[Medulloblastoma : management of a usually pediatric tumour in a young adult]. / Médulloblastome : prise en charge d'une tumeur habituellement pédiatrique chez un jeune adulte.

Medulloblastoma is a cerebellar grade IV tumour according to the WHO classification, mainly seen in children under the age of 15. This cancer can nevertheless occur in adults. We report the case of a 22-year-old patient with a medulloblastoma disseminated in the spine. The patient had a type 1 Arnold-Chiari malformation causing hydrocephalus treated by ventriculoperitoneal shunt. The current condition began with perineal and lower limb hypoesthesia, ataxic gait, erectile dysfunction and urinary incontinence. Subsequently, a predominant paraparesis of the right lower limb appeared. The patient was treated according to the PNET HR+5 protocol combining two courses of conventional chemotherapy followed by two courses of high-dose chemotherapy with autograft recovery. Given the excellent response, a proton therapy was then delivered to the whole cerebrospinal axis with boosts to the primary tumour sites. The case of this young adult patient shows on the one hand an atypical presentation, and on the other hand underlines, in the absence of a specific therapeutic strategy established for adults, the importance of collaboration between the adult and pediatric oncology departments, with management integrating innovations such as proton therapy and molecular typing.

Le médulloblastome est une tumeur cérébelleuse de grade IV selon l'Organisation Mondiale de la Santé, principalement observée chez les enfants de moins de 15 ans. Ce cancer peut néanmoins survenir chez l'adulte. Nous rapportons le cas d'un patient de 22 ans présentant un médulloblastome disséminé au niveau du rachis. Le patient est porteur d'une malformation d'Arnold-Chiari de type 1 provoquant une hydrocéphalie traitée par dérivation ventriculo-péritonéale. L'affection actuelle a débuté par une hypoesthésie du périnée et des membres inférieurs, une démarche ataxique, un trouble érectile et des troubles vésico-sphinctériens. Par la suite est apparue une paraparésie prédominant au membre inférieur droit. Le patient a été traité selon le protocole pédiatrique PNET HR+5 combinant deux cures de chimiothérapie conventionnelle suivies de deux cures de chimiothérapie à haute dose avec rattrapage par autogreffe. Vu l'excellente réponse, une protonthérapie a été administrée sur l'axe cérébrospinal avec surdosages sur les sites primaires de la tumeur. Le cas de ce jeune adulte illustre, d'une part, une présentation atypique et d'autre part, souligne, en l'absence de stratégie thérapeutique spécifique établie pour l'adulte, l'importance de la collaboration entre les services d'Oncologie adulte et pédiatrique, la prise en charge intégrant les innovations telles que la protonthérapie et le typage moléculaire.

Tinnitus distress: a paradoxical attention to the sound?

Tinnitus, the perception of sound in the absence of external stimuli, is often a disturbing symptom for which the underlying functional neuroanatomy still remains poorly understood. Most studies have focused solely on functional connectivity changes in the auditory cortex of tinnitus patients. The aim of this study was to investigate whether a correlation exists between tinnitus behavioural scores and functional brain connectivity of five resting-state networks comprising the auditory, the default mode, the external control left and right, and the salience network. For this purpose, a large sample of one hundred and thirty-five subjects underwent resting-state functional magnetic resonance imaging and their behavioural scores were obtained using clinical evaluations. Networks were extracted using independent component analysis, and functional connectivity patterns in the extracted networks were evaluated by a graph theoretical approach. The effects of tinnitus for each network were investigated by correlating the graph strength of all the regions with the tinnitus behavioural scores using stepwise fit regression analysis. Results indicated that alterations of functional interactions between key neural circuits of the brain are not limited to one single network. In particular, tinnitus distress showed a strong correlation with the connectivity pattern within and between the right executive control network and the other four resting-state networks, indicating that tinnitus distress is probably the consequence of a hyperactive attention condition. Among the behavioural scores, the strongest correlation was observed between age and hearing loss, while the tinnitus objective loudness was not correlated with any behavioural scores.

Connectivity graph analysis of the auditory resting state network in tinnitus.

Thirteen chronic tinnitus patients and fifteen age-matched healthy controls were studied on a 3T magnetic resonance imaging (MRI) scanner during resting condition (i.e. eyes closed, no task performance). The auditory resting-state component was selected using an automatic component selection approach. Functional connectivity (correlations/anti-correlations) in the extracted network was portrayed by integrating the independent component analysis (ICA) approach with a graph theory method. Tinnitus and control groups showed different graph connectivity patterns. In the control group, the connectivity graph was divided into two distinct anti-correlated networks. The first one encompassed the auditory cortices and the insula. The second one encompassed frontoparietal and anterior cingulate cortices, brainstem, amygdala, basal ganglia/nucleus accumbens and parahippocampal regions. In the tinnitus group, only one of the two previously described networks was observed, encompassing the auditory cortices and the insula. Direct group comparison showed, in the tinnitus group, an increased functional connectivity between auditory cortices and the left parahippocampal region surviving multiple comparisons. We investigated a possible correlation between four tinnitus relevant measures (tinnitus handicap inventory (THI) and tinnitus questionnaire (TQ) scores, tinnitus duration and tinnitus intensity during the scanning session) and the connectivity pattern in the tinnitus population. We observed a significant positive correlation between the beta values of the posterior cingulate/precuneus region and the THI score. Our results show a modified functional connectivity pattern in tinnitus sufferers and highlight the role of the parahippocampal region in tinnitus physiopathology. They also point out the importance of the activity and connectivity pattern of the posterior cingulate cortex/precuneus region to the development of the tinnitus associated distress. This article is part of a Special Issue entitled: Tinnitus Neuroscience.

Ectopic ureter associated with renal dysplasia.

Two patients, aged 22 and 68, were admitted for recurrent orchi-epididymitis and septicemia respectively. On digital rectal examination, a right pararectal mass was palpated. CT showed in both patients unilateral renal agenesia with a dilated blind ectopic ureter and an enlarged pseudocystic seminal vesicle. MRI obtained in one patient demonstrated a hyperintense content of the blind ureter and the seminal vesicle. Cross section imaging findings were in agreement with deferentography. Histology confirmed the diagnosis of renal dysplasia, with a blind ectopic ureter, opening in the seminal vesicle.

Unusual CT and MRI appearance of carbon monoxide poisoning.

Unilateral low attenuation areas within the right putamen, globus pallidus and thalamus were observed on CT in a patient after exposure to carbon monoxide. A transient bilateral appearance was found on subsequent CT examinations. Hemorrhagic infarction of the right putamen, and ischemic lesions in both thalami were visualized on MRI 2 weeks later.

[Inflammatory spondylodiscitis as a unique radiological manifestation of the SAPHO syndrome]. / La spondylodiscite inflammatoire comme manifestation radiologique unique du SAPHO.

We describe two cases of SAPHO with an exclusive spinal involvement. Diagnosis was established by sterno-clavicular arthralgias, palmar and plantar pustulosis and by radiological signs of inflammatory spondylodiscitis and vertebral osteitis. Spondylodiscitis and medullary edema resolved as shown by MRI after administration of steroids in one case and methotrexate in the other.

Percutaneous catheter drainage of external fistulas of the pancreatic ducts.

The aim of this study was to describe catheterization techniques and report the results of percutaneous drainage of external pancreatic fistulas. Twenty patients with external pancreatic fistulas in whom medical therapy had failed, were referred for radiologically guided treatment. Fifteen patients had postoperative and five primary fistulas. Sixteen were high-output fistulas (H-OF) and four were low-output fistulas (L-OF). All patients were treated percutaneously. Percutaneous catheter drainage was successful in 16 of 20 patients (80 %). The fistula healed in 13 of 15 postoperative cases (86.6 %) and in three of five primary fistulas (60 %). Treatment was successful in 14 of 16 patients (87.5 %) with H-OF and in two of four patients with L-OF. Percutaneous catheterization of the pancreatic ducts was successful in eight of 20 patients (40 %); seven of these patients were cured. Catheterization was not achieved in 12 patients and treatment failed in three (25 %). Conservative treatment of external pancreatic fistulas with percutaneous catheter-directed drainage is thus a reasonable alternative to surgery, particularly in patients with H-OF.

Neurosarcoma of the face: MRI.

Neurosarcoma is a rare tumour originating from the sheath of peripheral nerves. Facial lesions have been reported in about 20 patients. We describe the MRI appearances of neurosarcoma with histological correlation in three patients. The lesions lay in the submandibular region, the left parapharyngeal space and the right orbit. MRI showed a well-defined mass with mixed components. The lesions were moderately heterogeneous on T1-weighted images in two cases and on T2-weighted images in all cases. Gadolinium enhancement occurred in all cases to variable degrees. In two cases, small high signal foci were seen on T2-weighted sequences. MRI appearances of neurosarcoma are not specific.