[Case report Brown-Vialetto-Van laere syndrome]. / Un caso clinico. Sindrome di Brown-Vialetto-Van Laere.

A case of a young man (36 years old) suffering from a progressive bulbo-pontine palsy, bilateral central deafness and respiratory failure, clinically known as Brown-Vialetto-Van Laere syndrome (BVVL) is reported. The illness occurred at 24 years and, after a period of quiescence of 12 years, led to a serious dependence from mechanical ventilation. In tyhis report the most important points of view about resuscitation and intensive treatment, such as neuromuscular failure and locked-in condition, are discussed.

Evaluating the effects of functional communication training in the presence and absence of establishing operations.

We conducted functional analyses of aberrant behavior with 4 children with developmental disabilities. We then implemented functional communication training (FCT) by using different mands across two contexts, one in which the establishing operation (EO) that was relevant to the function of aberrant behavior was present and one in which the EO that was relevant to the function of aberrant behavior was absent. The mand used in the EO-present context served the same function as aberrant behavior, and the mand used in the EO-absent context served a different function than the one identified via the functional analysis. In addition, a free-play (control) condition was conducted for all children. Increases in relevant manding were observed in the EO-present context for 3 of the 4 participants. Decreases in aberrant behavior were achieved by the end of the treatment analysis for all 4 participants. Irrelevant mands were rarely observed in the EO-absent context for 3 of the 4 participants. Evaluating the effectiveness of FCT across different contexts allowed a further analysis of manding when the establishing operations were present or absent. The contributions of this study to the understanding of functional equivalence are also discussed.

[Second surgery in the management of ovarian cancer]. / La seconda chirurgia nel trattamento del cancro ovarico.

The aim of this study was to evaluate the value of second surgery in the combined and multidisciplinary treatment of mullerian ovarian cancer. A retrospective study was carried out in 86 consecutive patients with advances stages of mullerian ovarian cancer (stage 3-4) referred to the Division of Gynecological Surgical Oncology of the Oncological Reference Centre at Aviano for continuation of treatment following initial surgery and chemotherapy performed in various peripheral institutions. Second surgery only revealed 11.6% of complete endocelomatic pathological responses (10 patients) to earlier treatments; among the 76 patients with persistent disease it was possible to achieve optimal redebulking in 50 (65.8%) (31 R0 and 19 R1 after second surgery), whereas it was not possible to perform adequate second surgery in 26 (34.2%). The impact of second surgery on the probability of survival (mean survival rate) was highly significant in the 50 patients in whom it was possible to perform adequate second surgery compared to the non-operated group (14.34 months versus 6.10, chi square = 12.671, p = 0.0004). The authors underline both the prognostic value of lymph node status with increased mortality among patients with positive retro-peritoneal lymph nodes (LN+), and the value of retroperitoneal re-evaluation in predicting endo-peritoneal recidivation in patients with free abdomen who subsequently relapsed.

Use of descriptive and experimental analyses to identify the functional properties of aberrant behavior in school settings.

We conducted descriptive and experimental analyses of aberrant behavior in school settings with 2 children with autism, using teachers as assessors. Experimental functional analyses carried out by the investigators were followed by training teachers to conduct a descriptive analysis and a classroom experimental analysis. A comparison of the assessment procedures showed that each procedure identified negative reinforcement as a maintaining variable for aberrant behavior. The teacher implemented an intervention based on the assessment with mixed results. We then replicated the initial results by having the first teacher train a second teacher to carry out the two assessment procedures. The results of these analyses were also in agreement, again identifying negative reinforcement as a variable maintaining aberrant behavior. An intervention based on negative reinforcement was then successfully implemented. These results suggest the applicability and utility of functional analyses carried out in school settings.

Adjuvant hormone treatment and chemotherapy in postmenopausal women with operable breast cancer: a retrospective analysis.

Two hundred consecutive postmenopausal women with operable breast cancer and metastatic axillary nodes were treated during the period January - December 1981 with adjuvant chemotherapy (CMF) or hormonal treatment (tamoxifen). The distribution of receptor status (estrogen or progesterone), number of axillary metastatic nodes (less than = 3 or greater than 3), surgical treatment and size of the primary tumor were homogeneous in both groups. Receptor status and number of axillary lymph nodes were correlated with adjuvant treatment efficacy. Ten-year disease-free survival (DFS) was higher in the TAM-treated (72%) than in the CMF-treated group (52%) (p less than 0.01). In patients with less than = 3 axillary metastatic nodes, those treated with TAM had a higher DFS rate than those treated with CMF (75% vs 59%, p less than 0.01). There was no difference in DFS between CMF-and TAM-treated groups within the greater than 3 metastatic lymph node patients. In ER + primary tumors, DFS was higher in the subset treated with TAM (62%) than with CMF (51%) (p less than 0.05), whereas no difference in DFS was observed in ER- patients between the two treatment groups. Considering the TAM group, DFS was better (p less than 0.01) for ER+ cases than for ER- cases only at 5 years of observation. In the CMF group, DFS was not influenced by ER status. PgR content did not affect DFS in either adjuvant treatment group.

Using parents as therapists to evaluate appropriate behavior of their children: application to a tertiary diagnostic clinic.

We conducted a preliminary analysis of maintaining variables for children with conduct disorders in an outpatient clinic. Eight children of normal intelligence between the ages of 4 and 9 years were evaluated during 90-min sessions. The children's parents conducted the assessments by varying task demands (easy and difficult) and parental attention (attention and no attention) within a multielement design. The assessment focused on appropriate child behavior and was conducted to formulate hypotheses regarding maintaining contingencies. Results demonstrated that the children's appropriate behavior varied across assessment conditions and, for 7 of the 8 children, occurred at a higher rate during one condition than during other conditions. In addition, treatment integrity data demonstrated that parents were able to implement the procedures as intended. The recommended treatments were rated as being both effective and acceptable to parents for up to 6 months following the evaluation. Our results extend previous studies of functional analytic procedures conducted by trained experimenters with severely handicapped children in more controlled settings.

Use of negative reinforcement in the treatment of self-injurious behavior.

Behavioral assessment procedures were used to determine the maintaining conditions of self-injury exhibited by 2 children with severe multiple handicaps. For both children, negative reinforcement (escape from grooming activities) was determined to be the maintaining reinforcer for self-injury (hand/arm biting) within an alternating treatments design. The treatment packages involved the use of negative reinforcement (brief escape from grooming activities) contingent upon a behavior that was incompatible with self-injury (reaching and pressing a microswitch that activated a pre-recorded message of "stop"). Treatment was evaluated with a reversal design for 1 child and with a multiple baseline across grooming activities for the 2nd child. The treatment led to a marked decrease in self-injury for both children. At follow-up, high rates of self-injury were reported for the 1st child, but low rates of self-injury and an increase in task-related appropriate behavior were observed for the 2nd child.

Unprogrammed effects of training high-status peers to interact with severely handicapped children.

We examined the effects of a peer initiation intervention with high- and low-status nonhandicapped students on the behavior of untrained peers toward handicapped students. In the context of a counterbalanced withdrawal design, high- and low-status nonhandicapped students were taught to direct social initiations to eight severely handicapped students during recess activities. The interactions of the high-status students resulted in higher levels of initiations by untrained peers toward the handicapped students than did the interactions of the low-status students. Social response levels were also differentially affected by the status of the peer initiator.

Correlation between estrogen receptor levels and clinical response to ovariectomy in premenopausal women with advanced breast cancer.

From 1974 to 1980, according to a controlled clinical trial, 66 premenopausal women with advanced breast cancer underwent bilateral ovariectomy (BO). Of the hormonal parameters evaluated, the present study deals with estrogen receptors (ER). The relation between the presence or the absence of ER and clinical response was evaluated, without considering their concentration in neoplastic tissue. The response was evaluated in relation to threshold values of 5 fmol/mg protein (considered at the time of the study the cut-off value of negativity and positivity) and 10 fmol/mg protein (recently found to be a more accurate cut-off value). The results of BO in relation to different receptor status showed an analogous response. Furthermore, the higher the receptor concentration, the better the response to BO, especially when the concentration was higher than 25 fmol/mg protein.