The prevalence of spondyloarthropathy in fibromyalgia patients.

OBJECTIVE: To document the prevalence of Spondyloarthropathy (SpA) with an evaluation of patients previously diagnosed with fibromyalgia syndrome (FMS). METHODS: The patients diagnosed with FMS before telephoned and asked three questions to determine for inflammatory back pain. American College of Rheumatology (ACR) Fibromyalgia criteria 1990 and ACR 2010 and for diagnosing patients with SpA; criteria from the European Spondyloarthropathy Study Group (ESSG), and Amor were applied. RESULTS: FMS was diagnosed according to 1990 ACR criteria in 14 (60.8%) SpA patients who were diagnosed with SpA according to the Amor criteria alone and in 10 (43.4%) patients who were diagnosed according to ESSG criteria alone, while it was diagnosed in 9 (33.3%) patients who were diagnosed with SpA according to Amor and ESSG criteria together and in 15 (65.2%) patients diagnosed with SpA according to Amor and/or ESSG criteria. The most tenderness was experienced in the bilateral shoulder supraspinatus tendon insertion region (57.7%) and in the sacroiliac joint (40.8%). Fourteen (60.8%) patients diagnosed according to ACR 1990 and 17 (51.6%) patients diagnosed according to ACR 2010 had plantar fasciitis and/or Achilles enthesopathy on foot radiography. CONCLUSIONS: There is a meaningful section of patients who are SpA or FMS and SpA are together in the patients thought to be FMS or the patients diagnosed with FMS according to ACR's criteria can be said.

Imaging findings and treatment in coccydynia - update of the recent study findings.

BACKGROUND: Coccydynia is one of the most overlooked symptoms in clinical practice. The diagnosis and radiologic findings of traumatic coccyx can be more easily detected unless it is delayed and postponed. For idiopathic coccydynia, which accounts for one third of cases, patients present with long-standing pain and multiple physician visits. METHOD: The keywords coccyx, coccydynia, coccygodynia were searched in PubMed, Embase, Scopus databases in the last 5 years. Research articles, reviews and case reports were analyzed. The studies conducted in the last 5 years were presented under the headings of etiology, radiologic assessment, interventional and surgical treatments. RESULTS AND CONCLUSION: The first step is dynamic X-ray of the coccyx in standing and sitting position. In this way, morphologic parameters and hypermobility causing idiopathic coccydynia can be evaluated. Morphologic and morphometric features of the coccyx described in previous CT and MR studies have explained the relationship with coccydynia. The key features are as follows: Type II coccyx morphology, subluxation of the intercoccygeal joint, presence of bony spicules. Knowledge of these definitions as well as the differential diagnosis in this anatomical region will help in reaching the correct diagnosis. The treatment of coccydynia is stepwise like the diagnosis. Conservative treatments are initiated first. Manipulations, ganglion impar block, injections, radiofrequency and shock treatments and finally coccygectomy are treatment methods reported. Radiologists should not overlook this region and should be familiar with the coccyx's morphologic appearance and the sacrococcygeal region's differential diagnosis. KEY POINTS: · The etiology of coccydynia usually includes trauma, obesity and female sex, special coccyx morphology, and coccygeal hypermobility.. · Coccyx fractures are defined into three groups as flexion type 1, compression type 2, and extension type 3.. · When evaluating coccydynia, the first step is a dynamic X-ray examination of the coccyx in standing and sitting position.. · Hypermobility is defined as more than 25 % posterior subluxation while sitting or more than 25° flexion while sitting.. · More than 35° posterior subluxation is considered significant hypermobility..

Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia.

A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemianopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation. Laboratory evaluations revealed dual antineutrophil cytoplasmic antibodies (ANCA) positivity [anti-proteinase (anti-PR3) ANCA and anti-myeloperoxidase (anti-MPO) ANCA], anticardiolipin (aCL) antibodies, and low titers of antinuclear antibodies (ANA). There was no evidence of active systemic lupus erythematosus (SLE), ANCA-related vasculitis, or other risk factors for cerebral occlusion, such as antiphospholipid syndrome (APS). Dual positivity for both cytoplasmic (c-ANCA) and perinuclear (p-ANCA) antineutrophil antibodies has been found previously in a small number of reports, but to our knowledge, this case represents the first case of moyamoya disease associated with dual ANCA positivity.

Effect of Liver Transplant on Neurologic Manifestations and Brain Magnetic Resonance Imaging Findings in Wilson Disease.

OBJECTIVES: Wilson disease is an autosomal recessive disorder of copper metabolism. It leads to copper accumulation in various organs (liver, eye, brain) and deteriorates their functions. Symptoms usually appear in the second and third decades of life. Neurologic symptoms and manifestations may appear 2 to 5 years after liver involvement, and neurologic symptoms are usually movement disorders. The main treatment objective is to decrease accumulation of copper by increasing urinary copper excretion. With early diagnosis and treatment, the quality of life of patients with Wilson disease evolves. In this study, we aimed to evaluate the effects of liver transplant on neurologic manifestations and radiologic findings in patients with Wilson disease. MATERIALS AND METHODS: Since 1988, our center has performed 642 liver transplant procedures. Fifty-three patients with Wilson disease received a liver transplant during this period, with 15 adults patients included in our study. All study patients were evaluated by the same neurologist and radiologist. Tremor was scored by the glass scale test. Radiologic evaluations were made by cranial magnetic resonance imaging. RESULTS: Before liver transplant, 4/15 study patients had tremor. In 1 patient, tremor was accompanied by dystonia; the patient's imaging findings and neurologic manifestations had regressed posttransplant. In the other 3 study patients with tremor, tremor decreased without any change in imaging findings. New-onset tremor was seen in 1 patient after liver transplant, but this patient had no observed imaging changes. This situation was correlated with immunosuppressive therapy. CONCLUSIONS: Neurologic recovery can be achieved in patients with Wilson disease with early diagnosis and treatment. Radiologic findings can be improved after therapy.

Assessment of Subclinical Atherosclerosis in Vitamin D Deficiency.

PURPOSE: Patients with vitamin D deficiency (VDD) have potential to have increased cardiovascular morbidity and mortality. In this study, we aimed to discover the findings of early atherosclerosis in patients with VDD by measuring carotid intima-media thickness (CIMT) and epicardial fat thickness (EFT). MATERIALS AND METHODS: The study population includes 52 patients with VDD (n = 30 [57% female], mean ± SD age 54.28 ± 8.77 years, mean ± SD serum 25-hydroxyvitamin D (25 [OH] D) 11 ± 2.4 ng/mL) and 82 participants for control group (n = 52 [63.4% female], mean ± SD age 56.40 ± 7.90 years, mean ± SD serum (25 [OH] D) 53 ± 4.2 ng/mL) who have no cardiovascular disease. Carotid intima-media thickness was assessed by using ultrasonography, and EFT was measured on the free wall of the right ventricle at end diastole from the parasternal long-axis views by standard transthoracic 2-dimensional echocardiography. RESULTS: Both CIMT and EFT were significantly higher in patients with VDD compared with controls (0.75 ± 0.16 mm vs 0.68 ± 0.21 mm, P < 0.05, and 0.66 ± 0.15 cm vs 0.56 ± 0.15 cm, P < 0.001). CONCLUSIONS: Patients with VDD seem to have increased CIMT and EFT, which are predictors of atherosclerotic process. Further studies are needed to confirm these results.

Reliability of Real-Time Sonoelastography in the Diagnosis of Supraspinatus Tendinopathy.

ABSTRACT: The practicality of real-time sonoelastography in the diagnosis of tendinopathy is being discussed since the beginning of its use in musculoskeletal system. The aims of this study were to compare the elasticity of pathologic supraspinatus tendon with the uninvolved side by sonoelastography and to determine the relationship between the sonoelastographic findings and magnetic resonance imaging (MRI) grade of the tendinosis. Eighty-2 patients (50 males, 32 females, mean ± SD age = 53.61 ± 16.15 years, range = 20-84 years) with unilateral supraspinatus tendinosis were included in this study. Three grades of tendinosis were identified in MRI (grade 1: mild, grade 2: moderate, and grade 3: severe). The strain ratio (SR) of the tendinosis area to the healthy normal area of the same tendon tissue and SR of the tendinosis area to ipsilateral subcutaneous fat tissue were evaluated with sonoelastography. The SRs of the tendinosis areas were also compared with the control (contralateral) side tendon tissue and subcutaneous fat tissue of the same patients. Between-groups comparisons were also done according to the MRI grading. Statistical analysis was done using paired t test (P < 0.005 was considered statistically significant). There was a statistically significant difference in the comparison of the SRs of the tendinosis areas to subcutaneous fat tissues on ipsilateral shoulders (TA/SFT) and the healthy supraspinatus tendon area (TA/ST) of the same shoulder. There was also statistically significant difference when compared with the control side measurements (P < 0.01). In patients who have grade 1 and grade 3 tendinosis on MRI, there was statistically significant difference between elastrographic evaluation of affected and unaffected sides. Real-time sonoelastography is a reliable diagnostic method in patients with rotator cuff tendinosis and shall be kept in mind as a noninvasive, inexpensive, and practical diagnostic test in suitable cases.

The effects of interferon beta-1a on proton MR spectroscopic imaging in patients with multiple sclerosis, a controlled study, preliminary results.

To evaluate the effects of interferon beta-1a(INFbeta-1a) on brain metabolites in patients with multiple sclerosis (MS), we performed Magnetic Resonance Spectroscopy Imaging (MRSI) on five patients treated with INFbeta-1a (Rebif 44 microg), and on five untreated patients. Six healthy volunteers were used as controls. Patients were evaluated at the beginning, in the first, third, sixth, and twelfth month. There were no significant differences in normal appearing white matter (NAWM) metabolite peaks of the control group and patients with MS. However, in white matter lesions (WML) and NAWM there was significant differences between the basal and the other months' metabolic peaks (p < 0.05) in the treatment group although no differences emerged in the untreated group. These data suggest that INFbeta-1a has a favorable effect on restoration of metabolites in MS lesions.

Two cases of glycine encephalopathy accompanied by pes equinovarus.

Glycine encephalopathy is a rare autosomal recessive metabolic disease characterized by glycine accumulation in body fluids owing to a defect in the glycine cleavage system. There are several forms of glycine encephalopathy. In the classic or neonatal form, symptoms usually develop as neurologic symptoms in the first few days of life. It characteristically presents with hypotonia, lethargy, apnea, and seizures and usually results in death by 1 year of age. In this report, we present two cases of neonatal glycine encephalopathy accompanied by isolated pes equinovarus deformity.

Intraosseous angiolipoma of the frontal bone with a unique location: a clinical and pathological case illustration and review of the literature.

Here, we report a case of a 16-year-old female patient was referred with scalp swelling and headache. Her neurological examination was normal and imaging of the skull revealed a well-defined lytic lesion measuring 15 mm × 6 mm to the right of the frontal bone. She was operated on with a prediagnosis of Langerhans cell histiocytosis. A wide excision with negative margins was made and the defect was reconstructed with a titanium plate. Subsequently, the lesion was histopathologically diagnosed as an angiolipoma of the frontal bone. The postoperative period was uneventful and she remained well during 1-year follow-up with no evidence of recurrence. Angiolipomas are rare benign lipomatous lesions located mostly in subcutaneous tissue of the forearm or trunk and frequently occur before puberty or in young adults. They are not common in bones. To the best of our knowledge, this is the first angiolipoma of the frontal bone reported.

Diagnosis of early sacroiliitis in seronegative spondyloarthropathies by DWI and correlation of clinical and laboratory findings with ADC values.

PURPOSE: Sacroiliitis is one of the diagnostic criteria of seronegative SpA. The purpose of our study is to show the signal characteristics of the sacral and iliac surfaces by DWI which may contribute in early diagnosis of sacroiliitis and investigate the correlation between ADC values and clinical and laboratory parameters. MATERIALS AND METHODS: 62 patients with inflammatory low back pain, with a history or suspect of seronegative SpA are enrolled into the study. 40 age and sex-matched subjects without SpA constituted the control group. After obtaining routine T1 and T2 weighted sequences, echo planar imaging at b values of 0, 400 and 800 was performed. ADC values on both surfaces of the both sacroiliac joints were measured in all subjects. The CRP and sedimentation results and the presence of arthritis and enthesitis were also correlated with the ADC values. RESULTS: ADC values on both surfaces of the both sacroiliac joints were found 0.23 × 10(-3)mm(2)/sn in the control group. In the patient group, mean ADC value of 0.48 × 10(-3)mm(2)/sn was obtained (p<0.001), which was statistically significant, compatible with the increased diffusion due to medullary edema in early sacroiliitis. There was a slight correlation between CRP and ADC values; presumed to be showing the relation between the activity of the disease and the active inflammation on DWI. There was no correlation between arthritis and enthesitis and the ADC values (p>0.001). CONCLUSION: DWI, by measuring ADC values, adds significant information in the early diagnosis of sacroiliitis and may help to evaluate the efficiency of the treatment.

Embolic infarct presented with bilateral thalamic lesions possibly initiated by an emboli to the artery of Percheron.

Bilateral thalamic infarcts are rare and present with varying symptoms. Cardioembolism and small artery disease are the most common etiologic factors. The occlusion of a rare arterial variant called the artery of Percheron results in bilateral thalamic infarcts. Herein, we present the imaging findings of an embolic infarct starting from bilateral thalami, probably due to an emboli to the artery of Percheron first.

A collodion baby with facial dysmorphism, limb anomalies, pachygyria and genital hypoplasia: a mild form of Neu-laxova syndrome or a new entity?

Neu-Laxova syndrome is a rare, lethal, autosomal recessive disorder characterized by intrauterine growth retardation, central nervous system anomalies, skin findings, such as ichthyosis, edema, collodion baby and harlequin fetus, facial dysmorphic features, limb anomalies and genital hypoplasia. Although it is generally a lethal condition, cases of such patients who lived beyond 6 months and 10 months of age have been reported. Here, we describe an 8-year-old boy who was born with collodion membrane, facial dysmorphic features, limb anomalies, genital hypoplasia and pachygyria. He had no major health problems over the course of 8 years of follow-up, except for mild mental/motor retardation, ichthyosis, facial dysmorphic features and limb anomalies. Based on these features, we suggest that because Neu-Laxova syndrome represents a heterogeneous phenotype, our case may be a milder variant of this syndrome or a new genetic entity.

Tissue Doppler study of the right ventricle with a multisegmental approach: comparison with cardiac magnetic resonance imaging.

OBJECTIVE: We investigated whether systolic right ventricular (RV) function and myocardial performance index (MPI) studied with a multisegmental approach improve the accuracy of RV function estimation based on tricuspid lateral annulus. METHODS: Systolic and isovolumic contraction velocities, isovolumic acceleration, regional MPI from the tricuspid lateral and septal annulus, and basal and apical RV free wall and blood pool MPI were obtained in 69 patients and compared with RV ejection fraction (EF) by cardiac magnetic resonance. RESULTS: Average systolic velocity from 2 annular sites had the highest correlation to the RVEF (r = 0.74; P < .001) and highest accuracy to estimate RVEF > 45% (cutoff = 7.0 cm/s; area under the curve 0.908; 95% confidence interval, 0.84-0.98; sensitivity 83%; specificity 86%; P < .0001). Average annular systolic velocity correlated with the RVEF more strongly than the lateral annular systolic velocity in patients with and without dilated RVs and in patients with and without pulmonary arterial hypertension. Four-region average MPI correlated with the RVEF (r = 0.70; P < .001) more strongly than regional MPI and blood pool MPI, with a higher accuracy to estimate RVEF > 45% (cutoff = 0.66; area under the curve 0.849; 95% CI, 0.76-0.94; sensitivity 86%; specificity 75%; P < .0001). Lateral annular measurements were mostly determined by the RVEF, whereas septal annular measurements were almost equally influenced by RVEF and left ventricular ejection fraction (LVEF). Consequently, when the RVEF and LVEF were discordant, only the lateral annular systolic velocity and MPI determined RVEF. CONCLUSION: Average systolic velocity from 2 tricuspid annular sites provides the most accurate estimate of RVEF if the RVEF and LVEF are not discordant.

Brachydactyly short-stature hypertension syndrome: a case with associated vascular malformations.

Brachydactyly short-stature hypertension syndrome, also known as hypertension-with-brachydactyly (HTNB) syndrome, is a rare autosomal dominant disorder that was first described by Bilginturan and colleagues in 1973. Many familial cases of HTNB have been reported, but the first sporadic case of this condition was published only recently. This article describes a case of HTNB syndrome in a 16-year-old boy. Although Doppler ultrasonography of the kidneys and renal arteries showed normal findings, magnetic resonance angiography showed an aberrant right posterior inferior cerebellar artery, early bifurcation of the left renal artery, and irregularity and stenosis of the inferior dominant branch of this artery. The patient's father was in chronic renal failure of which the primary pathology was unknown. We speculate that the described case is the second documented sporadic case of HTNB syndrome. This disorder should be included in the differential diagnosis of patients with short stature and hypertension of unknown aetiology. Such individuals should be carefully examined for brachydactyly and for cerebral-cerebellar and renal vascular malformations.

Value of whole-body turbo short tau inversion recovery magnetic resonance imaging with panoramic table for detecting bone metastases: comparison with 99MTc-methylene diphosphonate scintigraphy.

OBJECTIVE: The objectives of this study were to assess the efficacy and reliability of whole-body turbo short tau inversion recovery (STIR) magnetic resonance imaging (MRI) for detecting skeletal metastasis and to compare the results with those of bone scintigraphy. METHODS: Twenty-six patients with primary cancer (mean age=56 years, age range: 34-75 years) were assessed for bone metastasis with whole-body MRI and bone scintigraphy. Eight bone regions in each patient were assessed (total of 208 sites) with each of these 2 techniques. A turbo STIR sequence and panoramic table were used during MRI. Whole-body MRI and scintigraphy findings were compared with biopsy or follow-up imaging results. RESULTS: After at least 12 months of follow-up, 9 patients had bone metastases in a total of 31 sites. Whole-body MRI showed 29 metastases (94%) in the total 208 skeletal sites investigated in the 26 patients. Bone scintigraphy revealed metastases in 16 (52%) of the 208 sites. CONCLUSION: Whole-body turbo STIR MRI is a reliable method for screening patients with suspected skeletal metastases. This technique is also advantageous in that it reveals extraskeletal organ and soft tissue metastases.

Familial arachnoid cysts in association with autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder well-known for its association with intracranial aneurysms. Recently, intracranial arachnoid cysts have also been reported to be associated with ADPKD. We describe a father and daughter who each has a posterior fossa arachnoid cyst and asymptomatic ADPKD. To our knowledge, this is the first report of familial occurrence of arachnoid cysts in association with ADPKD.