Osteoblastoma of the temporal articular tubercle misdiagnosed as a temporomandibular joint disorder.

The case of a 17-year-old female with a benign osteoblastoma in the temporomandibular joint (TMJ) is reported. The patient had a 2.5-year history of reduced mouth opening accompanied by tenderness and swelling in the left TMJ. Initial treatment included stabilization of the occlusion with a splint, jaw exercises, and analgesics. At first the patient's symptoms decreased, but they then increased 18 months later, prompting a cone beam computed tomography (CBCT) evaluation of the joint. The radiographic findings showed a somewhat ill-defined, radiolucent, expansile lesion containing small scattered calcifications located in the temporal articular tubercle. The lesion was removed under general anaesthesia and sent for histopathological examination. At the 12-month follow-up, the patient had normal TMJ function without clinical symptoms. CBCT examination showed a small recurrence of 3mm. Another 12 months later, CBCT showed a 1-mm increase in the recurrence. Her function was normal, with slight tenderness lateral to the left TMJ. The decision from a multidisciplinary meeting was further annual follow-up. The present case illustrates the importance of initial radiographic examination together with clinical examination in patients with TMJ dysfunction.

Underlying sources of cognitive-anatomical variation in multi-modal neuroimaging and cognitive testing.

Healthy adults have robust individual differences in neuroanatomy and cognitive ability not captured by demographics or gross morphology (Luders, Narr, Thompson, & Toga, 2009). We used a hierarchical independent component analysis (hICA) to create novel characterizations of individual differences in our participants (N=190). These components fused data across multiple cognitive tests and neuroanatomical variables. The first level contained four independent, underlying sources of phenotypic variance that predominately modeled broad relationships within types of data (e.g., "white matter," or "subcortical gray matter"), but were not reflective of traditional individual difference measures such as sex, age, or intracranial volume. After accounting for the novel individual difference measures, a second level analysis identified two underlying sources of phenotypic variation. One of these made strong, joint contributions to both the anatomical structures associated with the core fronto-parietal "rich club" network (van den Heuvel & Sporns, 2011), and to cognitive factors. These findings suggest that a hierarchical, data-driven approach is able to identify underlying sources of individual difference that contribute to cognitive-anatomical variation in healthy young adults.

Cognitive and anatomical data in a healthy cohort of adults.

We present data from a sample of 190 healthy adults including assessments of 4 cognitive factor scores, 12 cognitive tests, and 115 MRI-assessed neuroanatomical variables (cortical thicknesses, cortical and sub-cortical volumes, fractional anisotropy, and radial diffusivity). These data were used in estimating underlying sources of individual variation via independent component analysis (Watson et al., In press) [25].

The Veterans Affairs Saint Louis University mental status exam (SLUMS exam) and the Mini-mental status exam as predictors of mortality and institutionalization.

OBJECTIVE: To evaluate predictive validity of cognitive dysfunction of the Saint Louis University mental status (SLUMS) exam or mini-mental state exam (MMSE) for institutionalization and mortality after 7.5-years. DESIGN: Longitudinal study. SETTING: Geriatric Research, Education and Clinical Center Veterans Affairs Hospital St. Louis, MO. PARTICIPANTS: Patients (N=705) were screened for cognitive dysfunction in 2003 using the SLUMS exam and MMSE, and mortality and institutionalization up to 7.5-years later were evaluated as outcome measures. MEASUREMENT: The associations between outcome measures and MMSE and SLUMS exam total scores, and cognitive status were examined using Kaplan-Meier curves and Cox proportional-hazards regression. RESULTS: Five hundred thirty-three charts were reviewed, 176/533(33%) patients had died and 31/526 (6%) were institutionalized during 7.5-year follow-up period. All subjects were male with a mean age of 75 years and most had high school education or greater (71%). MMSE dementia, SLUMS dementia (ps<.001) and MCI (p<.05) groups had significantly lower survival rates than normal cognition group in the Kaplan-Meier curves. Scores classified as dementia on SLUMS (HR=2.4, 95% CI 1.6-3.7; p <.001) or MMSE (HR=2.3, 95% CI 1.5-3.6; p <.001) both predicted mortality and, also, institutionalization (SLUMS: HR=3.5, 95% CI 1.3-9.1; p <.01; MMSE: HR=3.8, 95% CI 1.6-9.0; p <.001) after adjustment for covariates. Unadjusted SLUMS exam MCI predicted morality (HR=1.5, 95% CI 1.1-2.2; p <.019) but not institutionalization. CONCLUSION: The SLUMS exam and MMSE both predict mortality and institutionalization for male patients screened as positive for dementia.

Call-Fleming syndrome associated with subarachnoid haemorrhage: three new cases.

The Call-Fleming syndrome (CFS) comprises acute severe recurrent (thunderclap) headaches, occasional transient or fluctuating neurological abnormalities and reversible segmental cerebral vasoconstriction. It is a benign condition with an excellent prognosis, yet because it is often clinically and radiologically similar to a number of commonly encountered conditions, diagnostic difficulties may arise, leading to inappropriate, and even potentially harmful, investigative and therapeutic approaches. Three personal cases are presented to highlight the occurrence of subarachnoid haemorrhage (SAH) as part of CFS. In two patients with a positive CT head, SAH involved the sulci in the upper cerebral convexity, an unusual location in aneurysmal SAH. SAH is not an uncommon feature of CFS, occurring in approximately 25% of reported cases, and may pose a diagnostic challenge. CFS has a relatively characteristic spectrum of features, allowing a confident diagnosis in most cases, even when atypical features such as SAH are present. Recognising the spectrum of abnormalities seen in CFS, including particularly SAH, allows a sound approach to a safe diagnosis.

The ectomesenchymal chondromyxoid tumor: a review.

The ectomesenchymal chondromyxoid tumor is a relatively recently described neoplasm that appears to involve uniquely the oral cavity, particularly the tongue. Thirty well-accepted cases have been reported since the initial description of this lesion in 1995. While a wide age range (9-78 years) has been documented, most of these tumors are diagnosed from the third to sixth decades of life. No sex predilection is seen. The size of the neoplasm is typically <2 cm, and most affect the anterior dorsal tongue. The duration of the lesion was difficult to gauge, probably due to the asymptomatic nature of the process. Some tumors, however, were well documented to have been present for as long as 10-20 years. Histopathologically, the ectomesenchymal chondromyxoid tumor is characterized by a well circumscribed, but unencapsulated, lobular growth pattern. Varying degrees of cellularity are noted, with the lesional cells often set in a myxoid, chondroid or hyalinized background. Immunohistochemical studies reveal positivity of the lesional cells for antibodies directed against glial fibrillary acidic protein, cytokeratins, S-100 protein and CD-57 in the majority of tumors. Treatment consists of conservative surgical excision, and while recurrence is possible, it has been noted in <10% of reported cases.

Review of linear epidermal nevus with oral mucosal involvement--series of five new cases.

INTRODUCTION: Linear epidermal nevi are sporadic hamartomatous alterations of the epidermis and superficial dermis that clinically appear as verrucous papules and plaques distributed in a linear pattern following Blaschko's lines. Their extent varies from unilateral involvement (nevus unius lateris) to extensive bilateral involvement (ichthyosis hystrix). Oral mucosal lesions have rarely been described. AIMS: We review the literature, focusing on the rare intraoral manifestations of linear epidermal nevus. CASE SERIES: We present a series of five new cases with oral mucosal involvement. Four cases had associated cutaneous lesions and one case had oral lesions exclusively. Histopathologic evaluation of lesional tissue in four cases showed hyperkeratosis, acanthosis, epithelial hyperplasia, and papillomatosis. Dental abnormalities, consisting of enamel hypoplasia and congenitally missing teeth, were noted in one patient adjacent to the oral lesions.

Call-Fleming syndrome associated with subarachnoid haemorrhage: three new cases.

BACKGROUND: The Call-Fleming syndrome (CFS) comprises acute severe recurrent (thunderclap) headaches, occasional transient or fluctuating neurological abnormalities and reversible segmental cerebral vasoconstriction. It is a benign condition with an excellent prognosis, yet because it is often clinically and radiologically similar to a number of commonly encountered conditions, diagnostic difficulties may arise, leading to inappropriate, and even potentially harmful, investigative and therapeutic approaches. CASES: Three personal cases are presented to highlight the occurrence of subarachnoid haemorrhage (SAH) as part of CFS. In two patients with a positive CT head, SAH involved the sulci in the upper cerebral convexity, an unusual location in aneurysmal SAH. RESULTS: SAH is not an uncommon feature of CFS, occurring in approximately 25% of reported cases, and may pose a diagnostic challenge. CFS has a relatively characteristic spectrum of features, allowing a confident diagnosis in most cases, even when atypical features such as SAH are present. CONCLUSIONS: Recognising the spectrum of abnormalities seen in CFS, including particularly SAH, allows a sound approach to a safe diagnosis.

Training improves medical student performance in image interpretation.

The aim of this study was to objectively quantify the effects of radiological teaching on student performance in interpreting radiological images, and to establish whether training location affects performance. 114 clinical students were asked to anonymously interpret radiological cases before and after 26 weeks clinical training. Improvements were assessed by comparing performance on the median question in each assessment using the Kruskal-Wallis analysis. Variations according to different placements were assessed by the Mann-Whitney U-test. There was a highly significant (p<0.001) improvement in the performance of the group with training. The proportion of correct answers to the median question improved from 8% to 43%. Differences between training placements were non-significant. Our study suggests that radiology teaching significantly improves student performance. Future work should look to define the contribution of "clinical exposure" towards this improvement, as well as the various teaching methods employed.

The association between Viadent use and oral leukoplakia.

Recently, leukoplakia of the maxillary vestibule was described in patients with no traditional risk factors but who had used Viadent products. We designed a case-control study to evaluate the hypothesis that Viadent users were more likely to have lesions. One hundred and forty-eight cases and controls were identified through the Section of Oral and Maxillofacial Pathology. Cases and controls were administered a questionnaire about Viadent use and other known risks. Results of crude, stratified, and logistic regression analyses showed that use of Viadent products was a risk indicator for leukoplakia (adjusted OR = 9.7, 95% CI = 4.7-21.6), with a strong dose-response relation.

Modification of Rab5 with a photoactivatable analog of geranylgeranyl diphosphate.

A photoprobe analog of geranylgeranyl diphosphate (2-diazo-3,3,3-trifluoropropionyloxy-farnesyl diphosphate or DATFP-FPP) inhibits mevalonate-dependent prenylation of in vitro translated Rab5 in rabbit reticulocyte lysate, suggesting that it competes for lipid binding to the Rab geranylgeranyl transferase. Modification of Rab5 with DATFP-FPP, demonstrated by gel mobility shift and Triton X-114 phase separation experiments, confirms that the enzyme uses the analog as a substrate. The sedimentation of DATFP-modified Rab5 as a larger mass complex on sucrose density gradients indicates that it binds to other factors in rabbit reticulocyte lysate. Most importantly, DATFP-Rab5 cross-links to these soluble factors upon exposure to UV light. Immunoprecipitation with antibodies raised against proteins known to interact with Rab5 reveals that the cross-linked complexes contain Rab escort protein and GDI-1. DATFP-Rab5 also associates with membranes in a guanosine-5'-O-(3-thiotriphosphate)-stimulated manner. However, although prenylated Rab5 can be cross-linked to two unknown membrane-associated factors by the chemical cross-linker disuccinimidyl suberate, these proteins fail to be UV cross-linked to membrane-bound DATFP-Rab5. These results strongly suggest that membrane-associated factors bind Rab5 through protein-protein interactions rather than protein-prenyl interactions. The modification of Rab5 with DATFP-FPP establishes a novel photoaffinity technique for the characterization of prenyl-binding sites.

Paraneoplastic pemphigus: a review of the literature.

Paraneoplastic pemphigus is a relatively rare but highly significant acquired mucocutaneous disorder. The condition typically presents in patients with previously diagnosed lymphoreticular disease, primarily malignancies (non-Hodgkin's lymphoma; chronic lymphocytic leukemia). Multiple sites of involvement are common, with the skin affected by a polymorphous eruption that includes lichenoid plaques as well as bullae. The oral lesions occur in all cases and present as painful widespread shallow ulcers with hemorrhagic crusting of the lips. Conjunctival involvement is also seen and may result in scarring. Pulmonary lesions are an ominous sign. Histopathologically, intraepithelial and subepithelial clefting associated with interface dermatitis are seen. A variety of autoantibodies directed against desmoplakins and desmogleins can be identified with immunoprecipitation studies. Treatment with immunosuppressive agents may result in some resolution of the disease, but the prognosis for paraneoplastic pemphigus is considered to be poor.

Angiosarcoma of the mandible: a case report and review of the literature.

Angiosarcoma is a rare malignancy that is characterized by endothelial cell differentiation. In the head and neck area, most of these lesions arise in the scalps of elderly individuals. Less commonly, angiosarcomas can be found within bone. The purpose of this report is to describe an example of angiosarcoma involving the floor of the mouth and right body of the mandible. The histopathologic and immunopathologic features of these lesions are also reviewed.

[Hybrid 3D visualization and virtual endoscopy in cochlear implants]. / Hybride 3D-Visualisierung und virtuelle Endoskopie von Cochlea-Implantaten.

PURPOSE: The aim of this study was to demonstrate on a complex anatomical structure the possibilities and the advantages of a superimposition of a color-coded surface and volume rendering (hybrid rendering) method with the possibility of the performance of a virtual endoscopy. MATERIAL AND METHOD: In 6 patients with cochlear implants a high-resolution spiral computed tomography of the petrous bone was performed. The cochlear implants, the middle and inner ear structures were visualized using a color-coded surface rendering method, either shaded or as a grid. The petrous bone was visualized using a transparent volume rendering method. RESULTS: The hybrid 3D visualization uses the advantages of both the color-coded 3D surface and volume rendering method. In comparison to the axial source images, the hybrid 3D visualization thus facilitates a clearer representation and better assessment of the complex topographical relationship without loss of diagnostic information. The virtual endoscopy facilitates an intraluminal visualization and inspection of all color-coded 3D surface- and volume rendered structures. CONCLUSIONS: The hybrid rendering and virtual endoscopy make the morphological assessment of cochlear implants easier by the simultaneous visualization of the surrounding structures and thereby support the diagnostic imaging methods. This image processing method can be used pre-operatively for the individual planning, simulation, training and further development of surgical procedures and interventions and post-operatively for the control of the position and further developments of implants.

Enhanced prenyltransferase activity and Rab content in rat liver regeneration.

Rabs are small GTP-binding proteins with a regulatory role in intracellular vesicular traffic. The modulation of their levels and activity in different physiological situations is poorly understood. During the first cell cycle of rat liver regeneration we observed a differential regulation of some Rabs, with a progressive increase of those involved in exocytosis and a progressive decrease of one involved in endocytosis. This could be related with the need of exposing growth factor receptors and prolonging the transduction of their signal in preparation for mitosis. Moreover, we observed an increased activity of protein prenyltransferases, the enzymes responsible for the prenylation of several proteins involved in crucial processes of proliferation, without a corresponding increase in the amount of prenyltransferase protein.