Pre-treatment risk markers for hemorrhagic transformation in posterior circulation acute ischemic stroke treated with reperfusion therapy.

BACKGROUND: Hemorrhagic transformation (HT) is an uncommon complication of posterior circulation acute ischemic stroke (PCS) compared to anterior circulation stroke. Nevertheless, it remains a major concern especially following reperfusion therapy. This study aimed at identifying potential predictive factors associated with HT in PCS. METHODS: Consecutive patients, from a multicenter cohort, with PCS treated by IVT or EVT or the combination of both, were included from December 2015 to May 2019. The European Cooperative Acute Stroke Study criteria was used to identify HT. Potential risk factors were analyzed using univariate and multivariable testing models. RESULTS: A total of 96 patients were included in our study. Median age was 66 (57-83) years, 54 patients (56%) were male and median baseline NIHSS was 8 (4-14). 77 patients (80%) received IVT and 54 patients (56%) benefited from EVT. HT occurred in 19 patients (20%), while sHT occurred in 3 patients (3%). HT was found to be associated with poor functional status at 3 months in univariate analysis (p = 0.0084). Multivariable analysis confirmed that higher baseline NIHSS (OR 1.1008; 95% CI [1.0216-1.1862]; p = 0.0117) and lobar topography of ischemia (OR 4.4275; 95% CI [1.3732-14.2753]; p = 0.0127) were independent predictors of the occurrence of HT. DISCUSSION: HT is associated with increased morbidity in patients with PCS; higher NIHSS and lobar ischemia were independent predictors of HT in our population. Easy-to-use predictive markers may help to tailor therapeutic management of patients with PCS.

Otological Manifestations in Adults with Primary Ciliary Dyskinesia: A Controlled Radio-Clinical Study.

Primary ciliary dyskinesia (PCD) is a rare genetical disease characterized by an abnormal structure or function of the cilia, causing sinusitis, otitis, and bronchiectasis. Hearing loss affects 60% of PCD patients, but data are lacking concerning hearing and temporal bone imaging in adults. Our aim was to describe clinical and radiological ear disease in adults with genetically confirmed PCD. Data were recorded from January 2018 to December 2019. PCD patients were compared with controls with bronchiectasis without PCD. Clinical examination included otomicroscopy and auditory tests. A temporal bone CT scan (TBCT) was systematically performed. Seventeen patients (34 ears) were included in each group. The eardrums were abnormal in 25 (74%) PCD ears versus 8 (24%) ears in the controls (p < 0.05). Conductive hearing loss was more frequent in the PCD group (24% vs. 12% in controls). TBCT were abnormal in 94% PCD patients vs. 32% in the controls (p < 0.05). The Main CT-scan images in PCD were middle ear inflammation (65%), mastoid condensation (62%), or ossicular anomalies (35%). With its excellent sensitivity, TBCT gives typical arguments for PCD diagnosis, adding otological signs to the usual sinus CT signs (hypoplasia, aplasia). Systematic TBCT could be useful in the initial evaluation of patients with suspicion of PCD.

Stability of the standard incus coupling of the Carina middle ear actuator after 1.5T MRI.

Limited data is available concerning the safety of active middle ear implants (AMEI) during Magnetic Resonance Imaging (MRI). Measurements in temporal bones are the gold standard for preclinical assessment of device safety. In this study the coupling stability of an actuator as used in a fully implantable AMEI was determined in temporal bones. Eleven temporal bones were implanted with the actuator according to the manufacturer's surgical guidelines. The actuator was coupled on the incus short process as recommended for sensorineural hearing loss. Temporal bones were exposed 10 times to the MRI magnetic field by entering the MRI suite in a clinically relevant way. Computed Tomography (CT) images were acquired before and after the experiment to investigate the risk of actuator dislocation. Based on the electrical impedance of the actuator, the loading of the actuator to the incus was confirmed. Relative actuator displacement was determined on the CT images by comparing the initial with the final actuator position in 3D space. Impedance curves were analyzed after each exposure to check the loading of the actuator to the ossicles. Analysis of CT images with a 0.30.6 mm in-plane resolution indicate no actuator displacement. The maximum detected change in impedance for all actuators was 8.43 Ω at the actuator's resonance frequency. Impedance curves measured when the actuator was retracted from the short process after the experiment still indicate the presence of a clear resonance peak. No actuator displacement or dislocation could be detected in the analysis of CT images and the measured impedance curves. Impedance curves obtained when the actuator was retracted from the incus short process still show a clear resonance peak, indicating the device is still functional after the MRI exposures.

Angiographic Signatures of the Predominant Form of Familial Transthyretin Amyloidosis (Val30Met Mutation).

PURPOSE: To describe abnormalities in choroidal and retinal vasculature associated with Val30Met familial transthyretin amyloidosis (V30M-FTA) using fluorescein and indocyanine green (ICG) angiography. DESIGN: Prospective, cross-sectional study. METHODS: This study was conducted at the French National Reference Center for FTA. We included 18 consecutive genetically confirmed V30M-FTA patients (36 eyes) who underwent complete neurologic examination, including staging with polyneuropathy disability (PND) score, and complete ophthalmic evaluation, including staging of intraocular amyloid deposits and fluorescein and ICG angiograms (ICG-A). The grading of choroidal and retinal angiopathy, and their association with neurologic functional impairment, were the main outcome measures. RESULTS: Eleven men and 7 women, mean age 61.6 ± 12.1 years, were included. Retinal amyloid angiopathy (RAA) was detected in 24 eyes (92%) of 13 patients, with microaneurysms, retinal hemorrhages, and retinal ischemia of variable extent. Three patients (5 eyes) had neovascular glaucoma and 2 (2 eyes) had preretinal neovascularization. ICG-A indicated choroidal amyloid angiopathy (CAA) in all patients, with 3 distinct patterns-diffuse (9/18 patients), focal (5/18 patients), or punctiform (4/18 patients)-based on the extent of late hypercyanescence along the choroidal arteries. PND scores were significantly higher in patients with diffuse CAA (firework pattern) compared to those with limited CAA (focal and punctiform patterns) (2.89 vs 1.78, P = .045). CONCLUSION: RAA is a frequent and severe complication of V30M-FTA that may lead to anterior and posterior segment neovascularization. CAA was detected in all patients, with a late hypercyanescent delineation of the choroidal arterial vasculature, which was more extensive with increased disease severity.

[Techniques and progress in the imaging of the ear]. / Les techniques et avancées dans l'imagerie de l'oreille.

Ear and temporal bone imaging is essential for the diagnostic and preoperative management of middle ear lesions. The scanner is the exam of choice to analyze the walls and the contents of the middle ear. MRI is used to characterize the opacities of the middle ear and to evaluate possible neurological complications. Modern imaging techniques allow intraoperative guidance in otological surgery. Hearing implants are not always a contraindication to MRI but require precautions according to the type of implant.

Sunitinib for the treatment of benign and malignant neoplasms from von Hippel-Lindau disease: A single-arm, prospective phase II clinical study from the PREDIR group.

Von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary cancer syndrome that predisposes affected individuals to the development of multiple benign and malignant tumors. One of the main manifestations of VHL is renal cell carcinoma (RCC). RCC is increasingly being treated with targeted therapies, which offer an alternative treatment option for patients with VHL disease. This study investigated the effectiveness of sunitinib in VHL patients with advanced tumors or tumors unsuitable for surgery.This multicenter, phase II, open-label study from the PREDIR VHL network, treated patients with genetically-confirmed advanced VHL disease with oral sunitinib (50 mg/day for 28 days then a 2-week rest period) until progression. Lesions were performed using magnetic resonance imaging (MRI) and computed tomographic (CT) scan. The primary endpoint was objective response rate; secondary endpoints included tolerability and overall survival.All five patients showed stable disease as best response at 6 months. Two patients showed impressive transitory clinical improvement during early cycles. No patient died during sunitinib treatment. Reasons for discontinuing sunitinib therapy were disease progression (n=1), unacceptable toxicity (n=3) and lack of clinical improvement after 7 cycles (10.5 months) with unacceptable toxicity (n=1).In conclusion, sunitinib was of limited benefit in patients with advanced VHL disease, but had better efficacy against metastatic RCC than other VHL-related lesions. Treatment-related toxicity is an important limiting factor in this frail patient population. New agents with different mechanisms of action are required to treat this disease.

Diffusion tensor magnetic resonance imaging of trigeminal nerves in relapsing herpetic keratouveitis.

BACKGROUND: Corneal hypoesthesia is the landmark of HSV and VZV keratitis and can lead to neurotrophic keratitis. Diffusion tensor imaging (DTI) is a new magnetic resonance imaging (MRI) derived technique, which offers possibilities to study axonal architecture. We aimed at assessing the potential impact of recurrent HSV or VZV-related keratitis on the axonal architecture of trigeminal nerves using DTI. DESIGN: Prospective non-interventional study. PARTICIPANTS: Twelve patients and 24 controls. METHODS: DTI using MRI of the trigeminal fibers and corneal esthesiometry using the Cochet-Bonnet esthesiometer were acquired for patients affected by unilateral and recurrent HSV or VZV-related keratitis (3 months after the last corneal inflammatory event), and control subjects with no history of ocular or neuronal disease affecting the trigeminal pathways. MAIN OUTCOME MEASURES: Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were compared between the 2 eyes of both patients and controls, and correlated with corneal esthesiometry. RESULTS: FA was lower in the trigeminal fibers ipsilateral to the affected eye compared to the non-affected side (0.39±0.02 versus 0.46±0.04, P=0.03). This difference was more important than the intra-individual variability observed in controls. Concomitantly, the asymmetry in ADC results was significantly correlated with the loss of corneal sensitivity in the affected eye. CONCLUSIONS: Corneal hypoesthesia related to HSV and VZV keratitis is associated with persistent modifications in the architecture and functionality of the trigeminal fibers. These results add further explanation to the pathogenesis of HSV and VZV-induced neurotrophic keratitis, which may occur despite an apparent quiescence of the disease.

Endoscopic removal of large orbito-ethmoidal osteoma in pediatric patient: Case report.

INTRODUCTION: Osteoma is a benign slow growing bone tumor with a prevalence of 3% of all benign paranasal sinuses tumors, with a peak incidence between the fourth and sixth decades, mostly involving frontal sinuses.(1) PRESENTATION OF CASE: We present a case of a large right ethmoidal sinus osteoma in a 12-year-old boy, complaining of frontal headaches and excessive lacrimation of the right eye. CT scan showed a very large tumor in the right anterior ethmoidal sinus (30mm×25mm×15mm). DISCUSSION: Large osteomas of the paranasal sinuses are usually resected by external approaches. However, the minimally invasive endonasal approach, which minimizes external facial scarring, is challenging for such large lesions in pediatrics. In the presented case, the osteoma was successfully resected exclusively by endoscopy-guided endonasal approach assisted by neuronavigation, with no peri or postoperative complications. CONCLUSION: An endoscopic approach assisted with neuronavigation may be a minimally invasive and safe procedure for managing large osteoma of the ethmoidal sinus in pediatrics patients.

Computed tomography of the anterior skull base in Kallmann syndrome reveals specific ethmoid bone abnormalities associated with olfactory bulb defects.

CONTEXT: Kallmann syndrome (KS) is characterized by congenital hypogonadotropic hypogonadism (CHH) and an impaired sense of smell related to defective development of the olfactory system. OBJECTIVE: The aim of the study was to use high-resolution computed tomography (CT) to detect specific abnormalities in the ethmoid bone region surrounding the olfactory bulbs in patients with KS. PATIENTS: Thirty-seven KS patients were compared to normosmic CHH (nCHH) patients (n = 15) and controls (n = 30) of similar age. DESIGN AND METHODS: We conducted a prospective study in a single referral center. Subjects underwent CT in bone windows with axial, coronal, and sagittal reconstructions centered on the olfactory fossa (OF) and cribriform plate (CP). We characterized the OF structure by measuring OF height, width, and surface area and a series of angles. The CP foramina were counted bilaterally. Olfactory bulb magnetic resonance imaging, performed in parallel, was compared with CT findings. RESULTS: OF height, width, and surface area were all significantly lower in KS patients than in nCHH patients and controls (P < .0001). KS patients also had wider angles than nCHH patients and controls (P < .0001). KS subjects with olfactory bulb agenesis on magnetic resonance imaging or who harbored KAL1 mutations had the most marked changes in OF measurements and angles. Coronal OF height distinguished KS patients from controls with the best sensitivity and specificity. The mean number of CP foramina was similar in KS, nCHH, and control subjects. CONCLUSIONS: KS is associated with specific ethmoid bone abnormalities. The preserved number of CP foramina in KS patients suggests that the integrity of olfactory structures is not mandatory for their formation during fetal development or their maintenance in adult life.

Surgical treatment of posttraumatic syringomyelia.

OBJECTIVE: The present study evaluates the effectiveness of 2 surgical procedures, shunting and untethering, for posttraumatic syringomyelia. METHODS: We retrospectively reviewed the medical charts of all surgical patients with posttraumatic syringomyelia in our department. Shunting was performed before 1997; after 1997, we used arachnoidolysis and untethering. RESULTS: Shunting was performed in 15 patients, and 19 patients underwent arachnoidolysis. Statistical analysis found that the 2 groups did not differ in age or initial clinical or radiological presentation. All patients suffered from progressively worsening symptoms. Reconstruction of the subarachnoid space by arachnoidolysis and untethering the cord allowed us to improve or stabilize 94% of our patients. Shunting exposed the patients to a higher rate of clinical recurrence and reoperation. Comparisons between the 2 groups found a significant difference (better results) in favor of arachnoidolysis for the McCormick classification (P = .03), American Spinal Injury Association motor score of the lower extremities (P = .02), and subjective grading (P = .001). There was no significant difference in the evolution of pain or the Vaquero index between the 2 groups; however, a tendency appeared in favor of arachnoidolysis for cyst evolution in regard to the extent of the cyst and the Vaquero index (P = .05). CONCLUSION: Our results confirmed that arachnoidolysis is an effective and safe treatment for posttraumatic syringomyelia. Because the majority of patients were stabilized, we concluded that surgery should be performed as soon as possible in patients with clearly progressing clinical features.

Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases.

OBJECTIVE: To determine the long-term outcome of surgically treated Chiari-related syringomyelia. METHODS: The medical charts of 157 consecutive surgically treated patients with Chiari-related syringomyelia were retrospectively analyzed. Factors predicting outcome, either clinical or radiological, are discussed, and our results are compared with those of other large series in the literature. RESULTS: The study included 74 men and 83 women (age range, 16-75 years; mean age at surgery, 38.3 years). Pain and sensory disturbance were the most frequent initial symptoms. The average duration of preoperative symptoms was 8.2 years. The follow-up period ranged from 82 to 204 months (median, 88 months). At the end of the study, 99 patients (63.06%) had improved, 48 (30.58%) had stabilized, 9 (5.73%) had worsened, and 1 (0.63%) had died during the postoperative period. Factors predicting improvement or stabilization were young age at the time of surgery and clinical signs of paroxysmal intracranial hypertension. Factors associated with a poor outcome were older age at the time of surgery, arachnoiditis, and a clinical feature of long-tract impairment syndrome. The presence of arachnoiditis or of basilar invagination was associated with poor clinical presentation (P = 0.05 and 0.0001, respectively). The extent of the cyst on postoperative magnetic resonance imaging was a predictor of poor clinical outcome (P = 0.002). CONCLUSION: Our results confirmed that surgery is an effective and safe treatment of Chiari-related syringomyelia, with a 90% chance of long-term stabilization or improvement on average. Surgery should be proposed as soon as possible in patients with clearly progressing clinical features.

Intramedullary spinal ependymomas: analysis of a consecutive series of 82 adult cases with particular attention to patients with no preoperative neurological deficit.

OBJECTIVE: Surgery should be considered for patients with intramedullary spinal ependymomas (ISE), particularly those presenting with a neurological deficit preoperatively. In contrast, it is still a debatable matter whether to recommend the same approach for patients with no neurological impairment. To investigate this matter, we analyzed the data of 82 consecutive patients with ISEs treated at our institution. METHODS: We reviewed the medical charts of all ISE patients undergoing operation at our institution between 1985 and 2000. Particular attention was given to patients without neurological deficit before surgery. RESULTS: Eighty-two consecutive ISE patients were included in this study. Preoperatively, a neurological deficit of variable severity was present in 72 patients (Group A, 88%) and absent in 10 patients (Group B, 12%). In the latter group, seven patients had progressive and nonspecific pain as the only presenting symptom; two had arm dysesthesias; and in one patient, ISE was diagnosed incidentally. The mean duration of their symptoms was 21 months. We achieved a total tumor excision in nine patients and subtotal removal in one. At the last follow-up assessment (mean, 45 mo), all Group B patients remained at Grade I of the McCormick classification, except one, who deteriorated to Grade Ib. Furthermore, nonspecific pain diminished in three patients, stabilized in four, and worsened in one. Arm dysesthesias diminished in one patient and stabilized in the other. No surgery-related complication or recurrence was recorded in these patients. CONCLUSION: Surgery should be carefully considered for ISE patients with no objective neurological deficit preoperatively because, in our experience, it resolves their preoperative complaints in 30% of cases, stabilizes them in 60%, and worsens them in 10%.