bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Importance of D-1 and D-2 Receptors
Authors: Beaulieu Michèle 
Published: July 2016

ABSTRACT:The existence of subtypes of dopamine receptors as defined by Kebabian and Calne is now well accepted. The Dl receptor is typically associated with stimulation of adenylate cyclase, while the D2 receptor is either independent of adenylate cyclase or mediates its inhibition. Considerable interest has been generated by the potential physiological and clinical roles of these receptor subtypes. Availability of agonists and antagonists specifically acting at the D1 or D2 receptor site has stimulated research to characterize the functional effects of each receptor subtype. This might facilitate the development of effective compounds to control the signs and symptoms of Parkinson's disease and perhaps prevent the induction of debilitating side effects. Recent evidence indicates that Dl receptor stimulation is required to obtain full expression of the D2 receptor site, which has typically been associated with the clinical benefits of dopaminergic therapy. Both pre- and postsynaptic location of the receptors must also be taken into consideration as well as involvement of other neuronal systems. It appears that in PD, progressive involvement of the dopaminergic pathways is the principal pathological course, however, noradrenergic and serotonergic pathways are likewise involved. This multineuronal involvement suggests that drugs acting specifically at receptor sites, located on both pre- and postsynaptic neurons, might be required at different times during the course of the disease process to control its symptoms and/or the complications occurring after long-term treatment with a given drug.
Systemic Therapy of Dystonia
Authors: Fahn Stanley 
Published: July 2016

ABSTRACT:A number of pharmacologic agents have been found to be effective for the dystonias. Anticholinergic drugs have been shown to be the most effective in terms of percentage of subjects who receive moderate to marked benefit. About 50% of children and 40% of adults obtain such improvement. Peripheral adverse effects are usually overcome by pyridostigmine. It may be necessary to utilize pilocarpine eyedrops for blurred vision. Central adverse effects, such as forgetfulness, can be reduced only by a reduction in dosage of the anticholinergic. In comparing trihexyphenidyl and ethopropazine, we found that children tend to have better tolerance of the former and adults tend to have better tolerance of the latter. The antidopaminergics are the group of drugs that were found to be the next most effective agents in terms of percentage of patients who respond. However, these drugs, particularly the dopamine receptor blockers, have the capacity to induce tardive dyskinesia and tardive dystonia. Tardive syndromes are difficult to treat and can persist indefinitely. Other agents that have shown usefulness in controlling dystonia are levodopa, baclofen, carbamazepine, and the benzodiazepines, either alone or in combination with each other and with the anticholinergics. Stereotactic thalamotomy is particularly useful in contralateral hemidystonia. The risk of adverse effects is less than with bilateral thalamotomy, which may need to be employed when generalized dystonia is severely disabling and not responsive to pharmacotherapy.
Task Dependent Variations of Ocular Lateropulsion in Wallenberg’s Syndrome
Authors: Kirkham T.H. , Guitton D. , Gans M. 
Published: July 2016

SUMMARYThe eye movements of a patient with a left lateral medullary infarct (Wallenberg’s syndrome) were recorded using the scleral search coil in magnetic field technique. When asked to look at spontaneously appearing targets, saccades to the left were generally accurate hut those to the right reached the target by multiple step refixation saccades. Large amplitude rightward saccades were possible between two continuously visible targets or when making voluntary saccades in the dark.Vertical saccades, up or down, between spontaneously appearing targets were always associated with a leftward eye movement (lateropulsion). Voluntary vertical saccades between continuously visible targets showed that upward movements had left lateropulsion but downward movements were normal. Vertical voluntary saccades in the dark were oblique, upward saccades showing left lateropulsion and downward saccades showing rightward deviation. The aberrant horizontal components of vertical saccades had amplitude and velocity characteristics for components of normal oblique saccades. Possibly impaired assessment of vertically with incorrect eye position information produced by the infarct accounts for the lateropulsion of saccades in Wallenberg’s syndrome.
Effect of Therapy on Cerebral Blood Flow Following Aneurysm Surgery
Authors: Weir Bryce , Deleo Roberto , Menon Devidas 
Published: July 2016

SUMMARYThe authors report on a patient with a ruptured anterior communicating aneurysm treated surgically on the day following her subarachnoid hemorrhage (SAH). Repeated measurements of cerebral blood flow (rCBF) were carried out using the inhalational Xenon 133 method. Dramatic acute increases in rCBF occurred, coincident with clinical improvement, following mannitol, CSF drainage and dopamine. Later, further elevation to normal levels occurred following ventriculo-peritoneal shunting.
The Computerized Tomographic Assessment of Brain Infarcts
Authors: Tubman D.E. , Ethier R. , Melançon D. , Bélanger G. , Taylor S. 
Published: July 2016

SUMMARY:The computed tomographic (CT) examinations of one hundred and sixty patients with a clinical diagnosis of cerebral infarction were reviewed. A characteristic CT pattern was noted. Infused scans and follow-up studies allowed for considerable accuracy in diagnosis.
Neuropsychological Changes Following Carotid Endarterectomy
Authors: Bornstein Robert A. , Benoit Brien G. , Trites Ronald L. 
Published: July 2016

SUMMARY:Fifty-five subjects undergoing carotid endarterectomy were grouped according to side of operation, i.e. right, left and bilateral. These subjects were administered a comprehensive neuropsychological battery prior to and six months following surgery. The performance of these subjects was compared with a control group undergoing surgical procedures that did not in volve the brain or cerebral vasculature, and a second control group composed of patients with cerebrovascular symptoms who were not operated. It was found that as a group the endarterectomy subjects improved on a greater percentage of measures. When various subgroups were examined, it was found that the right operated stroke patients improved on significantly more measures than any other group. The difference between right and left stroke patients was significant, but there was no difference between right and left TIA patients in the extent of improvement. These findings were discussed in terms of possible underlying mechanisms.
The Effect of Plasmapheresis on Post-Thymectomy Ocular Dysfunction
Authors: Olanow C.W. , Roses A.D. , Fay J.W. 
Published: July 2016

SUMMARY:Five myasthenia gravis patients with post-thymectomy residual ocular signs were treated with plasmapheresis. Despite a significant reduction in AChR antibody titer, there was no clinical improvement. Subsequently, there was a dramatic response to prednisone. The AChR antibody titer did not correlate with the clinical state of the individual patient. It is suggested that plasmapheresis may operate by removing a thymic factor and that prednisone acts by a different mechanism.
Brain Stem Glioma in Childhood: Acute Hemiplegic Onset
Authors: Rothman S.J. , Olanow C.W. 
Published: July 2016

SUMMARY:Two children, age seven and 16 years, are described with the abrupt onset of a pure motor hemiplegia as the initial manifestation of a brain stem neoplasm. Subsequent rapid neurological deterioration localized the lesion to the brain stem and glioblastoma multiforme was diagnosed by surgical biopsy. It is suggested that brain stem gliomas with this unusual presentation are likely to be highly malignant and prone to rapid bulbar deterioration.
Life Effects of Narcolepsy in 180 Patients from North America, Asia and Europe Compared to Matched Controls
Authors: Broughton Roger , Ghanem Quais , Hishikawa Yasuo , Sugita Yoshiro , Nevsimalova Sonia , Roth Bedrich 
Published: July 2016

SUMMARY:A questionnaire survey has been made of the life effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, with 180 similarly distributed age and sex matched controls. Life-effects were attributed by the patients to the primary symptoms of excessive daytime drowsiness, sleep attacks, cataplexy, vivid hypnagogic hallucinations and sleep paralysis, and also to other frequent symptoms such as visual problems (blurring, diplopia) and memory impairment. Occupational problems were prevalent (over 75%) and included statistically significant deleterious effects upon performance, promotion, earning capacity, fear of or actual job loss and increased disability insurance. Driving was greatly affected and patients fell asleep at the wheel more frequently (66%), had near or actual accidents from drowsiness or falling asleep at the wheel (67%), and could experience cataplexy (29%) or sleep paralysis (12%) while driving. Work or home accidents attributed to sleepiness or sleep (49%) or related to smoking (49%) were much more common in patients. There were also deleterious effects on education, recreation and personality related to the disease. Narcolepsy can produce a variety of life-effects probably more serious and pervasive than, for instance, those of epilepsy, therefore emphasizing the importance of early diagnosis and treatment.
Ultrastructural Observations on Spinal Ganglion Biopsy in Friedreich’s Ataxia: A Preliminary Report
Authors: Lamarche J. , Luneau C. , Lemieux B. 
Published: July 2016

ABSTRACT:We report the preliminary study of a dorsal root ganglion obtained at biopsy during a surgical intervention in a patient with Friedreich’s ataxia. There was an apparent decrease in the number of large myelinated fibers without necrosis, but with numerous axonal swellings consisting mainly of dense accumulated neurofilaments. Large amounts of lipofuscin were also found as well as some onion bulb formations suggesting a process of axonal atrophy.
Oral Lecithin and Linoleic Acid in Friedreich’s Ataxia: I. Design of the Study, Material and Methods
Authors: Melancon S.B. , Dallaire L. , Potier M. , Vanasse M. , Marois P. , Geoffroy G. , Barbeau A. 
Published: July 2016

SUMMARY:A clinical and biochemical evaluation of twenty-two patients with Friedreich’s Ataxia and ten normal controls was undertaken in 1980 to assess the effect of lecithin and linoleic acid supplements on the course of the disease. The trial consisted of two consecutive six months periods on either supplements in a double-blind crossover fashion. Clinical appraisal was performed with regards to the following parameters: joints mobility, muscle strength, equilibrium, coordination, motor accuracy, speech and numerous day to day activities. Blood samples were obtained at the beginning and in the course of the trial for enzymatic determinations. This paper describes the methodology of the study.
Tissue Lipids in Acute Acrylamide Intoxicated Rats
Authors: Huang Y.S. , Wong P. , Blache D. , Barbeau A. , Davignon J. 
Published: July 2016

ABSTRACT:A preliminary survey of tissue lipid composition in acrylamide intoxicated rats is reported. The animals were injected intraperitoneally with acrylamide 50 mg/kg body weight per day for 10 days. Liver cholesterol, mainly in the ester fraction, was decreased in treated rats. When fatty acid composition of liver cholesterol esters was examined, the proportions of linoleate and stearate were found to be decreased and were compensated by the increase of palmitate. Atrophy of epididymal fat pad resulted in severe triglyceride depletion and a relative increase in the proportion of phospholipids and cholesterol. There was also a reduction of linoleate, palmitate and palmitoleate in triglycerides and phospholipids of this tissue. There were, however, only minor changes in the fatty acid profile of the sciatic nerve.
A Possible Genetic Pattern of Taurine Urinary Excretion in Friedreich’s Ataxia
Authors: Barbeau A. , Patenaude F. , Nadon G. , Charbonneau M. , Cloutier T. 
Published: July 2016

SUMMARY:The taurine urinary excretion pattern, before and after an oral load of 250 mg taurine, was studied in normal control subjects and in patients with typical Friedreich’s ataxia. It was demonstrated that in both situations the ataxic patients fell within the sub-types of “intermediate” and “high taurine excretors”, while none were “low taurine excretors”. It was also demonstrated that the excretion of taurine after a load in the obligate heterozygotes parents of the ataxic patients was intermediate between normal controls and patients. It is postulated that patients with Friedreich’s Ataxia lack normal regulation of the high affinity-low capacity uptake system for taurine (the TH system) in the brush border of kidney tubules. The low affinity-high capacity uptake system in the same membranes (the TL system) appears to be normal in Friedreich’s patients. The normal allele could be called THN and the variant THF and this trait would be inherited in an autosomal recessive fashion if it is linked to the Freidreich phenotype. Whether this finding is or is not the basic genetic defect in Friedreich’s Ataxia will require more studies to clarify, but it is of interest to note that a similar pattern appears to be present in the fibroblasts of these patients.
Pyruvate Dehydrogenase Activity in the Liver, Brain and Adipose-Tissue of Lipid-Deprived Developing Rats. Effect of Minute Amounts of Polyunsaturated Fatty Acids
Authors: Loriette C. , Launay M. , Lapous D. , Raulin J. 
Published: July 2016

ABSTRACT:The present experiment was carried out using the following diets:FF, fat-free, andLPthe same diet with 0.7% sunflower oil - given to the progeny of females kept on theFFdiet since the mating. After 10 mM Mg2+ activation of the PDH phosphatase, the rate of [1-14C] pyruvate decarboxylation into acetyl-CoA ester units was determined in the liver, brain and adipose-tissue of the pair-fed developing rats.Results: In the male progeny, pyruvate dehydrogenase (PDH) activity was higher (61%) in theLPgroup livers than in theFFgroup livers, at the end of the 13 week experiment. Such a difference was not observed in the two group brains up to the 91 days postweaning, but was even larger (94%) between adipose-tissues of theLPandFFgroups. In the female progeny kept 12 weeks on the diets, PDH activity in theLPgroup tissues was also higher than in theFFgroup tissues: 63% in the liver, 43% in adipose-tissues, and less than 10% in the brain. Therefore, a minute amount of lipids high in linoleic acid appeared to increase PDH activity, and especially in the liver and adipose-tissues of animals kept on a strictly fat-free diet. This stimulation of the PDH activity seems closely related to the phospholipid rehabilitation in the tissues (decrease in the trienoic: tetraenoic acid ratio values).
Current Views on Parkinson’s Disease
Authors: Calne Donald B. 
Published: July 2016

SUMMARY:Since the introduction of levodopa therapy, there have been major advances in the understanding and treatment of Parkinson’s disease. However, the problems and limitations associated with this form of treatment are now becoming increasingly apparent. This article reviews some of the current knowledge concerning the etiology and pathophysiology of Parkinson’s disease and discusses some-practical approaches to drug therapy.
Ventricular Dilation Following Intraventricular Hemorrhage in the Premature Infant
Authors: Hill Alan 
Published: July 2016

SUMMARY:Periventricular/intraventricular hemorrhage occurs commonly in the premature newborn. Recent studies indicate an incidence of 35–45%. Following PVH/IVH, the likelihood of developing hydrocephalus is related to the severity of the hemorrhagic lesion. Ventricular dilation may be due to an obliterative arachnoiditis affecting principally the posterior fossa or, less commonly, due to obstruction of flow of cerebrospinal fluid within the ventricular system by clots or other debris. With moderate to severe hemorrhagic lesions, ventricular dilation may occur at the time of PVH/IVH. More commonly, progressive dilation begins one to three weeks following PVH/IVH. The classical clinical signs of hydrocephalus, ie. bulging of anterior fontanelle and inappropriate increase in head circumference, may not appear for days to weeks following onset of ventricular dilation. The precise significance of such normal-pressure hydrocephalus in the genesis of brain injury in the newborn is unknown. Following diagnosis of PVH/IVH, close surveillance of ventricular size with ultrasound scans is indicated. When there is slowly progressive ventricular dilation with normal intracranial pressure, the choice of therapy is made difficult because of frequent spontaneous arrest in such cases. Several modes of therapy have been reported including drugs to decrease the formation of cerebrospinal fluid and the use of serial lumbar punctures. When ventricular dilation is rapid with intracranial hypertension, ventricular drainage is indicated.
The Pattern Electroretinogram in Optic Nerve Demyelination
Authors: Kirkham Trevor H. , Coupland Stuart G. 
Published: July 2016

SUMMARY:The pattern electroretinogram (PERG) is probably generated by the ganglion cell layer of the retina whose axons constitute the conductive fibres of the optic nerves. A study was undertaken of the PERG in a group of patients with clinically definite multiple sclerosis who had suffered optic nerve demyelination to assess the value of recording the PERG as a method for electrophysiological determination of optic nerve dysfunction. Pattern visual evoked potentials (PVEP) were simultaneously recorded, each eye being stimulated separately, and four different check sizes were used. Although individual patients showed PERG abnormalities the results as a group showed no significant difference from the normal population so far as latency or amplitude of the PERG was concerned. Thus we conclude that the PERG, while an interesting measurement, does not satisfactorily reflect optic nerve dysfunction. Further, our results indicate that it is not such a useful measure as the pattern visual evoked response for detecting optic nerve disease.
Hypothesis: Phylogenetic Diseases of the Nervous System
Authors: Sarnat Harvey B. , Netsky Martin G 
Published: July 2016

SUMMARYA few human diseases may be viewed from a phylogenetic perspective. Some metabolic or degenerative diseases selectively affect recently evolved or exclusively mammalian structures of the brain and spare the older structures. Examples include Krabbe’s leukodystrophy, olivopontocerebellar atrophy, Friedreich’s ataxia, Pick’s disease, and Leber’s optic atrophy. Some pathologic conditions in man are similar to normal anatomy in other species, although the mechanisms may differ. Congenital muscle fiber-type disproportion in rodents, Dandy-Walker cyst in birds, and agenesis of the corpus callosum in marsupials are representative of this category. Loss of basal dendritic spines from pyramidal cells in Pick’s disease is reminiscent of certain large neurons normally found in the cortex of reptiles. Changes in metabolism in the evolution of mammals in general and of man in particular may explain some aspects of’phylogenetic diseases’. Some potential examples are the shift from predominantly phospholipids to galactolipids in myelin composition as mammals evolved, and the greater toxicity of cyanide and other poisons of oxidative metabolism in mammals than in other vertebrates because of less reliance on anaerobic metabolism as an alternative energy source.
Serial Pattern Shift Visual Evoked Potentials in Multiple Sclerosis
Authors: Becker Werner J. , Richards Irene M. 
Published: July 2016

SUMMARYForty patients with MS initially tested in our laboratory were recalled for repeat PSVEP testing approximately two years later. Twelve normal controls were tested in a similar manner approximately two years apart. The PSVEP positive peak latency changed little in the 24 control eyes (mean 1.4 msec, range 0-6) over the study interval. Most MS patient eyes also showed little change in PSVEP latency over the two year study interval. Fifty-eight eyes changed 8 msec or less. Eighteen eyes showed a PSVEP latency increase of 10 msec or more. Six of these eighteen eyes were symptomatic (attack of clinical optic neuritis), twelve asymptomatic during the study interval. Symptomatic eyes tended to have greater latency increases during the study interval than asymptomatic eyes.Significant latency increases occurred with equal frequency in previously normal eyes (normal PSVEP on first test) and abnormal eyes (abnormal PSVEP on first test or previous clinical optic neuritis). Significant latency increases occurred with greater frequency in patients with a mixed or progressive course than in patients with a remitting-relapsing course, and in patients with greater disability ratings (Kurtzke 3-7) than in patients with lower disability ratings (Kurtzke 0-2). Bilateral latency increases occurred during the study interval more frequently than expected by chance. Patient age and disease duration did not significantly influence the number of PSVEP latency increases seen during the study interval.Four eyes decreased in latency by 10 msec or more during the study interval. All these eyes had had an episode of acute optic neuritis which began in the 5 weeks immediately preceding the 1st PSVEP test.In our MS patients, 13% of eyes per year developed latency increases of 10 msec or more. These may represent new demyelinating lesions. If so, one-third of these lesions were clincally symptomatic.
Retrograde Amnesia in Parkinson’s Disease
Authors: Freedman Morris , Rivoira Peter , Butters Nelson , Sax Daniel S. , Feldman Robert G. 
Published: July 2016

ABSTRACT:Retrograde amnesia was assessed in demented and non-demented Parkinson’s patients using a test of remote memory spanning the years from 1920-1979. Results indicated that the demented patients 1) scored significantly below normal controls and 2) had equal impairment for all time periods. This pattern was like that seen in other dementing illnesses (i.e., Huntington’s and Alzheimer’s diseases), but different from that in amnesic disorders, such as Korsakoff s syndrome. The data, therefore, suggest qualitative differences in pattern of remote memory loss between the dementias and amnesic syndromes.
The University of Toronto Head Injury Treatment Study: A Prospective, Randomized Comparison of Pentobarbital and Mannitol
Authors: Schwartz Michael L. , Tator Charles H. , Rowed David W. , Reid S. Ross , Meguro Kotoo , Andrews David F. 
Published: July 2016

ABSTRACTFifty-nine patients were treated in a prospective, randomized comparison of pentobarbital and mannitol for the control of intracranial hypertension resulting from head injury. Patients with elevated intracranial pressure (ICP) after evacuation of intracranial hematomas were randomized to one of two treatment groups; mannitol initially or pentobarbital initially, followed by the second drug as required by further elevation of ICP. Similarly, patients with raised ICP but without hematomas requiring evacuation were randomly assigned to two treatment groups in an identical paradigm.Those with ICP elevation and no hematoma treated with pentobarbital as initial therapy had a 77% mortality compared to a 41% mortality for those with mannitol as initial treatment. Patients with evacuated hematomas had mortalities of 40% and 43% (no significant difference) for pentobarbital and mannitol respectively. In both no-hematoma and hematoma streams pentobarbital was less effective than mannitol for control of raised ICP.Multivariate statistical analysis indicates that pentobarbital coma is not better than mannitol for the treatment of intracranial hypertension and may be harmful in no-hematoma patients with intracranial hypertension after head injury.