Posters - esmrn 2015

Posters
Poster discussion will take place 15th May, between 12.40 and 13.50.
Presenters are asked to be available near their posters during that time.
ESMRN 2015
1
Concurrence among abnormal MRI scans, pathological EEG and intellectual disability in autism spectrum disorder.
F. Piras 1, C. Masala 4, M. Carta 1, D. Serra 1, G. Bitti 3, M.T. Peltz 3, R. Fadda 2, G. Doneddu 1.
1
Center for Pervasive Developmental Disorders, AOB, Cagliari, Italy. 2 Department of Pedagogy, Psychology and Philosophy, University of Cagliari, Italy. 3 Department of Diagnostic Imaging, AOB, Cagliari, Italy. 4 Department of Biomedical Sciences, Section of
Physiology, University of Cagliari, Monserrato (CA), Italy.
Introduction: Abnormal MRI scans and pathological EEG are frequent in Autism Spectrum Disorders (ASD), especially
with Intellectual Disability (ID). However, little is still known about the concurrence between ID, pathological EEG and
abnormal MRI scans in ASD. Our study aimed to evaluate abnormal MRI scans, pathological EEG and their relation with
ID in individuals with non-syndromic ASD.
Methods: MRI scans and EEG investigations were studied in 100 children and adolescents. Based on recommended clinical screenings, we excluded patients with infectious, metabolic or genetic diseases, or any other neurological symptoms.
Mean chronological age was (2-24 years old). EEG investigations were recorded according to 10-20 system during sleep
and MRI scans were acquired with a 1.5-Tesla Signa GE.
Results: 68% of individuals had ID, while 32% had typical intellectual development. Moreover, 45% of individuals
showed pathological EEG and 46% had abnormal MRI scans. The most frequent MRI abnormalities were cortical dysplasia/atrophy (37%), bilateral periventricular leukomalacia (32.6%), cystic formations (13%), distended optic nerve sheaths
(6.5%), vascular malformations (6.5%) and basal ganglia malformation (4.3%). Furthermore, 56.5% individuals had pathological EEG coupled with abnormal MRI scans and 22.2% exhibited pathological EEG, abnormal MRI scans and severe
ID.
Conclusion/discussion: In line with two previous studies (1,2), our results indicate a considerable percentage of patients
with EEG abnormalities, ID and abnormal MRI scans.
References: 1) Stanfield A. et al., 2008. 2) Ünal Ö. et al., 2009.
Poster # 1
ESMRN 2015
2
Functional connectivity of the language network in left temporal lobe epilepsy.
Widmann C 1, Nenning KH 2, Langs G 2, Prayer D 1, Kasprian G 1.
1
Division of Neuro- and Musculoskeletal Radiology, Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Austria. 2Computational Image Analysis and Radiology Lab, Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Austria.
Introduction: Atypical language representations are frequently seen in left hemispheric temporal lobe epilepsy (lTLE)
patients. fMRI based language connectome analysis offers a novel approach to study neuronal networks, linked to language function. The aim of this study was to identify alterations of these networks and characterize the language connectome (LC) in a cohort of lTLE patients.
Methods: The functional LC was calculated on the basis of preoperatively acquired functional MRI data (3 Tesla,
TE=35ms, TR=3000ms, slice thickness: 4mm, 32 slices, 96x96 matrix, 2.4x2.4x4mm, 100 dynamics, verb generation task,
FreeSurfer, FSL). The language connectome of 8 patients with non lesional lTLE (median age 35) and 12 lTLE patients
with hippocampal sclerosis (HS) (median age 42) were compared to a reference LC based on 13 healthy controls (median
age 38). Variations in the functional connectome analysis were quantified using the network-based statistics (NBS) approach.
Results: The language connectome of non lesional lTLE patients and HS patients showed a significantly increased interhemispheric connectivity (p<0.001), compared to healthy controls. While a stronger connectivity in the default mode
network was found in controls, both - non lesional lTLE and HS patients showed an increased node degree in the Broca
region.
Conclusion/Discussion: Task-based NBS analysis reveals widespread alterations in the language connectome of lTLE
patients. The recruitment of interhemispheric connections may be linked to structural alterations of the temporal lobe and/
or seizure activity. These observations refine existing theories of language reorganization in TLE.
Poster # 2
ESMRN 2015
3
Dual pathology in epilepsy - clinical challenges due to pathological diversity - an illustrated review.
Tiago Gil Oliveira 1,2, José Manuel Amorim 1, João Paulo Soares-Fernandes 1.
1
Hospital de Braga, Division of Neuroradiology, Braga, Portugal. 2 Life and Health Sciences Research Institute (ICVS), School of
Health Sciences, University of Minho, Braga, Portugal.
Introduction: A major challenge in the management of epilepsy in clinical cases with potential indication for surgical
ablation is the presence of dual pathology.
Methods: A revision of the clinical MRI records of our institution was performed. A selection of representative cases of
dual brain pathology in a pediatric population with clinical evidence of seizures is presented.
Results: We present 5 patients with a combination of at least two pathological findings identified on imaging studies that
could be either responsible for the potential epileptic foci or relevant for management and treatment. Patient#1 presents
hypoplastic corpus callosum, nodular subependymal heterotopia and an ectopic neurohypophysis. In Patient#2, a cavernous malformation in the cingulate gyrus and a focal cortical dysplasia were found. Patient#3, a neonatal Sturge-Weber
syndrome case, presented bilateral pial angiomas and accelerated myelination. Patient#4 demonstrated bilateral mesial
temporal lobe sclerosis. In Patient#5 mesial temporal lobe sclerosis was associated with anterior temporal lobe gliosis.
Conclusion/Discussion: In this revision we highlight the role of a thorough imaging evaluation in patients with refractory epilepsy. We further discuss the challenges of defining if one or more lesions are responsible for the presentation of
seizures, or their consequence. The identification and detailed characterization of the imaging findings in epilepsy are
fundamental for the proper management of each patient.
Poster # 3
ESMRN 2015
4
Fetal brain abnormalities associated with congenital heart disease: report of two cases.
Valentina Ribeiro 1, José Eduardo Alves 1, João Teixeira 1, Maria Céu Rodrigues 2.
1
Service of Neuroradiology, Centro Hospitalar do Porto, Portugal. 2 Prenatal Diagnosis Center, Centro Hospitalar do Porto, Portugal.
Brain and heart development occur simultaneously in the human fetus. Fetuses with congenital heart disease (CHD) have
a high incidence of structural prenatal central nervous system abnormalities (CNS), both malformative and acquired.
Objective and methods: We report fetal brain and neck MRI abnormalities of two fetuses with isolated congenital heart
disease at ecochardiography at Centro Materno Infantil do Porto, who underwent MRI ranged 20-24 weeks gestation.
Results: Fetus 1 had a truncus arteriosus and MRI showed corpus callosum agenesis, cerebellar hypoplasia and germinolytic cysts. Fetus 2 presented with coarction of the aorta and MRI revealed a cervical cystic hygroma and brain left
ventricular dilatation. In both cases, extensive investigation excluded genetic defects and malformative syndromes.
Discussion: Our cases are in line with the literature that both malformations and acquired brain abnormalities are already
present prior to birth in fetuses with CHD. Abnormalities in utero cerebral blood flow are associated with abnormal brain
development in newborns with CHD, although further studies with fetal MRI are necessary to confirm this suggestion.
Our case also show that there is a relationship between cystic hygroma and CHD and NT (nuchal translucency) measurement in the first trimester is potentially useful for screening for fetal major CHD.
Poster # 4
ESMRN 2015
5
Fatal acenocoumarol-related in utero subdural hemorrhage.
Mariana Diogo 1, Carla Conceição 1, Nisa Félix 2, Cláudia Rijo 2.
1
Department of Neuroradiology, Centro Hospitalar de Lisboa Central, Lisbon, Portugal, 2 Gynecology and Obstetrics Department,
Maternidade Alfredo da Costa, Lisbon, Portugal.
Introduction: Subdural hematoma (SDH) is a frequent finding in newborns, but antenatal SDH is exceptional. Risk
factors include trauma and other maternal or fetal conditions: coagulopathies, infections and iatrogenic effect of drugs,
namely vitamin K antagonists; they cross placental barrier and exposure in the second or third trimesters is associated
with central nervous system (CNS) malformations or hemorrhagic complications.
We present a rare case of extensive, acenocoumarol-associated SDH occurring in the third trimester, emphasizing the
importance of anticoagulation as a cause of antenatal intracranial bleeding and the major role played by fetal magnetic
resonance imaging (MRI) in its diagnosis.
Case Description: A 24-year-old woman under anti-coagulation for prosthetic mitral valve became pregnant. Due to
maternal high thromboembolic risk, it was decided to maintain treatment with acenocoumarol throughout the gestation,
with bi-weekly ultrasound (US) evaluations. Routine US at 35 weeks of gestation was suggestive of enlarged extra-cerebral space and fetal MRI performed the following day revealed an extensive SDH involving both hemispheric convexities
and the tentorium, with significant mass effect. The fetus died 2 days later and autopsy confirmed the imaging diagnosis.
Conclusion/Discussion: Anticoagulant-associated fetal hemorrhage is a rare but severe event. There is no alternative
regimen that can effectively replace vitamin K antagonists in women with mechanical heart valves. Although US studies
are valuable for regular monitoring, MRI offers a more detailed assessment of fetal CNS and provides a better characterization of both hemorrhagic and malformative pathology.
Poster # 5
ESMRN 2015
6
Fetal and postnatal MRI of aicardi syndrome.
Mariana Diogo 1, Carla Conceição 1, Amets Irañeta 2, Eulália Calado 3.
1
Department of Neuroradiology, Centro Hospitalar de Lisboa Central, Lisboa, Portugal. 2 Department of Neurosurgery, Centro Hospitalar de Lisboa Central, Lisboa, Portugal. 3 Department of Neuropediatrics, Centro Hospitalar de Lisboa Central, Lisboa, Portugal.
Introduction: Aicardi syndrome (AS) is classically defined by a triad of corpus callosum agenesis, chorioretinal “lacunae” and infantile spasms. In recent years and with the advent of MRI, further imaging findings have been described:
cortical polymicrogyria, periventricular heterotopia, choroid plexus anomalies, intracranial cysts and gross asymmetry of
the hemispheres, that probably represents a specific malformation complex and is highly suggestive of AS.
The purpose of this exhibit is to describe a case of AS detected in fetal MRI and confirmed in postnatal clinical assessment and imaging studies.
Case Description: A pregnant woman was referred to our neuroradiology department for an avascular inter-hemispheric
cyst detected in prenatal ultrasound. Fetal MRI was performed at 35 weeks of gestation, and depicted several intracranial
anomalies, including agenesis of the corpus callosum with associated inter-hemispheric, multiseptated cyst, a space occupying lesion of the III ventricle, subependymal heterotopias and thickening and irregularity of the frontal cortex. After
birth the newborn developed partial seizures and a brain MRI was performed, corroborating the prenatal findings and
better depicting the cortical malformation (compatible with polymicrogyria). Diagnosis of Aicardi syndrome was later
confirmed by ophthalmologic findings.
Conclusion/Discussion: We present a case of Aicardi syndrome with pre and postnatal MRI. Prenatal images were quite
elucidative, but since it is a clinical-imagiological entity, definitive diagnosis wasn’t possible until postnatal evaluation.
Imaging findings in these patients are paramount for the diagnosis as callosal agenesis is a mandatory feature.
Poster # 6
ESMRN 2015
7
Altered brain development in second trimester fetuses with Tetralogy of Fallot in fetal MRI.
Christoph Schellen 1, Ernst Schwartz 2, Gerlinde M. Gruber 3, Michael Weber 4, Peter C. Brugger 3, Georg Langs 2, Prayer Daniela 1,
Gregor Kasprian 1.
1
Division of Neuroradiology and Musculoskeletal Radiology, Department of Radiology, Medical University of Vienna, Austria.
2
Computational Image Analysis and Radiology Lab, Department of Radiology, Medical University of Vienna, Austria. 3 Center for
Anatomy and Cell Biology, Department of Systematic Anatomy, Integrative Morphology Group, Medical University of Vienna,
Austria. 4 Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Austria.
Introduction: Abnormal brain growth has previously been described in fetuses with congenital heart disease (CHD).
However, little is known about the precise timing of altered brain development and its occurrence in specific CHD subgroups. This in utero MRI study aimed to identify early (median, 25 gestational weeks [GW]) changes in fetal total brain
(TBV), gray matter (GMV), and subcortical brain (SBV) volumes in Tetralogy of Fallot (TOF) cases.
Methods: Fetal MRI (1.5 Tesla) was performed in 24 fetuses with TOF and 24 age-matched controls (20-34 GW). TBV,
GMV, SBV, and intracranial cavity (ICV), cerebellar (CBV), ventricular (VV), and external cerebrospinal fluid (eCSFV)
volumes were quantified by manual segmentation (ITK-SNAP) of T2-weighted coronal sequences. Mixed model analyses
of variance (ANOVA) and t-tests were performed to compare cases and controls.
Results: TBV was significantly lower (p<0.001) in early (<25 GW) and late TOF cases. Both GMV (p=0.003) and SBV
(p=0.001) were reduced. GMV to SBV ratios declined in fetuses with TOF (p=0.026). VV was increased (p=0.0048).
eCSFV was enlarged in relation to head size (p<0.001). ICV (p= 0.314) and CBV (p= 0.074) were not significantly reduced.
Conclusion/Discussion: Fetuses with TOF have reduced gray and white matter volumes and enlarged CSF spaces at
25 GW and earlier. Previously, fetuses with right-sided obstructive lesions were assumed to be less affected by altered
cerebral perfusion than fetuses with left ventricular dysfunction. However, the changes in the brains of fetuses with TOF
presented in this study indicate a severe impact of TOF on early antenatal brain development
Poster # 7
ESMRN 2015
8
Diffuse benign villous hyperplasia - prenatal MRI diagnosis and postnatal follow up.
Mariana Diogo 1, Carla Conceição 1, Amets Irañeta 2, Alvaro Cohen 3.
1
Neuroradiology Department, 2 Neurosurgery department, 3 Gynecology and Obstetrics Department, Centro Hospitalar de
Lisboa Central, Lisbon, Portugal.
Introduction: Diffuse benign villous hyperplasia (DVH) is a rare congenital condition characterized by bilateral enlargement of the choroid plexus and increased ventricular size. MRI is the imaging modality of choice in establishing the
diagnosis. We present a case of DVH diagnosed by fetal MRI (fMRI) and compare it to postnatal brain MRI findings. To
our knowledge, no other cases have been diagnosed in utero using this imaging modality.
Case Description/Results: A primiparous mother had an ultrasound at 21 weeks’ gestation, which showed asymmetrical enlargement of the lateral ventricles. Brain fMRI was performed at 23 weeks’ gestation (and again at 27w), showing
moderate lateral ventricle dilatation with enlarged choroid plexus, occupying both atria and occipital horns. Biparietal
and fronto-occipital diameters were above P90. Given the findings, DVH was considered the most probable diagnosis and
parents decided to carry pregnancy to term. After birth (at 39 weeks) physical and neurological examination was normal,
apart from cephalic perimeter (CP) above 95th percentile. Postanatal brain MRIs were performed at 5 weeks and 9 months
and corroborated the prenatal findings. No other abnormalities were noted. Ventricular size remained stable, with no need
for shunting.
Conclusion/Discussion: Enlarged lateral ventricles are a common reason of referral for fMRI. This case depicts a rare
entity, diagnoses by fMRI and illustrating the crucial role of this imaging modality in prenatal CNS evaluation.
DVH is a benign entity demanding differential diagnosis with more ominous disorders, like CP tumors.
Poster # 8
ESMRN 2015
9
Is fetal vermian lobulation quantifiable?
Dovjak G 1, Brugger PC 2, Gruber G 2, Schwartz E 3, Bettelheim D 4, Prayer D 1, Kasprian G 1.
1
Division of Neuro- and Musculoskeletal Radiology, Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Austria. 2 Center for Anatomy and Cell Biology, Department of Systematic Anatomy, Integrative Morphology
Group, Medical University of Vienna, Austria. 3Computational Image Analysis and Radiology Lab, Department of Biomedical
Imaging and Image-guided Therapy, Medical University of Vienna, Austria. 4Department of Obstetrics and Gynecology, Medical
University of Vienna, Austria.
Introduction: This MRI study aims to quantitatively assess normal vermian development in human fetal brains in vivo.
Furthermore the accuracy of fetal MRI based vermian visualization is determined by comparing in vivo with post-mortem segmentation results.
Methods: 29 fetuses (18-30 gestational weeks - GW, mean 25.55GW) were scanned prenatally (1.5 Tesla, T2-TSE, resolution 0.7/0.7/4.4mm before 24GW and 0.9/0.9/4.4mm after 24GW) and 7 fetuses (16-30GW, mean 21.9GW, 3 Tesla,
CISS sequence, resolution: 0.33/0.33/0.33mm) scanned within 24 hours post-mortem were selected for postprocessing. A
T2-weighted midline sagittal slice was identified and 2D vermian segmentation was performed using ITK snap.
Results: 7 of the 9 Vermian lobules could be discriminated prenatally and post-mortem. The mean proportional percentage of each vermian lobule was (in vivo vs. post mortem): 6.88% vs. 6.82% (Lingula), 10.13% vs. 11.42% (Centrum),
27.67% vs. 29.87% (Culmen), 21.9% vs. 21.42% (Declive+ Folium+ Tuber), 13.84% vs. 15.24% (Pyramis), 15.8% vs.
10.88% (Uvula), 3.78% vs. 4.35% (Nodule) across all gestational ages (18-30GW). The standard deviation ranged between
1.08-3.97 (in vivo) and 1.94%-3.31% (post mortem) per lobule. 3D models of post-mortem cerebellar hemispheres were
generated.
Conclusion/Discussion: Different vermian segments can be reliably visualized between 18 and 30GW by fetal MRI.
Proportions of the vermian lobules correlate between post-mortem and in vivo measurements and remain stable during
this observational period. In future, comparative analysis will help to further classify cases of hindbrain malformations
at prenatal stages.
Poster # 9
ESMRN 2015
10
Brain Magnetic Resonance Imaging findings after in utero toxic exposure.
José Manuel Amorim, Tiago Gil Oliveira, Jaime Rocha, João Soares Fernandes.
Serviço de Imagiologia - Departamento de Neurorradiologia, Hospital de Braga.
Introduction: Central Nervous System (CNS) malformations are mainly caused by genetic defects. However, when pregestational and/or gestational toxic exposure occurs, a teratogenic etiology should be considered.
Methods: We reviewed our institution’s neuroimaging archive and collected a series of 5 illustrative cases of children
who suffered in utero exposure to alcohol (cases 1 and 2), alcohol and valproate (case 3), cocaine and opiates (case 4) and
isotretinoin (case 5). Brain and spine MRI findings are described.
Results: Polysubstance abuse was present in 2 of the 5 cases. In the cases related to in utero alcohol exposure only, imaging findings ranged from brain abnormalities (including midline malformations and simplified gyral pattern) to craniocervical junction (CCJ) malformations and spine segmentation or fusion disorders. In valproate and alcohol consumption
we found brain midline malformations, CCJ malformations and diastematomyelia. In cocaine and opiate abuse, callosal
hypogenesis and subependymal hemorrhages were found. The use of retinoic acid was associated with cerebellar malformations, short corpus callosum and brain volume loss.
Conclusion: A wide range of CNS malformations are associated with in utero toxic exposure. The recognition of characteristic imaging abnormalities plays an important role in the diagnosis and/or confirmation of suspected drug-induced
CNS malformations. This has important medicolegal implications and also clinical significance to avoid unsuccessful and
misleading genetic testing.
Poster # 10
ESMRN 2015
11
A pauci-simptomatic presentation of Leukoencephalopathy with Vanishing White Matter.
Vera Cruz e Silva 1, Marcelo Mendonça 2, Gonçalo Matias 3, Marjo Van der Knaap 4, Joana Graça 1.
1
Neuroradiology Department, Hospital Egas Moniz (CHLO), Lisboa. 2 Neurology Department, Hospital Egas Moniz (CHLO), Lisboa. 3 Neurology Department, Hospital Geral das Forças Armadas, Lisboa. 4 VU University Medical Center, Amsterdam.
Introduction: Leukoencephalopathy with Vanishing White Matter (LVWM) is an autosomal recessive hereditary disease
and one of the more prevalent leukoencephalopathies in children. It presents mostly with long term onset and clinical presentation usually includes cerebellar ataxia and progressive neurological deterioration. Beyond the pediatric phenotype,
some pauci-symptomatic presentations have been reported in older patients.
Case description: We present a case of a 15-year-old male with normal psycho-motor development presenting a generalized seizure after being hit in the face by a ball. Three months later, in a similar context, describes a 3-minute episode of
isolated sudden conscious loss. Neurological examination depicted only hyperactive deep tendon reflexes. Electroencephalogram showed diffuse slowing, predominant in the frontal lobes, without paroxysmal activity. MRI presented extensive bilateral and symmetric hyperintensity on T2-weigthed images of white matter associated with cystic degeneration,
without basal ganglia or thalamic abnormalities, nor increased signal after gadolinium. The diagnosis of LVWM was
considered and confirmed by genetic test: 2 pathogenic compound heterozygous mutations in the gene EIF2B5. A 3-year
follow-up showed no clinical or imaging deterioration.
Discussion: The pediatric phenotype of LVWM is well known and usually points towards the diagnosis, which is afterwards confirmed by MRI studies. However, in cases with mild or unsuspicious clinical presentation such an advanced imaging presentation is usually rare and surprising. We suggest this entity to be considered among the differential diagnosis
of young onset leukodistrophies, even in the absence of typical clinical findings.
Poster # 11
ESMRN 2015
12
The role of MRI in the diagnosis of Wolfram syndrome - a review about three cases.
Mariana C. Diogo, Carla Conceição, Catarina Perry Câmara.
Neuroradiology Department, Hospital Dona Estefânia, CHLC, Lisbon, Portugal.
Introduction: Wolfram syndrome (WFS) is a neurodegenerative genetic disorder, also known by the acronym DIDMOAD, describing its main features: diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy, and deafness. Although genetic studies offer the definite diagnosis, magnetic resonance imaging (MRI) plays a preponderant role in
showing an array of suggestive findings.
Methods: We reviewed 3 cases of genetically confirmed WFS, imaged and diagnosed in our center, in which MRI played
a preponderant role in the diagnosis. Major findings in this syndrome are discussed, with special focus on the imaging
aspects.
Results: Three cases of WFS in non-related adolescent girls are described. A summary of each clinical presentation,
as well as imaging findings, are presented. Age of first symptoms’ onset varied from 18 months to 11 years of age, with
several years until diagnosis. MRI findings included: absence of the neurohpophysis physiological signal, thinning of the
optic nerves and chiasm, and atrophy and signal anomalies of the cerebral cortex, brain stem, and cerebellum.
Using these 3 cases as a starting point we review the classic and atypical imaging findings and their correlation with the
clinical presentation in WFS.
Conclusion/Discussion: Diagnosis of WFS is not always straightforward, as symptoms can develop over a long period of
time and concern different medical specialties. Neuroradiolgists should be aware of findings typical of WFS and suggest
the possibility of this diagnosis in the association of loss of neurohypophysis high signal and optic atrophy in patients
performing MRI for deafness or visual alterations.
Poster # 12
ESMRN 2015
13
Acute necrotizing encephalitis of children (ANEC).
Ibrahim Shoukry Cairo University.
Tarek Farid National Research center Cairo.
Background: Acute necrotizing encephalitis is a catastrophic disease presenting as encephalopathy and complicated by
motor and cognitive disability. Liver affection is a common association. MRI is diagnostic showing always bilateral thalamic involvement in addition to brain stem and tegmentum in some cases. This work aims at analyzing outcome of this
condition in relation to neuron-imaging.
Methods: This study comprises 3 boys and 2 girls, aged between 10 and 22 months. They presented with fever and disturbed sensorium. MRI, EEG, CSF analysis, liver and coagulation profile were done.
Results: Seizures were present in three cases two focal and one generalized. Hypotonia and sluggish tendon reflexes
were evident in four cases. Bulbar manifestations were present in two, one case had asymmetrical facial palsy and one
had ophthalmoplagia. MRI showed bilateral symmetrical thalamic necrosis in all cases. Subcortical white matter lesions
in parasagital and parietal areas were present in four cases and brain stem signals in two. Elevated transaminase and
hypoprothrombinemia were found in all cases. CSF showed increased Protein in four cases. EEG showed hypoactivity
and featureless background in two cases. One case recovered completely after hydrocortisone. Two cases with brain stem
and white matter lesion developed psychomotor retardation and were assigned on intervention program. The two cases
showing hemorrhagic necrosis in thalami died in ICU.
Conclusion: Despite thalamic necrosis in all ANEC cases, those with hemorrhagic necrosis have the worst prognosis.
Brain stem and white matter lesions are associated with neurological dysfunction and intellectual retardation.
Poster # 13
ESMRN 2015
14
Isolated bulbo-medullary atrophy/hypoplasia - anoxic-ischemic or malformative origin? A cause for
severe central hypoventilation in a neonate.
Carolina Figueira, Joana Pinto, Teresa Garcia, Rui Pedro Pais.
Serviço de Imagem Médica - Neurorradiologia, Hospital Pediátrico - Centro Hospitalar e Universitário de Coimbra, Portugal.
Introduction: Systemic hypotension and other conditions of reduced perfusion in the fetus with early or late onset in
gestation, may result in symmetrical longitudinal columns of infarction in the midbrain, pons and medulla oblongata. Isolated malformations involving the brainstem and bulbo-medullary junction are extremely rare and usually are associated
with cerebellar disorders. Magnetic Resonance Imaging (MRI) is helpful, although diagnosis during life can be quite a
challenge.
Case Report: A thirty-two weeks premature male newborn with monitorized pregnancy, revealed intrauterine growth
delay, polyhydramnios and alterations in the doppler ultrasound. An urgent cesarean was performed with required intubation and aggressive mechanical ventilatory support. The MRI performed at the 39th day of life revealed segmental
bulbo-medullary atrophy/hypoplasia with 1 mm in the antero-posterior axis. No abnormalities were found in the cerebellum and in the cranial nerves VI, VII, VIII, IX and X. During hospitalization within intensive care unit the patient
showed severe clinical deterioration and died at the fifth month and fourteenth day of life. Anatomopathological autopsy
was performed.
Conclusion/Discussion: In our case, the hypothesis of a severe anoxic-ischemic injury during fetal life was considered
rather than a malformation. This cases rarely appear in the literature and, to our knowledge, there is no published case
report with similar MRI findings.
Poster # 14
ESMRN 2015
15
Clinical and MRI multicentric prospective study for diagnosis of leptomeningeal angiomata and
Sturge-Weber syndrome in neonates with upper facial port-wine stain.
Chateil JF, Dutkiewicz AS, Havez-Enjolras M, Ezzedine K, Léauté-Labrèze C, Bessou P.
University hospital Pellegrin, Departments of Pediatric Radiology and Dermatology, Bordeaux, France.
Introduction: congenital facial port-wine stain (PWS) located in the V1 territory is at risk for leptomeningeal angiomata
(LMA), leading to Sturge Weber syndrome. The authors compared patterns of PWS and MRI.
Methods: clinical data and MRI were
prospectively collected in 71 infants. Face photographs were analysed, PWS were
classified into 6 distinct patterns. MRI with at least T1, T2 and post-contrast sequences were blindly evaluated by 2 radiologists, to search for LMA or indirect signs: white matter signal asymmetry, ventricular and choroid plexus size, pericerebral spaces, atrophy, calcification. A composite MRI score evaluated the probability for LMA, and compared to PWS
patterns and neurological signs, at least after 6 months.
Results: 71 patients were enrolled, 66 were finally included. PWS involving midline, scalp, nose, temporal areas were associated with an increased risk of LMA, several at-risk patterns were recognized. Mean age of MRI was the first 4 months
of life. 11 patients presented LMA, with significant composite score; there was one false negative with early MRI. Most
sensitive signs were white-matter signal asymmetry on T2, asymmetry of size hemispheres and choroid plexus, visibility
of the LMA after injection. Diffusion imaging can contribute to diagnosis; SWI is promising but has not been carried out
systematically in our series.
Conclusion: LMA is not always present at birth. Recognition of indirect MRI signs is crucial. The protocol that can be
proposed for babies with at-risk PWS patterns should include: Sagittal T1, axial T1 IR, axial T2, diffusion, SWI, T1 after
contrast.
Poster # 15
ESMRN 2015
16
Central nervous system imaging in childhood LCH : Preliminary findings.
Luciana Porto 1, Stefan Schöning 2, Elke Hattingen 1, Jan Sörensen 2, Thomas Lehrnbecher 2.
1
Neuroradiology Department. 2 Hospital for Children and Adolescents. Johann Wolfgang Goethe University Frankfurt/Main, Germany.
Objective: Langerhans cell histiocytosis (LCH) is a systemic disease with variable impact on the central nervous system
(CNS). The aim of this study was to evaluate the cerebral MR imaging abnormalities in children with LCH.
Material and Methods: Two experienced neuroradiologists retrospectively reviewed 31 cranial MR examinations available out of 94 children and adolescents with LCH. The incidence of typical cerebral pathologies of LCH was rated regarding their signal intensity on T2-w images and on contrast-enhanced T1-w images.
Results: The most common MR changes were osseous (55%), followed respectively by pineal enlargement (45%), pituitary stalk mass (32%) and signal changes of dentate nucleus (32%). Occasionally, hyperintensity in hippocampus, parenchymal and meningeal enhancement, as well as white matter hyperintensity also occurred. The inter-rater agreement was
69-100%. The lowest agreement was found for the pineal region and the dentate nucleus.
Conclusion: The most common sites of manifestation in LCH after the bone were the pineal gland and the hypothalamic-pituitary system. We showed that neurodegeneration can begin at early age and that the CNS may be affected not only
as primary site, but also as secondary degeneration with the involvement of the cerebellum and the white matter. It is
important to increase the sensitivity of the radiologist staff to specific MRI changes related to LCH, especially for the
pineal region and the dentate nucleus.
Poster # 16
ESMRN 2015
17
Methionine-PET in the initial work-up of suspected brain tumours: is there an added value?
V. Velickaite, T. Danfors, N. Canto-Moreira.
Uppsala University Hospital, Sweden.
Introduction: The uptake of C11-Methionine in PET (MET-PET) reflects protein synthesis. The normal brain has a low
uptake, so the method helps in the workup of suspected tumours, mostly to evaluate tumour size and, indirectly, both cell
and vessel proliferation. Its value is known in the adult population, but few data exist in the special group of pediatric
brain tumours.
Methods: We reviewed brain MET-PET examinations performed since 2004 on patients younger than 18 years, for evaluation after MRI of a new suspected tumour (n=31). Preoperative PET and MRI were compared regarding matching of
lesion size/location, suspected diagnosis and potential added value for patient management. The surgical results (WHO
grading) were compared 1) to maximum MET uptake (“hotspot”), and 2) to the type of match between MRI (FLAIR and
T1-contrast) and PET.
Results: Comparing to the suspected MRI diagnosis, PET did not add relevant clinical information in 19 (61%) patients,
was less performing in 3 (10%), and added significant information in 9 (29%). In 3 of these, PET changed the proposed
treatment. Amongst the patients that had surgery (n=18) there was no correlation between WHO grade and maximum
MET uptake. A correlation (<. 05) existed between the pattern of PET/ FLAIR match and WHO grade, but not concerning
T1-contrast.
Conclusion: MET-PET is useful in the initial workup of pediatric tumours, but does not correlate with WHO grade.
Should be interpreted jointly with MRI studies.
Poster # 17
ESMRN 2015
18
A case report based discussion concerning extra-axial tumors in pediatric age - can we suggest a gliosarcoma diagnosis?
Luísa Sampaio, Irene Bernardes, José Fonseca.
Neuroradiology Department, Centro Hospitalar de São João, Porto, Portugal.
Introduction: Extra-axial tumors in pediatric age are rare and often have the burden of a tricky differential diagnosis. In
spite of this, they are almost always forgotten when discussing pediatric tumors. We intent to discuss them, based on a
clinical case and focusing on three differential diagnosis.
Case description: An 8 year-old boy presented to the emergency department with headaches during for the last 2 months,
without neurologic deficits. CT scan showed a volumous extra-axial left temporo-parietal occupying-space lesion, mildly
heterogeneous (vascularized with necrotic-cystic areas) that enhanced avidly on postcontrast images (except in the necrotic-cystic areas); bone algorithm depicts thinning of the overlying calvaria. Brain MR confirmed the extra-axial location
of the tumor, showing well defined boundaries. For its heterogeneous morphology contributed an antero-medial necrotic
area with hemorrhagic residues and vascular flow voids. The postcontrast enhancement was intense and homogenous outside the mentioned heterogeneous areas. This lesion was surrounded by moderate vasogenic edema. No “dural tail sign”
was identified. He was submitted to surgery and the pathologic examination diagnosed gliosarcoma.
Conclusion/discussion: We believe that the main differential diagnosis to consider were anaplastic hemangiopericytoma,
atypical meningioma and gliosarcoma. We believe that atypical meningioma was the less likely, because of the thinning of
the overlying bone, that most frequently shows hyperostosis. Regarding the other two diagnosis, the imaging differential
is virtually unachievable. Both can display necrotic areas, vascular voids and thinning of the nearby calvaria. Also, they
may or may not have an associated “dural tail sign”.
Poster # 18
ESMRN 2015
19