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Biomedical subjects

N Martin-Duverneuil

Publications and source records attributed to N Martin-Duverneuil.

At least 19 recordsLinked to original sources

MRI features of neurodegenerative Langerhans cell histiocytosis.

CNS complications of LCH include "space occupying" lesions corresponding to histiocytic granulomas and "neurodegenerative" presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH.

Adolescent↗

Facial malignant peripheral nerve sheath tumors.

BACKGROUND/PURPOSE: Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, only sporadically reported involving the maxillo-mandibular region (ten cases with CT or MR data). We present here five additional cases with CT and MR findings along with an extensive review of the literature. RESULTS: Accurate diagnosis of MPNSTs is difficult because pathological like radiological criteria are often non specific. Radiological features display a large spectrum of abnormalities from a well-delineated heterogeneous appearance simulating benign schwannoma to extensive erosive patterns. Their development along the mandibular nerve, the absence of any target or central dot sign, their strong predominant peripheral enhancement must suggest the diagnosis of MPNSTs while irregular bone destruction or the detection of poorly defined margins with muscular infiltration are the most reliable criteria of malignancy. Unfortunately, MPNSTs also display a considerably varied histology. Careful clinical and radiological correlation should bring pathologists to examining large samples of the lesion to better evaluate the overall organisation of the lesion and detect some evocative criteria often only present in some areas of the sample as the peculiar curlicue or whorled arrangement of the spindle cells or the alternation of densely cellular fascicles with hypocellular, myxoid zones. Focal, limited immunostaining for S-100 protein is one of the most important additional criterion. CONCLUSION: If accurate early diagnosis often remains difficult, careful correlation of clinical, pathological and radiological data should in most cases suggest a diagnosis of MPNSTs which display a poor prognosis and requires early and adapted treatment.

Adult↗

[Primary central nervous system lymphoma presenting with central neurogenic hyperventilation. A case report and review of the literature].

INTRODUCTION: Central neurogenic hyperventilation (CNH) in an awake patient is a rare entity. OBSERVATION: We report here a 54-year-old patient who developed central neurogenic hyperventilation as the initial presentation of a primary central nervous system lymphoma located in the brainstem. CONCLUSION: The patient's hyperventilation resolved completely with chemotherapy for primary CNS lymphoma. Most of the cases reported in the literature are related to a diffuse tumor of the brainstem with an intriguing overrepresentation of primary CNS lymphoma. The pathogenesis of CNH is discussed.

Brain Stem Neoplasms↗

Correlations between molecular profile and radiologic pattern in oligodendroglial tumors.

OBJECTIVE: To investigate possible correlations between tumor location and genetic alterations in a series of oligodendrogliomas. METHODS: A series of 158 consecutive oligodendrogliomas were retrospectively reviewed. In each case, the radiologic picture and the chromosome 1p (chr 1p) status of the tumor detected by the loss of heterozygosity technique were analyzed. Correlation between tumor location and molecular profile was made by chi2 tests. RESULTS: Eighty-eight of the 158 patients had low-grade oligodendrogliomas, and 70 had anaplastic oligodendrogliomas. Overall, oligodendrogliomas with chr 1p loss were located preferentially in the anterior part of the brain, whereas tumors with intact chr 1p affected mainly the posterior part of the brain (p = 0.0038). In terms of lobar involvement, a preferential location of oligodendrogliomas with chr 1p loss was found in the frontal lobes as compared with the temporal, parietal, and occipital tumors (p < 0.01). CONCLUSION: There is a significant correlation between loss of heterozygosity on chromosome 1p and tumor location in oligodendrogliomas, suggesting that subtypes of oligodendrogliomas could derive from site-specific precursors.

Adult↗

[Post-irradiation neuromyotonia of the masseter muscle].

INTRODUCTION: Neuromyotonia is a late and rare complication of radiation therapy, consisting of involuntary sustained muscle contractions with a delay in relaxation. OBSERVATION: We report the case of a 68-year-old man who developed neuromyotonia of the masseter muscle 6 years after irradiation for tonsil carcinoma. CONCLUSION: This observation underlines the importance of a correct diagnosis that can lead to an efficient treatment by carbamazepine.

Aged↗

[Radiation-induced aneurysm of the cavernous internal carotid artery].

We report a case of intra-cranial aneurysm of the left cavernous internal carotid artery occurring 27 years after radiotherapy of the pterygopalatine fossa for Hodgkin disease. The development of the aneurysm within the irradiation field, the long latency after radiotherapy, the normality of carotid angiography before radiotherapy and the absence of other etiologies led to the diagnosis of radiation-induced aneurysm. The main characteristics of radiation-induced intra-cranial aneurysms are reviewed.

Carotid Artery, Internal↗

[The sphenoid: tumoral pathology].

The interest and difficulty of sphenoid tumor imaging relate mainly to its great diversity. Detection may be difficult due to variable tumor size and often misleading clinical data. Because the sphenoid is located at the center of the skull base, tumor extension may be quite variable and difficult to assess, requiring sufficient knowledge of anatomy and imaging features to detect tumor spread, especially retrograde perineural spread, since this has a negative impact on prognosis.

Diagnosis, Differential↗

Intravascular malignant lymphomatosis.

Intravascular malignant lymphomatosis is a rare and probably often overlooked disease characterised by massive intravascular proliferation of lymphoid cells, usually with a poor prognosis. CT and MRI appearances are nonspecific; the most suggestive finding being both asymmetrical, bilateral, contrast enhancing high-signal areas on T2 weighting and infarct-like lesions of the cortex and basal ganglia. We report two patients with previously unreported dural and spinal cord involvement.

Adult↗

[Parapharyngeal chondrosarcoma and Ollier's disease: a case report and review of the literature].

OBJECTIVES: Ollier's disease is a constitutional pathology of unknown etiology. It is characterized by bone dysplasia generating numerous enchondromas. The malignant degeneration of this dysplasia is well known. The aim of this article is to study the diagnostic, therapeutic and prognostic characteristics of these lesions. MATERIAL AND METHODS: We report a case of parapharyngeal chondrosarcoma extended to the base of the skull in a patient with Ollier's disease. The treatment was a surgical removal by a cervicotransoral incision combined with a preauricular incision and with a mastoidectomy. It was therefore possible to control the skull base, the parapharyngeal space, the infratemporal fossa and the major neurovascular structures. The removal of the lesion was completed at the level of the clivus and sphenoid with optics (30 and 70 degrees ). We discuss this treatment and the follow up on the bases of literature data. RESULTS: The neoplastic degeneration of enchondromas is estimated between 25 to 50% of cases. The most frequent location is the pelvic bones. Chondrosarcomas are slow growing tumors and their metastatic potential is less significant as we note it in our case report. Their diagnostic is essentially based on histological criteria's and their treatment is surgical. CONCLUSION: Chondrosarcomas of the ENT area occurring with Ollier's disease is rare. Their prognostic is good if the surgical treatment is well done.

Adult↗

Radiographic findings in 37 cases of primary CNS lymphoma in immunocompetent patients.

Because of the increasing incidence of primary central nervous system lymphoma (PCNSL), it is essential to recognize this disease in order to start appropriate treatment. We present the characteristic CT and MRI features of this tumour. The findings of 32 CT and 31 MR of 37 immunocompetent patients with biopsy-proved PCNSL are reviewed. The main features are presented and analysed, and are discussed in comparison with proven literature data. Primary central nervous system lymphoma presents as supratentorial solitary lesions in approximately 80% of the patients and multiple lesions in 20%. In contrast to classical data, the lesions are located in deep structures only in one-third of the cases, and involve posterior fossa in 10% of cases. Most of the lesions are hyperdense or isodense (92%) on CT, hypointense or isointense on T1-weighted images, and only about 40% are hyperintense on T2-weighted images. Nearly all the lesions enhance, except after corticosteroid administration. They produce mild oedema and mass effect. Meningeal or ventricular enhancement are rare but suggestive. Calcification, haemorrhage or necrosis are scarce. Although PCNSL in immunocompetent patients have a variable CT and MR appearance, the imaging data often suggest the diagnosis.

Brain Neoplasms↗

Multiple glioblastomas: CT and MR features.

The aim of this study was to analyze the CT and MR features of multiple glioblastomas, and to determine the best imaging modality for the initial diagnosis. The CT (four exams) and MR imaging (eight exams) of eight patients with proven multiple glioblastomas were reviewed by two neuroradiologists. The lesions were always hypo- or isodense on CT and hyperintense on T2-weighted images (100%). They were usually hypo- or isointense on T1-weighted images (90%). Edema and mass effect were very variable. After contrast media administration, the enhancement was mostly strong (71% on CT and 70% on MR), often either heterogeneous or ring-like. The different lesions of a patient often had a different pattern on MR (75% of cases). Meningeal or ventricular enhancement, suggestive of a possible way of dissemination, was rare. In case of multiple cerebral masses, multiple glioblastomas should be considered as a possible diagnosis in addition to the better known diagnosis of brain metastases, abscesses, or multifocal lymphomas. Moderate edema and mass effect on MR associated with strong and heterogeneous enhancement are suggestive of feature of multiple glioblastomas. Magnetic resonance allows rarely the visualization of a dissemination route.

Brain↗