Sign Of Weakness Research Articles

Costly Conversations in South Asia: 1962 and Beyond

Costly Conversations in South Asia:1962 and Beyond John H. Gill (bio) The "costly conversations" model that Oriana Skylar Mastro develops in her new book The Costs of Conversation: Obstacles to Peace Talks in Wartime provides a useful new approach for interpreting the 1962 Sino-Indian War. To assess the applicability of this model, Mastro examines the behaviors of the Chinese and Indian governments during this brief but consequential conflict. As a limited interstate war fought in Asia during the first two decades of the Cold War, this conflict makes a good match for the chapters she devotes to China in the Korean War and North Vietnam during the Vietnam War and affords an opportunity to investigate the actions and policies of both belligerents. This essay will review these China and India studies and suggest another South Asian situation that might benefit from analysis through the same theoretical lens. Mastro defines China's diplomatic stance in 1962 as "open" both prior to and during the war. That is, Beijing expressed a desire for direct communications with New Delhi without imposing any preconditions. Indeed, it made such offers publicly and repeatedly. New Delhi, on the other hand, insisted throughout the war that it would only engage in talks after Chinese forces had withdrawn to prewar positions. Its diplomatic stance was, therefore, "closed" in terms of the costly conversations model. Mastro then proceeds to apply her approach to explicate why the two sides made these strategic choices by assessing the respective leaderships' views of the "strategic costs of conversation" through the prism of two factors: (1) "the likelihood that the enemy will interpret an open diplomatic posture as a sign of weakness" (p. 14) and (2) "how the enemy may change its strategy in response to such an interpretation" (p. 7). She posits that a state will only turn to an open posture if it thinks that its own strength and resiliency have been demonstrated and concludes that the enemy lacks the "strategic capacity" to prolong or escalate the war. She looks first at China (chap. 3) and then at India (chap. 4), a division that could cause some confusion of chronology for readers not familiar with the 1962 war. However, this [End Page 180] structure facilitates the focus on each in sequence and is thus suited to the study's aim of examining each state's diplomatic stance during the conflict. The 1962 Sino-Indian War resulted from the confluence of multiple developments in the post–World War II era. In part, it was caused by a dispute over the possession of territory encompassing more than 130,000 square kilometers along the 3,500-kilometer border that divides the two countries. Though still unresolved today, this dispute was especially acute at the time because it involved two large, newly independent powers who were proud and highly sensitive to issues of sovereignty as they sought to establish themselves as global leaders in the evolving postwar, postcolonial order.1 Of particular importance for China was the status of Tibet, which was harshly incorporated into the People's Republic in 1951 and the scene of unrest and rebellion through 1959.2 India's efforts to assert its claims along the border and to contest what New Delhi saw as illegal occupation of Indian soil thus deepened Beijing's anxieties regarding foreign support for Tibetan separatism.3 These fears were accentuated in 1961 when India, pursuing Prime Minister Jawaharlal Nehru's ill-conceived "forward policy," advanced small army patrols and outposts into the mountains to counter what Indian leaders saw as Chinese encroachment. There was also an ideological element of the dispute. Mao Zedong and other Chinese leaders viewed "big bourgeoisie India" as part of the "anti-China tide" sponsored by the United States and other capitalist enemies.4 Determined to teach India an enduring lesson, China launched a series of well-planned "self-defensive counterattacks"5 on October 20, 1962, that tore apart India's thin defenses. Chinese forces halted on October 24 to create an opportunity for talks, but India rejected negotiations without a Chinese withdrawal. After waiting three weeks, Chinese forces resumed their offensive on November 16, inflicted further humiliating defeats on...

A Pre-Experimental Study to Evaluate the Effectiveness of Structured Reminiscence Psychotherapy on the Level of Depression among Elderly Residing in Old Age Home

Depression is not a sign of weakness. It can happen to anyone, at any age, no matter your background or your previous accomplishments in life. Depression is common problem in older adults. And its symptoms affect every aspect of life, including energy, appetite, sleep and interest in work, hobbies and relationships. Elderly people are highly prone to mental morbidities due to aging of the brain, problems associated with physical health, socioeconomic factors such as break down of the family support systems, decrease in economic independence. Reminiscence refers to recollection of memory from the past. Reminiscence involves exchanging memories with the old and young, friends and relatives, with care giver and professional, passing on information, wisdom and skills, therapeutic reminiscence not only enhances the cure of souls in early old age; it also help to close the gap between the depressing expectation of younger generation and the reality of continued growth in the last half of life. Elderly adults gain satisfaction, confidence and sense of identity from reminiscing. “A pre-experimental study to evaluate the effectiveness of structured reminiscence psychotherapy on the level of depression among elderly residing in old age home at selected areas of tricity.” Total 60 elderly adults of age group 60–80 years were taken by using convenient sampling technique. The pre-test was done by using standardized Beck's Depression Inventory on the level of depression, it contained 21 items. Structured reminiscence psychotherapy was administered to reduce the level of depression. The post-test was conducted by using same depression scale. Collected data was analyzed by descriptive and inferential statistics. The results showed that the level of depression was assessed with Beck's Depression Inventory with 21 items. The level of depression was classified according to severity. In pre-test 28 (46.7%) subjects were moderate level of depression, 26(43.3%) had severe level of depression, 6(10%) had mild mood disturbance and none of them had borderline clinical depression and extreme level of depression. The analysis revealed that the mean value of pre-test was 28.016 with Standard Deviation 6.593 and the mean value of post-test was 21.366 with Standard Deviation 6.436 with t-value 5.834. So there was significant difference between the mean pre-test and post-test scores at p>;0.01 level. Thus it was evident that after intervention the score of post-test was less from pre-test score. It indicates that the structured reminiscence psychotherapy was an effective psychotherapy to reduce the level of depression among elderly.

Workplace stress in senior executives: Coaching the ‘uncoachable’

PurposeWorkplace stress is becoming an acknowledged problem, and employee assistance programmes are widely invested into reducing workplace stress. However, there is still a group of people who are neglected: senior executives. There is a taboo among this group about admitting to overwhelming stress, as the executives themselves can consider it a sign of weakness. Executive coaches often pick up symptoms of underlying stress, including sleep deprivation, or substance abuse in leadership development programmes or executive coaching sessions. However, unless the coach is a trained stress expert, medical doctor, or psychotherapist, he or she may feel unequipped to help a senior executive deal with stress. This article introduces an interpretative protocol to gage senior executive stress to flag up potential stress issues and areas to be addressed in coaching or by health professionals.MethodsBased on a literature review on common workplace stress factors and symptoms, we identified five areas in which senior executive stress may arise. In a next step, following a psychodynamic approach, we developed an interpretative protocol that can be used in conversational or observational approach to gage stress in these areas.ConclusionsOur intent was to achieve a delicate balance between flexibility, face validity, and academic rigour. The protocol helps the coach to detect and interpret stress indicators despite possible self-deception and impression management by the coachee.

Stand on the Sky by Erin Bow

Reviewed by: Stand on the Sky by Erin Bow Wesley Jacques Bow, Erin Stand on the Sky. Houghton Mifflin Harcourt, 2019 [336p] Trade ed. ISBN 978-1-328-55746-9 $16.99 E-book ed. ISBN 978-1-328-63007-0 $9.99 Reviewed from galleys R Gr. 4-7 Twelve-year-old Aisulu notices her older brother Serik’s new limp, but believing that their nomadic Kazakh family wouldn’t welcome any sign of weakness, he makes her promise to keep his ailment a secret. Her breaking of that promise saves his life as a hospital visit reveals Serik has cancer, but it leaves Aisulu alone to be tended to by her university-educated uncle and his Tuvan wife. Aisulu soon takes it upon herself to retrieve a baby golden eagle left behind when her brother’s leg failed, and as she worries about her family’s future, her unique bond with the growing eagle becomes undeniable. Bow uses wistful narrative flourishes to paint this meticulously detailed portrait of a nomadic family and their profound relationship with the wildlife and unforgiving land, and the story compellingly tracks Aisulu’s gradual maturation into someone who challenges not only the gender expectations of her family and culture but also herself. As her family is finally reunited, Aisulu and her eagle must enter a competition at the Golden Eagle Festival—covered comically by ESPN producers attuned to the inherent spectacle of a girl eagle hunter—to win the money needed for Serik’s treatment and prosthesis. With her community behind her in ways she [End Page 289] could not have imagined and an eagle that has been a surrogate brother to her for some time, she’s ultimately triumphant on the big stage in a fashion young readers will find heartwarmingly earned and inspiring. Copyright © 2019 The Board of Trustees of the University of Illinois

Electrophysiology of Seizure Disorders May Hold Key to the Pathophysiology of Psychiatric Disorders

Despite the increasing burden of mental illness, social stigma and fears that psychological and emotional problems are a sign of character weakness prevent most sufferers from seeking treatment. These barriers are reinforced by diagnostic ambiguity, frequent drug side effects, variable treatment success, and a lack of clarity about the cause of mental illness. Much more progress has been made with epilepsy, a closely related group of disorders for which the pathophysiology is better understood. Although psychiatric disorders and seizure disorders are known to be distinctly different conditions, they have many shared features including their disruptive effects on mentation, their migratory nature, and their responsiveness to anticonvulsant drugs. In addition, a comparative analysis of the two disorder-types strongly suggests that they have shared mechanisms of symptom production, symptom progression, and symptom prevention. In this side-by-side comparison of the two disorder-types, I will discuss how the electrophysiological patterns that underlie seizure initiation and migration help explain how psychiatric symptoms develop and morph into one another, thus providing important insights into the pathophysiology of mental illness and potentially serving as a guide to the development of more effective treatments.

White Eagle, Black Madonna: One Thousand Years of the Polish Catholic Tradition by Robert E. Alvis

Reviewed by: White Eagle, Black Madonna: One Thousand Years of the Polish Catholic Tradition by Robert E. Alvis David Curp White Eagle, Black Madonna: One Thousand Years of the Polish Catholic Tradition. By Robert E. Alvis. (New York: Fordham University Press. 2016. Pp. xvi, 349. $125.00 clothbound. ISBN 978-0-8232-7170-2; $35.00 paperback. ISBN 978-0-8232-7171-9.) The central contention of Robert Alvis' study, that the Catholic Church in Poland holds a peculiarly central place in that country's history and culture, is inarguable. The Catholic Church has shaped the relationships between its Polish faithful and Poland's non-Catholic populations within its changing borders, as well as between Poland and its various neighbors in Europe, and even globally in the places touched by Polonia—Poland's diaspora. In turn, Poles also have enriched the Catholic faith in ways that until relatively recently—during and after St. John Paul II's pontificate—many English speakers were unaware of. Alvis' White Eagle, Black Madonna seeks to introduce and make intelligible to this audience some of the wide and deep crosscurrents that Catholicism's instantiation in Poland have generated in that country and for the world. The work is divided into ten chapters that focus on distinct eras of Polish history and the role of the Catholic Church therein, though it is heavily weighted towards the modern era. The sketches of general Polish history are generally judicious, and interweave Poland's changing geopolitics, internal political life, social development, and broader cultural changes with the story of its Catholic religious life. Several of the chapters are particularly important in demonstrating the dynamic ways in which Polish Catholics integrated their faith into their peculiar circumstances. In his discussion of the Reformation, for example (the strongest chapter in his work), Alvis notes Poland's uniqueness as a country that for over a century saw much of Polish Catholicism's lay leaders (especially among the nobility—the szlachta) and prominent clergy resist the pull of religious violence that engulfed Europe. This was due to their society's distinctive, pre-existing multi-religious pluralism as well as the degree to which, even as Catholics, Poles had previously found themselves targeted by the crusading zeal and predatory geopolitical vison of the Teutonic Knights in the high Middle Ages. Polish resistance to religious violence was both politically pragmatic—the szlachta understood that a state strong enough to enforce religious uniformity would be strong enough to subordinate them—but also principled and faithful, as Polish Catholic lay believers and many of their clergy grasped the importance of a free religious conscience. There are many ironies of Poland's Reformation. These [End Page 345] include how the papacy (and some Polish churchmen then and later) considered this refusal to use coercion a sign of religious and political weakness, as well as how this resistance played a key role in the resurgence of Catholicism in the late sixteenth century as the Church in Poland turned to teaching and evangelization instead of force to answer the challenge of Protestantism. Finally, perhaps the supreme irony of this graced historical moment is that while Polish Catholics resisted the pull of religious persecution during the Reformation, in a changed—and charged—geopolitical and cultural moment a century later, Polish Catholic lay and clerical elites enacted restrictions on religious life that their forefathers had rejected. This is an ambitious book that points to just how much needs to be done to make a vastly different experience of Catholicism accessible to a wider audience. While this reviewer wishes that the author had been able to develop at greater length the development of Poland's rich Catholic spirituality and customs, as well as the sustained work of education, charitable works, and burgeoning social movements that were a primary mode of experiencing Catholicism for many Poles, Professor Alvis has offered us a concise and rewarding survey. David Curp University of Ohio Copyright © 2019 The Catholic University of America Press

The obesity disease

Obesity in a dog is not a sign of moral weakness in either the animal or its owner – but a genuine disease requiring prompt and effective veterinary attention, delegates attending BSAVA Congress 2019 will hear.

WAJAH KERAHIMAN ALLAH DALAM PERJANJIAN LAMA

The theme is taken for this Jubilee is "Compassionate like God - Like the Merciful Father" (Lk 6:36).God is essentially show mercy, even declared himself the Almighty through His mercy. We must realize that God's mercy was not a sign of weakness, but a sign of power. Since the Old Testament, God invites each individual to reflect on his mercy, as proclaimed by the prophet Zephaniah. God has to get rid of the punishment that fell upon His people (Zephaniah 3:15). God is also present in the midst of His people (Zephaniah 3:17) expressed his compassion and solidarity. Moreover, God refurbish the people with His love (Zephaniah 3:17). God's mercy is transformed and entered into the history of mankind, in the person of Jesus Christ. He is the perfect face of God's mercy. Finally, everyone is called to show the face of the compassion of God through everyday life. God's love is so great that it revealed first of the works of creation. His love for man revealed in action by creating the universe and it is all provided for humans. Because after all there (the earth and its contents) God created man in the image of Himself.

Gestion de l’incertitude et compétence médicale : une réflexion clinique et pédagogique

Uncertainty arises when information is not sufficient to predict the prognosis or the outcome following an intervention. It is omnipresent in medical daily practice, and will follow each practitioner all along his career. The communication or merely even the feeling of uncertainty is frequently perceived as a negative experience. Nevertheless, rather than a sign of weakness, feeling uncertain reflects a dynamic state of self-reassessment which should be experienced by each doctor involved in providing his patients with the best care. Furthermore, uncertainty may lead to disastrous consequences for practitioners who have not been properly prepared to it, involving the sphere of emotions (stress, anxiety, burn-out) as well as the behavioral field (misdiagnosis, excessive testing, impaired communication…). A growing number of observations, clinical or educational studies, supports the idea that learning uncertainty should be a critical prerequisite to a valuable medical practice. Through this literature review, we propose herein a conceptual glance on uncertainty. Then, we expose some sources of uncertainty in daily practice and teaching, its consequences, and the main factors that contribute to the wide variety of the individual relationship with uncertainty. We finally aim to prompt a global reflexion, falling within an uncertainty competency-based education approach, assuming that our obligation to professionalize the students necessitates making them skilled in dealing with uncertainty.

Left ventricular dysfunction in hypertensive patients: an echocardiographic assessment

Introduction: Echocardiography is noninvasive procedure to assess heart. Ventricular mass increases due to left ventricular hypertrophy and performance of left ventricle decreases with increase in blood pressure. Left ven­tricular diastolic dysfunction (LVDD) is early sign of heart weakness which can be picked up early with Echo. This study was aimed to study cardiac anatomic and functional parametric alteration in echocardiography among hypertensive patients. 
 Methods: This was a non-invasive, cross sectional hospital based retrospective review of the patient record from echo room among the hypertensive patient who undergone echocardiographic as­sessment from September 2017-February 2018 in Shree Birendra Hospital (SBH), Chhauni, Kathmandu. 
 Results: Among 447 hypertensive cases, 232(51.9%) were having normal diastolic function while rest 215(48.1%) were having diastolic dysfunction of the heart. Among total cases evaluated, only 12(2.7%) patients were having ejec­tion fraction lower than 55%, while in rest it was normal. There were 53 (11.9%) cases having some form of as­sociated complication. Gender, Concentric Left Ventricular Hypertrophy and left atrial dilatation has significant difference (p<0.05) with LVDD. Statistically significant differences in age distribution of individuals with different categories of left ventricular systolic dysfunction (LVSD) and LVDD (p˂0.001) was observed. 
 Conclusion: Signifi­cant number of hypertensive individuals were having LVDD though in most of the individual LV ejection fraction was maintained.

Hyoid bone position related to gender and aging using lateral cephalometric radiographs

sPurposeThe purpose of this study was to assess hyoid bone position related to gender and aging using lateral cephalometric radiographs in 22 to 84 years old patient.Materials and methodsLateral cephalometric radiographs of 459 patients were reviewed retrospectively. The position of the hyoid bone was assessed in terms of horizontal position, rotation, and vertical position. Data were compared between age groups for both genders. Two-factor analysis of variance and Tukey’s honest significant difference (Tukey’s HSD) test were performed.ResultsMean vertical distance in the 22–29-year age group and the 60–84-year age group was 0.2 mm and 9.7 mm, respectively, among males, and −4.3 mm and −0.3 mm, respectively, among females. There was a trend toward larger measurements in the vertical position in older males (P < 0.001). Among males, all measurements tended to increase significantly with age (P < 0.001). In the over 60-year age group, the posterior position and rotation of the hyoid bone were almost the same for males and females.ConclusionAge-related changes in hyoid bone position may decrease swallowing safety. Oral frailty, an early sign of orofacial muscle weakness and functional deterioration, negatively influences dental treatment and swallowing. The hyoid bone shifted to a posterior, rotated, and lower position with increasing age. Especially among males in the over 60-year age group, the hyoid bone had shifted significantly to a lower position. We should focus into the hyoid bone position in lateral cephalometric radiograph.

2047 Mental illness public stigma, culture, and acculturation among Vietnamese Americans

2047 Mental illness public stigma, culture, and acculturation among Vietnamese Americans

Irresistible: The Rise of Addictive Technology and the Business of Keeping Us Hooked

Irresistible: The Rise of Addictive Technology and the Business of Keeping Us Hooked

Irresistible: The Rise of Addictive Technology and the Business of Keeping Us Hooked

Irresistible: The Rise of Addictive Technology and the Business of Keeping Us Hooked

Detecting depression stigma on social media: A linguistic analysis

BackgroundEfficient detection of depression stigma in mass media is important for designing effective stigma reduction strategies. Using linguistic analysis methods, this paper aims to build computational models for detecting stigma expressions in Chinese social media posts (Sina Weibo). MethodsA total of 15,879 Weibo posts with keywords were collected and analyzed. First, a content analysis was conducted on all 15,879 posts to determine whether each of them reflected depression stigma or not. Second, using four algorithms (Simple Logistic Regression, Multilayer Perceptron Neural Networks, Support Vector Machine, and Random Forest), two groups of classification models were built based on selected linguistic features; one for differentiating between posts with and without depression stigma, and one for differentiating among posts with three specific types of depression stigma. ResultsFirst, 967 of 15,879 posts (6.09%) indicated depression stigma. 39.30%, 15.82%, and 14.99% of them endorsed the stigmatizing view that “People with depression are unpredictable”, “Depression is a sign of personal weakness”, and “Depression is not a real medical illness”, respectively. Second, the highest F-Measure value for differentiating between stigma and non-stigma reached 75.2%. The highest F-Measure value for differentiating among three specific types of stigma reached 86.2%. LimitationsDue to the limited and imbalanced dataset of Chinese Weibo posts, the findings of this study might have limited generalizability. ConclusionsThis paper confirms that incorporating linguistic analysis methods into online detection of stigma can be beneficial to improve the performance of stigma reduction programs.

Miłosierdzie jako kategoria życia społecznego

Refleksja nad rzeczywistością miłosierdzia w życiu społecznym, a więc także w przestrzeni pedagogicznej, prowadzi do poszukiwania odpowiedzi na pytanie, czy miłosierdzie jest oznaką słabości i rezygnacji z wymagań, czy przeciwnie – jest raczej znakiem rzetelnej troski o jakość życia społecznego i rozwój młodego pokolenia. Artykuł jest próbą poszukiwań odpowiedzi na to kluczowe pytanie i wypływające z niego problemy szczegółowe, w oparciu o wiedzę teologiczną i pedagogiczną.

Failures and Successes

Failures and Successes Jonathan P. Lewis Teaching the Works of Eudora Welty: Twenty-First-Century Approaches. Edited by Mae Miller Claxton and Julia Eichelberger. Jackson: UP of Mississippi, 2018. xiv, 224 pp. Softcover $30.00. Among physicians, morbidity and mortality conferences are essential learning tools. While I cannot imagine the terror of being questioned about medical choices that lead to the death of my patient, only through transparent discussions of errors, willful or not, can bad practices or judgments be driven out and successful paradigm shifts created. We teachers are perhaps too often loath to publicly admit failure, perhaps for fear of showing a lack of credibility or control in a political and social climate that leaps on any sign of weakness. Even when high school and college teachers get together, as I do each June for the Advanced Placement Literature and Composition Exam grading, there is often not enough of a focus on what doesn’t work in the literature and writing classrooms over what does. Teaching the Works of Eudora Welty: Twenty-First-Century Approaches offers both failures and successes, both what led to student disengagement with Welty’s works and what led to stronger critical thinking and writing skills. The collection’s essays are lean and to the point, and the book is quite useful in this regard as it gives instructors a wide range of approaches to this important writer. The book is broken into seven sections, and each section contains four or five short essays, each about eight pages long. The initial section covers elements of Welty’s biography, but from there, each of the sections engages a different aspect of approaching her works: through region, race, the body, international perspectives, the First Year Writing Classroom, and then a concluding section on teaching Welty through broader contexts outside the literature classroom. The writers’ backgrounds skews largely towards professors from southern universities with some from the high school level and a few international [End Page 147] scholars and Welty biographers/curators. This regional imbalance is perhaps to be expected, and several contributors note that teaching Welty outside the South can be challenging as there is a perception that non-southern students cannot understand her contexts, and that the questions of race and the depictions of racism can be quite difficult for non-southern teachers to deal with. Such concerns are understandable, but the strength of the collection comes from the pedagogical discussions, particularly the approaches that did not work, often for trying to avoid these presumed difficulties. For example, in her essay “Teaching Welty’s Narrative Strategies in Delta Wedding,” Sarah Gilbreath Ford says, “One approach that did not work was discussing the scholarship detailing the dense allusions to other texts that occur on every page of the novel” as “the students felt left out of the conversation” (32). Ford writes that she “was not starting from [her] students’ own interests or expertise” (33). To bridge this gap, Ford says that she then assigned each student a character from the novel “to follow when reading the novel,” tracking “appearances, descriptions, and opinions” and building a visual reference to the character through sticky notes as textual markers (33). All students became invested in their characters, which Ford says “led the students to the intertextuality that I had wanted them to see,” including seeing allusions to the Persephone/Hades myth which in turn led Ford to recommending that students explore the extant scholarship (34). In another example of a failed strategy, Christin Marie Taylor describes how her students came to Welty through social movements—in this case the story “A Curtain of Green” through the Civil Rights Movement—and became comfortable with literature “about political rebellion, racial oppression, and social change” alongside Martin Luther King’s “Letter from Birmingham Jail,” Paul Laurence Dunbar’s “Sympathy,” and Claude McKay’s “If We Must Die” (72). But “Curtain” was met with “faint scowls and averted eyes” because Welty offers them no sure footing—no “champion, no victim” (72). Taylor says she abandoned lecture to employ a “jigsaw” method: students break into small groups, assigned “sets of questions, related images, background information, and instructions that will help them arrive at unique...

Private Security and Paramilitarism in Colombia: Governing in the Midst of Violence

The article examines the links between paramilitary groups and the Colombian state within a context of pervasive violence. Colombia represents a particularly interesting case as high-intensity violence is accompanied by the preservation of a relatively strong institutional framework. Most interpretations of this relationship consider it to be either a sign of state weakness or a centralized strategy to outsource violence. Taking a different stance, the paper argues that the existence of paramilitary groups compels us to analyze government through practices vis-à-vis the treatment of violence. A policy linking private security and counterinsurgency, crafted in the early 1990s and known as Convivir, provides an illustration of this approach.

Correlation Between Transperineal 3-Dimensional Ultrasound Measurements of Levator Hiatus and Female Sexual Function.

The aim of this study is to investigate the association of sexual functions with levator hiatus biometry measurements and levator ani muscle defect. In 62 heterosexual, sexually active premenopausal women without pelvic floor disorders or urinary incontinence, 3-dimensional transperineal ultrasound imaging was used. Two 3-dimensional volumes were recorded, one at rest and one on Valsalva maneuver. Levator biometry measurements and levator defect were evaluated in an axial plane. Sexual function was assessed by a validated questionnaire, Female Sexual Function Index (FSFI). The primary outcome measure was correlation of sexual functions with the levator hiatus area, transverse and anteroposterior diameters, levator ani muscle thickness, vaginal length, and changes in measurements with Valsalva and levator defect. Forty-two women (67.7%) had low total FSFI scores (<26.55). Levator defect rates were similar in female sexual dysfunction (7/42, 16.7%) and women without female sexual dysfunction (5/20, 25%). The FSFI was negatively and weakly correlated with Δhiatal anteroposterior diameter (r = -0.33, P < 0.009) in the study population. There was a weak and inverse correlation between Δhiatal anteroposterior diameter and arousal (r = -0.35, P < 0.002), desire (r = -0.38, P < 0.001), and orgasm (r = -0.33, P < 0.007). Pain and lubrication did not correlate with any measurement. Hiatal area and diameters at rest are not related to sexual functions. Changes in anteroposterior diameter of the levator hiatus during Valsalva, which may be a sign of pelvic floor laxity or levator muscle weakness, are weakly associated with sexual functions, particularly desire, arousal, and orgasm domains.

P.128 - Broad clinical spectrum observed in patients with scapuloperoneal spinal muscular atrophy (SPSMA) caused by an c.806G > A (p. Arg269His) mutation in the TRPV4 gene

Scapuloperoneal spinal muscular atrophy (SPSMA) is a rare autosomal dominant disorder caused by heterozygous mutations in the transient receptor potential vanilloid 4 (TRPV4) gene. It is characterized by scapuloperoneal weakness and congenital contractures. Herein, we present two Polish SPSMA families harboring the same p.Arg269His mutation in TRPV4. In the first, the mutation was identified by direct Sanger sequencing of TRPV4 in the proband and then confirmed in other affected family members. For the second proband, clinical exome sequencing (TruSight One, Illumina) revealed the presence of the same de novo mutation. The mutation is known (HGMD, Clinvar) and was found not only in SPSMA individuals, but also in patients with Charcot-Marie-Tooth 2C. It is localized in a conserved protein region and predicted in silico to be pathogenic. The clinical spectrum observed in the first family was variable, although all members showed symptoms of skeletal dysplasia and mildly or non-progressive lower motoneuron disorder. The clinical presentation varied from mild (shortness of the clavicles with no sign of muscle weakness) to severe (hypotonia, severe congenital contractures, delayed motor milestones, short stature, global weakness and respiratory insufficiency). The proband from the second family, the first child of healthy young parents, presented with bilateral congenital talipes equinovarus, hypotonia and delayed motor milestones. At the age of five years, she was able to walk with calipers. In both families, EMG analysis revealed a neurogenic pattern, while her sensitive and motor nerve conduction velocities were normal. The SPSMA is characterized by an extremely variable clinical picture. We have observed an intrafamilial heterogeneity with a more severe phenotype in the following generations that is suggestive of anticipation.