bluuebunny/crossref_metadata_2025_split huggingface dataset:
Correlation of CT and MR with Impedance Monitoring and Histopathology in Stereotactic Biopsies
Authors: Gorecki J. , Dolan E.J. , Tasker R.R. , Kucharczyk W. 
Published: July 2016

ABSTRACT:Magnetic Resonance Imaging (MR) and Computer Assisted Tomography (CT) guided stereotaxis combined with intraoperative impedance monitoring and multiple sequential biopsies provides the opportunity to correlate the results of radiologic imaging with impedance and histopathology. The authors present the methods used and preliminary results obtained from 30 stereotactic biopsies with complete correlation in 12 cases. Impedance changes correlate accurately with lesion margins as defined by histology. CT images of enhancing lesions correlate quite closely to the histopathologic lesion margins whereas the appearance of the lesion on MR images is often larger than subsequently identified by either impedance or at pathologic examination. Impedance monitoring is a useful adjunct to stereotactic biopsy as it helps to accurately define lesion margins and can help direct the choice of biopsy sites.
Lack of Association Between Two Restriction Fragment Length Polymorphisms in the Genes for the Light and Heavy Neurofilament Proteins and Alzheimer's Disease
Authors: Lacoste-Royal Ginette , Mathieu Martine , Nalbantoglu Josephine , Julien Jean-Pierre , Gauthier Serge , Gauvreau Denis 
Published: July 2016

ABSTRACT:The etiology of Alzheimer disease (AD) remains unknown. The hypothesis of genetic factors playing a role in the causation of the disease, at least in its familial form, has been borne out by results showing linkage in several early-onset AD families to a locus on the proximal part of the long arm of chromosome 21. Linkage was not detected in several other families using the same markers. The metabolism of neurofilaments is perturbed in AD, as indicated by the presence of neurofilament epitopes in neurofibrillary tangles, as well as by the severe reduction of the expression of the gene for the light neurofilament subunit in AD brain. To detect a possible anomaly that might relate to the disease, we have searched for an association between the genes for the light subunit and the heavy subunit of the neurofilament triplet, and AD. Genotypes for restriction fragment length polymorphisms (RFLP) at each of the two loci were determined for an AD group and a control group. Allelic frequencies at a Taql-defined RFLP for the gene for the light neurofilament subunit were 0.70 for the 3.7 kb allele and 0.30 for the 2.9 kb allele. Hindi detected an RFLP for the heavy neurofilament subunit gene with frequencies of 0.31 for the 18.0 kb allele and 0.69 for the 6.8 kb allele. Frequencies were found to be similar in the two groups for both light and heavy neurofilament subunit loci. Although it cannot be excluded that mutations at other sites of the neurofilament genes are relevant to AD, the data reported here do not support an association between these genes and the disease.
Add-on Trial of Clobazam in Intractable Adult Epilepsy with Plasma Level Correlations
Authors: Guberman A. , Couture M. , Blaschuk K. , Sherwin A. 
Published: July 2016

ABSTRACT:Clobazam, a novel benzodiazepine, was used as an add-on agent in 47 adult patients with intractable epilepsy and a variety of seizure types. A >75% reduction in seizure frequency was observed in 42% (18) of the patients and a >50% reduction in 65% (29) of the patients which was sustained over a mean follow-up period of 13.3 (±5.6) months. Nine patients had to discontinue the drug due to minor adverse effects or increased seizures. Possible tolerance developed in 6 patients. Plasma levels of the active metabolite N-desmethylclobazam were linearly related to dosage and appeared to correlate with both therapeutic and toxic responses. Clobazam appears to be a safe and effective add-on antiepileptic for a wide variety of seizure types in intractable epilepsy.
Transfer Constants for Blood-Brain Barrier Permeation of the Neuroexcitatory Shellfish Toxin, Domoic Acid
Authors: Preston Edward , Hynie Ivo 
Published: July 2016

ABSTRACT:The cause of the toxic mussel poisoning episode in 1987 was traced to a plankton-produced excitotoxin, domoic acid. Experiments were undertaken to quantitate the degree to which blood-borne domoic acid can permeate the microvasculature to enter the brain. Pentobarbital-anesthetized, adult rats received an i.v. injection of 3H-domoic acid which was permitted to circulate for 3-60 min. Transfer constants (Ki) describing blood-to-brain diffusion of tracer were calculated from analysis of the relationship between brain vs plasma radioactivity with time. Mean values (mL.g-1.s-1 x 106) for permeation into 7 brain regions (n = 10 rats) ranged from 1.60 ± 0.13 (SE) to 1.86 ± 0.33 (cortex, ponsmedulla respectively), and carrier transport or regional selectivity in uptake were not evident. Nephrectomy prior to domoic acid injection resulted in the elevation of circulating plasma tracer level and brain uptake. The Ki values are comparable to those for other polar compounds such as sucrose, and indicate that the blood-brain barrier greatly limits the amount of toxin that enters the brain. Together with absorbed dosage, integrity of the cerebrovascular barrier and normal kidney function are important to the outcome of accidentally ingesting domoic acid.
Analgesic Effects of Vibration and Transcutaneous Electrical Nerve Stimulation Applied Separately and Simultaneously to Patients with Chronic Pain
Authors: Guieu Régis , Tardy-Gervet Marie-Françoise , Roll Jean-Pierre 
Published: July 2016

ABSTRACT:The analgesic effects of transcutaneous electrical nerve stimulation (TENS) and vibratory stimulation (VS), used both separately and simultaneously, were compared in 24 patients suffering from chronic pain. We tested the hypothesis that these combined procedures might improve the pain reducing effects obtained with a single type of stimulation, since they make it possible to recruit a larger number of large diameter afferents and/or to increase the discharge frequencies. Four 35-minute treatment sessions (VS, TENS, VS+TENS, Sham stimulation) were run with each patient. The vibrations (100 Hz) and TENS (100 Hz) were applied to the surface of the painful region. The sham stimulation treatment consisted of positioning the TENS electrodes without actually delivering any current. The short form of the McGill pain questionnaire was used to assess the subjects' pain levels. The assessments took place immediately after any treatment (Oh.), and again 4 hours and 24 hours later. The results showed that dual stimulation not only alleviated pain in more cases than either VS or TENS alone, but also had stronger and more long-lasting analgesic effects. On the other hand, all three types of stimulation used produced stronger analgesic effects than those obtained with the sham stimulation.
Graves' Thyrotoxicosis and Moyamoya Disease
Authors: Kushima Kenji , Satoh Yutaka , Ban Yoshio , Taniyama Matsuo , Ito Kunihiko , Sugita Koujiro 
Published: July 2016

ABSTRACT:Cerebrovascular accidents due to Moyamoya disease, a disorder characterized by arterial stenosis at the base of the brain accompanied by typical net-like collateral vessels, occurred in two young Japanese women with Graves' disease when they were in thyrotoxicosis. In one patient, a second attack of cerebral infarction occurred with the recurrence of thyrotoxicosis. Association of Moyamoya disease and Graves' thyrotoxicosis is rare and the pathegenetic relationship is discussed.
Spasmodic Torticollis — Review of 220 Patients
Authors: Rondot P. , Marchand M.P. , Dellatolas G. 
Published: July 2016

ABSTRACT:220 patients with isolated and idiopathic spasmodic torticollis were followed and treated over a 14 year period. Each patient was given a short questionnaire leading to the present retrospective data analysis. In most areas, including female preponderance and frequency of postural tremor, the findings confirmed previous studies and highlighted particular points: importance of psychopathological antecedents and association with stressful life-events. The discussion deals with some of the conflicting debates surrounding this unusual disorder. What is the role of psychopathological factors? What is the therapeutic prognosis? The best therapeutic results were obtained by combining anticholinergic drugs, local injections and rehabilitation.
Correlation between Cerebral Blood Flow, Somatosensory Evoked Potentials, CT Scan Grade and Neurological Grade in Patients with Subarachnoid Hemorrhage
Authors: Fazl M. , Houlden D.A. , Weaver K. 
Published: July 2016

ABSTRACT:Cerebral blood flow (CBF) and central conduction time (CCT) were recorded from 58 subarachnoid hemorrhage patients and from 49 age-matched controls. CBF was calculated following Xenon inhalation and CCT was determined from somatosensory evoked potentials (SSEP's) following median nerve stimulation. Each patient had a CT scan on the day of admission which was graded from I-IV. CBF, CCT and neurological grade (Hunt and Hess classification) were concomitantly recorded 1, 4, 7 and 14 days after subarachnoid hemorrhage. Mean CBF was highest in patients with neurological grades I and II (48.6 ± 12.3 and 48.1 ± 10.3 ml/lOOgm/min respectively) and lowest in patients with neurological grade IV (37.3 ± 9.6 ml/lOOgm/min). Patients in neurological grade I or II had mean CBF and CCT measurements that were significantly different from those obtained from patients in neurological grade IV (P < 0.05). Neurological grade and CT scan grade correlated with CBF (P < 0.0001) better than CCT (P = 0.015). Unexpectedly low CBF's from patients in neurological grades II and III (< 37 and < 31 ml/lOOgm/min respectively) failed to significantly prolong CCT suggesting CCT is unable to detect marginal ischemia. A significant correlation between CBF and CCT occurred only when CBF was < 30 ml/lOOgm/min (R = 0.75, P = 0.05). It appears that prolonged CCT is associated with a drop in CBF only when CBF drops below a certain threshold.
3-Nitropropionic Acid - Exogenous Animal Neurotoxin and Possible Human Striatal Toxin
Authors: Ludolph A.C. , He F. , Spencer P.S. , Hammerstad J. , Sabri M. 
Published: July 2016

ABSTRACT:3-Nitropropionic acid (3-NPA) — a suicide inhibitor of succinate dehydrogenase — is a widely distributed plant and fungal neurotoxin known to induce damage to basal ganglia, hippocampus, spinal tracts and peripheral nerves in animals. Recent reports from Northern China indicate that 3-NPA is also likely to be responsible for the development of putaminal necrosis with delayed dystonia in children after ingestion of mildewed sugar cane. This article discusses the role of 3-NPA in the causation of the disease in China, its neurotoxic effects in animals and the potential role for this compound as a probe of selective neuronal vulnerability.
Chronic Aluminum-Induced Motor Neuron Degeneration: Clinical, Neuropathological and Molecular Biological Aspects
Authors: Strong Michael J. , Garruto Ralph M. 
Published: July 2016

ABSTRACT:The monthly intracisternal inoculation of young adult New Zealand white rabbits with low-dose (100 Μg) aluminum chloride induces aggregates of phosphorylated neurofilament that mimics the intraneuronal inclusions of amyotrophic lateral sclerosis. The chronic progressive myelopathy and topographically-specific motor neuron degeneration that occurs in the absence of suppressions of neurofilament messenger RNA levels in this model contrasts with the acute fulminant encephalomyelopathy and nonspecific gene suppressions that occur subsequent to high-dose (1000 Μg) aluminum chloride inoculations. Further analysis of this unique model of chronic motor system degeneration can be expected to provide additional insights into the pathogenesis of amyotrophic lateral sclerosis.
Preoperative Electroencephalographic Investigation in Temporal Lobe Epilepsy: Extracranial and Intracranial Recordings
Authors: Gloor Pierre 
Published: July 2016

ABSTRACT:Preoperative EEG investigations of patients with temporal lobe seizures include extracranial interictal and ictal recordings during wakefulness and sleep, including long-term EEG and video-monitoring. Interictal epileptiform discharges when evaluated conservatively and in conjunction with other EEG and non-EEG localizing information, provide valuable guidance for the identification of the area to be resected, as do ictal recordings. When extracranial EEG features in conjunction with non-EEG data provide conflicting localizing information, intracranial recordings with stereotaxically implanted depth and epidural electrodes are used. Intracranial recordings must be designed to avoid biasing the exploration strategy in favor of one's preferred localizing hypothesis. Patients with evidence for bitemporal epileptogenic dysfunction in extracranial EEG recordings are suitable candidates for intracranial recordings. The majority of the patients explored in this manner show that all or more than 80% of their seizures arise from one temporal lobe. Excision of that lobe yields satisfactory results in a fair proportion of these patients. The number of satisfactory outcomes is, however, still somewhat less than in patients with unilateral temporal foci in extracranial EEG recordings.
Tailoring of Cortical Excisions for Frontal Lobe Epilepsy
Authors: Rasmussen Theodore 
Published: July 2016

ABSTRACT:Follow-up data of 2 to 49 years' duration are presented on 257 patients with cortical excisions for non-tumoral frontal lobe epilepsy. Twenty-six percent have become and remained seizure free. Another 30% have had a marked reduction of seizure tendency. Thus a total of 56% have had a long term complete or marked reduction of seizure tendency after the cortical resection. The cortical resections were classified into 5 regional categories. Forty-seven percent of patients with anterior frontal resections were in the seizure free category, as were 35% of patients with excisions limited to the convexity cortex and 32% of patients with maximal or near maximal frontal lobectomy. Only 18% of patients with frontal parasaggital excisions were in the seizure free category, and 10% of the patients who had excision of frontal cortex plus limited excision of adjacent central and/or temporal cortex.
Origin of Friedreich's Disease in Quebec
Authors: Barbeau A. , Sadibelouiz M. , Roy M. , Lemieux B. , Bouchard J.P. , Geoffroy G. 
Published: July 2016

AbstractWe have been able to trace 40 cases of classical Friedreich's disease from 14 previously unrelated French Canadian kindreds to one common ancestral couple arriving in New France in 1634: Jean Guyon and Mathurine Robin.One member of this couple presumably introduced one gene for Friedreich's disease into the French Canadian population. This gene has now been traced over 12 generations to both parents of the present cases. We plan to use this knowledge to study the spectrum of clinical manifestations of this gene and to carry out gene chromosomal localization studies, using the techniques of linkage and of molecular biology. Such studies in rare autosomal recessive disorders have previously been judged to be almost impossible.
A simple device to obtain reliable foot radiographs of ambulatory and non-ambulatory friedreich's ataxia patients
Authors: Billimoria M. , Allard P. , Sibille J. , Sirois J.P. , Duhaime M. , Geoffroy G. 
Published: July 2016

AbstractA simple radiographic apparatus standardizing foot position and simulating weight-bearing effects in the feet is described in this paper. It provides an effective means of obtaining reliable radiographic data for both ambulatory and nonambulatory patients. This device is presently used in an on-going study on pes cavus in Friedreich's ataxia.
Lysosomal enzymes in ataxia: discovery of two new cases of late onset hexosaminidase A and B deficiency (adult sandhoff disease) in French Canadians
Authors: Barbeau A. , Plasse L. , Cloutier T. , Paris S. , Roy M. 
Published: July 2016

AbstractWe have measured in leukocytes the following lysosomal enzymes in II Friedreich disease cases, 11 “atypical” recessive ataxias, 13 neurological controls and 16 normal controls: hexosaminidase A and B; (3-galactosidase and neuraminidase (labile and cold stable, or A and B). The lysosomal enzyme deficiencies known to produce certain forms of spinocerebellar degeneration were not present in Friedreich's disease or the Charievoix-Saguenay syndrome. The very small scale survey of “atypical” recessive ataxias revealed 3 cases of severe deficiencies in hexosaminidase activity. Two adult brothers presenting with the clinical phenotype of Kugelberg-Welander disease (one also with ataxia), were shown to have a severe deficiency of both HEX A and HEX B activity (Sandhoff biochemical pattern). This is the first such report. A further adult female patient, unrelated to the others, had a severe isolated deficiency of HEX B and presented with a very slowly progressive and mild ataxia with severe internal strabismus. These patients and their families are being studied clinically and biochemically in greater detail and will be reported elsewhere. However these preliminary findings justify screening for such lysosomal defects in all cases of “atypical” recessive ataxia.
Plasma Cholesteryl Sulfate in Friedreich's ataxia
Authors: Nestruck A.C. , Huang Y.S. , Eid K. , Dufour R. , Boulet L. , Barbeau A. , Davignon J. 
Published: July 2016

AbstractAlteration of membrane fluidity and anomalies of membrane structural proteins have been suspected in Friedreich's ataxia. Plasma lecithinxholesterol acyltransferase (LCAT) activity is also lowered in this disease, presumably because of a substrate effect. The membrane-stabilizing effect of cholesteryl sulfate (CS) and its inhibitory effect on LCAT activity prompted us to measure this substance in the plasma of Friedreich's ataxia patients as well as in normal subjects and in patients with Charlevoix-Saguenay disease. Plasma cholesteryl sulfate concentrations were significantly higher in Friedreich's ataxia, with levels above the upper limit of normal in nearly half of the cases. This increase was unrelated to age, sex or plasma cholesterol levels, but closely associated with the severity of the disease and thus considered to be secondary. A similar phenomenon (except the association with severity) was observed in Charlevoix-Saguenay ataxia. Levels also tended to be higher in first-degree relatives of Friedreich cases. The significance of these findings is discussed in the light of recent knowledge and experimental data obtained in this laboratory on rats made deficient in essential fatty acids. The highest concentrations of CS observed in Friedreich's ataxia (1097 µ-g/dL, 6 times the normal mean) was only 25% as high as the concentrations reported to inhibit LCAT activity.
The Brain of the Planarian as the Ancestor of the Human Brain
Authors: Sarnat Harvey B. , Netsky Martin G. 
Published: July 2016

ABSTRACT:The planarian is the simplest living animal having a body plan of bilateral symmetry and cephalization. The brain of these free-living flatworms is a biiobed structure with a cortex of nerve cells and a core of nerve fibres including some that decussate to form commissures. Special sensory input from chemoreceptors, photoreceptor cells of primitive eyes, and tactile receptors are integrated to provide motor responses of the entire body, and local reflexes. Many morphological, electrophysiological, and pharmacological features of planarian neurons, as well as synaptic organization, are reminiscent of the vertebrate brain. Multipolar neurons and dendritic spines are rare in higher invertebrates, but are found in the planarian. Several neurotransmitter substances identified in the human brain also occur in the planarian nervous system. The planarian evolved before the divergence of the phylogenetic line leading to vertebrates. This simple worm therefore is suggested as a living example of the early evolution of the vertebrate brain. An extraordinary plasticity and regenerative capacity, and sensitivity to neurotoxins, provide unique opportunities for studying the reorganization of the nervous system after injury. Study of this simple organism may also contribute to a better understanding of the evolution of the human nervous system.
The Nucleus Basalis of Meynert
Authors: Ezrin-Waters Cheryl , Resch L. 
Published: July 2016

ABSTRACT:The nucleus basalis of Meynert has been studied extensively in the recent literature. Interest in this nucleus has resulted from the discovery that it is a major source of cortical cholinergic input and that there is neuronal loss in the nucleus basalis in some dementing illnesses. Consistent and severe involvement of the nucleus basalis of Meynert has been found in Alzheimer's disease and in the dementia accompanying Parkinson's disease. Occasional involvement is present in other dementing illnesses, such as progressive supranuclear palsy, Parkinsonism-Dementia complex of Guam, dementia pugilistica, Pick's disease, Korsakoff's syndrome, Down's Syndrome and Creutzfeldt- Jakob disease. Huntington's disease spares this nucleus. However, the role of the nucleus in cognitive function is as yet undetermined. Even its alteration with normal aging remains controversial. This review details the pathological studies of this region to date, with particular emphasis on the dementias. Its role in the dementias of Alzheimer's disease and Parkinson's disease is specifically addressed.
Cytoplasmic RNA in Nervous System Tumours in Children: A Fluorochromic Histochemical Study using Acridine Orange
Authors: Sarnat Harvey B. , Curry Bernadette , Rewcastle N.B. , Trevenen Cynthia L. 
Published: July 2016

ABSTRACT:Acridine orange was used as a fluorochromic histochemical stain of nucleic acids, applied to 78 neoplasms of the central and peripheral nervous systems of 60 children. Some cases were compared with 5 adults and 4 other cases of chronic reactive gemistocytic gliosis. Opposite concentration gradients of cytoplasmic ribonucleic acid (RNA) was demonstrated in tumours of the neuronal/neuroectodermal series, and those of the glial/neuroepithelial series. Minimal AO-RNA fluorescence was seen in 8 cerebellar medulloblastomas and in a retinoblastoma; strong AO-RNA fluorescence occurred in one cerebellar medulloblastoma and in 3 primitive neuroectodemal tumours of the cerebral cortex. Intermediate intensity of fluorescence was found in neuroblastomas, and strong fluorescence was shown in well differentiated ganglioneuroma cells and in cells of chromaffin tumours. Among glial tumours, by contrast, the most anaplastic cells displayed the most RNA fluorescence, while better differentiated astrocytoma cells showed much less. Gradients also were found within some astrocytomas, corresponding to zones of relative anaplasia. Minimal or no fluorescence was detected in reactive gemistocytes or in oligodendroglioma cells. Ependymomas were weakly fluorescent and choroid plexus papillomas showed more fluorescence, similar to the findings in normal ependyma and choroid plexus. Several non-neuroepithelial tumours of the nervous system and Schwannomas also were studied. The acridine orange technique applied to either frozen or paraffin sections of nervous system tumours, has value as an adjunct in the diagnosis and grading of these neoplasms and perhaps in distinguishing reactive gliosis from benign astrocytoma.