bluuebunny/crossref_metadata_2025_split huggingface dataset:
Primary Malignant Tumours of the Trigeminal Nerve
Authors: Maroun F.B. , Sadler M. , Murray G.P. , Mangan M. , Mathieson G. , Jacob J.C. , Kwan A. 
Published: July 2016

ABSTRACT:We report a case of primary malignant schwannoma of the trigeminal nerve, and review the 5 previously reported cases. Clinical presentation, along with radiological and pathological features are discussed.
Pharmacological Modification of Bradykinin Induced Breakdown of the Blood-brain Barrier
Authors: Raymond Jennifer J. , Robertson David M. , Dinsdale Henry B. 
Published: July 2016

ABSTRACT:Internal carotid artery infusion of bradykinin caused extensive breakdown of the blood-brain barrier to protein as demonstrated by the extravasation of the marker, horseradish peroxidase, into vessel walls and the adjacent parenchyma. Pretreatment of the animals with indomethacin, trifluoperazine, or imidazole significantly reduced the quantity of abnormally permeable vessels as determined by light microscopy. By electron microscopy, it was determined that bradykinin caused an intense increase in the number of pinocytotic vesicles in the permeable segments, but no change in the interendothelial junctions. After imidazole pretreatment, although the extent of the permeability change was markedly reduced, the intensity of pinocytotic activity in the involved areas was not altered.
Cerebral Evoked Potentials in Multiple Sclerosis
Authors: Javidan Manouchehr , McLean Donald R. , Warren Kenneth G. 
Published: July 2016

ABSTRACT:Multimodal evoked potentials were analyzed from 58 possible, 62 probable and 100 definite (total 220) multiple sclerosis (MS) patients. Visual evoked potentials (VEP) were most frequently abnormal yielding 39%, 69%, 84% in the three diagnostic groups respectively. Median nerve sensory evoked potentials (SEP) yielded abnormalities in 26%, 65%, 79% respectively. Brainstem auditory evoked responses (BAER) were abnormal in 17%, 39%, 66% respectively. We measured the combined amplitude (CA) of waves III, IV, V in the BAER of these patients as an objective measure of amplitude asymmetry. The CA was considered abnormal if it was 1SD below the lowest CA value in the control group. The CA was abnormal in 9.2% of BAER with normal central conduction time. The BAER diagnostic yield in MS patients increased 11% by using CA analysis.
Cardiac Tachyarrhythmias in Hereditary Long QT Syndromes Presenting as a Seizure Disorder
Authors: Sundaram M.B.M. , McMeekin J.D. , Gulamhusein S. 
Published: July 2016

ABSTRACT:Patients with hereditary long QT disorders — Romano-Ward Syndrome and Jervell Lange-Nielsen Syndrome — sometimes present with seizures due to cardiac tachyarrhythmias. Two such patients are presented, emphasizing diagnostic clues — syncope, seizures with onset in early life, precipitation of attacks by emotional or physical stress, positive family history of sudden death and/or seizures, normal EEG and prolonged QT interval on ECG. Treatment is usually with adequate doses of sympathetic betablockers.
Effects of Extracellular Matrix Proteins on the Growth and Differentiation of an Anaplastic Glioma Cell Line
Authors: Rutka James T. 
Published: July 2016

ABSTRACT:Efforts to determine the factors responsible for reversing malignancy in the central nervous system may not only increase our understanding of the growth of primary human brain tumors, but may eventually prove to be of therapeutic benefit as well. We therefore devised a model system to study the effects of extracellular matrix (ECM) proteins on the malignant phenotype of an anaplastic glioma line, U-343 MG-A. Well-characterized cultures derived from normal human leptomeninges were grown to confluence and maintained for 2 weeks. The pia-arachnoid cells were then removed with detergent and base, leaving behind an ECM enriched in laminin, fibronectin, types I and IV collagen, and procollagen III. U-343 MG-A tumor cells planted on top of this normal ECM were profoundly growth inhibited, developed multiple slender cytoplastic processes similar to those of normal astrocytes, and expressed more GFAP per cell than did tumor cells growing on plastic alone. The growth of U-343 MG-A tumor cells in flasks coated with purified fibronectin or laminin was not significantly inhibited. However, U-343 MG-A cultures grown in flasks coated with type I or IV collagen showed decreased cellular proliferation and altered cell morphology. Conditioned medium from U-343 MG-A tumor cells growing on plastic alone contained a 64 kD activated metalloprotease. U-343 MG-A tumor cells growing on the pia-arachnoid ECM do not demonstrate such proteolytic activity. We conclude that the tumor cell microenvironment is extremely important in modulating the growth and differentiation of an anaplastic glioma cell line. It is hoped that an increased knowledge of the production of ECM components and their effects on malignant glioma cell growth, migration and differentiation will lead to the development of new approaches to improve the prospects of patients with primary malignant brain tumors.
Serial Recordings of Multimodality Evoked Potentials in Multiple Sclerosis:
A Four Year Follow-Up Study
Authors: Iragui Vicente J. , Wiederholt Wigbert C. , Romine John S. 
Published: July 2016

ABSTRACT:Pattern reversal visual, brain-stem auditory, and short latency median nerve somatosensory evoked potentials (EPs) were evaluated in a prospective study over 4 years in 20 patients with clinically definite sclerosis (MS). Standardized neurological examinations were done at regular intervals and correlated with EP findings. The highest incidence of EP abnormalities occurred in the visual system followed by the somatosensory and auditory systems. Clinical relapse was usually accompanied by EP deterioration, but clinical improvement often occurred without parallel EP recovery. EP changes were not always related to clinical symptoms and often took place during remission periods in the absence of clinical changes. There was no significant correlation between clinical and electrophysiological progression within any given sensory modality. The progression of clinical disability, however, showed a fairly good correlation with the overall progression of EP abnormalities. We conclude that EPs complement the neurological exam in the evaluation of MS and may have a place in the investigation of the effects of therapeutic agents on the neurological status in MS.
The Value of Prolonged Electroencephalographic and Video Monitoring in Diagnosis of Seizure Disorders
Authors: Willmer J.P. , Brunet D.G. 
Published: July 2016

ABSTRACT:Prolonged electroencephalographic and video monitoring of ictal events is useful in aiding the diagnosis of complicated seizure disorders. Forty eight patients admitted to a neurology service were assessed with this technique. Twenty five (54%) had their diagnoses changed, six (14%) had their diagnoses confirmed and the remaining patients had inconclusive studies. Therapy is modified by the results and in addition both patient and staff education is enhanced through the use of this equipment.
Intravenous Abuse of Propylhexedrine (Benzedrex®) and the Risk of Brainstem Dysfunction in Young Adults
Authors: Fornazzari Luis , Carlen Peter L. , Kapur Bhushan M. 
Published: July 2016

ABSTRACT:In 1949, amphetamine sulfate was replaced by propylhexedrine in the nasal decongestant agent
Benzedrex® because of psychosis, sudden death, and widespread abuse. Propylhexedrine is not without risks, and reported cases of psychosis, myocardial infarction, pulmonary vascular disease and pulmonary hypertension, and sudden death are well documented in the medical literature. We are reporting 2 cases of definite brainstem dysfunction and 5 cases of transient diplopia secondary to IV abuse of Benzedrex®. This widely abused drug is prepared by heating Benzedrex® and hydrochloric acid, and the resulting crystals are dissolved in water for injection. This agent is called “stove-top speed”. All 7 patients had transient diplopia, within seconds after injection. One patient had evidence of a right-internuclear opthalmoplegia, and another had a depressed right gag reflex and paralysis of the right half of the tongue. The deficits in these two patients, persisted for many months. In young adults with history of drug abuse, the IV use of Benzedrex® should be considered in the differential diagnosis of transient or permanent focal brainstem deficits.
The Significance of White Matter Lucencies on CT Scan in Relation to Cognitive Impairment
Authors: Steingart A. , Lau K. , Fox A. , Diaz F. , Fisman M. , Hachinski V. , Merskey H. ,   University of Western Ontario Dementia Study Group
Published: July 2016

Abstract:As part of a prospective clinicopathological study a cohort of “normal” elderly volunteers (n = 110) has been investigated with CT scans, psychometric testing (Extended Scale for Dementia) and neurological examination. CT scans were evaluated by a neuroradiologist for the presence or absence of white matter lucencies (WML). WML were defined as patchy or diffuse areas of decreased attenuation involving only white matter and with no change in adjacent ventricles or sulci.The 12 subjects with WML had lower scores on the ESD than the 98 subjects without WML (mean ESD with WML 229.5± 14; without WML 236.7±8.6, t-test p<.01) and the difference remains significant even after adjusting for the possible confounding effects of age (ANCOVA, P<.043).
Transmitter-replacement Therapy in Alzheimer's Disease Using Intracerebroventricular Infusions of Receptor Agonists
Authors: Gauthier S. , Leblanc R. , Quirion R. , Carlsson G. , Beaulieu M. , Bouchard R. , Dastoor D. , Ervin F. , Gauthier L. , Gauvin M. , Henry J. , Palmour R. , Robitaille Y. 
Published: July 2016

Abstract:Neurotransmitter replacement therapy in Alzheimer's Disease is currently being attempted using bethanechol chloride (Urecholine) infused intracerebroventricularly with an Infusaid continuous infusion pump. The rationale of this therapy is based on the severe cortical pre-synaptic cholinergic deficit in the presence of relatively normal post-synaptic muscarinic receptor density. Patients are selected on the basis of strict clinical criteria at a functional stage 4 or 5 of Reisberg. A cortical biopsy at the time of pump and catheter implantation confirms the diagnosis by histological and biochemical examination. Pre-operative, post-operative and serial mental status assessments combined with functional ADL assessments monitor changes in behavior. A 6 months double-blind treatment period is done in every patient, who is then free to continue if he has improved on active treatment. This specific study is part of a multi-centre trial. Other therapeutic trials using somatostatin analogs, such as Sandostatin, could then be done. The biological effects of the latter compound are being studied currently in adult Green Vervet monkeys, prior to its use in Alzheimer patients. Furthermore autoradiography of bethanechol and peptides labeled with 14C administered in these animals by intracerebroventricular infusion will allow a better knowledge of their pharmacological site of action.
Chromatin Structure in Scrapie and Alzheimer's Disease
Authors: McLachlan D.R. Crapper , Lukiw W.J. , Cho H.J. , Carp R.I. , Wisniewski H. 
Published: July 2016

Abstract:Scrapie affected brains exhibit a number of pathological features in common with the human neurodegenerative condition, Alzheimer's disease. The present report describes studies on chromatin structure seen in these two disease processes.Chromatin associated proteins influence transcriptional activity of DNA through an effect upon chromatin structure. We examined chromatin structure by: (1) measuring the capacity of the enzyme micrococcal nuclease to release mono- and dinucleosomes from isolated nuclei and (2) measuring DN A-histone interactions by examining the effect of ambient tonicity upon the release of chromatin proteins.In two strains of mice infected with two strains of scrapie agent there was reduced accessibility to micrococcal nuclease and an increased content on dinucleosomes of the histone HI and Hl° types. These changes precede clinical signs of scrapie and resemble those found in the human conditions of Alzheimer's and Pick's disease. Scrapie mouse brain differs from Alzheimer brain in that scrapie does not alter histone-DNA interactions as monitored by ionically induced histone release from chromatin. Despite similarities, the scrapie agent appears to operate upon different molecular mechanisms than those found in Alzheimer's disease.
Family Studies of Alzheimer's Dementia: Results and Prospects
Authors: Heston Leonard L. , Morris Marcia L. 
Published: July 2016

Abstract:Studies of families located through a proband with dementia of the Alzheimer type have demonstrated transmission of the disorder within families, probably through shared genes. Increasing closeness of genetic relationship and increasing severity of illness are both associated with increasing risk to relatives. Families a.t high risk are especially valuable for studies of the biology of dementing illness including, in particular, their molecular genetics. Associations at several levels between Down's syndrome and Alzheimer's dementia provide important clues for molecular hypotheses.
Challenges and Opportunities of Clinicopathological Investigation in Longitudinal Studies of Alzheimer's Disease
Authors: Ball M.J. 
Published: July 2016

Abstract:After organizational involvement in a clinicopathological investigation of Alzheimer's Disease for a decade, the present appears an appropriate time to reflect upon both the major challenges encountered as well as the exciting opportunities presented by such a longitudinal study.Problematic areas have included: (a) brevity of research grant intervals (generally one- or two-year); (b) turnover of support personnel, as a consequence; (c) limited biostatistical and data management expertise dedicated to the Study objectives; (d) limited neuropsychological manpower in this specialized sphere; (e) “distillate” effect of postmortem retrieval, by which only some of the many clinical cases expire during any grant period, only some of those receive autopsy permission, only some of those demonstrate (pure) Alzheimer's Disease neuropathologically, and only some are harvested quickly enough for specialized (e.g. biochemical) analyses; (f) ensuring the scientific optimization of available tissue samples; and (g) paucity of cases dying in the early stages of the illness.Significant achievements include: (a) demonstration of the opportunities for young researchers committed to careers in behavioral neurology, psychogeriatrics or neurodegenerative pathology; (b) development of improved testing protocols for psychometric, electroencephalographic and neuroradiological evaluation of the demented elderly; (c) ethical enrolment both of a large cohort of Alzheimer patients and a sizeable normative (control) population; (d) public cooperation permitting a postmortem compliance rate exceeding 75%; (e) rapid autopsy retrieval times (50% < 6 hours); (f) utilization of human postmortem synaptosomal preparations for neurochemical investigations; (g) availability of fresh autopsy tissues for other specialized techniques (e.g. magnetic resonance spectroscopy, in situ hybridization); and (h) a collegial forum for the regular exchange of scientific data.While the challenges to be met are certainly not unique to our Study, the interdisciplinary and longitudinal nature of this approach could magnify their potentially retardatory effect upon research quality. By contrast, however, surmounting these hurdles enables the participant scientists to share in an incomparable opportunity for observational insights into the cellular and pathogenetic mechanisms underlying the cognitive decline of Alzheimer patients. The vigour with which my numerous collaborators at the University of Western Ontario meet such challenges may serve as a model for other Alzheimer centres where a similar research system is likewise expected to justify the anticipation of its supportive funding agencies, and of the patients whom we are pledged to comfort.
Post-Mortem Neurochemical Changes in Alzheimer's Disease Compared With Normal Ageing
Authors: Rossor M. , Mountjoy C.Q. 
Published: July 2016

Abstract:Selective neuronal degeneration with concomitant changes in neurotransmitter systems are features of both normal ageing and Alzheimer's disease. There are, however, important neurochemical differences in cerebral cortex. Choline acetyltransferase declines with age in frontal cortex in contrast to the prominent change in the temporal cortex in Alzheimer's disease. The loss of somatostatin, and the recently reported reciprocal change in corticotropin-releasing factor and receptors are not seen with ageing. However, age itself may have an important influence on the neurochemical deficits in Alzheimer's disease which are restricted in the older patients. The problems of post-mortem studies in the analysis of ageing and possible approaches to this are discussed.
In Vitro31P NMR Spectroscopy Detects Altered Phospholipid Metabolism in Alzheimer's Disease
Authors: Miatto Orietta , Gonzalez R. Gilberto , Buonanno Ferdinando , Growdon John H. 
Published: July 2016

Abstract:In order to study possible metabolic derangements in Alzheimer's disease (AD), we performed phosphorus 31 nuclear magnetic resonance (31P NMR) spectroscopy on brain samples obtained at autopsy from 7 patients with AD and 9 control subjects. Aqueous solutions of brain tissue contained well-defined peaks of intermediate compounds in phospholipid metabolism, including the phosphomonoesters phosphocholine and phosphoethanolamine, and the phosphodiesters glycerophosphorylcholine and glycerophosphorylethanolamine. 3IP NMR spectra also displayed the inorganic phosphorus signal, which provides an index to the in vivo concentration of high-energy compounds.We found evidence for altered phospholipid metabolism in that relative levels of phosphomonoesters were decreased, and phosphodiesters increased, in frontal and parietal regions of patients with AD compared to control subjects. The inorganic phosphorus resonance peaks were similar in AD and control subjects, suggesting that energy stores are not diminished in AD. These preliminary data are consistent with the hypothesis that abnormalities in phospholipid metabolism contribute to possible neuronal membrane dysfunction and impaired cholinergic neurotransmission in AD.
Quantitative X-Ray Computed Tomography (CT) in Dementia of the Alzheimer Type (DAT)
Authors: Luxenberg J.S. , Friedland R.P. , Rapoport S.I. 
Published: July 2016

Abstract:Dementia of the Alzheimer type (DAT) has proven to be difficult to diagnose using computerized X-ray tomography (CT). To improve the identification of DAT with CT, several different quantitative approaches have been tried. Brain parenchymal density measurements and a variety of linear indices of ventricular size have failed to reliably separate DAT patients from age matched controls. Measures of ventricular volume improve discrimination, but overlap with controls persists. The inadequacy of a single CT study to diagnose DAT is clearly related to the overlap of brain atrophy in DAT and healthy aging, a finding which has also been noted in post-mortem studies. Estimating the rate of ventricular enlargement from quantitative measurements of ventricular size on successive CT scans may allow the physician to take advantage of the progressive nature of DAT, improving separation of DAT patients from healthy controls.
Physiology of the Basal Ganglia and Pathophysiology of Parkinson's Disease
Authors: Lee Robert G. 
Published: July 2016

ABSTRACT:This paper reviews current concepts concerning the anatomical organization and functions of the basal ganglia focusing mainly on the role of the basal ganglia in control of movement. Results of several studies which have been carried out on patients with Parkinson's disease are also reviewed and discussed as examples of altered normal physiological mechanisms.
Aging of the Nigrostriatal Pathway in Humans
Authors: Calne Donald B. , Peppard R.F. 
Published: July 2016

ABSTRACT:Progressive degeneration of functionally related groups of neurons occurs in certain infective, toxic, nutritional and genetically determined neurological diseases. It also takes place in normal aging, and several of the regions that undergo selective decay with the passage of time seem to be the same target regions that are afflicted in degenerative disorders such as Parkinson's disease, Alzheimer's disease and amyotrophic lateral sclerosis (ALS). Infective etiology is relatively easy to exclude by a combination of immunological tests and transfer experiments. Genetic causation can be rendered unlikely when large kindreds are available for study. Nutritional deprivation and acute or subacute toxicity are accessible to explanation by examining the environment. The most difficult mechanism of pathogenesis to refute is chronic toxic damage, where the lesion may derive from long-term exposure to a relatively widespread noxious agent or agents. Variations in involvement of individuals within a population may stem from differing capacities to activate or inactivate a toxin. Inherent in this concept of etiology is recognition that compensatory potential within the central nervous system may contribute to prolonged existence of subclinical lesions so that a latent period may exist for several decades, between causal event and the onset of symptoms. Furthermore, progressive clinical deterioration may take place even though the cause may have been transient, many years before. The histological features associated with Parkinson's disease, Alzheimer's disease and ALS may be nonspecific indicators of neuronal “illness”, there being a predilection for certain morphological markers to appear more frequently in particular circumstances and particular regions associated with the pathology of particular diseases.
The Use of the MPTP-Treated Mouse as an Animal Model of Parkinsonism
Authors: Heikkila Richard E. , Sonsalla Patricia K. 
Published: July 2016

ABSTRACT:The MPTP-treated mouse has proven to be a valuable model of parkinsonism. For example, C57 black mice treated with MPTP exhibit a large decrement in the neostriatal content of dopamine and its metabolites, a marked reduction in the capacity of neostriatal synaptosomal preparations to accumulate [3H]dopamine, a large decrease in neostriatal tyrosine hydroxylase activity, a marked loss of nerve cells in the zona compacta of the substantia nigra, and pronounced behavioral deficits. These biochemical, pathological and behavioral deficits are similarly observed in MPTP-treated primates and in humans with idiopathic parkinsonism. A great deal of our current knowledge concerning MPTP has come from experimentation carried out in the mouse.
Hypothesis: A Role for Quinolinic Acid in the Neuropathology of Glutaric Aciduria Type I
Authors: Heyes Melvyn P. 
Published: July 2016

ABSTRACT:Glutaric aciduria type I is an autosomal recessive metabolic disorder of children associated with severe dystonic motor disturbances and degeneration in the cerebral cortex, striatum and cerebellum. Biochemical studies demonstrate a deficiency in the enzyme glutaryl-CoA dehydrogenase. This enzyme metabolizes substrate derived from dietary tryptophan that could otherwise be converted to quinolinic acid within the brain. The law of mass action predicts that the production of quinolinic acid should be increased in glutaric aciduria type I. Quinolinic acid is a potent neurotoxin and convulsant when it is injected into the central nervous system of experimental animals. This paper argues that quinolinic acid may accumulate within the brain and cause the neuropathology of glutaric aciduria type I.