Anti-Ma2 antibody encephalitis associated with Sjogren's syndrome.

INTRODUCTION: Onconeuronal antibodies directed against intracellular antigens are strongly associated with paraneoplastic syndromes and their detection in the absence of cancer is unusual. We herein report a case of anti-Ma2 encephalitis associated with Sjogren's syndrome (SS). CASE REPORT: An 81-year-old woman followed for a cutaneous lupus with vasculitis associated with SS presented a flare of her disease with neurological worsening including walking difficulty, hypersialorrhea and dysphagia. A paraneoplastic origin of the symptoms was suspected and anti-Ma2 antibodies were positive in serum. The search for an underlying neoplasia was negative. The diagnosis of anti-Ma2 encephalitis secondary to a SS was made. In the literature, the association of anti-Ma2 encephalitis and SS has been previously reported twice. Cases of patients with other onconeuronal antibodies associated with SS have been also reported. Anti-Ma2 encephalitis is a rare condition with a wide spectrum of symptoms associated with a cancer in more than 90% of the cases. Anti-Ma2 encephalitis has also been described after the use of immune check points inhibitors underscoring the role of autoimmunity in its pathogenesis. CONCLUSION: Anti-Ma2 encephalitis is essentially associated with neoplasia but can occur in Sjogren's syndrome.

Cystic form of cervical lymphadenopathy in adults. Guidelines of the French Society of Otorhinolaryngology (short version). Part 2-etiological diagnosis procedure: Clinical and imaging assessment.

INTRODUCTION: The authors present the guidelines of the French Society of Otorhinolaryngology (SFORL) for clinical and radiological assessment of cystic neck lymphadenopathy of unknown primary in adults. Most cases concern head and neck carcinoma metastasis, often in the oropharyngeal area, or less frequently differentiated thyroid carcinoma or non-keratinizing nasopharyngeal carcinoma. METHODS: A multidisciplinary task force was commissioned to carry out a review of the literature on the etiological work-up in cystic neck lymphadenopathy in adults: clinical examination, conventional imaging (ultrasound, CT, MRI) and metabolic imaging. Guidelines were drafted based on the articles retrieved, and graded A, B, C or expert opinion according to decreasing level of evidence. RESULTS: Oriented clinical examination, cervical and thyroid ultrasound scan and contrast-enhanced neck and chest CT scan are recommended in the assessment of cystic neck lymphadenopathy of unknown primary in adult patients. PET-CT is recommended prior to panendoscopy, to identify the primary tumor. CONCLUSION: Clinical and radiological assessment is fundamental for etiologic diagnosis of cystic neck lymphadenopathy in adult patients, and should be completed by cytological examination before in initiating treatment.

Cystic form of cervical lymphadenopathy. Guidelines of the French Society of Otorhinolaryngology - Head and Neck Surgery (SFORL). Part 1: Diagnostic procedures for lymphadenopathy in case of cervical mass with cystic aspect.

OBJECTIVES: The authors present the guidelines of the French Society of Otorhinolaryngology - Head and Neck Surgery Society on diagnostic procedures for lymphadenopathy in case of a cervical mass with cystic aspect. METHODS: A multidisciplinary work-group was entrusted with a review of the scientific literature on the topic. Guidelines were drawn up, then read over by an editorial group independent of the work-group, and the final version was drawn up. Guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. RESULTS: In adults presenting a cystic cervical mass, it is recommended to suspect cervical lymphadenopathy: in order of decreasing frequency, cystic metastasis of head and neck squamous cell carcinoma, of undifferentiated nasopharyngeal carcinoma, and of thyroid papillary carcinoma (Grade C). On discovery of a cystic cervical mass on ultrasound, architectural elements indicating a lymph node and a thyroid nodule with signs of malignancy should be screened for, especially if the mass is located in levels III, IV or VI (Grade A). Malignant lymphadenopathy should be suspected in case of cervical mass with cystic component on CT (Grade B), but benign or malignant status cannot be diagnosed only on radiological data (CT or MRI) (Grade A), and 18-FDG PET-CT should be performed, particularly in case of inconclusive ultrasound-guided fine needle aspiration biopsy (Grade C).

[Intramedullary hemangioblastomas]. / Les hémangioblastomes intramédullaires.

AIM AND BACKGROUND: Intramedullary hemangioblastomas are rare lesions representing 1 to 5% of spinal tumors. The aim of this study was to review our experience with the surgical management of intramedullary hemangioblastomas. MATERIALS AND METHODS: We performed a retrospective analysis of all the patients with intramedullary hemangioblastomas operated on between 1993 and 2011 in our department. All the patients were screened for Von Hippel Lindau disease. The minimum follow-up was 3 years. The clinical presentation, radiological findings, surgical procedure and outcomes were recorded and analyzed. RESULTS: Our consecutive series included 59 patients with a total of 65 tumors. The mean age at diagnosis was 38 years. Forty-two patients (72.5%) had Von Hippel Lindau disease. The main symptom was pain (58% of cases). The most common location was cervical spinal cord. The average size was 15mm. The resection was complete in 95% cases resulting in clinical improvement in 12% cases, stability in more than 86% of cases and deterioration in less than 2% cases. CONCLUSION: All patients with intramedullary hemangioblastoma should have a screening for the Von Hippel Lindau disease and if the diagnosis is correct, close monitoring should be initiated. Surgical removal is strongly advised in cases of neurological deficits or radiological progression of the tumour.

The vestibulocochlear nerve (VIII).

The vestibulocochlear nerve (8th cranial nerve) is a sensory nerve. It is made up of two nerves, the cochlear, which transmits sound and the vestibular which controls balance. It is an intracranial nerve which runs from the sensory receptors in the internal ear to the brain stem nuclei and finally to the auditory areas: the post-central gyrus and superior temporal auditory cortex. The most common lesions responsible for damage to VIII are vestibular Schwannomas. This report reviews the anatomy and various investigations of the nerve.

The lower cranial nerves: IX, X, XI, XII.

The lower cranial nerves innervate the pharynx and larynx by the glossopharyngeal (CN IX) and vagus (CN X) (mixed) nerves, and provide motor innervation of the muscles of the neck by the accessory nerve (CN XI) and the tongue by the hypoglossal nerve (CN XII). The symptomatology provoked by an anomaly is often discrete and rarely in the forefront. As with all cranial nerves, the context and clinical examinations, in case of suspicion of impairment of the lower cranial nerves, are determinant in guiding the imaging. In fact, the impairment may be located in the brain stem, in the peribulbar cisterns, in the foramens or even in the deep spaces of the face. The clinical localization of the probable seat of the lesion helps in choosing the adapted protocol in MRI and eventually completes it with a CT-scan. In the bulb, the intra-axial pathology is dominated by brain ischemia (in particular, with Wallenberg syndrome) and multiple sclerosis. Cisternal pathology is tumoral with two tumors, schwannoma and meningioma. The occurrence is much lower than in the cochleovestibular nerves as well as the leptomeningeal nerves (infectious, inflammatory or tumoral). Finally, foramen pathology is tumoral with, outside of the usual schwannomas and meningiomas, paragangliomas. For radiologists, fairly hesitant to explore these lower cranial pairs, it is necessary to be familiar with (or relearn) the anatomy, master the exploratory technique and be aware of the diagnostic possibilities.

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.

Sinonasal Wegener's granulomatosis: CT characteristics.

Wegener's granulomatosis (WG) is a severe and potentially lethal granulomatosis. Even though no specific radiological criteria exist, CT may suggest the correct diagnosis at an early stage. Recent improvement in the prognosis is related to earlier diagnosis, allowing the initiation of efficient and specific treatment before any severe complications occur. We reviewed a series of WG cases in order to establish the CT diagnostic criteria.

[MRI sequences in the detection of cavernous angiomas]. / Séquences de résonance magnétique dans la détection de cavernomes.

We prospectively evaluated 30 patients with CNS cavernous angioma with a high field (1.5T) magnetic resonance imager. In all patients the MRI protocol included: gradient echo T1*-weighted imaging, spin echo T2*-weighted imaging and gradient echo T2*-weighted imaging. We evaluated each case for the number of lesions detected on each sequence. Gradient echo T2*-weighted imaging with a long TE (TE: 35ms) was the most sensitive sequence for the detection of cavernous angiomas allowing detection of small lesions that were not identified on the other sequences.

[Imaging of syringomyelia]. / L'imagerie des syringomyélies.

This review reports the value of current imaging techniques in the diagnosis of syringomyelia excluding tumoral causes. The value of imaging techniques for evaluation of extension, detection of associated anomalies, and pre- and postoperative evaluation are detailed.

[Imaging of syringomyelia]. / Imagerie des syringomyélies.

MRI is the best imaging method to evaluate syringomyelia. It is important to study from the posterior cranial fossa to the sacro-lumbar region and also the supra-tentorial structures. This complete analysis is essential to classify the syringomyelia and to investigate other associated malformations. Radiographs and CT scan are useful to analyze bone structures. For MRI, the new sequences with phased-array coils are also very important to study the entire spinal cord and the posterior fossa. It is essential to study the spinal cord with sagittal and axial spin echo T1 and fast spin echo T2 weighted images with sometimes coronal view, particularly when the patient presents a scoliosis, to have a correct morphological and functional evaluation. MRI gives an excellent study of the spinal cord with an excellent analysis of a primitive or foraminal syringomyelia, but also traumatic, infectious or post arachnoiditis syringomyelia. Spin echo T1 weighted images with injection of gadolinium can be used if an intra-medullary tumor is suspected. MRI is also useful for the post-operative follow up to evaluate the persistence of the medullary cyst and the enlargement of the foramen magnum.

[Neurocysticercosis]. / La neurocysticercose.

The neurocysticercosis is an infestation of the central nervous system by larvae of Taenia solium. The disease is endemic in a few countries of Latin America, Asia, and Africa and is becoming increasingly prevalent in the United States and Europe. The immigration of individuals from endemic areas and the tourism in these areas require a good knowledge of this disease. A retrospective study evaluating the neurocysticercosis lesions with MR and CT is proposed. Parenchymal, subarachnoid and intraventricular cysts are evaluated. We were able to recognise four CT and MR stages. MR was useful in detecting the cysts of neurocycticercosis in the first stage but was inferior in the detection of parenchymal calcifications.

[MRI symptomatology of non-tumoral myelopathies]. / Sémiologie irm des myélopathies non tumorales.

We present a retrospective study in order to analyze the abnormalities noted on MRI in 27 cases of myelopathy excluding tumors, explored between 1994 and 1996. The different lesions were: Multiple Sclerosis (n = 11), Spondylotic myelopathy (n = 3), Neurosarcoidosis (n = 4), CMV Myelitis (n = 1), Radiation Myelopathy (n = 1), Spinal Dural Arteriovenous Fistula (n = 1), Intramedullary Cysticercosis (n = 1), Infarct (n = 5). The exams have been made on 1.5 Tesla Magnetom Vision Siemens or GE Signa machine. All patients have had axial and sagittal views with coronal complementary study in 4 cases. Sequences were Spin echo pT1 (TR: 560, TE: 12), Fast Spin echo pT2 (TR: 3 500, TE: 99 or 128), and gradient echo pT2 (TR: 700, TE: 22, Angle: 25 degrees). Intravenous injection of Gadolinium has been made in 16 cases (0.1 mmol/kg). We have studied the presence or not of a signal abnormality in pT1 and/or in pT2, of enhancement, and its topography (cervical, thoracic, lumbar). We classified lesions in central and/or peripheral and according, to their topography in anterior, posterior or lateral type. The form has been classified in four types (nodular, triangular, "pen like", plage). Extension in transversal (superior or inferior to half medullary surface) and cranio-caudal directions (inferior to one vertebrae, between one and two vertebrae, superior to two vertebrae) has been also classified. Others intra or perimedullar and encephalic abnormalities have been noted. We analyzed the results for each pathology and underline the essential diagnosis criteria noted (low cranio-caudal and transversal extension with frequent triangular form of Multiple Sclerosis lesions, frequent suggestive abnormalities of the encephale (82%) in Multiple Sclerosis, intra and perimedullar enhancement with deformations of the surface of the spinal cord in Sarcoidosis' lesions, extended dorsolumbar "pen like" lesions with inconstant enhancement of infarcts, focal plage lesions centered on degenerative changes of the spinal canal in spondylotic myelopathy, bony lipomatous involution in front of intramedullary radiation plage lesion...) and also review the literature and confront their results to it. We insist on the difficulties in classifying myelopathy (radio-clinical terminology discordances, identical signal abnormalities frequently caused by different illness, necessity to compare to pathologic results). We propose a MRI study protocol that should interest the whole spinal cord and comport T1 weighted without and after gadolinium sequences, T2 weighted sequences (with always a gradient echo type). 2 or better 3 different plans should be made. A complementary study of the brain by MRI is often useful. Clinical study, biology, evolution, MRI and when possible pathology all are necessary to better understand myelopathy's mechanisms.

[XII cranial nerve pseudoneurinoma due to spontaneous fibrous transformation of an aneurysm of the vertebral artery]. / Pseudoneurinome du XII par transformation fibreuse spontanée d'un anévrisme de l'artère vertébrale.

The authors report an exceptional case report of tumor like evolution of a completely thrombosed aneurysm of the right vertebral artery suggestive of neurinoma of the XII nerve. We describe CT, MRI, MR-angiography. The diagnosis has been established by pathologic study after surgical extraction. Our case demonstrate the possibility of growth of totally thrombosed aneurysms and we discuss various mechanisms.