Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

Background/aim: Non-Wilms renal tumors (NWRTs) are rarely encountered in children. The aim of this study is to determine the treatment strategies, prognosis, outcomes, and survival of children with NWRTs at Erciyes University in Kayseri, Turkey. Materials and methods: Medical records of all patients (n = 20) treated for NWRTs over a 23-year period (1995­2018) were reviewed retrospectively. Results: There was male predominance (female/male: 7/13); the median age at diagnosis was 3.2 years old (0.1­13.5 years old). The major histological groups included mesoblastic nephroma (MBN), (n: 5, 25%), malignant rhabdoid tumor (MRT), (n: 5, 25%), renal cell carcinoma, (n: 3, 15%), inflammatory myofibroblastic tumor (n: 2, 10%), multilocular cystic renal tumors (n: 2, 10%), metanephric adenoma (n: 1, 5%), renal neuroblastoma (n: 1, 5%), and bilateral renal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) (n: 1, 5%). All of the patients with NWRTs had radical nephrectomy except the child with bilateral renal ES/PNET. Six children died because of progressive disease; the mortality rate was 30% (n: 6). Conclusion: We have made the first report of bilateral renal involvement of ES/PNET in the English medical literature. Physicians dealing with pediatric renal masses should be alert to the high mortality rate in children with MRT, MBN, and ES/PNET and they should design substantial management plans for NWRTs.

The use of rapamycin in patients with tuberous sclerosis complex: Long-term results.

PURPOSE: The purpose of this study was to evaluate the long-term results of eight cases diagnosed with tuberous sclerosis complex (TSC) and receiving rapamycin therapy because of epileptic seizures and/or accompanying TSC findings. METHOD: Rapamycin therapy was initiated at a dose of 1.5 mg/m2. Seizure frequency, electroencephalographic (EEG) findings, renal and cranial imaging findings, and cutaneous lesions over 3- to 6-month periods during follow-up and treatment were evaluated. RESULTS: Four girls and four boys aged 4-16 years at the start of rapamycin therapy and now aged 9-24 years were evaluated. Duration of rapamycin therapy was 1-5 years, and the monitoring period after commencement of rapamycin therapy lasted 5-8 years. Positive effects were observed at 9-12 months in three out of six cases of renal angiomyolipoma (AML) and in the second year of treatment in one. An increase in AML dimensions was observed in three cases after treatment was stopped. Seizure control was established in the first year of rapamycin therapy in all cases. An increased frequency of seizures was observed in three cases after the second year of treatment. No seizure recurrence was determined in the second year of treatment with rapamycin in five out of eight cases. Recurrence of seizure was observed in 6-12 months after the discontinuation of rapamycin in three cases. CONCLUSION: Rapamycin therapy exhibits positive effects on epileptic seizures in cases of TSC in 1-2 years but these positive effects on seizure control of rapamycin therapy decline after the second year. Larger case series are still needed to determine the duration and effectiveness of treatment in childhood.

Evaluation of symmetrical increased echogenicity of bilateral caudothalamic grooves detected on cranial ultrasonography by comparing with susceptibility-weighted imaging.

OBJECTIVE: To assess symmetrical increased echogenicity of bilateral caudothalamic grooves (SIEBCG) detected on newborn cranial ultrasonography (CUS) using magnetic resonance susceptibility-weighted imaging (SWI). MATERIALS AND METHODS: A total of 14 newborns (8 girls; 12 premature with mean gestational age of 30 weeks and 5 days, 2 mature) who were detected to have SIEBCG on routine serial CUS and underwent cranial magnetic resonance imaging (MRI) were recruited for the study. The cranial MRI examinations including SWI acquired on the same day of SIEBCG detection and serial CUS to assess the progress of SIEBCG lesions in the following 6 month period were retrospectively evaluated and compared for the presence of germinal matrix hemorrhage. RESULTS: On SWI, solely one patient (7, 1%) had signal alteration on caudothalamic groove compatible with grade 1 germinal matrix hemorrhage. Two patients (14, 2%) had parenchymal (on cerebellar and parietal white matter) millimetric hemorrhagic foci. Seven patients (50%) had signs of presumptive hypoxic insult including hyperintense dots on centrum semiovale and periventricular white matter in five, and increased signal intensity on the globus pallidi in two, on T1-weighted images. Four patients (28, 6%) had normal findings. Of these, 10 patients became normal on follow-up CUS at postterm-equivalent age, whereas four were missing. CONCLUSION: Symmetrical increased echogenicity of bilateral caudothalamic grooves seen on newborn CUS may be the indicator of other pathologies as ischemic insult or focal parenchymal hemorrhage. In the presence of SIEBCG, further examination with SWI should be performed.

Pediatric central nervous system tumors in the first 3 years of life: pre-operative mean platelet volume, neutrophil/lymphocyte count ratio, and white blood cell count correlate with the presence of a central nervous system tumor.

PURPOSE: The aim of this study is to describe the relationship of pre-operative complete blood count parameters [mean platelet volume (MPV), neutrophil/lymphocyte count ratio (NLCR), and white blood cell count (WBC)], with the clinical, radiological, and histopathological features and the management options for patients under 3 years of age with a newly diagnosed central nervous system tumors. METHODS: Children with central nervous system (CNS) tumors in the first 3 years of life admitted in the Erciyes University Hospital between April 2004 and April 2014 were enrolled in this study. The CBC parameters were compared with those of an age- and sex-matched normal control group. RESULTS: In the study group, the means of MPV and WBC were 8.00 ± 1.24 fl, and 10,855 ± 3642/mm3 respectively; the median (25-75%) of NLCR was 0.98 (0.66-1.46). For the control group, the means of MPV and WBC were 6.8 ± 0.73 fl and 8565 ± 2522/mm3; the median (25-75%) of NLCR was 0.52 (0.36-0.70). The MPV, WBC, and NLCR were higher in the study group. The median overall survival (OS) of the patients was 60 months (range 0-81.6 months); and median event free survival (EFS) was 24 months (range 0-70.1 months). The formulation of MPV, NLCR, and WBC was found to be predictive for the diagnosis of CNS tumor in children with nonspecific symptoms. The univariate and multiple binary regression analyses showed a positive association of MPV, NLCR, and WBC and the risk of a diagnosis of CNS tumor. There was no relationship between MPV, WBC, NLCR, and histological subgroups. However, there were no associations between CBC parameters and OS or EFS of the patients. CONCLUSIONS: By causing suspicion, MPV, NLCR, and WBC may provide both an earlier radiological investigation decision and thereby an early diagnosis of CNS tumor in children with nonspecific symptoms in the first 3 years of life.

Imaging Evaluation Of Pediatric Orbital Pathologies.

The orbital pathologies commonly detected during the childhood period substantially differ from the lesions that arise in adult orbit. The advance in imaging modalities including computed tomography (CT) and particularly magnetic resonance imaging (MRI) might enable the radiologists and clinicians who would be involved in either medical or surgical care of orbital pathologies, to confidently establish a definite diagnosis prior to histopathologic examination. The purpose of this pictorial assay is to present relatively common paediatric orbital pathologies with regard to CT and MRI findings.

The Age-Related Development of Maxillary Sinus in Children.

INTRODUCTION: Paranasal sinuses are complex structures and show individual variation. Providing normative values for paranasal sinus size and their changes related to age could be helpful in evaluating the presence of some diseases related to sinonasal region. The purpose of the current study was to investigate the development of maxillary sinuses and evaluate the volume changes according to age and sex by using stereological and ellipsoidal formula methods after that to compare these approaches with each other in children. MATERIALS AND METHODS: This retrospective volumetric computed tomography (CT) study was carried out on 361 individuals (180 females, 181 males) between 0 and 18 years old (10 females, 10 males in each group, only 14 age group includes 11 males) with no signs of sinus pathology volumetric estimations determined on CT images using point-counting approach of stereological methods and ellipsoid formula by using morphometric data. RESULTS: Maxillary sinus volume measurements that were obtained using 2 methods were increased with age in both sexes until 16 years old. There was a significant correlation determined between 2 methods (ICC 0.894-1.000 for right and 0.862-0.999 for left maxillary sinus measurements). According to the sex, the right and left mean maxillary sinuses volumes were determined at 8.30 ± 5.19 and 8.57 ± 5.53 cm(3) in male and at 7.60 ± 4.57 and 7.99 ± 4.73 cm(3) in female by using ellipsoid formula respectively. By the stereological method these values were 8.28 ± 5.26, 8.44 ± 5.35 cm(3) and 7.64 ± 4.55, 7.85 ± 4.73 cm(3) respectively. There was no statistically significant difference between the volume of maxillary sinuses with sex and side using both methods. CONCLUSIONS: This study presents the basic data for studies relative to the development of the maxillary sinus in children according to 2 methods. The current study demonstrated that the point-counting method and ellipsoid formula are both effective in determining volume estimation of maxillary sinuses and are well suited for CT studies.

Inflammatory Myofibroblastic Tumor of the Kidney and Bilateral Lung Nodules in a Child Mimicking Wilms Tumor With Lung Metastases.

Renal inflammatory myofibroblastic tumor (IMT) is an extremely rare lesion especially in children. This report describes a case of renal IMT accompanied by multiple lung nodules mimicking Wilms tumor with lung metastasis in a 3-year-old boy. To our knowledge, this is a unique case of IMT which has not been reported in the literature previously.

MR imaging findings in children with pseudotumor cerebri and comparison with healthy controls.

OBJECTIVE: The aim of this study was to discuss the MR imaging findings of pseudotumor cerebri in children by comparing with healthy controls. MATERIALS AND METHODS: Forty-two pseudotumor cerebri patients from hospital records between 2003 and 2011 were retrospectively reviewed. Between 2007 and 2011 25 patients (16 boys, 9 girls) whose brain MR images were on PACS workstation were included. Thirty MR imaging examinations (14 boys, 16 girls) which were interpreted as normal constituted the control group. Two pediatric radiologists reviewed each MRI for optic nerve sheath distension, intraocular protrusion of the optic nerve, posterior globe flattening, horizontal tortuosity of the optic nerve, and decreased pituitary gland size. The sensitivity, specificity, and overall accuracy of the findings on MRI were calculated. RESULTS: Optic nerve sheath enlarged in the PTC group (mean value, 4.3 mm) than in the control group (mean value, 3.2 mm). It had 88 % sensitivity and 80 % specificity. Pituitary gland size was decreased in PTC group (mean value, 3.63 mm) than in the control group (mean value, 5.05 mm). It had a sensitivity of 64 % and specificity of 90 %. Posterior globe flattening had 56 % sensitivity and 100 % specificity, intraocular protrusion of the optic nerve had 40 % sensitivity and 100 % specificity, and horizontal tortuosity of the optic nerve had 68 % sensitivity and 83 % specificity. CONCLUSIONS: According to our study, posterior globe flattening, intraocular protrusion of the optic nerve, horizontal nerve sheath tortuosity, optic nerve sheath distension, and decreased pituitary gland size are reliable neuroradiological diagnostic markers for pediatric pseudotumor cerebri.

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.

Gastric signet ring carcinoma in a patient with ataxia-telangiectasia: a case report and review of the literature.

Ataxia-telangiectasia (A-T) is an autosomal recessive primary immunodeficiency disease characterized by progressive cerebellar ataxia, telangiectasia, sinopulmoner recurrent infections, and cancer susceptibility. Individuals with A-T are known to be at increased risk of certain malignancies including leukemia, lymphoma, and breast and gastric cancer. We present an 18-year-old case of A-T with Hashimoto thyroiditis who admitted with complaints of nausea, vomiting, anorexia, and weight loss. An upper endoscopic biopsy revealed gastric signet ring cell carcinoma. To the best of our knowledge, we report the first case of signet ring cell carcinoma in the patient with A-T. Our experience with occurrence of Hashimoto thyroiditis and gastric signet ring cell carcinoma in the same case of A-T underlines that the clinicians handling A-T must be vigilant about both malignancy and autoimmune disorders.

Intracranial hemorrhages and late hemorrhagic disease associated cholestatic liver disease.

Deficiency of vitamin K predisposes to early, classic or late hemorrhagic disease of the newborn (HDN); of which late HDN may be associated with serious and life-threatening intracranial hemorrhage. Late HDN is characterized intracranial bleeding in infants aged 1 week to 6 months due to severe vitamin K deficiency. Late HDN is still an important cause of mortality and morbidity in developing countries where vitamin K prophylaxis is not routinely practiced. Children with cholestatic liver disease are at risk for developing secondary vitamin K deficiency because of fat malabsorbtion and inadequate dietary intake. In this study, we described 11 infants with cholestatic liver disease with different etiologies exhibiting intracranial hemorrhage (ICH). Six patients underwent surgical evacuation of ICH, following the administration of vitamin K and/or fresh frozen plasma. The possibility of cholestatic liver disease should be considered in the treatment of ICH due to vitamin K deficiency.

Neurofibromas of the bladder in a child with neurofibromatosis type 1 causing chronic renal disease.

Neurofibromatosis type 1 (NF1) is an autosomal-dominant inherited disorder and its prominent feature is the neurofibroma and renal involvement includes renal artery stenosis and renal artery aneurysms causing renovascular hypertension. The genitourinary tract may be rarely involved, leading to urinary symptoms of obstruction and hydronephrosis. Herein, we report a 12-year-old boy with chronic renal failure associated with neurofibromas of the bladder, which leads to urinary obstruction.

Polymicrogyria: correlation of magnetic resonance imaging and clinical findings.

AIM: The aim of this study is to evaluate the correlation between clinical presentation and the extent of cortical involvement in patients with polymicrogyria. MATERIALS AND METHODS: The magnetic resonance imaging findings of 26 patients were evaluated for the location and distribution of polymicrogyria. Presence of asphyxia at birth and serological tests for TORCH infections, the presence and type (spastic, flaccid) of motor deficits, mental development,microcephaly, and epilepsy were noted. RESULTS: Nineteen patients had bilateral, whereas seven had unilateral involvement. Patients with unilateral polymicrogyria presented later with milder symptoms. The most encountered symptom in patients with bilateral involvement was mental motor retardation (MMR) (89%) and speech problems (84%).The clinical presentations of patients with asphyxia and positive serological tests for cytomegalovirus (CMV) were worse.All patients with positive serological tests for CMV had bilateral involvement. The perisylvian region was affected in five(71%) patients with unilateral involvement. The most encountered presenting symptom in these patients was epilepsy. Cerebral palsy was seen in three (43%) of the patients, and all of them had left hemiparesis. Microcephaly, MMR, and speech delay were detected in one (14%) of the patients. CONCLUSIONS: Late presenting epilepsy may be a predictor of aunilateral polymicrogyria and is associated with relatively good prognosis. CMV infection and the presence of asphyxia are predictors of worse prognosis.

Computed Tomographic Thickness of Retrobulbar Optic Nerve Is Decreased in Glaucoma Patients and Is Negatively Correlated With Disease Severity.

Purpose The purpose of the study was to quantitatively measure the width of the retrobulbar optic nerve-sheath complex by computed tomography (CT) in glaucoma and evaluate its relationship with optic nerve changes and visual field loss parameters. Methods Sixty-six eyes of 33 patients with bilateral asymmetric glaucomatous optic nerve damage and 20 eyes of 20 age- and sex-matched control subjects without glaucoma were included. Axial retrobulbar optic nerve-sheath complex was measured by CT in the eyes with advanced glaucomatous damage (group 1); in the fellow eyes of the same patients with moderate glaucomatous damage (group 2); and in control subjects (group 3). Measurements were obtained at three different points: just behind the globe, near the optic canal, and in the middle. Results Mean age and sex distribution between groups were comparable (p>0.05). The mean diameters of the retrobulbar optic nerve at three measurement points in group 1 (4.00±0.42mm, 3.49±0.44mm, 3.18±0.45mm) were significantly (for each, p<0.05) lower when compared with the corresponding points of group 2 eyes (4.24±0.41mm, 3.77±0.47nn, 3.47mm±0.44mm) and normal controls (4.58±0.44mm, 4.15±0.45mm, 3.92±0.48mm). Optic disc changes and visual field parameters were negatively correlated with retrobulbar optic nerve diameter (for each, p < 0.05). Conclusion Radiological alterations of the retrobulbar optic nerve in glaucomatous eyes revealed a decreased optic nerve diameter which correlated with disease severity. Optic nerve dimensions below the lower limit for normal individuals may be considered pathologically reduced and, therefore, CT measurements of the retrobulbar nerve may be additive to the traditional triad of raised intraocular pressure, field defects, and optic disc changes in some cases with opaque optic media preventing the fundus examination or with optic nerve anomalies.

Identifying the effects of excess weight, metabolic syndrome and insulin resistance on liver stiffness using ultrasound elastography in children.

BACKGROUND: Metabolic syndrome (MetS) and insulin resistance (IR) are known predictors of nonalcoholic fatty liver disease (NAFLD) which is one of the significant comorbidities of obesity. Obese children with MetS and IR are reported to be more likely to have advanced liver fibrosis compared to those without MetS or IR. The aim of this study is to determine the effects of excess weight, MetS and IR on liver fibrosis assessing liver stiffness in children using ultrasound elastography and compare gray scale ultrasonographic findings of hepatic steatosis (HS) with liver fibrosis. METHODS: The study group involved 131 overweight/obese children. The control group involved 50 healthy lean children. Groups were adjusted according to body mass index (BMI) and BMI-standard deviation scores (SDS). Liver stiffness measurements which are expressed by shear wave velocity (SWV) were performed for each individual. The study group was further subgrouped as children with MetS and without MetS, with IR and without IR. RESULTS: The mean SWV of liver was 1,07 ± 0,12 m/s in the control group and 1,15 ± 0,51 m/s in the study group. The difference was significant (p=0,047). SWV of liver was weakly correlated with age, BMI, BMI-SDS, Homeostatic Model Assessment-Insulin Resistance and high-density lipoprotein cholesterol. The mean SWV of the liver in the study group for children without MetS was 1,1 ± 0,44 m/s, with MetS was 1,23 ± 0,70 m/s. The difference was not significant (p=0,719). The mean SWV of the liver in the study group for children without IR was 1,02 ± 0,29 m/s, with IR was 1,24 ± 0,61 m/s. The difference was not significant (p=0,101). In multivariate regression analysis, the only independent factor affecting liver stiffness was BMI-SDS (OR:2,584, 95% CI: 1,255- 5,318, p=0,010). CONCLUSIONS: Obesity itself, regardless of MetS or IR seems to be the major problem affecting liver stiffness in this study. However, large scale longitudinal studies might clarify this issue.

Intracranial hemorrhage in children with congenital factor deficiencies.

BACKGROUND: Intracranial hemorrhage (ICH) is a life-threatening situation in childhood. Congenital factor deficiencies (CFD) like hemophilia may cause ICH, and ICH may be the initial presentation in some cases. METHODS: From 2000 to 2010, 107 children with CFD from Erciyes University, Faculty of Medicine, Department of Pediatric Hematology, were evaluated. The ICH episodes were identified by medical history, general physical examination, detailed neurological examination, and CT or MR scan. The management strategies, surgical intervention, and outcome were noted. RESULTS: Twenty-one episodes of ICH were seen in 18 patients (16.8%) out of 107 CFD patients. The mean age of the patients was 42.1 months. Fourteen out of 18 patients were male, and four were female. Twelve (57.1%) out of 21 ICH episodes were caused by trauma, and nine (42.9%) were non-trauma related. Epidural hematoma was most frequently observed. All patients survived, but four had decrease in intellectual capacity and motor deficit. CONCLUSION: The optimal management of ICH in children with CFD depends on immediate recognition and prompt replacement therapy to ensure hemostatic balance with adequate surgical intervention.

Evaluation of pneumonia in children: comparison of MRI with fast imaging sequences at 1.5T with chest radiographs.

BACKGROUND: Although there has been a study aimed at magnetic resonance imaging (MRI) evaluation of pneumonia in children at a low magnetic field (0.2T), there is no study which assessed the efficacy of MRI, particularly with fast imaging sequences at 1.5T, for evaluating pneumonia in children. PURPOSE: To investigate the efficacy of chest MRI with fast imaging sequences at 1.5T for evaluating pneumonia in children by comparing MRI findings with those of chest radiographs. MATERIAL AND METHODS: This was an Institutional Review Board-approved, HIPPA-compliant prospective study of 40 consecutive pediatric patients (24 boys, 16 girls; mean age 7.3 years ± 6.6 years) with pneumonia, who underwent PA and lateral chest radiographs followed by MRI within 24 h. All MRI studies were obtained in axial and coronal planes with two different fast imaging sequences: T1-weighted FFE (Fast Field Echo) (TR/TE: 83/4.6) and T2-weigthed B-FFE M2D (Balanced Fast Field Echo Multiple 2D Dimensional) (TR/TE: 3.2/1.6). Two experienced pediatric radiologists reviewed each chest radiograph and MRI for the presence of consolidation, necrosis/abscess, bronchiectasis, and pleural effusion. Chest radiograph and MRI findings were compared with Kappa statistics. RESULTS: All consolidation, lung necrosis/abscess, bronchiectasis, and pleural effusion detected with chest radiographs were also detected with MRI. There was statistically substantial agreement between chest radiographs and MRI in detecting consolidation (k = 0.78) and bronchiectasis (k = 0.72) in children with pneumonia. The agreement between chest radiographs and MRI was moderate for detecting necrosis/abscess (k = 0.49) and fair for detecting pleural effusion (k = 0.30). CONCLUSION: MRI with fast imaging sequences is comparable to chest radiographs for evaluating underlying pulmonary consolidation, bronchiectasis, necrosis/abscess, and pleural effusion often associated with pneumonia in children.

Long-Term Follow-Up of Patients with a Diagnosis of Posterior Reversible Encephalopathy Syndrome.

OBJECTIVE: The essential characteristics of posterior reversible encephalopathy syndrome (PRES) are the presence of acute onset neurologic symptoms, focal vasogenic edema at neuroimaging, and reversible clinical and/or radiologic findings. This study aimed to evaluate the clinical findings, causes, radiologic findings, and prognoses of patients with PRES. METHODS: Patients with PRES confirmed with clinical and radiologic findings by a pediatric neurologist were evaluated retrospectively. RESULTS: Seventeen patients with PRES were evaluated (mean age at onset, 10.23 ± 4.65 years; range, 2-17 years; girls, 29.4% [n = 5]). The mean length of follow-up was 6 ± 2.3 years (range, 3.4-10 years). Mortality due to primary disease occurred in 4 patients (23.5%) during follow-up. PRES was derived from renal diseases in 10 patients (58.8%), hematologic diseases in 6 patients (35.3%), and liver disease in one patient (5.9%). Hypertension was present in 16 patients (94.1%) at onset of PRES (>99th percentile). Seizure, the most frequent initial symptom, was observed in 82.4% (n = 14). Blurred vision and headache were the initial symptoms in 3 patients (17.6%). Sequelae were observed at magnetic resonance imaging (MRI) in 6 patients. Development of epilepsy was determined as a sequela in 4 patients (23.5%) and mental motor retardation in 2 patients (11.8%). CONCLUSION: Epilepsy is uncommon in patients who have recovered from PRES. The presence of gliosis on MRI and interictal epileptic discharges on electroencephalograms are major risk factors for the development of epilepsy. Antiepileptic treatment can be stopped in the early period in patients with normal MRI and electroencephalogram by eliminating the factors that trigger the seizures.

Magnetic resonance imaging of childhood Guillain-Barre syndrome.

OBJECTIVE: To describe the spinal magnetic resonance imaging (MRI) features in children with Guillain-Barre syndrome (GBS) and to investigate the correlation with the clinical/laboratory findings. MATERIAL AND METHODS: Clinical/laboratory findings of 40 children (mean age 5.7 years; range, 3 months-15 years) who had a final diagnosis of GBS were retrospectively reviewed. Clinical severity was graded according to Hughes classification. Electromyogram and cerebrospinal fluid analysis of the patients were recorded. All patients had a contrast-enhanced spinal MRI. The contrast enhancement pattern was determined, and the diameters of anterior and posterior spinal nerve roots were measured. The clinical/laboratory findings were correlated with the MRI findings. RESULTS: Twenty-eight patients had an electromyogram examination, and 25 of them revealed findings consistent with GBS. Cerebrospinal fluid analysis of 37 out of 40 patients showed albumino-cytologic dissociation. All but two patients had thickening and contrast enhancement of the nerve roots and cauda equina on spinal MRI. The most common MRI finding was enhancement of both the anterior and the posterior nerve roots of cauda equina which was prominent anteriorly. The mean anteroposterior diameter of the anterior nerve roots was 2.19 mm (range, 1.38-3.30 mm) and the posterior nerve root was 1.80 mm (range, 1.07-2.97 mm). CONCLUSION: Spinal MRI is a reliable imaging method for the diagnosis of GBS as it was positive in 38 of 40 patients. The severity on MRI does not correlate with severity of the clinical condition. MRI can be used as a supplementary diagnostic modality to clinical and laboratory findings of GBS.