The Epidermal Growth Factor Domain of the Mutation Does Not Appear to Influence Disease Progression in CADASIL When Brain Volume and Sex Are Taken into Account.

BACKGROUND AND PURPOSE: By studying the evolution of brain volume across the life span in male and female patients, we aimed to understand how sex, brain volume, and the epidermal growth factor repeat domain of the mutation, the 3 major determinants of disability in CADASIL, interact in driving disease evolution. MATERIALS AND METHODS: We used validated methods to model the evolution of normalized brain volume with age in male and female patients using nonparametric regression in a large, monocentric cohort with prospectively collected clinical and high-resolution MR imaging data. We used k-means clustering to test for the presence of different clinical course profiles. RESULTS: We included 229 patients (mean age, 53 [SD, 12] years; 130 women). Brain volume was larger in women (mean size, 1024 [SD, 62] cm3 versus 979 [SD, 50] cm3; P < .001) and decreased regularly. In men, the relationship between brain volume and age unexpectedly suggested an increase in brain volume around midlife. Cluster analyses showed that this finding was related to the presence of a group of older male patients with milder symptoms and larger brain volumes, similar to findings of age-matched women. This group did not show specific epidermal growth factor repeat domain distribution. CONCLUSIONS: Our results demonstrate a detrimental effect of male sex on brain volume throughout life in CADASIL. We identified a subgroup of male patients whose brain volume and clinical outcomes were similar to those of age-matched women. They did not have a specific distribution of the epidermal growth factor repeat domain, suggesting that yet-unidentified predictors may interact with sex and brain volume in driving disease evolution.

MRI evaluation of the endolymphatic space in otosclerosis and correlation with clinical findings.

PURPOSE: The purpose of this study was to investigate the clinical features of ears with otosclerosis and their correlation with endolymphatic hydrops and blood-labyrinth barrier (BLB) impairment on 3T magnetic resonance imaging (MRI). MATERIALS AND METHODS: This was a single-center retrospective imaging study. Thirty-nine ears from 29 patients (17 men, 12 women; mean age 52±12 [SD] years; range 27-74 years) with non-operated otosclerosis were included. All patients underwent three-dimensional fluid attenuated inversion recovery (FLAIR) MRI sequences performed 4hours after the intravenous administration of a single dose of gadolinium-based contrast material. MRI examinations were analyzed by two radiologists for the presence of saccular hydrops (SH) and BLB impairment. Results of MRI examinations were compared with clinical findings, hearing levels and extent of otosclerotic lesions based on high-resolution computed tomography findings. BLB impairment was evaluated using the signal intensity ratio, ratio of intensities between the basal turn of the cochlea and the medulla. RESULTS: SH was observed in 1/39 (3%) otosclerotic ears and BLB impairment in 8/39 (21%) while 8/29 patients with otosclerosis (28%) had vertigo. No significant associations were found between SH or BLB impairment on MRI, and the presence of vertigo or the degree of sensorineural hearing loss. CONCLUSION: Clinical manifestations of otosclerosis (sensorineural hearing loss and rotatory vertigo) were not significantly associated with MRI findings such as BLB impairment and endolymphatic hydrops. SH was only observed in one patient with obstruction of the vestibular aqueduct by an otosclerotic focus.

Comparison of Enhancement of the Vestibular Perilymph between Variable and Constant Flip Angle-Delayed 3D-FLAIR Sequences in Menière Disease.

BACKGROUND AND PURPOSE: Endolymphatic hydrops in patients with Menière disease relies on delayed postcontrast 3D-FLAIR sequences. The purpose of this study was to compare the degree of perilymphatic enhancement and the detection rate of endolymphatic hydrops using constant and variable flip angles sequences. MATERIALS AND METHODS: This was a retrospective study performed in 16 patients with Menière disease who underwent 3T MR imaging 4 hours after gadolinium injection using two 3D-FLAIR sequences with a constant flip angle at 140° for the first and a heavily-T2 variable flip angle for the second. The signal intensity ratio was measured using the ROI method. We graded endolymphatic hydrops and evaluated the cochlear blood-labyrinth barrier impairment. RESULTS: Both for symptomatic and asymptomatic ears, the median signal intensity ratio was significantly higher with the constant flip angle than with the heavily-T2 variable flip angle (7.16 versus 1.54 and 7.00 versus 1.45, P < .001). Cochlear blood-labyrinth barrier impairment was observed in 4/18 symptomatic ears with the heavily-T2 variable flip angle versus 8/19 with constant flip angle sequences. With heavily-T2 variable flip angle sequences, endolymphatic hydrops was observed in 7-10/19 symptomatic ears versus 12/19 ears with constant flip angle sequences. We found a significant association between the clinical symptomatology and the presence of endolymphatic hydrops with constant flip angle but not with heavily-T2 variable flip angle sequences. Interreader agreement was always perfect with constant flip angle sequences while it was fair-to-moderate with heavily-T2 variable flip angle sequences. CONCLUSIONS: 3D-FLAIR constant flip angle sequences provide a higher signal intensity ratio and are superior to heavily-T2 variable flip angle sequences in reliably evaluating the cochlear blood-labyrinth barrier impairment and the endolymphatic space.

Anatomo-radiological study supporting the use of ipsilateral nasoseptal flap for the transpterygoid management of temporo-sphenoidal meningoceles. A review of 21 cases.

OBJECTIVES: Surgical treatment of temporo-sphenoidal meningoceles involves the reduction of the meningocele, watertight closure and defect coverage with a nasoseptal flap (NSF). It can be performed contralaterally or ipsilaterally: in the latter situation, the pedicle of the flap must be dissected into the pterygopalatine fossa. The objective of this study was to evaluate the benefit of using an ipsilateral NSF in transpterygoid approaches for the management of temporo-sphenoidal meningoceles, compared to a contralateral NSF, based on a radiological study. MATERIAL AND METHODS: Retrospective monocentric study of 21 cases, between 2002 and 2018. Measurement of the NSF lengths, and lengths needed to cover the defect were evaluated on the preoperative scanner. Early and later failure and complication rates were evaluated. RESULTS: Seventeen cases of temporo-sphenoidal meningoceles with available CT scan were identified. The mean duration of follow up was 27.9 months [1-147]. Theoretical lengths of the ipsi and contralateral NSF were comparable: 71.4±7.8mm vs. 78.8±8mm, P=0.729. In 8 cases/18 (42%), the theoretical length of the contralateral NSF was not long enough to cover the defect beyond the V2 (mean lack of 8.87±6.6mm). In all cases, the theoretical length of the ipsilateral NSF was sufficient to cover the defect. In the case series, failure and complication rates were similar. CONCLUSION: The use of an ipsilateral NSF for the transpterygoid management of temporo-sphenoidal meningoceles, although more complex, allows a better coverage of the defect, compared to the contralateral NSF, which is not long enough in 42% of cases.

Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI.

PURPOSE: The purpose of this study was to assess whether the volume of the vestibular endolymphatic space correlates with the degree of hearing loss using heavily T2-weighted fast imaging employing steady-state acquisition with cycle phase (FIESTA-C) MRI. MATERIALS AND METHODS: A total of 23 patients with vestibular schwannoma, as diagnosed on typical image findings, who underwent FIESTA-C MRI were included. There were 13 women and 10 men with a mean age of 63.5±9.3 (SD) years (range: 49-88years). Two radiologists independently evaluated the volume of the utricle and saccule. Correlation between tumor volume, vestibular endolymphatic space volume and degree of hearing loss - as evaluated with the levels of pure-tone average and speech recognition threshold - were searched for. RESULTS: The mean saccular, utricular and tumor volumes were 3.17±1.1 (SD) mm3 (range: 1.45-5.7mm3), 14.55±5 (SD) mm3; (range: 6.6-23.9mm3) and 17.4±5.5 (SD) mm3; (range: 8.3-25.4mm3), respectively. There was a moderate correlation between the volume of the utricle and the degree of hearing loss as evaluated with the levels of pure-tone average (rho=0.5; P=0.015) and speech recognition threshold (rho=0.58; P=0.004). There were no significant correlations between saccular and tumor volumes and the degree of hearing loss. CONCLUSION: The volume of the utricle in patients with obstructive vestibular schwannoma moderately correlates with the degree of hearing loss.

Postoperative MR imaging features after superior semicircular canal plugging in Minor syndrome.

PURPOSE: To report the postoperative magnetic resonance imaging (MRI) features after superior semicircular canal plugging in patients with Minor syndrome. MATERIALS AND METHODS: The MRI examinations with 3D T2-weighted SPACE sequence of 12 patients with superior semicircular canal dehiscence syndrome (SCDS) were retrospectively assessed. Two radiologists independently evaluated the presence of a filling defect of the superior semicircular canal above the superior ampulla and the common crus using an oblique plane parallel to the superior semicircular canal (Pöschl's plane). RESULTS: Postoperative MRI showed a filling defect above the ampulla of the superior semicircular canal and the common crus in 8/12 patients (67%). Three patients (3/12; 25%) had a filling defect involving also the superior ampulla that caused postoperative labyrinthitis with labyrinthine enhancement on MRI in 2 patients. One patient (1/12; 8%) had incomplete plugging of superior semicircular canal with abnormal functional tests and remaining symptoms. CONCLUSION: Postoperative MRI shows a normal plugging aspect of the superior semicircular canal in 67% of patients. MRI can reveal complications that may have therapeutic implications.

Infratemporal fossa tumors: When to suspect a malignant tumor? A retrospective cohort study of 62 cases.

OBJECTIVES: Infratemporal fossa (ITF) tumors are rare and little is known about their general epidemiology, making it sometimes difficult for clinicians, who seldom encounter them, to distinguish between benign and malignant forms on the basis of the initial clinical and radiological work-up alone. The objectives of this retrospective study were: (i) to determine the respective prevalences of the various histologic types of ITF tumor, and (ii) to assess associations between certain clinical and radiological features and malignancy. METHODS: A single-center observational study in a university hospital included all new consecutive cases of ITF tumor treated from January 2000 to December 2016. Histologic type, demographics, clinical presentation and imaging findings were analyzed. RESULTS: In total, 62 patients were included. 74% of tumors were benign (n=46) and 26% malignant. Juvenile nasopharyngeal angiofibroma, adenoid cystic carcinoma and schwannoma were the most frequent histologic types, accounting for 47%, 16% and 10% of cases, respectively. The only clinical or imaging signs significantly associated with malignancy were trismus, facial pain, facial hypoesthesia and neural invasion on magnetic resonance imaging (all P-values<0.05). CONCLUSION: This study provides general epidemiological data on ITF tumors, and identified several clinical and radiologic signs to help clinicians suspect malignancy.

Morphological and functional imaging of neck paragangliomas.

OBJECTIVE: To review the optimal techniques for localization and characterization of neck paragangliomas (PGL). MATERIAL AND METHODS: Systematic review of the literature from the PubMed/Medline database. RESULTS: Neck PGL are hypervascular tumours essentially arising from paraganglionic tissue situated at the carotid bifurcation (carotid body) and along the vagus nerve. Morphological and functional imaging are indicated to confirm the diagnosis, identify multifocal disease and for local and regional staging. MR angiography is the noninvasive technique of choice. CT scan and especially CT angiography are excellent alternatives for diagnosis and staging. Conventional arteriography remains useful preoperatively for embolization and occlusion tests. Functional imaging allows localization and characterization of PGLs. Somatostatin receptor scintigraphy (SRS) was the reference imaging technique for staging of sporadic PGLs. The indications for PET imaging have been extended over recent years in parallel with the development of new tracers such as [18F]-FDOPA PET or 68Gallium-labelled DOTA peptides. 68Gallium-labelled DOTA peptides has become the first-line imaging modality in the evaluation of cervical PGLs, regardless of the genetic background. CONCLUSION: Morphological and functional imaging is essential for the staging of neck PGL.

Ossifying fibromas of the paranasal sinuses: diagnosis and management.

Fibro-osseous benign lesions rarely affect the sinonasal tract and are divided into 3 different entities, namely osteoma, fibrous dysplasia and ossifying fibroma. They share several clinical, radiological and histological similarities, but have different behaviours. Ossifying fibroma, and in particular the "juvenile" histological subtype, may have a locally aggressive evolution and a high risk for recurrence if removal is incomplete. The purpose of the present study is to compare the clinical behaviour of ossifying fibroma with the other benign fibro-osseous lesions; highlight different behaviour between the histological subtypes; compare the advantages, limitations and outcomes of an endoscopic endonasal approach with reports in the literature. We retrospectively reviewed 11 patients treated for sinonasal ossifying fibroma at a tertiary care centre. All patients underwent CT scan, and MRI was performed in cases of cranial base involvement or recurrence. Pre-operative biopsy was performed in cases where it was possible to use an endoscopic approach. One patient underwent pre-operative embolisation with ipsilateral visual loss after the procedure. Depending on its location, removal of the tumour was performed using an endoscopic (n = 7), or an external (n = 3) or combined (n = 1) approach. Histopathologically, 5 patients presented the conventional type, 5 the juvenile psammomatoid variant, which was associated in 1 case with an aneurismal bone cyst, and 1 case presented the trabecular juvenile variant. Three patients affected by the juvenile psammomatoid histological variant presented invasion of the skull base and underwent a subtotal removal that subsequently required, due to the regrowth of the remnant, a transbasal approach. Clinical, radiological and histological findings should all be considered to establish differential diagnosis among fibrous osseous lesions. More studies are necessary to conclude if the localisation and extension of the disease at the time of diagnosis is more important than the histological variant. An endoscopic approach is the first choice in most of cases even if an external open approach may be necessary in selected patients.

[Chordoma]. / Les chordomes.

PURPOSES: To review in the literature, all the epidemiological, clinical, radiological, histological and therapeutic data regarding chordomas as well as various notochordal entities: ecchordosis physaliphora, intradural and intraparenchymatous chordomas, benign notochordal cell tumors, parachordomas and extra-axial chordomas. To identify different types of chordomas, including familial forms, associations with tuberous sclerosis, Ollier's disease and Maffucci's syndrome, forms with metastasis and seeding. To assess the recent data regarding molecular biology and progress in targeted therapy. To compare the different types of radiotherapy, especially protontherapy and their therapeutic effects. To review the largest series of chordomas in their different localizations (skull base, sacrum and mobile spine) from the literature. MATERIALS: The series of 136 chordomas treated and followed up over 20 years (1972-2012) in the department of neurosurgery at Lariboisière hospital is reviewed. It includes: 58 chordomas of the skull base, 47 of the craniocervical junction, 23 of the cervical spine and 8 from the lombosacral region. Similarly, 31 chordomas in children (less than 18 years of age), observed in the departments of neurosurgery of les Enfants-Malades and Lariboisière hospitals, are presented. They were observed between 1976 and 2010 and were located intracranially (n=22 including 13 with cervical extension), 4 at the craniocervical junction level and 5 in the cervical spine. METHODS: In the entire Lariboisière series and in the different groups of localization, different parameters were analyzed: the delay of diagnosis, of follow-up, of occurrence of metastasis, recurrence and death, the number of primary patients and patients referred to us after progression or recurrence and the number of deaths, recurrences and metastases. The influence of the quality of resection (total, subtotal and partial) on the prognosis is also presented. Kaplan-Meier actuarial curves of overall survival and disease free survival were performed in the entire series, including the different groups of localization based on the following 4 parameters: age, primary and secondary patients, quality of resection and protontherapy. In the pediatric series, a similar analysis was carried-out but was limited by the small number of patients in the subgroups. RESULTS: In the Lariboisière series, the mean delay of diagnosis is 10 months and the mean follow-up is 80 months in each group. The delay before recurrence, metastasis and death is always better for the skull base chordomas and worse for those of the craniocervical junction, which have similar results to those of the cervical spine. Similar figures were observed as regards the number of deaths, metastases and recurrences. Quality of resection is the major factor of prognosis with 20.5 % of deaths and 28 % of recurrences after total resection as compared to 52.5 % and 47.5 % after subtotal resection. This is still more obvious in the group of skull base chordomas. Adding protontherapy to a total resection can still improve the results but there is no change after subtotal resection. The actuarial curve of overall survival shows a clear cut in the slope with some chordomas having a fast evolution towards recurrence and death in less than 4 years and others having a long survival of sometimes more than 20 years. Also, age has no influence on the prognosis. In primary patients, disease free survival is better than in secondary patients but not in overall survival. Protontherapy only improves the overall survival in the entire series and in the skull base group. Total resection improves both the overall and disease free survival in each group. Finally, the adjunct of protontherapy after total resection is clearly demonstrated. In the pediatric series, the median follow-up is 5.7 years. Overall survival and disease free survival are respectively 63 % and 54.3 %. Factors of prognosis are the histological type (atypical forms), localization (worse for the cervical spine and better for the clivus) and again it will depend on the quality of resection. CONCLUSIONS: Many different pathologies derived from the notochord can be observed: some are remnants, some may be precursors of chordomas and some have similar features but are probably not genuine chordomas. To-day, immuno-histological studies should permit to differentiate them from real chordomas. Improving knowledge of molecular biology raises hopes for complementary treatments but to date the quality of surgical resection is still the main factor of prognosis. Complementary protontherapy seems useful, especially in skull base chordomas, which have better overall results than those of the craniocervical junction and of the cervical spine. However, we are still lacking an intrinsic marker of evolution to differentiate the slow growing chordomas with an indolent evolution from aggressive types leading rapidly to recurrence and death on which more aggressive treatments should be applied.

Radionecrosis of the frontal lobe as a consequence of malignant ethmoid tumor management: incidence, diagnosis, risk factors, prevention and management.

Malignant ethmoid tumors are treated by surgery followed by radiotherapy. This study aimed to evaluate the incidence, risk factors and outcome of radionecrosis of frontal lobe and determine preventive measures. Retrospective study of ethmoid malignancies treated from 2000 to 2011. All patients underwent surgery with/without anterior skull base resection using endoscopic or external approaches followed by irradiation (mean dose 64 Gy). Median follow-up was 50 months. Eight of 50 patients (16 %) presented with fronto-basal radionecrosis, connected to duraplasty, with a latent interval of 18.5 months. Although asymptomatic in six, radionecrosis triggered seizures and required surgery in two cases. Survival was not impacted. Risk factors included dyslipidemia, occurrence of epilepsy and dural resection. Radionecrosis may result from the combination of anterior skull base resection and radiotherapy for the treatment of ethmoid malignancies. Preventive measures rely on improving the duraplasty and optimization of the Gy-dose delivery.

[The impact of early cranioplasty on cerebral blood flow and its correlation with neurological and cognitive outcome. Prospective multi-centre study on 24 patients]. / Impact de la cranioplastie sur l'hémodynamique cérébrale comme facteur pronostic de l'amélioration clinique chez les patients craniectomisés pour traumatisme crânien grave.

INTRODUCTION: Cranioplasty after decompressive craniectomy in patients suffering from severe head injury often leads to a functional improvement although, to date, the pathophysiology of this phenomenon remains unclear. A few hypotheses have been proposed. The impact of cranioplasty on cerebral perfusion could be one explanation. We have evaluated the impact of cranioplasty on the functional status of patients undergoing decompressive craniectomy for severe head injury with its influence on cerebral perfusion. MATERIALS AND METHODS: Twenty-four patients undergoing craniectomy for severe head injury were included in this multi-centric and prospective study. All of them had a cranioplasty within 12 weeks following decompressive craniectomy. A clinical and radiological evaluation was performed prior to and after cranioplasty. Neurological and cognitive evaluation was performed with the Glasgow Outcome Score (GOS), the Frontal Assessment Battery (FAB) and the Mini Mental State Examination (MMSE). Radiological evaluation was performed by perfusion CT scan and transcranial Doppler. RESULTS: A statistically significant neurological and cognitive improvement was observed in 92% of patients at 6 months follow-up (F-U). Brain perfusion was improved at 6 weeks F-U, predominantly in the affected hemisphere. Systolic and diastolic blood velocity flow were improved in both middle cerebral arteries. CONCLUSION: Cranioplasty after decompressive craniectomy for patients suffering from severe head injury probably improves the functional outcome of these patients, thanks to a global improvement of cerebral perfusion.

[Complications from sinonasal surgery]. / Complications de la chirurgie rhinosinusienne.

Endoscopic sinonasal surgery is the main procedure in sinonasal pathology. Complications are rare but potentially severe given the close relationship between the nasal cavities and sinuses and the orbit, skull base and carotid arteries. The different types of surgeries along with the mechanisms of injury, presenting signs and symptoms, and imaging features of the different surgical complications will be reviewed. We will also review the anatomical variants increasing the surgical risk that radiologists should describe on preoperative imaging studies.

Corpus callosum abnormalities in Wilson's disease.

INTRODUCTION: Wilson's disease (WD) with neurological presentation is associated with brain lesions classically localised in globus pallidus, putamen, thalamus, mesencephalon, pons and dentate nucleus. Lesions of corpus callosum (CC) have not been studied in a broad population of patients with WD. OBJECTIVE: Evaluation of the frequency of CC lesions in patients with neurological symptoms related to WD. METHOD: The authors included all patients with neurological expression of WD, followed in the French national centre for WD who had a brain MRI between March 2006 and December 2008. The localisation of brain lesions was analysed and the frequency of lesions in CC evaluated. All patients were assessed using the Unified Wilson's Disease Rating Scale. For patients with abnormalities located in CC, a clinical dysconnexion syndrome was investigated. RESULTS: Among 81 patients (45 men, mean age: 34.8 years, from 12 to 74 years) with neurological expression, 42% had white-matter lesions on fluid-attenuated inversion recovery MRI. 23.4% of patients presented CC lesions, limited to the posterior part (splenium). The severity of disability estimated by Unified Wilson's Disease Rating Scale was correlated with the presence of CC lesions on MRI. CONCLUSION: Abnormalities in CC are not unusual (23.4%). Together with lesions of basal ganglia, CC signal changes should suggest the diagnosis of WD.

Skull bone flap fixation - reliability and efficacy of a new grip-like titanium device (Skull Grip) versus traditional sutures: a clinical randomized trial.

BACKGROUND: After completing a craniotomy, it is important to replace the removed bone flap in its natural position in order to guarantee brain protection as well as improve cosmesis. A skull defect can expose the brain to accidental damage, and in cases of larger defects it may also cause the patients psychosocial problems. The ideal fixation device should provide reliable attachment of the flap to the skull and promote fast bony healing to avoid possible pseudo-arthrosis and/or osteolytic changes. MATERIALS AND METHODS: This is a pilot randomized clinical trial on a series of 16 patients undergoing different craniotomies for benign brain lesions in which the bone flaps were replaced using traditional sutures (Prolene 0.0) in 8 cases and with a new skull fixation device (Skull Grip) in the other 8 (randomly allocated). All patients underwent CT scans of the head with 3D reconstruction at day 1 and day 90 postoperatively to evaluate bone flap position and fusion. These scans were independently reviewed by a neuroradiologist. Cosmesis was also evaluated clinically by the surgeon and radiologically by the neuroradiologist in the 2 patient groups. RESULTS: The new "Skull Grip" device has shown stronger fixation qualities with optimal bone flap fusion and increased cosmetic healing features vs. traditional sutures. CONCLUSION: The "Skull Grip" has shown to be a reliable, effective and stronger bone flap fixation device when compared to traditional sutures.

Eustachian tube melanoma treated by double surgical approach.

OBJECTIVES: We report a case of an eustachian tube melanoma treated by a double surgical approach. MATERIAL AND METHODS: A 56-year-old man consulted for a recurrent left serous otitis media. RESULTS: CT-scan and MRI showed a lesion filling completely the middle ear, extending along the Eustachian tube up to the nasopharynx, with enhancement after injection. The biopsy of the lesion confirmed the diagnosis of malignant melanoma. After left internal carotid artery occlusion, the removal of the lesion was performed by a double surgical approach: combination of an endoscopic transnasal and a retroauricular approach. Radiotherapy was adjuvant therapy. CONCLUSION: Eustachian tube melanoma is a rare occurrence. Surgical treatment must be radical, what represents a challenge considering the localization; it can be obtained with the double surgical approach proposed in our case report.

Sphenoid fungus balls: clinical presentation and long-term follow-up in 24 patients.

Fungus balls are a non-invasive form of fungal infection involving the maxillary sinus in most cases. Sphenoid sinus fungus balls (SSFB) are rare and their clinical presentation is not well described. We intended to define the clinical presentation of sphenoid fungus balls, and retrospectively reviewed 24 cases of SSFB seen at our institution over a 10-year period, identified through pathological reports. Presenting symptoms were separated into three groups: headache, rhinological and asymptomatic. Headaches were subdivided into acute and chronic, unilateral and diffuse. Radiological clues leading to diagnosis were reviewed. Prognosis was determined from medical files or by phone calls. Sixty-seven per cent of patients were female. The mean age at presentation was 65 years. Sixty-two per cent presented with headache, 36% unilateral, mainly in the first trigeminal branch territory. Rhinological symptoms were seen in 21%. In 16% of patients the SSFB was asymptomatic and found during routine tests. SSFB, even if non-invasive, did lead to recurrent bacterial infections and central nervous system complications in three patients. Of 15 patients presenting with headache, 10 were significantly improved post surgery. The prognosis is good, with no recurrence of fungal infection after a main follow-up of 2.3 years. Our study underlines that SSFB present with headaches, often unilateral and in the fronto-orbital region. Proper imaging of the sphenoid sinus is useful in patients with unexplained headache. The neurologist has to be aware of radiological clues suggesting fungal sinus infection, since surgery is the main treatment, with good prognosis and frequent resolution of headaches.

[Association of an arachnoid cyst with heterotopic neuroglial tissue in the internal auditory canal]. / Association d'un kyste arachnoïdien et d'une hétérotopie de tissu glial dans le méat auditif interne.

OBJECTIVES: We report a case of an association of an arachnoid cyst with heterotopic neuroglial tissue in the internal auditory canal. MATERIAL AND METHODS: A 66-year-old woman consulted for cochleovestibular syndrome. RESULTS: MRI demonstrated a lesion with spontaneous hypersignal on T1- and T2-weighted images, instigating surgical exploration. We discovered a hematic arachnoid cyst associated with heterotopic neuroglial tissue arising in the internal auditory canal. CONCLUSION: An arachnoid cyst arising within the cerebellopontine angle or the internal auditory canal is a rare occurrence. Clinical manifestations are identical with those produced by a cochleovestibular schwannoma. MRI usually demonstrates a nonenhancing isointense cystic mass with cerebrospinal fluid on all sequences (hypointense on T1-weighted and hyperintense on T2-weighted images). These lesions are usually monitored. Spontaneous hypersignal on T1- and T2-weighted images makes diagnosis difficult, as in our case, leading to surgical exploration.

Sphenoid and optic nerve sheath meningioma revealed by recurrent brain infarctions.

Meningioma, though benign, may invade adjacent structures such as bone, soft tissues, dural sinuses and arteries. However brain infarctions secondary to meningioma involving the cavernous sinus and encasing and narrowing the intracranial carotid artery are rare. We report the case of a young man with recurrent left carotid artery infarctions due to a left sphenoid meningioma infiltrating the posterior optic nerve sheath through the optic canal and circumscribing the intracranial carotid artery. The patient had a gradually progressive occlusion of the middle cerebral artery, the distal internal carotid artery and finally the anterior cerebral artery ipsilateral to the sphenoid meningioma.