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Background:Bronchiectasis has been defined as the abnormal and permanent dilation of the bronchi. It is still an important problem in many developing countries. Aim:The aim of this study was to identify the chacteristics and underlying etiology of children followed with the diagnosis of noncystic fibrosis bronchiectasis. Materials and Methods:Children with bronchiectasis confirmed with high-resolution computed tomography were enrolled into the study. The data of the patients, including symptoms of the disease, age at the onset of symptoms, findings of physical examination, labrotory investigations performed in order to identify the etiology of bronchiectasis, etiology of bronchiectasis if found, radiologic findings and treatment modalities were noted. Results: Sixty-six children between 1 and 17 years were included in the study retrospectively. Forty-four of them were males (66.7%) and 22 (33.3%) were females. The most common presenting symptoms were cough (100%) and sputum expectoration (50%). An underlying etiology was identified in 44 (66.7%) of the study subjects. The four most common underlying causes were found as infections (21.2%), asthma (16.7%), aspiration syndromes and/or gastroesophageal reflux disease (9.1%) and immunodeficiency syndromes (7.6%), respectively. Conclusion:Identifying an underlying etiology will have a significant effect on the management of noncystic fibrosis bronchiectasis. Defining the cause of bronchiectasis may also decrease its incidence, progression and complications.

Abstract Centronuclear myopathies (CNM) describe a group of rare muscle diseases typically presenting an abnormal positioning of nuclei in muscle fibers. To date, three genes are known to be associated to a classical CNM phenotype. The X-linked neonatal form (XLCNM) is due to mutations in MTM1 and involves a severe and generalized muscle weakness at birth. The autosomal dominant form results from DNM2 mutations and has been described with early childhood and adult onset (ADCNM). Autosomal recessive centronuclear myopathy (ARCNM) is less characterized and has recently been associated to mutations in BIN1, encoding amphiphysin 2. Here we present the first clinical description of intrafamilal variability in two first-degree cousins with a novel BIN1 stop mutation. In addition to skeletal muscle defects, both patients have mild mental retardation and the more severely affected male also displays abnormal ventilation and cardiac arrhythmia, thus expanding the phenotypic spectrum of BIN1-related CNM to non skeletal muscle defects. We provide an up-to-date review of all previous cases with ARCNM and BIN1 mutations.

A 14-year-old male presented with paresthesias on the right upper and lower extremities, headache, and vomiting. In addition to worsening paresthesia and weakness on the right side of his body, blurred vision, fever, and skin lesions developed. He also had skin lesions characterized with 3-10 mm papules with a white atrophic center surrounded by pink rim mostly on the trunk and lower extremities. Brain magnetic resonance imaging showed chronic subdural effusion and encephalomalacia of the left cerebral hemisphere. Cerebrospinal fluid (CSF) examination revealed increased protein levels. Electromyography was consistent with diffuse polyradiculoneuropathy. Skin biopsy confirmed the diagnosis of a rare vasculopathy: Degos disease. A case presenting with chronic subdural effusion, encephalomalacia, elevated CSF protein, and polyradiculopathy should be carefully examined for skin lesions, which may suggest the diagnosis of Degos disease.

Fibromuscular dysplasia is a rare, idiopathic and nonatheromatous disease. It is rarely encountered as a cause of stroke in children. We report a nine-year-old girl with stroke in whom extensive fibromuscular dysplasia of intracranial vessels was established. She also had familial combined hyperlipidemia as an additional risk factor. This case suggests that additional risk factors like hyperlipidemia in cases with fibromuscular dystrophy may facilitate the occurrence of stroke at early ages.

This study describes the clinical and radiological features of tuberculosis in infants under one year of age. Medical records were reviewed for infants aged 12 months or less with proven tuberculosis. Six patients' data were evaluated. Cough and tachypnea were the major symptom and sign, respectively. Contact with an adult case of tuberculosis was present in five of the cases. Tuberculin skin test was positive in only one case. Mycobacterium tuberculosis was cultured from gastric aspirates of four of five infants and from cerebrospinal fluid in one case. Consolidation was the most common parenchymal lung lesion occurring in four of the patients. Mediastinal or hilar lymphadenopathies were also detected in four of the patients and calcifications were seen within the enlarged nodes in two of them. Antituberculous treatment appeared to be well tolerated without significant adverse effects. Significant radiological improvement was noted after a mean period of 4.6 months.

Objective: The present study assessed whether there is a correlation between hepatic artery resistive index (HARI) and increase of body mass index and hepatosteatosis grading in children with non-alcoholic fatty liver disease (NAFLD) reflecting hemodynamic effects of hepatosteatosis., Methods: Thirty three healthy children [body mass index(BMI): mean+/- standart deviation(SD), min-max: 20.1+/-1.14(18.5-23.7), 33 overweight [BMI:25.1+/-2.2 (18.5-23.7)] and 66 obese [BMI:31.1+/-2(25.6-40)] adolescents were enrolled into the study. To search the relation of HARI with fatty liver degree, study subjects subdivided into groups according to their degree of fatty liver at ultrasonography(US)., Results: Increase of HARI was correlated with increase in BMI (p<0.0001, r=0.533). Increase of HARI was well correlated with increase in degree of fatty liver (p<0.0001, r=0.630)., Conclusion: The present study results suggest that there are positive correlations of HARI with BMI and hepatosteatosis grade in obese children with NAFLD. HARI may be a candidate parameter to determine early alarming hemodynamic changes in hepatic tissue of obese children with fatty liver before development of severe stages NAFLD.

Megalencephalic leukoencephalopathy with subcortical cysts is a rare leukodystrophy that is characterized by macrocephaly and a slowly progressive clinical course. It is one of the most commonly reported leukoencephalopathies in Turkey. Mutations in the MLC1 gene are the main cause of the disease. We report two patients with megalencephalic leukoencephalopathy with subcortical cysts with confirmed mutations in the MLC1 gene. The mutation in the second patient was novel. We also review identified mutations in the Turkish population.

The pathological nature of Leigh syndrome is highly variable and depends on the underlying mitochondrial or nuclear genome defect. Mitochondrial m.8993T>G and m.8993T>C mutations are responsible for both NARP (neurogenic weakness, ataxia and retinitis pigmentosa) and Leigh syndrome depending on the amount of mutant mtDNA. The clinical findings of Leigh syndrome caused by the m.8993T>C mutation are less severe than those associated with the m.8993T>G mutation, and ragged red fibers, oligoclonal bands in cerebrospinal fluid, and additional deficiencies of respiratory enzyme complexes are usually not found. This report presents a two year old girl with Leigh syndrome caused by a m.8993T>C mutation. Interestingly she had ragged red fibers in muscle tissue, oligoclonal bands in CSF and focal deficient histochemical staining for complexes I and IV.

The aim of this study is to evaluate the carotid artery intima media thickness and serum lipids in pediatric patients with epilepsy treated with valproic acid. The study included 44 pediatric epileptic and 40 healthy children. Intima media thickness of left common carotid artery and fasting lipid profile (total cholesterol, triglycerides, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol) were assessed. Although we did not observe any differences regarding serum lipid profiles, intima media thickness of common carotid artery was significantly higher in epileptic patients treated with valproic acid. We suggest that this increase in intima media thickness of common carotid artery may be due to epilepsy and/or valproic acid treatment.

Aim: The aim of this study was to evaluate the clinical, pathological and radiological survey of patients affected by Leigh syndrome., Methods: Eleven patients with Leigh disease were evaluated at Dokuz Eylül University, School of Medicine, Department of Pediatric Neurology. All patients underwent neurological evaluation with detailed medical and family history. Muscle biopsy from quadriceps muscle, brain magnetic resonance imaging and brain magnetic spectroscopy were obtained., Results: The patients were aged between 1 month and 8 years (mean age: 2.29+/-2.58 years). The most common presentation findings were psychomotor retardation and acute metabolic encephalopathy. All patients had elevated lactate in the blood and/or cerebrospinal fluid. Except in two patients, brain magnetic resonance imaging revealed abnormal symmetrical lesions in the brainstem and basal ganglia. Brain magnetic resonance spectroscopy revealed abnormal lactate peak in all patients. The muscle biopsy of two patients showed cytocrom-c oxidase deficiency and measurement of respiratory chain complex in one patient revealed complex I and IV deficiency. One patient was found to carry mitochondrial T8993C mutation., Conclusions: There are no specific markers for Leigh disease which lead to extensive work-up. The disease should be considered in patients who present progressive neurologic symptoms involving brainstem and basal ganglia.

Chronic recurrent multifocal osteomyelitis is an unusual inflammatory process of unknown origin involving multiple osseous sites, often recurrently. Selective immunoglobulin M (IgM) deficiency is a rare primary immunodeficiency disease, which can be associated with autoimmune diseases such as systemic lupus erythematosus, Hashimoto's disease, or hemolytic anemia. Here we report a case of a chronic recurrent multifocal osteomyelitis coexisting with selective IgM deficiency.

Aneurysmal bone cyst is a rare benign tumor of the bone that can be difficult to distinguish from malignant tumors, especially when it presents in an unusual location. Herein, we report a six-year-old girl with a primary aneurysmal bone cyst in an uncommon location. It originated from the 4th rib and she presented with a huge chest wall mass. Despite the large size of the cyst, the only symptom was persistent cough. She was successfully treated with total excision of tumor without any complication. The patient has been followed up for 56 months without any recurrence.

We evaluated the predictors of renal scar in children with urinary tract infections (UTIs) having primary vesicoureteral reflux (VUR). Data of patients who were examined by dimercaptosuccinic acid (DMSA) scintigraphy between 1995 and 2005 were evaluated retrospectively. Gender, age, reflux grade, presence/development of scarring, breakthrough UTIs, and resolution of reflux, were recorded. The relation of gender, age and VUR grade to preformed scarring and the relation of gender, age, VUR grade, presence of preformed scarring, number of breakthrough UTIs and reflux resolution to new scarring were assessed. There were 138 patients [male/female (M/F) 53/85]. Multivariate analysis showed that male gender [odds ratio (OR) 2.5], age > or = 27 months in girls (OR 4.2) and grades IV-V reflux (OR 12.4) were independent indicators of renal scarring. On the other hand, only the presence of previous renal scarring was found to be an independent indicator for the development of new renal scar (OR 13.4). In conclusion, while the most predictive variables for the presence of renal scarring among children presenting with a UTI were male gender, age > or = 27 months in girls, and grades IV-V reflux, the best predictor of new scar formation was presence of previous renal scarring.

Mycoplasma pneumoniae is an important pathogen which causes nervous system disorders during or after the course of a respiratory tract infection. The exact pathogenic mechanism which causes neurological disorders still remains unknown. Although meningoencephalitis and acute disseminated encephalomyelitis are common complications, there are few cases of acute transverse myelitis and isolated abducens nerve palsy associated with M. pneumoniae infection in childhood. The association between ocular myasthenia gravis and M. pneumoniae infection has not been described before. Here, we describe five patients with different nervous system complications associated with M. pneumoniae infection and discuss the pathological features of central nervous system involvement.

Vertebral artery dissection as a cause of stroke is rarely reported in children. The association between vertebral artery dissection and Klippel-Feil syndrome is also very rare. We report on a case of vertebral artery dissection with posterior circulation involvement in a child with Klippel-Feil syndrome after a hard physical-training lesson. She was also diagnosed with Wildervanck syndrome, with additional clinical findings. Vertebral artery dissection should be considered in patients with Klippel-Feil syndrome who present with acute-onset neurologic signs. Movements such as hyperextension with rotation of the neck should be avoided in these cases.

Although cerebellitis is common in childhood but cerebellitis with cerebellar swelling is rarely reported. Pulsed high dose methylprednisolone treatment is the choice of treatment for cases who have non-progressive symptoms. An 8-year-old girl presented acutely with vertigo, headache, and vomiting. Brain magnetic resonance imaging showed marked bilateral cerebellar swelling with increased signal on T2-weighted imaging. Following treatment with standard dexamethasone dose, the clinical and radiological signs resolved in 1 week. We conclude that standard dexamethasone treatment should be used in mild cases of acute cerebellitis in order to avoid adverse reactions of pulsed high dose methylprednisolone treatment.

Untimely bilateral testicular enlargement greater than 3 ml is suggestive of precocious puberty, in which an underlying organic disease is more common in boys than in girls. We describe a 7 1/2 year-old boy presenting with testicular enlargement due to testicular microlithiasis. Following hormonal tests, diagnosis was based on ultrasonographic findings. Three years follow-up of the patient revealed normal pubertal progress and no malignant evolution. Testicular microlithiasis is a rare cause of testicular enlargement and pediatricians should take this disease into account in the differential diagnosis of suspected precocious puberty.

Lipofibromatosis is a recently described rare benign soft tissue tumor of childhood. We report a case of a lipofibromatosis in a two-year-old girl with a painless mass on the plantar aspect of her right foot who developed local recurrence eight months after surgery. The tumor has a high rate of non-destructive recurrence, but there is no metastatic potential. Complete surgical resection is the mainstay of treatment. Nevertheless, the rareness of its presentation should be taken into account by the pediatric pathologist, and considerable surgical judgment is of great importance.

Purpose: The aim of this study was to determine the value of power Doppler ultrasonography in the differential diagnosis of intracranial extraaxial fluid collections in enlarged subarachnoid spaces., Materials and Methods: The study included 11 patients referred to our hospital's radiology department between April 2001 and February 2002 who were determined to have intracranial extraaxial effusion based on the results of cranial ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI). Transfontanel examinations of the enlarged subarachnoid spaces, including cortical vascular structures, with a 4-7 MHz linear probe were used to make differential diagnoses of extracerebral fluid collections., Results: In 8 of the 11 patients, the diagnosis was an enlarged benign extraaxial subarachnoid space. In one case, the differentiation of mirror artifacts of an epidural hematoma from a cephalhematoma was constructed by the cortical vascular structure with power Doppler US. In 2 cases, subdural hematomas were seen., Conclusion: Power Doppler US is a safe and complementary method to cranial US that is useful in the differential diagnosis of extraaxial intracranial fluid.

Hemoptysis as a result of pulmonary or bronchial pathologies is a rare but potentially serious problem in childhood. The presented case is an eight-year-old previously healthy girl who was admitted to the emergency department because of recurrent hemoptysis. Because high resolution computerized tomography (HRCT) showed an abscess cavity, antibiotic therapy was continued about six weeks. Lobectomy was done when massive hemoptysis recurred. Histopathological examination revealed follicular bronchiolitis, which is a very rare entity, particularly in childhood. Although HRCT imaging is of great value in the diagnosis of this disease, in our case it failed to show any evidence of follicular bronchiolitis. In conclusion, the definitive diagnosis of follicular bronchiolitis always requires histopathologic examination of open lung biopsy.

Cerebral volumetric measurements based on magnetic resonance imaging have been established as advanced morphometric techniques with anatomic and clinical utility in adults and children with epilepsy. This study investigated the cerebral and hippocampal volumes in children with idiopathic partial epilepsy to detect the factors correlated with volume reduction. Magnetic resonance imaging volumetric measurements were performed of the total cerebral and hippocampal formation volumes in 30 patients with idiopathic partial epilepsy between 3 to 18 years old. The cerebral and the total, right, and left hippocampal volumes of the study and control patients were detected using volumetric magnetic resonance imaging, and the volumes were compared between the 2 groups. In study patients, the correlation between volumetric findings and seizure characteristics was evaluated. The results suggested that children with idiopathic partial epilepsy had significant hippocampal volume reduction that was not influenced by the age of onset and the duration of epilepsy.

Prothrombin G20210A mutation is an important prothrombotic condition for venous thrombosis. Recently, some studies have also considered it to be a risk factor for arterial ischemic stroke in children. A 10-year-old boy with basilar artery thrombosis who was heterozygous for prothrombin G20210A mutation is described. In concordance with the previous literature, the present case suggests that prothrombin G20210A mutation may be a risk factor for arterial ischemic stroke in childhood.

Idiopathic thrombocytopenic purpura usually presents with minor bleeding such as petechia and purpura. Rarely, life-threatening events as intracranial and intra-abdominal bleedings can be seen. We would like to present a rare case diagnosed as idiopathic thrombocytopenic purpura, presenting with abdominal pain and paleness. In this 17-year-old female patient, extensive abdominal sensitivity was revealed on physical examination and massive intra-abdominal hemorrhage secondary to distended ovarian follicle rupture was seen on ultrasonography and abdominal computed tomography. The case was treated successfully with intravenous immunoglobin, thrombocyte suspension, and pulse methylprednisolone.

In this study the aim was to evaluate the intrathecal sICAM-1 production in multiple sclerosis (MS) patients during relapse and remission. In addition to this, we assessed whether there is a correlation between intrathecal sICAM-1 production and other disease activity markers such as IgG index and gadolinium enhancement in magnetic resonance imaging (MRI). Twenty four relapsing-remitting MS patients were included in the study. Serum and cerebrospinal fluid (CSF) samples were obtained both during relapse and remission. The soluble form of ICAM (sICAM) was measured by the ELISA method in serum and CSF. Cranial MRI with triple dose gadolinium injection was performed for each patient both during relapse and remission. Serum levels of sICAM-1 (245.23 +/- 92.88 ng/ml) were higher during relapse than those in remission (219.90 +/- 110.94 ng/ml), but the difference was not statistically significant. In relapse periods CSF levels of sICAM-1 (1.304 +/- 0.92 ng/ml) were higher than those in remission (1.06 +/- 0.86 ng/ml), but this was not significant. However, during relapse periods patients had significantly higher sICAM-1 index values (1.76 +/- 0.60) than those found during remission periods (1.01 +/- 0.44) (p < 0.05). The IgG index values were higher in relapse periods than in remission (0.88 +/- 0.37 vs. 0.67 +/- 0.28) (p < 0.005). On T1 weighted images following triple dose Gd injection, at least two or more enhancing lesions were present in 22/24 of the patients (91%) in relapse and 4/24 of the patients (19%) in remission. There was strong correlation both between the sICAM-1 index and Gd enhancement (r =0 .72 p < 0.05) and sICAM-1 index and IgG index in relapse (r = 0.69 p < 0.05). In conclusion, there is association between high sICAM-1 and IgG indices, as well as between high sICAM-1 index and Gd enhancing MRI lesions in relapsing MS patients.

The aim of this study was to evaluate the correlation of ultrasonography-proven fatty liver with liver functions, serum lipid levels and anthropometric measurements in children with exogenous obesity. Three hundred and twenty-two patients (183 girls, 56.8%) with a mean age of 11.4+/-3.2 years (4-18 years) who presented with the complaint of obesity were enrolled. In 38 (11.8%) patients, increased liver echogenicity resembling fatty liver was found (Group 1). The body mass index percentages of group 1 patients were significantly higher than of those without fatty liver (Group 2) (157.7+/-18.0 vs 151.3+/-17.8, p=0.038). Alanine and aspartate aminotransferase levels of group 1 patients were significantly higher than of group 2 (p=0.002 vs p=0.028, respectively). Triglyceride levels were significantly higher in group 1 patients (120.8+/-88.8 vs 100.5+/-58.5 mg/dl, p=0.044). In conclusion, ultrasonography is an easy and noninvasive method for the diagnosis of fatty liver in children with obesity. Body mass index and serum lipids were higher in group 1 patients. The diagnosis and early treatment of obesity in childhood is important for the prevention and better treatment of related complications. Thus, ultrasonography should be a part of the early evaluation of obese children.

Glutaric aciduria type 1 is an inborn error of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-coenzyme A dehydrogenase. The disease often appears in infancy with an encephalopathic episode that results in acute basal ganglia and white matter degeneration. The neuroimaging findings in glutaric aciduria type 1 have been well defined. However, the changes in magnetic resonance spectroscopy, a noninvasive tool for identifying the biochemical state of the brain, are scarce in glutaric aciduria type 1. This report presents the magnetic resonance spectroscopy findings in a 19-month-old male with glutaric aciduria type 1. Magnetic resonance spectroscopy of right frontal white matter and right lentiform nuclei revealed decreased N-acetylaspartate/creatine ratio, slightly increased choline/creatine ratio, and increased myoinositol/creatine ratio, compared with the age-matched control patients. We thought that these changes were in accordance with neuroaxonal damage, demyelination, and astrocytosis in these areas. In conclusion, proton magnetic resonance spectroscopy provides a tool for assessing metabolic disturbances and the extent of brain damage noninvasively in glutaric aciduria type 1.

Pyogenic liver abscess is a rare and life-threatening disease in children. Our case is noteworthy because of the rapid advancement of liver abscesses without any other systemic disorder. A 16-year-old girl was admitted to the hospital with fatigue, pallor, weight loss and high fever. In physical examination a fluctuating mass was observed under the scapular area and hepatosplenomegaly was found. In computed tomography, three septated cystic lesions which looked like abscesses were demonstrated in the liver. The abscess was drained through percutaneous route. Right pleural empyema with clinical features of adult respiratory distress syndrome appeared after the first day of treatment. Bacteroides sp. was isolated from pus. On the twentieth day of the therapy, control abdominal computed tomography revealed two new abscesses in the liver. They were drained and the antibiotic therapy was continued with ticarcillin-clavulanate, fluconazole and levofloxacin. By the end of the first week of the therapy, the fever of the patient had abated. This therapy was continued for four weeks; 15 days after the end of the therapy there was prominent healing of the liver lesions with only one necrotic remnant 2 cm in diameter on abdominal computed tomography.

The aim of this study was to determine the spectrum of clinical abnormalities in the agyria-pachygyria complex, to identify possible causes, and to correlate the clinical features with the extent of the lesions on magnetic resonance imaging. On the basis of the magnetic resonance imaging findings, 37 patients (22 males, 15 females; mean age 21.1 +/- 31.2 months) with agyria-pachygyria complex were separated into two groups: Group 1 (18 children) manifested generalized or bilateral gyral malformation, and Group 2 (19 children) manifested localized or unilateral gyral malformation. The ratio of generalized seizures in Group 1 was significantly higher, whereas partial seizures were more common in Group 2. Group 1 patients had seizures significantly more frequently than Group 2 patients. Diffuse electroencephalographic abnormalities were significantly more common in Group 1, as were the localized abnormalities in Group 2. Hemipareses were the most frequent neurologic deficit among Group 2 patients. Spastic quadriparesis and microcephaly were more common in Group 1. In conclusion, the extent of agyria-pachygyria complex varies widely and the clinical features are accordingly diverse. Patients with bilateral or generalized gyral anomalies have poor prognosis for outcome of epilepsy and neurologic disability. The recognition of these lesions with higher-resolution techniques of magnetic resonance imaging is important for planning proper treatment and genetic counseling.

Craniovertebral junction surgery requires knowledge regarding the anatomy of this region, particularly the C1 vertebra. Both C1 laminectomy and C1-2 instrumentation necessitate preoperative information about bony landmarks and the vertebral artery. This study compares the results obtained from anatomic and computed tomographic measurements of C1 bony landmarks. 31 C1 cervical vertebrae were measured; the C1 AP diameter, and C1 transverse diameter, the facet diameter, the distance between the anterior tubercle and the anterior aspect of the C1 lateral mass on a lateral view, the distance between the midline and the vertebral artery groove on the outer cortex of the posterior arch of C1 anatomically and computed tomographically. Anatomic measurements were performed by an anatomist using a Vernier caliper accurate to 0.1 mm, whereas the computed tomographic measurements were performed by a radiologist on bone window computed tomography (CT). The mean values and the differences between two measurement modalities were analysed using a paired t-test. There was no statistical difference between the results obtained by anatomical and radiological measurements for six parameters. There was, however, a statistically significant difference between two modalities regarding the distance between the midline and vertebral artery groove on the outer cortex of posterior arch of C1, while slightly different, the difference is within 1 mm and, therefore, not clinically significant. It is concluded that CT reflects most anatomical details of bony landmarks of C1.

Objective: The purpose of this study was to determine the role and cost effectiveness of surveillance imaging at the management of pediatric brain tumors., Materials and Methods: In this study, the imaging and clinical finding of 59 patients who had been diagnosed and followed by the Dokuz Eylul University Pediatric Oncology Group as primary central nervous system tumors between 1988 and 2000 were retrospectively evaluated., Results: We found that the 87.5% of tumor recurrence occurs within 21 months and 93.8% occur within 29 months. About 25% of these recurrences were asymptomatic and these (n=16) could be detected by surveillance imaging with a frequency rate of 1.59%. The cost of imaging for our patients was calculated to be 788 US$ (mean) for a follow-up period of 24 months which would have been 739 US$ if a standard surveillance protocol would have been followed., Conclusion: Surveillance imaging is an effective follow-up in detecting symptomatic recurrence in pediatric brain tumors.

Nitric oxide (NO) molecules have one of the most important roles in the pathogenesis of multiple sclerosis (MS). It has been stated that a continuous and high concentration of NO metabolites in CSF and in the serum of MS patients in relapse may cause toxic damage to myelin and oligodendroglia. The aim of this study was to investigate whether NO is a marker of disease activity and is correlated with other disease activity markers such as active lesions on brain magnetic resonance imaging (MRI) and increased immunoglobulin G (IgG) index. Cerebrospinal fluid (CSF) and peripheral serum (PS) samples were taken from patients with definite MS (n = 24) during relapse and remission and from control subjects (n = 18). The Griess reaction was used to measure the NO metabolites, nitrite and nitrate in CSF and PS. Cranial MRI was carried out with triple dose (0,3 mmol/kg) gadolinium and the IgG index was determined. Nitrite and nitrate concentrations (NNCs) of CSF were 11.16 +/- 8.60 micromol/ml in relapse and 6.72 +/- 3.50 micromol/ml in remission, whereas in PS they were 12.89 +/- 7.62 micromol/ml during relapse and 12.35 +/- 6.62 micromol/ml during remission. In control subjects NNCs in CSF and PS were 7.42 +/- 2.81 micromol/ml and 4.37 +/- 1.63 micromol/ml respectively. NNCs in CSF during relapse period were significantly higher than those of both remission phase and control subjects (p = 0.000). Although serum NNCs did not differ in relapse and remission, they were still higher than normal controls. Validity analysis revealed that NNC measurement in CSF was 71 % specific and 66 % sensitive to disease activity. The most important result was the significant correlation of increased NNCs with the existence of active lesion in cranial MRI and an increase in IgG index (p < 0.05).In conclusion, these results add background data to assist in further outlining the possible role of NO in the pathogenesis of MS. Together with the other markers it may be used as an activity marker in relapses of MS.

Schilder's myelinoclastic diffuse sclerosis is a rare sporadic demyelinating disease that usually affects children between 5 and 14 years old. The disease often mimics intracranial neoplasm or abscess. We report a 9-year-old girl with Schilder's disease who presented with left hemiparesis. Cranial computed tomography and magnetic resonance imaging (MRI) showed large lesions in the subcortical white matter of the occipital and parietal lobes of both hemispheres that were indistinguishable from an abscess. A cerebrospinal fluid oligoclonal band test was found positive. A diagnosis of acute disseminated encephalomyelitis was then suspected. Serial MRI examinations revealed regression of parenchymal lesions, but there were new developing corpus callosum lesions. After 2 months, the patient presented with right-sided visual loss. MRI examination revealed a right optic nerve lesion. The patient had a dramatic clinical response to corticosteroid therapy. Subsequent control MRI examination revealed regression of both lesions. At 24 months of observation, the patient continued to do well without any complaints or neurologic sequelae.

Cerebral sinus thrombosis (CST) is known to be related to a number of underlying aetiologies including otitis media, trauma, pregnancy, birth control pills, tumours, malnutrition, dehydration, haematologic disorders and malignancy (Fishman, 2000; Raizer and Abbott, 2000). We present the case of a patient with breast cancer receiving the antioestrogen drug tamoxifen who developed CST. A 40-year-old female presented as an emergency with a 10-day history of headache and left sided weakness. On questioning her past medical history included a diagnosis of breast cancer 3 years ago treated by radical mastectomy and tamoxifen 20 mg daily. At the time of admission, neurologic examination revealed a mild left sided hemiparesis and a present Babinksi sign. Non-contrast enhanced tomography was normal. Magnetic resonance imaging (MRI) showed thrombosis in the superior sagittal sinus, right lateral sinus and jugular vein in addition venous infarction in the right temporal lobe was present (Figs 1a and b). Routine haematology and biochemistry was normal. Anticoagulation tests, antithrombin III, protein S and C levels were also found to be normal. She was treated with anticoagulation therapy and her hemiparesis improved within 3 days. Control MRI showed the resorption of the venous infarction and resolution of the thrombosis (Fig. 1c).

Carotid-cavernous fistulas (CCF) are reported very rarely in childhood and their clinical course and prognosis are uncertain. We report a 9-year-old boy presented with left eye swelling, neck pain and headache. The MRI findings suggested a CCF with enlarged left superior ophthalmic vein. Ocular Doppler ultrasonography revealed enlarged left superior ophthalmic vein, and arterialization of Doppler wave form. The cerebral angiogram showed normal anatomy. Control Doppler examination findings supported the diagnosis of closure of fistula. The clinical and radiological findings of this unusual presentation are discussed.

A 12-year-old male with subacute sclerosing panencephalitis is presented. Magnetic resonance imaging revealed basal ganglia involvement without white matter changes for several months. Basal ganglia changes are not infrequent in subacute sclerosing panencephalitis, but they tend to appear in advanced clinical stages. Prominent basal ganglia involvement may occur very rarely in subacute sclerosing panencephalitis. In our patient, serial magnetic resonance imaging demonstrated the involvement of white matter after 2 years of magnetic resonance imaging follow-up. In contrast with the neuroradiologic progression, our patient's clinical status remained stable.

An 11-month-old patient with Sturge-Weber syndrome with the absence of facial angioma and normal mental development is presented. Noncontrast computed tomography revealed left parieto-occipital atrophy with heavy gyriform calcifications. Axial T(2)-weighed magnetic resonance imaging confirmed the presence of low-signal areas corresponding to the gyral calcifications evident on computed tomography. Contrast-enhanced T(1)-weighted axial and coronal images exhibited high signals in the left parieto-occipital cortical and subcortical areas, representing angiomatous malformations. The clinical appearance and pathologic features of the reported patient were compared with those of similar patients described in published reports.

Magnetic resonance imaging (MRI) appearance of fibromatosis colli has been reported in only two cases in the literature. We herein describe the MRI findings in a case of fibromatosis colli: the signal intensity of the fusiform mass on T2 weighted images was slightly less than on T1 weighted images, consistent with the presence of some fibrous tissue within the muscle mass.

The magnetic resonance imaging (MRI) findings in 14 patients with biopsy or polymerase chain reaction proven herpes simplex encephalitis were retrospectively reviewed to evaluate the diagnostic value of MRI in the early diagnosis of herpes simplex encephalitis in children. In addition to the early findings, follow-up MRI scans were obtained in four patients. Typical limbic system involvement was seen in 78 percent of the cases. Contrast-enhanced MRI was found to be superior to routine MRI sequences and computerized tomography (CT) in the early detection of inflammation. Follow-up MR images in four patients demonstrated the volume loss and late petechial hemorrhage in the involved regions. Magnetic resonance imaging is the method of choice in the diagnosis and follow-up of herpes simplex encephalitis.

Background and Objective: To evaluate the sensitivity of magnetic resonance imaging (MRI) in detecting retinal detachment (RD) in eyes filled with silicone oil, and to determine the MRI characteristics of silicone oil in vivo., Patients and Methods: Seven eyes of seven patients with RD and eight eyes of eight patients without RD after silicone oil surgery were included in the study. All of the patients had clear media. MRI was performed in these patients. The results of ophthalmic examinations were compared with MRI findings., Results: MRI detected six of the seven detached retinas. The multiplanar imaging capability of MRI helped in showing the RDs in various locations. The chemical shift artifact exaggerated the retinal thickness and made the detached retina visible. Fat suppressed images showed the silicone oil-corneal contact clearly., Conclusion: MRI is an effective technique for demonstrating RD in eyes filled with silicone oil and can be used successfully in eyes with opaque media.