Near-death experiences (NDEs) refer to profound psychological events that can have an important impact on the experiencers’ (NDErs) lives. Previous studies have shown that NDEs memories are phenomenologically rich. In the present study, we therefore aimed to extract the common themes (referred to as “features” in the NDE literature) reported by NDErs by analyzing all the concepts stored in the narratives of their experiences. A qualitative thematic analysis has been carried out on 34 cardiac arrest survivors’ NDE narratives. Our results shed the light on the structure of the narratives by identifying 10 “time-bounded” themes which refer to isolated events encountered during the NDE and 1 “transversal” theme which characterizes the whole narrative and generally appears as a retrospective comment of self-reflection on the experience. The division of narratives into themes provides us with detailed information about the vocabulary used by NDErs to describe their experience. This established thematic method enables a rigorous description of the phenomenon, ensuring the inclusion of all self-reported manifestations of themes in narratives. After facing a life-threatening situation, some people  report profound psychological experiences that may include features such as an intense feeling of peacefulness or joy, out-of-body experiences (OBEs), meeting a deceased relative or a spiritual figure, or seeing a brilliant light . Such epLife After Life” )One of the major contributions of our study is that it sheds a different light on the structure of the narratives by identifying 1 “transversal” theme and 10 “time-bounded” themes. More specifically, “transversal” themes characterize the whole narrative and do not correspond to a specific moment of the experience. Moreover, the transversal theme  is generally addressed retrospectively by NDErs as they reflect upon their experience. On the contrary, “time-bounded” themes have more limited time duration and are generally described as clear isolated events .The originality of our approach also resides in the design of the study intended to provide new insights regarding the extracted themes. Firstly, the aetiology group of our participants was not revealed to the readers until the end of the analysis, and secondly, they were not experts in the field of NDEs. Obviously, NDEs are regularly discussed in the lay literature and the readers may not have been fully blinded, however, we wanted to enhance methodological rigor and reduce measurement biases by limiting any substantial influence of existing literature and preconceived notions on the processing of the narratives. For example, researchers’ knowledge of existing quantitative scales  could hIn the end, we identified the “altered time perception” as a transversal theme. This loss of time marker has already been reported in the NDE literature as a defining NDE feature e.g., ,8). Conc. Conc8])In parallel, readers identified 3 additional themes that partly overlap with the features identified by Greyson  and RingTo our knowledge, no NDE questionnaire formally investigates those 3 themes and we believe that further work should be done to collect them more systematically. Furthermore, it could be interesting to explore the way they are described in narratives relating NDEs of other aetiologies.Overall, these results corroborate the content of existing tools such as the Greyson NDE-scale , but alsIn addition to outlining the structure of narratives, text analyses explore the context of reported themes and provide detailed descriptions of NDEs. In our sample, the “light”, usually qualified as “brilliant”, “unusual” or “mystical” in the NDE literature , is alsExisting questionnaires, such as the Greyson NDE scale, allowed researchers to gather data and identify NDErs. Nonetheless, these tools do not offer the possibility to distinguish between NDEs of different aetiologies, or between classical NDEs and “NDE-like” experiences. In addition to the use of those scales, we believe that thematic analysis, combined with other types of qualitative analysis methods such as discourse analysis , should Finally, some issues remain to be addressed. First, the sample size in the present study is limited. Qualitative research is, however, very intensive and time consuming, which makes analysis of large samples impractical. Indeed, sample size in qualitative research is frequently much smaller than in quantitative study and generally does not exceed 50 participants . BesidesGiven the increasing number of NDEs testimonies and the impact that these experiences may have on peoples’ lives , it appe


The dissolution of the USSR resulted in independence for constituent republics but left them battling an unstable economic environment and healthcare. Increases in injection drug use, prostitution, and migration were all widespread responses to this transition and have contributed to the emergence of an HIV epidemic in the countries of former Soviet Union. Researchers have begun to identify the risks of HIV infection as well as the barriers to HIV testing and treatment in the former Soviet Union. Significant methodological challenges have arisen and need to be addressed. The objective of this review is to determine common threads in HIV research in the former Soviet Union and provide useful recommendations for future research studies.In this systematic review of the literature, Pubmed was searched for English-language studies using the key search terms “HIV”, “AIDS”, “human immunodeficiency virus”, “acquired immune deficiency syndrome”, “Central Asia”, “Kazakhstan”, “Kyrgyzstan”, “Uzbekistan”, “Tajikistan”, “Turkmenistan”, “Russia”, “Ukraine”, “Armenia”, “Azerbaijan”, and “Georgia”. Studies were evaluated against eligibility criteria for inclusion.Thirty-nine studies were identified across the two main topic areas of HIV risk and barriers to testing and treatment, themes subsequently referred to as “risk” and “barriers”. Study design was predominantly cross-sectional. The most frequently used sampling methods were peer-to-peer and non-probabilistic sampling. The most frequently reported risks were condom misuse, risky intercourse, and unsafe practices among injection drug users. Common barriers to testing included that testing was inconvenient, and that results would not remain confidential. Frequent barriers to treatment were based on a distrust in the treatment system.The findings of this review reveal methodological limitations that span the existing studies. Small sample size, cross-sectional design, and non-probabilistic sampling methods were frequently reported limitations. Future work is needed to examine barriers to testing and treatment as well as longitudinal studies on HIV risk over time in most-at-risk populations. The Former Soviet Union (FSU)—a group of countries and republics spread out across a vast area spanning Europe and Asia—is harboring one of the fastest growing epidemics of Human Immunodeficiency Virus/Acquired Immune Deficiency Syndrome (HIV/AIDS) in the world.When the Soviet Union collapsed in 1991, the constituent republics  were left to transition to independence with limited resources and guidance. During the transition period, economic collapse and political turmoil catalyzed many societal changes.8Efficacy of HIV testing and access to treatment have been evaluated at both the individual and societal levels in many regions of the world, but studies of how vulnerable populations—especially IDU—access these services in the FSU have been limited.Furthermore, the body of literature on HIV in the FSU is just beginning to take shape. However, the eventual goal of building a substantive body of literature around the causes of and barriers to reduction of HIV is to identify why at-risk populations are more vulnerable to HIV, as well as the barriers underlying suboptimal access to testing and treatment.Search terms used on PubMed included “HIV”, “AIDS”, “human immunodeficiency virus”, “acquired immune deficiency syndrome”, “Central Asia”, “Kazakhstan”, “Kyrgyzstan” “Uzbekistan”, “Tajikistan”, “Turkmenistan”, “Russia”, “Ukraine”, “Armenia”, “Azerbaijan”, and “Georgia” (Countries were chosen on the basis of availability of research literature). Bibliographies of relevant articles and reviews were scanned for further studies.To be eligible, studies had to be published in English, contain primary data, and identify HIV risk factors and/or barriers to HIV testing or treatment as primary outcomes. These overarching themes are subsequently referred to as “risk” and “barriers”. Studies highlighting risks and barriers were chosen for review because HIV prevention and treatment efforts cannot be realized unless the underlying risks are understood.The literature collected was sorted into geographical categories by country. The number of studies from each are as follows: Armenia (n=3)The majority of studies employed a cross-sectional design (n=36). Of these studies, 10 also obtained biological samples to determine HIV status of participants. The only longitudinal study was from Georgia where investigators and implemented both HIV testing and biobehavioral surveys at two different time points, three years apart from each other.IDU were the target population in 17 studies (44%) and were conducted in each country except Turkmenistan. Other major populations were migrant workers (n=8), female and CSW (n=7).The most common sampling methods were purposive sampling (n=19), convenience sampling (n=6), and respondent-driven sampling  (n=5).The most frequently reported methodological challenges were cross-sectional study design (n=12), inability to obtain a representative sample (n=11), use of self-report (n=11), sub-optimal participant recruiting procedures (n=8), and/or a small sample size (n=6). Further methodological limitations included: data were found not to be generalizable outside of the country in which the research was conducted (n=5), specifically having used purposive or snowball sampling to recruit participants (n=5), low participation rates (including not having obtained data on specific groups that declined to participate) (n=4), translation issues and cultural misunderstanding of qualitative data (n=4), and likely underreporting of risky, illegal, and/or stigmatized behaviors in surveys and interviews (n=4).The most frequently reported categories of risks were condom misuse (n=9), risky intercourse (n=9), unsafe injection practices among IDU (n=8), and spread of infection through people who inject drugs (n=8) . AdditioThe most prominent barriers to testing for HIV status included the perception that it was shameful to test for HIV (n=2), that testing was inconvenient (n=2), and that test results would not be held confidential (n=2) .The most frequently reported barriers to obtaining treatment for HIV were based on a distrust in the treatment system and experience with the lack of efficiency in the structure of the treatment system . SpecifiThis is, to our knowledge, the first systematic review of the current body of research spanning the stages of HIV infection in the FSU, from risk/infection through testing and treatment. The results reveal several important areas in which the current state of research and knowledge is incomplete due to methodological limitations of many studies. As shown in CSW, men who have sex with men (MSM), IDU, and migrant workers have been found to be key players in the spread of HIV.Migration between Russia, the Caucuses, and Central Asia has been observed as a driver of the epidemic.9Throughout the FSU, the IDU population has been growing and is associated with harmful drug use and co-infections of TB and HCV.61This group consists of both men and women who engage in sex work for compensation and suffer tremendously from the stigma that accompanies their work. Among this group, female sex workers are more stigmatized than their male counterparts. Those who also inject drugs experience a form of double jeopardy.62th century. On the other hand, each country has its own history and ethno-cultural fabric, which is likely to uniquely affect the mentality and psychology of its people. Therefore, studying the risks and barriers within every FSU country is necessary in order to help each one best prepare and implement an approach to ameliorate the HIV/AIDS epidemic.One, studies have been conducted in many, but not all, of the countries of the FSU. Research in the field of HIV/AIDS is particularly limited in Ukraine and Georgia, along with being severely limited in Turkmenistan. While the number of studies from Russia and Kazakhstan, for instance, are relatively numerous, they cannot necessarily be generalized to other FSU countries. This could be explained by the simultaneous similar-and-different nature of the countries: on one hand, they shared some common elements of their political, social, and economic history for most of the 20Two, many of the studies used a cross-sectional design in collecting their data. It is difficult to make statements of causation from such designs. More longitudinal designs are needed to study the range of factors for any given at-risk group. For example, migrant workers may need to be studied throughout the migration process to evaluate the stage of the migration experience that introduces the most vulnerability to exhibit HIV risk behaviors.Three, many of the studies employed non-probabilistic sampling. It is difficult to know the probability with which the target population has been represented in the sample when using a non-probabilistic method of sampling. Such methods that have been employed in this review’s studies include convenience sampling, purposive sampling, and snowball sampling. The studies required participation by individuals who exhibit illicit, illegal, or stigmatized behaviors and are, therefore, socially marginalized. Convenience sampling, purposive sampling, and snowball sampling were used to gain access to such populations. Although migrant workers, IDU and CSW are understandably difficult to access and representatively sample, studies could be designed in a more rigorous way that takes into account these limitations of working with hard-to-sample populations that are hidden and lack most formal forms of rosters or lists of documentation, from which probabilistic samples could be obtained. In a separate search of the literature about HIV risk behavior studies in other parts of the world, including Thailand and Australia, it was interesting to find that many of the studies did not demonstrate a need for purposive or respondent-driven sampling techniques, for instance. Instead, the researchers often approached CSW, in many cases, in testing or treatment facilities.65Four, many of the studies reported that it was likely that subjects may have underreported stigmatized, illegal, or risky behaviors in self-report questionnaires and interviews. Although not verified, it was an observation made by researchers who were likely aware of the stigmatized nature of most-at-risk populations. For this reason, it was thought to be likely, given that study participants may have distrusted the researchers and feared that the interview results deemed confidential would be released to the police.There are several limitations of the review that must be noted. First, while most of the studies were generally accessible in English, several studies were available only in Russian (n=8) or full-text was inaccessible. Without the ability to identify and retrieve all relevant studies, the review’s scope may diminish from the ideal. Second, the study of HIV is a relatively new area of epidemiologic and public health focus in the FSU, and thus the number of relevant studies is limited, resulting in 39 eligible studies for review. Third, the methodological limitations that were extracted from the study manuscripts and tabulated above were based on what the authors had listed in their own evaluation of study limitations and/or from what was available in the methodological descriptions of the studies. For instance, purposive sampling—as a methodological limitation in a study—was determined from the methodological descriptions and/or from the discussion of limitations provided by the study’s authors. This means that certain methodological limitations deemed infrequent in this review—especially “distrust of researchers” (3% of studies reviewed)—should not be viewed as certainly infrequent. Many of the studies may have suffered from participant distrust, which may have either gone unnoticed and had an effect on data or sampling outcomes or have been noticed but not reported in the manuscript. Participant distrust of the researchers can have an effect on many parts of a study that involve most-at-risk populations: participation rate, sample size, and underreporting can all be affected, but it is important to note in FSU-based studies when distrust occurs in order to help determine ways to improve the relationship between most-at-risk populations and researchers.Today, the epidemic grows as risky behavior continues, and prevention and treatment programs face difficulties gaining a foothold in the still-transitioning atmosphere of the FSU. However, the findings of this review reveal a few particular ways in which the current state of knowledge is incomplete as a result of methodological limitations of many of the existing studies. For instance, while 20 studies reported risk factors for HIV infection, only seven of the 39 studies reported barriers to HIV testing, and 9 studies reported barriers to treatment. These numbers illustrate where the bulk of the research has been conducted in the HIV infection pathway  in the FSU. One reason for this finding may be that there are many more individuals at risk for or infected with HIV than there are individuals who have sought testing and/or treatment. Given that small sample size has been a limitation and concern among many of the reviewed studies, it may be a reason for the relative scarcity of studies on barriers to testing and treatment when compared to studies on the risk factors for HIV infection.Going forward, energy and resources would be best spent on research to study the barriers to getting tested and treated for HIV. Of the studies included, the number of which that looked barriers to testing and treatment was minimal compared to what was aimed at studying the risks of HIV infection. Future research would include a combination of studies that address the described methodological challenges and one or more attempts at meta-analysis of the data from thematically aligned studies. Efforts to apply the results from the above-mentioned research would assist in improving existing HIV programs and advising the development of new ones in the FSU.68


The experience of tinnitus does not equate to tinnitus distress. Tinnitus suffering might be understood as a function of tinnitus-related distress in that bothersome tinnitus is an illness rather than a disease. In bothersome (distressing) tinnitus, the perception of the characteristic sound is a very disturbing and bothersome experience because of maladaptive psychological responses. Several cognitive and behavioral theoretical frameworks attempting to explain the nature and cause of tinnitus suffering have been introduced in and will be summarized here. Current treatment approaches are generally based on models that aim to: alleviate the perceptional experience by focusing on the tinnitus perception for habituation or even soothing purposes; decrease awareness of the sound by attentional training and cognitive interventions; decrease the maladaptive responses and the resulting distress by behavioral methods . The cognitive behavioral fear-avoidance model may offer an integrative cognitive behavioral approach that can lead to a new set of paradigms for studying the underlying mechanisms explaining chronic tinnitus suffering as well for developing innovative strategies to treat bothersome tinnitus.Tinnitus is  The term “tinnitus” is usually defined as the continuous perception of sound in the absence of an external (or adequate) source. Although it describes the instance of perceiving a noise, this definition fails to recognize that for some individuals this perception coincides with severe anguish. Indeed, tinnitus is a fairly common “harmless” auditory sensation for up to 21% of the adult population ; howeverbothersome tinnitus as being an illness rather than a disease. A disease is defined as biological damage or malformation in tissue, anatomy, or physiology, whereas illness can be defined as the subjective experience of “being unwell” or “sick,” which concurs with the observation that tinnitus can cause, but is not by definition, a dysfunction that interferes with every aspect of daily living. In bothersome (distressing) tinnitus, the perception of the characteristic sound is a very disturbing and bothersome experience because of maladaptive psychological responses.Tinnitus is not a disease, but merely a symptom, as evidenced by the fact that the experience of tinnitus does not per se equate to tinnitus distress, which might add to the confusion regarding its terminology. Analogous to observations in chronic pain research , 56, onetinnitus aurium, can be defined as the phantom perception of continuous sound or noise in the absence of an external (or adequate) source. In order to address and include the intrinsic psychological component of the distressing tinnitus experience, the following definition may be appropriate: A bothersome tinnitus (causing illness) might be best described as a negative auditory experience coinciding with aversive emotional reactivity, associated with, or described in terms of, actual or potential  harm. In analogy with the definition used for describing Chronic Pain suffering [The symptom itself, uffering .Confined to the individual’s subjective perceptual and emotional experience, tinnitus is not measurable or quantifiable by objective physical recordings, and is furthermore not traceable to disease, injury, or pathology in the brain or elsewhere. A medical or pharmacological cure is unavailable , 24 and Several cognitive and behavioral theoretical frameworks attempting to explain the nature and cause of tinnitus suffering have been introduced in the past and will be summarized here. Current treatment approaches are generally based on models that aim to: alleviate the perceptional experience by focusing on the tinnitus perception for habituation or even soothing purposes; decrease awareness of the sound by attentional training and cognitive interventions; or decrease the maladaptive responses and the resulting distress by behavioral methods . Current theoretical frameworks have been explanatory on some level, and the resulting treatment approaches have alleviated complaints leading to reports of occasional recovery to a satisfactory quality of daily life in many patients. The cognitive behavioral treatments (CBT) for tinnitus have indeed been shown to be effective in decreasing tinnitus distress, anxiety, and annoyance as well as improving daily life functioning , 37, 39.The habituation model proposed by Hallam and colleagues  is oftenThe model has remained largely theoretical, although tinnitus treatment approaches, such relaxation therapy, attention diversion techniques (directing attention away from tinnitus), and stress reduction by means of cognitive restructuring methods  have been based on its main premises . ResearcThe habituation model inspired Jastreboff , 43, whoThe neurophysiological model (NP model) for chronic tinnitus is based on the premise that conditioned fear responses elicited by the tinnitus sound are the cause of the tinnitus becoming bothersome , an alternative, and more adaptive pathway (turning right), whereby a positive or neutral evaluation of the tinnitus results in no or low fear of the tinnitus, and no or lowered distress. In other words, the tinnitus sound is accepted by the system as being benign, therefore no unwanted attentional resources are needed, and in turn, avoidance and/or escape behaviors do not interfere with daily tasks; therefore, there is no severe disability due to tinnitus.Accumulating evidence indicates that a CBT protocol for tinnitus based on this FA model, which targets re-appraisal of and exposure to the tinnitus sound, significantly reduces tinnitus distress, decreases tinnitus suffering, and improves the quality and daily functioning of tinnitus patients , 37, 48.The psychological impact of tinnitus has puzzled clinicians and scientists for many years. The extreme anguish and suffering of some patients are well observed and recorded, and the strong negative emotional connotation of the tinnitus experience seems to be commonly agreed upon , 21. ForAlthough there are common elements discernible across CBT-based treatments for tinnitus, CBT approaches vary largely, e. g., with respect to the content of treatment sessions , number of treatment sessions, hours spent in therapy, group versus individual formats, face-to-face versus Internet based self-help therapies, combinations of different treatment elements, and tinnitus diagnostics and outcome assessments. Moreover, CBT treatments in general, and by extension in tinnitus practice and research as well, have evolved during the past 30 years, today often including elements of the so-called third-wave CBT interventions.The theoretical frameworks have strong conceptual overlap and are based on the premise that the initially neutral tinnitus signal receives an “alarm” value, through classical conditioning. In turn, this negative tinnitus valence exacerbates negative responses in cognitions, emotions, and behaviors, hindering the normal process of habituation. Tinnitus distress ensues, which is the very negative and aversive state in which processes of adaptation and the efforts thereto have failed to return the organism to equilibrium or homeostasis.It is important to note that treatment avenues sometimes seem to be contradictory. The NP model’s widely applied Tinnitus Retraining Therapy  approacIn the early years in CBT for tinnitus, a large part of treatment time was allocated to relaxation as a means of stress reduction as well as an attention-diversion method. In addition, emphasis was placed on cognitive processes. Control techniques and attention refocusing training, as well as the promotion of masking the tinnitus by sound enrichment (purported to increase control over tinnitus) were claimed to be essential in tinnitus control and therefore relief. These techniques were indeed helpful in the short term. Later on, other cognitive and behavioral components entered the CBT protocols for tinnitus, which promoted to decrease avoidance behaviors toward the tinnitus experience to decrease fearful reactions. The recent emphasis on countering avoidance behaviors, increasing moment-to-moment awareness, and being attentive toward tinnitus are illustrated in the growing application of exposure-based CBT elements and the use of third-wave CBT interventions, such as acceptance and commitment therapy (ACT) and mindfulness, in tinnitus health care.Throughout the literature on effective tinnitus management, it is hard to find either CBT or sound-based approaches as the sole treatment strategy. In order to effectively manage complex tinnitus problems, treatments usually consist of a mixture of treatment approaches, combining psychologically informed education, sound therapy, and CBT approaches, have been proposed to effectively reduce the impact of the tinnitus on functioning , 10. HowThe cognitive behavioral FA model might offer an integrative cognitive behavioral framework that can lead to a new set of paradigms for studying the underlying mechanisms of chronic tinnitus suffering. This FA approach integrates previous hypotheses and might prove helpful both for discovering new venues of investigation as well as offering a means of determining why not only cognitive but also behavioral treatment approaches are repeatedly found to be successful. The FA approach also offers a means for discerning which treatment-components work best for whom.Tinnitus is not traceable to a single disease or pathology, but a symptom with a psychological impact that has puzzled clinicians and researchers for many years.Several cognitive and behavioral theoretical frameworks have been proposed to explain the nature, cause, and chronicity of tinnitus suffering.In the current report, the habituation model, the neurophysiological model, the cognitive behavioral model, and the fear-avoidance model are described, as well as the treatment methods arising from these.To effectively manage complex tinnitus problems, a combination of treatment approaches have been proposed to reduce the impact of tinnitus on daily functioning.The cognitive behavioral fear-avoidance model may offer an integrative approach that can help explain the underlying mechanisms of chronic tinnitus suffering and contribute toward the development of innovative strategies for treating bothersome tinnitus.


Henri Meige was a French neurologist now known for the eponymous “Meige’s syndrome”, which is a neurological condition characterized by blepharospasm and orofacial dystonic movements.Born on February 11, 1866 in Moulins-sur-Allier in France, into a family with a rich medical tradition, Meige represented the fourth generation of doctors. He graduated from high school in his hometown, moved to Paris to study medicine and, following his graduation, qualified for a position at Salpêtrière Hospital. His medical training was conducted under the guidance of Jean-Martin Charcot, the Head and the Chair of the Clinic of Nervous System Diseases at Salpêtrière. Meige had the advantage of being one of Charcot’s last interns and, until the end of his life, Meige remained under the great influence of his master’s genius. A few years after Charcot’s death, Meige wrote a short monograph, glorifying not only Charcot’s artistic interests, but also the artistry of his clinical skills and unmistakable diagnostic accuracy; the book was re-edited in 1925 [In 1893, the year of Charcot’s death, Meige obtained his doctorate with a thesis entitled “The Study of Certain Neuropathological Travelers: The Wandering Jew at the Salpêtrière”. Meige prepared his doctoral dissertation under Charcot’s tutelage, basing it on his and Charcot’s observations of Jews hospitalized at the Salpêtrière. However, he also incorporated some conclusions derived from non-academic papers on “wandering Jews”; part of which comprised hideously anti-Semitic trends. Meige noticed in his Jewish patients an irrepressible need to wander continuously, “from city to city, from clinic to clinic” in a search for “an efficacious remedy”. However, at the same time, he also noted these patients’ tendency to withdraw swiftly from any proposed therapy with new medications which were allegedly much more effective. Diagnosing “an obsession with constant traveling” or a compulsive unsteadiness in his patients, Meige reached the conclusion that this was a kind of putatively congenital and race-specific disease touching the Jewish population, and that racial factors were responsible for their inability to settle in a certain place or become part of a certain nation. Words that, in the context of the future Holocaust, are abhorrent.Fortunately, Meige’s doctoral analysis is not what represents his considerable contribution to neurology . In 1894Meige’s interest at that time was focused on acromegaly and the skeletal changes caused by the disease. In 1895, with Brissaud’s cooperation, Meige accurately concluded that gigantism in teenagers and acromegaly in adults shared the same pathogenesis and was actually the same disease entity, although with a different age of onset .Later, in collaboration with Eugene Feindel, Meige took up a subject that Georges Gilles de la Tourette had dealt with a decade or so earlier. In 1885, Tourette precisely reported the case of the Marquise de Dampierre, who was touched by a disease presenting with a composition of vocal and motor tics. Her illness began in childhood and the tics increased in number and severity over time. Although Tourette was awarded the honour of having the syndrome named after him, he proposed an explanation of the disease pathogenesis that reflected the spirit of the time: tics were a consequence of inherited nervous system degeneration due to an accumulation of the effects of immoral behavior in previous generation , 5.In 1902, Meige presented his conceptions on tic pathogenesis, symptomatology and treatment in a monumental monograph entitled “Les tics et leur traitement” . The booMeige continued his studies on involuntary movements and, in an article written in 1910, produced a report on the combination of blepharospasm and facial, mandibular, oral, lingual, and laryngeal spasms. Paradoxically, this short paper, with the first precise description of blepharospasm and orofacial dystonia, became his most important contribution to neurology and “Meige’s syndrome” remains one of the most recognizable medical eponyms in neurological practice .The tragedy of the First World War brought new challenges for the medical staff of the time. Along with others, French neurologists had to face the consequences of a large-scale war never experienced before: millions of soldiers suffered severe psychological and somatic injuries and new types of weapons meant new types of wounds. Throughout the Great War, Meige continued to carry out his research work, combining the care of injured soldiers with his scientific interests. Together with his Salpêtrière colleague Pierre Marie, Meige concentrated on the pathogenesis and clinical spectrum of neuropathy, using a modern method of electrical nerve stimulation. Their studies contributed new insights into knowledge of diseases of the peripheral nervous system. In 1915, Meige and Marie published a paper on localizing symptomatology in neuropathies and nerve injuries .Clinical neurology was not the only love of Meige’s life—he also dedicated himself to art. When bored with long lectures and discussions, Meige used to follow his attraction to art by unobtrusively escaping from medical congresses to visit local galleries and museums. Guided by another of Charcot’s former assistants, Professor Paul Richer, Meige developed his talent and gained a position at the École des Beaux-Arts—a famous art school in Paris. His artistic skills and invention helped him to assume the position of editor of two important French medical journals at the turn of the twentieth century: “Nouvelle iconographie de la Salpêtrière” and “Revue Neurologique”. Moreover, many of his articles were illustrated with drawings of his own creation. He also—surprisingly for a neurologist—had considerable knowledge of entomology and botany, frequently sketching pictures of the insects or plants he saw and described to his friends. In 1922, Meige succeeded Richer as a professor and the Head of the Chair of Artistic Anatomy at the École des Beaux-Arts.In his friends’ memoirs, Meige is recalled as a tall gentleman, who listened attentively to others, had a long face, was rather distant, calm and courteous, but liked by his colleagues and loved by his students; because of his musketeer mustache, his co-workers facetiously called him the “Count of Fère”, a title given to Athos, one of “The Three Musketeers” in the novel by Alexandre Dumas .Henri Meige passed away on September 29, 1940, but lives on in the vocabulary of neurology in the eponymous syndrome he had described 30 years earlier .


In the developmental literature, the idea has been proposed that young children do not understand the specificity of non-literal communicative acts. In this article, I focus on young children’s ability to produce and understand different forms of humor. I explore the acquisition of the communicative contexts that enable children to engage in humorous interactions before they possess the capacity to analyze them in the terms afforded by a full-fledged theory of mind. I suggest that different forms of humor share several basic features and that we can construct a continuum from simple to sophisticated forms. In particular, I focus on teasing, a form of humor already present in preverbal infants that is also considered a typical feature of irony. I argue that all forms of humor can be regarded as a type of interaction that I propose to call “playing with expectations.” In studies of communication, the interpretation of non-literal, indirect or figurative meaning occupies a distinct place. Various views have been advanced on this topic. In pragmatics, the classical two-stage theories distinguish a primary literal interpretation from a non-literal secondary interpretation, which can be developed only through the analysis and failure of the former . SubsequNon-literal communication includes numerous forms, such as indirect speech acts, metaphors, jokes, irony, and hyperbole. These forms occur commonly in everyday adult communication. Do they also occur in children’s communication? Certainly, when communicating with children, adults do not refrain from using non-literal expressions. Consider, for instance, the following examples:Wanna go for a bike ride?- You have a full dish in front of you.- Your brother is an ogre.- Tom’s cat is as big as an elephant.- I love children who keep their rooms clean.- When are children able to master these forms ? Are all of them cognitively equivalent? The few systematic studies that have been conducted suggest that acquisition does not follow a unique progression. Some forms are simpler to master than others . It is tOne hypothesis asserts that different forms of non-literal communication can be distinguished based on the role of theory of mind (ToM) abilities. The most demanding tasks require developed ToM abilities to comprehend the speaker’s meaning. For instance, according to An important question arises concerning the relationship between use and interpretation. Adults and children differ markedly with respect to this relationship. Theories may differ on how the chain of inferences that enables interpretation is constructed. Nonetheless, adults are undoubtedly able to interpret non-literal communication. If an adult laughs at a joke, we presume that s/he has understood its humor, and if s/he produces a joke, we assume that s/he has intentionally produced humor. When children produce humor, however, we are unsure whether they do so intentionally. Does the fact that a child laughs at a joke indicate that s/he has understood it? If the child makes us laugh, did the child do so intentionally, or was the humor unintended such that we, the audience, are creating it? In the case of adults, we do not pose the problem of the meaning of comprehension. Instead, we presume that use and comprehension are linked. In the case of children, this link remains unclear.In the developmental literature, the idea is often advanced that young children do not understand the specificity of non-literal communicative acts and cannot distinguish, for instance, between an ironic statement or a hyperbole and a lie . For youare able to do. I believe this perspective might help to reconstruct the developmental path and thus to more effectively understand mature comprehension of non-literal communication. In this article, I focus specifically on young children’s ability to produce and understand different forms of humor.This perspective is limited; it highlights only the tasks at which young children fail. Conversely, I aim to understand what young children My argument proceeds as follows. I identify the forms of humor that children typically use through several examples drawn primarily from parents’ reports. I then discuss the difficulties highlighted in the literature regarding the definition and categorization of different forms of humor. I specifically address the relationship between humor and irony. I explore the acquisition of the communicative contexts that constitute the background that enables children to engage in humorous interactions before being able to analyze them using full-fledged ToM abilities. I assume that young children react differently to lies and to non-literal communication. Finally, I present a theoretical proposal: I argue that different forms of humor share some basic features and that we can construct a continuum from simple to sophisticated forms. I focus on teasing, a form of humor already present in preverbal infants that is also considered a typical feature of irony. I conclude that all forms of humor can be considered a type of interaction that I propose to call “playing with expectations.”1 communicative interactions from a very young age , a 2.5-year-old girl answers, “Moo” (Muh) and laughs.Another parent reports an incident with her daughter, also 2.5 years old: “I asked Becky, ‘What is the cat’s call?’ (Come fa il gatto?). She answered ‘chirp’ (cip cip) and laughed. Then, she corrected herself: ‘No, mom, it meows! .”provocative non-compliance, offer and withdrawal of an object or of the self and disrupting others’ activities. In all of these types, children playfully disturb an interaction by performing “the mis-expected” (ibid.).xpected” . As theselational or linked to newly acquired skills. As examples of the first situation, consider the cases of contradicting expectations of kissing or hugging, withdrawing at the last moment, or playing with parents’ fears of approaching a dangerous or precious (and forbidden) object and withdrawing at the last moment.More precisely, based on my analysis of the existing literature and the parents’ reports I collected, it appears that parents’ expectations exploited by young children may be either rOne example was observed in a 2.3-year-old girl. “The aunt asked her, ‘Marta, will you give me a kiss?,’ to which she replied: ‘No, never!’ . The aunt looked sad, and [the girl] smothered her with kisses.”A good example of fears is reported in Common examples of playing with skills include those introduced earlier, such as deliberately attributing the wrong calls to animals, calling the father “mom” or the mother “dad,” or claiming that the sister (or the grandmother or the aunt) is a male, whereas the brother (or the grandfather or the uncle) is a female. Children typically play with newly acquired skills, a tendency confirmed in the literature. What form of communication does this type of humor represent? In at least one of the examples mentioned, the child explicitly indicated that her first response was not a mistake but an intentional joke. Often, however, the child makes no explicit declaration but displays what parents identify as a pert or ironic smile.mocking .If we consider the development of teasing in older children, we may add a new category to the categories identified by Reddy, namely, Consider an example mentioned by corpus. A 3.3-year-old boy, exaggerating his mother’s thinking mood, says, “Let us see, let us see…” .Consider an example from my However, older children also use forms of humor typically used by younger children, such as offer/withdrawal: “Mom, I brought you a cookie!” says a 4.7-year-old boy. When the mother, thanking him, approaches her son to obtain the cookie, the child eats it.The following example illustrates a case of playing with expectations regarding new skills: a 6.3-year-old boy tells his mother, “Today the teacher scolded me because I was not able to read…I got an A! (10 e lode).”The following example demonstrates play with relational expectations. A mother reports an incident with her 6.5-year-old daughter: “We are at the table, and my daughter looks at us and says, ‘You are old, but dad is the oldest in the house! Ah, ah! I am kidding, you are the most beautiful parents in the world,’ and she gets up and hugs us.”The following example is of disrupting others’ activity (3.1-year-old girl): the grandmother is counting money aloud, and her granddaughter says numbers at random to confuse her.In conclusion, teasing represents an intriguing form of humor because it develops precociously, yet older children and adults also use it. Consider the following instances of other forms of humorous interactions drawn from parents’ reports.(1) “The child, looking at the rain, says, ‘What a beautiful day, mom! It is ideal to go to the beach!’ .” (2) “I said, ‘Today I have prepared pizza,’ and my child said, ‘Mom, how disgusting! You made the wurstel pizza!’ (Mamma che schifo! Hai fatto la pizza con i wurstel!), his preferred pizza, and he plunged [toward his plate] to eat it (e si è tuffato a mangiarla).” (3) “Today, during lunch at [the] grandparents’ [home], grandfather made [a] noise when eating his spaghetti. She [the girl] started laughing and said, ‘Grandpa you are very elegant!’ (Lei ha cominciato a ridere e a dire ‘Nonno sei molto elegante!’).” (4) “She says, ‘Mom let’s go to the beach. Today we have a fine weather’ , while outside it rains like all the other days.” (5) “Sofia dropped her glass of water, and she looked at me in a loving mood (modo amorevole) and said, ‘It was not me! It was the glass, which did not want to live anymore!’ (non sono stata io! è stato il bicchiere che non voleva più vivere).” (6) “While I was doing the dishes, two glasses slipped from my hands and broke. Martina immediately commented, ‘Eh, mom, you are really good at doing the dishes!’ .” (7) “He plays with the sand, rolling over (rotolandosi) and getting dirty all over (sporcandosi tutto); he makes castles with a bucket and spade. I tell him, ‘You really like playing with sand, eh?’ And he says: ‘No, I don’t like it at all!’ .” (8) “Today, while she was playing with her doll, she said, ‘Mom, she is your child! She wants chocolate, she, no me!’ .” (9) “At lunch, she spilled water all over herself. She started laughing and said, ‘Bath I take!’ (Bagnetto ho fatto!).” Examples 1–3 appear to be typical ironic utterances. In fact, the children who performed them were in the age range in which, according to the literature, we can expect irony production . Are theIn the following sections, I discuss different ways of interpreting children’s ability to use and understand these forms of non-literal communication. First, however, I introduce additional examples of humor that children typically perform from a young age.(10) “Hello, my little doll” (Ciao bambolotto mio). A 4.11-year-old girl uses the phrase “little doll” to refer to her father imitating her mother, who had greeted her with the words, “Hello, little doll” (bambolotta mia). Note that when addressing the father correctly, the child transitions from the feminine form to the masculine form.(11) A 3.3-year-old boy tells his mother, “Mind that if you don’t behave I’ll call the traffic policemen” . In this case, the child employs a typical expression used by Italian parents to calm overly excited or misbehaving children.Examples 11 and 12 are noteworthy because the humor is produced by the fact that children use expressions with parents that the latter normally use with them and that become incongruous in this inverted form.Consider another example:(12) A 6.7-year-old girl is seated with a group of adults and children at a coffee shop. When everyone orders hot chocolate, she exclaims, “Well…I’ll take a beer!”In this example, irony results from the child’s incongruous appropriation of a behavior that the child cannot yet perform.Examples 10–12 indicate that a typical way that children use to produce humor is by uttering a statement that is normal when spoken by an adult but becomes incongruous when uttered by a child. Note that this incongruity applies to different forms of humor. In clowning, we may observe gestures rather than words, such as when a child wears her mother’s heels or walks with a grandparent’s cane. It applies also to teasing, as in Example 11, and to irony, as in Examples 10 and 12.Based on the analysis of the reviewed examples of humor, the following observations are evident:- If we consider humor a form of communicative interaction, distinguishing different forms of humor by age is not straightforward. At all ages, we observe both simple and more complex forms of humor. Although, we identify two forms of humor typical of young children, namely, clowning and teasing, we have seen that older children use these forms as well. We also note instances of very young children performing what is generally considered the most sophisticated form of humor, irony.- Children’s humor is multifaceted, but we can nonetheless detect typical manifestations.Thus far, we have analyzed forms of humor observed in children of different ages. We have not closely examined the current definitions of humor and irony. The next section discusses these definitions from a more theoretical perspective.This section presents the theoretical assumptions of my work. I propose that the relationship among humor, irony and teasing may be clarified by considering them different forms of a more general communicative ability that appears early in development and is characterized by playing with others’ expectations.represent the incongruity, an ability that children acquire at approximately 18 months of age. Shultz considers a necessary condition to be the resolution of incongruity, an ability that children acquire at 6 years of age. Other authors, by contrast, consider the detection of incongruity to be sufficient  and the degree of premeditation .- Different games may depend on the degree of complexity of knowledge that constitutes the common ground enabling the expectations, which are unfulfilled .Because all of the identified components are already present in young children’s teasing acts, I propose that teasing is the prototypical form of humor.Therefore, we can draw the following two conclusions:- If regarded as communicative games, different forms of humor cannot be differentiated by age.- All forms of humor, even the more sophisticated instances, can be characterized by playing with expectations, and every form of humor includes a teasing component.2 that could be considered a display of irony. In these studies, observations were completed for a predefined limited time in specific contexts.In the developmental literature, a clear distinction has been proposed between the acquisition of spontaneous forms of humor, which is typical of infants and young children, and sophisticated forms of humor, including irony. The use of simple humor has been observed in children’s familiar contexts. For these forms, the problem of comprehension has not been posed. By contrast, the comprehension of sophisticated forms of humor is considered a conceptual attainment that must be assessed with classical experimental procedures. Most experimental studies have shown that children’s understanding of irony does not begin before 5 or 6 years of age . Accordiseem ironic, but in claiming that they are ironic, we would be attributing to the child an intentionality that has not been proven. Considering these utterances ironic would constitute an over-interpretation. This perspective is supported by the fact that in experimental studies, young children do not seem to understand the ironic character of utterances.The late acquisition of irony is explained in terms of the ToM. The comprehension of irony implies the attribution of second-order beliefs to the speaker, or a full-fledged ToM , 2010. HI believe these two assumptions should be questioned. On the one hand, it is not clear that adults produce ironic utterances deliberately . It has implicit , would persist in older children and adults when the capacity of explicit reasoning has developed.The previous considerations prompt us to reconsider the relationship between the use of sophisticated forms of humor and ToM abilities. A result of this reconsideration might be to extend the concept of ToM. A number of recent studies have shown that infants can attribute epistemic states to agents, including false beliefs . These fThese results are abundantly debated in the developmental literature. The core of the debate centers on resolving the discrepancy between these results and the fact that 3-year-old children fail the classical false-belief tasks . More geTo explain the discrepancy between infants’ and older children’s performances on false belief tasks, Other authors have emphasized the influence of social experiences, which may help to explain individual differences in the development of ToM abilities . Also BaI believe these findings show that young children are able to monitor others’ behavior and to adapt to their actions regardless of how precocious forms of intuitive comprehension of agents’ actions are characterized . Studiescommunity of minds . It is reasonable to infer that a 3.3-year-old girl who laughs and tells her mother, “I am not sucking my thumb,” while actually sucking her thumb is not lying but playing a teasing game. If the child intended to lie, she would have employed a hiding strategy, regardless of its naiveté or effectiveness. As noted, other typical situations exist in which the use of irony constitutes an alternative to lying—for example, when a child cannot conceal a wrongdoing such as not having eaten a disliked food or having soiled an article of clothing. In these cases, irony is used as a possible escape. As the 3.3-year-old girl who soiled her T-shirt with ice cream says while looking at her mother, “This way, I can also eat the ice cream at home!” (‘Così mangio il gelato anche a casa!’).I have argued that the results of numerous experimental studies indicate only where young children fail in attempting to interpret non-literal communication. Children are unable to explicitly appreciate the nature of non-literal communication. We can now add what young children can accomplish with respect to this particular form of non-literal communication: humor. Children intentionally produce teasing communicative acts. Among these forms of teasing communicative acts, we also find incipient forms of irony.corpus that involves a young child. A 3-year-old boy is playing in a park. He sees another little boy approaching and tells his mother, “Look! My friend is coming!” Here, too, is a specific and succinct comment about a situation. The difference from the earlier example is that the presuppositions are simpler: the boy shares with his mother the knowledge that he does not like the other boy.Consider a hypothetical example of irony in adult communication. Two colleagues are standing near a coffee machine and see another colleague approaching. One of them exclaims, “Here is the hidden gem!” This exclamation is a perfect example of a “specific and succinct” comment about a situation in which the richness derives from the presuppositions shared between the two interlocutors. We can imagine that the two colleagues share the knowledge that the newcomer is particularly praised by his superiors, that he considers himself worthy of this consideration and that they, on the contrary, do not think that he deserves it. Let us now consider an actual example from our In conclusion, children may produce and understand non-literal communication without having developed a full-fledged ToM. Teasing communicative games do not differ in principle from serious communicative games; in fact, they are acquired in an identical way and become increasingly complex with age. Many elements may relate to their development, such as an improvement in language abilities, executive functions, social and ToM skills. Importantly, however, the considerably more sophisticated adult forms of communication involve the development of communicative formats that are already present in childhood.This work aimed to delineate a developmental framework for humor. The intent was twofold: to examine the different forms that humor can assume in childhood and to use the analysis of children’s humor to illuminate typical problems that are debated in research on humor in general.The existing literature typically distinguishes between spontaneous forms of humor, which infants and young children perform, and refined forms of humor, which only older children and adults can produce and understand. Based on this distinction, young children would be able to perform only forms of humor that generate immediate laughter. Other forms of humor that adults perform, such as irony, are generally regarded as considerably more complex and thus beyond the capacity of children to perform. In particular, young children are regarded as lacking the ability to produce and understand such forms of humor because they cannot comprehend their non-literal character. This task of comprehension would require inferring others’ mental states and, hence, the development of a full-fledged ToM.If, however, we conduct naturalistic observation, the situation appears differently. When asked to record their children’s humorous interactions in everyday life, parents report that even young children may use complex forms of humor appropriately. I have contended that young children acquire complex forms of humor within communicative games and that this acquisition does not require the ability to explicitly express an understanding of the implied mental states.I argue that to more fully comprehend the problem of acquiring complex forms of humor, it is helpful to analyze what humor entails as a form of communication in general. To facilitate this analysis, I proposed to focus on a form of humor that begins developing very early in life and is present in a more advanced form in adults, namely, teasing. I discuss several examples of teasing that are typical of different ages. Thus, I propose the possibility of a basic form of human communication characterized by playing with others’ expectations by teasing. Children acquire this form of communication very early in their interactions with adults. Such communication games may assume different forms that become increasingly sophisticated during development, aided by the development of ToM abilities. However, even young children may acquire communicative games that include sophisticated forms of humor and use them. For instance, irony may be part of a game of justification. Children may know that a parent who laughs at a self-ironic description of a misdeed will likely be more indulgent and will abstain from scolding the child. Another example is the appropriation of a communicative game typical of adults. This is a simple move, and it is clear that this appropriation is unexpected and provokes laughter in the audience. We can thus see that acquiring the ability to perform more complex acts of humor does not differ from the way an interactional perspective explains the acquisition of the ability to perform the simplest acts of humor. Consider the case of a child who discovers that if she wears her mother’s shoes, she will provoke laughter in the audience. These acts exemplify young children’s communicative cleverness. This cleverness is not apparent if we ask children to provide explicit explanations of conceptual differences. The same claim could be made of other communicative acts. We do not doubt that when children make a request, they produce an intentional act, even if they are unable to define a request as a communicative act.Therefore, we can assert that in early stages of development, a child’s use of the communicative game is deliberate, whereas irony is not . Later iFrom a cognitive perspective, the degree of complexity of the different forms of humor depends on the complexity of the communicative game. Thus, rather than claiming that one form of humor is simple while another form  is difficult, I suggest that the difficulty or simplicity of producing and comprehending a given instance of humor derives from the combination of several constituents that construct the specific communicative game. Most remarkable among these constituents is common ground. Every utterance draws its communicative meaning from a common ground that the interlocutors share. Common ground constitutes the context for comprehension. Nonetheless, the aspects considered when identifying common ground may differ considerably . For insAnother factor that may influence the ease of comprehension is the degree of indirectness. Planning an indirect act to hurt someone’s feelings, as in the case of sarcasm, is considerably more difficult than directly mimicking an interlocutor’s behavior to ridicule him or her.At least two research directions are apparent. I propose several characteristics as relevant for defining different forms of humor. However, it is possible that other characteristics could be considered. I contend that such additional characteristics would make the present model more elaborate but would not invalidate it. Another direction that could be examined in depth is the relationship between comprehension and production. Are these two processes symmetrical in acts of humor? Only a systematic study could indicate whether production and comprehension develop simultaneously.Comparing production and comprehension is not easy because of the different methods that may be utilized to study these two aspects. With respect to the production of humor, the only effective method is an observation technique. We cannot provoke the use of humor in an experimental situation. Moreover, we must resort to parent reports, which are observations made by non-professional observers. Naturally, parents are given precise instructions; for instance, they are asked to describe the context in which any specific humorous utterance is produced. The main problem involved in the use of this method is that it does not allow precise quantitative analysis because it is impossible to ensure that all parents devote the same attention to the observation of their children’s behavior. However, these limitations are balanced by the possibility to access the child’s spontaneous behavior at any time. I expect that future work will confirm that even very young children use a wide range of humorous utterances. Moreover, I expect to find similar typologies of humor in all children, namely, the forms that we have observed in our sample.In contrast, comprehension can be assessed through experiments. Experiments may also be used to evaluate the factors that influence performance in humor tasks. According to the theoretical assumptions expressed in this paper, one would expect no direct correlation between performance in humor tasks and performance in ToM verbal tasks. This is the result that we obtained in My perspective is compatible with the point of view expressed by I believe my perspective is advantageous to explain the fact that young children may produce sophisticated forms of humor, as the empirical evidence shows, without attributing them ToM abilities that are not demonstrated in other domains. The latter is true particularly for deceit, which children do not perform until a later age . AcquiriThis perspective is also useful for obtaining a better understanding of humor in general and of the relationship among humor, irony and teasing in particular. Studies on humor aim to define and categorize the different forms of humor. They encounter difficulty with the fact that humor manifests multifariously and that it is difficult to formulate definitions that allow the construction of a categorization without overlaps. The fact of laughter cannot be a criterion because several forms of humor exist in which an association with laughter is indirect and even loose or absent. We also cannot identify a function that characterizes all forms of humor. Sometimes humor represents a simple way to express immediate amusement, whereas other times it may function primarily to strengthen the relationship between the interlocutors by stressing and confirming shared knowledge. It may also be used to indirectly criticize an interlocutor in ways that can range from mild to harsh.I propose the construction of a unifying cognitive framework underlying the communicative games from which different manifestations of humor arise. I argue that this framework can be constructed by analyzing one of the most precocious and pervasive forms of communication, namely, teasing. Teasing is a feature that can be found in all forms of humor, whether simple or complex. I thus propose to characterize humor as a form of communication that has a teasing component and that plays with expectations. Children acquire this general communicative format during their initial interactions with adults. This communicative format becomes increasingly flexible and articulated with age and with cognitive acquisitions, including language abilities, ToM and relational competence.Consider a final example of a child’s utterance. When his mother’s car does not start, the 3.6-year-old boy asks, “Are we going to sleep here, mom?” How might we determine, in this case, whether this utterance is ironic? I would propose this remark as a typical form of teasing and, therefore, of humor.The author confirms being the sole contributor of this work and approved it for publication.The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Preserving upper extremity (UE) function in patients with Duchenne muscular dystrophy (DMD) is extremely important as it is related to independence and quality of life. For clinical decision making, knowledge of variables associated with UE function is necessary. This knowledge is, however, limited. Therefore, this study aims to gain more insight into the variables associated with UE function in DMD. Data from an international web-based questionnaire on UE function, obtained from 213 DMD patients, were used. Six dependent variables regarding UE function were used in multivariable linear regression analyses. In addition, 26 independent variables regarding patient characteristics, medication, therapy, supportive aids, pain, stiffness and participation were used. Twelve independent variables showed a significant relation to UE function. Variables with a negative relation to UE function were: later disease stage, occurrence of scoliosis, higher age, use of UE splints, more frequent stiffness complaints, more limitations due to stiffness, more frequent elbow pain, and having physical therapy. A positive relation with UE function was seen for going to school or work, use of corticosteroids, higher BMI, and higher age at diagnosis. These variables explained 56–81 % of the variation of the different measures of UE function. Knowledge of variables associated with UE function is very important in the clinical management of DMD patients. The results of this study suggest that corticosteroid use and participation in school and work related activities are positively related to UE function in DMD patients, as well as reducing pain and stiffness and preventing scoliosis. Duchenne muscular dystrophy (DMD) is one of the most common neuromuscular disorders. DMD is an X-linked recessive disorder affecting about 1:5000 live born males . The disUntil now no cure has been found for DMD, however, life expectancy has rapidly increased over the last few decades. Currently, life expectancy is about 30–40 years , 18, 19,Several studies have indicated that treatment with corticosteroids has beneficial effects on the preservation of UE function in DMD patients , 6, 12. To our knowledge there are no observational studies that have investigated variables associated with UE function in DMD, such as ‘participant characteristics’, ‘pain’, ‘stiffness’ and ‘participation’. However, this information could play an important role in clinical decision making with regard to the preservation (or perhaps even improvement) of UE function. Therefore, this study aimed to gain more insight into the variables associated with UE function in DMD using multivariable linear regression analysis of data obtained through a large international web-based survey .This study was part of a larger study in which 344 participants from 14 different countries responded to a web-based questionnaire . ParticiThe web-based questionnaire consisted of 224 items in total. Some items were extracted from existing questionnaires such as the capabilities of upper extremity questionnaire (CUE) , the ABIFor this study we used a subset of items from the total questionnaire Table . To findMedian values and ranges were used to describe the continuous variables. Valid percentages were used to describe categorical variables. Univariable regression analysis and stepwise multivariable linear regression analysis were performed to identify variables associated with the measures of UE function (dependent variables). Independent variables consisted of items from the sub categories ‘patient characteristics’, ‘medication’, ‘therapy’, ‘supportive aids’, ‘participation’, ‘pain’ and ‘stiffness’ Table . Data weIn total, 213 participants were included in this study, of which 198 participants filled in the complete questionnaire and 15 participants filled in only a part of the questionnaire, as they ended the questionnaire prematurely. Table p value <0.2 were entered in the multivariable linear regression analysis.The results of univariable linear regression analyses of potential variables associated with UE function in patients with DMD are presented in Table Multivariable stepwise linear regression analysis revealed a total of 12 different variables associated with one or more aspects of UE function Table . These aThe aim of our study was to gain insight into the variables associated with UE function in boys and men with DMD. Knowledge of these variables is essential for the clinical management of these patients. In this study we found four variables that were positively associated with UE function and eight variables that had a negative association with UE function.The finding that use of corticosteroids was positively related to UE function is not surprising, as it has been proven that this medication can retard disease progression , 12, 26.r > 0.6), as a result of which stiffness severity did not add to the explained variance of UE function in the multivariable model. The finding that use of splints and physical therapy showed a negative association with UE function is probably caused by the likelihood that these interventions are recommended more often to relatively severely affected patients [With regard to the variables that have a negative relationship with UE function, a later disease stage and a higher age are well conceivable based on the progressive nature of DMD. Although we found no studies that related the occurrence of scoliosis to UE function, it can be expected that deformity of the spine has a negative effect on sitting balance and reduced sitting balance has a negative influence on UE function , 10, 11.patients , 28. In A limitation of this study is that our results are based on a questionnaire that was primarily designed to gain insight in UE function in patients with DMD, not for the identification of variables associated with UE function. Thus, the possible variables associated with UE function in DMD were limited to those addressed in this questionnaire, leaving the possibility that there might be other variables associated with UE function that were not investigated. Another limitation is that the cross-sectional design of our study does not allow any inferences with regard to the nature of the observed relationships (cause vs. consequence). Third, our results are entirely subjective in nature, as no objective tests of UE function, pain or stiffness were performed. Therefore, the results of this study should be interpreted with caution. Nevertheless, our study addressed 26 possible variables associated with UE function in more than 200 patients with DMD, which provides a good basis for further  prognostic studies, using both subjective and more objective outcome measures, to improve our understanding of the most essential variables associated with function in DMD.It is important to realize that several of the variables associated with UE function in DMD that were identified in this study can be influenced by proper clinical management. For example, use of corticosteroids and living an active life by participating in school and work related activities can be stimulated by clinicians. In addition, prevention of scoliosis, maintaining a stable sitting balance, and reduction of pain and stiffness complaints may be attainable by regular attention from physical and occupational therapists, including the prescription of optimal assistive devices. Future longitudinal research should investigate whether proper clinical management of patients with DMD can indeed slow down the progression of UE impairments, UE activity limitations, and related participation restrictions.


Health care providers (HCP) and clinical scientists (CS) are generally most comfortable using evidence-based rational decision-making models. They become very frustrated when policymakers make decisions that, on the surface, seem irrational and unreasonable. However, such decisions usually make sense when analysed properly. The goal of this paper to provide a basic theoretical understanding of major policy models, to illustrate which models are most prevalent in publicly funded health care systems, and to propose a policy analysis framework to better understand the elements that drive policy decision-making. The proposed policy framework will also assist HCP and CS achieve greater success with their own proposals. The Oxford dictionary defines policy as "A course or principle of action adopted or proposed by an organization or individual" . It is dIncremental models were defined in 1959 by Lindblom who referred to them as the science of “muddling through” . IncremeRational policy models are grounded in some well-defined economic, scientific, social, or other framework with a pre-hoc defined ultimate goal . The curPolicy decisions in the real world can be complex and may contain both incremental and rational components. Etzioni described this middle ground as a hybrid “mixed-scanning” model, which involves making small incremental decisions leading to some overall rational goal .Figure In an era of evidence-based health care, HCP and CS strive to work in a scientific rational policy arena. However, it is naïve to think that policy decisions are only driven by scientific reasoning. Policymakers are influenced by many other factors, in addition to what makes sense scientifically.Figure The following is a brief synopsis of the individual policy assessment lenses. HCP and CS are generally most experienced, and comfortable, with the first four “lenses”, i.e. epidemiology, efficacy, effectiveness, and ethics.Epidemiology is the study of the occurrence and distribution of health-related states or events in specified populations, including the study of the determinants that influence such states, and the application of this knowledge to control the health problems .From a policy perspective, the greater the “burden” of disease or illness , the more likely the issue will be recognized as being important. In addition, specific target populations may also influence recognition. Examples include children, women , men , and the elderly . There are also issues that cut across target populations, such as initiatives focused on drunk driving .Efficacy relates to how well the proposal performs under ideal controlled conditions, with multicentre randomized controlled trials being the most representative methodology of such. In other words, what are the best outcomes that can be expected in an ideal setting?Effectiveness relates to how well the proposal performs under real world conditions, with prospective observational and retrospective studies being the most representative methodologies of such.Economics is an important consideration in publicly funded health care systems. This is especially evident in pharmacare where governments have to make funding decisions across a field of competing pharmacological interests. In the public realm, resources are scarce and getting the most net benefit amongst a number of competing options is the goal.If a new initiative is less expensive and more effective than the current standard of care, then it “dominates” other strategies. In contrast, if a new initiative is more expensive but less effective, it is unlikely to be adopted. However, if a new initiative is more expensive and more effective, how much “more effective” does it need to be to justify adoption?  This question is addressed by four health economic indicators: cost minimization (CM), cost-effectiveness (CE), cost utility (CU), and cost-benefit (CB).In CM, the outcomes of the options being considered are equal and the most economical option becomes the most desirable.CE and CU are measures of the net additional benefit gained by the new proposal compared to the existing standard(s) of practice. CE is the net cost ($) per additional natural units, e.g., year of life gained. CU is the net cost ($) per additional quality-adjusted units, e.g., year of life gained . CE and CU are commonly expressed as incremental cost-effectiveness ratios (ICER). The question of what is an acceptable threshold, or incremental cost-effectiveness ratio (ICER), is debatable. In 1992, Laupacis, et al.  proposedOne of the limitations of the CE/CU approach is that it is difficult to compare health policy proposals with different outcomes. For example, when comparing quality adjusted life years  against cavity-free children , the CE/CU approach will not work. Policymakers, especially at higher levels, may want to compare and contrast long lists of possible health care resource allocations. This is where a CB economic analysis can be applied. In a CB analysis, all outcomes, such as potential years of life saved, disability-free days, progression-free survival, etc., are converted to dollar values. Putting a value on a year of life is somewhat controversial but it is not dissimilar to setting a CE/CU threshold. The dollar values for the various outcomes are generally estimated through a “willingness to pay” exercise where individuals are asked how much they would be willing to pay per unit of outcome. This allows a rank listing of diverse health programs and services comparing how much each item costs compared to the dollar value of the outcome. CB is commonly referred to as getting the best “bang for your buck”.Table In health care, the most commonly cited ethics framework has been published by Tom Beauchamp and James Childress .The four key elements of their framework include:Autonomy – patients have a right to choose or refuse careBeneficence – health care workers have a duty to act in the best interest of their patientsNon-maleficence – “first do no harm” (primum non nocere).Justice – relates to the fair and equitable distribution and access to health care resourcesEthical issues can be complex, especially when there is a conflict between ethical elements. For example, a patient may want a health care service for which the health care provider feels may cause them more harm than good, resulting in a conflict between patient autonomy and non-maleficence.The current debate about low-dose CT screening for lung cancer is a good example of the challenges of making programmatic and resource decisions at a targeted population level. Does low-dose CT lung cancer screening cause more good than harm? Initial results from the National Lung Screening Trial (NLST) are promising from an epidemiology point of view . HoweverPolicy decisions are strongly influenced by existing legislation. If decisions are contrary to such, they can be challenged. Governments may choose to amend legislation to accommodate new policy directions but this takes time, especially if the change is beyond an order in council . For example, a substantive change to an act generally requires three readings in the house , approval of the senate for federal statutes, and royal ascent at both levels.As well, existing laws can be challenged. For example, the Canadian Supreme Court recently ruled (February 2015) that laws prohibiting assisted death of terminally-ill patients were unconstitutional and violated Section 7 of the Canadian Charter of Rights and Freedoms . The SuIn addition, health care policy may be influenced by multiple levels and branches of government  and this needs to be taken into account when analysing the feasibility of the proposal.Elected officials constitute a good portion of those who directly or indirectly influence law-making and policy decisions. The opinions of the electorate, and the public, at large, can be influential, especially when combined with election cycles.For example, Sznitman, et al.  have pubBurstein  has condPoliticians, collectively referred to as “government”, are responsible for making laws and having a significant influence on resource allocations. At the end of the day, and put simplistically, the government can only influence behaviour in three ways: incentives , disincentives , or exhortation . In the Canadian system, as is the case in most democratic countries, politicians are elected for fixed terms. As such, politicians, and especially the political party that has a majority or minority lead in Parliament or Legislatures, are sensitive to election cycles and the need for appropriate “positioning” for re-election purposes. This may disadvantage longer term projects that extend beyond election cycles.A proposal, which takes into account election cycle timing and provides positive feedback for a politician or a political party, will probably fare much better than one that is naïve to such factors.In addition, understanding, in real time, what is driving public opinion is important as resources may be directed accordingly. One may ask how does their proposal relate to or address issues important to the government. The more aligned the proposal is to the mandate or issue of the day, the more likely it will be supported.Political ideology is also important. For example, if a ruling government believes that publicly funded services should be delivered in publically administered facilities, it may be difficult to gain approval for a publically funded service being delivered in a private setting.There are a number of other factors which may influence policy and resource allocation. They include security crises , natural disasters , epidemics , social/economic factors , and international affairs , to name a few.MRI services were first offered Winnipeg at Saint Boniface General Hospital in 1990 . Figure This data suggests that factors, other than clinical demand, appear to drive health care resource allocation. Increased capacity during the 2007/08 and 2011/12 election years was achieved by increasing shifts  as well as by the temporary addition of a portable MRI for the 2007/08 election year. It would appear that factors, such as election cycles and associated or anticipated public opinion, drove such resource allocation decisions. That is, increased resources were allocated to reduce the elective MRI wait times to correspond to Provincial election cycles. It is interesting to note that over three election cycles, this was not observed in the more historic 2003/04, and more recent April 2016, MB Provincial election.On the face of it, policy decisions can appear irrational. However, when a more comprehensive set of factors is considered, such decisions may be made understandable. It is important to be comfortable with factors outside of scientific reasoning, such as public opinion, politics, economics, and legal issues. This will achieve two goals: first, a better understanding and comfort level with what influences policy decisions, and second, how to make one’s own research or health service proposals more relevant and successful. It is hoped that the provided model of a set of policy assessment “lenses” will help achieve these goals.