bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fatal Nemaline Myopathy in Infancy
Authors: McMenamin Joseph B. , Curry Bernadette , Taylor Glen P. , Becker Laurence E. , Murphy E. Gordon 
Published: July 2016

ABSTRACT:The clinical and neuropathological findings in two infants with congenital nemaline myopathy are described. One patient presented at birth with severe hypotonia, respiratory failure and contractures and died shortly after the neonatal period. The other presented at age two months with hypotonia and, following a period of clinical stability, died at age seven months from respiratory failure. Pathological findings in the fatal neonatal case revealed numerous rod bodies in lingual, pharyngeal, diaphragm and limb muscles, correlating with clinical findings. Significant, but less rod body involvement was found in the diaphragm and limb muscles of the second patient. Although a neural basis has been suggested for this disorder, no abnormalities were found in the central nervous system or in the peripheral nerves of these two severely affected patients.
The History and Pharmacology of Dopamine Agonists
Authors: Lataste X. 
Published: July 2016

ABSTRACTThe recognition of the dopaminergic properties of some ergot derivatives has initiated new therapeutical approaches in endocrinology as well as in neurology. The pharmacological characterization of the different ergot derivatives during the last decade has largely improved our understanding of central dopaminergic systems. Their development has yielded valuable information on the pharmacology of dopamine receptors involved in the regulatory mechanisms of prolactin secretion and in striatal functions.The clinical application of such new neurobiological concepts has underlined the therapeutical interest of such compounds either in the control of prolactin-dependent endocrine disorders or in the treatment of parkinsonism. Owing to their pharmacological profiles, dopaminergic agonists represent a valuable clinical option especially in the management of Parkinson’s disease in view of the problems arising from chronic L-Dopa treatment.
Epidemiology of Parkinson’s Disease
Authors: Rajput A.H. 
Published: July 2016

ABSTRACTEpidemiology of parkinsonism for 13 years (1967 to 1979) in Rochester, Minnesota is evaluated. For each patient, two age- and sex-matched controls with comparable medical follow-up (mean 40 years before diagnosis) were selected from the same community. Smoking habits, dementia, acute and chronic illnesses and mortality patterns in the two groups are compared. The annual incidence rate was 20.5/100,000, which is virtually unchanged from previous reports on Rochester population. Cigarette smoking habits of cases and the controls before the index date were not significantly different. Prior to diagnosis of Parkinson’s disease, dementia was diagnosed significantly more frequently among the patients than the controls (P = 0.048). After diagnosis of parkinsonism dementia emerged nearly three times more frequently in patients than among the controls. Mortality rate in the entire parkinsonian population was similar to that reported from the same community between 1935 and 1966. Only 50% of the patients were treated with levodopa. Among those treated with levodopa the mortality rate was comparable to their controls. Because there were no definite criteria for administering levodopa therapy, higher frequency of dementia and prolonged survival in those treated with this drug could be attributed to the bias of case selection for treatment.
Dopamine Agonists in Parkinson’s Disease
Authors: Calne Donald B. , Burton Keith , Beckman Jeff , Wayne Martin W.R. 
Published: July 2016

ABSTRACTDopamine agonists have yielded two important advances to our understanding of the basal ganglia – they have facilitated the subdivision of different classes of dopamine receptors, and they have established the fact that important dopaminergic effects can be achieved by activation of dopamine receptors in a manner that is unrelated to anoxal impulse traffic in dopaminergic neurons – a phenomenon similar in its diffuse, slow, characteristics to an endocrine effect.The tangible clinical benefit of dopamine agonists has been evident in patients with prominent dyskinesia or wearing off reactions. It is possible that earlier use of agonists, in low doses combined with similarly low doses of levodopa, may improve the long term treatment of Parkinson’s disease, but as yet there is no firm evidence.In the future, we can expect to see agonists with more prolonged effects, deriving from the formation of active metabolites. We can also hope to gain further insight into the correlations between the various animal models of dopaminomimetic activity, and specific aspects of drug efficacy and toxicity in parkinsonian patients. Such information should allow the design of improved pharmacotherapy.
Should Dopamine Agonists be Given Early or Late in the Treatment of Parkinson’s Disease?
Authors: Rascol A. , Montastruc J.L. , Rascol O. 
Published: July 2016

ABSTRACTThe long term consequences of the use of a dopamine agonist, bromocriptine, in the treatment of Parkinson’s disease are reported. In a first study in 82 patients showing late side effects of levodopa, bromocriptine permitted a significant decrease of the gastro-intestinal adverse effects. In contrast, no significant improvement of end of dose deterioration from levodopa was noted. In cases where levodopa had ceased to be active, bromocriptine produced an improvement in the clinical state. The drug was ineffective in the very advanced stages of the disease or in the cases of dyskinesias without “on-off” effects. Bromocriptine did not significantly improve freezing or “on-off” effects, but reduced other side effects of levodopa, in particular dystonia. In a second group of 29 patients who had never received levodopa treatment, bromocriptine was shown to be very effective as a first treatment of the disease. The most important finding was the absence of long term side effects similar to those usually observed under levodopa: in this group and in comparison with 38 patients taking levodopa, dyskinesia, dystonia, oscillations in performance and especially “on-off” effects were not noted. However, a partial loss of efficacy of bromocriptine was observed in 27% of cases. In a third group of 10 patients, bromocriptine introduced according to a low and slow protocol was found to be active in a limited number of patients only.
Intrauterine Hydrocephalus and Ventriculomegaly: Associated Abnomalies and Fetal Outcome
Authors: Cochrane D. D. , Myles S. T. , Nimrod C. , Still D. K. , Sugarman R. G. , Wittmann B. K. 
Published: July 2016

ABSTRACT:Advances in fetal diagnostic techniques have opened many areas to prenatal anatomical scrutiny. Intrauterine hydrocephalus and ventriculomegaly are conditions which are readily diagnosed. Fetal intervention has been undertaken in humans in order to minimize the craniofacial disfigurement and to maximize the growth potential of the brain. To justify such an approach, the significance of all anomalies should be recognized prior to treatment. The authors have reviewed 41 cases of hydrocephalus diagnosed in utero in order to define associated anomalies and patient outcome.75% of our personal series and 72% of the reviewed literature cases had other anomalies of the central nervous system. Other system malformations, some of which proved fatal, were seen commonly. Prenatal diagnostic techniques did not always reveal these additional problems.The outcome of these pregnancies is not good. Approximately one third of these fetuses have survived to be treated postnatally and to be followed up clinically. Only 7.5% of this series were felt to have attained normal developmental milestones. The remainder of the survivors have various focal and/or global cerebral deficits.
Adenoid Cystic Carcinoma: An Unusual Neurosurgical Entity
Authors: Dolan Eugen J. , Schwartz Michael L. , Lewis Anthony J. , Kassel Edward E. , Cooper Perry W. 
Published: July 2016

ABSTRACT:Adenoid cystic carcinoma, or cylindroma, usually presents in the salivary glands or in the upper respiratory passages. We report an unusual case of adenoid cystic carcinoma in a 45-year-old woman who presented with a three-year history of progressive right trigeminal sensory palsy, most pronounced in the second division. There was, in addition, a right trigeminal motor palsy and a partial right 6th nerve palsy. CT scanning showed only a small soft tissue mass spanning an enlarged right foramen ovale. Solid adenoid cystic carcinoma was resected from Meckel’s cave via a middle cranial fossa approach. A subsequent biopsy of the right maxillary antral mucosa found tumor tissue. We concluded that the tumor originated in the maxillary antrum and spread posteriorly along the infraorbital nerve to enlarge in the foramen ovale. Radiation to a total of 5,000 cGy was given. At 22 months there was neither radiographic nor clinical evidence of recurrence. At 30 months, the development of unsteady gait signalled the presence of recurrent tumor extending backwards from Meckel’s cave into the right cerebello-pontine angle.
Longitudinal Evoked Potential Studies in Hereditary Ataxias
Authors: Taylor M.J. , Chan-Lui W.Y. , Logan W.J. 
Published: July 2016

ABSTRACT:We studied multimodal evoked potentials (EPs) longitudinally in a series of children with Friedreich’s ataxia and ataxia telangiectasia to determine both their diagnostic utility and their correlation with clinical regression.The auditory brainstem responses (ABRs) were abnormal only in the children with Friedreich’s ataxia. The abnormality seen in these patients was a rostral-caudal loss of the ABR waves. The visual EPs (VEPs) were abnormal in many of the patients; those with ataxia telangiectasia had unusually low amplitude or absent VEPs, occasionally with increased latencies, whereas those with Friedreich’s ataxia had normal amplitude VEPs, often at increased latencies. The somatosensory EPs were usually of increased latency or absent in these patients. Unlike the ABR and VEPs, they did not serve to differentiate the groups.Changes in the EPs appeared to reflect clinical deterioration; patients with little change in their EPs over several years were regressing very slowly, whereas others had rapid deterioration in both EPs and clinical status. We suggest that the EPs are diagnostically of value in degenerative ataxias and may be of value in monitoring these patients and their response to therapy.
Persistent Hemiballismus with Lesions Outside the Subthalamic Nucleus
Authors: Lang Anthony E. 
Published: July 2016

ABSTRACT:Two patients are reported with hemiballismus due to lesions in the striatum as documented on CT scan. The involuntary movements persisted for one and five years respectively. A third patient with more typical self-limited hemiballismus had no evidence of a causative lesion on CT scan. It is proposed that lesions outside the subthalamic nucleus of Luys may interfere with the compensatory mechanisms which can result in the spontaneous resolution of hemiballistic movements. None of the patients benefited from the use of sodium valproate therapy, however, pimozide reduced the severity of the dyskinesia in the two with longstanding hemiballismus.
Amantadine Therapy for Fatigue in Multiple Sclerosis
Authors: Murray T.J. 
Published: July 2016

ABSTRACT:We carried out a double blind control study of fatigue in 32 patients with multiple sclerosis, comparing amantadine hydrochloride 100 mg twice a day and placebo. On amantadine 31% had marked improvement; 15.6% moderate improvement; 15.6% mild improvement; and 36.5%unchanged. On placebo, none noted marked improvement; one claimed moderate improvement on either amantadine or placebo. 18.7% reported mild improvement on placebo; and most of them had similar or more response to amantadine. No patient selected placebo over amantadine at the end of the trial. Overall improvement was seen in 62.5% of patients on amantadine and 21.8% on placebo. Additional experience up to two years suggests continued benefit but common and important side-effects.
The Carotid Siphon: A Scanning Electron Microscope Assessment of its Embolie Potential
Authors: Saunders F.W. , Shedden P. 
Published: July 2016

ABSTRACT:The role of the carotid siphon as a source of embolic material has had limited morphological or clinical study. The morphologic data available suggests that the siphon plays only a small role in embolic cerebrovascular disease. Clinical studies, however, suggest that it has a significant ischemic potential. To clarify this apparent discrepancy, we have designed a scanning electron microscope study of the carotid siphon. Eighty percent of the specimens from the carotid bifurcation and 30% of the siphon specimens showed evidence of damaged endothelium with attached red cell and platelet debris. The embolic potential of the siphon is estimated to be between ⅓ to ½ that of the carotid bifurcation. This supports the most recent clinical studies.
Fluctuation of Plasma Cortisol Levels in Multiple Sclerosis
Authors: Butterworth F. M. , Ristow G. E. , Collarini E. 
Published: July 2016

SummaryUsing radioimmunoassay, the levels of plasma cortisol of 23 patients with multiple sclerosis and 10 control subjects were determined at three or more times over a one-year period. The status of the disease was ascertained by neurological examination at the same time. Twelve of the patients were considered stable because they showed no changes in their disease status during the year. The remaining 11 patients were designated active since each had one or more relapses. The levels of cortisol of the control and stable groups remained relatively constant during the one-year period, but the levels of the active group fluctuated significantly more than either of the other two groups. Furthermore the active patients who had more than one episode had the greatest fluctuation in their cortisol levels. The fluctuations could indicate some endocrinologie al aberration in patients with active multiple sclerosis or may be secondary to the influence of the disease.
Brain Resuscitation
Authors: Ventureyra Enrique C.G. , Ivan Leslie P. 
Published: July 2016

All those related to the medical profession are familiar with the term “resuscitation” which usually means restoration of normal cardio-respira-tory activity in someone who has suffered from the effects of severely impaired functions of these two organs.The concept of “brain resuscitation” has emerged during the past few years and to the best of our knowledge the term was probably first coined by SAFAR (1977). This term is used more freely by anesthetists. Neuro-surgeons, when they relieve otherwise fatal herniations of the brain, are not in the habit of calling the effort resuscitation. Since, in our opinion, the term brain resuscitation focuses attention on a set of measures in an acute catastrophic situation, we would like to support its use.
Brain Lesions in Friedreich's Ataxia
Authors: Oppenheimer D.R. 
Published: July 2016

SummaryAn estimate is given of the frequency of pathological changes (assessed by cell populations and from myelin staining) in the brains of 15 cases of Friedreich's ataxia. Mention is made of the special problem of distinguishing primary degenerative lesions from those due to circulatory disturbances arising from the patients cardiac disease.
Taurine and Beta-Alanine Uptake in Cultured Human Skin Fibroblasts from Patients with Friedreich's Ataxia
Authors: Melancon S.B. , Grignon B. , Potier M. , Dallaire L. 
Published: July 2016

SummaryTaurine and ß-alanine uptake kineiics were studied in cultured skin fibroblasts from 9 patients with Friedreich's Ataxia and 8 controls. No significant difference was observed. The data support the presence of normal ß-amino acid carrier protein in Friedreich's Ataxia cell membrane.
Evaluation of Attributional Change in a Relapse Prevention Program for Child Molesters
Authors: Larsen Jillian , Hudson Stephen M. , Ward Tony 
Published: July 2016

Relapse prevention is a multimodal, cognitive-behavioural approach to treating, among other types of clients, child molesters. Rather than positing a cure as the outcome of treatment, it emphasises self-management and personal responsibility for avoiding or coping with situations that threaten self-control. The motivation to use these self-control strategies is likely to vary according to beliefs about the causes of their offending behaviour, particularly as a function of the degree to which they are seen as controllable. Fifteen child molesters, classified as preferential or situational type, and familial or nonfamilial, from the Kia Marama unit at Rolleston Prison reported on their causal beliefs concerning their offence-related behaviour at four points in their description of their most typical or recent relapse. This assessment was carried out at four points in the 35-week relapse-prevention based treatment program.All participants made clinically positive changes in their causal ascriptions over treatment. Preferential participants judged the cause of their offending to be less controllable, and more stable at the time of offence, and more global than situational offenders. Compared with non-familial participants, incestuous participants evaluated the cause of their offending to be less stable at the time of their offence and less global across all the assessment points. The results are discussed in terms of the utility of attributional assessment as an interim measure of progress, particularly with respect to motivation to avoid reoffending.
“Abuse” of Dietary Fibre in a Woman With Bulimia Nervosa
Authors: Bulik Cynthia M. 
Published: July 2016

In addition to vomiting, clinicians and researchers have outlined several means by which women with bulimia nervosa purge, including fasting, exercise, laxatives, emetics, diuretics, thyroid hormone, fluoxetine, sorbitol, enemas, heroin, and tobacco (Bulik, 1992; Mitchell, Hatsukami, Eckert, & Pyle, 1985; Pyle, Mitchell, & Eckert, 1981). The following report presents an additional method of purging, namely with excessive dietary fibre, which was particularly reinforcing as it satisfied the need to binge and purge simultaneously.Ms. A was a 27-year-old single white female who presented for outpatient treatment with a 1-year history of “out of control” binge-eating. She reported no prior history of any eating or weight disturbance. At the beginning of treatment she was 172.7 cm and weighed 64.5 kg (BMI = 21.6). She reported her desired weight to be 59 kg. The onset of her eating disorder was 1 year prior to treatment after moving, taking a new job, and beginning a relationship with a man whose diet consisted mainly of junk food.During the first few months of the relationship, she gained (4–4.5 kg) and became quite concerned. She was distraught at not fitting into her clothes and disliked her body at that weight. She began a very restrictive diet, losing 6 kg in 2 weeks. Following that 2-week period, she “lost control” and began to binge-eat. This pattern of alternating binge and fast periods and 5 kg weight fluctuations continued for 1 year. With every subsequent binge period, she reported feeling more out of control.
Feinberg on Rescue, Victims, and Rights
Authors: Conter David 
Published: July 2016

Quite by accident, Scarlett is in imminent danger of losing life or limb. You know this, and can rescue her without any unreasonable cost, inconvenience, or danger to yourself. Yet you stand by idly, and she suffers accordingly. Assume that morally speaking, you failed to do your duty; you ought to have done your best to rescue her. Should there be a criminal law penalizing your omission?
Joel Feinberg imposes the following two constraints — which may be called “liberal” — on any answer to our question. First, if it is reasonable that there should be a criminal law requiring rescue, then whenever there is a morally wrong omission to rescue, there must be someone who is victim of that omission. There must be no victimless crimes. Secondly, all criminal law must protect individual moral rights. When law protects people who need to be rescued and when others can rescue them at no unreasonable inconvenience, cost, or danger, the imperilled people must have a moral right to be rescued. If so, what precisely is the moral right that comes into play? Who are the victims? Joel Feinberg offers answers to these questions, answers intended to support the view that it is indeed reasonable for there to be a criminal law penalizing omissions to rescue, where rescue would involve no unreasonable cost, inconvenience, or danger to the potential rescuer.
Punishment, Feminism, and Political Identity: A Case Study in the Expressive Meaning of the Law
Authors: Hampton Jean 
Published: July 2016

In the Spring of 1995,1 was asked to testify as an expert witness in a case in Canada that raised a number of different philosophical and jurisprudential issues. The case concerned whether prisoners sentenced to two years or more in a Canadian penitentiary had the right to vote. For many years, Canada has denied those incarcerated in its prisons voting rights (following the British practice of doing so), but after the enactment of the Canadian Charter of Rights and Freedoms in 1982, which grants each citizen of Canada the right to vote, that practice was challenged; in a series of court cases, prisoners maintained that denying them the right to vote during their incarceration amounted to denying them one of their basic constitutional rights as Canadian citizens.One of the most important issues raised by this case was the nature of Canada’s political identity. The fact that the political identity of a state can be partly at stake in a law is, I believe, important and insufficiently recognized. A law can be not only a tool for the organization of the community (e.g., by promoting order, or coordination, or public wellbeing), but also a significant expressive force in that community, symbolizing the community’s sense of its values and (what I will call) its “political personality”. Indeed, for countries which are not culturally homogeneous and in which the unity of the community is primarily purchased through the principles of its polity, the expressive nature of certain laws can be essential in the creation, maintenance or revision of a unifying identity for that society; this is an identity that not only helps to hold the pluralist society together but also helps people to have a sense of themselves as members of that political community. I hope to argue that the controversy surrounding the issue of whether or not prisoners’ voting rights should be suspended reflects controversy about what kind of state Canada is and shows the ways in which law can be expressive.