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BACKGROUND AND PURPOSE: There is a paucity of data regarding the incidence of structural brain lesions in children with new-onset unprovoked seizures. Our aim was to determine the frequencies and types of epileptogenic lesions detected on a dedicated epilepsy protocol MR imaging according to age group, the presence of developmental delay, and the number and types of seizures. MATERIALS AND METHODS: Consecutive children between 6 months and 18 years of age with new-onset unprovoked seizures were included. The frequencies and types of epileptogenic lesions were determined and then stratified according to sex, age groups, the presence of developmental delay, and the number and types of seizures at presentation. Multivariate analysis was used to identify variables significantly associated with the presence of epileptogenic lesions. RESULTS: One thousand children were included. An epileptogenic lesion was identified in 26%, with malformations of cortical development being the most common lesion (32%), followed by hypoxic-ischemic injury (20%) and vascular etiologies (16%). Univariate analysis showed a significant increase in the frequency of epileptogenic lesions with decreasing age, the presence of developmental delay, and the number and types of seizures at presentation. The presence of developmental delay and seizure type at presentation remained significant in a multivariate analysis. CONCLUSIONS: We documented a relatively high rate of epileptogenic lesions in children with new-onset seizures, with the presence of developmental delay and specific seizure types being associated with a higher likelihood of detecting an epileptogenic lesion on neuroimaging. This study fulfills the requirements of the study design recommended by the Practice Committee of the American Academy of Neurology, and we hope that our results will assist the relevant societies and committees in formulating neuroimaging guidelines for children with new-onset seizures.

Objective To determine the incidence of pituitary incidentalomas in the paediatric population and among its different age subgroups as well as to identify the characteristics of these lesions. Additionally, we aim to give a perspective on the management and follow-up of these patients. Design and patients We retrospectively studied MRI of children aged 18 years or below who underwent MRI with sellar region within their field of view between January 2010 and December 2018. Measurements Pituitary lesions were considered incidental according to the definition by the Endocrine Society. We reported the size, location and signal characteristics of each lesion. Medical charts of the subjects were reviewed for age, sex, the MRI indication and the hormonal assays levels. Results We identified 40 pituitary lesions of which 31 were incidental lesions. The incidence of pituitary incidentaloma in our cohort was 22 per 1000 patients with female predisposition ( 64.5%) and a mean age of 11 ± 6 years. Rathke's cleft cyst was the most prevalent lesion, accounting for 67.7% followed by cystic pituitary lesions and microadenomas. The most common indications for imaging were growth disturbance (12.9%) followed by headache (9.7%). Abnormal laboratory workup was present in 13% of the subjects. Incidental lesions were more common in the older age groups compared to young children. Conclusion Incidental pituitary lesions in the paediatric population are relatively infrequent and increases with age. Rathke's cleft cyst is the most common incidentally encountered pituitary lesion followed by cystic pituitary lesions and microadenomas.

Previous studies on possible accumulation of gadolinium-based contrast agents (GBCA) in the brain suggest that macrocyclic GBCA are less likely to accumulate than linear GBCA. However, conflicting results have been reported, especially in MS. The aim of this study is to investigate retrospectively the correlation between gadoterate-meglumine (macrocyclic GBCA) use and T1 signal intensity changes (SI) in the dentate nucleus and the GP on unenhanced T1-weighted images in a large cohort of MS patients.Unenhanced T1-weighted images of 232 MS patients who previously received multiple intravenous administrations of 0.1 mmol/kg of gadoterate-meglumine were reviewed. The change in T1 SI ratios of dentate nucleus/central pons (DN/CP) and globus pallidus/centrum semiovale (GP/CSO) was calculated between the first and last MRIs and correlated with age, number of injections, time interval between MRIs, disease duration, activity, and therapy.DN/CP ratio showed no significant changes whereas the GP/CSO ratio showed a significant decrease (p0.0001) between the first and last MRIs. Multivariable analyses of both ratios, controlling for age, disease duration, and time interval between MRIs, showed no significant correlation between the number of gadolinium injections and the differences in DN/CP (standardized beta = -0.018, p = 0.811) or GP/CSO SI ratios (standardized beta = -0.049, p = 0.499).Repeated administration of gadoterate-meglumine in MS patients did not result in increased T1 SI in the DN or the GP. The significant decrease of GP/CSO ratio between the first and last MRIs is not due to gadolinium accumulation but rather to varying MR parameters.

The regular administration of intrathecal chemotherapy has significantly reduced the risk of central nervous system leukemia in patients with acute lymphoblastic leukemia. We report the case of 28-year-old man who developed intrathecal methotrexate induced myelopathy; a rare but serious side effect of intrathecal chemotherapy. In the light of absent effective treatment strategies, description of the case, along with reviewing similar cases published in the literature will help shed a light on the possible pathophysiologic mechanisms behind this injury. To this date, there are no specific clinical, biochemical and imaging signs that would allow timely detection of intrathecal methotrexate induced myelopathy. This in turn is causing delayed treatment of this injury, resulting in significant morbidity and mortality.

Obstructive sleep apnoea (OSA) is a common condition usually treated with continuous positive airway pressure (CPAP). No reports have linked it to an acute subdural haematoma. A 54-year-old white man who had hypertension well controlled with an angiotensin II receptor blocker, presented with a 2-week history of occipital headache with no other focal neurological symptoms. The headache began 12 days after he had started using CPAP for OSA. A brain MRI performed 2 weeks later showed bilateral subdural haematomas which were chronic on the left and sub-acute/acute on the right. Since the patient was clinically stable with no focal neurological deficits, he received prednisone for 3 weeks and was followed up with consecutive CT scans demonstrating gradual regression of the haematomas. This is the first report showing that subdural haematomas could be linked to CPAP use. LEARNING POINTS Primary care physicians, pulmonologists and neurologists should be alert for unexplained headache in a patient on continuous positive airway pressure (CPAP). Subdural haematoma may be a rare reported side effect of CPAP use. Subdural haematoma in a stable patient with no focal neurological deficits can be treated conservatively with close monitoring and follow-up.

Background Diagnoses of thalamic atrophy in children are based on experts’ judgments. No normative measures exist for aiding objective diagnoses. Our aim was to determine normative two-dimensions(2D) and volume measurements of the thalamus in normally developing children. Methods MRI images of 245 patients were retrospectively collected. Only participants with normal brain MRIs were included in this cross-sectional study. Anterior-posterior (AP), transverse (T), and craniocaudal (C) diameters were measured. Volumetric masks of the thalamus were manually drawn, whereas volumetric measurements of the brain were automated. Results 124 patients were male (50.6%). We tabulated our measurements from birth until 18 years old. No significant differences in the thalamus measurements are found between the two hemispheres nor between sexes. The most remarkable increase in the thalamus volume and AP dimension is noted in the first four years of life, following which the values seem to stabilize. Craniocaudal diameters seem to increase in the first year of life, whereas transverse diameters increase until the age of 14 before plateauing. Conclusion We report normative values of the thalamus in 2D and 3D from birth until 18 years of age. A rapid increase in the thalamic size is noted during the first four years of life followed by stabilization.

Contains fulltext : 206714.pdf (Publisher’s version ) (Open Access) Objective: This pilot study aims to identify white matter (WM) tract abnormalities in Autism Spectrum Disorders (ASD) toddlers and pre-schoolers by Diffusion Tensor Imaging (DTI), and, to correlate imaging findings with clinical improvement after early interventional and Applied Behaviour Analysis (ABA) therapies by Verbal Behaviour Milestones Assessment and Placement Program (VB-MAPP). Methods: DTI scans were performed on 17 ASD toddlers/pre-schoolers and seven age-matched controls. Nine ASD patients had follow-up MRI twelve months following early intervention and ABA therapy. VB-MAPP was assessed and compared at diagnosis, six and twelve months after therapies. Tract-Based Spatial Statistics (TBSS) was used to measure fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial (RD) diffusivity Results: VB-MAPP scores improved at six and twelve months after early intervention and ABA therapy compared to scores at baseline. TBSS analysis showed significant FA decrease and/or RD increase in ASD patients before therapy versus controls in inferior fronto-occipital fasciculi, uncinate fasciculi, left superior fronto-occipital fasciculus, forceps minor, left superior fronto-occipital fasciculus, right superior longitudinal fasciculus, corona radiata bilaterally and left external capsule. A significantly FA increase in 21 tracts and ROIs is reported in post- versus pre-therapy DTI analysis. Conclusion: DTI findings highlighted ASD patient WM abnormalities at diagnosis and confirmed the benefits of twelve months of early intervention and ABA therapy on clinical and neuro imaging outcomes.

Background and purpose The aim of this prospective study is to investigate and evaluate in clinical practice the diagnostic impact of 3DFLAIR in regards to 2DT2/PD in terms of infratentorial lesions detection in multiple sclerosis (MS). Material and methods 164 MS patients from the OFSEP database were reviewed retrospectively. MR examinations were performed on 1.5T or 3T systems from four different centers. Infratentorial lesions were counted and allocated to different regions of the posterior fossa by three raters independently (junior resident, resident with an expertise in neuroradiology, and senior neuro-radiologist) on the 3DFLAIR and 2DT2/PD. Both sequences do not have the same spatial resolution but reflect what is recommended by most of the consensus and done in clinical practice. Results With an overall number of 528 for Rater-1 and 798 for Rater-2 infratentorial lesions, 3DFLAIR had a significantly higher number of lesions detected than 2DT2/PD (303 for Rater-1 and 370 for Rater-2). The prevalence of trigeminal lesions detected by using 3DFLAIR was also significantly higher than 2DT2/PD. ROC analysis showed 3DFLAIR to be more specific and sensitive than 2DT2/PD. An overall difference between all three Raters has been observed. The more the Rater is experienced the more lesions he detects. Conclusion Along with the radiologist ability to detect lesions based on his level of experience, the OFSEP optimized 3DFLAIR can significantly improve infratentorial lesion detection in MS compared to 2DT2/PD. This is important in MS follow-up that takes into account new lesions number to adapt patients’ treatment.

Radiation-induced medulloblastoma is exceedingly rare. Four cases of adult medulloblastoma were previously reported to have occurred following whole brain or craniospinal irradiation. Development of systemic malignancies secondary to proton beam therapy is extremely uncommon, with a lifetime risk lower than 1 %. A 39-year-old woman presented with gait instability and a right cerebellar mass following an 8-year latency period post-fractionated proton therapy for an acoustic neuroma. To date, no cases of new onset medulloblastoma have been reported following proton beam therapy. The differential diagnosis by imaging studies includes brain radiation necrosis. The current report highlights the possible, although rare, risks and complications associated with proton beam radiation. Further studies are needed to address issues related to the management and treatment of radiation-induced medulloblastomas.

Pediatric ischemic stroke still represents a burden, and more than half of the survivors will experience cognitive or motor disabilities. The objective of this study was to investigate the role of thrombophilia in a cohort of children with arterial ischemic stroke. The records of infants and children with clinically and radiologically confirmed stroke were reviewed. Patients with venous or perinatal stroke were not included. Thirty-three patients were diagnosed with arterial ischemic stroke. The male/female ratio was 1.75:1. The median age was 4 years. The most frequent clinical manifestations were hemiparesis (54.5%) and seizures (33.3%). Genetic thrombophilia testing was available on 24 patients. Nine of the 24 patients (37.5%) were heterozygous for factor V Leiden. None of the patients carried the factor II G20210A variant. Ten patients (41.7%) were heterozygous and 3 (12.5%) were homozygous for methylenetetrahydrofolate reductase (MTHFR) C677T variant. Fifteen patients (62.5%) had one or more genetic polymorphism. Factor V Leiden was significantly associated with arterial ischemic stroke (P < 0.001). Stroke recurred in 2 children with multiple risk factors and MTHFR C677T mutation. Factor V Leiden is one of the major genetic risk factors for pediatric arterial ischemic stroke in Lebanon. MTHFR C677T was prevalent among patients with recurrent stroke.

We explore the safety, and therapeutic benefit of intrathecal injection of ex-vivo expanded autologous bone marrow derived mesenchymal stem cells (BM-MSCs) in 10 patients with advanced multiple sclerosis (MS). Patients were assessed at 3, 6 and 12 months. Assessment at 3-6 months revealed Expanded Disability Scale Score (EDSS) improvement in 5/7, stabilization in 1/7, and worsening in 1/7 patients. MRI at 3 months revealed new or enlarging lesions in 5/7 and Gadolinium (Gd+) enhancing lesions in 3/7 patients. Vision and low contrast sensitivity testing at 3 months showed improvement in 5/6 and worsening in 1/6 patients. Early results show hints of clinical but not radiological efficacy and evidence of safety with no serious adverse events.

Background: Endoscopic third ventriculostomy (ETV) has gained wide popularity as a first-line treatment for obstructive hydrocephalus (OHC). We have been performing ETV since 1998. We report our experience with this technique in the management of OHC. Methods: Between 1998 and 2007, we performed 49 ETV procedures in 46 patients suffering from OHC. Medical records were retrospectively reviewed. Success was defined as shunt-free survival. Results: There were 29 males and 17 females with a mean age of 23 years (6 months–65 years). Aqueductal stenosis and tectal tumor were the most common etiologies (63%). Seven patients (15.6%) had early ETV failure. Of 38 patients with initial success and available follow-up, shunt independence was achieved in 29 patients (76.3%) after a mean follow-up of 37 months. Kaplan-Meier analysis yielded a 70% 5-year shunt-free survival rate. On multivariate analysis, no variables could predict early or late ETV failure. Transient complications occurred in 6 patients (13%), but there were no ETV-related deaths or permanent morbidity. Conclusion: ETV is a safe and effective treatment for OHC, resulting in a high rate of long-term shunt independence with a low risk of complications. ETV should be considered the treatment of choice for patients with OHC and its development as a substitute to shunt placement should be encouraged in neurosurgically developing countries.

Cervical aortic arch (CAA) is a rare congenital aortic anomaly. It can be associated with several cardiovascular abnormalities including aortic aneurysm, valvular disease, and pseudocoarctation. In this report, we present a case of CAA with combined association of both aneurysm formation and pseudocoarctation. This combination of anomalies is extremely uncommon, described in only 4 cases in the literature. In addition, our patient had an anomalous subaortic left brachiocephalic vein that, to our knowledge, was not previously described in the literature to be associated with CAA. The radiographic, multidetector computed tomography scan, and magnetic resonance angiography imaging findings are presented.

Hereditary spastic paraplegia (HSP) is a heterogeneous group of hereditary disorders first described by Strumpell in 1880 characterized by degeneration of the corticospinal tracts and posterior columns of the spinal cord. In spite of the advances in our clinical and genetic understanding of HSP's, the radiological correlates of the disease were never defined. Most of the studies reporting MRI findings, describe non-specific brain abnormalities and spinal cord atrophy. We report two identical twins sisters with the diagnosis of HSP in whom MRI of the spinal cord revealed posterior displacement of the central ependymal canal suggestive of posterior column atrophy.

Antunes NL, 2003, PEDIATR NEUROL, V28, P190, DOI 10.1016-S0887-8994(02)00508-8; Bartynski WS, 2001, AM J NEURORADIOL, V22, P1901; Bartynski WS, 2006, AM J NEURORADIOL, V27, P2179; Bushara KO, 1997, PEDIATR NEUROL, V17, P185, DOI 10.1016-S0887-8994(97)00079-9; CHATURVEDI S, 1994, STROKE, V25, P1215; Chen CY, 1996, AM J NEURORADIOL, V17, P295; Chou Mei-Chun, 2004, Kaohsiung Journal of Medical Sciences, V20, P381; Delanty N, 1997, NEUROLOGY, V49, P686; Duru F, 1997, LEUKEMIA LYMPHOMA, V26, P377; Ferraz-Filho JR, 2006, ARQ NEURO-PSIQUIAT, V64, P718, DOI 10.1590-S0004-282X2006000500003; FLEISCHHACK G, 1994, KLIN PADIATR, V206, P334, DOI 10.1055-s-2008-1046626; GUGLIOTTA L, 1992, EUR J HAEMATOL, V49, P63; Hinchey J, 1996, NEW ENGL J MED, V334, P494, DOI 10.1056-NEJM199602223340803; Ito Y, 1998, AM J NEURORADIOL, V19, P415; Kieslich M, 2003, J PEDIAT HEMATOL ONC, V25, P484, DOI 10.1097-00043426-200306000-00011; Kinoshita T, 2003, CLIN IMAG, V27, P307, DOI 10.1016-S0899-7071(02)00592-2; Larson RA, 1998, LEUKEMIA, V12, P660, DOI 10.1038-sj.leu.2401007; Lee JH, 2000, CLIN NEUROL NEUROSUR, V102, P33, DOI 10.1016-S0303-8467(99)00076-1; Morris EB, 2007, PEDIATR BLOOD CANCER, V48, P152, DOI 10.1002-pbc.20703; NAKASU Y, 1995, AM J NEURORADIOL, V16, P1185; Narbone MC, 2006, NEUROL SCI, V27, P187, DOI 10.1007-s10072-006-0067-y; Nomura K, 1995, Rinsho Shinkeigaku, V35, P64; Norman JK, 2007, PEDIATR BLOOD CANCER, V49, P198, DOI 10.1002-pbc.20597; NowakGottl U, 1996, EUR J HAEMATOL, V56, P35; OHNUMA T, 1970, CANCER RES, V30, P2297; Osborn A, 2004, DIAGNOSTIC IMAGING B; Rangi PS, 2005, NEURORADIOLOGY, V47, P586, DOI 10.1007-s00234-005-1376-6; Rathi B, 2002, PEDIATR NEUROSURG, V37, P203, DOI 10.1159-000065395; Rogers LR, 2003, NEUROL CLIN, V21, P167, DOI 10.1016-S0733-8619(02)00066-X; Sanchez-Carpintero R, 2001, PEDIATR NEUROL, V24, P145, DOI 10.1016-S0887-8994(00)00242-3; SCHWARTZ RB, 1995, AM J ROENTGENOL, V165, P627; Shin RK, 2001, NEUROLOGY, V56, P388; SIBAI BM, 1949, AM J OBSTET GYNECOL, V163, P1054; Suminoe A, 2003, J PEDIAT HEMATOL ONC, V25, P236, DOI 10.1097-00043426-200303000-00010; VAUGHN DJ, 1993, AM J NEURORADIOL, V14, P1014

The aim of this chapter is to describe the neuroimaging findings of neurobrucellosis. Neurobrucellosis can present with different clinical and neuroradiological signs. The imaging findings are variable and mainly divided into four categories: normal imaging, inflammatory changes in the brain and meninges, white matter changes, and vasculopathic lesions. The different radiological patterns of neurobrucellosis will be discussed thoroughly on CT and MRI. Knowledge of the multiple radiologic findings of neurobrucellosis will enable radiologists and neurologist to include neurobrucellosis in the differential diagnosis and may allow reliable diagnosis.

Alter BP, 1996, AM J HEMATOL, V53, P99, DOI 10.1002-(SICI)1096-8652(199610)53:299::AID-AJH73.0.CO;2-Z; Alter BP, 2007, J MED GENET, V44, P1, DOI 10.1136-jmg.2006.043257; Alter BP, 2003, CANCER, V97, P425, DOI 10.1002-cncr.11046; AUERBACH AD, 1993, EXP HEMATOL, V21, P731; BUTTURINI A, 1994, BLOOD, V84, P1650; CERVENKA J, 1981, PEDIATRICS, V67, P119; DECHADAREVIAN JP, 1985, ARCH PATHOL LAB MED, V109, P367; Frappart PO, 2007, EMBO J, V26, P2732, DOI 10.1038-sj.emboj.7601703; Hirsch B, 2004, BLOOD, V103, P2554, DOI 10.1182-blood-2003-06-1970; Joenje H, 2001, NAT REV GENET, V2, P446, DOI 10.1038-35076590; Rosenberg PS, 2003, BLOOD, V101, P822, DOI 10.1182-blood-2002-05-1498; Ruud E, 2001, ACTA PAEDIATR, V90, P580, DOI 10.1080-080352501750197764

To prospectively evaluate the accuracy of magnetic resonance (MR) imaging for the preoperative diagnosis of deep pelvic endometriosis and extension of the disease.One hundred ninety-five patients (mean age, 34.2 years; range, 20-71 years) who were suspected of having pelvic endometriosis were recruited at two institutions. Two experienced radiologists evaluated the MR images independently. Deep pelvic endometriosis was defined as implants or tissue masses that appeared as hypointense areas and/or hyperintense foci on T1- or T2-weighted MR images in the following locations: torus uterinus, uterosacral ligaments (USLs), vagina, rectovaginal septum, rectosigmoid, and bladder. MR imaging results were compared with surgical and pathologic findings. Sensitivity, specificity, predictive values, and accuracy of MR imaging for prediction of deep pelvic endometriosis were assessed.Pelvic endometriosis was confirmed at pathologic examination in 163 (83.6%) of 195 patients. Endometriomas, peritoneal lesions, and deep pelvic endometriosis were diagnosed on the basis of surgical findings, alone or combined with pathologic findings, in 111 (68.1%), 83 (50.9%), and 103 (63.2%) of 163 patients, respectively. Torus uterinus and USL were the most frequent sites of deep pelvic endometriosis. The sensitivity, specificity, positive and negative predictive values, and accuracy of MR imaging for deep pelvic endometriosis were 90.3% (93 of 103), 91% (84 of 92), 92.1% (93 of 101), 89% (84 of 94), and 90.8% (177 of 195), respectively. The sensitivity, specificity, and accuracy, respectively, of MR imaging for the diagnosis of endometriosis in specific sites were as follows: USL, 76% (57 of 75), 83.3% (100 of 120), and 80.5% (157 of 195); vagina, 76% (16 of 21), 95.4% (166 of 174), and 93.3% (182 of 195); rectovaginal septum, 80% (eight of 10), 97.8% (181 of 185), and 96.9% (189 of 195); rectosigmoid, 88% (53 of 60), 97.8% (132 of 135), and 94.9% (185 of 195); and bladder, 88% (14 of 16), 98.9% (177 of 179), and 97.9% (191 of 195).MR imaging demonstrates high accuracy in prediction of deep pelvic endometriosis in specific locations.

Objective To determine the accuracy of transvaginal sonography (TVS) for the diagnosis of deep pelvic endometriosis. Methods In a prospective study, 142 women with clinical signs of endometriosis underwent TVS followed by surgical and histopathological investigations. The presence and extent of endometriosis involving the uterosacral ligaments, vagina, rectovaginal septum, intestines, bladder and ovaries shown by TVS were compared with surgical and histological findings. The sensitivity, specificity, predictive values and accuracy of TVS for predicting deep pelvic endometriosis were assessed. Results Ovarian and deep pelvic endometriosis were found by surgery and histology in respectively 83 (58.5%) and 79 (55.6%) of the 142 patients. The sensitivity, specificity, and positive and negative predictive values of TVS for the diagnosis of deep pelvic endometriosis were 78.5%, 95.2%, 95.4% and 77.9%, respectively. The sensitivity and specificity of TVS for endometriotic involvement of the uterosacral ligaments, vagina, rectovaginal septum and intestines were 70.6% and 95.9%, 29.4% and 100%, 28.6% and 99.3%, and 87.2% and 96.8%, respectively. The sensitivity and specificity of TVS for bladder involvement were 71.4% and 100%, respectively. Conclusion TVS accurately diagnoses intestinal and bladder endometriosis, but is less accurate for uterosacral, vaginal and rectovaginal septum involvement. Copyright © 2004 ISUOG. Published by John Wiley & Sons, Ltd.

Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that arise most commonly in the pleura but have been increasingly reported in extrapleural sites. The authors report the case of an SFT of the scalp that manifested as an enlarging scalp mass in a 2-year-old boy. The mass was surgically excised. Histological examination showed it to be composed of fusiform cells of variable cellularity with a central hyalinization zone. Immunohistochemical staining was positive for CD34 and negative for smooth muscle actin, S100 protein, desmin, and h-caldesmon. On 1-year follow-up, there was no recurrence of the mass. This case presented some diagnostic difficulty because of the wide range of possible diagnoses for a pediatric scalp mass; however, the distinct immunohistochemical profile helped to eliminate other more frequent fibrous tumors of the scalp. The behavior of scalp SFTs is usually benign, but their course can be unpredictable, and recognition of these lesions is essential.

The authors report a case of intraspinal neurenteric cyst in a 22-month-old child, who presented with acute paraplegia following a vesicourethrogram. Despite 8 days' delay in surgical decompression, he made a complete neurological recovery. Neurenteric cysts are rare congenital lesions of the spinal canal lined with an epithelium of endodermal origin. They are usually located at the cervicothoracic junction and present with progressive mild to moderate signs of myelopathy. This is a unique case in regard both to its clinical presentation and to the excellent outcome after 8 days of complete paraplegia.

We herein describe a 16-year-old child with acute lymphoblastic leukemia and acute pancreatitis who developed Wernicke's encephalopathy secondary to prolonged total parenteral nutrition (TPN) that lacked vitamin B1 supplementation. The patient showed a direct and complete response to thiamine therapy. Diagnostic challenges are discussed and recommendations for prophylactic vitamin B1 supplementation in children with leukemia who are placed on TPN are made.

We present the case of an 11-year-old boy with humoral immunodeficiency on monthly intravenous immunoglobulins (IVIG) infusions, evaluated for recurrent, brief, neurological deficits secondary to cerebral sinus thrombosis without any identifiable hypercoagulability state. Etiologic possibilities for the thrombotic event are presented with special discussion of IVIG-related cerebral thrombosis. To the best of our knowledge, our patient represents the first reported case of Bruton's disease on IVIG therapy developing a cerebral ischemic event and the second reported case of cerebral sinus thrombosis associated with IVIG use for any disease. Potential concerns regarding the risk of cerebral thrombosis during IVIG therapy in this and other disorders are reviewed.

A 30-year-old man who developed acute blurred vision in the right eye and right periocular pain on eye movement proved to have a bilateral optic neuropathy and imaging evidence suggestive of bilateral optic nerve sheath meningiomas. This is an unusual presentation for optic nerve meningioma but a reminder that this entity may mimic optic neuritis.

Hydrocephalus resulting from bilateral thrombosis of the jugular veins has been rarely described in the literature. We report the case of an 18-month-old girl who was diagnosed with this condition following total parenteral nutrition. We also review the literature relevant to this subject and discuss the pathophysiology of hydrocephalus in the setting of bilateral jugular venous thrombosis.

Fibroblastic and myofibroblastic tumors of the head and neck are a heterogeneous group of disorders characterized by the proliferation of fibroblasts, myofibroblasts, or both. These tumors may be further subclassified on the basis of their behavior as benign, intermediate with malignant potential, or malignant. There are different types of fibroblastic and myofibroblastic tumors that can involve the head and neck including desmoid-type fibromatosis, solitary fibrous tumor, myofibroma/myofibromatosis, nodular fasciitis, nasopharyngeal angiofibroma, fibrosarcoma, dermatofibrosarcoma protuberans, fibromatosis coli, inflammatory myofibroblastic tumor, ossifying fibroma, fibrous histiocytoma, nodular fasciitis, fibromyxoma, hyaline fibromatosis and fibrous hamartoma. Although the imaging characteristics of fibroblastic and myofibroblastic tumors of the head and neck are nonspecific, imaging plays a pivotal role in the noninvasive diagnosis and characterization of these tumors, providing information about the constitution of tumors, their extension and invasion of adjacent structures. Correlation with the clinical history may help limit the differential diagnosis and radiologists should be familiar with the imaging appearance of these tumors to reach an accurate diagnosis.

Acute appendicitis is one of the most common conditions requiring emergency surgery. However, acute appendicitis presenting with left lower quadrant abdominal pain is extremely rare. Imaging, particularly CT , plays an important role in establishing an accurate and prompt diagnosis, as delay in diagnosis may occur due to lack of uniformity in the clinical signs and symptoms. We report a rare case of a 10-year-old boy who presented with persistent left lower quadrant pain of several days duration, in which the CT scan of the abdomen and pelvis was essential in establishing the correct diagnosis. The malpositioned inflamed appendix was clearly identified in the left side of the abdomen, with the characteristic CT findings of uncomplicated intestinal malrotation. Left-sided acute appendicitis should be considered in the differential diagnosis of young patients presenting with left lower quadrant pain, in order to avoid delay in diagnosis and guide the surgical intervention.

Traumatic eye injuries are associated with significant visual impairment and are a major cause of monocular blindness. Rapid assessment and examination following trauma to the eye is crucial. A thorough knowledge of potential injuries is imperative to ensure rapid diagnosis, to prevent further damage to the eye, and to preserve visual capacity. One consequence of ocular trauma is cataract formation. We report a case and image of a 5-year-old boy who presented after sustaining a blunt head trauma. CT scan of the head revealed a markedly hypodense left eye lens.

To report a rare case of bilateral superior semicircular canal dehiscence (SCCD) in a child. Case report, 11-year-old female patient. Descriptive case report. Audiological findings of bilateral symmetrical low frequency sensorineural hearing loss with ascending curves and bilateral superior semicircular canal dehiscence on a high resolution computed tomography (CAT) scan. The young child presented with bilateral fluctuating hearing loss with no vestibular symptoms. She was found to have bilateral superior semicircular canal dehiscence. We hereby present a rare case of bilateral semicircular canal dehiscence found incidentally on high resolution CAT scan in a young child during work up for bilateral sensorineural hearing loss.

Objective To report a case of PHACE syndrome— Posterior fossa brain abnormalities, Hemangioma (usually facial), Arterial anomalies, Coarctation of the aorta along with cardiac defects, and Eye abnormalities—in a 16-yearold female patient with a port-wine stain of the right periorbital area present since birth in conjunction with hypoplasia of the contralateral internal carotid artery. Methods Thyroid-stimulating hormone, free thyroxine, and growth hormone (GH) levels were measured, and insulin-induced hypoglycemia and arginine infusion tests were done. Radiologic investigations included sagittal enhanced T1-weighted magnetic resonance imaging of the brain and the pituitary gland as well as computed tomography and magnetic resonance angiography of the head and neck. Results The patient had a normal karyotype. Her height and weight were below the 5th percentile for her chronologic age, and she had amenorrhea. Laboratory investigations revealed both thyroid and GH deficiencies and confirmed the diagnosis of hypogonadotropic hypogonadism. The imaging studies showed a right intraorbital hemangioma as well as an enhancing mass in the right internal auditory canal at the cerebellopontine angle, consistent with a posterior fossa hemangioma. Initiation of both thyroid and GH replacement therapy improved her growth rate and yielded a good clinical outcome. Conclusion In patients with facial or neck hemangiomas, PHACE syndrome should be suspected, and brain imaging and cardiac, ophthalmologic, and endocrinologic evaluations are recommended to screen for other potential PHACE abnormalities. (Endocr Pract. 2010;16:255-259)

BACKGROUND AND PURPOSE: Hereditary spastic paraplegia (HSP) is a disorder characterized by degeneration of the corticospinal tracts and posterior column of the spinal cord. Previously described radiologic findings included nonspecific brain abnormalities such as brain atrophy and white matter lesions, as well as atrophy of the spinal cord. In our study, we aimed to better characterize brain and spine MR imaging findings in a series of patients with HSP. MATERIALS AND METHODS: Nine patients from 4 different Lebanese families with the autosomal recessive form of HSP were included in the study. All patients underwent brain and whole-spine MR imaging. We assessed the presence of white matter abnormalities mainly along the corticospinal tracts, brain atrophy, thinning of the corpus callosum, and the presence of spinal cord atrophy or abnormal signal intensity. RESULTS: Imaging revealed mild brain atrophy (44%), atrophy of the corpus callosum (55%), white matter lesions (67%), abnormal T2 high signal intensity in the posterior limb of the internal capsule (55%), and mild spinal cord atrophy (33%). CONCLUSIONS: The MR imaging findings of HSP are nonspecific and variable; however, the most prominent features include atrophy of the corpus callosum, T2 signal intensity in the posterior limb of the internal capsule, and spinal cord atrophy.

Noninvasive diagnosis of brain lesions is important for the correct choice of treatment. Our aims were to investigate whether 1) proton MR spectroscopic imaging ((1)H-MRSI) can aid in differentiating between tumors and nonneoplastic brain lesions, and 2) perfusion MR imaging can improve the classification.We retrospectively examined 69 adults with untreated primary brain lesions (brain tumors, n = 36; benign lesions, n = 10; stroke, n = 4; demyelination, n = 10; and stable lesions not confirmed on pathologic examination, n = 9). MR imaging and (1)H-MRSI were performed at 1.5T before biopsy or treatment. Concentrations of N-acetylaspartate (NAA), creatine (Cr), and choline (Cho) in the lesion were expressed as metabolite ratios and were normalized to the contralateral hemisphere. Dynamic susceptibility contrast-enhanced perfusion MR imaging was performed in a subset of patients (n = 32); relative cerebral blood volume (rCBV) was evaluated. Discriminant function analysis was used to identify variables that can predict inclusion in the neoplastic or nonneoplastic lesion groups. Receiver operator characteristic (ROC) analysis was used to compare the discriminatory capability of (1)H-MRSI and perfusion MR imaging.The discriminant function analysis correctly classified 84.2% of original grouped cases (P.0001), on the basis of NAA/Cho, Cho(norm), NAA(norm), and NAA/Cr ratios. MRSI and perfusion MR imaging had similar discriminatory capabilities in differentiating tumors from nonneoplastic lesions. With cutoff points of NAA/Choor =0.61 and rCBVor =1.50 (corresponding to diagnosis of the tumors), a sensitivity of 72.2% and specificity of 91.7% in differentiating tumors from nonneoplastic lesions were achieved.These results suggest a promising role for (1)H-MRSI and perfusion MR imaging in the distinction between brain tumors and nonneoplastic lesions in adults.

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder consisting of external ophthalmoplegia, retinitis pigmentosa, ataxia and heart block. Magnetic resonance imaging (MRI) shows abnormal T2 high signal intensity in the deep gray matter nuclei, the cerebellar and the subcortical white matter. We report an unusual MR pattern of KSS, where the T2 images revealed radially oriented, hypointense stripes in hyperintense white matter, a characteristic MRI pattern of lysosomal disease not previously reported in KSS.

Purpose To investigate whether in vivo proton magnetic resonance spectroscopic imaging (MRSI) can differentiate between 1) tumors and nonneoplastic brain lesions, and 2) high- and low-grade tumors in children. Materials and Methods Thirty-two children (20 males and 12 females, mean age = 10 ± 5 years) with primary brain lesions were evaluated retrospectively. Nineteen patients had a neuropathologically confirmed brain tumor, and 13 patients had a benign lesion. Multislice proton MRSI was performed at TE = 280 msec. Ratios of N-acetyl aspartate/choline (NAA/Cho), NAA/creatine (Cr), and Cho/Cr were evaluated in the lesion and the contralateral hemisphere. Normalized lesion peak areas (Chonorm, Crnorm, and NAAnorm) expressed relative to the contralateral hemisphere were also calculated. Discriminant function analysis was used for statistical evaluation. Results Considering all possible combinations of metabolite ratios, the best discriminant function to differentiate between nonneoplastic lesions and brain tumors was found to include only the ratio of Cho/Cr (Wilks' lambda, P = 0.012; 78.1% of original grouped cases correctly classified). The best discriminant function to differentiate between high- and low-grade tumors included the ratios of NAA/Cr and Chonorm (Wilks' lambda, P = 0.001; 89.5% of original grouped cases correctly classified). Cr levels in low-grade tumors were slightly lower than or comparable to control regions and ranged from 53% to 165% of the control values in high-grade tumors. Conclusion Proton MRSI may have a promising role in differentiating pediatric brain lesions, and an important diagnostic value, particularly for inoperable or inaccessible lesions. J. Magn. Reson. Imaging 2006. Published 2005 Wiley-Liss, Inc.