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Introduction Onconeuronal antibodies directed against intracellular antigens are strongly associated with paraneoplastic syndromes and their detection in the absence of cancer is unusual. We herein report a case of anti-Ma2 encephalitis associated with Sjogren's syndrome (SS). Case report An 81-year-old woman followed for a cutaneous lupus with vasculitis associated with SS presented a flare of her disease with neurological worsening including walking difficulty, hypersialorrhea and dysphagia. A paraneoplastic origin of the symptoms was suspected and anti-Ma2 antibodies were positive in serum. The search for an underlying neoplasia was negative. The diagnosis of anti-Ma2 encephalitis secondary to a SS was made. In the literature, the association of anti-Ma2 encephalitis and SS has been previously reported twice. Cases of patients with other onconeuronal antibodies associated with SS have been also reported. Anti-Ma2 encephalitis is a rare condition with a wide spectrum of symptoms associated with a cancer in more than 90% of the cases. Anti-Ma2 encephalitis has also been described after the use of immune check points inhibitors underscoring the role of autoimmunity in its pathogenesis. Conclusion Anti-Ma2 encephalitis is essentially associated with neoplasia but can occur in Sjogren's syndrome.

Resume Introduction Les auteurs exposent les recommandations de la Societe francaise d’oto-rhino-laryngologie et de chirurgie de la face et du cou (SFORL) concernant le bilan clinique et d’imagerie d’une adenopathie cervicale d’allure kystique sans porte d’entree chez l’adulte. En majorite, il s’agit de metastase d’un carcinome des voies aerodigestives superieures, le plus souvent oropharynge, plus rarement d’un carcinome differencie de la thyroide ou d’un carcinome non keratinisant du nasopharynx. Objectif Un groupe de travail multidisciplinaire a ete charge de realiser une revue de la litterature scientifique concernant le bilan etiologique a effectuer en presence d’une adenopathie cervicale d’allure kystique chez l’adulte, comprenant les donnees cliniques et d’imagerie conventionnelle (echographie, TDM, IRM) et metaboliques. Les recommandations ont ete redigees a partir de ces textes et de l’experience de chacun puis classees en grade A, B, C ou accord professionnel selon un niveau scientifique decroissant. Resultats Il est recommande de realiser un examen clinique oriente, une echographie cervicale et thyroidienne, une TDM cervicothoracique injectee chez un adulte presentant une adenopathie cervicale d’allure kystique sans porte d’entree. La TEP-TDM est recommandee avant l’endoscopie pour rechercher la tumeur primitive. Conclusion La realisation d’un bilan clinique et d’imagerie est essentielle pour le diagnostic etiologique d’une adenopathie cervicale kystique de l’adulte et sera associe aux examens cytologiques avant prise en charge therapeutique.

Objectives The authors present the guidelines of the French Society of Otorhinolaryngology - Head and Neck Surgery Society on diagnostic procedures for lymphadenopathy in case of a cervical mass with cystic aspect. Methods A multidisciplinary work-group was entrusted with a review of the scientific literature on the topic. Guidelines were drawn up, then read over by an editorial group independent of the work-group, and the final version was drawn up. Guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. Results In adults presenting a cystic cervical mass, it is recommended to suspect cervical lymphadenopathy: in order of decreasing frequency, cystic metastasis of head and neck squamous cell carcinoma, of undifferentiated nasopharyngeal carcinoma, and of thyroid papillary carcinoma (Grade C). On discovery of a cystic cervical mass on ultrasound, architectural elements indicating a lymph node and a thyroid nodule with signs of malignancy should be screened for, especially if the mass is located in levels III, IV or VI (Grade A). Malignant lymphadenopathy should be suspected in case of cervical mass with cystic component on CT (Grade B), but benign or malignant status cannot be diagnosed only on radiological data (CT or MRI) (Grade A), and 18-FDG PET-CT should be performed, particularly in case of inconclusive ultrasound-guided fine needle aspiration biopsy (Grade C).

Limited data is available concerning the safety of active middle ear implants (AMEI) during Magnetic Resonance Imaging (MRI). Measurements in temporal bones are the gold standard for preclinical assessment of device safety. In this study the coupling stability of an actuator as used in a fully implantable AMEI was determined in temporal bones. Eleven temporal bones were implanted with the actuator according to the manufacturer’s surgical guidelines. The actuator was coupled on the incus short process as recommended for sensorineural hearing loss. Temporal bones were exposed 10 times to the MRI magnetic field by entering the MRI suite in a clinically relevant way. Computed Tomography (CT) images were acquired before and after the experiment to investigate the risk of actuator dislocation. Based on the electrical impedance of the actuator, the loading of the actuator to the incus was confirmed. Relative actuator displacement was determined on the CT images by comparing the initial with the final actuator position in 3D space. Impedance curves were analyzed after each exposure to check the loading of the actuator to the ossicles. Analysis of CT images with a 0.30.6 mm in-plane resolution indicate no actuator displacement. The maximum detected change in impedance for all actuators was 8.43 Ω at the actuator’s resonance frequency. Impedance curves measured when the actuator was retracted from the short process after the experiment still indicate the presence of a clear resonance peak. No actuator displacement or dislocation could be detected in the analysis of CT images and the measured impedance curves. Impedance curves obtained when the actuator was retracted from the incus short process still show a clear resonance peak, indicating the device is still functional after the MRI exposures. ispartof: Plos One vol:15 issue:4 ispartof: location:United States status: Published online

Introduction The authors present the guidelines of the French Society of Otorhinolaryngology (SFORL) for clinical and radiological assessment of cystic neck lymphadenopathy of unknown primary in adults. Most cases concern head and neck carcinoma metastasis, often in the oropharyngeal area, or less frequently differentiated thyroid carcinoma or non-keratinizing nasopharyngeal carcinoma. Methods A multidisciplinary task force was commissioned to carry out a review of the literature on the etiological work-up in cystic neck lymphadenopathy in adults: clinical examination, conventional imaging (ultrasound, CT, MRI) and metabolic imaging. Guidelines were drafted based on the articles retrieved, and graded A, B, C or expert opinion according to decreasing level of evidence. Results Oriented clinical examination, cervical and thyroid ultrasound scan and contrast-enhanced neck and chest CT scan are recommended in the assessment of cystic neck lymphadenopathy of unknown primary in adult patients. PET-CT is recommended prior to panendoscopy, to identify the primary tumor. Conclusion Clinical and radiological assessment is fundamental for etiologic diagnosis of cystic neck lymphadenopathy in adult patients, and should be completed by cytological examination before in initiating treatment.

Purpose To describe abnormalities in choroidal and retinal vasculature associated with Val30Met familial transthyretin amyloidosis (V30M-FTA) using fluorescein and indocyanine green (ICG) angiography. Design Prospective, cross-sectional study. Methods This study was conducted at the French National Reference Center for FTA. We included 18 consecutive genetically confirmed V30M-FTA patients (36 eyes) who underwent complete neurologic examination, including staging with polyneuropathy disability (PND) score, and complete ophthalmic evaluation, including staging of intraocular amyloid deposits and fluorescein and ICG angiograms (ICG-A). The grading of choroidal and retinal angiopathy, and their association with neurologic functional impairment, were the main outcome measures. Results Eleven men and 7 women, mean age 61.6 ± 12.1 years, were included. Retinal amyloid angiopathy (RAA) was detected in 24 eyes (92%) of 13 patients, with microaneurysms, retinal hemorrhages, and retinal ischemia of variable extent. Three patients (5 eyes) had neovascular glaucoma and 2 (2 eyes) had preretinal neovascularization. ICG-A indicated choroidal amyloid angiopathy (CAA) in all patients, with 3 distinct patterns—diffuse (9/18 patients), focal (5/18 patients), or punctiform (4/18 patients)—based on the extent of late hypercyanescence along the choroidal arteries. PND scores were significantly higher in patients with diffuse CAA (firework pattern) compared to those with limited CAA (focal and punctiform patterns) (2.89 vs 1.78, P = .045). Conclusion RAA is a frequent and severe complication of V30M-FTA that may lead to anterior and posterior segment neovascularization. CAA was detected in all patients, with a late hypercyanescent delineation of the choroidal arterial vasculature, which was more extensive with increased disease severity.

Points essentiels L’imagerie de l’oreille et de l’os temporal est essentielle a la prise en charge diagnostique et preoperatoire des lesions de l’oreille moyenne. Le scanner est l’examen de choix pour analyser les parois et le contenu de l’oreille moyenne. L’IRM permet de caracteriser les opacites de l’oreille moyenne et d’en evaluer les complications neurologiques possibles. Les techniques modernes d’imagerie permettent un guidage peroperatoire dans la chirurgie otologique. Les implants auditifs ne sont pas toujours une contre-indication a l’IRM mais necessitent alors des precautions en fonction du type d’implant.

Resume Les hemangioblastomes intramedullaires HIM sont de rares lesions representant 1 a 5 % des tumeurs de la moelle epiniere. Le but de ce travail est de presenter les caracteristiques cliniques, radiologiques ainsi que le resultat chirurgical a long terme des HIM. Materiels et methodes Nous avons analyse tous les dossiers de patients operes entre 1993 et 2011 d’un hemangioblastome intramedullaire confirme a l’examen anatomopathologique. Tous ces patients ont beneficie d’un depistage de la maladie de Von Hippel Lindau. Un suivi minimal etait de 3 ans. Les donnees cliniques, radiologiques, chirurgicales et les resultats postoperatoires etaient analyses. Resultats Il s’agit d’une serie consecutive comprenant 59 patients (65 lesions) avec un âge moyen de 38 ans. Quarante-deux patients (72,5 %) etaient porteurs de la maladie de Von Hippel Lindau. Le principal facteur revelateur etait la douleur (58 % des cas). La plupart des tumeurs etait de topographie cervicale. La taille moyenne etait de 15 mm de hauteur. L’exerese chirurgicale etait complete dans 95 % des cas. Cliniquement, une amelioration postoperatoire etait observee dans 12 % des cas, une stabilite dans plus de 86 % des cas et une deterioration dans moins de 2 % des cas. Conclusion Tout patient porteur d’hemangioblastome intramedullaire devrait avoir un depistage pour la maladie de Von Hippel Lindau et si le diagnostic est avere, une surveillance etroite doit etre initiee. En cas de deficit neurologique ou de croissance radiologique, l’exerese chirurgicale doit etre proposee.

Resume Les tumeurs intracanalaires sont des tumeurs rares dont l’evolution est lente, et la symptomatologie clinique variee et peu specifique. Les tumeurs extra-medullaires sont principalement developpees aux depens des gaines nerveuses et de la meninge (schwannome et meningiome respectivement). Les tumeurs intra-medullaires les plus frequentes sont les tumeurs gliales (l’astrocytome chez l’enfant et l’ependymome chez l’adulte), mais il y a de nombreuses autres tumeurs qui ne sont pas si rares. Le diagnostic se fait essentiellement par l’imagerie par resonance magnetique (IRM). L’interet de l’imagerie est de determiner le siege de la lesion. La localisation tumorale a un espace anatomique (epidural, intradural et intramedullaire) permet la meilleure approche diagnostique. Le suivi therapeutique par IRM permet un diagnostic precoce des recidives ou l’evaluation de reliquat.

The lower cranial nerves innervate the pharynx and larynx by the glossopharyngeal (CN IX) and vagus (CN X) (mixed) nerves, and provide motor innervation of the muscles of the neck by the accessory nerve (CN XI) and the tongue by the hypoglossal nerve (CN XII). The symptomatology provoked by an anomaly is often discrete and rarely in the forefront. As with all cranial nerves, the context and clinical examinations, in case of suspicion of impairment of the lower cranial nerves, are determinant in guiding the imaging. In fact, the impairment may be located in the brain stem, in the peribulbar cisterns, in the foramens or even in the deep spaces of the face. The clinical localization of the probable seat of the lesion helps in choosing the adapted protocol in MRI and eventually completes it with a CT-scan. In the bulb, the intra-axial pathology is dominated by brain ischemia (in particular, with Wallenberg syndrome) and multiple sclerosis. Cisternal pathology is tumoral with two tumors, schwannoma and meningioma. The occurrence is much lower than in the cochleovestibular nerves as well as the leptomeningeal nerves (infectious, inflammatory or tumoral). Finally, foramen pathology is tumoral with, outside of the usual schwannomas and meningiomas, paragangliomas. For radiologists, fairly hesitant to explore these lower cranial pairs, it is necessary to be familiar with (or relearn) the anatomy, master the exploratory technique and be aware of the diagnostic possibilities.

Resume Le nerf facial (VII) est issu de noyaux a la partie posterieure du pont. Il est accompagne du VIII dans son trajet cisternal ainsi qu’au niveau du meat auditif interne. Son trajet petreux comprend un segment labyrinthique, un segment horizontal tympanique et un segment vertical mastoidien jusqu’au foramen stylo-mastoidien. Il se poursuit ensuite au niveau de la parotide. L’atteinte pontique s’associe generalement a une autre symptomatologie neurologique. Les lesions de l’angle pontocerebelleux (meningiome et schwannome plus frequemment) donnent en premier lieu une atteinte du VIII, l’atteinte du VII etant au second plan. L’atteinte peripherique (hors contexte traumatique) est le plus souvent due a une paralysie faciale a frigore

Resume Les dernieres paires crâniennes assurent l’innervation du pharynx et du larynx par les nerfs (mixtes) glossopharyngien (IX) et vague (X) et l’innervation motrice des muscles du cou par le nerf accessoire (XI) et de la langue par le nerf hypoglosse (XII). La symptomatologie provoquee par leur atteinte est souvent discrete et rarement au premier plan. Comme pour tous les nerfs crâniens, le contexte et l’examen cliniques, en cas de suspicion d’atteinte des dernieres paires crâniennes, sont determinants pour guider l’exploration en imagerie : en effet, cette atteinte peut sieger dans le tronc cerebral, dans les citernes peribulbaires, dans les foramens ou encore dans les espaces profonds de la face, et la localisation par la clinique du siege probable de la lesion permet de choisir le protocole adapte en IRM et de completer eventuellement par un scanner. La pathologie intra-axiale est dominee dans le bulbe par l’ischemie cerebrale (avec en particulier le syndrome de Wallenberg) et par la sclerose en plaques. La pathologie cisternale est tumorale avec deux tumeurs, le schwannome et le meningiome, dont la frequence est nettement moindre qu’au niveau des nerfs cochleovestibulaires mais aussi leptomeninges (infectieuse, inflammatoire ou tumorale). Enfin, la pathologie foraminale est tumorale avec, en dehors des habituels schwannomes et meningiomes, les paragangliomes. Pour les radiologues, assez reticents a l’exploration de ces dernieres paires crâniennes, il faut dominer (ou reapprendre) l’anatomie, maitriser la technique d’exploration et connaitre les gammes diagnostiques.

The facial nerve (CN VII) emerges from the facial nerve nucleus in the pons. It is accompanied by CN VIII along its cisternal pathway, as well as at the internal auditory meatus. Its petrous pathway includes a labyrinthine segment, a horizontal tympanic segment and a vertical mastoid segment until the stylomastoid foramen. It then continues to the parotid gland. Pontine impairment is usually associated with other neurological symptoms. Lesions of the cerebellopontine angle (most often meningioma and schwannoma) initially result in impairment of CN VIII. The impairment of CN VII takes second place. Peripheral impairment (outside of a traumatic context) is most often due to Bell's palsy.

Resume Le nerf cochleovestibulaire (8e paire crânienne) est un nerf sensoriel. Il est divise en deux nerfs, le nerf cochleaire qui transmet les perceptions sonores et le nerf vestibulaire qui gere l’equilibre. C’est un nerf intracrânien pur, depuis les recepteurs sensoriels situes au niveau de l’oreille interne jusqu’aux noyaux situes dans le tronc cerebral et enfin jusqu’aux aires auditives : gyrus post-central et cortex auditif temporal superieur. Les lesions les plus frequemment responsables d’une atteinte du VIII vont etre les schwannomes vestibulaires. Ce travail rappelle l’anatomie et les differentes explorations de ce nerf.

Skull base osteomyelitis is a rare but serious infection. It typically afflicts immunosuppressed patients and should be suspected in patients with persistent otitis complicated by cranial nerve palsy (VII, IX and XII). The most frequent germ is pseudomonas aeruginosa. Contiguous spread of infection occurs along neurovascular structures and weaker regions of the skull base, then into the soft tissue compartments of the face and nasopharynx. Diagnosis and treatment should be made early for this disease with poor prognosis and high mortality.

The clinical manifestations of vascular lesions of the head and neck may be variable (hemorrhagic, ischemic, compressive). Diagnosis often is made at the time of acute presentation, but delayed manifestations, sometimes long after the initial presentation, should not be overlooked. Hemorrhagic manifestations are characterized by epistaxis, corresponding mainly to lesions of the nasal cavities but involvement of the internal carotid artery should be excluded (life threatening). In addition, some vascular malformations may lead to severe hemorrhage. Ischemic manifestations typically result from arterial dissection. A venous origin is also possible. Carotid-cavernous fistulas rarely lead to hemorrhagic or ischemic manifestations and tend to result in ocular manifestations, typically delayed and sometimes misleading.

Le fratture della rocca petrosa sono frequenti, in quanto le loro cause principali sono gli incidenti stradali. La loro diagnosi e la loro gestione iniziale devono essere realizzate rapidamente in un quadro di urgenza vitale che puo farle misconoscere. La stretta collaborazione tra rianimatori, radiologi e otorinolaringoiatri (ORL) e fondamentale. Le fuoriuscite di liquor cerebrospinale e la paralisi facciale sono le due complicanze la cui gestione iniziale e fondamentale. Tuttavia, le complicanze ritardate richiedono un follow-up clinico e radiologico a lungo termine.

Las fracturas del penasco (porcion petrosa del hueso temporal) son frecuentes, porque sus causas principales son los accidentes de trafico. Su diagnostico y su tratamiento inicial deben realizarse con rapidez en un contexto de urgencia vital que puede hacer que se pasen por alto. La colaboracion estrecha entre reanimadores, radiologos y otorrinolaringologos (ORL) es fundamental. Las fugas de liquido cefalorraquideo y la paralisis facial son las dos complicaciones cuyo tratamiento inicial es fundamental. Sin embargo, las complicaciones diferidas requieren un seguimiento clinico y radiologico a largo plazo.

Cord injuries are frequent and severe lesions resulting in significant disability, most frequently in younger subjects. The area of cord injured results in clinical syndromes (Brown-Sequard, motor and/or sensory deficit...). Cord and rootlet injuries are best depicted on MRI. Diffusion tensor imaging with tractography enables depiction of the most severe cord lesions and some prediction of tissue viability which may provide an idea of the potential functional prognosis and patient recovery. MRI is optimal to demonstrate areas of cord hemorrhage or compression, partial or complete cord transsection, nerve root avulsion...

Intramedullary hemangioblastomas are rare lesions representing 1 to 5% of spinal tumors. The aim of this study was to review our experience with the surgical management of intramedullary hemangioblastomas.We performed a retrospective analysis of all the patients with intramedullary hemangioblastomas operated on between 1993 and 2011 in our department. All the patients were screened for Von Hippel Lindau disease. The minimum follow-up was 3 years. The clinical presentation, radiological findings, surgical procedure and outcomes were recorded and analyzed.Our consecutive series included 59 patients with a total of 65 tumors. The mean age at diagnosis was 38 years. Forty-two patients (72.5%) had Von Hippel Lindau disease. The main symptom was pain (58% of cases). The most common location was cervical spinal cord. The average size was 15mm. The resection was complete in 95% cases resulting in clinical improvement in 12% cases, stability in more than 86% of cases and deterioration in less than 2% cases.All patients with intramedullary hemangioblastoma should have a screening for the Von Hippel Lindau disease and if the diagnosis is correct, close monitoring should be initiated. Surgical removal is strongly advised in cases of neurological deficits or radiological progression of the tumour.

Resume Objectif Illustrer l’interet des sequences tenseur de diffusion et tracking de fibres dans le bilan lesionnel initial d’une myelinolyse centro-pontique et dans le suivi evolutif. Observation Une patiente de 29 ans, en denutrition severe par anorexie mentale a presente une myelinolyse centro-pontique confirmee sur les sequences IRM 1,5 T sagittales T1 et T2. Un tracking de fibres base sur une sequence de tenseur de diffusion 6 directions et centre sur la protuberance, effectue a M0, et M4 a ete compare avec cinq temoins sains, et des mesures du coefficient apparent de diffusion (ADC) et de Fraction d’Anisotropie (FA) ont ete effectuees en zone lesionnelle et comparees aux valeurs normales. Le tracking de fibres a objective une alteration du faisceau cortico-spinal Droit, ceci en concordance avec un discret deficit du Membre inferieur Gauche. Les fibres pedonculaires cerebelleuses moyennes apparaissaient refoulees sans lesion, de meme que les faisceaux posterieurs sensitifs. Ces lesions presentaient un ADC augmente (ADC = 1,58 10 -3 mm 2 /s pour une normale a 0,76 10 -3 mm 2 /s) et une FA diminuee (FA = 0,2 pour une normale a 0,38). Lors des bilans evolutifs, les valeurs d’ADC et de FA ne se sont pas normalisees (ADC = 1,36 10 -3 mm 2 /s, FA = 0,22), alors que la patiente s’est amelioree cliniquement et que l’aspect du faisceaux cortico-spinal gauche s’est normalise sur le tracking de fibres. Conclusion Le tenseur de diffusion et le tracking de fibres sont un nouvel outil permettant l’evaluation qualitative des faisceaux de la substance blanche. Il peut etre utilise pour evaluer la localisation precise et l’extension des lesions dans le bilan initial des myelinolyses centro-pontiques ainsi que pour le suivi evolutif.

Resume L'exploration des infections chroniques de l'oreille moyenne repose en premiere intention sur la tomodensitometrie (TDM) des rochers. La TDM permet le diagnostic etiologique des inflammations chroniques ainsi qu'un bilan anatomique precis preoperatoire. L'imagerie par resonance magnetique (IRM) est utilisee en seconde intention en cas de doute diagnostique s'il existe des complications intracrâniennes et en postoperatoire a la recherche d'une recidive ou pour eviter le second temps operatoire, systematique jusqu'a ces dernieres annees.

Wegener's granulomatosis (WG) is a severe and potentially lethal granulomatosis. Even though no specific radiological criteria exist, CT may suggest the correct diagnosis at an early stage. Recent improvement in the prognosis is related to earlier diagnosis, allowing the initiation of efficient and specific treatment before any severe complications occur. We reviewed a series of WG cases in order to establish the CT diagnostic criteria.

The vestibulocochlear nerve (8th cranial nerve) is a sensory nerve. It is made up of two nerves, the cochlear, which transmits sound and the vestibular which controls balance. It is an intracranial nerve which runs from the sensory receptors in the internal ear to the brain stem nuclei and finally to the auditory areas: the post-central gyrus and superior temporal auditory cortex. The most common lesions responsible for damage to VIII are vestibular Schwannomas. This report reviews the anatomy and various investigations of the nerve.

INTRODUCTION Osteoma is a benign slow growing bone tumor with a prevalence of 3% of all benign paranasal sinuses tumors, with a peak incidence between the fourth and sixth decades, mostly involving frontal sinuses.1 PRESENTATION OF CASE We present a case of a large right ethmoidal sinus osteoma in a 12-year-old boy, complaining of frontal headaches and excessive lacrimation of the right eye. CT scan showed a very large tumor in the right anterior ethmoidal sinus (30 mm × 25 mm × 15 mm). DISCUSSION Large osteomas of the paranasal sinuses are usually resected by external approaches. However, the minimally invasive endonasal approach, which minimizes external facial scarring, is challenging for such large lesions in pediatrics. In the presented case, the osteoma was successfully resected exclusively by endoscopy-guided endonasal approach assisted by neuronavigation, with no peri or postoperative complications. CONCLUSION An endoscopic approach assisted with neuronavigation may be a minimally invasive and safe procedure for managing large osteoma of the ethmoidal sinus in pediatrics patients.

Context: Kallmannsyndrome (KS) is characterized by congenital hypogonadotropic hypogonadism (CHH) and an impaired sense of smell related to defective development of the olfactory system. Objective: The aim of the study was to use high-resolution computed tomography (CT) to detect specific abnormalities in the ethmoid bone region surrounding the olfactory bulbs in patients with KS. Patients: Thirty-seven KS patients were compared to normosmic CHH (nCHH) patients (n=15) and controls (n=30) of similar age. Design and Methods: We conducted a prospective study in a single referral center. Subjects underwent CT in bone windows with axial, coronal, and sagittal reconstructions centered on the olfactory fossa (OF) and cribriform plate (CP). We characterized the OF structure by measuring OF height, width, and surface area and a series of angles. The CP foramina were counted bilaterally. Olfactory bulb magnetic resonance imaging, performed in parallel, was compared with CT findings. Results: OF height, width, and surface area were all significantly lower in KS patients than in nCHH patients and controls (P < .0001). KS patients also had wider angles than nCHH patients and controls (P < .0001). KS subjects with olfactory bulb agenesis on magnetic resonance imaging or who harbored KAL1 mutations had the most marked changes in OF measurements and angles. Coronal OF height distinguished KS patients from controls with the best sensitivity and specificity. The mean number of CP foramina was similar in KS, nCHH, and control subjects. Conclusions: KS is associated with specific ethmoid bone abnormalities. The preserved number of CP foramina in KS patients suggests that the integrity of olfactory structures is not mandatory for their formation during fetal development or their maintenance in adult life.

Skull base osteomyelitis is a rare but serious infection. It typically afflicts immunosuppressed patients and should be suspected in patients with persistent otitis complicated by cranial nerve palsy (VII, IX and XII). The most frequent germ is pseudomonas aeruginosa. Contiguous spread of infection occurs along neurovascular structures and weaker regions of the skull base, then into the soft tissue compartments of the face and nasopharynx. Diagnosis and treatment should be made early for this disease with poor prognosis and high mortality.

The clinical manifestations of vascular lesions of the head and neck may be variable (hemorrhagic, ischemic, compressive). Diagnosis often is made at the time of acute presentation, but delayed manifestations, sometimes long after the initial presentation, should not be overlooked. Hemorrhagic manifestations are characterized by epistaxis, corresponding mainly to lesions of the nasal cavities but involvement of the internal carotid artery should be excluded (life threatening). In addition, some vascular malformations may lead to severe hemorrhage. Ischemic manifestations typically result from arterial dissection. A venous origin is also possible. Carotid-cavernous fistulas rarely lead to hemorrhagic or ischemic manifestations and tend to result in ocular manifestations, typically delayed and sometimes misleading.

Cord injuries are frequent and severe lesions resulting in significant disability, most frequently in younger subjects. The area of cord injured results in clinical syndromes (Brown-Sequard, motor and/or sensory deficit...). Cord and rootlet injuries are best depicted on MRI. Diffusion tensor imaging with tractography enables depiction of the most severe cord lesions and some prediction of tissue viability which may provide an idea of the potential functional prognosis and patient recovery. MRI is optimal to demonstrate areas of cord hemorrhage or compression, partial or complete cord transsection, nerve root avulsion...

Purpose To study the trigeminal fibres (TGFs) using diffusion tensor magnetic resonance imaging (DTMRI) in relapsing herpetic kerato-uveitis. Methods The TGFs of 11 patients (average age: 57+/-9 years), addressed for relapsing unilateral kerato-uveitis related to Herpes simplex virus (HSV) or varicelle-zoster virus (VZV), were tested using DTMRI. The diagnosis of HSV- or VZV-related disease was based on PCR or specific antibodies production within the aqueous humor, or on medical history (zoster). At the time of DTMRI, the kerato-uveitis was in a quiescent period (no recent increase of inflammation). Data were retrospectively assessed by a blinded examiner to compare the affected-side with the non-affected one and with data from sex- and age-matched normal subjects. Results The DTRMI processing showed a significant difference in the mean isotropic fraction within trigeminal fibres innervating the affected side, compared to the non-affected side (0.41 versus 0.49, p = 0,003). There also was a trend in difference between results of the affected side of patients and those of control subjects (0.41 versus 0.44, p =0,12), whereas it was not the case for DTRMI results of non-affected sides (p=0.23). Conclusion DTMRI is an emerging technique that allows an accurate analysis of neuronal networks in patients. Our results suggest that multiple HSV or VZV reactivation episodes induce persistent trigeminal fibres injuries (as shown by reduction of the mean isotropic fraction), that probably reflects a significant decrease in the amount of nerve endings.

Introduction Le virus varicelle-Zona (VZV) est un virus a ADN du groupe herpes avec un tropisme neuro-cutane. Nous rapportons un cas de meningo-radiculite a VZV mimant un infarctus cerebral. Observation Un homme de 67 ans, aux antecedents d’infarctus du myocarde, a consulte pour la survenue brutale d’une dysphagie avec fausses routes, d’une dysphonie et au second plan d’une hypoacousie gauche. L’examen clinique a montre une atteinte des nerfs crâniens VIII, IX et X gauches (hypoacousie, paralysie de la corde vocale, signe du voile du palais). Il n’a pas ete retrouve d’eruption vesiculeuse. Le patient etait apyretique. Le bilan biologique standard etait normal (absence de syndrome inflammatoire). L’IRM cerebrale non injectee a permis d’eliminer un infarctus du bulbe rachidien. Une prise de contraste leptomeningee des nerfs crâniens IX et X gauches au niveau de leur trajet cisternal et une prise de contraste meningee du meat acoustique interne gauche ont ete retrouvees sur les sequences injectees. L’etude du LCR a mis en evidence une meningite lymphocytaire et une positivite de la PCR VZV. Dans le sang, la serologie VZV etait positive en IgG et la PCR virale etait negative. Le scanner thoraco-abdomino-pelvien etait normal. La serologie VIH etait negative. Un traitement intraveineux par Aciclovir (10 mg/kg/8 h) a ete poursuivi pendant 12 jours, sans amelioration clinique. La ponction lombaire de controle a montre une disparition de la meningite. Discussion La reactivation du VZV peut etre responsable de manifestations neurologiques (meningoencephalite, meningoradiculite, vascularite et cerebellite notamment). L’eruption cutanee est inconstante. L’atteinte des nerfs crâniens IX et X est rarement rapportee. Le diagnostic est porte devant la mise en evidence d’une prise de contraste des nerfs crâniens et la positivite de la PCR VZV dans le LCR. Conclusion Une meningoradiculite a VZV avec atteinte des nerfs crâniens IX et X peut mimer un infarctus cerebral. L’absence d’ischemie a l’IRM cerebrale doit conduire a la realisation de sequences injectees et d’une ponction lombaire.

Background Corneal hypoesthesia is the landmark of HSV and VZV keratitis and can lead to neurotrophic keratitis. Diffusion tensor imaging (DTI) is a new magnetic resonance imaging (MRI) derived technique, which offers possibilities to study axonal architecture. We aimed at assessing the potential impact of recurrent HSV or VZV-related keratitis on the axonal architecture of trigeminal nerves using DTI. Design Prospective non-interventional study. Participants Twelve patients and 24 controls. Methods DTI using MRI of the trigeminal fibers and corneal esthesiometry using the Cochet-Bonnet esthesiometer were acquired for patients affected by unilateral and recurrent HSV or VZV-related keratitis (3 months after the last corneal inflammatory event), and control subjects with no history of ocular or neuronal disease affecting the trigeminal pathways. Main Outcome Measures Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were compared between the 2 eyes of both patients and controls, and correlated with corneal esthesiometry. Results FA was lower in the trigeminal fibers ipsilateral to the affected eye compared to the non-affected side (0.39±0.02 versus 0.46±0.04, P=0.03). This difference was more important than the intra-individual variability observed in controls. Concomitantly, the asymmetry in ADC results was significantly correlated with the loss of corneal sensitivity in the affected eye. Conclusions Corneal hypoesthesia related to HSV and VZV keratitis is associated with persistent modifications in the architecture and functionality of the trigeminal fibers. These results add further explanation to the pathogenesis of HSV and VZV-induced neurotrophic keratitis, which may occur despite an apparent quiescence of the disease.

International audience; OBJECTIVES: To illustrate the value of diffusion tensor imaging and tractography in the diagnosis and follow-up of central pontine myelinolysis. CASE REPORT: We report a case of central pontine myelinolysis in a 29 year old woman, also anorexic, studied using MR Diffusion Tensor Imaging (DTI) and Fibre Tracking (FT) focused on the pons, and compared with the studies of 5 normal volunteers. Tractography showed a swollen aspect of the right corticospinal fiber tract correlating with mild left lower extremity deficit at clinical evaluation. The pontine fibers were posteriorly displaced but intact. The sensory tracts were also intact. Apparent Diffusion Coefficient values were increased and Fractional Anisotropy was decreased in the lesions. Follow up imaging showed persistent abnormal ADC and FA values in the pons although the left cortico-spinal tract returned to normal, consistent with the clinical outcome. CONCLUSION: Diffusion Tensor Imaging MR and Fiber tractography are a new method to analyse white matter tracts. It can be used to prospectively evaluate the location of white matter tract lesions at the acute phase of central pontine myelinolysis and follow up.

We prospectively evaluated 30 patients with CNS cavernous angioma with a high field (1.5T) magnetic resonance imager. In all patients the MRI protocol included: gradient echo T1*-weighted imaging, spin echo T2*-weighted imaging and gradient echo T2*-weighted imaging. We evaluated each case for the number of lesions detected on each sequence. Gradient echo T2*-weighted imaging with a long TE (TE: 35ms) was the most sensitive sequence for the detection of cavernous angiomas allowing detection of small lesions that were not identified on the other sequences.

In a chronic alcoholic patient with progressive confusion, which was consistent with the clinical diagnosis of Wernicke encephalopathy, T2-weighted, FLAIR and diffusion weighted (DWI) MR imaging depicted brain abnormalities located in both medial thalamic nuclei. Apparent Diffusion Coefficient (ADC) measurements in these regions shown unexpected normal values, referring to Wernicke pathological findings and DWI data. DWI may be helpful to diagnose early basal nuclei abnormalities, but may fail to compute ADC values in these locations.

The central canal of the spinal cord is present at birth and becomes progressively obliterated. Cadaver studies have shown that it may persiste partially or completely. To our knowledge, this entity has not been described on MRI. We reviewed 794 MRI studies of the spinal cord, and found 12 patients (aged 14 to 65 years) who had an intramedullary cavity. The cavity was at the junction of the ventral 1/3 and dorsal 2/3 of the spinal cord, except at the level of the lumbar enlargement, where it was central. It was filiform in most cases, although sometimes fusiform (3 to 4 mm in diameter), and had regular contours. The cavity were thoracic in 69 % of cases. The clinical features were totally unrelated to the image, and there were no anatomical factors (Chiari malformation, dysraphism) predisposing to syringomyelia. The images were perfectly compatible with a persistent central canal, which we interpret as a variant of normal anatomy. Therefore it is important to regard these findings as normal, to avoid unnecessary treatment and follow-up.

The neurocysticercosis is an infestation of the central nervous system by larvae of Taenia solium. The disease is endemic in a few countries of Latin America, Asia, and Africa and is becoming increasingly prevalent in the United States and Europe. The immigration of individuals from endemic areas and the tourism in these areas require a good knowledge of this disease. A retrospective study evaluating the neurocysticercosis lesions with MR and CT is proposed. Parenchymal, subarachnoid and intraventricular cysts are evaluated. We were able to recognise four CT and MR stages. MR was useful in detecting the cysts of neurocycticercosis in the first stage but was inferior in the detection of parenchymal calcifications.

Objectifs pedagogiques Connaitre les indications et les protocoles de realisation de l’imagerie devant une infection cervico-faciale chez l’adulte. Connaitre les portes d’entree de ces infections et les voies d’extension (espaces cervico-faciaux de diffusion, hematogenes et lymphatiques). Connaitre les formes d’extension (encephaliques et orbitaires, thoraciques) et les complications vasculaires. Connaitre les indications, les techniques et les protocoles d’imagerie devant une infection cervicale en pediatrie. Savoir evoquer une anomalie congenitale devant une infection cervicale chez l’enfant. Connaitre les indications de l’imagerie des sinus dans la pathologie infectieuse de l’enfant. Savoir explorer les complications aigues des otites chez l’enfant.

MRI is the best imaging method to evaluate syringomyelia. It is important to study from the posterior cranial fossa to the sacro-lumbar region and also the supra-tentorial structures. This complete analysis is essential to classify the syringomyelia and to investigate other associated malformations. Radiographs and CT scan are useful to analyze bone structures. For MRI, the new sequences with phased-array coils are also very important to study the entire spinal cord and the posterior fossa. It is essential to study the spinal cord with sagittal and axial spin echo T1 and fast spin echo T2 weighted images with sometimes coronal view, particularly when the patient presents a scoliosis, to have a correct morphological and functional evaluation. MRI gives an excellent study of the spinal cord with an excellent analysis of a primitive or foraminal syringomyelia, but also traumatic, infectious or post arachnoiditis syringomyelia. Spin echo T1 weighted images with injection of gadolinium can be used if an intra-medullary tumor is suspected. MRI is also useful for the post-operative follow up to evaluate the persistence of the medullary cyst and the enlargement of the foramen magnum.

Results of CT and MRI explorations in 8 patients with cerebrospinal fluid (CSF) fistulae are reported and compared with surgical findings in 7. Sensitivity of MRI (CISS and fast T2 spin-echo sequences) was excellent giving perfect correlations with surgical findings in 7/7 cases. CT and MRI were found to provide complementary information suggesting they should be used in combination as first intention explorations of suspected CSF fistulae. Computerized cisternography should be used if MRI is contraindicated or if a clinically and biologically proven CSF fistulae is not visualized by CT or MRI.

Objectifs Connaitre l’anatomie du nerf trijumeau. Maitriser le choix des techniques d’exploration. Savoir explorer une algie faciale en IRM. Connaitre la gamme diagnostique des atteintes trijeminales. Points cles L’innervation sensitive de l’angle mandibulaire et de la region retro-auriculaire est assuree par une branche d’origine rachidienne (C2) et non par le V. L’exploration d’une algie faciale en IRM doit comporter des coupes T2 descendant jsuqu’au niveau de C2. La realisation d’une sequence T2 haute resolution est systematique, en particulier a la recherche d’un conflit neurovasculaire. Les croisements pathogenes nerfs vaisseaux siegent a quelques millimetres de l’emergence protuberantielle du V. Il faut toujours analyser les muscles masticateurs dont l’atrophie temoigne d’une atteinte du V3. Resume L’exploration d’une algie faciale passe par la connaissance de l’anatomie du nerf trijumeau, de son noyau tres etendu (jusqu’au niveau de C2), a sa portion cisternale, au ganglion trijeminal jusqu’a ses trois branches de division et aux foramen de la base permettant le passage de ces branches. L’IRM est l’outil principal de cette exploration : sa realisation doit comporter une exploration encephalique complete descendant jusqu’en cervical haut, une sequence en T2 haute resolution qui permet entre autres la mise en evidence d’un conflit neuro-vasculaire, une sequence sans et avec injection en coupes fines. Le scanner peut completer cette exploration pour mettre en evidence les eventuelles lesions osseuses ou dentaires. Cette exploration doit rechercher : une pathologie intra axiale (la scslerose en plaque, une pathologie vasculaire, une syringomyelie), une lesion de la portion cisternale (conflit neurovasculaire, tumeur), du cavum trijeminal et du sinus caverneux (pathologie de voisinage : anevrysme, infection, adenome…) ou enfin des branches de division (infection ou extension tumorale perineurale).

The authors report an exceptional case report of tumor like evolution of a completely thrombosed aneurysm of the right vertebral artery suggestive of neurinoma of the XII nerve. We describe CT, MRI, MR-angiography. The diagnosis has been established by pathologic study after surgical extraction. Our case demonstrate the possibility of growth of totally thrombosed aneurysms and we discuss various mechanisms.

Magnetic resonance imaging (MRI) gives an accurate analysis of Meckel's cave variability. Images were acquired in 50 patients with several sections for anatomical comparison. Using several sections, MRI is a suitable method for better analysis of the trigeminal cistern. The most frequent findings are symmetrical trigeminal cisterns. Expansion of Meckel's cave or its disappearance has pathological significance.

Fifty files were evaluated to determine the normal anatomy of the cranial nerves. All the cranial nerves were studied including the labyrinth, in different planes with a 3DFT-CISS imaging technique. The 3DFT-CISS is especially interesting to study cranial nerves because of the excellent contrast with CSF-fluid and the possibility of thin sections. It might be essential for the diagnosis of neuralgia and cranial nerves paralysis.

We present a retrospective study in order to analyze the abnormalities noted on MRI in 27 cases of myelopathy excluding tumors, explored between 1994 and 1996. The different lesions were: Multiple Sclerosis (n = 11), Spondylotic myelopathy (n = 3), Neurosarcoidosis (n = 4), CMV Myelitis (n = 1), Radiation Myelopathy (n = 1), Spinal Dural Arteriovenous Fistula (n = 1), Intramedullary Cysticercosis (n = 1), Infarct (n = 5). The exams have been made on 1.5 Tesla Magnetom Vision Siemens or GE Signa machine. All patients have had axial and sagittal views with coronal complementary study in 4 cases. Sequences were Spin echo pT1 (TR: 560, TE: 12), Fast Spin echo pT2 (TR: 3 500, TE: 99 or 128), and gradient echo pT2 (TR: 700, TE: 22, Angle: 25 degrees). Intravenous injection of Gadolinium has been made in 16 cases (0.1 mmol/kg). We have studied the presence or not of a signal abnormality in pT1 and/or in pT2, of enhancement, and its topography (cervical, thoracic, lumbar). We classified lesions in central and/or peripheral and according, to their topography in anterior, posterior or lateral type. The form has been classified in four types (nodular, triangular, "pen like", plage). Extension in transversal (superior or inferior to half medullary surface) and cranio-caudal directions (inferior to one vertebrae, between one and two vertebrae, superior to two vertebrae) has been also classified. Others intra or perimedullar and encephalic abnormalities have been noted. We analyzed the results for each pathology and underline the essential diagnosis criteria noted (low cranio-caudal and transversal extension with frequent triangular form of Multiple Sclerosis lesions, frequent suggestive abnormalities of the encephale (82%) in Multiple Sclerosis, intra and perimedullar enhancement with deformations of the surface of the spinal cord in Sarcoidosis' lesions, extended dorsolumbar "pen like" lesions with inconstant enhancement of infarcts, focal plage lesions centered on degenerative changes of the spinal canal in spondylotic myelopathy, bony lipomatous involution in front of intramedullary radiation plage lesion...) and also review the literature and confront their results to it. We insist on the difficulties in classifying myelopathy (radio-clinical terminology discordances, identical signal abnormalities frequently caused by different illness, necessity to compare to pathologic results). We propose a MRI study protocol that should interest the whole spinal cord and comport T1 weighted without and after gadolinium sequences, T2 weighted sequences (with always a gradient echo type). 2 or better 3 different plans should be made. A complementary study of the brain by MRI is often useful. Clinical study, biology, evolution, MRI and when possible pathology all are necessary to better understand myelopathy's mechanisms.