Extremely delayed renal cell carcinoma metastasis mimicking convexity meningioma.

Cerebral extra-axial metastases mimicking meningioma are extremely rare. Imaging characterictics may not always differentiate between meningioma and metastatic tumors. A 68-year-old-woman who had been operated for renal cell carcinoma 20 years ago presented with new symptoms of an intracranial mass lesion. A large extra-axial convexity mass destroying the calvarium and dura was excised with Simpson Grade I removal. The pathology examination revealed metastatic carcinoma. Such tumors that satisfy several criteria for a diagnosis of meningioma, but prove instead to be metastatic carcinoma form the focus of our discussion. A meticulous clinical evaluation and histopathological diagnosis is essential in patients with an intracranial mass whether the lesion looks like a primary or metastatic tumor on the first evaluation.

Pitfalls in the management of Cushing's disease.

Cushing's disease is caused by functional corticotroph adenomas of the pituitary gland, most commonly noninvasive microadenomas. Transsphenoidal microsurgery is an effective means of control for patients with adrenocorticotrophic hormone-producing microadenomas. However, a wide variation of clinical outcomes and recurrence rates has been reported. The major causes of surgical failure in the treatment of Cushing's disease lies in inadequate preoperative evaluation, unsuccessful identification of the adenoma and inexperience of the surgeon. Furthermore, appropriate use of combination therapy, including surgery, radiotherapy, radiosurgery and adrenalectomy can improve the outcome. For optimal results in this rare disease, endocrinological, radiological and surgical procedures should be co-ordinated in a specialized center. In this review, factors affecting preoperative evaluation, surgical success and outcome are outlined in the light of current knowledge.

Microsurgical anatomy of the hypoglossal nerve.

The aim of the present study was to review the surgical anatomy of the hypoglossal nerve (HN), to reveal its relationships on its course and to provide some landmarks to its identification. Ten cadaveric head dissections (20 sides) were performed using microsurgical techniques. The anatomical relationships between the HN and other nerves, muscles, arteries and veins were carefully recorded, and some measurements were made between the HN and related structures. Thus, various landmarks were determined for the easy identification of the HN. In addition, the hypoglossal triangle, which containes major vascular structures, is described. The HN is divided into three main parts: cisternal, intracanalicular and extracranial. The HN arises from the medulla as a line of rootlets situated along the anterior margin of the lower two-thirds of the olive in the preolivary sulcus. It is the newly described 'hypoglossal' triangle in the anterior neck that is bordered by the descending hypoglossus laterally, transverse hypoglossus inferiorly and inferior border of the stylohyoid muscle superiorly. In our specimens, we determined that the HN was 3-7 mm (mean 5 mm) inferior to the digastric tendon, as well as mostly superficial. The occipital artery arose from the posterior surface of the external carotid artery (ECA) 6-9 mm (mean 7 mm) above the carotid bifurcation. There is also an important 'cross' between the occipital artery and the HN. In all cadavers, this crossing point was 7-9 mm (mean 8 mm) superior to the emergence of the occipital artery from the ECA. In conclusion, understanding the detailed anatomy of the HN and using landmarks to identify the nerve are crucial for surgery in the region.

Molecular genetic analysis of two large kindreds with intracranial aneurysms demonstrates linkage to 11q24-25 and 14q23-31.

BACKGROUND AND PURPOSE: Both environmental and genetic factors contribute to the formation, growth, and rupture of intracranial aneurysms (IAs). To search for IA susceptibility genes, we took an outlier approach, using parametric genome-wide linkage analysis in extended IA kindreds in which IA is inherited as a simple Mendelian trait. We hereby present the molecular genetic analysis of 2 such families. METHODS: For genome-wide linkage analysis, we used a 2-stage approach. First, using gene chips in affected-only analysis, we identified genomic regions that provide maximum theoretical logarithm of odds (lod) scores. Next, to confirm or exclude these candidate loci, we genotyped all available family members, both affected and unaffected, using polymorphic microsatellite markers located within these regions. RESULTS: We obtained significant lod scores of 4.3 and 3.00 for linkage to chromosomes 11q24-25 and 14q23-31, respectively. CONCLUSIONS: Molecular genetic analysis of 2 large IA kindreds confirms linkage to chromosome 11q and 14q, which were suggested to contain IA susceptibility genes in a previous study of Japanese sib pairs. Independent identification of these 2 loci strongly suggests that IA susceptibility genes lie within these regions. While demonstrating the genetic heterogeneity of IA, these results are also an important step toward cloning IA genes and ultimately understanding its pathophysiology.

Risky anatomic variations of sphenoid sinus for surgery.

We searched for the surgically risky anatomic variations of sphenoid sinus and aimed to compare axial and coronal tomography in detection of these variations. Fifty-six paranasal tomography images (112 sides) were evaluated for coronal, axial and both coronal and axial images. Tomographic findings including bony septum extending to optic canal or internal carotid artery; protrusions and dehiscences of the walls of internal carotid artery, optic nerve, maxillary nerve and vidian nerve; extreme medial course of internal carotid artery; patterns of aeration of the anterior clinoid process; and Onodi cells were evaluated. The results were classified as "present, absent, suspicious-thin (only for dehiscence) or no-consensus". The results of each plane were compared with that of the result of the both planes together. Kappa coefficient and Chi-square tests were used to compare both planes. Twelve cadaveric dissections were performed to reveal the proximity of sphenoid sinus to surgically risky anatomic structures. Endoscopy was applied to five cadavers. 18 evaluations were classified as 'no-consensus'. We detected 34, 35, 34 and 40 protrusions of internal carotid artery, optic nerve, maxillary nerve, vidian nerve, respectively. Dehiscences were present in 6, 9, 4 and 8, and suspicious-thin in 8, 10, 16 and 25 in canals of internal carotid artery, optic nerve, maxillary nerve and vidian nerve, respectively. Bony septum to internal carotid artery and optic nerve was observed in 30 and 22 cases. We observed 9 extreme medial courses of internal carotid artery, 27 aerated clinoid process and 9 Onodi cells. Axial images were superior in detection of bony septum to internal carotid artery and Onodi cells; while the coronal images were more successful in detection of protrusion of optic nerve and vidian nerve, and dehiscense of maxillary nerve and vidian nerve (P<0.05). In cadaveric dissections, the septa were inserted into the bony covering of the carotid arteries in two sinuses (8.3%). Detailed preoperative analysis of the anatomy of the sphenoid sinus and its boundaries is crucial in facilitating entry to the pituitary fossa and reducing intraoperative complications. Coronal tomography more successfully detects the sphenoid sinus anatomic variations.

Prevalence of primary tethered cord syndrome associated with occult spinal dysraphism in primary school children in Turkey.

The prevalence and associated factors of primary tethered cord syndrome (PTCS) in primary school children were investigated. A cross-sectional study was performed in four demographically different primary schools in Turkey. Demographic, familial and physical data were collected from 5,499 children based on enuresis as a predominant symptom and dermatologic and orthopedic signs as clues of occult spinal dysraphism. Statistical analysis and input of the data were carried out with the SPSS package program 10.00, and logistic regression analysis was used to identify discriminating factors between enuretic children with or without neurologic signs. Of 5,499 analyzed children, 422 (7.7%) had enuresis nocturna, and 19.9% of 422 children had also daytime incontinence. Sixteen of these 422 enuretic children (3.8%) had several dermatologic signs. Five of them had spina bifida on plain radiographies, and 4 of them had cord tethering on lumbar MRI. Fifteen of 422 enuretic children (3.7%) had gait disturbances and orthopedic anomalies without cutaneous manifestations. Six of 15 children had spina bifida on plain graphies and 2 of them had tethered cord syndrome on MRI. The general prevalence of PTCS was found to be 0.1% of 5,499 analyzed children and 1.4% of enuretic children. A good outcome after untethering was found in 83.0% in this series. Practitioners should be aware of these clues of occult spinal dysraphism and resort to further radiologic and neurosurgical assessment. Early surgical intervention may halt the progression of the neurologic deficits and stabilize or reverse symptoms.

Bilateral carpal tunnel syndrome associated to familial Mediterranean fever.

A unique case of bilateral severe carpal tunnel syndrome due to familial Mediterranean fever is reported. The syndrome was diagnosed by clinical examination and electrophysiological studies. Bilateral transverse carpal ligaments were released and the biopsy specimens revealed systemic type A amyloidosis. Up to our knowledge, the co-existence of bilateral carpal tunnel syndrome and familial Mediterranean fever has not been reported previously in the literature.

Proximally situated osseous septum in complex spina bifida. Case report.

In Type I split cord malformation (SCM) lesions, the osteocartilaginous spur and its dural sleeve are almost always located in the caudal extent of the median cleft. The authors present a case of lumbar myelomeningocele associated with a thoracic Type I SCM in which an osseous septum is uniquely situated in the proximal extent of the median cleft. Split cord malformations are cord-tethering lesions, which may be associated with a myelomeningocele that causes additional tethering, even in the opposite direction; therefore, both lesions should be treated. In view of this unique case, however, the surgical approach used for tethering lesions in such cases of complex spina bifida should perhaps be tailored. Whenever feasible, meticulous preoperative examination of a patient with spina bifida, including entire craniospinal magnetic resonance imaging followed with perioperative dynamic evaluation, is important for the effectiveness and safety of the surgery.

Postoperative spinal ultrasonography findings in spinal dysraphia.

Diastematomyelia is a form of spinal dysraphism involving sagittal clefting of the spinal cord, conus medullaris, and/or filum terminale into two hemicords. It can be an isolated finding or can be associated with meningomyelocele or meningocele. In this report, we present postoperative spinal ultrasonography findings in a patient with diastematomyelia and a tethered cord.

Suprasellar germ cell tumor with subarachnoid seeding MRI and MR spectroscopy findings.

A case of suprasellar germinoma with subarachnoid seeding is presented. Patient had a history of depression unresponsive to drug therapy and recently developed diabetes insipidus. MR imaging revealed a huge suprasellar mass, heterogenous in signal intensity due to cystic components. MR spectroscopy of the mass showed prominent lipid peak suggesting high malignant potential.