bluuebunny/crossref_metadata_2025_split huggingface dataset:
Parkinson’s Disease: Advanced Disease, Motor Complications, and Management
Authors: Fox Susan H. , Shah Binit , Walsh Richard , Lang Anthony 
Published: February 2014

Abstract
Advanced Parkinson’s disease is characterized by progressive worsening of motor symptoms with bradykinesia, rigidity, tremor, and bulbar, gait, and balance problems. All these complications lead to increased functional disability and reduced quality of life. With ongoing disease, many of these symptoms become less responsive to medical therapy. In addition, patients develop a range of fluctuating symptoms in response to levodopa therapy that mar the quality of antiparkinsonian response. Management of these levodopa-responsive and non-levodopa-responsive symptoms requires a combination of pharmacological, surgical, and non-pharmacological approaches.
Progressive Supranuclear Palsy and Corticobasal Degeneration
Authors: Williams David R. 
Published: February 2014

Abstract
Progressive supranuclear palsy (PSP) is the second most common form of neurodegenerative parkinsonism after idiopathic Parkinson’s disease. Patients most often present with an insidious change in balance, personality, or cognitive function that characteristically leads to severe postural instability, vertical supranuclear gaze palsy, and death within seven or eight years; this classic clinical presentation is now know as Richardson syndrome (also Steele–Richardson–Olszewski syndrome). Less commonly, patients present with bradykinesia and rigidity (PSP–parkinsonism) which is only later followed by the emergence of falls, eye movement abnormalities, and cognitive impairment; others may present with pure akinesia and gait freezing (PSP–PAGF), corticobasal syndrome (PSP–CBS), or progressive non-fluent aphasia (PSP–PNFA). The characteristic neuropathological findings of abnormal accumulations of neurofibrillary tau protein in the subthalamic nucleus and other brainstem regions define the disease, and because of these findings PSP is included amongst the primary ‘tauopathies’.
Corticobasal degeneration (CBD) is another rarer tauopathy characterized by the accumulation of neurofibrillary and oligodendroglial tau in the cortex and basal ganglia. The classic ‘motor’ presentation of CBD is known as ‘corticobasal syndrome’: a combination of limb apraxia, cortical sensory loss, alien limb, focal myoclonus, and limb dystonia, as well as rigidity and bradykinesia that do not respond to dopaminergic medications. Corticobasal syndrome is not specific to CBD and is also known to occur in other diseases such as PSP, Alzheimer’s diseas,e and the frontotemporal dementias. The ‘cognitive’ presentation of CBD typically includes behavioural disturbance, including personality change and executive dysfunction, and progressive non-fluent aphasia.
There is some clinical and pathological overlap between these two conditions, and progressive decline over six to twelve years is usual in both. There are no disease-modifying therapies for PSP or CBD, and treatment is focused on optimizing independence, minimizing the complications of the advancing parkinsonism, and supporting care-givers.
Essential Tremor and Other Tremors
Authors: Paschen Steffen , Deuschl Günther 
Published: February 2014

Abstract
Tremor is one of the most frequent movement disorders, with the majority of subjects suffering from either essential tremor or enhanced physiological tremor. However, patients presenting to the movement disorder specialist mostly suffer from tremor of Parkinson’s disease or essential tremor and a large variety of relatively infrequent tremor syndromes such as dystonic tremor, task-specific tremor, cerebellar tremor, orthostatic tremor, tremor in peripheral neuropathies, Holmes tremor, palatal tremor, and psychogenic tremor. Correct diagnosis is critical for the selection of appropriate treatments. Medical therapy is available for most tremors. Deep brain stimulation is established as treatment for drug-resistant cases for most of these indications.
Primary Dystonia
Authors: Elia Antonio A. , Albanese Alberto 
Published: February 2014

Abstract
Dystonia syndromes are defined by the occurrence of sustained muscle contractions, which frequently cause twisting and repetitive movements or abnormal postures. Classification of dystonia syndromes is based on three axes describing, for each patient, age at disease onset (early vs adult), distribution of involved body sites (from focal to generalized), and aetiology (primary, heredodegenerative, or symptomatic forms). Primary dystonias are further subdivided into primary pure (with dystonia only), primary-plus (with other associated movement disorders) and primary paroxysmal (characterized by intermittent symptoms).
The phenomenology of dystonia encompasses a combination of movements and postures to generate sustained muscle contractions, repetitive twisting movements, and postural (torsion) abnormalities. Dystonic postures can precede the occurrence of movements, and in rare cases can persist without the appearance of movements (so-called fixed dystonia). In dystonic movements speed of contractions may be slow or rapid, but is commonly sustained at the peak, making movements acquire a consistent directional or posture-assuming character. Dystonia has some unique activation/deactivation features: geste antagoniste (or sensory tricks), mirroring, and overflow.
It has been shown that both genetic predisposition and environmental factors play a significant role in the aetiology of primary dystonia syndromes. Genetic defects are known for two primary pure dystonias (namely, DYT1 and DYT6) and for several primary-plus, paroxysmal, and heredodegenerative dystonia syndromes.
There is no aetiological or neuroprotective treatment for primary pure dystonias. Symptomatic treatments aim to relieve involuntary movements, correct abnormal postures, prevent contractures, reduce pain and embarrassment, and improve function. Botulinum toxin is the first choice treatment for most types of focal dystonia. Neurosurgical treatments have a growing role in the symptomatic management plan since long-term electrical stimulation of the globus pallidus internus has been established to be effective for primary generalized or segmental dystonias.
Huntington’s Disease Look-alikes
Authors: Wild Edward J. , Tabrizi Sarah J. 
Published: February 2014

Abstract
Around 1 per cent of patients in whom Huntington’s disease (HD) is suspected clinically lack the genetic mutation that causes HD. Such patients, referred to as having HD look-alike syndromes, HD phenocopies, or HD-like disorders, present a diagnostic challenge. This chapter offers a definition of HD look-alikes and reviews the causes and features of HD look-alike syndromes, including HD-like syndromes 1, 2 and 3, familial prion disease, , spinocerebellar ataxias 17, 1, 2 and 3, dentatorubro-pallidoluysian atrophy (DRPLA), brain iron accumulation disorders including pantothenate kinase associated neurodegeneration (PKAN) and neuroferritinopathy, choreoacanthocytosis, MacLeod syndrome, Wilson’s disease, Friedreich’s ataxia, mitochondrial disease, and benign hereditary chorea, and considers possible acquired causes. The evidence for the relative frequencies of each genetic cause is reviewed. The yield from exhaustive genetic investigation of such patients is only 2 per cent, which is important when it comes to genetic counselling. We propose a rational framework for the investigation of HD look-alike patients based on genetic frequencies and associated clinical features. The exclusion of treatable causes is a top priority. Expert genetic counselling and multidisciplinary care via a neurogenetics clinic remain the mainstay of care for such patients.
Myoclonus
Authors: Tijssen Marina A. J. 
Published: February 2014

Abstract
Myoclonus is characterized by quick involuntary jerks and is classified as one of the hyperkinetic movement disorders. Myoclonus can be caused by muscle contractions (positive myoclonus) or by interruptions of tonic muscle (negative myoclonus). The main classification of myoclonus is based on the anatomical origin of the myoclonic jerks: cortical, subcortical, spinal, and peripheral myoclonus, each with distinctive physiological mechanisms. Clinical characteristics, aetiology, and treatment options are related to the anatomical classification of myoclonus.
The epidemiology of myoclonus is poorly documented, as myoclonus has many different causes and a spectrum of clinical presentations. A single study showed a lifetime prevalence of 8.6 cases per 100 000. The aetiology of myoclonus is diverse and in 70 per cent of cases is symptomatic. Over the last 10 years new genes have been discovered for several myoclonic disorders. Recent pathophysiological studies illustrate an important role for the cerebellum in generating both cortical and subcortical myoclonus, but further studies addressing the precise mechanism of myoclonus are required. Therapy is usually symptomatic, as treatment of an underlying cause is often impossible or ineffective. Therapeutic options are mainly based on a low level of evidence as randomized controlled trials are not available.
Sleep-related Movement Disorders
Authors: Reading Paul J. 
Published: February 2014

Abstract
As in many areas of sleep medicine, it is often a challenge deciding whether or not movements during sleep reflect truly abnormal nocturnal phenomenona or are simply physiological variants. Indeed, when asleep, subconscious shifts of body position occurring every 15 minutes or so are considered entirely normal, as are minor jerks of the extremities and face, particularly during periods of rapid eye movement (REM) sleep. However, if nocturnal movements are observed to be excessive, violent, or arousing, either to the subject or bed partner, they will usually indicate a defined disorder, especially if the events are stereotypic through the night. Although the second edition of the International Classification of Sleep Disorders (ICSD) was revised in 2005 to include a new section dedicated to movement disorders occurring specifically during the state of sleep, diagnostic precision of such phenomena is often lacking and evidenced-based treatment protocols, when indicated, are poorly developed. Furthermore, if a sleep-related movement disorder is recognized, it can be very difficult to determine its true clinical significance for sleep quality and subsequent daytime wakefulness, especially in the elderly patient.
This chapter addresses those movement disorders that are intimately or exclusively related to the state of sleep or the sleep–wake transition. By convention, parasomnias causing abnormal arousals from deep non-REM sleep, such as sleepwalking or night terrors, will not be covered, although restless legs syndrome is discussed, partly because of its close association with periodic limb movements during sleep.
Psychogenic Movement Disorders
Authors: Pareés Isabel , Edwards Mark J. 
Published: February 2014

Abstract
Psychogenic movement disorders (PMD) are commonly encountered in movement disorder practice and are part of the wider spectrum of “functional” neurological disorders. These disorders raise complex questions regarding aetiology, with some regarding them as fabricated, part of a purely psychiatric disturbance, or misdiagnosed organic illness. Here we detail the positive clinical criteria that separate these disorders from typical organic movement disorders, and show how scientific evidence points to a neurobiological explanation for symptoms. We discuss treatment, demonstrating how recent studies suggest that recovery is possible in many patients.
Problems before agreed psychiatric classifications were available
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
The problems listed serve to illustrate some of the main problems that develop in the absence of an agreed “common language” that can form the basis of international communication. They range from the diagnoses of individual patients to large-scale national statistics. Sometimes these problems reflect differences in national diagnostic traditions and schools of thought, the study of which is potentially constructive, but sometimes they are due to political and ideological ways of thinking that cannot stand up to rational examination and scientifically-based new knowledge.
Large-scale collaborative studies on diagnosis
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
The methods of interviewing and diagnosis just described in Chapter 2 made possible two large-scale collaborative projects on diagnosis. The first was the cross-national US/UK Diagnostic Project between London and New York, and the second, which overlapped in time, was the set of international educational and investigative programmes A,B,C, and D of the WHO. Using the PSE and the Diagno computer program of the MSS, plus the Glossary to ICD-8, The US/UK Diagnostic Project demonstrated that the much larger mental hospital admission rates for schizophrenia in New York were due to differences between the diagnostic concepts of the hospital psychiatrists rather than differences in the clinical states of the patients. Programme A of the WHO was a set of international seminars aimed at providing a basis for the forthcoming chapter V of ICD-9. Programme B was to test whether interviewing and rating methods, such as the PSE, could be translated and used in different cultures; this test took the form of setting up a network of Field Research Centres as a basis for the International Pilot Study of Schizophrenia(IPSS), carried out in Denmark, Columbia, Chekoslovakia, India, London, Moscow and Washington. Assuming that Programme B was successful, Programme C was planned to be an epidemiologically based international collaborative study on severe mental disorders; the Determinants of Outcome of severe Mental Disorders (DOSMED) study was the eventual result. Programme D was a long- term project aimed at the training and education of mental health workers in different countries in methods of epidemiological research
The first internationally understandable epidemiological studies
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
For the International Pilot Study of Schizophrenia of the WHO (IPSS) eight Field Research Centres were set up, in(Denmark), Cali (Columbia), Agra (India), Ibadan (Nigeria), Taipei (Taiwan), Washington (USA) and London (UK). A ninth, Prague (Czechoslovakia) was added in 1967. In each centre a “convenience sample” of 120 patients likely to be suffering from schizophrenia were was assessed by means of the PSE and other schedules; diagnoses were made according to the Glossary to ICD-8. Patients with typical syndromes of schizophrenia were easily found in all centres, and followed up for 2 years. An unexpected finding emerged in the follow-up, in that there were higher proportions of patients in the favourable outcome categories in the centres in the under-developed countries (such as India and Nigeria) than in the developed countries (such as Denmark, London and the USA). This finding was one of the principal motivations for the subsequent epidemiologically-based Determinants of Outcome of Severe Mental Disorders (DOSMED) study. For this, a wider set of cultures was covered by the addition of FRCs in Chandigarh (India), Nagasaki (Japan), Honolulu (USA) and Dublin (Ireland). In the UK, Nottingham replaced London. Each FRC defined a catchment area of at least several hundred thousand persons aged 15- 54 years, from which all persons who developed a new (lifetime first) potentially schizophrenic illness were assessed and included in a two-year follow-up study. The tendency for a better outcome for patients in the developing centres was confirmed, but remained unexplained. Using a comparatively narrow definition of schizophrenia, its incidence was not significantly different between the centres, but using a wider definition some differences were found. These findings were confirmed again by the latest WHO report published as the International Study of Schizophrenia (ISOS). In this, the IPSS and the DOSMED data are put together with data from a number of other similar studies sponsored by the WHO, to give follow-up assessment on a total of more than 1000 patients in 14 countries, over follow-up periods of 15-20 years.
Some problems with research methods used in diagnostic surveys
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
Since the presence of psychiatric disorders in the subjects of a survey is assessed by means of an interview, the training of the interviewer and the nature of the method of questioning will have a large effect upon what is recorded. A lay interviewer using the DIS or CIDI records the complaints of the subject whereas a psychiatrically trained interviewer using the PSE or SCAN records the symptoms. The extent to which these are equivalent for diagnostic purposes has been studied several times, and the conclusion is that they have considerable differences. In the absence of independent diagnostic tests, whether symptoms are preferable to complaints is a matter of opinion to be decided by the designers of each survey. In all survey schedules, the rating scales used for the questions are likely to be more useful if they are short rather than long and detailed. Designers of surveys also need to be aware of the differences between bottom-up and top-down schedules.
Translation and use of interviewing schedules for use in more than one language and culture
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
The idea that the significance of behaviour and language can be assessed only within their own culture has been superceded by the view that with due precautions, realistic comparisons and equivalences can be reached. Attention needs to be paid to equivalence, which takes account of the setting in which behaviour occurs, and authenticity which refers to the ability of the interviewer to get direct and ‘genuine’ answers from the respondent. When translating, the translation needs to be checked for its semantic, conceptual, and technical equivalences. The extent to which these different types of equivalence can be achieved depends to some extent on the properties of the schedule being translated, and the structured semi-standardized types are the best for this. Translation needs to be done by bi-lingual psychiatrists, using the re-iterative back translation method in cooperation with local mono-lingual experts. Video- or tape-recordings can be used for some of these procedures, but face-to-face discussions are likely to be more productive. Some simple rules for the translation processes are given.
Communication between health care professions
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
The first purpose of the classifications of mental disorders produced by psychiatrists has always been to summarize the current knowledge about mental disorders (and the links and limits between them) and to facilitate communication among psychiatrists about their recognition and management. The compilers of the classifications assumed that other professions involved in the care of the mentally ill (e.g. psychiatric nurses and general practitioners) would wish to use the same classification, and that the classifications would also be used for public health purposes. But these expectations were not fulfilled, in that different professional groups produced their own classifications of mental disorders. In addition, it soon became necessary to produce classifications for other purposes — for example the reimbursement of costs for care and the reports on the performance of health services. The consequence has been that a number of classifications have been produced and used, but often in forms that are not easily translatable into each other. As a result, communication and reporting about mental disorders and their treatment between health care professionals has often been of poor quality. The World Health Organization attempted to resolve this problem by issuing the ICD-10 Chapter V(F) in three versions — one for daily clinical work by psychiatrists, one for use by researchers, and one for use in primary health care. Care was taken to ensure that these three versions were easily translatable into each other. For a variety of reasons the use of these three classifications has not become universal, and the problem of ensuring cross-disciplinary communication and uniform reporting about mental health care continues to be a major challenge.
Understanding classification
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
In ordinary daily language, to classify things means simply to place them into groups which have some obvious features in common. This produces useful lists which are of great practical use in everyday life. More formally, the process of classification is part of the discipline of taxonomy, which can be applied to any type of objects, so long as there is a systematic and preferably quantifiable body of knowledge about the objects. Agreement needs to be reached about standard names and descriptions for all objects to be classified, so that an agreed nomenclature, glossary of terms and definitions will allow the objects to be arranged in similar groups or taxons, and perhaps even into a series of levels to form a hierarchy. Two well-known, very useful but different classifications are briefly examined, first, the biological classification of animals and second, the periodic table of the elements, to see if any points emerge that might be useful in producing future classifications of mental disorders. It is concluded that classifications based initially only upon descriptions of obvious physical characteristics may be very similar to later versions based upon more sophisticated techniques of measurement.
Classification beyond the diagnosis
Authors: Cooper John E. , Sartorius Norman 
Published: February 2014

Abstract
The diagnosis of the disorder from which a patient is suffering does not necessarily convey much information for that individual about the severity or duration of any associated disability. But since recording of the diagnosis is traditionally given much emphasis, and is easily available, it is often used by administrators for purposes for which it is not suited. In particular, ‘diagnosis-related groups’ (DRG) are a poor substitute for properly worked out measures of severity and length of disability. Some contemporary diagnoses are focused too much on the individual patient, and do not reflect adequately the effects on other persons and family members; there are now some signs that these deficiencies may be addressed in DSM-V and ICD-11, as relationship disorders. In the past, treating terms such as impairment, disability and handicap as synonyms has caused confusion, but special rating scales are now available which give specific meanings to these terms. The most recent example is the International Classification of Functioning, Disability and Health (ICF) produced by the WHO.