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.

[Skull base osteomyelitis]. / Ostéite de la base du crâne.

Skull base osteomyelitis is a rare but serious infection. It typically afflicts immunosuppressed patients and should be suspected in patients with persistent otitis complicated by cranial nerve palsy (VII, IX and XII). The most frequent germ is pseudomonas aeruginosa. Contiguous spread of infection occurs along neurovascular structures and weaker regions of the skull base, then into the soft tissue compartments of the face and nasopharynx. Diagnosis and treatment should be made early for this disease with poor prognosis and high mortality.

[Vascular emergencies of the head and neck]. / Urgences vasculaires cervicofaciales.

The clinical manifestations of vascular lesions of the head and neck may be variable (hemorrhagic, ischemic, compressive). Diagnosis often is made at the time of acute presentation, but delayed manifestations, sometimes long after the initial presentation, should not be overlooked. Hemorrhagic manifestations are characterized by epistaxis, corresponding mainly to lesions of the nasal cavities but involvement of the internal carotid artery should be excluded (life threatening). In addition, some vascular malformations may lead to severe hemorrhage. Ischemic manifestations typically result from arterial dissection. A venous origin is also possible. Carotid-cavernous fistulas rarely lead to hemorrhagic or ischemic manifestations and tend to result in ocular manifestations, typically delayed and sometimes misleading.

[Traumatic cord and nerve root injuries: imaging features at the acute and chronic phases]. / La moelle et les racines traumatiques: imagerie dans la prise en charge aiguë et chronique.

Cord injuries are frequent and severe lesions resulting in significant disability, most frequently in younger subjects. The area of cord injured results in clinical syndromes (Brown-Sequard, motor and/or sensory deficit...). Cord and rootlet injuries are best depicted on MRI. Diffusion tensor imaging with tractography enables depiction of the most severe cord lesions and some prediction of tissue viability which may provide an idea of the potential functional prognosis and patient recovery. MRI is optimal to demonstrate areas of cord hemorrhage or compression, partial or complete cord transsection, nerve root avulsion...

[Diffusion tensor imaging and tractography of central pontine myelinolysis]. / Suivi évolutif d'une myelinolyse centro-pontique en irm de tenseur de diffusion et de tracking de fibres.

OBJECTIVES: To illustrate the value of diffusion tensor imaging and tractography in the diagnosis and follow-up of central pontine myelinolysis. CASE REPORT: We report a case of central pontine myelinolysis in a 29 year old woman, also anorexic, studied using MR Diffusion Tensor Imaging (DTI) and Fibre Tracking (FT) focused on the pons, and compared with the studies of 5 normal volunteers. Tractography showed a swollen aspect of the right corticospinal fiber tract correlating with mild left lower extremity deficit at clinical evaluation. The pontine fibers were posteriorly displaced but intact. The sensory tracts were also intact. Apparent Diffusion Coefficient values were increased and Fractional Anisotropy was decreased in the lesions. Follow up imaging showed persistent abnormal ADC and FA values in the pons although the left cortico-spinal tract returned to normal, consistent with the clinical outcome. CONCLUSION: Diffusion Tensor Imaging MR and Fiber tractography are a new method to analyse white matter tracts. It can be used to prospectively evaluate the location of white matter tract lesions at the acute phase of central pontine myelinolysis and follow up.

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.

Diffusion-weighted imaging in a case of wernicke encephalopathy.

In a chronic alcoholic patient with progressive confusion, which was consistent with the clinical diagnosis of Wernicke encephalopathy, T2-weighted, FLAIR and diffusion weighted (DWI) MR imaging depicted brain abnormalities located in both medial thalamic nuclei. Apparent Diffusion Coefficient (ADC) measurements in these regions shown unexpected normal values, referring to Wernicke pathological findings and DWI data. DWI may be helpful to diagnose early basal nuclei abnormalities, but may fail to compute ADC values in these locations.

[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.

Visibility of the central canal on MRI.

The central canal of the spinal cord is present at birth and becomes progressively obliterated. Cadaver studies have shown that it may persist partially or completely. To our knowledge, this entity has not been described on MRI. We reviewed 794 MRI studies of the spinal cord, and found 12 patients (aged 14 to 65 years) who had an intramedullary cavity. The cavity was at the junction of the ventral 1/3 and dorsal 2/3 of the spinal cord, except at the level of the lumbar enlargement, where it was central. It was filiform in most cases, although sometimes fusiform (3 to 4 mm in diameter), and had regular contours. The cavity were thoracic in 69 % of cases. The clinical features were totally unrelated to the image, and there were no anatomical factors (Chiari malformation, dysraphism) predisposing to syringomyelia. The images were perfectly compatible with a persistent central canal, which we interpret as a variant of normal anatomy. Therefore it is important to regard these findings as normal, to avoid unnecessary treatment and follow-up.

[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.

[The place of MRI in the study of cerebrospinal fluid fistulas]. / Place de l'IRM dans l'exploration des fistules de liquide céphalo-rachidien.

Results of CT and MRI explorations in 8 patients with cerebrospinal fluid (CSF) fistulae are reported and compared with surgical findings in 7. Sensitivity of MRI (CISS and fast T2 spin-echo sequences) was excellent giving perfect correlations with surgical findings in 7/7 cases. CT and MRI were found to provide complementary information suggesting they should be used in combination as first intention explorations of suspected CSF fistulae. Computerized cisternography should be used if MRI is contraindicated or if a clinically and biologically proven CSF fistulae is not visualized by CT or MRI.