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Resume Comme recommande par la Haute Autorite de sante, l’IRM fait partie du bilan d’un trouble cognitif et/ou comportemental. Cet examen permet d’identifier un pattern d’atrophie qui peut evoquer une maladie neuro-evolutive, et permet egalement d’evaluer une charge vasculaire ischemique et hemorragique et de diagnostiquer des lesions ni neurodegeneratives ni vasculaires pouvant expliquer les troubles. Cet examen est necessaire mais non suffisant pour un diagnostic precis et necessite des investigations complementaires telles que les evaluations neurologiques cliniques et neuropsychologiques et dans certains cas, l’utilisation de marqueurs biologiques et de metabolisme.

Resume L’Angiopathie Amyloide Cerebrale sporadique (AAC) est une micro angiopathie cerebrale frequente, liee a l’âge, caracterisee par l’accumulation de peptide amyloide Aβ dans la paroi des arteres lepto-meningees et des vaisseaux corticaux. C’est la cause principale d’hemorragies intracerebrales lobaires de la personne âgee. Son origine reste largement meconnue. Un defaut de clearance du peptide Aβ est largement evoque dans sa physiopathologie. L’etude de sujets porteurs de mutations dans les rares formes hereditaires montre qu’une longue periode pre-symptomatique est probable. L’hemorragie intracerebrale lobaire, avec son risque de recidive eleve, constitue l’avenue clinique la mieux connue. L’AAC est egalement a l’origine directe de declin cognitif avec un risque evolutif eleve vers une demence. Elle est egalement a l’origine de manifestations neurologiques focales deficitaires transitoires (TFNE). L’existence d’une forme a composante inflammatoire suggere l’association d’une reponse auto-immune specifique. L’identification de biomarqueurs permet de mieux en caracteriser l’histoire naturelle et le risque evolutif : IRM (micro-saignements, micro infarctus corticaux, hemosiderose corticale, saignements meninges, atrophie corticale, hyper signaux de la substance blanche, dilatation des espaces peri vasculaires) TEP amyloide, biomarqueurs amyloides du liquide cerebro-spinal, contribuent a creer une d’empreinte de la maladie. Si l’anatomo-pathologie reste un element essentiel du diagnostic, ils ont permis d’affiner la probabilite du diagnostic de la maladie (criteres de Boston modifies). Aucune therapeutique specifique n’est a ce jour disponible mais nombre de developpements sont en cours. Le controle strict de la pression arterielle, et la strategie therapeutique vis-a-vis des anticoagulants chez les patients a haut risque embolique sont des elements cles de la prise en charge.

Multi-state models provide an extension of the usual survival/event-history analysis setting. In the medical domain, multi-state models give the possibility of further investigating intermediate events such as relapse and remission. In this work, a further extension is proposed using relative survival, where mortality due to population causes (i.e. non-disease-related mortality) is evaluated. The objective is to split all mortality in disease and non-disease-related mortality, with and without intermediate events, in datasets where cause of death is not recorded or is uncertain. To this end, population mortality tables are integrated into the estimation process, while using the basic relative survival idea that the overall mortality hazard can be written as a sum of a population and an excess part. Hence, we propose an upgraded non-parametric approach to estimation, where population mortality is taken into account. Precise definitions and suitable estimators are given for both the transition hazards and probabilities. Variance estimating techniques and confidence intervals are introduced and the behaviour of the new method is investigated through simulations. The newly developed methodology is illustrated by the analysis of a cohort of patients followed after an allogeneic hematopoietic stem cell transplantation. The work has been implemented in the R package mstate.

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Large artery intracranial stenosis (ICS) is a common finding in stroke patients, but is much less prevalent in Western countries than in Asia and in young adults than in the elderly. We investigated the prevalence and causes of ICS among French young adults with ischaemic stroke. Clinical and radiological data of patients aged 18-54 years treated consecutively for acute ischaemic stroke in the anterior circulation at a tertiary stroke centre were analysed retrospectively. Patients with50% ICS were identified. ICS was evaluated using TOF-MRA, vessel wall-MRI, digital subtraction angiography and CT-angiography. A total of 316 patients were included. ICS was diagnosed in 29 patients, resulting in a prevalence of 9.2% (95% CI, 6.2 to 13.3). The leading cause of ICS was atherosclerosis (n=13), ahead of moyamoya disease (n=4), dissection (n=2), vasculitis (n=2), and reversible cerebral vasoconstriction syndrome (n=1). The cause of ICAS could not be determined in 7 patients. ICS was found in nearly one in 10 ischaemic strokes among French young adults. Atherosclerosis was the leading cause of ICS. The cause of ICS could not be determined in almost a quarter of the patients.

A 40-year-old woman and her 38-year-old brother developed since childhood optic neuropathy, absence epilepsy, cognitive disability, cerebellar syndrome, hypertrophic cardiomyopathy, and scoliokyphosis, due to a mitochondriopathy associating MTO1 mutation and an MT-FT variant, both genes involved in mitochondrial tRNA modification.1 Brain MRIs were normal during childhood, but cortical, symmetric hyperintensities of precentral gyri on diffusion-weighted imaging (figure) arose apart from any new symptom, different from previously reported MTO1 mitochondriopathies, only noncortical,2 from status epilepticus, generally unilateral and associated with a thalamic or corpus callosum splenium hypersignal, and from Creutzfeldt-Jakob disease, usually more posterior and associated with pulvinar and basal ganglia hyperintensities.

Aggresssive pituitary tumors are defined as radiologically invasive, exhibiting a rapid growth and a poor response to the medical and surgical treatment options. The role of magnetic resonance imaging (MRI) is fundamental to assess tumor aggressiveness before surgical exploration. Distinction between cavernous sinus invasion and cavernous sinus compression is often challenging and cannot be solved always by using the Knosp criteria. Ideally, T2W images demonstrating the ruptured internal dural wall of cavernous sinus is the ultimate proof of cavernous sinus invasion. Subtle tumor volume increase in a short time can be shown when sequential MR images are rigorously replicable. A microcystic pattern observed on T2W images frequently reflects a potentially aggressive tumor as observed in silent corticotroph pituitary adenomas.

Less than 1% of cases of acromegaly are secondary to ectopic secretion of Growth Hormone Releasing Hormone (GHRH), usually from a neuroendocrine tumor. Symptoms of ectopic acromegaly did not differ from classical acromegaly from pituitary origin. GH and IGF 1 values are in the same range. GHRH measurement only could make the correct diagnosis but is not routinely proposed in acromegaly. MRI of the pituitary gland is considered not very effective in ectopic acromegaly. In the literature (1), different patterns are described: pituitary enlargement (46%), adenoma (30%), empty sella (2%) or normal (20%). But T2MRI signal of the pituitary is never mentioned nor illustrated. Finally, in about 30 % of published cases, pituitary surgery, of course inefficient, was performed. These data enhance the poor contribution of imaging studies in the published cases of ectopic acromegaly. We have been able to obtain and read MRIs and particularly T2WI of 27 acromegalic patients- 20 female, 7 male- due to GHRH hypersecretion from a neuroendocrine tumor –principally bronchial carcinoid and pancreatic NET- which have been published or not. Remarquably, T2 sequences were available in only 27/61 cases we have collected. In all these 27 cases but two, the T2 signal was clearly hypointense if compared with the brain cortex, as it has been described in densely granulomated somatotropinomas (2). In one case with T2 hyperintensity, the pituitary mass presented the same imaging characters as multiple brain metastases from a bronchial carcinoïd. In one case, T2 signal was isointense. In 3 cases, tiny millimetric T2 hyperintense images were disseminated within pituitary hyperplasia. In several cases where pituitary MRI was considered as normal, correlation of the patient’age with pituitary size could make suspect an enlarged gland. In a case labeled empty sella, T2MRI signal of the pituitary remnant was hypointense. When coupling T2 and T1 gadolinium enhanced sequences, no pituitary adenoma was visualized and normal pituitary tissue was never identified along with pituitary hyperplasia. In conclusion, T2 MRI hypointense signal of the pituitary gland is a better hallmark than pituitary hyperplasia for the diagnosis of acromegaly due to GHRH ectopic secretion. Analysis of T2 MR signal in these cases is essential to avoid unnecessary interventions to the pituitary. References 1 Ghazi A Endocrine 2013 2 Potorac J Endocr Relat Cancer 2015

Background and Purpose— Convexity subarachnoid hemorrhage (cSAH) is an increasingly recognized presentation of cerebral amyloid angiopathy (CAA), usually revealed by transient symptoms, but data on its outcome are limited. We compared the risk of future intracerebral hemorrhage (ICH), cSAH, and death in patients with CAA after cSAH and after lobar ICH. Methods— Consecutive patients with probable CAA, based on the Boston criteria, presenting with cSAH (CAA-cSAH) or lobar ICH (CAA-ICH) were included. We obtained baseline clinical and magnetic resonance imaging data and follow-up information. Univariable and multivariable analyses were used to compare incidence rate for symptomatic ICH, symptomatic cSAH, and late-death (beyond 30 days) between patients with CAA-cSAH and CAA-ICH. Results— Among 105 patients (mean age, 76.7±7.5 years) enrolled, 44 participants presented with CAA-cSAH and 61 with CAA-ICH. The median follow-up was 22.2 months (interquartile range, 12.6–34.4). The symptomatic ICH rate (per person-year) was 10.5% (95% CI, 5.6–19.4) in patients with CAA-cSAH compared with 8.5% (95% CI, 4.4–16.4) in those with CAA-ICH (adjusted hazard ratio, 1.05; 95% CI, 0.32–3.43). The annual incidence rates of symptomatic cSAH (9.9% versus 3.8%; adjusted hazard ratio, 1.77; 95% CI, 0.43–7.28) and death (9.5% versus 17.8%; adjusted hazard ratio, 0.56; 95% CI, 0.22–1.43) were not significantly different between patients with CAA-cSAH and those with CAA-ICH. Conclusions— Patients with CAA-related cSAH have a poor outcome, with similar high risk of future ICH and long-term mortality than CAA patients after lobar ICH. Our findings may have important prognostic implication and guide management of patients with cSAH in CAA.

Background In Moyamoya Angioplasty (MMA), increased apparent diffusion coefficient (ADC) in frontal white matter (WM) with a normal appearance has been associated with frontal hypoperfusion and executive dysfunction. Multiple burr-hole surgery enables the revascularization of large frontal areas. Goal: To assess the effect of multiple burr-hole surgery on the ADC and cognitive functions in adults with MMA. Methods ADC was measured in 26 brain hemispheres of 14 consecutive adults with MMA (9 women, mean age ± SD: 38.1 ± 10.7 years) prior to and 6 months after burr-hole surgery. ADC was obtained from regions of interest located in frontal and posterior (temporo-occipital) normal-appearing WM. Ten patients had neuropsychological assessment that focused on executive and attentional functions before and after surgery. Results Anterior and posterior ADC values did not differ before surgery (815.8 ± 60.1 vs. 812.1 ± 35.3 mm2/s, p = 0.88). After surgery, frontal ADC was lower than prior to surgery (789.9 ± 64.5 vs. 815.8 ± 60.1 mm2/s; p p = 0.31). Trail-making test part B median z-score increased from − 1.47 to − 0.21 (p = 0.018), suggesting improved cognitive flexibility. Conclusion In adults with MMA, indirect revascularization with burr-hole is followed by a decrease of ADC in normal-appearing frontal WM and may have improved some executive functions in the flexibility process. Change in ADC may reflect the improvement in cerebral perfusion after surgery. The measuring of ADC may be a promising tool in exploring potentially reversible microstructural WM damage related to hypoperfusion and cognitive change in MMA.

Purpose New multiple sclerosis (MS) disease-modifying therapies (DMTs), which exert beneficial effects through prevention of relapse, limitation of disability progression, and improvement of patients’ quality of life, have recently emerged. Nonetheless, these DMTs are not without associated complications (severe adverse events like. progressive multifocal leukoencephalopathy). Patient follow-up requires regular clinical evaluations and close monitoring with magnetic resonance imaging (MRI). Detection of new T2 lesions and potential brain atrophy measurements contribute to the evaluation of treatment effectiveness. Current MRI protocols for MS recommend the acquisition of an annual gadolinium (Gd) enhanced MRI, resulting in administration of high volume of contrast agents over time and Gd accumulation in the brain. Methods A consensus report was established by neuroradiologists and neurologists from the French Observatory of MS, which aimed at reducing the number of Gd injections required during MS patient follow-up. Recommendations The French Observatory of MS recommends the use of macrocyclic Gd enhancement at time of diagnosis, when a new DMT is introduced, at 6-month re-baseline, and when previous scans are unavailable for comparison. Gd administration can be performed as an option in case of relapse or suspicion of intercurrent disease such as progressive multifocal leukoencephalopathy. Other follow-up MRIs do not require contrast enhancement, provided current and previous MRI acquisitions follow the same standardized protocol including 3D FLAIR sequences.

Key Points Question Are early brain changes seen on magnetic resonance imaging (MRI) associated with functional outcomes among adult patients with severe herpes simplex encephalitis (HSE)? Findings In adult patients with HSE, extensive brain changes seen on MRI during the first month after intensive care unit admission were independently associated with poor functional outcome at 90 days. Thalamic diffusion signal changes were frequently observed and were associated with poor prognosis, mainly in older patients. Meanings These findings suggest that, among adult patients with severe HSE, early MRI data were associated with neurological outcomes, both in terms of brain lesion extension and critical focal MRI signal changes., This cohort study investigates the association between early collected magnetic resonance imaging (MRI) data and functional outcomes 90 days after intensive care unit admission among adults with severe herpes simplex encephalitis., Importance Current guidelines recommend brain magnetic resonance imaging (MRI) for clinical management of patients with severe herpes simplex encephalitis (HSE). However, the prognostic value of brain imaging has not been demonstrated in this setting. Objective To investigate the association between early brain MRI data and functional outcomes of patients with HSE at 90 days after intensive care unit (ICU) admission. Design, Setting, and Participants This multicenter cohort study was conducted in 34 ICUs in France from 2007 to 2019 and recruited all patients who received a clinical diagnosis of encephalitis and exhibited cerebrospinal fluid positivity for herpes simplex virus DNA in the polymerase chain reaction analysis. Data analysis was performed from January to April 2020. Exposures All patients underwent a standard brain MRI during the first 30 days after ICU admission. Main Outcomes and Measures MRI acquisitions were analyzed by radiologists blinded to patients’ outcomes, using a predefined score. Multivariable logistic regression and supervised hierarchical classifiers methods were used to identify factors associated with poor outcome at 90 days, defined by a score of 3 to 6 (indicating moderate-to-severe disability or death) on the Modified Rankin Scale. Results Overall, 138 patients (median [interquartile range {IQR}] age, 62.6 [54.0-72.0] years; 75 men [54.3%]) with an admission median (IQR) Glasgow Coma Scale score of 9 (6-12) were studied. The median (IQR) delay between ICU admission and MRI was 1 (1-7) days. At 90 days, 95 patients (68.8%) had a poor outcome, including 16 deaths (11.6%). The presence of fluid-attenuated inversion recovery MRI signal abnormalities in more than 3 brain lobes (odds ratio [OR], 25.71; 95% CI, 1.21-554.42), age older than 60 years (OR, 7.62; 95% CI, 2.02-28.91), and the presence of diffusion-weighted MRI signal abnormalities in the left thalamus (OR, 6.90; 95% CI, 1.12-43.00) were independently associated with poor outcome. Machine learning models identified bilateral diffusion abnormalities as an additional factor associated with poor outcome (34 of 39 patients [87.2%] with bilateral abnormalities had poor outcomes) and confirmed the functional burden of left thalamic lesions, particularly in older patients (all 11 patients aged >60 years had left thalamic lesions). Conclusions and Relevance These findings suggest that in adult patients with HSE requiring ICU admission, extensive MRI changes in the brain are independently associated with poor functional outcome at 90 days. Thalamic diffusion signal changes were frequently observed and were associated with poor prognosis, mainly in older patients.

Resume Introduction La sclerodermie lineaire, ou morphee, est une maladie fibrosante affectant la peau et parfois les tissus plus profonds. Les sclerodermies lineaires de la face s’accompagnent parfois de troubles neurologiques. Nous en decrivons un cas associe a des anomalies neurologiques particulieres, non rapportees jusqu’ici dans la litterature. Observation Un patient de 16 ans consultait en 2009 pour une sclerodermie lineaire hemifaciale gauche. Un traitement de 14 mois par methotrexate entrainait une stabilisation de la maladie. En 2013, devant une aggravation des lesions cutanees et l’apparition d’un ptosis homolateral, une IRM cerebrale etait realisee qui montrait des anomalies de signal unilaterales hemispheriques sous la forme d’une plage d’hypersignal T2 des noyaux gris centraux et des hyposignaux T2* homolateraux correspondant a des microsaignements. En 2014 et 2015, le patient presentait trois episodes brefs de paresthesies de l’hemicorps droit (avec pour le premier episode, une aphasie transitoire puis des cephalees). L’IRM cerebrale montrait une aggravation des lesions en faveur d’une microangiopathie cerebrale evolutive. Discussion Les anomalies neurologiques associees aux sclerodermies localisees de la face ne sont pas toujours connues des cliniciens mais ne sont pas exceptionnelles. Leurs manifestations cliniques sont variees. Sur le plan radiologique, il s’agit le plus souvent de calcifications et de lesions de la substance blanche hyperintenses en T2. A notre connaissance, les microsaignements observes chez notre patient n’avaient jamais ete rapportes. L’evolution de ces anomalies neurologiques, dont la nature est inconnue, ne serait pas correlee a celle de l’atteinte dermatologique. Il est important que les dermatologues connaissent ces complications neurologiques de la sclerodermie lineaire de la face.

Non-functioning pituitary adenoma may be totally asymptomatic and discovered “incidentally” during radiological examination for some other indication, or else induce tumoral signs with compression of the optic chiasm and pituitary dysfunction. Non-functioning adenomas are mainly gonadotroph, but may also be “silent”. Treatment strategy depends on initial clinical, biological, ophthalmological and radiological findings. The present French Society of Endocrinology Consensus work-group sought to update the pitfalls associated with hormone assay and outline a hormonal exploration strategy for diagnosis and follow-up, without overlooking the particularities of silent adenoma. We also drew up basic rules for initial exploration and radiological follow-up of both operated and non-operated pituitary adenomas.

Resume La thrombose veineuse cerebrale (TVC) est une urgence qui peut engager le pronostic vital. Le polymorphisme clinique renforce l’importance de l’imagerie en coupes pour poser le diagnostic. Tant le scanner que l’IRM permettent de porter le diagnostic d’occlusion d’un sinus veineux mais l’IRM est superieure au scanner pour detecter un caillot dans les veines corticales ou profondes. Le scanner peut montrer le caillot spontanement hyperdense et l’angioscanner le defect endoluminal. L’IRM detecte egalement ce thrombus, dont le signal varie en fonction du temps : a la phase aigue, il est en hyposignal marque en T2* alors que T1 et T2 peuvent apparaitre faussement rassurants ; a la phase subaigue, il est en hypersignal sur toutes les sequences (T1, T2, FLAIR, T2*, diffusion). L’IRM montre facilement la souffrance ischemique, voire hemorragique, du parenchyme cerebral en cas de TVC. Enfin, l’imagerie permet parfois de reveler une autre pathologie a l’origine de la TVC, comme une fracture de la voute, une infection, une tumeur, ou encore une fistule durale, voire de l’hypotension intracrânienne.

La nevralgie trigeminale est une affection ancienne, entrainant une douleur invalidante de la face localisee sur une ou plusieurs branches du nerf trijumeau. Elle affecte plus souvent la femme avec une incidence qui augmente avec l’âge. Son diagnostic est clinique sur sa forme typique, mais des examens complementaires sont a envisager en fonction des presentations symptomatiques. Le traitement medical de premiere intention est base sur la carbamazepine ou l’oxcarbazepine. Chez les patients âges et polymediques, des interactions medicamenteuses et le risque d’effets secondaires genants doivent etre pris en compte. L’option chirurgicale doit etre envisagee rapidement en cas d’echec medicamenteux chez les patients resistant au traitement medical.

Objective Both antitumor and antisecretory efficacies of dopamine agonists (DA) make them the first-line treatment of macroprolactinomas. However, there is no guideline for MRI follow-up once prolactin is controlled. The aim of our study was to determine whether a regular MRI follow-up was necessary in patients with long-term normal prolactin levels under DA. Patients and methods We conducted a retrospective multicenter study (Marseille, Paris La Pitie Salpetriere and Nancy, France; Liege, Belgium) including patients with macroprolactinomas (largest diameter: >10 mm and baseline prolactin level: >100 ng/mL) treated by dopamine agonists, and regularly followed (pituitary MRI and prolactin levels) during at least 48 months once normal prolactin level was obtained. Results In total, 115 patients were included (63 men and 52 women; mean age at diagnosis: 36.3 years). Mean baseline prolactin level was 2224 ± 6839 ng/mL. No significant increase of tumor volume was observed during the follow-up. Of the 21 patients (18%) who presented asymptomatic hemorrhagic changes of the macroprolactinoma on MRI, 2 had a tumor increase (2 and 7 mm in the largest size). Both were treated by cabergoline (1 mg/week) with normal prolactin levels obtained for 6 and 24 months. For both patients, no further growth was observed on MRI during follow-up at the same dose of cabergoline. Conclusion No significant increase of tumor size was observed in our patients with controlled prolactin levels on DA. MRI follow-up thus appears unnecessary in patients with biologically controlled macroprolactinomas.

Objectif Etudier les caracteristiques IRM des neuromyelites optiques associees aux anticorps anti-MOG. Materiel et methodes Etude retrospective, multicentrique, incluant 53 patients anti-MOG+, ayant presente au moins une nevrite optique (43 [81,1 %]) et/ou une myelite (13 [24,5 %]) entre 2011 et 2017. Toutes les IRM ont ete analysees par deux neuroradiologues. Resultats et discussion Les nevrites optiques apparaissaient en hypersignal T2 (100 %) et prenaient le contraste (84,7 %). Elles predominaient dans les segments anterieurs du nerf optique (96,6 % dans les segments intra-orbitaires, 71,2 % dans le segment intra-canalaire). Contrairement aux nevrites optiques associees aux anti-AQP4, les lesions associees aux anti-MOG respectaient le chiasma et les bandelettes optiques (1,7 %). L’atteinte bilaterale (26,4 %), l’œdeme papillaire radiologiquement visible (52,5 %), ainsi que l’inflammation perinerveuse (32 %) etaient frequents et evocateurs. Les myelites etaient en hypersignal T2 (100 %), extensives (hauteur superieure a 3 segments vertebraux) ou courtes (hauteur inferieure a 2 segments vertebraux), pouvant toucher toute la moelle epiniere, mais avec une predilection pour le cone medullaire (50 %). Elles touchaient la substance grise et de la substance blanche sur la section axiale du cordon medullaire (62,5 %), et presentaient une prise de contraste souvent floue (83,3 %). Nous rapportons un nouveau signe original, evocateur d’atteinte medullaire anti-MOG : la pseudo-dilatation du canal central de l’ependyme, visualise quasi systematiquement (90 %), et possiblement associee a une prise de contraste ependymaire lineaire (16,7 %). Conclusion Les nevrites optiques aigues associees aux anti-MOG sont souvent bilaterales, œdemateuses, et se presentent avec une atteinte longitudinale extensive touchant quasi systematiquement toutes les portions intra-orbitaires du nerf optique. Nous illustrons la diversite des lesions de myelite et nous rapportons un nouveau signe original : la pseudo-dilatation du canal central ependymaire, possiblement evocateur d’atteinte medullaire aux anti-MOG.

Central nervous system (CNS) involvement in systemic disease (SD) is unusual. MRI features of such lesions are unfamiliar to most radiologists. The diagnosis of SD is still based on clinical features and laboratory findings but some characteristic MRI findings exist for each SD: micronodular leptomeningeal enhancement in sarcoidosis, diffuse or focal pachymeningeal involvement in Wegener disease, dentate nuclei and brain stem lesions in Langerhans cell histiocytosis, meningeal masses, dentate nuclei lesions and periarterial infiltration in Erdheim-Chester disease, meningeal masses in Rosai-Dorfman disease, veinular pontic lesions and cerebral vein thrombosis in Behcet, supratentorial microvascular lesions in lupus and antiphospholipid and Gougerot-Sjogren syndrome. In this work, we explain, describe and illustrate the most characteristic MRI findings for each disease.

Les adenomes hypophysaires somatotropes expriment majoritairement les recepteurs a la somatostatine (SSTR) SSTR2a et SSTR5. Cette expression est variable. Ces recepteurs sont la cible des analogues de la somatostatine. Des etudes retrospectives suggerent une association significative entre l’expression des SSTR et la reponse au traitement par octreotide LP (OCT-LP) ou pasireotide LP (PAS-LP). D’autres facteurs pourraient etre predictifs de reponse, notamment un signal IRM T2 hypointense de l’adenome. EOLIA est une etude observationnelle francaise qui a pour objectif d’evaluer ces donnees de facon prospective. Quatre-vingt patients acromegales initiant un traitement par analogue de la somatostatine seront inclus dans l’etude (50 patients OCT-LP, 30 PAS-LP) et suivis 6 mois. Les parametres biologiques, anatomopathologiques et radiologiques seront evalues de maniere centralisee. L’objectif principal sera d’evaluer la correlation entre l’expression des recepteurs SST2a et SST5, evaluee par immunohistochimie et le pourcentage de reduction de la GH a 6 mois dans chacun des groupes. Les principaux objectifs secondaires seront de decrire les correlations suivantes dans chacun des groupes : reponse biologique (reduction de GH a 3 mois, reduction d’IGF-1 a 3 et 6 mois) et expression des SSTR2a et SSTR5, reponse biologique et intensite du signal IRM prechirurgical T2, expression des recepteurs SSTR2a et SSTR5 et intensite du signal IRM prechirurgical T2, reponse biologique et granulation de l’adenome, expression des SSTR2a et SSTR5 et granulation de l’adenome (population totale). La tolerance a OCT-LP et PAS-LP sera egalement evaluee. L’etude EOLIA fournira des donnees prospectives importantes pour optimiser et personnaliser le traitement des patients acromegales.

We report an atypical case of cerebral toxoplasmosis (CT) in a 70-year-old woman with a history of breast cancer. Contrast-enhanced computed tomography revealed a single ring-enhancing lesion in the pons with perifocal oedema and mass effect. Toxoplasma encephalitis was suggested by means of diffusion weighted imaging, MR perfusion and MR spectroscopy, leading to the discovery of HIV infection. The patient was put on antitoxoplasma therapy. Subsequent clinical and radiological improvements confirmed the diagnosis.

RADIOLOGIE ET IMAGERIE MEDICALE : Musculosquelettique - Neurologique - Maxillofaciale - 30-650-A-10

RADIOLOGIE ET IMAGERIE MEDICALE : Musculosquelettique - Neurologique - Maxillofaciale - 30-650-A-11

L'angiographie cerebrale numerisee, toujours consideree comme l'examen de reference dans l'exploration des vaisseaux intracrâniens, est depuis quelques annees progressivement remplacee par de nouvelles techniques d'imagerie non invasives (angioscanner et angio-imagerie par resonance magnetique [IRM]) de plus en plus fiables et sensibles. L'angiographie conventionnelle conserve quelques indications, notamment dans le cadre du bilan diagnostique des angeites cerebrales, et surtout dans le bilan pretherapeutique et le suivi des malformations vasculaires cerebrales (malformations arterioveineuses [MAV] et fistules arterioveineuses [FAV]), et des anevrismes intracrâniens. Ainsi, la connaissance des modalites de realisation de cet examen invasif ainsi que l'angioanatomie des vaisseaux intracrâniens sont indispensables pour la pratique de la neuroradiologie interventionnelle (NRI), mais egalement pour une meilleure comprehension de la pathologie vasculaire cerebrale. Dans ce chapitre, la radioanatomie des vaisseaux intracrâniens et leurs principales variations anatomiques sont presentees, ainsi que les principales complications pouvant survenir au cours ou au decours de cet examen. Enfin, la place de l'angiographie cerebrale par rapport aux autres modalites d'imagerie non invasive dans la demarche diagnostique des pathologies vasculaires intracrâniennes est discute

RADIOLOGIE ET IMAGERIE MEDICALE : Musculosquelettique - Neurologique - Maxillofaciale - 31-658-C-10

Pituitary incidentalomas are a by-product of modern imaging technology. The term ‘incidentaloma’ is neither a distinct diagnosis nor a pathological entity. Rather, it is a collective designation for different entities that are discovered fortuitously, requiring a working diagnosis based on the input of the radiologist, endocrinologist and often a neurosurgeon. In addition to pathological conditions affecting the pituitary gland, a thorough knowledge of the radiological characteristics of normal variants and technical artifacts is required to arrive at an accurate differential diagnosis. After careful radiological and hormonal evaluation, the vast majority of pituitary incidentalomas turn out to be non-functioning pituitary microadenomas and Rathke’s cleft cysts (RCCs). Based on the low growth potential of non-functioning pituitary microadenomas and RCCs, periodic MRI surveillance is currently considered the optimal management strategy. Stricter follow-up is required for macroadenomas, as increases in size occur more frequently.

Linear scleroderma is a fibrotic disease affecting the skin and sometimes the deeper tissues. We describe a case of scleroderma associated with neurological anomalies not previously reported in the literature.A 16-year-old male patient presented in 2009 for hemifacial linear scleroderma. Treatment with methotrexate for 14 months resulted in stabilization of the disease. In 2013, we noted worsening of the patient's skin lesions as well as homolateral ptosis. Head MRI revealed unilateral hemispherical signal abnormalities with T2 hypersignal in the basal gangliaand punctate foci of T2* hyposignal corresponding to microbleeds. In 2014 and 2015, the patient presented three brief episodes of right hemicorpus paresthesia (with temporary aphasia followed by headache during the first episode). The head MRI showed worsening of the anomalies, suggesting progressing cerebral microangiopathy.Clinicians may not always be familiar with the neurological abnormalities associated with localized facial scleroderma even if such abnormalities are not uncommon (their exact prevalence is unknown). Clinical signs vary but, in most cases, the radiological features are calcifications and hyperintense foci of white matter lesions in T2. As far as we are aware, there have been no reports to date of microbleeding as observed in our patient. The worsening with time of these neurological anomalies of unknown origin does not appear to be correlated with the dermatological lesions. It is important for dermatologists be aware of these complications of facial linear scleroderma.

Resume Toute exploration cerebrale en TDM et IRM comprend des coupes axiales passant par le mesencephale dont la forme de cœur caracteristique est facilement reconnaissable. A cet etage, une grande variete de structures anatomiques est visible dont l’atteinte peut etre responsable de symptomes varies. L’etude de ces coupes permet egalement de reconnaitre les signes d’hypertension intra-crânienne et d’apprecier la gravite de pathologies sus et sous-tentorielles pour adapter la prise en charge therapeutique.

Resume Toute exploration cerebrale en TDM et IRM comprend des coupes axiales passant par le mesencephale dont la forme de cœur caracteristique est facilement reconnaissable. A cet etage, une grande variete de structures anatomiques est visible dont l′atteinte peut etre responsable de symptomes varies. L′etude de ces coupes permet egalement de reconnaitre les signes d′hypertension intra-crânienne et d′apprecier la gravite de pathologies sus et sous-tentorielles pour adapter la prise en charge therapeutique.

MRI is the sole radiological examination to be obtained if abnormal menses, galactorrhea or anovulation evokes the diagnosis of prolactinoma. Nevertheless, iatrogenic hyperprolactinemia or presence of big prolactin has to be ruled out before MRI is asked for. MRI of the pituitary gland must be technically optimal; analysis of the images has to be directed by clinical and biological data.

There have been somewhat conflicting reports published about the significance of linear meningeal thickening and enhancement adjacent to peripherally located cranial mass lesions on contrast-enhanced magnetic resonance (MR) images. Most of the authors consider this so-called "dural tail sign" or "flare sign" almost specific for meningioma. This review illustrates the MR imaging findings of a wide spectrum of disorders that show this dural sign. Causes include other extra-axial lesions and also peripherally located intra-axial lesions such as neuromas, chloromas, metastases, lymphoma, gliomas, pituitary diseases, granulomatous disorders, and also cerebral Erdheim-Chester disease. The dural tail sign is not specific to a particular pathological process. Nevertheless, useful conclusions can be drawn from the morphology of the lesion, its enhancement pattern, and its solitary or multifocal presentation. The final diagnosis must be based on cerebrospinal fluid studies or histological studies after biopsy.

Objectif Evaluer l’influence de la visualisation des thrombus en T2* (SVS) sur la recanalisation, le bon pronostic clinique a 3 mois et l’etiologie de l’accident vasculaire cerebral des patients traites par thrombectomie mecanique. Materiels et methodes Cette etude observationnelle portait sur une base de donnee prospective des infarctus cerebraux traites par thrombectomie mecanique recueillant des donnees cliniques et radiologiques. Les criteres d’inclusion etaient : avoir un infarctus cerebral diagnostique sur une IRM avec une sequence T2*, traite par thrombectomie mecanique avec ou sans thrombolyse intra veineuse. La visualisation (SVS+) ou non (SVS−) du thrombus en T2* etait realisee independamment par deux observateurs. La recanalisation etait evaluee en de fin de procedure par le score TICI. L’handicap residuel (evalue par le score mRS), et l’etiologie de l’infarctus etaient etablis a distance par un neurologue. Resultats Sur 180 patients, un SVS+ etait retrouve chez 137 (76 %) patients et SVS− dans 43 (24 %) cas. Le coefficient de variabilite inter-observateur etait de k = 0,609 avec un IC 95 % [0,485–0,732]. La recanalisation TICI2b/3 etait significativement associee a la presence du SVS+ avec un OR = 3,11, IC 95 % [1,29–7,57], p = 0,01 apres analyse multivariee. L’association entre SVS+ et mRS 0–2 n’etait pas significative, OR = 0,59, [0,27–1,28], p = 0,15. Comme pour le SVS et la cause cardioembolique OR = 1,81, IC 95 % [0,77–4,34], p = 0,18. La TIV, n’etait pas significativement associee a une recanalisation satisfaisante ou un bon pronostic a 3 mois en multivariee. Conclusion La presence ou l’absence du SVS est un biomarqueur, fiable et accessible, predictif d’une meilleure recanalisation lors de la thrombectomie mecanique independamment du traitement par TIV.

Objectifs Decrire une approche originale d’analyse volumique de l’IRM de perfusion dans le suivi des glioblastomes, et d’en comparer les resultats a ceux obtenus par la methode classique, dans le diagnostic de pseudoprogression. Materiel et methodes Etude retrospective de 26 patients, en progression lesionnelle sur l’IRM realisee un mois apres la fin du traitement par RT-TMZ. Sur les sequences de DSC-perfusion, toutes realisees a 3 Tesla, deux analyses des cartographies de CBV ont ete realisees : 1° calcul classique de rCBVmax par disposition manuelle de ROI, 2° etude de l’ensemble des CBV du volume tumoral prealablement segmente sur la sequence 3DT1 injectee. Resultats L’analyse de l’IRM realisee a 3 mois de la fin de la RT, a l’aide des criteres RANO, a permis de classer les patients en deux groupes : pseudoprogression (PSP, n = 14) et vraie progression (VP, n = 12). Lors de l’etude par ROI, le rCBVmax moyen etait de 5 ± 1,4 pour les PSP et 7,4 ± 2,2 pour les VP (p = 0,13). Lors de l’etude volumique le rCBVmax moyen etait de 8,5 ± 1,4 pour les PSP et 8,8 ± 1,2 pour les VP (p = 0,72). L’application d’un seuil de rCBV > 2, nous a permis d’obtenir un pourcentage de volume tumoral hypervascularise par rapport au volume tumoral total prenant le contraste, en moyenne de 27 % pour les PSP et 47 % pour les VP (p = 0,02). Conclusion L’analyse volumique des CBV tumoraux a permis de mettre en evidence un nouveau parametre perfusionnel : %rCBVmax > 2, refletant l’importance de la neo-angiogenese tumorale, ameliorant les performances de l’IRM de perfusion dans le diagnostic precoce de pseudoprogression et vraie progression.

Objectif Evaluer l’interet de l’injection de gadolinium dans le suivi IRM des patients SEP sous Tysabri. Materiel et methode Etude retrospective monocentrique des IRM cerebrales de suivi des patients consecutifs traites par Tysabri entre janvier 2014 et janvier 2017. Les prises de contraste en T1 apres Gadolinium et les nouveaux hypersignaux T2/FLAIR etaient etudies. Resultats Sur les 156 patients traites pendant cette periode, 27 n’ont pas eu leurs IRM dans notre institution. Chez les 129 patients restants, 929 IRM de suivi ont ete realisees pendant la duree du traitement par Tysabri, dont 735 (80 %) avec du Gadolinium. Aucune nouvelle lesion prenant le contraste n’a ete detectee chez les patients sous Tysabri de maniere continue. Une nouvelle prise de contraste a ete mise en evidence chez 3 patients (2,3 %) qui avaient arrete transitoirement le traitement. Chez 16 patients (12,4 %) l’IRM a montre un nouvel hypersignal T2/FLAIR : dans 11 cas, il s’agissait d’hypersignaux apparus entre l’IRM de reference avant mise en route du Tysabri et la premiere IRM apres le debut du traitement. Ces hypersignaux pouvaient donc correspondre a un reste d’activite de la maladie avant la pleine efficacite du traitement. Il y avait 1 cas de LEMP et 4 cas de nouvelle lesion dont 3 apres arret transitoire du traitement. Conclusion Aucune lesion prenant le contraste n’a ete detectee lors du suivi en IRM des patients SEP traites de maniere continue par Tysabri. L’injection systematique de Gadolinium ne semble pas presenter d’interet dans cette population.

Objectifs L’aspect du thrombus dans les accidents vasculaires ischemiques (AVCi) peut etre analyse en IRM ou scanner. En retirant les thrombus, la thrombectomie mecanique (TM) nous permet egalement une analyse, macroscopique et histopathologique. Le but de notre etude etait d’analyser les relations, entre l’aspect du thrombus en IRM, sa composition en histopathologie et les donnee clinico-radiologiques. Materiels et methodes Nous collections les thrombus retires lors des TM dans notre centre depuis octobre 2016. Ils etaient fixes et inclus en paraffine puis analyses grâce aux colorations hematoxyline eosine (HE), et Martius Scarlet Blue (MSB). Nous collections egalement les donnees clinico-radiologiques tels que : le traitement anti-agregant ou anticoagulant ou thrombolyse IV, l’aspect du thrombus en T2* (susceptibility vessel sign [SVS]), etiologie de l’AVCi, et evolution clinique a 3 mois. Concernant la TM, nous collections egalement les informations sur le score de recanalisation (TICI), le nombre de passage et la technique qui ont permis de retirer le thrombus. Resultats L’analyse portait sur plus de 100 thrombus retires entre octobre 2016 et novembre 2017 dans notre centre. Les analyses preliminaires retrouvaient des correlations entre la composition des thrombus et la presence du SVS. Le traitement medical par anti-agregants ou anticoagulants, etait correle avec une composition particuliere des thrombus.

X-linked acrogigantism (X-LAG) is a new syndrome of pituitary gigantism, caused by microduplications on chromosome Xq26.3, encompassing the geneGPR101, which is highly upregulated in pituitary tumors. We conducted this study to explore the clinical, radiological, and hormonal phenotype and responses to therapy in patients with X-LAG syndrome. The study included 18 patients (13 sporadic) with X-LAG and microduplication of chromosome Xq26.3. All sporadic cases had unique duplications and the inheritance pattern in two families was dominant, with all Xq26.3 duplication carriers being affected. Patients began to grow rapidly as early as 2–3 months of age (median 12 months). At diagnosis (median delay 27 months), patients had a median height and weight standard deviation scores (SDS) of >+3.9 SDS. Apart from the increased overall body size, the children had acromegalic symptoms including acral enlargement and facial coarsening. More than a third of cases had increased appetite. Patients had marked hypersecretion of GH/IGF1 and usually prolactin, due to a pituitary macroadenoma or hyperplasia. Primary neurosurgical control was achieved with extensive anterior pituitary resection, but postoperative hypopituitarism was frequent. Control with somatostatin analogs was not readily achieved despite moderate to high levels of expression of somatostatin receptor subtype-2 in tumor tissue. Postoperative use of adjuvant pegvisomant resulted in control of IGF1 in all five cases where it was employed. X-LAG is a new infant-onset gigantism syndrome that has a severe clinical phenotype leading to challenging disease management.

Normal anatomical structures and lesions characterized by low T2W signal intensity are reviewed in this pictorial essay. The purpose is to demonstrate how evaluation of the appearance, shape and exact anatomical location of the T2W hypointense sellar region structure, correlated with its T1W signal intensity, can based on the clinical context lead to an appropriate differential diagnosis.

MRI is the sole radiological examination to be obtained if abnormal menses, galactorrhea or anovulation evokes the diagnosis of prolactinoma. Nevertheless, iatrogenic hyperprolactinemia or presence of big prolactin has to be ruled out before MRI is asked for. MRI of the pituitary gland must be technically optimal; analysis of the images has to be directed by clinical and biological data.

Introduction Les traumatismes crâniens entrainent des dommages cerebraux (TBI) et s’associent frequemment a des troubles neuroendocriniens. Ces anomalies endocriniennes restent cependant sous-evaluees dans le cadre de la medecine d’expertise. Patients et methodes Nous etudions les donnees biologiques et d’IRM hypophysaire d’une serie de 65 patients adresses par des medecins experts pour exploration endocrinienne. Les patients ont subi un TBI leger-modere en milieu professionnel ou sur la voie publique : ils sont explores en moyenne 2 ans apres l’accident. Les patients dont les symptomes restent inexpliques beneficient d’une mise au point endocrinienne de base et un test au glucagon. Un deuxieme test insulinique, de confirmation, est pratique si necessaire. Un IRM hypophysaire est demandee en cas d’anomalies endocriniennes et les donnees radiologiques obtenues a l’admission pour TBI sont egalement revues. Resultats La prevalence de troubles neuroendocriniens dans cette serie est de 35 % (23/65). Les patients ont un âge moyen de 45 ± 14 ans (10 H/13 F) et un IMC 29 ± 4 kg/m2. Un premier test au glucagon est anormal chez 21/23 patients, un deuxieme test insulinique est anormal chez 9/10 patients. Les explorations retrouvent un deficit somatotrope (15/23), gonadotrope (7/23), corticotrope (7/23), une prolactine Conclusions Par sa frequence, la recherche d’un deficit neuroendocrinien est necessaire lors du bilan d’un patient avec TBI et merite d’etre integree dans la medecine d’expertise.