bluuebunny/crossref_metadata_2025_split huggingface dataset:
Current Status of Levodopa Therapy in Idiopathic Parkinson's Disease
Authors: Gauthier S. , Gauthier L. 
Published: July 2016

ABSTRACT:Levodopa is currently used at all stages of Parkinson's disease, particularly from Stage 3 onward. Most patients start levodopa within four years of the onset of disease, earlier in the akineto-rigid patients (average delay of 2.1 years) than in those where resting tremor predominates (average delay of 3.4 years). Advanced age (>80) is no deterrent to the use of levodopa if required. Wearing off is most noticeable in Stage 3 or after 10 years of therapy with levodopa. An inverse correlation was found between age and wearing off, suggesting a marked sensitivity of younger patients to levodopa. Future studies on the use of dopamine agonists should thus be stratified according to age.
Bromocriptine and the Clinical Spectrum of Parkinson's Disease
Authors: Riopelle Richard J. 
Published: July 2016

ABSTRACT:As the direct agonist with the widest clinical use, bromocriptine provides a unique window into the clinical spectrum of Parkinson's disease. The efficacy of bromocriptine for therapy of de novo Parkinson's disease has recently been confirmed using a double-blind design with L-Dopa (Sinemet). Over a period of 5.5 months, bromocriptine was found to be as effective as L-Dopa in reducing the functional and neurological disability of Parkinson's disease. This study complements others and demonstrates a role for bromocriptine as de novo therapy. A longitudinal study comparing bromocriptine with L-Dopa is underway, but previous observations with bromocriptine suggest modest, transient beneficial effects with significantly less fluctuation of disability and less dyskinesia when used alone or in combination with L-Dopa. The transient benefits of bromocriptine on progressive disability suggest that both pre-and post-synaptic defects are eventually involved in Parkinson's disease. While agonists with improved efficacy and minimal side effects are required for symptomatic treatment of Parkinson's disease, strategies to protect pre- and post-synaptic neuron populations against progressive dysfunction must be developed.
D-1 and D-2 Agonists in Parkinson's Disease
Authors: Lieberman Abraham N. , Goldstein Menek , Gopinathan Govindan , Neophytides Andreas 
Published: July 2016

ABSTRACT:We have evaluated 5 DA agonists-bromocriptine, lergotrile, lisuride, pergolide, and mesulergine in studies encompassing 278 patients with advanced PD. In most of our patients the DA agonist was added to levodopa. Most of our patients were no longer satisfactorily responding to levodopa. Previous attempts at managing these patients by changing the dose of levodopa (increasing or decreasing it), the treatment schedule, or the ratio of levodopa to carbidopa or by temporarily discontinuing levodopa [drug holiday] were unsuccessful. The majority of our patients had diurnal fluctuations in performance, either “wearing off” or “on-off” phenomena. The addition of a DA agonist resulted in a decrease in parkinsonian disability in most patients and a decrease in the severity of the diurnal fluctuations in performance. Improvement in many patients was maintained for at least 2 years. Adverse effects included mental changes, dyskinesias, orthostatic hypotension, and nausea. All of the adverse effects were reversible when the agonist was decreased or discontinued. As a group the agonists behaved similarly but individual patients often responded better to one agonist than another. The main role of agonists is in combination with levodopa in the treatment of patients with early PD who have not yet developed dyskinesias or diurnal fluctuations in performance.
Multiple Sclerosis: Assessment of Disease Progression and Effects of Treatment
Authors: Paty D.W. 
Published: July 2016

ABSTRACT:MS could well be a two stage disease. The first stage involves the sequential development of multiple small lesions, mostly inflammatory, that accumulate at a given rate. The second stage could be that of consolidation and confluence of lesions that involves not only demyelination but gliosis. MRI now gives us an opportunity to watch the evolution of these processes and also to monitor treatment effects. It is only after the evolution of this process is understood that we can design rational therapies directed toward the prevention of spasticity in MS.
Chorea
Authors: Klawans Harold L. 
Published: July 2016

ABSTRACT:A variety of neurotransmitters have been implicated in the pathophysiology of chorea as exemplified by Huntington's chorea. These include dopamine, serotonin, acetylcholine, GABA and a variety of neuropeptides including substance P and somatostatin. Despite biochemical data that suggests that alterations in other neurotransmitters may be of greater significance, pharmacologic data still supports a major role of dopamine in the actual clinical manifestation of chorea.
Progressive Supranuclear Palsy
Authors: Jankovic Joseph , Rohaidy Haydee 
Published: July 2016

ABSTRACT:Progressive supranuclear palsy (PSP) was first recognized as a distinct morbid entity by Richardson, Steele and Olszewski a quarter century ago. Subsequent experience has confirmed and extended their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigidity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is an important cause of parkinsonism. Its etiology remains obscure. Familial concentrations have not been observed.Some cases exhibit no oculomotor dysfunction. Dementia is usually mild. Recent neuropsychological studies have defined features consistent with frontal lobe cortical dysfunction. Seizures and paroxysmal EEG activity may occur.CT and MRI scans show midbrain atrophy early and later atrophy of the pontine and midbrain tegmentum and the frontal and temporal lobes. PET scans have shown frontal hypometabolism and loss of striatal D-2 dopamine receptors. Postmortem studies have documented involvment of both dopaminergic and cholinergic systems. Treatment remains palliative and unsatisfactory.
Paul Flechsig’s System of Myelogenetic Cortical Localization in the Light of Recent Research in Neuroanatomy and Neurophysiology Part I
Authors: Meyer Alfred 
Published: July 2016

SUMMARYIn the first part of the paper supplementary information on Flechsig’s personality and his attitude to work is given which had not been sufficiently appreciated in English biographical notices. This is followed by translated excerpts from “Gehirn and Seele”, published in 1896, in which Flechsig introduced his myelogenetic localization of the cerebral cortex.The second part is mainly a review of recent advances in neuroanatomical and neurophysiological research within those cortical regions in which Flechsig had been interested. A comparison between recent advances and Flechsig’s work demonstrates that Flechsig had definitive ideas on the solution of some of the outstanding problems but had been unable to resolve these with the techniques available at his time.
A Comparison of Visual, Brainstem Auditory, and Somatosensory Evoked Potentials in Multiple Sclerosis
Authors: Purves S.J. , Low M.D. , Galloway J. , Reeves B. 
Published: July 2016

SUMMARYMultimodality evoked potentials testing including PVEPs, SEPsand BAEPs was done in 112 patients who were known or suspected to have multiple sclerosis. The incidence of abnormal evoked potential findings in each of these systems was considered in patients in the different diagnostic categories of M.S. Results were also evaluated with respect to the presence of abnormal clinical visual, somatosensory, or brainstem signs. The PVEP was found to be the most frequently abnormal even in patients without clinical involvement in the visual system (45% of patients with definite, probable, or possible M.S.), the SEP was less frequently abnormal in the absence of clinical signs (35% in patients with M.S.), and the BA EP showed the lowest frequency of abnormalities in patients without brainstem signs (14% in patients with M.S.). Combining the three types of evoked potentials significantly increased the percentage of M. S. patients having abnormal findings, compared to any of these tests alone, with 97% of “definite” M.S. patients, 86% of “probable” M.S. patients and 63% of “possible” M. S. patients having at least one of these EP tests abnormal.
Polymyositis Presenting with Distal and Asymmetrical Weakness
Authors: Sundaram Mecheri B. , Ashenhurst Edward M. 
Published: July 2016

SUMMARY:A 52 year old female with polymyositis presenting with distal and asymmetrical weakness is presented. Similar reported cases are reviewed. These patients respond favorably to steroids. Increased awareness of this unusual distal polymyositis should avoid delay in diagnosis.
Spinal Cord Swelling in Multiple Sclerosis
Authors: Feasby Thomas E. , Paty Donald W. , Ebers George C. , Fox Allan J. 
Published: July 2016

SUMMARY:Three cases of suhacutely progressive spinal paraparesis had myélographie evidence of spinal cord swelling suggesling the diagnosis of intramedullary tumour. The diagnosis of multiple sclerosis (MS) was made in each case with the aid of CSF protein electrophoresis, evoked responses and CT scan. Because acute MS may produce cord swelling simulating tumor or syrinx, these additional investigations may he necessary to make a correct diagnosis.
Pasteurella Multocida Subdural Empyema: A Case Report
Authors: Khan M.I. , Chan R. 
Published: July 2016

SUMMARY:The first case of Pasteurella Multocida subdural empyema is reported. This was secondary to hematogenous spread from the pharynx to a chronic subdural hematoma. Positive cultures were obtained from the primary source, the blood and the subdural space. The patient was discharged with no neurological deficit following surgical drainage and parenteral antibiotics. The incidence, predisposing factors, clinical features, bacteriology, diagnostic procedures, treatment and mortality of subdural empyema are briefly discussed.
Abnormal Proximal Axons of Clarke’s Neurons In Sporadic Motor Neuron Disease
Authors: Averback P. , Crocker P. 
Published: July 2016

SUMMARY:Filamentous axonalmasses, similar to the abnormal enlargements found in the ventral horns, were found in Clarke’s nucleus in six cases of sporadic A LS. Clarke’s nucleus is generally thought to be normal in sporadic A LS, but the present findings, in conjunction with distal spinocerebellar degeneration which is often described, suggest that this system is also pathologically involved.
Viewpoint: What the Marine MolluscAplysiaCan Tell the Neurologist About Behavioral Neurophysiology
Authors: Ruben Peter , Goldberg Jeff , Edstrom Jon , Voshart Karen , Lukowiak Ken 
Published: July 2016

Only recently has man begun to regard himself as mundane and not divine. This conceptual liberation has allowed him to ask frank questions concerning the physical and chemical mechanisms which determine or affect his behavior. Unfortunately the answers to these questions have been slow in coming. The reasons for this are two-fold: Basic ethical considerations preclude the experiments necessary to investigate the neural substrates of human behavior in man. Further, man’s behavior and nervous system are both so enormously complex and subtle, it is therefore unlikely that much real fundamental knowledge could be gained from such experiments if performed. It is more expedient to study simple behavior in simpler organisms than man to understand how nervous systems operate in general and, it is hoped, to eventually gain a better understanding of the human in particular. This tactic is known as the “model systems” approach. By discovering the strategies adopted by less complex nervous systems to deal with simple situations one can devise a realistic model of the neural mechanisms that control more complex behavior in more advanced animals.Many animals have served as valuable sources of model systems. Among them the marine gastropod molluscAplysiahas received considerable attention. In comparison to the human nervous system with approximately 50 billion neurons, theAplysianervous system contains relatively few neurons — about 20,000. Furthermore the study of theAplysianervous system has several other advantageous characteristics. A number of forms of behavioral plasticity that are found in all higher metazoans including man are also found in theAplysia. These simple but non-trivial types of behavioral plasticity include habituation, sensitization and associative learning as well as easily defined qualities of neural function which we choose to call “behavioral states”. In addition the nervous system is composed of neurons which are large and, in many cases, easily identified by anatomical and physiological criteria so that the “same” cell can be studied in more than one animal under more than one set of experimental conditions. The cell bodies of the neurons inAplysia, from which electrical recordings can be fairly easily obtained, are electrically close to their dendrites so that changes in postsynaptic potentials occurring during modifications of behavior can be monitored.
An Electroretinal and Visual Evoked Potential Study in Friedreich’s Ataxia
Authors: Kirkham T. H. , Coupland S. G. 
Published: July 2016

SUMMARY:We made an electroretinographic (ERG) and visual evoked potential (VEP) study of 12 patients with Friedreich’s ataxia whose diagnosis was established using the Quebec diagnostic criteria. ERGs and VEPs were elicited to the same stimulating conditions. Flash evoked luminance changes and pattern-specific evoked potentials to check and diamond stimuli were used. Statistical analysis of the data was made using independent sample t-tests. Significant VEP delays were present under all test conditions. The presence of significant interocular and interhemispheric delays as well as evidence of abnormal temporal dispersion of the VEP response suggest there to be both diffuse anterior visual system disease and retrochiasmal involvement in Friedreich’s ataxia. The implicit times of the ERG b-waves were statistically within normal limits but the waveforms were of low amplitude and deformed and there were significant interocular implicit time differences. These ERG results suggest there are retinal conduction abnormalities in Friedreich’s ataxia which possibly play a role in the genesis of the abnormal VEPs.
Neonatal Myotubular Myopathy: Neuropathy and Failure of Postnatal Maturation of Fetal Muscle
Authors: Sarnat Harvey B. , Roth Sanford I. , Jimenez Jorge F. 
Published: July 2016

SUMMARY:The natural course of the pathologic features in striated muscle was studied in a full-term infant with myotubular myopathy. At 5 days of age a muscle biopsy revealed that more than 90 percent of muscle fibers fulfilled histologic, histochemical and electron microscopic criteria of fetal myotubes. The infant died unexpectedly at 9 months of age from spontaneous rupture of a multifocal cavernous hemangioma of the liver. Postmortem examination revealed that progressive maturation of the fetal muscle had not occurred postnatally, and more than 90 percent of myofibers were still apparent myotubes. This maturational arrest was generalized to all striated muscles. The only changes detected since the neonatal period were hypertrophy of the small population of large fibers, but with minor cytoarchitectural alterations, and loss of the incomplete histochemical differentiation with ATPase stains or dedifferentiation not attributed to postmortem diffusion. Involvement of the gubernaculum testis accounted for the undescended testicles. The brain and spinal cord appeared normal. Evidence of degenerating and regenerating axons in the sciatic nerve suggested that the etiology of this maturational arrest of fetal muscle may be neurogenic.
Stroke in Coronary Bypass Surgery
Authors: Martin W.R. Wayne , Hashimoto Stanley A. 
Published: July 2016

ABSTRACT:A retrospective review of 253 patients who underwent coronary bypass surgery was performed. Eight (3.2%) had suffered a stroke either intra-operatively (4) or during the early post-operative period (4). A comparison of pre-operative and intra-operative variables between the stroke group and the stroke-free group showed a significant difference only for the hemoglobin level (p < 0.01). All intraoperative strokes occurred in patients who had undergone femoral cannulation; it is suggested that this may have been a source of embolic material in these patients. An embolic origin for most of the strokes (both intra-operative and post-operative) is postulated on the basis of clinical and pathological findings.
Extramedullary Hematopoiesis Simulating Parasagittal Meningioma
Authors: Kandel R.A. , Pritzker K.P.H. , Gordon A.S. , Bilbao J.M. 
Published: July 2016

ABSTRACT:We report a patient with polycythemia vera and myeloid metaplasia of nine years duration who developed raised intracranial pressure related to a mass obliterating the sagittal fissure. Although clinically and radiologically simulating a meningioma, biopsy revealed extramedullary hematopoiesis involving the falx cerebri. Although uncommon, this case illustrates that myeloproliferative disease can present with symptomatology and radiologic features similar to primary intracranial tumours.
Scoliosis in Friedreich’s Ataxia
Authors: Allard P. , Dansereau J. , Thiry P.S. , Geoffroy G. , Raso J.V. , Duhaime M. 
Published: July 2016

SUMMARY:The preliminary results based on a three year study on the evolution and management of scoliosis in Friedreich’s ataxia are presented. Thirty-two patients were followed in the Neuromuscular Disease Clinic at Sainte-Justine Hospital where standardized spinal radiographs were taken periodically with the Scoliosis Chariot and the Throne.Pathomechanics — Between the age groups I (5 – 10 years) and II (10 – 15 years), a substantial increase in the Cobb values occurs. Associated with it, an increase was observed in the thoracic and thoraco-lumbar projected surface area indices. The relative torsion between the thoracic and lumbar segments was associated with a sudden increase in the Cobb measurements.A computer program which generates a tri-dimensional display of the spine from the coordinates obtained from the standardized radiographs has been adapted to study the importance and influence of the thoracic torsion on the progress of the scoliosis and on the evolution of the total spinal deformity.Management — Prevention of the progression of established curves was our main objective. Careful examination of the spine, depending on the age of the child, in our preliminary study, stimulated early orthopaedic treatment in any curve of 20° or more. There was always concern for curves of 30° or more. In the growing child, bracing which was recommended is now under a more thorough investigation. The use of spinal pacemakers is being clinically evaluated.In the older child, the curve was usually stable after sixteen years of age. Surgery was usually attempted in curves over 40° in the growing child. The same curve was usually stable after the growth period.For the non-ambulatory patients, the present study suggested the prescription of a molded seat with the following characteristics: i) a posterior lumbar support, ii) low thoracic lateral supports and Hi) a slight inclination of the seating system. This was presumed to be beneficial in maintaining stability of the spine.
Cushing’s Disease: 50 Years Later
Authors: Hardy Jules 
Published: July 2016

SUMMARY:The recent improvements in transsphenoidal microsurgery have once again placed the emphasis on the basophilic adenoma of the pituitary as the etiology of the disease originally described by Harvey Cushing fifty years ago.In 75 operated cases, tumors were found in 60 cases: 52 were microadenomas, 4 were larger adenomas and 4 were invasive. In 15 cases, no tumor was found at surgery; a selective partial central hypophysectomy was performed in 11 cases and a total hypophysectomy in 4. Among the latter, 3 additional microadenomas were found at pathology. The majority of the microadenomas were located in the center of the gland most often near or within the neural lobe.In 59 patients with a non invasive tumor, initial cure was obtained in 52 (88%); only 1 of the 4 patients with an invasive tumor was cured.In the 12 cases without surgical or pathological tumor, partial or total hypophysectomy resulted in 10 cures (83%).
Distribution of Dopamine in 35 Subregions of the Rat Caudate-Putamen: A High Performance Liquid Chromatography with Electrochemical Detection Analysis
Authors: Di Paolo Thérèse , Daigle Michel , Dupont André 
Published: July 2016

SUMMARY:Topographical variations in the endogenous content of dopamine were studied in the rat caudate-putamen. For this purpose, a high performance liquid chromatography with electrochemical detection procedure for the determination of norepinephrine, epinephrine and dopamine in nervous tissue samples is described. Tissue preparation required only homogenization in acidic solution and centrifugation prior to application to the chromatograph. Detection limits in the low picogram range were found for the catecholamines separated. This assay was used in combination with a micropunch dissection technique. The performance of this analytical technique is illustrated by the detailed mapping of dopamine content in 35 subregions of the rat caudate-putamen. The distribution of dopamine varies along the major axis of the nucleus.
Spino-Cerebello-Cerebral Degeneration with Amyloid Plaques (Gerstmann, Sträussler, Scheinker Syndrome)
Authors: Dolman Clarisse L. , Daly Lyle L. 
Published: July 2016

SUMMARY:We report the case of a man who died at age 40 after a 9 year illness resembling a hereditary spinocerebellar degeneration, but accompanied also by brain stem signs and dementia. Autopsy disclosed lesions of combined spinocerebellar and ponto-cerebellar degeneration, but also damage to many brain stem nuclei. Prominent amyloid plaques disseminated through the brain indicated a relationship to the Kuru-Creutzfeldt-Jakob group of diseases. Transmission experiments are underway at the National Institutes of Health.
The Neurology of Alice in Wonderland
Authors: Murray T.J. 
Published: July 2016

SUMMARY:Charles Dodgson, better known as Lewis Carroll, author of the famous Alice stories, developed migraine and associated visual symptoms late in life. There has been considerable speculation that the bizarre phenomena and weird visual imaginery in Alice stories was directly related to the author’s migraine.This paper reviews several aspects of the character and health of Lewis Carroll including his shy, introspective personality, his stuttering and his attraction to young girls. It is concluded that there is no connection between the visual symptoms of migraine and the phenomena described in the Alice stories which were written over 25 years before the author developed migraine in his mid-fifties.