European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB): An Update on the Pediatric CEUS Registry on Behalf of the "EFSUMB Pediatric CEUS Registry Working Group".

The European Federation of Ultrasound in Medicine and Biology (EFSUMB) created the "EFSUMB Pediatric Registry" (EFSUMB EPR) with the purpose of collecting data regarding the intravenous application of pediatric contrast-enhanced ultrasound (CEUS). The primary aim was to document the current clinical practice and usefulness of the technique and secondarily to assess CEUS safety in children. We issue the preliminary results of this database and examine the overall practice of CEUS in children in Europe.

Seventeen years of prenatal magnetic resonance imaging at the Institute of Mother and Child in Warsaw.

PURPOSE: The purpose of this paper is to summarise and to present the experience of the main Polish centre for prenatal magnetic resonance imaging (MRI) and to discuss the place and role of MRI in antenatal diagnosis, management, and counselling. MATERIAL AND METHODS: Retrospective analysis of the examinations performed in the years 2001-2017. RESULTS: In total, 1221 medical records and/or image files were collected. The full documentation of ultrasonography and MRI referrals, reports, and images was not available in every case. During the first three years 98 pregnant women were examined (approximately 33 per year, one study per fortnight). After purchase of own MR scanner, the number of examinations grew constantly, reaching 208 in 2017, which means almost one per day, and the highest number was eight in one day. We examined 45 pairs of twins, including three pairs of conjoined twins. CONCLUSIONS: MRI at our Institute is a practically used second-line foetal imaging tool, necessary to confirm, complete, or correct sonographic diagnoses, with important impact on clinical decisions concerning management of pregnancy and of the neonate, termination of pregnancy, choice of the time, place, and mode of delivery, and neonatal care, as well as on counselling. In experienced hands, MRI is to date the last and the best non-invasive method of diagnosis in utero. It is frequently requested by the interdisciplinary team for foetal diagnosis and therapy and routinely performed in everyday practice.

Diagnostic Imaging of Pregnant Women - The Role of Magnetic Resonance Imaging.

BACKGROUND: Presentation of magnetic resonance imaging (MRI) findings in pregnant women in the Department of Diagnostic Imaging, Institute of Mother and Child, Warsaw, Poland. MATERIAL/METHODS: Forty-three symptomatic pregnant women underwent MRI between 9 and 33 weeks of gestation (mean of 23 weeks). Moreover, we included 2 pregnant women who underwent fetal MRI and had incidental abnormalities. RESULTS: In 9 cases, we excluded the suspected brain abnormalities. In 4 cases, we found unremarkable changes in the brain without clinical significance. One patient was diagnosed with multiple sclerosis, one with cortical dysplasia, one with pineal hemorrhage and one with a brain tumor. On abdominal MRI, 2 patients had normal findings, one patient had colon cancer with a hepatic metastasis, one patient had a hepatic angioma, one patient had an extraadrenal pheochromocytoma, one patient had an abscess in the iliopsoas muscle, 9 patients had myomas, two patients had ovarian simple cysts, two endometrial cysts, three dermoid cysts, one patient had sacrococcygeal teratoma, one patient had a cystadenofibroma (partial borderline tumor), one patient had an androgenic ovarian tumor and two patients had hyperreactio luteinalis. One patient was diagnosed with transient osteoporosis of the hip and one with a stress fracture of the sacral bone. CONCLUSIONS: Magnetic resonance imaging is the best imaging modality for pregnant women. Although ultrasonography is the method of choice, doubtful cases as well as structures that cannot be examined with ultrasonography can be non-invasively evaluated with MRI.

Imaging of Complications After Limb Prostheses Implantation in Children with Bone Tumors.

BACKGROUND: Tumors of the developmental age are an important problem in clinical practice. Primary bone tumors constitute 1-1.5% of all tumors, and 7% of all tumors diagnosed in the developmental age. The aim of the study was to assess the capabilities of imaging methods in diagnosing complications of limb endoprostheses in children with osteoarticular tumors. MATERIAL/METHODS: We included 155 patients with limb endoprostheses, aged 7-26 years. There were 113 patients with knee prostheses, 1 patient with bilateral knee prostheses, 16 patients with shoulder prostheses, 14 patients with hip prostheses, 11 patients with hip and knee prostheses, and 1 patient with a femoral prosthesis. All patients underwent plain radiography and an ultrasound study. RESULTS: Complications were found in 50 patients (32% of all patients). The following complications were seen - inflammatory changes in soft tissues and bones (12 patients, 7.7%), stump fractures (11 patients, 7%), lack of elongation effect or shortening of the prosthesis (7 patients, 4.5%), prosthesis fractures (4 patients, 2.5%), crural fractures (4 patients, 2.5%), loosening of the prosthesis (3 patients, 1.9%), joint dislocation (2 patients, 1.3%), stump and prosthesis fracture (1 patient, 0.6%) and local recurrence (6 patients, 3.9%). Prostheses were exchanged for the following reasons - fractures (5 patients, 3.2%), inflammation (2 patients with 3 exchanges, 1.9%). Local recurrence and inflammation were confirmed by a biopsy. CONCLUSIONS: Radiography and ultrasonography are of crucial importance in monitoring patients after implantation of limb endoprostheses. The findings did not require confirmation in additional studies: CT and MRI. Suspicion of local recurrence and inflammation required a biopsy confirmation.

Circle of Willis abnormalities in children with neurofibromatosis type 1.

BACKGROUND AND PURPOSE: The aim of the study was to assess anatomical variants and abnormalities in cerebral arteries on magnetic resonance angiography in 67 children with neurofibromatosis type 1 (NF1). MATERIALS AND METHODS: The study included 67 children aged 9 months to 18 years (mean 6.6 years). Control group comprised 90 children aged 2-18 years (mean: 11.8 years). All patients were examined at 1.5T scanner. RESULTS: We found cerebral arteriopathy (moyamoya disease) in one child (1.5%) in the study group. No aneurysms were found. Twenty-nine NF1 children (43.3%) had arterial anatomical variants. In 13 of them, more than one variant was diagnosed (44.8% of group with variants, 19.4% of study group). In control group, 19 children (21.1%) had variants, including four children with more than one variant (21% of group with variants, 4.4% of control group). Arterial variants were more common in NF1 patients compared with control group (p=0.026, binomial test for two proportions). Percentage of multiple variants was higher in study group than in control group, but this difference was not significant. Variants were more frequent on left side than on the right one (significant difference in control group; p=0.022, McNemara test). In study group, the number of left-sided anomalies (25) was similar to that of right-sided ones (22). There was no correlation between gender and variants, unidentified bright objects and variants or between optic gliomas and variants. CONCLUSIONS: Occurrence of arterial variants in NF1 patients was twofold higher than in control group. Multiple variants were more frequent in the study group although the difference did not reach statistical significance. Features of cerebral arteriopathy were found in one child with NF1.

Cephalic index in the evaluation of surgical treatment of children with sagittal synostosis. Preliminary report.

AIM OF THE STUDY: To analyze changes of the cephalic index (CI) values in patients with sagittal synostosis operated on in the Department of Surgery of Children and Adolescents of the Institute of Mother and Child in Warsaw. MATERIAL AND METHODS: The study group consisted of 14 consecutive cases (11 boys and 3 girls). In 8 patients younger than 3 months of age (1st subgroup) the modified reversed-pi technique (craniotomy in the shape of the reversed Greek π letter) was utilized. In 6 older infants and in patients with severe head deformity - the radical cranial vault reconstructions were performed (2nd subgroup). The statistical analysis of the preoperative (preop) and postoperative (postop) cephalic index (CI) values was done. RESULTS: For the entire study group the preop CI values ranged from 61 to 77 (mean 68.14, median 68.5) and postop CIs from 73 to 89 (mean 77.64, median 76). In the 1st subgroup the preop CIs ranged from 63 to 77 (mean 69.625, median 69) whereas the postop CIs from 69 to 89 (mean 78.125, median 76.5). In the 2nd subgroup the preop CI values ranged from 61 to 72 (mean 66.1667, median 66) and postop from 73 to 81 (mean 77, median 77). All the differences were statistically significant. CONCLUSION: The values of cephalic index increased after both types of procedures. It signifies the correction of dolichocephaly in both subgroups of the patients. However, because of the small number of operated children, further studies are necessary.

First Experience with Neonatal Examinations with the Use of MR-Compatible Incubator.

BACKGROUND: Since 2003, very few publications have described brain examinations using neonatal MR-compatible incubator (INC). The authors present their first experience in these examinations, not limited to brain scans, with the use of an incubator equipped not only with head coil, but also with a coil designed for examinations of the spinal canal and spinal cord as well as the whole body, at the Institute of Mother and Child in Warsaw. MATERIAL/METHODS: Examinations were performed in 27 newborns (12 girls, 15 boys). Most of the neonates were prematurely born: 19 (70.4%) were born at gestational age of 23-37 weeks, mean of 30 weeks. They were examined at the corrected age of 26 weeks-1 month, mean of 36 weeks. Body weight of the newborns on the day of the study was 600-4,300 g, mean of 2,654 g. The study was performed with a GE Signa HDxT 1.5 T system with the use of a Nomag IC 1.5 incubator by Lammers Medical Technology Co., equipped with three coils: an eight-channel, phased-array head coil and a twelve-channel phased-array coil for the whole body, consisting of an eight-channel coil integrated in the incubator and a separate four-channel surface coil. RESULTS: Of the 27 children, 25 (92.6%) required a brain scan. Two children (7.4%) were referred to MRI for assessment of the spinal canal and the abdomen. We compared the results of transfontanelle ultrasound and MRI scans in 21 children. MRI provided significantly more diagnostic information in 18 cases (85.7%); in 3 cases (14.3%), no additional knowledge about the pathology was provided by the exam. CONCLUSIONS: The MR-compatible incubator increases the availability of MRI to newborns, especially premature newborns and those with low and extremely low body weight, for whom MR examinations are necessary to determine the extent of changes, not limited to the central nervous system, as well as to establish prognosis. Dedicated neonatal coils integrated with the incubator permit more accurate diagnosis than the previously used adult coils.

Miliary brain metastases from papillary adenocarcinoma of the lung - unusual MRI pattern with histopathologic correlation.

Miliary brain metastases are very rarely described in the literature but if they are, they are quite obvious on magnetic resonance imaging (MRI) and enhance after intravenous administration of the contrast medium. The authors presented a case of miliary metastatic spread to the brain which was invisible on computed tomography and hardly visible on MRI, i.e. as countless, tiny, slightly T1-hyperintense foci that did not enhance. The authors discussed a few T1-hyperintense brain lesions which did not include metastases (except for metastatic melanoma which was a radiological suggestion after brain MRI). Autopsy revealed papillary adenocarcinoma of the lung with numerous metastatic lesions in both cerebral and cerebellar hemispheres and the meninges.

Use of Fibrin Glue in the Treatment of Persistent Pneumothorax in Premature Infants at the Limit of Viability: Ethical Issues and Two and A Half Years Follow-Up.

INTRODUCTION: Due to the extreme immaturity of many internal organs, including lungs, infants at the limit of viability are more predisposed to a pneumothorax (PTX). In some cases, PTX becomes persistent. Previously, only a few attempts of PTX treatment with fibrin glue were reported. However, its impact on further lung development is unknown. CASE REPORT: We present a case of an extremely preterm infant with persistent PTX who was successfully treated with fibrin glue. In addition, we present a two-and-a-half-year corrected age follow-up focusing on respiratory problems, motor development and sensory organs. Furthermore, we touch upon the related ethical issues. CONCLUSIONS: Fibrin glue should be used to treat persistent PTX even in an extremely preterm infant. No adverse effects were observed. At the two-and-a-half-year corrected age follow-up, despite severe bronchopulmonary dysplasia development, no serious pulmonary problems were observed. However, the child's development is uncertain. This situation raises important ethical issues concerning saving the lives of infants at the limit of viability.

Postradiotherapeutic changes and their evolution in MRI in children with aggressive soft tissue tumors.

BACKGROUND: Magnetic resonance imaging is a commonly used method of monitoring of soft tissue tumours. The aim of the work was to describe precisely the typical changes within soft tissues and bones occurring after radiotherapy in children treated for sarcomas and other soft tissue tumours. With time, the changes undergo evolution and their characteristics and comparison with previous examinations help in a difficult differentiation between tumour lesions and posttherapeutic changes. MATERIAL/METHODS: Fifteen children and young adolescents (9 boys and 6 girls) aged between 2 and 22 years (mean age of 13.4 years) with diagnosed aggressive soft tissue tumours, were treated with radiotherapy. There were 102 MRI examinations analysed in the period from February 2004 to February 2008. The examinations were performed with a 1.5T MRI scanner in the following sequences: SE T1, SE T1+fatsat, before and after gadolinium administration (Gd), FSE T2 and STIR in three planes, and, in some selected cases, a dynamic gadolinium-enhanced (DCE MRI) examination with FAME sequence. HISTOPATHOLOGICAL EXAMINATIONS SHOWED: rhabdomyosarcoma (RMS) in 8 cases, synovial sarcoma - 3, agressive desmoid fibroma - 3, mesenchymoma mal. - 1. MRI examinations were performed at different postradiotherapeutic stages, several times in one patient (12 times at the most). RESULTS: Every postirradiation stage revealed a typical picture of posttherapeutic changes. We distinguished four stages and described changes in different sequences within soft tissues and bones, as well as features of contrast enhancement and enhancement curves in a dynamic study. The stages included: I stage - early, up to 3 months after rth, II stage - chronic, from 3 months to 12 months after rth, with some differences between the following periods: • 3-9 months; 9-12 months; III stage - late, from 1 to 3 years after rth, IV stage - distant, more than 3 years after rth. In the early stage, there were 2 cases with a suspicious, equivocal image of postradiotherapeutic changes. In the chronic stage, there was one recurrence and one case of increasing changes after the therapy. However, the changes resolved in subsequent examinations. In the distant stage, we found two cases of a local recurrence. CONCLUSIONS: 1. MRI is a method of choice in the monitoring of treatment of aggressive soft tissue tumours and in diagnosis of recurrence. 2. To interpret the examination results, it is very important to know the MRI characteristics of changes after radiotherapy and their evolution with time. 3. Interpretation of MRI images and differentiation between postradiotherapeutic and neoplastic changes is difficult, especially at an early postradiotherapeutic stage. 4. A dynamic MRI examination may be useful in the differentiation between postradiotherapeutic and neoplastic changes but it may be unreliable at an early postradiotherapeutic stage. 5. When interpreting the results, it is very important to compare the image with the previous ones. It is therefore indicated to carry out a baseline MRI in the early postradiotherapeutic stage, and then further follow-up images, at several-month intervals.

Pneumococcal Meningitis and Its Sequelae - A Devastating CNS Disease.

INTRODUCTION: In countries where Haemophilus influenzae type B vaccine is used, Streptococcus pneumoniae is the most common cause of bacterial meningitis in young children and notable cause of morbidity/mortality. The authors present material of magnetic resonance imaging (MRI) of patients with pneumococcal meningitis from archive of Department of Diagnostic Imaging of Institute of Mother and Child in Warsaw. MATERIALS AND METHODS: We performed 27 brain MRI scans and 1 follow-up computed tomography (CT) in 10 children (2 girls and 8 boys) aged from neonate to 5 years at disease onset with proven pneumococcal infection. RESULTS: Follow-up period range was 0-12 years. Two children underwent only one MRI, one of them died before follow-up and the other was lost from further observation. There was one case of relatively benign disease course with mild changes on MRI. In another seemingly benign case, acute transient hydrocephalus was observed. Six children developed hydrocephalus, and two required ventriculoperitoneal shunting complicated by neuroinfection, shunt malfunction and revisions. Two patients developed epilepsy. In six children, spastic paresis of various severity was diagnosed, up to quadriplegia in one who is under the longest observation (>12 years) and survived in vegetative state. Three other children suffer from delayed psychomotor development to severe intellectual disability. CONCLUSIONS: MRI shows perfectly the degree of central nervous system (CNS) damage during and after pneumococcal invasion. Despite appropriate treatment, disease course may be unpredictably serious. Attempts to eliminate the obligation to vaccinate are extremely irresponsible taking into account potential danger of death, vegetative state or another form of severe damage to CNS. Social and financial costs of care of survivors are very high with shunts placements and changes, (neuro)infections, rehabilitation, families breakdown, etc.

Neonatal brain and body imaging in the MR-compatible incubator.

BACKGROUND: The use of a specialized MR-compatible incubator (INC) is very poorly described in the literature and only with regard to brain imaging. OBJECTIVES: To present our own experience with brain and body magnetic resonance imaging (MRI) in the INC in a large cohort of neonates. MATERIAL AND METHODS: A total of 555 examinations were performed in 530 newborns with the use of a 1.5T system and Nomag IC 1.5 incubator, equipped with head and body coils. RESULTS: More than half of neonates (54%) were prematurely born at 22 + 6-36 + 6 gestational weeks. They were examined from the first to 153 days of life (median: 18.5, mean: 37.7) with body weights 600-5000 g (mean: 3051 g), 23% of less than 2500 g. The proportion of brain MRIs to other body regions was 533:85 = 86%:14%. In 36.6% of cases, MRI showed more abnormalities than ultrasound (USG), in a further 21.8%, MRI diagnosis was completely different, in 4.7%, a pathology described on a USG was ruled out on MRI. The superiority of MRI over USG was 63.1%. CONCLUSIONS: MR-compatible incubator significantly increased the availability of MRI to newborns, especially to premature and unstable newborns. The integration of body coils into the INC increased the spectrum of examinations and made possible the scanning not only of the brain but also the body. Dedicated neonatal coils improved image quality and allowed more accurate diagnosis than the previously used adult coils. Immobilization of the babies in the INC by means of Velcro belts and head fixation inserts is better than in adult coils. The closed space of the INC isolates newborns to a greater extent from the negative influence of noise in the MR environment.

CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999-2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%), 14 boys, aged 5-22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing's sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS), and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45%) than in other reports (0.7%). Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

Diffusion restriction in the corticospinal tracts and the corpus callosum in neonates after cerebral insult.

BACKGROUND: In neonatal brains diffusion restriction, which is not limited to the region of insult, but is also found in distant locations from it seems to be a frequent finding, called pre-Wallerian degeneration. OBJECTIVES: The purpose of this study was to describe these findings and to estimate the frequency of their occurrence with an attempt to determine their clinical significance. METHODS: 125 brain MRI examinations of neonates with confirmed brain damage performed or consulted in our Institute were retrospectively reviewed, focusing on the presence of restricted diffusion in corticospinal tracts (CST) and corpus callosum (CC). Apparent diffusion coefficients (ADC) were measured in callosal splenium and compared to normal neonatal brains. RESULTS: Restricted diffusion was found in 21 newborns (16.8%): in 4 in CST (3.2%), in 5 in CC (4.0%), in 12 in both (9.6%). Mean ADC value in CC was 0.638, standard deviation (SD): 0.211µm2/s and in the control group 0.995, SD: 0.162µm2/s (p=0,001). CONCLUSIONS: Neonatal brain MRI should be searched for DWI abnormalities which are not rare and require careful studying of ADC maps. Diffusion restriction in the corpus callosum and/or corticospinal tracts below the region of insult should not be mistaken for acute ischemia as it most likely reflects early phase of secondary neuronal degeneration called pre-Wallerian degeneration. This finding helps in prognostication and guides the management of the affected neonates.

[Vesicoureteral reflux in children with prenatal suspicion of urinary tract abnormalities]. / Odplyw pecherzowo-moczowodowy u dzieci z prenatalnym podejrzeniem wady ukladu moczowego.

AIM: The aim of the study was to assess indications to voiding cystourethrography (VCUG) in children with suspicion of urinary tract (UT) abnormalities in prenatal ultrasonography (US). MATERIAL AND METHODS: We studied 93 children aged 1 day to 7 months (mean 1.4 +/- 1.3 months). Prenatal USG was done between 18-41 week of gestational age (mean 32.6 +/- 5.6). Congenital anomalies of UT were suspected in 88% fetuses with dilatation of UT and in 12% fetuses with other changes in UT. Postnatal US was done in all children between 1-122 day of life (mean 18.2 +/- 21.5); VCUG between 1 day to 13 months (mean 2.1 +/- 2.3 months), in this in 75 (81%) infants in the first months of life. RESULTS: Abnormal result of postnatal US had 84 (90%) children (dilatation of pyelocalyceal system and/or ureter, duplex kidney, multicystic dysplastic kidney, ureterocele). We found normal VCUG in 67 (72%) children, abnormal in 26 (28%). Vesicoureteral reflux (VUR) to 28 kidneys was detected in 20 (21.5%) children (1st--1 kidney, 2nd--14, 3rd--3, 4th--6 and 5th--4 kidneys); another anomalies in 6 (in 2 posterior urethral valves). VUR was observed in 17.3% VCUG performed before 3 months of age and in 38.9% performed later (p<0.05). VUR was recognized: in 17.2% kidneys with normal prenatal US and in 16.4% kidneys with dilatation of UT (NS); in 6 (8%) kidneys with normal postnatal US and 22 (29.8%) with abnormal (p=0.05). There was not statistically significant difference in frequency of VUR in kidney with normal (0-5 mm) and abnormal postnatal renal pelvic diameter (RPD) (11.3% vs 19%, NS). The sensivity and specifity of normal postnatal USG in detection VUR were 79% and 56% respectively, and of normal postnatal RPD were 61% and 46% respectively. CONCLUSIONS: 1. Indication to performing VCUG in children with prenatally detected hydronephrosis should be persistent dilatation of pyelocalyceal system and/or ureter in postnatal US or another abnormalities in UT. 2. Normal prenatal and postnatal USG does not exclude presence of VUR. 3. VCUG in children with prenatally detected hydronephrosis should be performed about 3 months of life, in specially situation earlier.

Neurocutaneous melanosis in children with giant congenital melanocytic nevi.

Twenty-four children with giant congenital melanocytic nevi underwent brain MRI at 1.5 T scanner. Melanin deposits in the brain were found in seven children (29.2%) located in temporal lobes, thalamus, cerebellum, and pons. One patient showed leptomeningeal involvement. Six patients were asymptomatic, and one had epilepsy. As opposed to previous reports, localization of skin nevi on anterior part of trunk was correlated to central nervous system involvement. In all patients with brain involvement skin nevi showed picture of compound nevus with neurofibromatic component.

MRI patterns of hypoxic-ischemic brain injury in preterm and full term infants - classical and less common MR findings.

Hypoxic-ischemic brain injury occurring in antenatal, perinatal or early postnatal period constitutes an important diagnostic problem in both term and prematurely born neonates. Over the past several years magnetic resonance imaging (MRI) has become relatively easily accessible in Poland. On the basis of the central nervous system MRI, the experienced radiologist are able to determine the location of the hypoxic-ischemic lesions, their extent and evolution. Therefore he can help clinicians to answer the question whether the brain damage of the newborn is responsible for its clinical condition and he can contribute to determining the prognosis of the infant's future development. The aim of this study is to present the current knowledge of different types of hypoxic-ischemic brain lesions based on our personal experience and MR images from the archives of the Department of Diagnostic Imaging at the Institute of Mother and Child.

The value of magnetic resonance imaging in the early diagnosis of Creutzfeldt-Jakob disease - own experience.

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a rare progressive neurodegenerative disorder, caused by the deposition of the pathological isoform of prion protein PrPsc in the central nervous system. The classic triad of symptoms consists of: rapidly progressive dementia, myoclonus and typical electroencephalographic findings (intermittent rhythmic delta activity and periodic sharp wave complexes). Detection of 14-3-3 protein in the cerebrospinal fluid plays an important diagnostic role as well. Magnetic resonance (MR) images of the brain have been recently incorporated into the diagnostic criteria of sporadic Creutzfeldt-Jakob disease. CASE REPORT: MR examinations were performed in a 65-year-old man and a 54-year-old woman with delusional disorder and cognitive dysfunction, respectively. Diffusion restriction (hyperintense signal in DWI) was shown in the cortex of the left parietal and occipital lobe in the first patient and symmetrically in the cortex of both cerebral hemispheres except for precentral gyri in the second one. In both cases, the first examinations were misread, with the suspicion of ischemic infarcts as the first differential diagnosis. Consultations and subsequent MR examinations in which lesions in subcortical nuclei appeared allowed for a diagnosis of probable CJD. In the first case it was confirmed by clinical picture, EEG and finally - autopsy. In the second case, EEG was not typical for CJD but the clinical course of the disease confirmed that diagnosis. CONCLUSIONS: The authors present the cases of two patients with characteristic MR images that allowed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clinical picture appeared. Early diagnosis is nowadays important for the prevention of disease transmission and in the future - hopefully - for early treatment.

[Sonography of small bowel, colon and appendix in children with cystic fibrosis]. / Ocena ultrasonograficzna jelita cienkiego, grubego i wyrostka robaczkowego u pacjentów z mukowiscydoza.

AIM: To assess the utility of sonographic examination in estimation of the incidence and character of pathological changes in different parts of the intestine and other abdominal organs in children with cystic fibrosis (CF). MATERIAL AND METHOD: The study group consisted of 182 children (94 girls, 88 boys), aged from 2 months up to 22 yrs, with diagnosed cystic fibrosis or its suspicion, confirmed later. The control group consisted of 20 children with mean age 8 yrs. Sonography was performed with Philips equipment: 4000 HDI and iU 22 using convex, microconvex and linear probes. 329 examinations were performed in 182 children in 2.5 years, 109 underwent second US and 38 patients were examined for the third time. RESULTS: In children with CF, changes in the intestines were observed in 108 patients (59.3%), in the colon in 97 patients (53.3%), and in the small intestine in 49 patients (26.9%). In the control group the wall thickness of large intestine did not surpass 1.6 mm, of small intestine--1.4 mm. In the tested group the maximum thickness of large intestine wall was 7 mm and of small intestine--4.3 mm. Layer structure of the ileum wall was found in 46 children (25%), enlarged appendix in 21 patients (11.6%). Enlarged mesenteric lymph nodes were observed in 84 pts (46%). Hyperaemia of the bowel wall was not observed on Power Doppler examination. In 71 patients (39%) changes in the structure of the liver were observed (cirrhosis, steatosis, enlargement) and in 114 (62.6%) there were changes in the pancreas. CONCLUSIONS: Sonographic examination in patients with cystic fibrosis revealed a high frequency of intestinal changes which confirms the need of including this examination in the annual follow-up of children with CF. In cases of unidentified CF, abdominal sonography leads to the diagnosis.