Erdheim-Chester disease: a sinonasal lesion mimicking rhinoscleroma.
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Diagnosis, Differential↗
Biomedical subjects
K Marsot-Dupuch
Publications and source records attributed to K Marsot-Dupuch.
Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel-Lindau disease.
Von Hippel-Lindau disease (VHL) is a hereditary cancer syndrome caused by germline mutations of the VHL tumor suppressor gene. Major progress has been made in the last decade in both clinical and fundamental aspects of VHL. The VHL gene product, pVHL, has major and multiple functions: pVHL regulates not only first angiogenesis but also extracellular matrix formation and the cell cycle. A molecular diagnosis of VHL is now available, leading to a transformation in clinical management of patients and their families. Diagnosis of VHL has to be suspected in patients with a VHL-related tumor without familial history and especially in case of hemangioblastoma or endolymphatic sac tumors. Such patients should be systematically investigated for clinical and molecular evidence of VHL disease. Treatment of symptomatic hemangioblastomas remains mainly neurosurgical, often in emergency, but stereotactic radiosurgery is emerging as an alternative therapeutic procedure. In the future, antiangiogenic drugs could represent a potential medical treatment of CNS hemangioblastomas in view of their highly vascular structure. Lastly, visceral manifestations of VHL disease are also of critical importance and require early detection for effective treatment.
Central Nervous System Neoplasms↗
[Facial palsy, enhancement of cranial nerves and Lyme disease].
Lyme disease involves multiple organ systems including in 10-15% of cases, the nervous system. Cranial neuropathies are observed in the second stage of the disease. The facial nerve is the most frequently affected nerve (20-30%). Facial nerve enhancement may be associated with cochleovestibular nerve abnormalities and can mimick an intracanalicular pseudomass. We present post-gadolinium enhancement of multiple cranial nerves associated to an intracanicular enhancement illustrated in a patient referred for a facial nerve palsy and presenting a Lyme disease. We discuss the differential diagnosis.
Adult↗
[The foramen rotundum. Anatomy and radiological explorations. Pathology].
The foramen rotundum is a small canal deeply situated in the base of the skull which represents the way of exit of the maxillary nerve, second branch of the trigeminal nerve. Its precise individualization and analyse is difficult and necessitates a precise and adapted technique as well as a precise knowledge of its anatomical relationships. Its represents a frontier area between the endo- and exocranial spaces. Its involvement which is preferentially related with tumoral pathologies (and particularly with retrograde perineural invasion) profoundly modifies the prognosis of the disease and so should allow soon a multidisciplinary therapeutic discussion.
Bone Diseases↗
[Infectious aneurysms of the cavernous carotid artery].
We describe 3 patients who developed infectious aneurysms of the cavernous carotid artery. The aneurysms were due to sphenoidal sinusitis in two patients and due to endocarditis in one. The acute and septic onset of the cavernous sinus syndrome, suggested thrombophlebitis of the cavernous sinus in all 3 patients. The diagnosis was established by magnetic resonance imaging and magnetic resonance angiography. Therapeutic internal carotid artery occlusion was indicated for a fissuration of their aneurysm manifested (n=3) by an episode of epistaxis (n=2) and blood in sphenoid sinus (depicted by MRI) in one case. We discuss the pathophysiology and management of bacterial aneurysms of the cavernous carotid artery. Close clinical and imaging follow-up should be performed for patients under antibiotherapy. Selective angiography with therapeutic occlusion of the carotid artery is discussed in patients with persistence of symptoms or if clinical findings are suggestive of fissuration or if aneurysmal sac diameter increases on follow-up imaging studies.
Adult↗
Haemorrhage in the labyrinth caused by anticoagulant therapy: case report.
We report a patient who experienced a severe vertiginous episode with bilateral tinnitus and progressive right-sided hearing loss. She had Marfan's disease and was on anticoagulant treatment. The fluid in the labyrinth gave higher signal than cerebrospinal fluid on T1-weighted images, suggesting haemorrhage. The radiological follow-up is discussed.
Anticoagulants↗
CT and MR findings of Michel anomaly: inner ear aplasia.
In 1863, Michel described a condition characterized by a total absence of differentiated inner ear structures associated with other skull base anomalies, including an abnormal course of the facial nerve and jugular veins. Michel aplasia clearly differs from Michel dysplasia, in which arrest of embryologic development occurs later. Recently, the role of otic capsule formation on mesenchymal differentiation was reported as well as the impact of the genetic deletion of the homeobox gene on the development of the ear, cranial nerves, and hindbrain. We report two patients with a total absence of inner ear structures bilaterally, illustrating the characteristic appearance of Michel aplasia and associated skull base anomalies.
Adolescent↗
[Brain imaging in AIDS].
Encephalic diseases remain an important problem in AIDS patients despite improvement in the rate of disease spread. We provide data on the current situation and describe the clinical, pathological and imaging features commonly observed in encephalic diseases in AIDS patients.
AIDS Dementia Complex↗
[Imaging of the cochleo-vestibular system].
This article will review the main pathologies affecting the vestibulocochlear system from the sensorineural organs to the cerebral cortex. MR imaging has greatly improved detection of these pathologies. Thin slice MR imaging using different pulse sequences now allows improved depiction of anatomical structures. Vestibular schwannoma is by far the most common lesion, but MR imaging allows detection and characterization of rarer lesions in patients often presenting with similar symptoms.
Brain Diseases↗
SAPHO syndrome of the temporomandibular joint associated with sudden deafness.
We report a case of arthritis of the temporomandibular joint (TMJ) associated with sclerosing osteomyelitis of the mandible and temporal bone, causing deafness. The presence of a palmoplantar pustulosis established the diagnosis of SAPHO syndrome. SAPHO (an acronym referring to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis) syndrome is defined by the association of characteristic osteoarticular and dermatologic manifestations, with diffuse sclerosing osteomyelitis of the mandible being a part of this entity. We review the literature of SAPHO syndrome with mandibular manifestations and discuss the mechanisms of inflammatory spread from the TMJ to the cochlea. To our knowledge, this is the first description of skull base involvement in a patient with SAPHO syndrome leading to sudden deafness.
Acquired Hyperostosis Syndrome↗
[Imaging of post-traumatic tinnitus, vertigo and deafness].
Tinnitus, hearing loss, and more rarely disequilibrium are common sequela of temporal bone trauma. Hemotympanum may cause a transient and immediate conductive hearing loss. HRCT depicts ossicular dislocation (most frequently incus), producing a long-term conductive hearing loss. Labyrinthine trauma causing neurosensory hearing loss or/and acute vertigo may be depicted by MRI, showing an abnormal non-enhancing high signal T1 of the membranous labyrinthine fluid. MRI also may show low signal T1 and T2 fibrotic areas of the membranous labyrinth, especially of interest if cochlear implant surgery is planned. Perilymphatic fistulas are to be searched in case of fluctuant hearing loss. Both HRCT and MRI may show window damage: filling of the tympanic recess, rupture of the window membrane, intra-vestibular luxation of the stapes, or occasionally pneumolabyrinth.
Ear Ossicles↗
Slow-growing labyrinthine masses: contribution of MRI to diagnosis, follow-up and treatment.
We report the use of MRI in the diagnosis, follow-up and therapeutic management of three cases of intralabyrinthine Schwannoma. The diagnosis was based on the history and initial and follow-up MRI findings. The main feature suggesting the diagnosis was a nodular intralabyrinthine mass of low signal intensity on T2-weighted images, and high or isointense signal on T1-weighted images (relative to cerebrospinal fluid), which showed contrast enhancement. Follow-up imaging showed growth of the tumour in one patient. One patient underwent surgery for severe tinnitus. To detect these lesions, MRI should be focussed on the inner ear, using thin-section T2-weighted and T1-weighted images before and after contrast medium. MRI allowed informed surgical planning.
Adult↗
Perichiasmatic granuloma occuring after radical mastoidectomy: MR findings.
A case of chronic chemical meningitis occurring after a radical mastoidectomy is reported. Imaging and surgical findings were suggestive of a dissemination of cholesteatoma debris within the subarachnoid spaces. Chemical meningitis has been described in epidermoid and dermoid cyst rupture. This report illustrates that clinicians should be aware of this possible complication. Skull base imaging is mandatory before considering the diagnosis of idiopathic meningitis. Only treatment of the abnormal communication between cerebrospinal fluid and middle ear may eradicate the origin of this rare meningitis.
Adult↗
[Magnetic resonance angiography of the carotid artery: artifacts, anatomy, diseases].
Magnetic resonance angiography is now a technique commonly used in neurologic practice. We give a brief overview of the biophysical principles of this technique. Recent refinements and technical innovations are also noted. After some anatomic considerations about the carotid artery, we provide some data about the role of MRA in atherosclerotic and non atherosclerotic diseases (dissections, aneurysms, arteritis, post operative follow-up...) of the extra and intracranial carotid arteries. The purpose of this review is to concentrate on the role of magnetic resonance angiography in patients with various carotid artery diseases and to specify possibilities, limitations and risk of misinterpretation. Magnetic resonance angiography is a major, still evolving technique.
Adult↗
[A report of two familial cases of Michel syndrome (bilateral agenesis of the inner ear)].
In two siblings, wearing conventional hearing aid, presenting profound but not total congenital deafness, with no particular antecedents, the imaging destined to confirm the indication of a cochlear implant revealed a total bilateral agenesis of the inner ear. In one of the children, this imaging was confirmed by an exploration of the middle ear performed during a tonsillectomy that was otherwise necessary. We will summarize the literature concerning Michel's Disease, exceptional in its princeps form, and we will discuss the manner of action of conventional devices in these cases which are a priori without any sensorial element.
Adolescent↗
[Artifacts in magnetic resonance angiography].
Magnetic resonance angiography (MRA) has become a major tool in the management of vascular diseases. However, many artifacts that may lead to misinterpretations are encountered with this imaging modality. The purpose of this paper was to enumerate, explain and illustrate each kind of artifact encountered with "time of flight" or "phase contrast" imaging methods. For each artifact, we tried to provide one or several techniques to minimize its consequences.
Artifacts↗
Idiopathic granulomatous hypophysitis: clinical and imaging features.
Idiopathic pituitary granuloma is a rare disorder similar to lymphocytic adenohypophysitis. Few cases have been reported. We report a new histologically case proven with MRI. The patterns of clinical and radiological presentation and the management of this disorder are discussed. MRI findings suggestive of this condition include an intensely enhancing pituitary mass, associated with dural enhancement. Steroid therapy may be suggested avoiding unnecessary surgery.
Adenoma↗
Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel-Lindau disease. Case report.
This report describes a patient with von Hippel-Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.
Adenoma↗