bluuebunny/crossref_metadata_2025_split huggingface dataset:
Coexistence of Idiopathic Parkinson’s Disease and Creutzfeldt-Jakob Disease
Authors: Ezrin-Waters C. , Resch L. , Lang A.E. 
Published: July 2016

ABSTRACT:The association of idiopathic Parkinson’s disease and Creutzfeldt-Jakob disease has not been reported previously. We describe the clinical findings in a patient with both disorders, confirmed at autopsy. The differential diagnosis of parkinsonism with cognitive and psychiatric disturbances is reviewed with emphasis on conditions which may complicate the course of idiopathic Parkinson’s disease. The etiological implications of this combination are considered.
Occipital Bone Cyst Causing Lower Cranial Nerve Palsies
Authors: Banna M. , Bradley W. G. , Kalbag R. M. , Tomlinson B. E. 
Published: July 2016

SummaryA 20-year-old girl presented with neck and occipital pain for six weeks, which was found to be due to a unicameral bone cyst of the left occipital condylar region.Although there have been very few reports of simple bone cysts in bones than the long tubular bones, it appears that the present case is probably the first report of such a lesion occurring in the skull.
The Incorporation of 3H (G) L-Leucine into Single Muscle Fibers in Duchenne Dystrophy and Charcot-Marie Tooth Disease
Authors: Monckton George , Marusyk Halyna 
Published: July 2016

SummaryThe results are reported of single muscle fiber uptake of tritiated leucine in muscle biopsy material from Duchenne dystrophy and Charcot-Marie Tooth disease. The uptake in the two conditions is compared and suggests that the previously reported increase in synthesis of cytoplasmic proteins in muscular dystrophy are probably related to regenerative efforts by the muscle fibers.
Degenerative Progressive Myoclonic Epilepsy Electrokymographic Observations
Authors: Derakhshan Iraj , Lotfi Jamshid , Abbsssioun Kazem 
Published: July 2016

SummaryClinical and pathological findings in two cases of degenerative progressive myoclonic epilepsy (PME) are described. The clinically difficult task of differentiating a “cerebellar” tremor from an action myoclonus is emphasized. Simultaneous elect roencephalography and electrokymography was done, using capacity to ground transients for recording hand movements. This method was found useful in corroborating the cerebellar nature of the remaining disorder, after successful treatment of the myoclonic element with anticonvulsants.
Regulation of Respiration in Friedreich's Ataxia
Authors: Begin R. , Lupien L. , Bureau M. A. , Labbe J. , Lemieux B. 
Published: July 2016

SummaryFriedreich's Ataxia (F.A.) is a degenerative disease which commonly leads to premature death of cardio-respiratory origin. To explain the early death of these patients, previous investigations have established the existence of I) a cardiomyopathy in nearly 100% of cases, 2) a restrictive pulmonary syndrome of scoliotic origin and 3) a mild hypoxemia associated with slight respiratory alkalosis and a normal oxyhemoglobin dissociation curve. To further assess the cause of early death in patients with such neuromyo-pathy, we evaluated, in eleven FA. patients, the sensitivity of the respiratory centers to hypercapnia, hypoxia, and hyperoxia.Ventilatory (VE, VT, F, VT/Ti) and occlusion pressure (P0.1) responses were taken as indices of the respiratory centers output during progressive hypercapnia (Read's method) and isocarhic hypoxia (Weil's method). We studied lì Friedreich's Ataxia patients and II age, sex, and arm-span matched controls. The responses of patients to hypercapnia were significantly greater than controls hut their responses to hypoxia were similar to controls.Our study establishes that the respiratory centers are functioning adequately in early Friedreich's Ataxia and do not contribute to cardio-respiratory insufficiency in such neuromyopathy.
Oculomotor Abnormalities in Friedreich's Ataxia
Authors: Kirkham T.H. , Guitton D. , Katsarkas A. , Kline L.B. , Andermann E. 
Published: July 2016

SummaryA clinical neuro-opthalmo-logical and electro-oculographic study was made on fourteen patients with Friedreich's ataxia. None had evidence of optic nerve dysfunction. No patient complained of oscillopsia although all had ocular motor deficits of varying degrees, which appeared to be related to the severity of the general manifestations of the disease. The defects comprised square wave jerks, jerky pursuit with inability to maintain eccentric gaze resulting in gaze paretic nystagmus and rebound nystagmus. There was failure to suppress by fixation the vestíbulo-ocular reflex. The slow phase velocity of caloric nystagmus was always of reduced velocity. There was inability to augment the slow phase velocity of optokinetic nystagmus with increasing stimulus velocity. Abnormalities of the saccadic system were manifest particularly as hypermetria. These signs in combination are suggestive of disease involving the cere be I lar flocculus and vermis or their brain stem connections. No abnormalities were found in 17 parents or siblings.
Electromyography and Nerve Conduction Studies in Friedreich's Ataxia and Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)
Authors: Bouchard J.P. , Barbeau A. , Bouchard R. , Bouchard R.W. 
Published: July 2016

SummaryTwenty four ataxie patients were investigated with electromyography and nerve conduction studies. They were divided in two groups according to the area they came from, the evolution of the disease, and the clinical signs. Group I patients from the Rimouski area displayed all the clinical and electrophysiological signs of Friedreich's ataxia. Group II comprised patients who presented with a new syndrome known as the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Although the clinical evolution was better in the latter, there were more electromyographic signs of denervation and the motor conduction velocities were slower. Both groups showed identical and important abnormalities in sensory nerve conduction.The results of electrophysiological studies in spastic ataxia have not been reported to our knowledge. They underline the place of spastic ataxia as distinct f rom Friedreich's ataxia, spastic paraplegia, and the known familial neuropathies.
Electroencephalographic Findings in Friedreich's Ataxia and Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)
Authors: Bouchard R.W. , Bouchard J.P. , Bouchard R. , Barbeau A. 
Published: July 2016

SummaryElectroencephalographie studies have heen done in two groups of hereditary ataxia: a group hearing the classical features of Friedreich's ataxia and a group clinically different described as autosomal recessive spastic ataxia of Charlevoix-Saguenay (A RSA CS). The qualitative anomalies observed in the two Ķroups were similar and were comparable with the data reported in the literature. However, the main difference between the two groups is the greater incidence of EEG abnormalities in the A RSA CS group, which suggests more involvement of the cortical and subcortical structures. This is reinforced by the lower I.Q. performance in the latter patients. Some comments are made about focal EEG findings, behavior and I.Q. In general, EEG was not considered a valuable instrument for diagnosis since no qualitative electric pattern could be identified. With regard to prognosis, EEG cannot be used as a criterion, since there is no relation between the degree of anomalies and the severity of the disease and since EEG does not worsen with the progression of the disease.
Hemagglutination by Lectins in Friedreich's Ataxia
Authors: Steinberg M.S. , Magnani J. , Czarkowski N. , Coccia M.B. , Barbeau A. 
Published: July 2016

SummaryCoded erythrocyte samples from ten individuals with Friedreich*š ataxia, from parents of five of these individuals, and from five unrelated control individuals were subjected to lecţin agglutination tests at three temperatures; before and after trypsinization; and before and after treatment with echinocyte-producing sodium salicylate and stornato^ cyte-pröducing tetracaine followed by shape-fixation with glutaraldehyde. The aġglutinins tested were the polycationic poly-L-lysirie (PLL) and four lectins with different saccharide specificities: soybean agglutinin, wheat germ agglutinin, Ufex europeus agglutinin (VEA) and concana-valin A. Altogether, over 45,000 individual test wells were scored, the status of each blood donor with respect to diagnosis being disclosed to the experimenters only after all results were tabulated.The majority of these tests revealed no significant difference among the three groups of blood samples. A few tests did reveal statistically valid (p<0.0l) differences between groups, the most significant of which were the following: Trypsin ized control RBC were more sensitive, on average, to agglutination by VEA (fucose-inhibited) than were RBC of ataxies or their parents. Non-trypsinized control “stomatocytes” were less sensitive, on average, to agglutination by PLL than were those of ataxies or their parents. Trypsinization appeared, on average, to sensitive control but not ataxia or parent RBC to PLL-agglutination. Other differences of borderline (p-0.01-0.025) or near borderline (p = 0.025-0.05) significance were also noted. None of the statistically significant, Friedreich's ataxia-telated differences in median agglutination titers were large, the greatest being about threefold, and in every case the ranges of individual titers within the differing groups overlapped. Thus, none of these tests at present offers a method of pre clinic al diagnosis or carrier detection, and only further tests can establish whether even the differences observed in the present series of tests are reproducible.
Criminalization and Sharing Wrongs
Authors: Marshall S.E. , Duff R.A. 
Published: July 2016

In colloquial speech the terms ‘crime’ and ‘criminal’ have a use which, though connected to the strictly legal notion of criminality, goes well beyond it. It is a way of indicating a serious condemnation of an activity or action. Similarly, when people say of something “There ought to be a law against it,’ they are not necessarily speaking literally, though they might be. Nonetheless, even when they are speaking colloquially and figuratively in using this expression, it is clear that the ‘law’ here is the criminal law and not the civil law. The sense that ‘something ought to be done’ expressed in these usages reflects the idea that there is a kind of wrong which demands a collective response rather than just an individual one. The figurative, colloquial use trades, of course, on the literal one. However, pace Devlin, it requires more than a general feeling of outrage to characterise just what kinds of wrongs are appropriately categorised as crimes. The enduring debates concerning the distinction between crime and tort bear witness to the fact that such a characterisation is far from easy.These academic debates can be situated against the background of wider philosophical discussions about the relationship between the individual and the collective, the private and the public, and the way in which these pairs of concepts map onto one another. We might agree with George Fletcher that “Blackstone had a point in identifying crimes as public wrongs and torts as private wrongs”: the criminal law should deal with those kinds of wrong which are matters of public concern, and which therefore require a collective response from the whole community; wrongs which are, by contrast, properly the concern only of the private or individual victim should be dealt with under the civil law. What is far less clear, however, is how we should characterise that distinction.
Giving It All Away? Thomas Reid’s Retreat from a Natural Rights Justification of Private Property
Authors: Mackinnon K.A.B. 
Published: July 2016

[P]roperty must exist wherever men exist, and…the right to such property is the necessary consequence of the natural right of men to life and liberty.Thomas Reid 1788I proceed therefore to consider in what State or Order of Society there is the least temptation to ill conduct, and I confess that to me the Utopian System of Sir Thomas More seems to have the advantage of all others in this respect. In that System, it is well known there is no private Property. All that which we call Property is under the Administration of the State for the common benefit of the whole political Family.Thomas Reid 1794The few remarks on property that are found in the Essays on the Active Powers of the Human Mind of the eighteenth century Scottish “Common Sense” philosopher, Thomas Reid, have led at least one commentator to treat him as a fairly traditional advocate of the natural right to (private) property, albeit one with a concern for the very poor. In an article on William Paley and the rights of the poor, Thomas Home remarks in passing that Reid’s (and Adam Ferguson's)major concern was to justify natural rights to property and that their interest in the poor was so little that a reader who accidentally skipped a paragraph or a page would miss all they had to say on the topic.
Kant on International Law and Armed Conflict
Authors: Orend Brian 
Published: July 2016

In recent years, there has been tremendous interest in the contributions of the great German philosopher Immanuel Kant to on-going debates about the appropriate nature of international justice and law. The end of the Cold War, for example, seemed to bring with it promises of greater international cooperation of the kind envisaged by Kant. Furthermore, those interested in the more explicitly moral aspects and potentialities of international law, such as those regarding human rights protection, have looked to Kant for comfort and inspiration in the face of strong pressures from doctrines dismissive of such aspects, such as law-and-economics and the statist strictures of realpolitik.There has, however, been widespread scholarly disagreement over Kant’s precise heritage in this regard: some, for instance, have found in Kant’s international writings a harbinger of world government while others have criticized Kant for faihng to specify any concrete powers for his fabled cosmopolitan federation; some have accused Kant of sacrificing his exalted moral principles on the dubious altar of state-centred expediency while others dismiss Kant as one of the most naively optimistic, and hopelessly moralistic, international thinkers ever; and, finally, some have accused Kant of glorifying the resort to warfare while others insist that Kant actually advocated “an extreme pacifism.” There is, in short, a ferocious and complex debate regarding which set of ideas can properly be called the “Kantian” view of these foremost issues of international law and what, if any, value can be attached to it.
Privatising Human Rights and the Abuse of Power
Authors: Raday Frances 
Published: July 2016

The classical concept of protection of human rights is generally perceived as appertaining to the public sphere. It is concerned with the protection of the individual against the power of the State. However, current voices are heard asserting that human rights are not confined to that sphere. It is claimed that individuals are entitled to respect for their human rights not only as against the power of the state but also in their dealings with one another. Introducing human rights jurisprudence to the disciplines of private law would limit the autonomy of an individual where exercise of that autonomy would infringe the human rights of another person. Under this proposal, the rules of such fields as contract law, company law, torts law and family law, would be subject to human rights principles.The privatising of human rights is taken by this author to infer the attribution of a review jurisdiction to the courts to override provisions of private law the application of which would bring about infringement of the human rights of individuals. It is in the nature of constitutional human rights protection to transfer the power to determine the limits of rights and powers away from the legislature and bestow it on the courts. By this conception of privatising human rights, obviously, not every protection offered by a state to individuals against interference or injury by private persons or bodies can be classified as an application of human rights in the private sphere. The protections provided by the criminal law or by torts, contract and labour law are not privatised human rights in this sense. The clarification is especially important in order to distinguish the provisions of international human rights law from a concept of constitutional privatisation. The international human rights provisions are in essence directives to governments and may be implemented by legislation. Only where they become directly applicable against private persons in the courts of the state party to the Convention can they be regarded as instances of privatising human rights. The perception of a right as a privatised human right rather than a mere application of the norms of the legal system is hence based on the source of the right in the constitutional hierarchy. In the analysis which ensues, the assumption will be made that there is a privatised human right only where that right derives directly from a constitutional power bestowed on courts. In this article, I shall employ the phrase ‘privatising human rights’ to denote this concept. As it will emerge, the use of this term is appropriate not only to denote the process but also to raise policy analogies to a more commonly known form of “privatising”—the privatising of economic activities of the state.
Liberalism and the Problem of Cultural Membership: A Critical Study of Kymlicka
Authors: Lenihan Don 
Published: July 2016

Moral conviction embodies an inescapable element of passivity, Hegel argued, a constitutive identification with morality’s demands, that cannot arise from autonomous decision, but only from the training and socialization that creates our very sense of self.Charles LarmoreIn the opening pages of Liberalism, Community and Culture Will Kymlicka tells us that the book was motivated by two concerns:One is my discomfort with recent communitarian discussions of culture and community, and with the kinds of criticisms they have brought against liberalism. The other is a discomfort with the way liberals have responded with indifference or hostility to the collective rights of minority cultures (1).
The Counter-Majoritarian Difficulty and the Road not Taken: Democratizing Amendment Rules
Authors: Colón Ríos Joel I. 
Published: July 2016

Discussions about the democratic legitimacy of judicial review of legislation are usually framed in terms of the so called ‘counter-majoritarian’ difficulty, the idea that judicial review is a deviant institution in a democracy. How can a country be considered democratic if a group of non-elected judges have the faculty to strike down laws that have been adopted by a majority of the elected representatives of the people? In framing the question in those terms, however, we tend to forget that there is nothing in the counter-majoritarian difficulty suggesting that judicial review of legislation is necessarily problematic from a democratic perspective. An institutional arrangement that gives judges the faculty to strike down laws inconsistent with the constitution only creates a counter-majoritarian difficulty if the constitution cannot be amended by simple majorities. In not paying proper attention to the role played by a rigid amendment process in the existence of a counter-majoritarian difficulty, this article argues, we have missed the opportunity of democratizing processes of constitutional reform in important ways while at the same time maintaining in place a system of constitutional review in which judges retain the ability of striking down legislation.The idea of giving simple majorities the possibility of having the final word on the meaning and scope of rights is of course not new. In fact, it is the basic feature of the weak system of judicial review now present in several commonwealth countries. However, such a system does not go beyond courts and legislatures, and it is therefore open to the same types of critiques advanced by defenders of strong judicial review against systems of legislative supremacy. The article defends the view that in a democratic society, deliberation and decision-making about the meaning and content of the constitution should extend beyond the ordinary institutions of government. Under that conception, a more democratic approach to the counter-majoritarian difficulty would provide popular majorities (as opposed to legislatures) with the faculty of amending the fundamental law in order to respond to a judicial decision that invalidated (or validated) an ordinary law. For example, citizens could be able to engage in the activity of constitutional reform through non-constituent assemblies, triggered by popular referendum and having the specific mandate of deliberating about the judicial decision in question and the power to propose constitutional changes that would be subject to popular ratification.
Treaties of a Particular Type: The ICJ's Interpretative Approach to the Constituent Instruments of International Organizations
Authors: QUAYLE PETER 
Published: July 2016

AbstractThe ICJ has confirmed that, on the one hand, the customary status of the general rule of treaty interpretation in the Vienna Convention on the Law of Treaties makes it applicable to the constituent instruments of all international organizations, but, on the other hand, constituent instruments are ‘treaties of a particular type’ with special considerations. This article examines the resultant implication that the Court adopts an approach to constituent instrument interpretation that supplements or modulates the approach provided for in the Convention. In doing so, it examines both the Advisory Opinions that respond to direct questions of constituent instrument interpretation, as well as Opinions and Judgments in which a question of constituent instrument interpretation is incidental to a dispute with respect to another treaty or general international law. The article identifies nine interpretative propositions used by the Court: (1) sufficiently clear text is conclusive; (2) the text cannot be nullified; (3) working documents (travaux préparatoires) afford auxiliary interpretation; (4) consistent practice precludes contrived interpretation; (5) to be consistent, practice need not be unanimous; (6) consistent institutional practice is determinative; (7) purpose can supplement text, but cannot contradict practice; (8) practice is interpretation, unless institutionally overruled; and (9) interpretation cannot be gratuitous. It is argued that to the extent the Court diverges from the Convention, it in fact allows for a more efficient and effective approach to constituent instrument interpretation and accords institutional practice particular prominence.
The Attribution of Responsibility and Modes of Liability in International Criminal Law
Authors: JACKSON MILES 
Published: July 2016

AbstractIn 2012, James Stewart published an article in this journal. The piece – ‘The End of “Modes of Liability” for International Crimes’ – argued for the abolition of accomplice liability in international criminal law and the adoption of a unitary model of participation in crime. This article argues that Stewart's proposal is flawed. As a matter of moral responsibility, the distinction between principals and accomplices follows from the recognition of individuals as moral agents. Turning to ordinary criminal responsibility, neither practical benefits nor expressive benefits nor the mitigating effects of the distinctive institution of criminal sentencing justifies the abolition of the distinction between principals and accomplices. Moreover, despite the collective nature of many international crimes, international criminal law ought to strive to accurately differentiate, in the attribution of responsibility, among participants. Only a differentiated model of participation can accurately and defensibly capture the different ways that individuals contribute to wrongdoing.