 2% of control FJs (P<0.01); 46 LBP subjects (61%) and 9 control subjects (12%, P<0.01) had true-positive findings. Pain on provocation and FJ effusion and/or edema were significantly correlated only in patients with LBP. In conclusion, only true-positive findings (ie, concurrent effusion and/or edema and positive provocation test results in the same FJ) discriminate well enough between control subjects and subjects with current LBP, whereas neither effusion and/or edema nor FJ provocations tests alone are suitable to detect suspected FJ arthropathy.

Interventional radiology at the meetings of the German Radiological Society from 1998 to 2008: evaluation of structural changes and radiation issues.

OBJECTIVES: Evaluation of structural changes and the weight given to radiation exposure of interventional radiology (IR) contributions at the Congress of the German Radiological Association from 1998 to 2008. METHODS: All IR abstracts were evaluated for type of contribution, design, imaging modality, and anatomic region. Weight given to radiation exposure was recorded as general statement, main topic and/or dose reduction. Statistical analysis included calculation of absolute/relative proportions of subgroups and ANOVA regression analyses. RESULTS: Out of 9,436 abstracts, 1,728 (18%) were IR-related. IR abstracts significantly rose to a maximum of 200 (20%) in 2005 (P = 0.048). While absolute numbers of scientific contributions declined, educational contributions significantly increased (P = 0.003). Computed tomography (CT) and magnetic resonance imaging (MRI) were the main IR imaging modalities, with growing use of CT (P = 0.021). The main body regions were vessels (45%) and abdomen (31%). Radiation exposure was addressed as a general statement in 3% of abstracts, as a main topic in 2%, and for dose reduction in 1%, respectively. During the study interval a significant growth of dose reduction abstracts was observed (P = 0.016). CONCLUSIONS: IR emerged as a growing specialty of radiology, with a significant increase in educational contributions. Radiation exposure was rarely in the focus of interest but contributions relating to dose reduction demonstrated a significant growth during the study period. Main Messages • Interventional radiology emerged as a growing specialty at the German radiological congress. • Significant increments of educational and prospective research contributions could be observed. • Despite a significant trend towards computed tomography, radiation exposure of IR was rarely in the focus of interest. • Contributions related to dose reduction demonstrated a significant growth during the study period.

Efficacy of CT-guided biopsies of the spine in patients with spondylitis--an analysis of 164 procedures.

OBJECTIVE: To evaluate efficacy of CT-guided spinal biopsy (CTSB) in patients with spondylitis considering patient characteristics, technical issues, antibiotic therapy, histopathological, and microbiological findings. MATERIALS AND METHODS: All CTSB procedures performed between 1995 and 2009 in patients with proven spondylitis were re-evaluated. Patient sex and age, antibiotic treatment, biopsy approach, number of specimens, length of needle path, laboratory results (CRP, WBC), and histopathological/microbiological findings were documented and compared to the final diagnosis of spondylitis. Statistical analysis was performed using Chi-square test and Student's t-test. The p-value was set to 5%. RESULTS: 164 CTSB procedures were performed in 159 patients (mean age 65 years, 60% men) in which spondylitis was histopathologically verified in 95%. Neither patient sex nor age, positioning, localization of the spinal lesion, bioptic approach, number of specimens, or depth of the needle showed significant impact on the rate of positive histopathological findings. A causative germ was identified in 40/127 biopsies (32%) with Staphylococcus aureus being identified in 50%. Tuberculous spondylitis was diagnosed in ten cases (6%). CRP significantly correlated with bacterial growth (13.3±12.2 mg/dl versus 8.8±7.6 mg/dl; p=.015) whereas administration of antibiotics did not show any significant impact on bacterial growth (29% versus 36% in patients without antibiotics; p=0.428). Patients with histopathological signs of active spondylitis showed a significantly higher CRP (16.5±15.8 mg/dl versus 8.9±8.0 mg/dl, p<.001). Complication rate was 0.6% (one focal bleeding). CONCLUSION: CTSB of the spine in suspected spondylitis is an effective and safe procedure for establishing final histopathological diagnosis. However, microbiological yield is low regardless of technical issues and antibiotic therapy. Other than CRP values, laboratory investigations added little useful information to diagnose infection in our study group. CRP values significantly correlated with bacterial growth and with histopathological signs of active spondylitis.

Multidetector-CT angiography in pulmonary embolism-can image parameters predict clinical outcome?

OBJECTIVE: To assess if pulmonary CT angiography (CTA) can predict outcome in patients with pulmonary embolism (PE). METHODS: Retrospective analysis of CTA studies of patients with PE and documentation of pulmonary artery (PA)/aorta ratio, right ventricular (RV)/left ventricular (LV) ratio, superior vena cava (SVC) diameter, pulmonary obstruction index (POI), ventricular septal bowing (VSB), venous contrast reflux (VCR), pulmonary infarction and pleural effusion. Furthermore, duration of total hospital stay, necessity for/duration of ICU therapy, necessity for mechanical ventilation and mortality were recorded. Comparison was performed by logistic/linear regression analysis with significance at 5%. RESULTS: 152 patients were investigated. Mean duration of hospital stay was 21 ± 24 days. 66 patients were admitted to the ICU; 20 received mechanical ventilation. Mean duration of ICU therapy was 3 ± 8 days. Mortality rate was 8%. Significant positive associations of POI, VCR and pulmonary infarction with necessity for ICU therapy were shown. VCR was significantly associated with necessity for mechanical ventilation and duration of ICU treatment. Pleural effusions were significantly associated with duration of total hospital stay whereas the RV/LV ratio correlated with mortality. CONCLUSION: Selected CTA findings showed significant associations with the clinical course of PE and may thus be used as predictive parameters.

Cumulative effective dose associated with radiography and CT of adolescents with spinal injuries.

OBJECTIVE: The purpose of this study was to analyze the quantity and distribution of cumulative effective doses in diagnostic imaging of adolescents with spinal injuries. MATERIAL AND METHODS: At a level 1 trauma center from July 2003 through June 2009, imaging procedures during initial evaluation and hospitalization and after discharge of all patients 10-20 years old with spinal fractures were retrospectively analyzed. The cumulative effective doses for all imaging studies were calculated, and the doses to patients with spinal injuries who had multiple traumatic injuries were compared with the doses to patients with spinal injuries but without multiple injuries. The significance level was set at 5%. RESULTS: Imaging studies of 72 patients (32 with multiple injuries; average age, 17.5 years) entailed a median cumulative effective dose of 18.89 mSv. Patients with multiple injuries had a significantly higher total cumulative effective dose (29.70 versus 10.86 mSv, p < 0.001) mainly owing to the significantly higher CT-related cumulative effective dose to multiple injury patients during the initial evaluation (18.39 versus 2.83 mSv, p < 0.001). Overall, CT accounted for 86% of the total cumulative effective dose. CONCLUSION: Adolescents with spinal injuries receive a cumulative effective dose equal to that of adult trauma patients and nearly three times that of pediatric trauma patients. Areas of focus in lowering cumulative effective dose should be appropriate initial estimation of trauma severity and careful selection of CT scan parameters.

Growth plate fractures of the distal tibia: is CT imaging necessary?

PURPOSE: Comparison of conventional radiographs (CR) of distal tibial growth plate fractures [Salter-Harris (SH) fracture types I-V/triplane fractures I-III] with computed tomography (CT) as the reference standard and assessment of diagnostic benefit of CT imaging in the affected patients. MATERIALS AND METHODS: We retrospectively evaluated all growth plate injuries of the distal tibia with complete pre-therapeutic imaging material (CR and CT including MPR) obtained between August 2001 and December 2006. The imaging material was randomised and presented to two radiologists. Fracture of metaphysis, epiphysis and epiphyseal line were noted separately for distal tibia. In case of fracture, involvement of the articular surface, articular dehiscence and ridge formation, subluxation and number of tibial fragments were evaluated. All fractures were classified as SH type I-V or triplane fractures type I-III. Sensitivity, specificity, positive and negative predictive value and overall accuracy of CR were compared to CT. RESULTS: Thirty-three patients (mean age 14 ± 2 years) were evaluated. CR showed significantly less tibial fragments as compared to CT (1.39 ± 0.75 vs. 1.61 ± 1.25; p = 0.023). The overall accuracy of CR was <90% for fracture involving the metaphysis (82%), dehiscence of the articular surface (64%), ridge formation of the articular surface (61%) and subluxation (79%). The CR evaluation showed differing SH classification in CT in 10/33 cases (30%) with the highest misclassification rates in type-III SH fracture. For evaluation of triplane fractures, CR classification was incorrect in five cases (71%) out of seven. No misclassification occurred in types I and II SH fractures. CONCLUSION: The CR of distal tibial growth plate fractures showed a low overall accuracy for articular surface dehiscence, articular ridge formation and subluxation as compared to CT. CT revealed significantly more fragments. It is difficult to correctly classify type III/IV SH fractures and triplane fractures with CR thus emphasising the necessity of using CT in selected patients.

Paediatric radiology and scientific contributions to radiation dose at the meeting of the German Radiological Society--an analysis of an 11-year period.

AIM: Evaluation of the emphasis on themes pertaining to paediatric radiology and radiation dose at the Meeting of the German Radiological Society from 1998 to 2008 in comparison to international data. MATERIALS AND METHODS: Retrospective analysis of 9440 abstracts with documentation of type of contribution, imaging modality, and examined body region. Abstracts primarily dealing with paediatric radiology and those stating radiation dose were documented. Results were compared with a Pubmed query. RESULTS: 448 contributions in paediatric radiology were presented corresponding to 5% of all abstracts with an increase from 5 (1998) to 7% (2008). The proportion of prospective studies of all congress contributions was 10%, whereas in paediatric radiology, the share of prospective studies was 6%. From 1998 to 2008, the share of MRI fell from 48 to 38%, while CT contributions rose from 30 to 34%. Within paediatric radiology, the proportion of CT rose from 23 to 29%, while MRI and ultrasound fell from 63 to 48% and 35 to 19%, respectively. The share of abstracts dealing with radiation dose rose from 7 to 10% while that primarily pertaining to dose reduction grew from 2 to 4%. Of all abstracts concerning CT, 15% touched on radiation dose, whereas 6% primarily dealt with dose reduction. Among all abstracts dealing with paediatric radiology, 20 and 6% mentioned radiation dose and dose reduction, respectively. In the subgroup of paediatric radiology CT abstracts, radiation dose and dose reduction were mentioned in 34 and 16%, respectively. An online query produced 137,791 publications on CT, of whose abstracts 3% mentioned radiation dose and 0.5% mentioned dose reduction. 11% of all CT publications dealt with paediatric populations and 2% of these publications examined questions of radiation dose. CONCLUSIONS: In the last 11 years the Meeting of the German Radiological Society has presented a growing number of contributions pertaining to paediatric radiology. CT has shown the most pronounced growth of all contributions. Paediatric radiology has significantly more often dealt with questions of radiation exposure than those from general radiology. However, contributions with definite reference to radiation dose, both pertaining to all publications and specifically to those dealing with CT, remain a minority, albeit with a higher proportion when compared to international data.

MRI in lipid-lowering agent-associated myopathy: a retrospective review of 21 cases.

OBJECTIVE: The objective of our study was to identify disease-specific patterns of myopathic changes in patients with lipid-lowering agent (LLA)-associated myopathy using a dedicated MRI protocol. MATERIALS AND METHODS: Axial T1- and T2-weighted STIR images were obtained for the detection of lipomatosis and edema, respectively, of the thighs and legs. Information about patient age, sex, duration of dyslipidemia and of medication use, latency of symptom onset, maximum creatine kinase (CK) level, presence of myalgia and muscle weakness, and LLA or LLAs used was collected. RESULTS: Significant correlations resulted for the radiologic diagnosis of LLA-associated myopathy and maximum CK level; the presence of edema and maximum CK level; edema and weakness in the ventral thigh muscle group, thigh adductors, or deep dorsal muscle group of the legs; and lipomatosis and weakness of the deep dorsal muscle group of the legs and peroneal muscle group, respectively. CONCLUSION: MRI is a method that helps detect active pathologic processes in patients with suspected LLA-associated myopathy but does not reveal disease-specific patterns.

Paediatrician awareness of radiation dose and inherent risks in chest imaging studies--a questionnaire study.

OBJECTIVE: To assess paediatricians' knowledge regarding radiation exposure of chest imaging. MATERIALS AND METHODS: German paediatricians were surveyed using a questionnaire. Participants were asked to estimate effective dose (ED) of radiographs (CR) and computed tomography (CT). Further questions included dose-saving of paediatric CT-protocols, ALARA principle, and awareness of the link between radiation and cancer development. Length and type of occupation and amount of ordered procedures were evaluated. RESULTS: 137 paediatricians participated with 59% and 39% correctly estimating ED of an adult (0.01-0.1mSv) and newborn CR (0.01-0.1mSv), respectively. ED of an adult chest CT (1-10mSv) was underestimated by 28%, whereas ED of cardiac CT (10-100mSv) was underestimated by 54%. 35% of participants correctly estimated ED of a chest CT in an infant (10-100mSv) which was underestimated by 56%. Neither length nor type of occupation showed significant impact on dose estimations. 14% of paediatricians stated that MRI causes radiation, whereas 4% correctly estimated the potential of paediatric CT-protocols. 15% were familiar with the ALARA principle and 26% were aware of a publication concerning radiation and malignancy. CONCLUSION: Paediatricians demonstrated an increased level of awareness compared to previous surveys. However, estimation of ED of CT remained difficult. Increased information transfer and education seem pressing in the light of increasing radiological examinations.

Which labral lesion can be best reduced with external rotation of the shoulder after a first-time traumatic anterior shoulder dislocation?

INTRODUCTION: Immobilization in external rotation after a first-time traumatic anterior shoulder dislocation has been shown to improve the position of the labroligamentous lesion relative to the glenoid rim. The purpose of the present study was to evaluate the effect of the external rotation position of the shoulder on different types of labroligamentous lesions in patients with first-time traumatic anterior shoulder dislocation by using MRI. PATIENTS AND METHODS: We performed a standardized MRI in internal and external rotation of the shoulder after initial reduction in 34 patients with a first-time traumatic anterior shoulder dislocation. Labroligamentous lesions were classified as Bankart, Perthes, or nonclassifiable. Four distinct grades were used to classify the amount of plastic deformation of the anterior labroligamentous structures. The position of the labrum was defined relative to the tip of the glenoid rim by measuring the dislocation and separation. RESULTS: In all patients, dislocation and separation of the labrum relative to the rim of the glenoid were significantly improved in shoulders in the external rotation position compared to those in the internal rotation position. We observed 15 Bankart, 15 Perthes, and 4 non-classifiable lesions. No HAGL or GLAD lesions were found. Fourteen patients showed a plastic deformation grade I, 16 showed grade II, 3 showed grade III, and 1 showed grade IV. In regression analysis, the odds ratio was 1.100 for the type of lesion and 1.660 for the grade of plastic deformation. Perthes lesions (with an intact anterior scapular periosteum) and grade I plastic deformations showed the best labral reduction on the external rotation MRI. CONCLUSION: Placing the shoulder in external rotation after a first-time traumatic shoulder dislocation, significantly improves the position of the labroligamentous lesion on the glenoid rim. Perthes lesions that showed a low grade of plastic deformation displayed better reduction in external rotation and then compared to Bankart or other lesions that showed a high grade of plastic deformation. In conclusion, immobilization of the shoulder after a first-time traumatic shoulder dislocation is most effective in patients with Perthes lesions that show low grade plastic deformation.

Computed tomography-navigated transthoracic core biopsy of pulmonary lesions: which factors affect diagnostic yield and complication rates?

RATIONALE AND OBJECTIVES: Only a few studies have systematically evaluated risk factors for pneumothorax and pulmonary hemorrhage in computed tomographically (CT)-guided transthoracic lung biopsy (TLB). We evaluated the diagnostic yield of CT-guided TLB and determined risk factors for pneumothorax and hemorrhage. METHODS: One hundred seventy-two CT-guided TLBs were performed on 159 patients (mean age 66 +/- 11 years; 72% male) using a 16-gauge core biopsy needle. Lesion and patient characteristics, lung function analysis, CT signs of emphysema, histopathologic diagnoses, and complications were recorded. Statistical analysis was performed with multivariate regression analysis. RESULTS: Histopathologic diagnosis was established in 153 cases (89%). Although lesion size was higher (47 +/- 29 vs. 43 +/- 35 mm, P = .191) and depth was lower (22 +/- 23 vs. 6 +/- 23 mm, P = .350) in procedures with histopathologic diagnosis, no parameter showed significant impact on diagnostic yield. Sensitivity and specificity for detection of malignancy were 93% and 100%, respectively, whereas positive and negative predictive values were 100% and 88%. Overall accuracy was 95%. Pneumothorax occurred in 45 procedures (26%). Hemorrhage was recorded in 17 procedures (10%). There was higher frequency of pneumothorax in smaller lesions (35 +/- 23 vs. 50 +/- 31 mm, P = .003; odds ratio = .96) and greater depth (29 +/- 29 vs. 20 +/- 19 mm, P = .05; odds ratio = 1.03). CT signs of emphysema revealed higher incidence of hemorrhage (35% vs. 23%; P = .04; odds ratio=41.03). Other parameters were nonsignificant. CONCLUSIONS: The high diagnostic yield of CT-guided TLB was not affected by lesion characteristics or emphysema. Pneumothorax rate was influenced by lesion size and depth. Hemorrhage was associated with CT signs of emphysema.

Effectiveness of CT-guided percutaneous biopsies of the spine: an analysis of 202 examinations.

RATIONALE AND OBJECTIVES: The study goal was to retrospectively evaluate the effectiveness of computed tomography (CT)-guided spinal biopsies. MATERIAL AND METHODS: Two hundred two CT-guided vertebral biopsies performed between May 1999 and June 2004 in 187 patients were retrospectively analyzed. Patient characteristics (age, sex, antibiotic therapy), technical parameters (depth and number of biopsies, needle approach), lesion features (spinal level, osteolysis, fluid collections, soft tissue tumor), and complications were documented. Furthermore, histopathological and microbiological diagnoses were considered. RESULTS: There were two focal hematomas in our study group (complication rate: 1%). Histopathological diagnosis was established in 74% of examinations with spondylitis (41% of cases) being most common. In spinal tumors (27% of cases), malignant lesions were found in 52 of 54 examinations (96%). Osteolysis was diagnosed in 98% of patients with spondylitis and in 87% of patients with tumors (P < .01). Spinal tumors were most commonly seen in the sacrum (53%, P < .001), whereas spondylitis typically occurred in the lumbar spine (55%, P = .001). Neither patient age nor sex, needle approach, needle depth, or vertebral abnormalities showed a significant impact on diagnostic accuracy. Microbiological tests were performed in 98 patients (49%); 62 of 98 patients (65%) received antibiotic therapy. In 12 of 62 patients (19%) with antibiotic therapy and in 9 of 36 patients (25%) without antibiotic treatment, microbiological tests were positive (P = .153). Staphylococcus aureus was found in 9 of 21 examinations (43%). CONCLUSIONS: CT-guided vertebral biopsy is a safe and effective procedure to establish final diagnosis in spinal lesions of unclear origin. Patient characteristics, lesions features, and technical considerations did not influence sample quality. In spondylitis, which was commonly caused by Staphylococcus aureus, microbiological yield was low regardless of antibiotic therapy.

CT angiography in suspected pulmonary embolism: impact of patient characteristics and different venous lines on vessel enhancement and image quality.

OBJECTIVE: The objective of our study was to compare image quality, patient characteristics, and different catheters in pulmonary CT angiography (CTA) performed with bolus tracking and z-axis automated tube current modulation (ATCM) in patients with suspected pulmonary embolism. SUBJECTS AND METHODS: One hundred twenty-six patients were referred to undergo pulmonary CTA with bolus tracking and ATCM. Besides patient characteristics, the type, position, size, and side of venous catheters were documented. Pulmonary vessel enhancement and image noise were quantified; signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were calculated. Subjective vessel contrast was assessed by two radiologists in consensus. RESULTS: Patient age showed a moderate but significant positive correlation to vessel enhancement (r = 0.244, p = 0.006), CNR (r = 0.178, p = 0.046), and subjective image quality (r = 0.344, p < 0.001). Patient weight revealed a significant negative correlation to vessel enhancement (r = -0.496, p < 0.001), SNR (r = -0.446, p < 0.001), CNR (r = -0.425, p < 0.001), and subjective image quality (r = -0.422, p < 0.001). In univariate analysis, SNR and CNR were significantly higher in patients who received contrast medium through peripheral catheters (30 +/- 13 and 27 +/- 13, respectively) than in those in whom central catheters were used (22 +/- 8 and 19 +/- 7, p = 0.041 and p = 0.029, respectively). Neither patient sex nor catheter size, position, or side had any significant impact on image quality. CONCLUSION: Patient age and weight showed significant impact on vascular attenuation and image quality in pulmonary CTA with bolus tracking and ATCM, whereas patient sex and different peripheral catheters did not significantly influence image parameters.

Clinical and morphological phenotype of the filamin myopathy: a study of 31 German patients.

Mutations in the filamin C gene (FLNC) cause a myofibrillar myopathy (MFM), morphologically characterized by focal myofibrillar destruction and abnormal accumulation of several proteins within skeletal muscle fibres. We studied 31 patients from four German families to evaluate the phenotype of filaminopathy. All patients harboured the same p.W2710X mutation in FLNC. Haplotype analysis suggested a founder mutation in these German filaminopathy families. The mean age at onset of clinical symptoms was 44 +/- 6 years (range, 24-57 years). Slowly progressive muscle weakness was mostly pronounced proximally, initially affecting the lower extremities and involving the upper extremities in the course of disease progression, similar to the distribution of weakness seen in limb-girdle muscular dystrophies (LGMD). Patients frequently developed respiratory muscle weakness. About one-third of the patients showed cardiac abnormalities comprising conduction blocks, tachycardia, diastolic dysfunction and left ventricular hypertrophy indicating a cardiac involvement in filaminopathy. Serum creatine kinase levels varied from normal up to 10-fold of the upper limit. Magnetic resonance imaging studies showed a rather homogenous pattern of muscle involvement in the lower extremities differing from that in other types of MFM. Myopathological features included perturbation of myofibrillar alignment, accumulation of granulofilamentous material similar to that seen in primary desminopathies and abnormal intracellular protein deposits typical of MFM. Decreased activities of oxidative enzymes and fibre hypertrophy seem to be early features, whereas dystrophic changes were present in advanced stages of filaminopathy. Rimmed vacuoles were detected in only a few cases. The intracellular aggregates were composed of a variety of proteins including filamin C, desmin, myotilin, Xin, dystrophin and sarcoglycans. Therapy is so far limited to symptomatic treatment. The German filaminopathy cohort, the largest group of patients studied so far, shares phenotypic features with LGMD and presents with characteristic histopathological findings of MFM.

Unilateral hyperplasia of a cervical spinous process as a rare congenital variant of the spine.

Congenital variants of the cervical spine may mimic traumatic lesions and may cause recurrent episodes of pain. We report a 24-year-old female patient with chronic neck pain who had marked unilateral hyperplasia of the spinous process of the seventh cervical vertebra. CT and MRI clearly depicted the abnormality and, furthermore, ruled out posttraumatic spinal changes. To our knowledge, our case is the first patient reported with this rare congenital variant diagnosed by cross-sectional imaging.

Image quality and radiation exposure at pulmonary CT angiography with 100- or 120-kVp protocol: prospective randomized study.

PURPOSE: To prospectively compare 16-section multidetector computed tomography (CT) at 100 and 120 kVp for image quality and radiation dose. MATERIALS AND METHODS: The study had institutional review board approval; written informed consent was obtained. Sixty patients were referred for evaluation of suspected pulmonary embolism with CT angiography. Patients were randomly assigned to a 100-kVp (n = 30; 17 men, 13 women; mean age, 66 years +/- 17 [standard deviation]; range, 19-89 years) or 120-kVp (n = 30; 15 men, 15 women; mean age, 62 years +/- 15; range, 28-86 years) protocol. Other scanning parameters were kept constant. Contrast medium was injected automatically with bolus tracking. Pulmonary vessel enhancement and image noise were quantified; signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were calculated. Subjective vessel contrast was assessed by two radiologists in consensus. Effective dose was calculated on the basis of dose length product and volume CT dose index. Results of both protocols were compared by using the chi(2) test and Student t test. RESULTS: The 100-kVp protocol had a nonsignificantly higher mean vessel attenuation than the 120-kVp protocol (386.8 HU +/- 130.1 vs 317.9 HU +/- 112.5; P = .56) and a nonsignificantly higher image noise (16.9 HU +/- 5.8 vs 13.7 HU +/- 6.2; P = .84), which resulted in almost identical SNR (25.3 +/- 11.7 vs 27.0 +/- 14.5; P = .37) and CNR (22.0 +/- 11.2 vs 22.9 +/- 13.1; P = .51). There was no significant difference in subjective image quality between protocols. Mean effective dose for the 100-kVp protocol was significantly lower than that for the 120-kVp protocol (1.37 mSv +/- 0.39 vs 2.44 mSv +/- 0.97; -44%; P < .001). CONCLUSION: Reduction of kilovoltage from 120 to 100 kVp resulted in significant reduction of effective dose at pulmonary CT angiography, without significant loss of objective or subjective image quality.