Monostotic fibrous dysplasia of the clivus: image-guided endoscopic endonasal transsphenoidal surgery.

INTRODUCTION: Fibrous dysplasia is a non-heritable, congenital disease involving the bones. Its diagnosis relies on image examination and pathology. Treatment is usually conservative, but in cases of cranial nerve impingement or extension to the condyles, surgery should be considered. In this paper, we describe a singular case of monostotic fibrous dysplasia of the clivus in a child. CASE REPORT: A 14-year-old boy presented with chronic headache and diplopia. A neurological examination revealed palsy of the right VIth cranial nerve. The radiological findings were consistent with fibrous dysplasia, with a hypointense and isointense clival lesion on T (1)- and T (2)-weighted images, respectively. Surgical treatment was indicated by cranial nerve impairment. An endoscopic endonasal approach with an image-guided system was used. The patient was discharged four days after surgery and his diplopia improved postoperatively. DISCUSSION: A variety of surgical approaches have been used in the treatment of midline extra-axial cranial base tumors. Fibrous dysplasia of the clivus without expansion to the condylus can be removed with a transsphenoidal endonasal endoscopic approach. Preoperative imaging reveals the individual patient's anatomy and improves the intraoperative orientation. CONCLUSION: Image-guided transsphenoidal endoscopic surgery is recommended for its safety and minimal invasiveness.

Different perceived foreign accents in one patient after prerolandic hematoma.

Foreign accent syndrome (FAS), a rare disorder characterized by the emergence of a new accent perceived as foreign by listeners, is usually reported with left brain damage. We here report the case of a 28-year-old native Brazilian who appeared, to the examiner, to show a North American accent during recovery from Broca's aphasia. The lesion was due to a frontal hematoma. Without referring specifically to speech, we asked 10 independent observers to comment on a videotape of the patient's interview. Seven reported that the patient had a foreign accent, while 3 simply noted a 'strange' accent. The observers did not agree on the origin of the accent, 5 identifying it as Spanish, 1 as German, and 1 as south Brazilian. These findings suggest that FAS is not due to the acquisition of a specific foreign accent, but to impairment of the suprasegmental linguistic abilities (tone, accent, pauses, rhythm, and vocal stress) that make it possible to distinguish native language.