Understanding the diagnosis of superior mesenteric artery syndrome: analysis of the location of duodenal impression on upper gastrointestinal studies.

BACKGROUND: Upper gastrointestinal (GI) contrast studies are frequently requested to aid superior mesenteric artery syndrome diagnosis, a rare entity. Compression of the third duodenal part is expected to be mid-to-left of the midline where the superior mesenteric artery arises from the aorta; however, a duodenal impression to the right of the midline due to normal anatomic impression by the inferior vena cava (IVC) is often encountered and frequently misdiagnosed. OBJECTIVE: The purpose of this study was to determine the frequencies of (1) normal right-of-midline duodenal impressions and (2) mid-to-left of midline compressions in upper GI studies in a tertiary pediatric referral center. MATERIALS AND METHODS: All upper GI studies performed at our institution over 2 years were retrospectively evaluated to determine whether the duodenum had vertical duodenal impression to the right of the vertebral midline, mid-to-left of the vertebral midline, or no identifiable duodenal impression at all. RESULTS: In total, 538 upper GI studies were included in this analysis. A total of 275 male and 247 female patients between 0 and 17 years of age (median: 6 years, range: 1 month-17 years) were included. Of 538 total upper GI studies, there were 240 studies (44.6%) with a right-of-midline impression. There were only 10 studies (1.9%) with a mid-to-left of midline compression, and 9/10 also showed a concurrent right-sided impression sign. CONCLUSION: Right-of-midline duodenal impression is a normal anatomic finding caused by the IVC and should not be confused with superior mesenteric artery syndrome. In the presence of an appropriate clinical context, proximal duodenal dilation, "to-and-fro" motion of contrast, and duodenal impression at mid-to-left of midline, a diagnosis of superior mesenteric artery syndrome should be considered.

Desmoplastic Small Round Cell Tumor With Ascending Intraspinal Metastasis at Recurrence: Case Report and Review of the Literature.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy commonly involving the abdomen and/or pelvic peritoneum. Despite aggressive therapy, the prognosis remains poor. Central nervous system relapse is rare in abdominal/pelvic primary DSRCT. OBSERVATION: We report a case of a 10-year-old female with a large pelvic DSRCT and involvement of the rectosigmoid colon and liver. Following treatment with chemotherapy, and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy an initial response was noted. With progressive lower limb weakness, recurrence with perineural invasion in the lumbosacral nerve root involving the conus was noted 2.5 years from diagnosis. Cerebrospinal fluid showed tumor cells with a molecular confirmation. CONCLUSIONS: Perineural invasion and ascending paralysis secondary to primary abdominal DSRCT has not been previously reported to our knowledge. We recommend a high index of suspicion for early and accurate diagnosis of this rare presentation.

Biliary Rhabdomyosarcoma in an Infant Male With Neurofibromatosis Type 1.

Different types of malignancies can be seen in patients with neurofibromatosis type 1 (NF-1). Herein we present a rare combination of NF-1 and biliary rhabdomyosarcoma in a male infant. An 11-month-old boy, who was recently diagnosed with NF-1, presented to the outpatient clinic with a 3-month history of prolonged jaundice, and failure to thrive. Clinical examination showed >20 café au let spots distributed mainly over the abdominal trunk. Hepatomegaly (4 cm below the costal margin) was additionally observed. His father was diagnosed with NF-1. Radiologic imaging studies showed a 6×5×5 cm in diameter cystic mass with multiple septations in the segment 4A of the liver. Surgical excision of the left hepatic lobe followed by hepatojejunostomy was further performed. Histopathology examination showed embryonal type rhabdomyosarcoma originating from the biliary duct. Chemotherapy regimen consisting of cyclophosphamide, actinomycin D, and vincristine, and radiotherapy were then initiated. This treatment led to a significant improvement in the patient's clinical status, and radiologic finding portrayed attainment of complete resolution. He is still in complete remission without any sequelae for 8 years.

Evaluation of Ovarian Vascularity in Children by Using the "Superb Microvascular Imaging" Ultrasound Technique in Comparison With Conventional Doppler Ultrasound Techniques.

OBJECTIVES: The aim of this study was to assess the feasibility of the novel "superb microvascular imaging" (SMI) Doppler ultrasound technique to detect ovarian vascularity in healthy children and to compare it with the conventional Doppler ultrasound techniques. METHODS: This prospective study included 140 girls, aged 3 to 18 years, for which an abdominal ultrasound study was requested in the pediatrics outpatient clinics for suspicion of nonovarian pathologies. Nineteen individuals were excluded from the study due to incomplete ultrasound scanning. For the remaining 121 cases, gray scale ultrasound evaluation was performed followed by color Doppler imaging, power Doppler imaging, advanced dynamic flow, color SMI, and monochrome SMI to detect the presence and degree of vascularity in the ovaries. Ultrasound scanning was performed for all participants by the same doctor by using curvilinear probe via the suprapubic approach, and the images were independently evaluated by 3 different observers. The images were assessed with regard to the degree of vascularity and the presence of artifacts. RESULTS: A total of 146 ovaries (42 right, 54 left, and 25 bilateral) were examined by sonography. The mean interrater agreement regarding the detection of vascularity was moderate for the color Doppler imaging, power Doppler imaging, advanced dynamic flow, and monochrome SMI techniques (κ = 0.514-0.551) and substantial for the color SMI technique (κ = 0.636) (P < .001). The techniques were found to be statistically significant for detecting vascularity: monochrome SMI > color SMI > power Doppler imaging > color Doppler imaging > advanced dynamic flow (P < .001). CONCLUSION: SMI is a promising tool that can detect ovarian vascularity more effectively than the conventional Doppler ultrasound techniques.

Seeing the Unseen: Evaluating Testicular Vascularity in Neonates by Using the Superb Microvascular Imaging Ultrasound Technique.

OBJECTIVES: To evaluate testicular vascularity in neonates by using the novel Superb Microvascular Imaging (SMI; Toshiba Medical Systems Corporation, Tokyo, Japan) ultrasound (US) technique and to compare this technique to conventional Doppler US techniques. METHODS: A total of 140 healthy neonates between 2 and 60 days old who were admitted for routine screening were included in the study. However, in 9 cases, the Doppler study could not be completed. In the remaining 131 cases, color Doppler imaging (CDI), power Doppler imaging (PDI), Advanced Dynamic Flow (ADF; Toshiba Medical Systems), and monochrome SMI (mSMI) techniques were performed on a single representative testicle. Real-time cine images were captured for 5 to 10 seconds for each technique by using a high-frequency (7.2-14-MHz) linear transducer. The images were evaluated by 3 observers using a grading system to quantify the degree of vascularity and artifact interference. RESULTS: A total of 131 testicles (74 right and 57 left) were evaluated, and vascularity was shown in 110 (84%) testicles with CDI, 109 (83%) with PDI, 94 (72%) with ADF, and 128 (98%) with mSMI by the consensus of all observers. The Doppler techniques were ranked from highest to lowest in the following order based on the degree of vascularity detected: mSMI > PDI > CDI > ADF (P < .001). Based on the presence of artifacts, the Doppler techniques were ranked as follows from high to low: ADF > CDI > PDI > mSMI (P < .001). CONCLUSIONS: Superb Microvascular Imaging is a promising US technique that appears to detect testis vascularity better than conventional Doppler US techniques; however, further research is needed to support the results of this study.

Is real-time elastography helpful to differentiate acute from subacute deep venous thrombosis? A preliminary study.

PURPOSE: To detect and characterize changes in stiffness of thrombus in patients with acute and subacute deep venous thrombosis (DVT) by using real-time elastography (RTE). METHODS: Fifty-eight patients with acute or subacute DVT were prospectively evaluated by B-mode sonography (US), color Doppler US (CDUS), and RTE. Two radiologists evaluated the thrombus echogenicity, compressibility, and recanalization of the affected vein, and thrombus stiffness in consensus. The thrombi were classified into 3 groups as soft, intermediate, and hard on RTE images. RESULTS: The final study group consisted of 30 patients with acute DVT, among whom 10 were women (33%), and 19 patients with subacute DVT, among whom 6 were women (32%). The presence of hypoechoic thrombus, incompressible vein, and absence of recanalization on US and CDUS were significantly associated with acute DVT (P < .001 for all variables). The differences in elasticity pattern of the thrombi between acute and subacute DVT were not significant (P = .202). CONCLUSION: Venous thrombus hardens with age; however, elastography pattern on RTE, in its present form, may not be able to differentiate acute DVT from subacute DVT.

The utility of ADC measurement techniques for differentiation of low- and high-grade clear cell RCC.

PURPOSE: To evaluate the diffusion properties of clear cell renal cell carcinoma (ccRCC) on magnetic resonance imaging (MRI) concerning their Fuhrman nuclear grades and sizes, and to compare the diagnostic performance of two ROI placement techniques for apparent diffusion coefficient (ADC) measurement (entire mass vs. only the darkest region of the mass). MATERIAL AND METHODS: Fifty-one ccRCC were enrolled in the study and grouped into low-grade ccRCC (Fuhrman grade 1 and 2, n = 37) and high-grade ccRCC (Fuhrman grade 3 and 4, n = 14). Selective ADC (Sel-ADC) measurement was performed by placing a circular ROI that included the darkest region of the tumour on ADC map images. Extensive ADC (Ext-ADC) measurement was performed by drawing an ROI that covered the entire tumour. RESULTS: The Sel-ADC value was lower in high-grade ccRCC (p = 0.019), whereas the Ext-ADC value did not show a statistically significant difference (p = 0.42). Sel-ADC value of a ≤ 1.405 mm2/s has a sensitivity, specificity, positive predictive value, negative predictive value, and accuracy value of 78.6, 72.2, 73.87, 77.13, and 75.4, respectively, to differentiate high-grade from low-grade ccRCC. The size and Fuhrman grade of the ccRCC were inversely correlated with the Sel-ADC value; however, the correlations were weak (r = -0.322, p = 0.021 and r = -0.376, p = 0.006, respectively). There was no difference between ADC values of small (≤ 4 cm) and large (> 4 cm) ccRCCs. CONCLUSIONS: The ADC value of the darkest region in solid part of the ccRCC may play a role in predicting the nuclear grade of ccRCC.

Neurological Manifestations in Familial Mediterranean Fever: Results of 22 Children from a Reference Center in Kayseri, an Urban Area in Central Anatolia, Turkey.

Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis. Objective The purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings. Materials and Methods Our medical records database for 2003 to 2014 was screened retrospectively. In total, 104 patients with FMF were identified, 22 of whom had undergone neurological examination for CNS symptoms. Results Neurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), tremor in 2 patients (9.1%), and multiple sclerosis in 1 patient (4.5%). The most common MEFV gene mutation was homozygous M694V (40.9%). Conclusions Patients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF.

Imaging of childhood angiomatoid fibrous histiocytoma with pathological correlation.

BACKGROUND: Angiomatoid fibrous histiocytoma is a rare soft-tissue tumor that more often affects children and young adults. There is little information available regarding the imaging appearance of angiomatoid fibrous histiocytoma in children. OBJECTIVE: To describe the ultrasonographic (US) and magnetic resonance (MR) imaging findings of angiomatoid fibrous histiocytoma in children. MATERIALS AND METHODS: A retrospective analysis was done of US and MR imaging findings in children with angiomatoid fibrous histiocytoma. Clinical findings and histopathology with molecular analysis results were also collected. RESULTS: There were 7 children with angiomatoid fibrous histiocytoma with a median age of 6 years (age range: 16 months-14 years). Patients presented clinically with a soft-tissue mass in the extremities or in the trunk. Four children had anemia, and three of them had additional systemic symptoms. Two patients had US and three had MR imaging while the remaining two had both. Lesion size ranged from 1.3 cm to 7.2 cm. In four patients, angiomatoid fibrous histiocytoma presented as a nonspecific predominantly solid mass. The other three patients had a combination of the following imaging findings: intralesional blood-filled cystic spaces with fluid-fluid levels, enhancing fibrous pseudocapsule and hemosiderin deposition. These findings correlated well with histopathology. CONCLUSION: The imaging detection of intralesional blood-filled cystic spaces with fluid-fluid levels, enhancing fibrous pseudocapsule and hemosiderin deposition in a soft-tissue tumor in a child may suggest the diagnosis of angiomatoid fibrous histiocytoma. A history of systemic symptoms and anemia in the presence of a soft-tissue mass may also be a clue for the diagnosis of angiomatoid fibrous histiocytoma.

Determination of lingual vascular canals in the interforaminal region before implant surgery to prevent life-threatening bleeding complications.

OBJECTIVES: Profuse hemorrhage and airway obstruction may occur during or after the implant surgery in the interforaminal region. The prevention from this complication requires identifying the location of the mandibular lingual vascular canals (MLVCs). The purpose of this study was to evaluate the anatomical variations of MLVCs and to determine the safety margins for implant placement in interforaminal region. MATERIALS AND METHODS: Computer tomography (CT) images of 200 consecutive patients were reexamined retrospectively by a radiologist and a maxillofacial surgeon to evaluate the presence of the MLVCs entering the mandible. The diameter and the number of the canals, the distance between the entrance of the canal and mandibular midline, and the height of the entrances of the canals from the inferior mandibular margin were measured. RESULTS: Two hundred and thirty-six median lingual canals (MLCs) and 159 lateral lingual canals (LLCs) were found in 200 patients. Significant differences were found between the number of lingual canals in the midline and canine regions (P < 0.001). CONCLUSION: There is a potential risk of complications due to the injuries of the vessels entering the lingual cortical bone through a number of bone canals during implant placement in the interforaminal region.

Rapamycin has a beneficial effect on controlling epilepsy in children with tuberous sclerosis complex: results of 7 children from a cohort of 86.

PURPOSE: Tuberous sclerosis complex (TSC) is a genetic disorder characterized by the formation of hamartomas in various organ systems. We would like share our experience from 86 patients and the results of rapamycin treatment in seven children with TSC. METHODS: Eighty-six children with TSC were enrolled into this retrospective study. The clinical features of seven children treated with oral rapamycin were presented in detail. RESULTS: The most common complaint of administration was convulsion in 77 children (89.5%). Hypopigmented skin lesions, adenoma sebaceum, resistant epilepsy, intracardiac mass, renal angiomyolipomas, and West syndrome were detected (n = 83, 96.5%; n = 47, 54.7%; n = 36, 41.9%; n = 27, 31.4%; n = 18, 20.9%; and n = 13, 15.1%, respectively). Subependymal nodules were the most frequent finding in cranial imaging followed by cortical tubers and subependymal giant cell astrocytomas (n = 75, 87.2%; n = 71, 82.6%; and n = 8, 9.3%, respectively). Of the seven patients treated with rapamycin, the lesions of six children with facial adenoma sebaceum showed regression in various degrees. The frequency of convulsions decreased in five patients with resistant epilepsy within the first 6 months of the treatment, and complete control of convulsion for all patients was achieved in the second 6 months. CONCLUSION: This is the first study that showed that rapamycin is an effective agent for controlling epilepsy without any significant side effect in children with TSC. Rapamycin seems to be effective after 6 months of therapy, and we recommend tapering the dosage after successful management of epilepsy.

Prenatal diagnosis and postnatal outcome of fetal intracranial hemorrhage.

BACKGROUND: The aim of this study was to present our experience with six cases of fetal intracranial hemorrhage (ICH) in terms of prenatal diagnostic features, and postnatal outcome. METHODS: The database of prenatal diagnosis unit was searched for antenatally diagnosed ICH cases. Maternal characteristics, ultrasound (US), and magnetic resonance imaging (MRI) findings, clinical course, and postnatal outcome were noted. RESULTS: We evaluated six consecutive cases of fetal ICH. One case was terminated at 24 weeks, and remaining five cases were delivered between 34 and 38 weeks. Five cases (5/6) had intraventricular, and one (1/6) had intraparenchymal hemorrhage. Hemorrhages were right sided in five cases (5/6), left sided in one case (1/6). Dilated and echogenic ventricular wall were the common US findings. No predisposing factor was detected in four of the cases, and intrauterine growth restriction was an underlying factor in two fetuses. Intrauterine progression of the hydrocephaly, and parenchymal thinning was seen in four cases (4/6). In three of four cases (3/4) with progressive grade 3-4 hemorrhage and hydrocephaly, postnatal outcome were dismal, and one case had mild neurological impairment at three months. In one case which had non-progressive mild ventriculomegaly, the lesion regressed after 4 weeks, and had normal short-term outcome CONCLUSION: Fetal ICH can be accurately identified and categorized by antenatal sonography, and fetal MRI. Although intrauterine regression or normal short-term postnatal outcome is possible, the outcome is usually poor for fetuses with high grade and/or progressive lesions. Therefore, further studies assessing long-term postnatal outcome are needed.

Different etiologies of acquired torticollis in childhood.

INTRODUCTION: Torticollis can be congenital or may be acquired in childhood. Acquired torticollis occurs because of another problem and usually presents in previously normal children. The causes of acquired torticollis include ligamentous, muscular, osseous, ocular, psychiatric, and neurologic disorders. OBJECTIVE: We performed this study to evaluate the underlying causes of torticollis in childhood. MATERIAL AND METHODS: Ten children presented with complaints of torticollis between April 2007 and April 2012 were enrolled in this study. The additional findings of physical examination included neck pain, twisted neck, walking disorder, imbalance, and vomiting The identified etiologies of the enrolled children was acute disseminated encephalomyelitis in a 2.5-year-old boy, posterior fossa tumor in a 10-month-old boy, spontaneous spinal epidural hematoma in a 5-year-old hemophiliac boy, cervical osteoblastoma in a 3-year-old boy, arachnoid cyst located at posterior fossa in a 16-month-old boy, aneurysm of the anterior communicating artery in a 6-year-old girl, pontine glioma in a 10-year-old girl, and a psychogenic torticollis in a 7-year-old boy were presented. CONCLUSION: There is a wide differential diagnosis for a patient with torticollis, not just neurological in etiology which should be considered in any patient with acquired torticollis. Moreover, early diagnosis of etiological disease will reduce mortality and morbidity. Therefore, clinicians managing children with torticollis must be vigilant about underlying neurological complications.

Prospective evaluation of the impact of sonography on the management and surgical intervention of neonates with necrotizing enterocolitis.

BACKGROUND/AIM: Established indications for surgery in necrotizing enterocolitis (NEC) are pneumoperitoneum and failure to improve or clinical deterioration with medical treatment alone. It has been proposed that infants with intestinal necrosis may benefit from surgery in the absence of one of these indications yet the diagnosis of definitive intestinal necrosis is challenging. Recent data suggest that abdominal ultrasound (US) examination focused on the gastrointestinal tract and the peritoneal cavity may be of utility in this regard. The aim of this study was to evaluate the ability of abdominal US to detect intestinal necrosis in infants with radiographically confirmed NEC. METHODS: Twenty-six consecutive infants with Bell stage II or III NEC were prospectively included in the study between September 2013 and July 2014. Infants with a pre-existing indication for surgery were excluded. At least one abdominal US examination was performed in each patient using a standardized previously described method. Surgery was performed at the discretion of the attending surgeon based on clinical and imaging findings. Clinical, radiographic, US, and intra-operative data were recorded to allow comparison between US findings, surgical findings and outcome. RESULTS: US demonstrated signs of intestinal necrosis in 5 of the 26 patients. All of these five had laparotomy. Intestinal necrosis requiring resection was confirmed in four and the other was found to have NEC but no necrosis was identified. In 21 patients US did not suggest intestinal necrosis. Of these, only one had surgery in whom NEC but no necrosis was identified. The remaining 20 responded to medical treatment for NEC and were assumed not to have had intestinal necrosis based on improvement without surgical intervention. The sensitivity, specificity, positive predictive value and negative predictive values of US for the detection of bowel necrosis were calculated as 100, 95.4, 80.0, and 100%, respectively. CONCLUSION: Our prospective findings suggest that abdominal US can identify those infants with NEC who may need surgery by detecting bowel necrosis (prior to the development of perforation or medical deterioration) with high sensitivity and specificity. Early surgical intervention in the clinical pathway of NEC may lead to improved outcomes.

Evaluation of pediatric thoracic disorders: comparison of unenhanced fast-imaging-sequence 1.5-T MRI and contrast-enhanced MDCT.

OBJECTIVE: The purpose of this study was to investigate the efficacy of thoracic MRI with fast imaging sequences without contrast administration at 1.5 T for evaluating thoracic abnormalities by comparing MRI findings with contrast-enhanced MDCT findings. SUBJECTS AND METHODS: A prospective study included consecutively registered pediatric patients who from December 2009 to January 2012 underwent thoracic MDCT followed within 2 days by MRI for evaluation of thoracic abnormalities. The final study sample consisted of 71 children (36 boys, 35 girls; mean age, 8.6 ± 4.5 years; range, 2 months-16 years) and 71 paired thoracic MRI and MDCT studies. Thoracic MRI was performed in the axial and coronal planes with the following fast imaging sequences: T1-weighted fast-field echo inversion prepulse, T2-weighted balanced fast-field echo multiple 2D, T1- and T2-weighted turbo spin-echo cardiac-triggering parallel imaging technique without cardiac monitoring, and STIR. Thoracic MDCT was performed with i.v. contrast administration. Two pediatric radiologists independently reviewed each MRI and MDCT study for abnormalities in the lung, large airways, and mediastinal, pleural, and musculoskeletal structures. The sensitivity, specificity, and overall accuracy of MRI were calculated. Interobserver agreement was measured with the kappa coefficient. RESULTS: With MDCT as the reference standard, 51 of 71 (72%) patients had abnormal findings on MDCT studies, including infections in 21 (42%) cases, neoplasms in 19 (37%) cases, interstitial lung disease in seven (14%) cases, pleural effusion in three (6%) cases, and congenital bronchogenic cyst in one (2%) case. The overall diagnostic accuracy, sensitivity, and specificity of MRI for detecting thoracic abnormalities were 69 of 71 (97%), 49 of 51 (96%), and 20 of 20 (100%). Two undiagnosed findings with MRI that were detected with MDCT were mild bronchiectasis and small pulmonary nodule (3 mm). Almost perfect interobserver agreement was found between two reviewers with 70 of 71 agreements (κ = 0.97; 95% CI, 0.92-1.00; p < 0.001). CONCLUSION; MRI with fast imaging sequences without contrast administration is comparable to contrast-enhanced MDCT for detecting thoracic abnormalities in pediatric patients. Use of MRI with fast imaging sequences without contrast administration as a first-line cross-sectional imaging study in lieu of contrast-enhanced MDCT has the potential to benefit this patient population owing to reduced radiation exposure and i.v. contrast administration.

Fatty liver is a good indicator of subclinical atherosclerosis risk in obese children and adolescents regardless of liver enzyme elevation.

AIM: To investigate the presence of association between nonalcoholic fatty liver disease (NAFLD) and subclinical atherosclerosis using carotid intima media thickness (c-IMT) in obese children and adolescents. Additionally, we wished to investigate the relationship between fatty liver and elevated liver enzymes. METHODS: A total of 157 obese patients (78 boys and 79 girls, mean age: 11.3 ± 2.6 years, age range: 6-16 years) were enrolled in the study. Aminotransferase, fasting glucose and lipid levels were determined. An oral glucose tolerance test was performed. The c-IMT was measured. Infectious and metabolic causes of elevated liver enzymes were excluded. The diagnosis of NAFLD was based on ultrasound scan. RESULTS: Obese patients with NAFLD had markedly increased carotid IMT (mean: 0.48 mm, 95% CI: 0.47-0.49) than those without NAFLD (mean: 0.45 mm 95% CI: 0.44-0.45, p < 0.001). The presence of NAFLD significantly increased c-IMT whether the patient had elevated liver enzyme or not (ANOVA, p < 0.001). In a multiple-regression model, only the presence of NAFLD was associated with increased c-IMT (ß = 0.031, SE (ß) = 0.008, p < 0.001). CONCLUSION: Obese children and adolescents with NAFLD are at risk of early atherosclerotic changes. As liver function tests are not sufficient to identify patients with fatty liver, ultrasonographic evaluation of NAFLD might be considered in all obese children and adolescents.

Diffusion-weighted imaging in Sydenham's chorea.

PURPOSE: The purpose of this study was to determine of caudate nucleus changes in diffusion-weighted magnetic resonance imaging. METHODS: A total of 13 children (four males and nine females) with history of Sydenham's chorea and 13 healthy controls were included in to the study. Diffusion cranial magnetic resonance imaging was performed in all subjects before prednisone treatment. Prednisone (2 mg/kg/day, maximum dose 60 mg/day) was used during 4 weeks and then progressively discontinued (20 % of the initial dose was reduced at each 5 days). Two months later, magnetic resonance imaging was repeated. RESULTS: Before and after 8 weeks of prednisone treatment, apparent diffusion coefficients (ADCs) were calculated for right and left caudate nucleus. The ADC values were significantly different before treatment and 2 months after imaging. For the left caudate nucleus, ADC values before treatment (0.69 ± 0.038) were significantly lower than after treatment (0.95 ± 0.04). For the right caudate nucleus, ADC values before treatment (0.72 ± 0.06 × 10(-3)) were significantly lower than after treatment (0.93 ± 0.04 × 10(-3)). CONCLUSIONS: Although cranial and caudate nucleus magnetic resonance imaging findings were normal, the low ADC value findings in our study support the autoimmune inflammation in basal ganglia of Sydenham's chorea.

Iodine deficiency: a probable cause of neural tube defect.

INTRODUCTION: Iodine deficiency is the most devastating event in developing brain in the fetus and neonate. Iodine is absolutely necessary on the myelination, neuronal differentiation, and formation of neural processes, synaptogenesis, and neuronal migration by thyroidal hormones throughout pregnancy and shortly after birth. Neural tube defects (NTD) form after third and fourth gestational weeks and their etiologies are multifactorial. CASE REPORT: We herein present a male newborn with iodine deficiency and thoracic neuroenteric cyst bound to a myelomeningocele via a pedinculi. We hypothesize that iodine deficiency may be a cause of NTD, and iodine supplementation in preconception and pregnancy may prevent NTD.

Transverse myelitis and acute motor sensory axonal neuropathy due to Legionella pneumophila: a case report.

Guillain-Barré syndrome is a rapidly progressive symmetrical muscle weakness associated with acute inflammatory disease. Transverse myelitis (TM) is the inflammation of the spinal cord characterized by rapidly evolving muscle weakness in the lower extremities, defects in sensory level and sphincter dysfunction. Guillain-Barré syndrome, and TM association occurs very rarely in childhood. A 7-year-old girl presented with complaints of neck pain, spout-style vomiting, cough, shortness of breath, and acute paraparesis with sensory and sphincter disturbance. The patient was intubated because of increased respiratory distress. A positive direct fluorescein antigen test in bronchoalveolar lavage confirmed Legionella pneumophila infection. Imaging and neurophysiologic studies were diagnostic for TM with acute motor and sensory axonal neuropathy. She was treated with a combination of high-dose methylprednisolone and intravenous immunoglobulins, and we observed incomplete recovery. The presented case is the first child with concomitant TM and acute motor and sensory axonal neuropathy related to L. pneumophila infection.