We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7-37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%).

No abstract

BackgroundHistological grading typically reflects the biological behavior of solid tumors, thus providing valuable prognostic information. This is also expected in hepatocellular carcinoma (HCC), although limited access to biopsy samples and a lack of standardization might hinder its full predictive value in this cancer.ObjectivesIn order to better understand the current practices of histological grading in HCC, we examined the latest publications addressing its impact on the outcome of patients following surgical treatment.MethodsWe searched the PubMed (MEDLINE) database under the headings “hepatocellular carcinoma,” “grade OR grading,” and “prognosis.” Qualitative and quantitative assessment of publications was performed according to the reference they used to grade their tumors (e.g., Edmondson–Steiner, World Health Organization).ResultsWe reviewed a total of 216 articles: 114 enclosed adequate information and were included herein. Among these, we found divergences and inaccuracies in the histological grade assessment of this cancer, which might have led to a non-standardized grade distribution, with further impact on data analysis. Nevertheless, in most of them, poor tumor differentiation correlated with worse prognosis, expressed by lower overall and/or disease-free survival.ConclusionWhile histological grading of HCC has an important prognostic role, there is an unsatisfactory heterogeneity on the microscopic assessment of this tumor, urging for a movement toward standardization.

PURPOSE: To determine the reasons for choosing the medical profession by interviewing freshmen medical students from the Faculty of Medicine, University of São Paulo and investigating their socioeconomic and psychological profiles, as well as to determine whether there are gender differences. METHOD: One hundred and sixty three freshmen medical students answered a questionnaire regarding their socioeconomical profile. Of those, 30 female and 30 male students underwent a face-to-face interview regarding the career choice, the Thematic Apperception Test (TAT) and the 16 Personality Factor Questionnaire (16 PF). The results were analyzed qualitatively and by Mann-Whitney, χ 2 , Fisher's Exact, and r p coefficient tests. RESULTS: Most students were middle class, catholic, and had physicians in their families. Students of both genders had made an: early choice of the medical career and a persistence in taking exams many times, even after being discouraged. They also showed an awareness of the difficulties and limitations in developing their careers. The study revealed a strong valuation of the humanistic aspects of medicine; openness to new experiences; a deep personal identification with the choice of profession; a critical need for fulfillment in their careers; and conscious and unconscious desires to help people and be recognized for their usefulness, without being narcissistic. Female students were more sensitive (P <.001) and less imaginative (P = .005) than male students, who were more utilitarian and less grounded; female students tended to present greater emotional maturity while male students presented a greater tendency towards competition, and were more ambitious. CONCLUSIONS: Students of both genders have similar socio-economical profiles and features regarding their motivations for choosing the medical profession. Slight differences were found regarding some psychological aspects.