STIFF PERSON SYNDROME (SPS) THE IMPORTANCE OF EXTENSIVE PHYSICAL EXAMINATION

Stiff Person Syndrome (SPS) is a rare condition with a prevalence of one to two cases per million and an incidence of one case per year. Characterized by progressive muscle stiffening, rigidity and spasm, mainly involve the axial muscles, resulting in severely impaired ambulation. The pathogenesis is not that clear. Classic SPS presents with extreme and persistent rigidity and stiffness of truncal and proximal limb muscles, significant lumber/cervical lordosis resulting from simultaneous actions of opposing paraspinous muscles are the hallmark. Wide and unsteady gait (Frankenstein’s gait) resulting from stiff muscles increases the risk of fall and fracture. Patient’s ADLs get severely restricted progressively. Startle reflex which is a superimposed episodic muscle spasm precipitated by sudden movement, noise, or emotional upset is a sensitive and specific feature of SPS and can be visible and palpable. Autonomic dysfunctions are common and are common cause of death in patients with SPS. In cases of partial SPS, we can find that truncal muscles are spared and limb muscles are affected, hence the term stiff-limb syndrome is used sometimes. Can involve a limb or focal part of a limb. As for treatment, symptom control and improving mobility and overall functionality is the primary target. High dose benzodiazepine is the best initial treatment. Those who are refractory can be benefit from Baclofen, IVIG, Plasma exchange, biological agent, e.g. Rituximab. Treatment of primary malignancies in paraneoplastic SPS can achieve remission. Keywords: Stiff Person Syndrome, clinical features, diagnosis and treatment.

DESIGN INVESTIGATION ON FOOT DEFORMITIES WORKING FOOTWEAR

Footwear was planned to protect the feet from the environment. Choosing the right footwear based on foot conditions and requirements enabling to reduce the risk of injury, fast fatigue, and foot pains. Good footwear should provide stability to the joints of the foot and a foundation upon which the foot can function. The objective of this study is to provide information that will assist researchers and designers in identifying, designing and offering appropriate and better working footwear designs for those with foot deformities. Two identified workers from the footwear factory with congenital foot deformities participated in this prospective intervention study for over 12 months. An interview and a video on how the participant wears and walk with normal commercial shoes were recorded. The physical examination was also assessed including the footwear, feet, legs, and hips of the subjects. They were examined with a focus on the areas of deformities. Each leg has different shape and concerns. Hence, each footwear designed for those with foot deformities must be customized base on the factors of type of deformities, body size and the remaining skeletal structure and muscular. With proper modification, people who have foot deformities and unable to buy suitable footwear will be able to relieve the pain and at the same time increase the individual motivation in job satisfaction, motivation or enthusiasm to work and reducing levels of stress or emotional upset.

A Visual-Analogue Screening Tool for Assessing Mood and Quality of Daily Life Complications in Family Members of People Living With Cancer: Portuguese Version of the Emotion Thermometers: Burden Version

Cancer is a disease that impacts not only the patient but also affects the entire family. Family members experience high levels of distress. Therefore, screening for cancer-specific distress among family members of people with cancer is important but relatively unexplored. This cross-sectional study aims to analyze the psychometric properties of a screening tool for family members of people with cancer. We examined the usefulness of the emotional thermometers burden version (ET-BV) in detecting caregiver emotional distress. The ET-BV is a simple multidomain visual analogue scale distributed in two major domains: “emotional upset” and “impact.” A total of 364 cancer patients’ family members completed the ET-BV and Brief Symptom Inventory. Analyses were aimed to examine the diagnostic accuracy (receiver operating characteristic) of the ET-BV. A fair to good diagnostic accuracy was achieved for ET-BV. For emotional upset thermometers, a cutoff of ≥5 was determined and for impact thermometers, a cutoff of ≥4 was established. ET-BV seems to be a useful, quick, and simple tool for distress screening in family members of people with cancer. A revision of a specific thermometer is discussed in order to increase ET screening performance and clinical utility.

Study of urinary incontinence affecting the quality of life and health seeking behavior in women of an urban slum in Mumbai

Background: Urinary incontinence (UI) defined by the International Continence Society as the complaint of any involuntary leakage of urine, is a social or hygiene problem. The purpose of this study was to determine how UI affected quality of life (QOL) of women in urban slum in Mumbai and also their health care seeking behavior for the same.Methods: A pre-formed, pre-tested, semi-structured questionnaire was designed for a centre based study, to collect information from 1200 eligible and willing women over a period of 6 months. Of these 361 had complaints of UI how had the UI affected their quality of life and whether they sought health care for treatment of same was studied. Data was entered in Microsoft Excel and analysis was done accordingly.Results: Of the 361 women who had UI, the QOL was affected as in avoiding social interactions 45.15%, avoiding religious functions 49.03%, feeling of emotional upset/distress 59.8% and hampering of domestic work 57.8%. The extra financial burden felt due to UI was only in 7.7% women and only 1.9% complained of sexual life getting affected. Health care services for treatment of UI were sought by only 24.1% of the affected women.Conclusions: That UI affected the QOL of women affected with it in terms of reduced social interactions and attendance at religious programs and the feeling of emotional upset and hampering of domestic works. Subjects felt their financial burden had increased due to UI.

Familicide in Italy: An Exploratory Study of Cases Involving Male Perpetrators (1992-2015)

This article adds to the literature on familicide by providing specific insights from the Italian experience. It presents results of an exploratory investigation into male-perpetrated familicides in Italy between 1992 and 2015. Familicide is defined as the killing of the spouse or intimate partner, and at least one child, at the hand of the other spouse/partner. Incidents of familicide were collated from newspaper reports. We identified 90 cases, resulting in 207 deaths. On average, perpetrators were middle aged ( M = 46.8; age ranging from 25 to 76), and most ( n = 66; 73%) committed, or attempted to commit, suicide. While significant contributory roles were played by health problems and financial worries, the origin of the primary emotional upset for the killers tended to be interpersonal conflicts involving their partners. For those cases with available information ( n = 56; 62%), six types of familicide were also identified on the basis of the murderer’s homicidal motivations. Most frequent were three circumstances. Fifteen cases concerned the “doubly-protective familicide” (which corresponds to the “suicide-by-proxy”), characterized by the preservation of the family in the face of a presumed catastrophic event. Triggers included the killer’s financial distress, health troubles, or anxiety associated with other personal problems. Thirteen cases referred to “doubly-punitive familicide,” whose distinctive feature, in addition to punishing the partner because of her estrangement, her infidelity, or other disputes, is to directly involve the child(ren) in the punitive homicidal act. The children are viewed as contributory factors to the killer’s stress, or are considered to be in league with the mother. Twelve cases exemplified “indirectly-punitive familicide” (also termed “murder-by-proxy”), in which the victimized child(ren) are killed as an extension of the partner. Overall, this typology provides a more nuanced conceptualization of familicide occurrences than those examined in previous research.