bluuebunny/crossref_metadata_2025_split huggingface dataset:
Missed diagnosis in myotonic dystrophy: frequency, characteristics, consequences, and how to prevent it
Authors: Eymard Bruno , Dobon I 
Published: November 2023

Abstract
Myotonic dystrophy is one of the most frequent muscular dystrophies in adults, with a prevalence of around of 5 per I 00 000 (Harper and Rudel 1994). The clinical features include myotonia, distal weakness and systemic symptoms (cataract, atrio-ventricular block, dysrhythmias, endocrine dysfunction, mainly diabetes, and central nervous dysfunction). The diagnosis is theoretically easy for physicians if considered in the context of autosomal dominant inheritance, with a characteristic combination of muscular and extramuscular signs (Harper 2001 ). However, the diagnosis of myotonic dystrophy can be problematic for the following reasons: (I) difficulty in identifying specific symptoms as myotonia; (2) prominent systemic manifestations requiring initial advice from specialists other than neurologists; and (3) the occurrence of neonatal and infantile forms of myotonic dystrophy that may have a very atypical clinical presentation, with mental retardation and facial dysmorphy, whereas classical signs such as myotonia and distal weakness are absent before adolescence or early adulthood. In this chapter, our objective is to describe the features of misdiagnosis in myotonic dystrophy: the frequency of misdiagnosis, the nature of unrecognized symptoms, the categories of physicians involved, and the consequences of ignored diagnosis. First we present the French experience concerning a series of myotonic dystrophy patients assessed for missed diagnosis. Principles of a strategy for a proper and early diagnosis are proposed in the second section. Unless stated otherwise, all information given here refers to patients with the CTG expansion mutation.
Genetic counselling and genetic testing in myotonic dystrophy
Authors: Harper Peter S , Brunner Han G 
Published: November 2023

Abstract
Genetic counselling is undoubtedly one of the most important aspects of management for patients with myotonic dystrophy and their relatives. Initial diagnosis of the disorder in one person almost invariably leads to the recognition that other family members are at risk, or even affected, with a series of concerns and queries regarding their own status and the risks for their existing or future children, even if these concerns are not immediately raised or expressed.
Rural service delivery
Authors: Neese Jane B 
Published: November 2023

Abstract
To provide a thorough examination of psychogeriatric service delivery, this chapter presents a review of rural mental health, barriers and service delivery issues related to the older adult population. This chapter focuses on the United States and many of the issues identified are relevant to rural areas in other countries, but there are also likely to be differences. The term ‘rural ‘ carries many definitions depending upon the academic institution, politicians and country. According to Coward and colleagues ( 1994), an acceptable definition of rural has been elusive. Most traditional methods of identifying rural areas are based on population size, sparseness of towns, remoteness or distance to urban resources, degree of economic activity and commuting pattern (Magilvy et al., 1994).
The future of psychogeriatric services
Authors: Draper Brian , Brodaty Henry , Melding Pamela 
Published: November 2023

Abstract
What are going to be the key issues that shape psychogeriatric service delivery over the next few decades? In this chapter we will address this question from different perspectives ranging from developing countries to developed countries, from health policy considerations to specific new treatments and technology, and from ethics to economics. As described by John Snowdon and Tom Arie in Chapter I, psychogeriatrics is a young discipline with a formal history of only about 50 years. Much, however, can be learnt from this brief history when considering the future.
Evidence Supporting The Use Of Problem-Solving Treatment
Authors: Mynors-Wallis Dr Laurence 
Published: November 2023

Abstract
Problem-solving can be defined as the process by which an individual attempts to identify effective coping skills for specific problematic situations in everyday living. Problems are those situations for which an immediate and easily recognisable solution is not apparent. The impact of these problems upon an individual’s psychological functioning depends on their particular circumstances. An individual’s personal coping skills form a link between problems and whether psychological symptoms develop. These coping skills are the result not only of personality and previous experience, but also may fluctuate depending on whether the individual is suffering from a psychological illness. The appraisal of problems as challenging or threatening, and the individual’s self-perception as possessing robust or fragile coping skills, will also play a part in whether problems lead to the development of psychological symptoms.
The Seven Stages Of Problem-Solving Treatment
Authors: Mynors-Wallis Dr Laurence 
Published: November 2023

Abstract
Problem-solving treatment is a structured and focused treatment. There is an emphasis on getting the patient to quickly engage in activities which will bring about some early successes with the difficulties that they face. The challenge for the therapist is to facilitate the necessary patient activity and sense of achievement, without the patient feeling rushed and pushed into tasks for which they do not feel committed or adequately prepared. When patients, who have been treated with problem-solving, are asked what was helpful about the treatment, they identify not only the fact that they were helped to solve problems in a clear and structured way, but also that the therapist listened to their difficulties and helped to order their thoughts appropriately. The structure and focus of problem-solving treatment, therefore, must take place within a therapeutic context in which the patient feels supported and able to explain their thoughts and feelings.
Methodology and implementation of systematic reviews
Authors: McQuay H J 
Published: November 2023

Abstract
Systematic reviews and large randomized trials constitute the most reliable sources of evidence we can muster (Table 2.1). Put simply, they are the best chance we have to determine what is true. We discuss how to find trials, and how to appraise their quality. Further on there is advice on how to appraise the quality of systematic reviews themselves. The randomized controlled trial (RCT) is the most reliable way to estimate the effect of an intervention. The simple principle of randomization is that each randomized patient has the same probability of receiving any of the interventions being compared. Randomization abolishes selection bias because it prevents investigators influencing who has which intervention. Randomization also helps to ensure that other factors, such as age or sex distribution, are equivalent for the different treatment groups. Inadequate randomization, or inadequate concealment of randomization, lead to exaggeration of therapeutic effect.
Prophylactic drugs II: discussion summary
Authors: Schoenen J 
Published: November 2023

Abstract
The presentations, posters, and discussion during this session devoted to beta-blockers, amine antagonists, and antiepileptic drugs confirmed well-known concepts and provided some novel insights in migraine prophylaxis. Regarding trial methodology, it was pointed out that the outcome measure ‘migraine periods’ which covers the total duration of a migraine attack does not add much to ‘migraine attacks’ and ‘migraine days’ proposed in the IHS guidelines for clinical trials. Referring to valproate studies, attention was drawn to the fact that some cross-over studies, such as the Danish one, were superior in statistical power than certain large trials on parallel patient groups.
Antidepressants: mechanisms of action
Authors: Richelson E 
Published: November 2023

Abstract
Antidepressants, particularly tricyclic antidepressants, are widely used for the pro phylaxis of migraine headaches. In fact, the tricyclic antidepressant amitriptyline is reported to be the second most prescribed antimigraine drug, with propranolol being the first. In addition, amitriptyline is the only antidepressant for which there is consistent reports of efficacy for the prevention of migraine headaches, although 11 other antidepressants have been recommended for use. Interestingly, although their effects in the prophylaxis of migraine headaches appear to be unrelated to effects in treating depression, the general theoretical mechanisms of action involved in treating these two diseases are thought to be similar. That is, the down-regulation of certain receptors for serotonin (5-hydroxytryptamine or 5-HT) may play a role.. However, the serotonergic receptors that are down-regulated may be different for preventing migraine headaches than for treating depression.
Antagonizing peripheral sensitization in migraine
Authors: Bolton S , O’Shaughnessy C 
Published: November 2023

Abstract
Migraine is an episodic brain disorder characterized by attacks of throbbing head pain and sensitization of the cutaneous facial skin. Current research indicates that the dural blood vessels and their neural connectivity with the trigeminocervical complex play a fundamental role in the generation of these symptoms. As such, characterization of this pathway may provide an insight into the pathological processes occurring during a migraine attack and offer new targets for the development of novel anti-migraine treatment strategies. Utilizing various experimental techniques such as in vivo electrophysiology, intravital microscopy and fos immunohistochemistry, several studies have explored the consequences of trigeminal primary afferent neuronal activation both under ‘normal’ conditions and during sensitized conditions.
Migraine Prophylaxis With Drugs Influencing The Angiotensin System
Authors: Stovner L J , Tronvik E , Schrader H , Helde G , Sand, T , Bovim G 
Published: November 2023

Abstract
Various antihypertensive drugs have proven effective in migraine prophylaxis, most notably some of the -blockers and a calcium antagonist (flunarizin). Contraindications and side effects do, however, limit their use. Angiotensin converting enzyme (ACE) inhibitors and angiotensin II (All) receptor inhibitors are extensively used for both hypertension and cardiac failure, and with a very favourable side effect profile. A small open study with ACE inhibitors from 1995 indicated efficacy against migraine, and a meta-analysis involving 12 000 patients who were treated with an All receptor blocker for other conditions, but in whom headache was registered, indicated that the risk of headache was about one-third lower compared with those taking placebo. In addition, we tried on selected migraine patients first the ACE inhibitor lisinopril and later the All blocker candesartan, and these trials also indicated a prophylactic effect. For these reasons, our group con ducted two studies with almost identical designs, first with lisinopril (in 1999-2000), and later with candesartan cilexitil (in 2001-2002) for migraine prophylaxis.
What is the mechanism of action of ACE inhibitors in migraine prophylaxis?
Authors: Peatfield R 
Published: November 2023

Abstract
Cough is a common side effect of angiotensin converting enzyme(ACE) inhibitors. It is believed to be due to the inhibiting effect of these drugs on bradykinin metabolism, and in the management of hypertension it can be circumvented by substituting angiotensin II receptor blocking drugs, which control the blood pressure without inducing cough. I report a patient with frequent intractable migraine, who proved unresponsive to most prophylactic drugs and had side effects when on the only ones that influenced his migraine. He responded well to lisinopril, though the drug induced an unacceptable cough, and when losartan was substituted, his cough soon settled but the migraine recurred.
New targets I: discussion summary
Authors: Olesen J 
Published: November 2023

Abstract
Two posters from France demonstrated the potential of modern techniques for studying agonist receptor interaction and the subsequent intracellular cascade of events using dihydroergotamine (DHE). This substance is a 5-HT1A receptor agonist, a mechanism known to give antianxiolytic effects. The effect was shared with the main metabolite of DHE. DHE is also a potent agonist at 5-HT1B and 5-HTD receptors. At the 5-HT2B receptor both DHE and its metabolite induce desensitization, which perhaps could be responsible for its prophylactic effect. DHE is also a 5-HT7 receptor antagonist. Thus, DHE seems to have all the desired properties of a universal antimigraine agent in relation to the 5-HT receptors. Nevertheless, it is a fairly poor prophylactic agent as was illustrated by a large double-blind study, presented as a poster, that showed no significant difference between active and placebo.
Calcitonin gene-related peptide and migraine
Authors: Doods H , Just S , Schindler M , Eberlein W , Engel W , Rudolf, K , Arndt K 
Published: November 2023

Abstract
Although the so-called triptans are efficacious and are generally well-tolerated drugs for the treatment of migraine headache, there is still a need for improvement of migraine therapy. The relatively low number of patients becoming pain free in addition to the relatively high number of patients experiencing a recurrence of headache and lacking consistency of relief, together with the potential for cardio vascular adverse effects, reflect the limitations of the triptans as the present therapeutic standards. Since the introduction of triptans to the market, several other treatment approaches targeting different aspects of migraine pathogenesis have reached clinical status, but most of them failed or showed no real therapeutic advantage.
The prostaglandin-E1-analog misoprostol in the prophylactic treatment of refractory cluster headache and trigeminal neuralgia
Authors: Evers S , Frese A , Schwaag S , Lttmann, R , Husstedt 1.-W 
Published: November 2023

Abstract
Chronic cluster headache is a severe disease extremely affecting the quality of life. In many cases, the common prophylactic treatment options are unsatisfactory and do not lead to a continuous freedom from pain. Trigeminal neuralgia is a rare, although typical, brainstem symptom of multiple sclerosis (MS) occurring in about 2 % of all MS patients. In most cases, the semiology of this type of trigeminal neuralgia cannot be differentiated from the idiopathic form of trigeminal neuralgia and the principles of treatment are similar for both types. The pathophysiology of trigeminal neuralgia associated with MS is not fully understood. It has been suggested that demyelinating plaques in the entry zones of the trigeminal roots are responsible for the lancinating pain by ephaptic conduction. Inhibition of T-cell functions by prostaglandins could be a mechanism to decrease their inflammatory activity in the plaques and, thus, could be a specific therapy of this type of trigeminal neuralgia.
Managing disease
Authors: Campling Frankie , Sharpe Michael 
Published: November 2023

Abstract
If your illness is associated with a disease, part of managing your illness is to manage your disease. This requires that you get the right disease diagnosis, obtain and accept treatment for it, and monitor your progress. For a long-term illness, treatment for the disease almost always involves the people who are suffering from it taking an active part. For example, in diabetes, people have to check their blood sugar themselves and use diet, insulin or medicine appropriately. How well this is done influences not only how well you feel, but also the progress of the disease itself. Managing disease on an on-going basis is an essential part, though only a part, of managing your illness.
The epidemiology and aetiology of malignant mesothelioma
Authors: King J E , Hasleton P S 
Published: November 2023

Abstract
Introduction
Malignant mesothelioma is a primary tumour of the serosal membranes (mesothelium) which line the thoracic and abdominal cavities. It is most commonly described in the pleura, which accounts for approximately 75 per cent of cases in most series, but can also arise in the peritoneum, pericardium, and tunica vaginalis.
Viral factors in the pathogenesis of malignant mesothelioma
Authors: Powers A , Bocchetta M , Carbone M 
Published: November 2023

Abstract
Simian virus 40 (SV40) is a DNA tumour virus endogenous to Asian macaques that was first isolated in1960. Early studies demonstrated that the virus was highly oncogenic when injected into rodents and was capable of transforming cells from many species in tissue culture. Since then it has been used extensively in medical research because of its potent transforming ability.
Novel therapies for mesothelioma
Authors: Blackhall F , Fennell D , Baka S , Danson S , O’Byrne K , Ranson M 
Published: November 2023

Abstract
Malignant mesothelioma presents a major therapeutic challenge and new approaches are urgently required. Considerable progress has been made in understanding the molecular pathogenesis of cancer over recent decades. Despite the unique aetiology and clinical behaviour of mesothelioma compared with other common cancers, many of the molecular characteristics are shared. Malignant cell growth has been eloquently summarized as six essential alterations in cell physiology [1]: self-sufficiency in growth signals, insensitivity to antigrowth signals, evasion of programmed cell death (apoptosis), limitless replicative potential, sustained angiogenesis, and ability to metastasize.
Risk estimation in breast cancer
Authors: Evans D Gareth R , Kerr Bronwyn , Lalloo Fiona 
Published: November 2023

Abstract
Breast cancer is the most common form of cancer affecting women. One in nine to twelve women will develop the disease in their lifetime in the developed world. Every year 39 000 women develop the disease in England and Wales (population 55 million) and as a result, 13 000 will die (Cancer Research Campaign Statistics). While the gen- eral population risk of breast cancer that is widely quoted of 1 in 10, is a lifetime risk, the risk in any given decade is never greater than 1 in 34. This needs to be considered in the context of death rates from other causes (1) (Table 5.1).
Gödel incompleteness
Authors: Kaye Richard 
Published: November 2023

Abstract
This chapter is devoted to the proof of Godel’s first incompleteness theorem for recursively axiomatized theories extending PA−. Godel’s theorem is often considered because of its role in foundational issues in mathematics (in this context the first incompleteness theorem tells us that ‘arithmetic truth is not axiomatizable’). We shall be also interested in it for a different reason: the incompleteness theorem, in conjunction with the completeness theorem, equips us with a rich supply of interesting models. The techniques we employ, such as the representability of recursive functions in PA- and the diagonalization lemma, will also yield other well-known and interesting results in logic, such as Tarski’s theorem on the undefinability of truth and Godel's second incompleteness theorem.
Some number theory in PA
Authors: Kaye Richard 
Published: November 2023

Abstract
We will now start developing number theory in PA. One of our main goals is to prove a version of the Chinese remainder theorem. This will enable us to prove Godel’s lemma (Lemma 3.2) for arbitrary models of PA. As an application we will show how to derive Euclid’s theorem on the infinitude of the primes in PA. (This theorem, coincidentally, is the first ‘real’ mathematical theorem that Hardy proves in his Apology!) In fact, equipped with Godel’s lemma, the reader will see that it is routine to transcribe the proofs of many theorems in number theory into PA.
Prime models
Authors: Kaye Richard 
Published: November 2023

Abstract
If T is a complete consistent extension of PA there is a ‘smallest’ model of T having some very pleasant features. This chapter is about these models and the various ways in which they can be regarded as ‘small’. Section 8.2 contains an application (the famous MacDowell-Specker theorem) showing that any countable model of PA has an elementary end-extension.
Saturation
Authors: Kaye Richard 
Published: November 2023

Abstract
Recall from Chapter 8 that, given a theory T, a type over T is a set p(x) of formulas in finitely many free-variables x such that T⋃ p(x) is consistent. If M⊂T then the type p(x) is either realized or omitted by M, and there is always some model of T that realizes p(x). Chapter 8 showed that the models KT(for complete extensions T of PA) omit as many types as possible. In this chapter we shall look at those types that are realized in nonstandard models of PA.
The standard system
Authors: Kaye Richard 
Published: November 2023

Abstract
If M is a nonstandard model of PA, its ‘memo-pad’ is its standard system of of sets A⊆ℕ that are coded in M. In particular we can consider computations that consult this memo-pad to determine whether or not a given integer n is in some A ∈ SSy(M). In this chapter we shall investigate the structure of these standard systems further: in particular we will find certain closure conditions that characterize them exactly. This will allow us to construct models with a given standard system, and in Chapter 14 we will use these ideas together with the embedding theorems of the last chapter to construct new interesting initial segments.
Introduction
Authors: Pinch Enid R 
Published: November 2023

Abstract
The shortest distance between two points in a plane is obtained by drawing the straight line that joins them. The circle is the shape that encloses maximum area for a given length of perimeter. These well-known facts were known to the Ancient Greeks and are the oldest known solutions in the class of problems discussed in what we now call optimization theory. But, although the Greeks’ geometrical insight gave them the answers to a few problems in this field, it was not until the eighteenth century that a systematic theory began to emerge. That the theory of optimization continues to be an area of active research for mathematicians is an indication both of the inherent beauty of the subject and of its relevance to modern developments in science, industry and commerce.
Our aim is to understand the theory of optimal control, an area developed since the 1950s in response to American and Russian efforts to explore the solar system. The mathematical problems of space navigation include optimization problems. One wishes to construct trajectories along which a space vehicle, controlled by a small rocket motor, will reach its destination in minimum time or using the minimum amount of fuel. These new problems were not soluble by the methods that were currently available and the theory, whose roots lay back in the eighteenth century, had to be extended to meet the new challenge.
Submanifold theory
Authors: Willmore T J 
Published: November 2023

Abstract
In this chapter we shall consider the immersion of a manifold M into a Riemannian manifold (N, g), and examine the structures on M induced from the given structure on N. This is a natural generalization of the study of surfaces in Euclidean 3-space with properties induced from the Euclidean metric-which was the origin of the classical theory of surfaces.
Special Riemannian submanif olds
Authors: Willmore T J 
Published: November 2023

Abstract
We shall begin by studying the very special case of Willmore surfaces immersed in Euclidean 3-space. Even here there remain many unsolved problems. My interest in the subject dates from the early 1960s when I gave several talks on the topic at the Mathematical Institute, Ober- wolfach, Germany. This was followed by my first paper on the topic, Willmore (1965).