Advanced multiparametric magnetic resonance imaging of multinodular and vacuolating neuronal tumor.

BACKGROUND AND PURPOSE: Multinodular and vacuolating neuronal tumor (MVNT) of the cerebrum is a rare brain lesion with suggestive imaging features. The aim of our study was to report the largest series of MVNTs so far and to evaluate the utility of advanced multiparametric magnetic resonance (MR) techniques. METHODS: This multicenter retrospective study was approved by our institutional research ethics board. From July 2014 to May 2019, two radiologists read in consensus the MR examinations of patients presenting with a lesion suggestive of an MVNT. They analyzed the lesions' MR characteristics on structural images and advanced multiparametric MR imaging. RESULTS: A total of 64 patients (29 women and 35 men, mean age 44.2 ± 15.1 years) from 25 centers were included. Lesions were all hyperintense on fluid-attenuated inversion recovery and T2-weighted imaging without post-contrast enhancement. The median relative apparent diffusion coefficient on diffusion-weighted imaging was 1.13 [interquartile range (IQR), 0.2]. Perfusion-weighted imaging showed no increase in perfusion, with a relative cerebral blood volume of 1.02 (IQR, 0.05) and a relative cerebral blood flow of 1.01 (IQR, 0.08). MR spectroscopy showed no abnormal peaks. Median follow-up was 2 (IQR, 1.2) years, without any changes in size. CONCLUSIONS: A comprehensive characterization protocol including advanced multiparametric magnetic resonance imaging sequences showed no imaging patterns suggestive of malignancy in MVNTs. It might be useful to better characterize MVNTs.

[Basal ganglia calcification]. / Calcifications des noyaux gris centraux.

Calcifications of the basal ganglia are frequently seen on the cerebral CT scans and particularly in the globus pallidus. Their frequency increases physiologically with age after 50 years old. However, pathological processes can also be associated with calcium deposits in the gray nuclei, posterior fossa or white matter. Unilateral calcification is often related to an acquired origin whereas bilateral ones are mostly linked to an acquired or genetic origin that will be sought after eliminating a perturbation of phosphocalcic metabolism. In pathological contexts, these calcifications may be accompanied by neurological symptoms related to the underlying disease: Parkinson's syndrome, psychiatric and cognitive disorders, epilepsy or headache. The purpose of this article is to provide a diagnostic aid, in addition to clinical and biology, through the analysis of calcification topography and the study of different MRI sequences.

Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance.

Multinodular and vacuolating neuronal tumor of the cerebrum is a rare supratentorial brain tumor described for the first time in 2013. Here, we report 11 cases of infratentorial lesions showing similar striking imaging features consisting of a cluster of low T1-weighted imaging and high T2-FLAIR signal intensity nodules, which we referred to as multinodular and vacuolating posterior fossa lesions of unknown significance. No relationship was found between the location of the lesion and clinical symptoms. A T2-FLAIR hypointense central dot sign was present in images of 9/11 (82%) patients. Cortical involvement was present in 2/11 (18%) of patients. Only 1 nodule of 1 multinodular and vacuolating posterior fossa lesion of unknown significance showed enhancement on postcontrast T1WI. DWI, SWI, MRS, and PWI showed no malignant pattern. Lesions did not change in size or signal during a median follow-up of 3 years, suggesting that multinodular and vacuolating posterior fossa lesions of unknown significance are benign malformative lesions that do not require surgical intervention or removal.

Inaugural tumor-like multiple sclerosis: clinical presentation and medium-term outcome in 87 patients.

BACKGROUND: Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo's concentric sclerosis, Schilder's disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients. METHODS: Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively. Inclusion criteria were (1) a first clinical event (2) MRI showing one or more large demyelinating lesions (20 mm or more in diameter) with mass-like features. Patients with a previous demyelinating event (i.e. confirmed multiple sclerosis) were excluded. RESULTS: Mean age at onset was 26 years. The most common initial symptoms (67% of the patients) were hemiparesis or hemiplegia. Aphasia, headache and cognitive disturbances (i.e. atypical symptoms for demyelinating diseases) were observed in 15, 18 and 15% of patients, respectively. The mean largest diameter of the tumefactive lesions was 26.9 mm, with gadolinium enhancement in 66 patients (81%). Twenty-one patients (24%) had a single tumefactive lesion. During follow-up (median time 5.7 years) 4 patients died, 70 patients improved or remained stable and 12 worsened. 86% of patients received initial corticosteroid treatment, and 73% received disease-modifying therapy subsequently. EDSS at the end of the follow-up was 2.4 ± 2.6 (mean ± SD). CONCLUSION: This study provides further evidence that the clinical course of MS presenting with large focal tumor-like lesions does not differ from that of classical relapsing-remitting MS, once the noisy first relapsing occurred.

TIPIC Syndrome: Beyond the Myth of Carotidynia, a New Distinct Unclassified Entity.

BACKGROUND AND PURPOSE: The differential diagnosis of acute cervical pain includes nonvascular and vascular causes such as carotid dissection, carotid occlusion, or vasculitis. However, some patients present with unclassified vascular and perivascular changes on imaging previously reported as carotidynia. The aim of our study was to improve the description of this as yet unclassified clinico-radiologic entity. MATERIALS AND METHODS: From January 2009 through April 2016, 47 patients from 10 centers presenting with acute neck pain or tenderness and at least 1 cervical image showing unclassified carotid abnormalities were included. We conducted a systematic, retrospective study of their medical charts and diagnostic and follow-up imaging. Two neuroradiologists independently analyzed the blinded image datasets. RESULTS: The median patient age was 48 years. All patients presented with acute neck pain, and 8 presented with transient neurologic symptoms. Imaging showed an eccentric pericarotidian infiltration in all patients. An intimal soft plaque was noted in 16 patients, and a mild luminal narrowing was noted in 16 patients. Interreader reproducibility was excellent. All patients had complete pain resolution within a median of 13 days. At 3-month follow-up, imaging showed complete disappearance of vascular abnormalities in 8 patients, and a marked decrease in all others. CONCLUSIONS: Our study improved the description of an unclassified, clinico-radiologic entity, which could be described by the proposed acronym: TransIent Perivascular Inflammation of the Carotid artery (TIPIC) syndrome.

Facial nerve: from anatomy to pathology.

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.

[Imaging of subarachnoid hemorrhage]. / Imagerie de l'hémorragie sous-arachnoïdienne.

Even if acute subarachnoid hemorrhage (SAH) accounts for only 5% of strokes, its diagnosis is very important because its clinical consequences can be tragic. Recent technological advances in medical imaging have improved diagnostic and therapeutic management of patients with SAH. Nonenhanced CT of the head is the initial imaging modality in suspected SAH for the detection of ruptured intracranial aneurysms. Digital subtraction angiography (DSA) remains the reference exam. Multidetector row CT angiography may potentially replace DSA in the emergency setting, as it provides image data that allows evaluating aneurysmal morphology, the neck size or the visualization of vessels in the vicinity of the aneurysm. For SAH unrelated to aneurysm rupture (15% of cases), MRI and MRA can be added to the diagnostic work-up in order to exclude other differential diagnoses such as venous thrombosis or angiitis. Finally, transcranial color-coded duplex sonography, CT, or MRI are used in clinical practice in order to detect aggravating factors of SAH like hydrocephalus or vasospasm.

[Natural history and management of mycotic intracranial aneurysm]. / Anévrysme mycotique cérébral: histoire naturelle et prise en charge thérapeutique.

INTRODUCTION: mycotic intracranial aneurysms are a rare complication of infectious endocarditis. We report four cases of patients with endocarditis, complicated by an acute stroke, revealing a mycotic intracranial aneurysm. PATIENTS AND METHODS: four men (aged range 24 to 63 year old) were admitted for endocarditis, complicated by ischemic stroke in two cases and hemorrhagic stroke in the two other cases, including one with subarachnoid hemorrhage. Neuroimaging disclosed a mycotic cerebral aneurysm in all four cases. DISCUSSION: we will discuss the natural history and the management of mycotic intracranial aneurysm based on a review of the literature and our experience. Three therapeutic options are possible: medical treatment, surgery and endovascular embolisation. CONCLUSION: management of mycotic intracranial aneurysms is still controversial, frequently requiring a multidisciplinary strategy with priority given to endovascular interventions.

[Posterior reversible encephalopathy syndrome: about 2 cases related to the cyclosporine]. / Syndrome d'encéphalopathie postérieure réversible: à propos de 2 cas liés à la ciclosporine.

Following a pulmonary transplantation for cystic fibrosis, 2 patients exhibited a syndrome associating arterial hypertension, headache, visual trouble and generalized seizures. Cerebral magnetic resonance imaging revealed diffuse cortical and subcortical lesions predominantly in posterior regions. The exclusion of alternate diagnoses and the disappearance of the symptoms when the cyclosporine treatment was stopped confirmed the diagnosis of cyclosporine-related reversible posterior encephalopathy syndrome (PRES). Immediate appropriate management resulted in symptom disappearance and regression of radiological images.

[Symptomatic venous angioma. Report of a case]. / Angiome veineux symptomatique. A propos d'un cas.

We report a case of venous angioma revealed by seizure after a non hemorrhagic venous infarct. The initial neuroradiological work-up with CT scan, angiography and brain MRI evidenced the venous infarction with abnormal contrast uptake. The control MRI 3 months later showed clear involution of the venous infarct. Non-hemorrhagic venous infarction on venous angiomas are exceptional. Only 2 cases have been reported in the literature. Impaired venous drainage could be implicated.

Post-traumatic syringomyelia and post-traumatic spinal canal stenosis: a direct relationship: review of 75 patients with a spinal cord injury.

This study aims to demonstrate predictive factors for post traumatic syringomyelia (PTS), and in particular to correlate the role of insufficiency of reduction of a spinal fracture with the occurrence of syringomyelia. One hundred and twenty-eight spinal cord injured patients (SCI) were studied during the years 1992 and 1993. Among them, 75 underwent a complete and reliable evaluation including: review of the initial vertebral lesion, and of the surgery report, and a radiological study of the lesion site with standard X-rays, a CT scan, and an MRI. The CT Scan included slices in sagittal reconstructions and in the axial plane at the site of injury with the calculation of a percentage of canal stenosis in the two planes of the space. An MRI was carried out with T1 and T2 weighted images, including sagittal entire cord images in addition to sagittal and axial slices centred on the site of injury. A syrinx was diagnosed in 28% of the patients. The occurrence of a syrinx is significantly correlated with spinal canal stenosis in the sagittal plane (delta D) with a P < 0.001 and in the axial plane (delta S) (P < 0.05). This present study demonstrates the major role of the insufficiency of reduction of the vertebral lesion in the genesis of a syrinx. The quality of the initial treatment of the vertebral injury is the first step in the prevention of a syrinx. The treatment of a syrinx, besides techniques of drainage, must also take into account the spinal realignment.

Ehlers-Danlos syndrome with a spontaneous caroticocavernous fistula occluded by detachable balloon: case report and review of literature.

We report a caroticocavernous fistula (CCF) treated by an endovascular procedure in a young woman with Ehlers-Danlos syndrome type IV, with severe bilateral carotid and vertebral artery dysplasia. The CCF, which appeared after minor trauma, was successfully occluded by a detachable balloon introduced into the venous side by an arterial approach. Six previously published cases are reviewed, five successfully treated. The difficulties and risks of the endovascular procedure due to the vascular changes, are emphasised. The possibility of the venous approach is discussed.

[Dural fistula of the cavernous sinus. Clinical and angiographic aspects. Results of particulate intravascular treatment]. / Fistules durales de la loge caverneuse. Aspects cliniques et angiographiques. Résultats du traitement endovasculaire au moyen de particules.

Thirteen patients with dural fistula of the cavernous sinus were studied by angiography. Nine of them presented with ophthalmic symptoms (chemosis and oculomotor disorders caused by the fistula). In one patient the lesion was discovered by chance, and 2 other patients had consulted for a disabling tinnitus. Eight patients accepted to the treated by the endovascular route for embolization of the maxillary artery, using particles. Embolization was unilateral in 4 cases and bilateral in 4 other cases. All embolizations were followed by serial control angiography immediately performed. All subjects were seen again as out-patients at 3 month, and 5 of them accepted a control angiography. Three patients were then regarded as clinically and anatomically cured. Two patients with incomplete clinical and angiographic results had a second embolization which resulted in clinical and anatomical cure at a 4-month control examination. These 8 patients were re-examined clinically after one month of treatment and found to be symptomless. Only one complication (transient oedema of the face) was noted. Dural fistulae are lesions that are most probably acquired by alteration of the physiological dural arteriovenous shunts occurring soon after venous thrombosis. Their course is capricious, and they sometimes heal spontaneously. However, the cavernous sinus location with its repercussion on the eye usually requires treatment. This treatment is initially endovascular; surgery and multifascicular irradiation being reserved for failures. Particle embolization of maxillary arteries is a simple and efficient procedure which must be used initially. If it proves insufficient, embolization of other arterial feeders (but it is often more dangerous) or the venous route can be tried.