Primitive cerebral melanoma: a diagnostic and management challenge. About 2 cases.

In this report of two cases of solitary cerebral meningeal melanoma, a rare tumor that presents both diagnostic and management challenges, the diagnosis of these lesions was based on a solitary leptomeningeal mass on MRI, a high mitotic rate on histology and the absence of extracerebral localizations. Although the radiological patterns can mimic those of other melanocytic tumors, MRI is a useful diagnostic tool for narrowing the differential diagnosis. Surgical removal remains the only effective treatment of these lesions, and can lead to prolonged survival in a few cases.

[Clinical, radiographic, prognostic and therapeutic aspects of demelinating disease with tumefactive demyelinating lesions]. / Aspects cliniques, radiologiques, pronostiques et thérapeutiques des lésions démyélinisantes extensives.

BACKGROUND: Demyelinating diseases presenting with a tumefactive demyelinating lesion (TDL) raise questions about classification, diagnosis, prognosis, and treatment. Their long-term course is not well described in literature. PATIENTS/METHODS: In a retrospective study, we describe the main characteristics of 29 patients with TDLs. In a case control study, we compared two cohorts of multiple sclerosis (MS) patients: 24 MS patients with TDL versus a reference cohort of patients with relapsing remitting MS. We compared the extended disability status score (EDSS) concerning the first demyelinating event (DE) with TDL, EDSS score at the end of follow-up and treatment intake. The objective was to discuss the prognosis and the management of TDL. RESULTS: In our study, the prognosis was better for patients with non-prevalent TDL (first DE without TDL) compared with patients with prevalent TDL (first DE with TDL) and was not different compared with the MS reference cohort. At the end of follow-up, there was no significant difference between patients treated with immunosuppressors after a first DE with TDL and patients with classical MS. The EDSS at the end of follow-up was statistically more severe for untreated patients after a first DE with TDL than for classical MS patients (P=0.0047). DISCUSSION: The prognosis of patients with TDL is difficult to assess because of its multifactorial nature (underlying disease and treatment impact). In our cohort, outcome of MS patients whose first severe DE involved a TDL was better when they received an early immunosuppressive treatment.

[Bilateral optic neuropathy with loss of vision after an influenza vaccination in a patient suffering from mixed connective tissue disease]. / Névrite optique rétrobulbaire bilatérale cécitante après vaccination contre la grippe saisonnière chez une patiente présentant un syndrome de Sharp.

BACKGROUND: Optic neuropathy is a rare adverse reaction to vaccination. CASE REPORT: A 62-year-old-woman was hospitalized for bilateral optic neuropathy with loss of vision. The symptoms occurred 15 days after a seasonal influenza vaccination. Her past medical history included a mixed connective tissue disease with no immunosuppressive treatment for several years. Investigations did not reveal any obvious cause and the hypothesis of post-influenza vaccination bilateral optic neuropathy was retained although a complication of the connective tissue disease complication could not be ruled out. The patient was given intravenous methylprednisolone 1g daily. At a cumulative dose of 8 g, oral steroids were given and tapered off. DISCUSSION: Few similar case reports have been described in literature. The causal link between vaccination and optic neuropathy thus remains to be confirmed. Clinicians should however consider this etiology.

[Longitudinal study of health related quality of life in multiple sclerosis: correlation with MRI parameters]. / Étude longitudinale de la qualité de vie dans la sclérose en plaques : corrélation aux paramètres morphométriques de l'imagerie.

INTRODUCTION: Health related quality of life (HRQOL) is often affected in multiple sclerosis (MS). Nevertheless, to our knowledge, there is no longitudinal study in the literature about the correlation between MRI parameters and HRQOL in MS patients. METHODS: We included 28 patients with clinically definite relapsing remitting MS. All patients initiated subcutaneous interferon beta-1a therapy. To assess HRQOL, we used the SEP-59 scale, the French validated translation of MSQOL-54, and the MusiQoL scale. Conventional MRI was performed every year. Lesion load (LL) and brain atrophy were automatically measured using SepINRIA, a free software developed by INRIA in Sophia Antipolis. RESULTS: The mean EDSS score was 1.7 and disease duration was 2.5 years. Our results revealed that HRQOL was significantly correlated to T1 and T2-LL with both SEP-59 and MusiQoL scales. T1-LL was better correlated with physical dimensions and T2-LL was better correlated with mental components. At 1-year follow-up, patients whose MRI showed either an increase of T1 LL or at least one gadolinium enhancing lesion had a worse HRQOL at the end of the study. Initial brain parenchymal fraction (BPF) measure was also correlated with the long-term follow-up HRQOL. EDSS scored at the end of the study had not significantly changed (1.3; P>0.05). CONCLUSION: Our study revealed pertinent clinicoradiological correlations between HRQOL and MRI parameters in our cohort.

[De Seze et al. criteria: application to a series of 14 patients presenting a first severe acute demyelinating event]. / Application des critères de de Seze et al. sur une série de 14 patients ayant présenté un premier événement démyélinisant aigu sévère.

INTRODUCTION: The application of de Seze et al. criteria (2007) to patients presenting a first severe acute demyelinating event helps to distinguish acute disseminated encephalomyelitis (ADEM) from other CNS inflammatory diseases, with 83% sensitivity and 95% specificity. We applied these criteria to 14 patients who presented a first severe acute demyelinating event and whose later clinical course enabled clear identification of the neurological diagnosis. METHOD/PATIENTS: This study concerned 14 patients who presented a first acute demyelinating event. Initially, there were 16 patients but two were excluded because their initial clinical condition (isolated acute retrobulbar optic neuritis in one and acute cervical myelitis in the other) would have excluded them in the princeps article. We identified 11 women (78.6%) and three men (21.4%) with a mean age of 33.7+/-12.5 years. Follow-up ranged from three months to 11.5 years after the initial episode (average four years). At last follow-up, the diagnosis was ADEM in seven patients (50%) and multiple sclerosis (MS) in seven (50%). Five of seven patients in the MS group had a tumor-like presentation (71.4%), this parameter partly explaining the initial discrepancy in diagnosis. When applied to our series, de Seze criteria for ADEM exhibited 85.7% sensitivity and 71.4% specificity. CONCLUSION: Applying the new criteria, we did not find the same sensitivity, specificity, and positive and negative predictive values as in the original article. The lack of specificity arose from the misclassification of MS patients with a tumor-like presentation (two out of five false negatives). One of the explanations is that the clinical criteria used can be part of atypical forms of MS, in particular in its tumor-like presentation. De Seze et al. criteria can be an invaluable help for the clinician in the diagnosis of a first severe demyelinating event. Considering our results, these criteria should not be applied for patients with a tumor-like form of MS. A prospective study in a larger cohort is needed to confirm or invalidate these preliminary results.

[Approaches to segment multiple-sclerosis lesions on conventional brain MRI]. / Revue des approches de segmentation des lésions de sclérose en plaques dans les séquences conventionnelles IRM.

Different sets of criteria are currently used for the diagnosis of multiple sclerosis (MS). Some are based on clinical features, while others are related to imaging findings. Among the image processing systems, specific criteria include spatial dissemination of lesions in one image or their temporal dissemination in images acquired at different time points. In addition, the evolution of the lesion load can be used to evaluate treatment efficiency in MS clinical research. Consequently, obtaining a precise segmentation of the MS lesion appears to be crucial. In the literature, a number of semi-automated or completely automated approaches have been proposed enabling a reduction of the inter- and intra-expert variability for manual delineations. A comprehensive state-of-the-art classification of the most representative systems is presented here.

Treatment of newly diagnosed symptomatic pure low-grade oligodendrogliomas with PCV chemotherapy.

Based on studies relating to anaplastic oligodendroglioma (OG) chemosensitivity and benefit of time to progression or overall survival, chemotherapy for pure OG has been proposed. Several studies have reported the efficacy of various chemotherapeutic agents in a small number of patients with low-grade gliomas, e.g. pure astrocytomas, OG or mixed histologies. The 5-year survival rate varies from 61% to 89% with a mean time to progression of 5 years. We report the outcome of 33 consecutive patients with pure low-grade OG diagnosed between 1990 and 2006 systematically treated for residual or non-removable tumor with PCV chemotherapy regimen as the front-line treatment after surgery. All the tumors were low grade (grade II) pure OG according to the WHO classification. All patients were symptomatic at presentation and underwent neurosurgical procedure for histological diagnosis. Response was evaluated by clinical assessment and brain magnetic resonance imaging. Twenty-one men and 12 women with a mean age at pathological diagnosis of 46.5 years were studied. The most common first symptom was partial epileptic seizure (73.7%). Six patients (18%) had initial gadolinium enhancement, associated with methoxyisobutyl (MIBI) hypermetabolism (P < 0.001). The resection was partial in seven cases (21%), and 26 patients (79%) had biopsy only. Eleven patients (36%) had a malignant transformation during the follow-up with a median time to progression of 19 months. Favorable prognostic factors were lack of contrast enhancement (P < 0.0001), and age <40 years (P < 0.0003); 90% of patients were progression-free at 1 year. Survival rates at 2, 5 and 10 years were 85%, 75% and 50%, respectively. Up-front chemotherapy with PCV regimen is a good treatment for symptomatic pure low-grade OG, as it increases the number of progression-free patients and time to progression. These results suggest that radiotherapy could be postponed until the malignant transformation occurs to delay cognitive side effects of irradiation.

[Neurosarcoidosis treated with mycophenolate mofetil: two cases]. / Neurosarcoïdose et mycophénolate mofétil.

INTRODUCTION: Neurosarcoidosis is a rare (5 cases for one million) immune-mediated disease generally observed in young adults. Neurological symptoms are present in the half of patients, and symptoms remain limited to neurological system in 10p.cent. Histological criteria are mandatory to prove the diagnosis. The sensitivity and complications of biopsy are variable. The best sensitivity appears to be achieved with muscle biopsies which in addition have a lower risk of complications. Neurosarcoidosis is usually treated with corticosteroid therapy and immunosuppressive drugs (cyclophosphamide, cyclosporine, aziathoprine, methotrexate), but frequently resists standard schedules. In addition the many contraindications, side effects and cumulated toxicities of immunosuppressive drugs compromises their use. Knowledge of the effectiveness of other treatments would therefore be useful. Mycophenolate mofetil (MMF) has been used for treatment of many immune-mediated neurological diseases, like polymyositis, multifocal motor neuropathy, myasthenia or chronic inflammatory demyelinating polyradiculoneuropathy. MMF is efficient and well tolerated, but there is no case-report about neurosarcoidosis. CASE REPORT: We report two observations of young patients (14 and 27 years) with a diagnosis of resistant neurosarcoidosis treated with MMF (2 g/j) and corticosteroids. A significant and rapid effectiveness was clinically and radiologically observed, with good clinical and hematologic tolerance. CONCLUSION: The MMF seems to be an interesting rescue treatment for neurosarcoidosis. Further evaluation is needed.

[Subthalamic nucleus stimulation using a Fisher ZD stereotactic frame, MR-CT fusion guidance and peroperative orthogonal radiographs, in Parkinson disease]. / Evaluation de la stimulation du noyau sous-thalamique utilisant un cadre de Fischer adapté, un guidage par fusion IRM-scanner et un contrôle radiographique stéréotaxique peropératoire, dans la maladie de Parkinson.

BACKGROUND: We present the method and results of an original technique to implant electrodes in the subthalamic nucleus (STN) to treat Parkinson's disease, based on adaptations of the Fisher ZD stereotactic frame. METHODS: Targets coordinates were calculated after fusion of stereotactic CT-scan and MRI images. STN was localized by its theoretical coordinates according to AC-PC and by its direct visualization on T2 images. Electrodes were implanted after local anesthesia, using peroperative multicanal microrecordings and test stimulation. Electrodes location was checked by peroperative perpendicular radiographs. To avoid projection of the frame arm on the area of interest on anteroposterior and lateral radiographs, the arm was fixed at 45 degrees from the usual 90 degrees position. This original fixation needed a trigonometric transformation of the X and Y stereotactic coordinates. Radiopaque markers, fixed on the frame, were identified on the radiographs, allowing the calculation of the stereotactic coordinates of the electrode tip, which were then entered in the stereotactic MRI, to check its location from the defined target. RESULTS: No problem due to adaptations of the frame occurred in the 60 patients. In all cases, peroperative radiographs allowed to confirm the correct location of electrodes. Six months after surgery, UPDRS III score without medication was decreased by 52% with stimulation "on". UPDRS IV items 32, 33 and 39 scores were decreased by 75,7, 79,5 and 72%. Daily dopa-equivalent dose was decreased by 71%. One asymptomatic thalamic hematoma and two wound infections occurred. CONCLUSION: This method was efficient and safe to implant deep electrodes.

[Early detection of cognitive impairment in relapsing-remitting multiple sclerosis: functional-anatomical correlations and longitudinal follow-up]. / Troubles cognitifs et sclérose en plaques rémittente: intérêt de leur détection précoce: corrélations anatomo-fonctionnelles et suivi longitudinal.

INTRODUCTION: Cognitive impairment is frequent in relapsing remitting Multiple Sclerosis and is often diagnosed after disruption of occupational and social relations. METHODS: We studied at baseline a homogeneous population of 32 RRMS patients, diagnosed for less than 5 years, with spontaneous memory complaints, and 20 controls. Sixteen patients were followed for 2 years, combining physical examination, neuropsychological tests, and brain MRI. Neuropsychological tests used evaluated memory capacities, attentional capacities, executive functions, language, and visuo-constructive praxis. Lesion load on brain MRI was measured with semi-automatic segmentation procedures and manual control. RESULTS: Eighty percent of patients presented cognitive impairment, and this proportion was higher than that found in the literature. These disorders were more marked for verbal episodic memory, attention, and executive functions. Patients with brain MRI that initially fulfilled the Barkhof criteria and those with callous lesions had more memory disorders. No link between global T1 and T2 lesion loads and neuropsychological scores was found. A statistical link between posterior fossa lesions and attentional disorders was shown. In the longitudinal follow-up, patients had better performances in memory and attentional domains, and a lower number of cognitive domains with dysfunction for each patient. This improvement on neuropsychological tests, whereas EDSS levels were stable, underlined a possible test-retest effect. CONCLUSION: During the initial phase of the disease, most of the relapsing remitting patients present a mild cognitive impairment. Early detection, therapeutic propositions, and recognition of disorders are necessary.

[Relapsing remitting multiple sclerosis or multiphasic disseminated encephalomyelitis?]. / Sclérose en plaques rémittente ou encéphalomyélite multiphasique disséminée?

INTRODUCTION: The imaging presentation of some forms of multiple sclerosis may be misleading. In patients with a history of recent infection or vaccination, especially for adolescents or young adults, the differential diagnosis with acute disseminated encephalomyelitis can be difficult. CASE REPORT: We report an unusual clinical and radiological presentation of multiple sclerosis, mimicking acute disseminated encephalomyelitis. We discuss clinical and radiological differential diagnosis, and the outcome after immunosuppressive treatment. CONCLUSION: Distinguishing between acute disseminated encephalomyelitis and the first relapse of multiple sclerosis can be difficult. Brain imaging is a precious tool for differentiating between the two diseases.

[Recurrent aseptic meningitis associated with primary biliary cirrhosis]. / Méningite récurrente aseptique associée à une cirrhose biliaire primitive.

INTRODUCTION: The etiological diagnosis of recurrent aseptic meningitis often requires difficult investigations. We report a case of recurrent aseptic meningitis associated with a primary biliary cirrhosis. CASE REPORT: A 37-year-old woman presented four recurrences of isolated meningitic syndrome with fever. Intravenous steroid treatment was effective. Investigations revealed an old right tympano-labyrinthine fracture with, on MRI, an abnormal enhancement of the posterior wall of the internal auditory canal. Serum tests were also significantly positive for antimitochondria antibodies subtype M2. CONCLUSION: Definitive diagnosis was recurrent puriform aseptic meningitis secondary to an inflammatory non-septic ears-nose-throat focus in a latent autoimmune context of primary biliary cirrhosis. To our knowledge, this is the first case reported in literature.

Ehlers-Danlos syndrome with subependymal periventricular heterotopias.

A 24-year-old woman with Ehlers-Danlos syndrome had complex partial and right sensory motor seizures. MRI showed periventricular subependymal heterotopias, agenesis of the posterior part of the corpus callosum, mega cisterna magna, and aneurysms of the sinuses of Valsalva. This may constitute a new subtype of type 1 Ehlers-Danlos syndrome characterized by X-linked periventricular subependymal heterotopias and epilepsy.

Nontraumatic spinal epidural hematoma: report of four cases and review of the literature.

OBJECTIVE AND IMPORTANCE: Spontaneous spinal epidural hematoma is a rare entity. We report four cases of nontraumatic spinal epidural hematomas observed from 1990 to 1994. CLINICAL PRESENTATION: In two cases, the causes were determined to be an acquired coagulopathy and a vascular malformation; in the other two cases, no causes were detected. We reviewed 85 cases in the literature, with particular emphasis on the various causes detected (coagulopathy, vascular malformations, tumor) and the diagnostic methods used. INTERVENTION: All the patients were investigated by computed tomography and magnetic resonance imaging and underwent surgery. CONCLUSION: We considered the expression "nontraumatic epidural hematoma" to be less ambiguous than "spontaneous spinal epidural hematoma," which is the one that is usually used in the literature and corresponds to various definitions. In the absence of any signs suggestive of vascular malformation on magnetic resonance imaging, preoperative angiography is not essential and need not delay the surgical procedure, because the timing of the surgery, together with the preoperative clinical state, determines the quality of the clinical result.

[Image processing and radiotherapy].

Medical images are of great importance in radiotherapy, which became a privileged application field for image processing techniques. Moreover, because of the progression of the computers' performances, these techniques are also in full expansion. Today, the recent developments of the radiotherapy (3DCR, IMRT) offer a huge place to them. Effectively, they can potentially answer to the precision requirements of the modern radiotherapy, and may then contribute to improve the delivered treatments. The purpose of this article is to present the different image processing techniques that are currently used in radiotherapy (including image matching and segmentation) as they are described in the literature.

[Relapsing-remitting inflammatory disease of the central nervous system with normal MRI: multiple sclerosis or phenocopy in a series of 15 patients]. / Maladie inflammatoire du système nerveux central évoluant par poussées avec IRM conventionnelle normale: sclérose en plaques ou phénocopie? A propos de 15 observations.

Multiple sclerosis is a demyelinating disease of central nervous system. Although many sub-types and clinical forms are identified, diagnosis is clearly related to the detection of MS lesions on brain MRI. We report data of 15 patients admitted in Nice for suspicion of MS after clinical relapsing-remitting or progressive symptoms. Extensive screening tests (i.e blood sample, CSF, MRI, spectroscopy) were performed at onset and at each relapse. All patients had normal-appearing white matter on spinal cord and brain MRI. Nevertheless, 11 patients can be considered as MS according to McDonald criteria.

[Intravenous immunoglobulins for relapsing-remitting multiple sclerosis after failure of treatment with other immuno-modulators]. / Evaluation du traitement par immunoglobulines intraveineuses dans la sclérose en plaques progressive après échappement aux autres traitements immunomodulateurs.

Intravenous immunoglobulins are used in the treatment of different autoimmune diseases. Recent trials suggest their efficacy in relapsing remitting multiple sclerosis. We report the results of an efficacy and safety trial using monthly intravenous injections of immunoglobulins for patients with secondary progressive multiple sclerosis. Eighteen patients in clinical progression, who have been previously treated with immunomodulatory or immunosuppressive drugs, were given monthly intravenous immunoglobulin infusions (0.4 g/kg/d for 5 days). At the beginning, the mean EDSS score was 6.77. At the end of the study, an improvement of EDSS was noted in 61.1 p. cent of patients, with less than 1 and 0.75 for secondary and primary progressive diseases respectively. No worsening was reported. Surprisingly, some patients had partial improvement of neurological functions which were considered as sequelae. Indications for intravenous immunoglobulins in the treatment of the multiple sclerosis need to be evaluated.

[The contribution of automatic anatomical matching of sequential brain MRI scans in the monitoring of multiple sclerosis lesions]. / Intérêt du recalage automatique des IRM cérébrales dans le suivi des scléroses en plaques.

INTRODUCTION: Magnetic resonance imaging (MRI) has transformed management of patients with multiple sclerosis. The exact contribution of brain MRI remains a subject of debate, but it is generally considered to provide a more specific and more sensitive outcome measure for monitoring purposes and for testing new therapies. The choice of MRI techniques, and measurement reproducibility for multiple sclerosis brain lesions are not defined with precision for routine practice. There are many sources of error when comparing successive images which can be overcome to some extent with repositioning and image processing techniques. METHODS: We evaluated the impact of image repositioning on treatment decision-making for twelve relapsing remitting patients. Brain MRIs were performed every three months for a one-year period. Two neurologists interpreted the non-repositioned and repositioned images giving their analysis of changes in the lesions visualized on the T2 sequences and their therapeutic decisions. RESULTS: For the first neurologist, analysis of the non-repositioned images yielded six patients whose lesions had worsened while for the repositioned images there were only three. For the second neurologist, four patients had more lesions with the non-repositioned images and only three with repositioning. The subjective interpretations were the same for the two neurologists when they used repositioned images. CONCLUSIONS: Comparison by two neurologists of non-repositioned and repositioned MRI, with no other image processing, affected the analysis and in certain cases propositions for treatment.

[Treatment of progressive multiple sclerosis with monthly pulsed cyclophosphamide-methylprednisolone: predictive factors of treatment response]. / Association cyclophosphamide-méthylprednisolone en traitement de fond des scléroses en plaques de type progressif: facteurs prédictifs d'une réponse clinique.

INTRODUCTION: Cyclophospamide is used in the treatment of progressive multiple sclerosis. We were looking for predictive indicators of treatment response. MATERIAL AND METHODS: Forty-seven patients with secondary progressive multiple sclerosis and seven others with primary progressive received monthly infusions of cyclophosphamide (750mg/m2) and methylprednisolone (500mg). During the year before cyclophosphamide the EDSS had worsened one point in all patients with or without surimposed relapses. Evaluation was based on EDSS change at 6, 12, 24 months and 5 years. RESULTS: Among secondary progressive patients, 91 per 100 (43/47) were stable or improved at 12 months, 65 per 100 (26/40) at 24 months and 22 per 100 (5/23) at 5 years. Annual relapse rate decreased from 0.81 before treatment to 0.48 during treatment and 0.12 after treatment (p<0.001). At 24 months, efficacy was correlated to a progressive phase lasting less than 5 years (p<0.01) and to a rapid increase of EDSS of at least 2 points the year before treatment (p<0.05). There were no influences of age, EDSS and surimposed relapses at the beginning of treatment, and other immunoactive drugs administrated before cyclophosphamide. There was no significant difference in quality of response to treatment between patients with primary progressive and secondary progressive multiple sclerosis. CONCLUSION: Cyclophosphamide appears to be more efficient in early stage of progressive multiple sclerosis independently of age, relapses or neurological disability scale.

[Castleman's disease located in the central nervous system]. / Maladie de Castleman localisée au système nerveux central.

A 26-year-old woman complained of trijeminal nevralgy and ocular symptoms revealing a paracavernal tumor which had progressed for three months. Histopathological analysis after partial resection led to the diagnosis of Castleman's disease. Six months later, the patient was considered cured after focal adjuvant radiotherapy. Castleman's disease is a lymphoproliferative disorder. Solitary intracranial involvement is unusual. Unlike multifocal disease, localized Castleman's disease has an excellent prognosis.