Inter-rater reliability of the International Cooperative Ataxia Rating Scale (ICARS).

We assessed the inter-rater reliability of the 100-point International Cooperative Ataxia Rating Scale (ICARS). Three neurologists independently rated videotaped ICARS examinations of 22 subjects with genetically determined ataxias (spinocerebellar ataxia [SCA] Type 1 in 11; SCA Type 2 in 1; Friedreich's ataxia in 10) and 4 controls. Scores on live ICARS assessment had ranged from 0 to 7 for controls and 11 to 74 for ataxic subjects (clinically very mildly affected to wheelchair-bound). Inter-rater correlation was very high for the total score (Kendall's omega 0.994, 95% confidence interval, 0.988-0.997), and high to very high for each component subscore (0.791 for speech to 0.994 for posture/gait). All correlations were significant at P < 0.00001. The ICARS exhibits very high inter-rater reliability even without prior observer standardisation and is sensitive to a range of ataxia severities from very mild to severe.     

Sarcoid-like lymphadenopathy mimicking metastatic testicular cancer

A case of nonseminomatous testicular cancer and enlarged mediastinal lymph nodes, which were interpreted as metastases, is reported. When there was no change after two courses of chemotherapy, a mediastinoscopy was performed and the results showed sarcoidosis.     

Cloning and expression analysis of the chick DAN gene, an antagonist of the BMP family of growth factors.

Differential screening-selected gene aberrative in neuroblastoma (DAN) is a member of a cystine knot protein family that includes Cerberus and Gremlin. First isolated in a screen to identify genes down-regulated in transformed rat fibroblasts, DAN has subsequently been cloned in Xenopus, mouse, and human. Overexpression of DAN suppresses the transformed phenotype and retards the cell's entry into S phase. Biochemical analyses have demonstrated DAN's ability to bind bone morphogenetic proteins and antagonize their signaling activity. In this study, chick DAN was cloned and sequenced, revealing a conserved cystine knot region as well as an N-glycosylation site. A riboprobe was designed from the 3' chick DAN coding sequence and used for analysis of DAN in the developing chick embryo by in situ hybridization. Chick DAN was expressed beginning at stage 10 in the developing somites and the medial otic epithelium. Expression in the neural layer of the eye became apparent at stage 14. By stage 17, expression had expanded to the base of the hindbrain. Limb bud labeling began at stage 20, whereas expression in the branchial arches appeared at stage 25. Chick DAN expression generally corresponded to that of mouse DAN expression as shown by comparative in situ hybridization. However, chick DAN was found in the otic epithelium and notochord, whereas mouse DAN was restricted to the overlying otic ectomesenchyme and was absent from the notochord. This observation suggests that DAN may play different roles in chick and mouse otic and notochord development.     

Is endometrial infection with Candida albicans a cause of recurrent vaginal thrush?

OBJECTIVE--It was hypothesised that the endometrium might act as a reservoir for candida, thus infecting the vagina as the endometrium is shed during menstruation. DESIGN--A prospective study of women with recurrent vulvo-vaginal candidiasis. The endometrium was sampled and cultured for candida species. SETTING--Central London STD clinic. SUBJECTS--26 women were enrolled, of whom 20 completed the study. RESULTS--One patient had a positive endometrial culture for candida species, the isolate being Candida krusei. CONCLUSIONS--The endometrium is not a common resevoir for candida species and therefore, infection at this site is an unlikely cause of recurrent vaginal candidiasis.     

Problems with a ‘target’ approach to access in primary care: a qualitative study

We report an analysis of the qualitative phase of a study of patients' and carers' views of primary care services, focusing on their experiences of access to face-to-face general practitioner (GP) consultations during the period when new access policies were being implemented. Practices interpreted the new policy in various ways; restricted interpretations, including restriction of access to telephone booking, could cause distress to patients. Patients and carers welcomed flexible interpretations of the policy that offered choice, such as a choice of GP, or of booking in advance.     

A second look at second-order belief attribution in autism

Compared the performance of autistic and mentally retarded subjects, all of whom had passed a standard first-order test of false belief, on a new second-order belief task 12 autistic and 12 mentally retarded subjects, matched on verbal mental age (assessed by PPVT and a sentence comprehension subtest of the CELF) and full-scale IQ were given two trials of a second-order reasoning task which was significantly shorter and less complex than the standard task used in all previous research. The majority of subjects in both groups passed the new task, and were able to give appropriate justifications to their responses. No group differences were found in performance on the control or test questions. Findings are interpreted as evidence for the role of information processing factors rather than conceptual factors in performance on higher order theory of mind tasks.This study was supported by a grant from the National Institute of Deafness and Other Communication Disorders (1RO1 DC 01234). We thank Jason Barker for his extensive help with this study. We are also very grateful to the schools where the study was conducted including the League School, and the public school systems in the following towns in Massachusetts: Hanover, Hanson, Hingham, Milton, Plymouth, and Rockland. We offer special thanks to Alan Dewey, Mary Dollar, Sandy D'Giacomo, Herman Fishbein, William Griffin, Nancy Kearns, Judy Monahan, Debbie Newhall, Cay Riley, Robert Sherman, and Kathy Staska for their continued support of our research.