Congenital prosopagnosia in a child: Neuropsychological assessment, eye movement recordings and training.

Here we report the assessment and treatment of a 6-year-old boy (L.G.) who was referred to us for congenital prosopagnosia (CP). We investigated his performance using a test battery and eye movement recordings pre- and post-training. L.G. showed deficits in recognising relatives and learning new faces, and misrecognition of unfamiliar people. Eye movement recordings showed that L.G. focused on the lower part of stimuli in naming tasks based on familiar or unfamiliar incomplete or complete faces. The training focused on improving his ability to explore internal features of faces, to discriminate specific facial features of familiar and unfamiliar faces, and to provide his family with strategies to use in the future. At the end of the training programme L.G. no longer failed to recognise close and distant relatives and classmates and did not falsely recognise unknown people.

Peculiar body representation alterations in hemineglect: a case report.

We report the case of FP affected by personal and extrapersonal neglect and a body representation deficit characterized by delusional ideas. When FP performed the human figure, he placed body parts to the left, despite his extrapersonal neglect. Differently, when he performed the car figure, he placed all parts to the right, in line with his deficit. Comparing FP with a small patient group with the same clinical features without delusional ideas about body emerged that he was the only one to suffer from a specific body representation deficit characterized by a lack of body ownership sense.

Long-term cyclic intravenous iloprost in systemic sclerosis: clinical experience from a single center.

The aim of the present study was to retrospectively evaluate response to therapy in 73 patients affected by systemic sclerosis (SSc) who underwent long-term cyclic treatment with intravenous iloprost for peripheral vascular involvement (average duration of treatment 54.12±41.04 months). Seventy-three SSc patients were enrolled. Data were collected by reviewing clinical records and by phone or direct interview. Patients underwent a thorough physical examination at the end of follow up. The incidence of severe vascular manifestations was also assessed. Statistical analysis was performed by Wilcoxon's signed rank test and descriptive statistics using Statview software. In this study cohort, 55 of 73 (75.2%) patients had a history of ischemic digital ulcers (DUs); 28 patients (38.4%) had active DUs at the beginning of treatment. Skin ulcers healed completely in 25 of 28 patients (89.3%) at the end of the first treatment. However, 40 of 55 patients (72.6%) relapsed after an average of 24 months. There was a significant correlation between relapse rate and/or number of ulcers and clinical factors (diffuse subset, changes in results of Allen's test, NT-pro BNP levels). The annual incidence of pulmonary arterial hypertension (PAH) was 2.34 (95%CI: 0.94-4.83) per 100 person years, the rate of gangrene was 2.7%, and no cases of scleroderma renal crisis were recorded. The incidence of PAH and of digital gangrene was higher than that observed in unselected SSc case series. These data suggest that our patients treated with iloprost have a higher vascular involvement than large case series of unselected SSc patients. A number of clinical factors are correlated to the severity of vascular involvement and could have an impact on the response to therapy. The clinical significance of these findings requires clarification and further investigation is needed.

Evidence of taxonomy for Developmental Topographical Disorientation: Developmental Landmark Agnosia Case 1.

We report Developmental Landmark Agnosia (DLA) in a 6-year-old boy (L.G.) who was referred to us for congenital prosopagnosia (see Pizzamiglio et al., 2017 , in which both testing and rehabilitation of Congenital Prosopagnosia are reported). We investigated his performance using a neuropsychological battery and eye movement recordings. The assessment showed the presence of deficits in recognizing familiar places (along with Congenital Prosopagnosia), but not common objects. Eye movement recordings confirmed his problems in recognizing familiar landmarks and misrecognition of unfamiliar places. L.G. is the first evidence of a DLA, suggesting identification of taxonomy of navigational disorders in Developmental Topographical Disorientation is possible, as in the Acquired Topographical Disorientation.

A selective egocentric topographical working memory deficit in the early stages of Alzheimer's disease: a preliminary study.

The aim of this study was to determine whether an egocentric topographical working memory (WM) deficit is present in the early stages of Alzheimer's disease (AD) with respect to other forms of visuospatial WM. Further, we would investigate whether this deficit could be present in patients having AD without topographical disorientation (TD) signs in everyday life assessed through an informal interview to caregivers. Seven patients with AD and 20 healthy participants performed the Walking Corsi Test and the Corsi Block-Tapping Test. The former test requires memorizing a sequence of places by following a path and the latter is a well-known visuospatial memory task. Patients with AD also performed a verbal WM test to exclude the presence of general WM impairments. Preliminary results suggest that egocentric topographical WM is selectively impaired, with respect to visuospatial and verbal WM, even without TD suggesting an important role of this memory in the early stages of AD.

Impact of participation on rehabilitation results: a multivariate study.

BACKGROUND: Participation in inpatients is commonly considered as a relevant factor influencing rehabilitation results, but its effects are still not exhaustively investigated. AIM: To clarify and quantify the impact of level of participation in rehabilitation on functional outcome in inpatients. DESIGN: Prospective, observational multivariate study. SETTINGS: Rehabilitation hospital. POPULATION: Three hundred and sixty-two patients (mean age 59.41±12.85 years) with stroke or orthopedic diseases consecutively admitted to rehabilitation hospital. METHODS: Rehabilitation program participation was assessed by means of Pittsburgh Rehabilitation Participation Scale (PRPS). Patients who scored below 4 in 25% of the physical and occupational therapy treatment were classified as "low" participants. Multiple and logistic regressions were performed to identify variables associated not only with participation but also with rehabilitation results. RESULTS: Nearly one third of patients (33.88%, primarily stroke) showed low participation. Low early participation (within the first two weeks) was associated with disability and depressive symptoms at admission, and late participation with early participation, age and years of schooling. Both early and late participation were associated with effectiveness of treatment on both ADL and mobility, even if there was much unexplained variance in both models. Patients with low early participation had a greater risk (OR=2.45, 95% CI 1.27-4.71) of a low response to treatment on mobility than the patients who had participated more. Among other prognostic factors, early start of rehabilitation treatment and the presence of cognitive and neuropsychological impairments have significant roles. CONCLUSIONS: Our results confirm the importance of participation in rehabilitation programs, which should be encouraged. Further studies are needed to improve knowledge about the overall effects of participation. CLINICAL REHABILITATION IMPACT: Early participation should be considered a treatment target as well as a prognostic factor.

Maximum or minimum entropy production? How to select a necessary criterion of stability for a dissipative fluid or plasma.

Ten necessary criteria for stability of various dissipative fluids and plasmas are derived from the first and the second principle of thermodynamics applied to a generic small mass element of the system, under the assumption that local thermodynamic equilibrium holds everywhere at all times. We investigate the stability of steady states of a mixture of different chemical species at the same temperature against volume-preserving perturbations. We neglect both electric and magnetic polarization, and assume negligible net mass sources and particle diffusion. We assume that both conduction- and radiation-induced heat losses increase with increasing temperature. We invoke no Onsager symmetry, no detailed model of heat transport and production, no "Extended Thermodynamics," no "Maxent" method, and no "new" universal criterion of stability for steady states of systems with dissipation. Each criterion takes the form of--or is a consequence of--a variational principle. We retrieve maximization of entropy for isolated systems at thermodynamic equilibrium, as expected. If the boundary conditions keep the relaxed state far from thermodynamic equilibrium, the stability criterion we retrieve depends also on the detailed balance of momentum of a small mass element. This balance may include the nablap-related force, the Lorenz force of electromagnetism and the forces which are gradients of potentials. In order to be stable, the solution of the steady-state equations of motion for a given problem should satisfy the relevant stability criterion. Retrieved criteria include (among others) Taylor's minimization of magnetic energy with the constraint of given magnetic helicity in relaxed, turbulent plasmas, Rayleigh's criterion of stability in thermoacoustics, Paltridge 's maximum entropy production principle for Earth's atmosphere, Chandrasekhar' minimization of the adverse temperature gradient in Bénard's convective cells, and Malkus' maximization of viscous power with the constraint of given mean velocity for turbulent shear flow in channels. It turns out that characterization of systems far from equilibrium, e.g., by maximum entropy production is not a general property but--just like minimum entropy production--is reserved to special systems. A taxonomy of stability criteria is derived, which clarifies what is to be minimized, what is to be maximized and with which constraint for each problem.