bluuebunny/crossref_metadata_2025_split huggingface dataset:
PET, CT, and MRI Imaging of Neuronal Migration Anomalies in Epileptic Patients
Authors: Falconer John , Wada Juhn A. , Martin Wayne , Li David 
Published: July 2016

ABSTRACT:During the investigation of intractable epilepsy, neuronal migration anomalies [NMA] were discovered in three patients. The first patient had abnormally positioned gray matter within the walls of both lateral ventricles. The second patient had a post operative cystic area in the right parietal-occipital lobes and an area of NMA within the right temporal lobe. The third patient had abnormally thickened gray matter in the right operculum. Long term CCTV-EEG monitoring of these three patients revealed ictal discharges originating from the area of abnormal gray matter in patients 2 and 3. PET scanning showed the areas of NMA in all three patients to have similar metabolic activity to normal gray matter. These cases illustrate the value of various imaging modalities and suggest some interesting physiology of a spectrum of neuronal migration anomalies.
Cerebrospinal Fluid Pressure and Prolactin in Empty Sella Syndrome
Authors: Maira Giulio , Anile Carmelo , Marinis Laura De , Mancini Antonio , Barbarino Antonino 
Published: July 2016

ABSTRACT:In 58 female patients with the primary empty sella (PES) syndrome, a study of the CSF dynamics was done by evaluating both the absorptive reserve by a lumbar infusion test at constant rate, and/or the ICP increase occurring during REM phase of nocturnal physiological sleep. In 33, prolactin (PRL) dynamics were also investigated evaluating both the response to sequential stimulating test with thyrotropin-releasing hormone (TRH) and metoclopramide (MCP) and/or the circadian variation of PRL levels. Impairment of CSF dynamics was found in the 84% who had a hormonal pattern characterized by an increase of the PRL response to TRH and MCP and a decrease of the PRL circadian variation. Twenty-one patients with impaired CSF dynamics underwent CSF shunting procedures with disappearance of the signs of intracranial hypertension. They also had restoration of normal PRL dynamics but the endocrine alterations improved only moderately. Altered CSF dynamics play a role in the pathogenesis of the PES syndrome. A correlation between elevated ICP and the hypothalamo-hypophyseal control of PRL secretion may exist.
Regional Cerebral Glucose Metabolism in Turner Syndrome
Authors: Clark C. , Klonoff H. , Hayden M. 
Published: July 2016

ABSTRACT:Regional cerebral glucose metabolism was examined in females with Turner syndrome, a sex chromosome abnormality. Previous studies have found a visual/spatial cognitive anomaly in these women but, to date, no abnormalities in brain structure or function have been associated with the condition. In the present study, decreases in regional metabolism were found in the occipital and parietal cortex. The involvement of the occipital cortex, although consistent with the observed cognitive anomalies, has not been suggested previously as an area dysfunction. Because the occipital cortex is a primary sensory cortex, the reduction of glucose metabolism in the parietal cortex may reflect a lack of innervation from the occipital cortex. Besides insight into the functional specialization of the brain, these findings are also consistent with previous reports on animals regarding the effects of estrogen in brain maturation.
The High Incidence of Valproate Hepatotoxicity in Infants May Relate to Familial Metabolic Defects
Authors: Appleton R.E. , Farrell K. , Applegarth D.A. , Dimmick J.E. , Wong L.T.K. , Davidson A.G.F. 
Published: July 2016

ABSTRACT:The incidence of fatal hepatic failure associated with valproic acid (VPA) therapy is highest in children under the age of three years, particularly in those with developmental delay. The pathogenesis of VPA hepatotoxicity is unclear but may relate to the accumulation of a toxic metabolite of VPA which impairs fatty-acid oxidation. We describe two unrelated infants with developmental delay who developed hepatic failure while receiving VPA. Siblings of both children subsequently developed hepatic steatosis and intractable seizures without being exposed to VPA. This suggests that that the two children who developed liver failure when receiving VPA may have had a familial metabolic disorder. Familial metabolic disorders may account partly for the higher incidence of fatal hepatotoxicity described in infants receiving VPA.
The Value of CT Scans for Children with Headaches
Authors: Dooley J.M. , Camfield P.R. , O'Neill M. , Vohra A. 
Published: July 2016

ABSTRACT:We studied the value of CT scans for all children referred because of headache to one secondary and one tertiary pediatric centre during a 1 year period. Of 117 children who were seen by the Pediatric Neurology Service, at the I.W.K. Children's Hospital, 4 had CT scans and only 1 of these was abnormal. The consultant Pediatrician saw 40 children because of headache. CT scans were done on 3 of these patients and all were normal. None of the children who had a clinical assessment alone had unrecognized neurological disease during 20 months of follow-up. Therefore only 1 of 157 children had significant intracranial pathology. We conclude that CT scans have a limited role in the management of children with headache.
Clobazam as an Add-on Drug in the Treatment of Refractory Epilepsy of Childhood
Authors: Keene D.L. , Whiting S. , Humphreys P. 
Published: July 2016

ABSTRACT:We report the results of a double-blind cross-over study comparing clobazam and placebo in the treatment of refractory childhood epilepsy. Fifty-two percent of patients had greater than 50% reduction in their seizure frequency when taking the clobazam. During the placebo phase no patient recorded a greater than 50% reduction in seizure frequency. Sex, age, seizure type, intellect did not appear to differentiate clobazam responsive from nonresponsive patients. Only 2/21 patients had behavioral changes on the drug sufficiently severe to require the patient to drop out of the study prematurely. Drug interactions between clobazam and the other anticonvulsant medicines did not occur.
Hypervolemic-Hemodilution and Hypertension During Temporary Middle Cerebral Artery Occlusion in Rats: The Effect on Blood-Brain Barrier Permeability
Authors: Cole Daniel J. , Drummond John C. , Matsumura Jerry S. , Marcantonio Suzzanne , Chi-Lum Bonnie I. 
Published: July 2016

ABSTRACT:The effect of hypervolemic-hemodilution, with and without hypertension, on blood-brain barrier permeability was investigated in rats, after 180 minutes of middle cerebral artery occlusion (MCAo), and 60 minutes of reperfusion. One of the following conditions was maintained during MCAo: 1) Control — hematocrit and blood pressure were not manipulated; 2) Hypervolemic-Hemodilution/Normotension — the hematocrit was decreased to 30%; 3) Hypervolemic-Hemodilution/Hypertension — the hematocrit was decreased to 30% and mean arterial pressure increased by 30 mmHg with phenylphrine. In all groups, Evans Blue was administered, and its concentration determined by spectrophotometric assay. Evans Blue (μg{g-1 of brain tissue [mean ± SD]) was greater in the Hypervolemic- Hemodilution/Hypertension group (71 ± 20) versus the Control (13 ± 9) and Hypervolemic-Hemodilution/ Normotension (17 ± 10) groups (p < 0.05). No other differences were present. These results support the hypothesis that during MCAo, hypervolemic-hemodilution/hypertensive therapy effects an increase in blood-brain barrier permeability in the early period of reperfusion.
The Cycling Pool of Cells within Human Brain Tumors:In SituCytokinetics Using the Monoclonal Antibody Ki-67
Authors: Tsanaclis Ana Maria , Robert Françoise , Michaud Jean , Brem Steven 
Published: July 2016

ABSTRACT:Brain tumor growth results from the relative proportion of cells contained in three populations: a) cycling/proliferative; b) quiescent (Go)/static, and c) terminally differentiated/dying. The cycling compartment can be detected by the mouse monoclonal Ki-67 antibody, an available, rapid, safe, sensitive, and specific method for immunostaining of proliferative cells. We report the Ki-67 labeling index (LI) in 48 brain tumors. Malignant brain tumors have elevated Lis, ranging from 6.0% to 56.9%: anaplastic astrocytoma, 8.0 ± 7.3; glioblastoma multiforme, 10.1 ±4.2; germinoma, 11.7; medulloblastoma, 13.1 ± 6.6; metastases, 40.3 ± 13.1. By contrast, slow-growing tumors showed lower values (P < .001), approaching 1%: acoustic schwannoma, 0.4 ± 0.6; pituitary adenoma, 1.3 ± 1.9; meningioma, 1.2 ± 1.2; low-grade astrocytoma, < 1; pilocytic astrocytoma, 5.6. Human brain tumors can therefore be ranked according to the percentage of cycling cells with the acoustic schwannoma among the least proliferative and the metastatic carcinoma among the most proliferative. Within a given histotype, the Ki-67 LI may have prognostic and therapeutic implications for the individual patient. Already important for neuro-oncology research, the Ki-67 labeling index should be added to the armamentarium of the clinical neuropathologist to complement the standard histopathologic diagnosis with a cytokinetic analysis of cellular proliferation.
Familial Amyotrophic Lateral Sclerosis, 1850-1989: A Statistical Analysis of the World Literature
Authors: Strong M.J. , Hudson A.J. , Alvord W.G. 
Published: July 2016

ABSTRACT:We present clinical and pathologic data on four previously unreported familial ALS pedigrees and review and analyze by descriptive and exploratory statistical techniques all published cases of familial ALS (1850-1989). In contrast to the age-dependent incidence of sporadic ALS, the age of onset of familial ALS is normally distributed about a mean of 45.7 years (std. dev. 11.3 years). Survival curves for the familial ALS data also demonstrate a skewed distribution with a median survival time of 24 months with 74% surviving at 12 months, 48% at 24 months and 23% surviving at 60 months. The patient characteristics of age at onset of disease, sex and focus of disease onset are unrelated variables and age at onset of disease is the only predictor of survival (Cox's proportional hazard model, chi-square 14.74, p = 0.0001). By applying accelerated failure time models with a log-normal baseline distribution, estimated probabilities for survival adjusted by age at onset were calculated. It was found that the older the age at disease onset, the shorter the survival.
Single Photon Emission Computed Tomography Using 99mTc-HM-PAO in the Routine Evaluation of Alzheimer's Disease
Authors: Hurwitz T.A. , Ammann W. , Chu D. , Clark C. , Holden J. , Brownstone R. 
Published: July 2016

ABSTRACT:Regional cerebral blood flow was studied in 7 patients with clinically suspected Alzheimer's disease and 10 normal controls by single photon computed emission tomography (SPECT) using HM-PAO. All patients with Alzheimer's disease and no controls had parietal lobe hypoperfusion which was usually bilateral. In patients with more severe dementia hypoperfusion extended into the frontal lobes. Parietal lobe hypoperfusion corresponds to parietal lobe degeneration which is the one of the first neocortical regions to show the typical degenerative changes of Alzheimer's disease. SPECT with HM-PAO is a non-invasive investigation available in most nuclear medicine departments and complements existing tests in the routine evaluation of patients presenting with dementia.
Assessment of Cognitive Impairment: The Role of CT
Authors: Varga Marica , Wortzman George , Freedman Morris 
Published: July 2016

ABSTRACT:The value of CT as a routine screening procedure in the investigation of cognitive impairment is being increasingly challenged. To address this issue, we reviewed the records of 175 patients with intellectual deficits admitted to a Behavioural Neurology Unit over a two-year period. In the vast majority of cases, ie. 82%, the CT served essentially to confirm the clinical impression and added no new diagnostic information that impacted the management of the presenting problem. In 15% of cases the CT scan was helpful for diagnosis, especially in the differentiation between Alzheimer's disease and multi-infarct dementia.
The Effect of Tricyclic Antidepressants on Cortex- and Amygdala-Kindled Seizures in the Rat
Authors: Yacobi R. , Burnham W.M. 
Published: July 2016

ABSTRACT:The anticonvulsant effects of amitriptyline, imipramine, nortriptyline and desipramine were tested against focal and generalized seizures, triggered from either the amygdala or the cortex in fully kindled rats. Tests were administered on a 72- or a 24-hour schedule. Significant seizure suppression was achieved with only one drug, amitriptyline, and it occurred only at toxic or near-toxic doses. The differential, low-dose suppression of amygdala-kindled seizures, reported in earlier studies, was not seen in the present experiments. It may occur only in the short-interval test paradigms used by previous experimenters.
Synaptic Dysgenesis
Authors: Becker L.E. 
Published: July 2016

ABSTRACT:Synapse formation is a complex, incompletely understood process that has received only limited investigation in man despite the importance of synaptic dysfunction in common disorders such as epilepsy and mental retardation. This review explores synaptic differentiation, focussing on the morphologic maturation of synapses. Since differentiation depends on many antecedent developmental events, synaptogenesis can be affected by several factors: errors in neuronal proliferation, migration, and differentiation. The challenge to the neurobiologist is to detect and evaluate the minor alterations in neuronal differentiation that could account for the structural basis of the clinical manifestations. Trisomy 21 is an example of a condition in which the cytoarchitecture of the cerebral cortex is not obviously altered, yet mental retardation is consistently present; research neurobiologic techniques are making possible documentation of its structural basis. Epilepsy is another example in which examination of surgically removed cerebral cortex reveals subtle cortical dysplasias helpful in understanding the basis for the abnormal electrical discharge. Further exploration of synaptogenesis, particularly the influence of gene products and epigenetic factors on synapse maturation, will increase our understanding of the pathogenesis of conditions in which “morphology” seems normal but function is abnormal.
The Case for Conservative Management of Venous Angiomas
Authors: Kondziolka Douglas , Dempsey Peter K. , Lunsford L. Dade 
Published: July 2016

ABSTRACT:Venous angiomas (developmental venous anomalies) are vascular malformations increasingly recognized in general neurosurgical or neurological practice. They are associated with intracranial hemorrhage, seizures, or progressive neurological deficits or found as incidental findings in patients who present with headaches or have neuroimaging studies for investigation of unrelated neurological disorders. Since venous angiomas drain normal cerebral tissue within a functionally normal arterial territory, resection can lead to venous infarction. This report studies 27 patients with venous angiomas, all of whom had conservative treatment. The venous angioma was considered to be responsible for the onset of neurological symptoms in 14 patients (7 with hemorrhage, 3 with hemorrhage and seizures, 2 with seizures, one with an extrapyramidal movement disorder, and one with motor deficit). Thirteen patients had incidental lesions (8 with headache, and 5 with unrelated neurological symptoms). Ten venous angiomas were in the posterior fossa; seven in the cerebellum. Location did not correlate with symptomatic presentation. No patient with hemorrhage required surgical evacuation of the hematoma. No patient died or had significant morbidity during the follow-up interval (mean of 3.7 years). Venous angiomas are low flow, low resistance vascular malformations, many of which are not associated with neurological sequelae. Our series supports the concept that surgical removal or radiosurgical obliteration should not be performed unless a patient has a second life threatening hemorrhage.
Treatment of Heredo-Degenerative Ataxias with Amantadine Hydrochloride
Authors: Botez M.I. , Young Simon N. , Botez Thérèse , Pedraza Olga L. 
Published: July 2016

ABSTRACT:Amantadine hydrochloride (AH) was administered (200 mg/day) for more than three months to 17 patients with Friedreich's ataxia (FA) and to 12 patients with olivopontocerebellar atrophies (OPCA) in an open clinical trial. Reaction time (RT) and movement time (MT) with the right and left hand were measured before and after treatment. A striking improvement on both RT and MT was observed in the OPCA group (on seven out of eight measures), whereas in the FA patients improvement was seen only in two out of four MT measures with no improvement in RT. Both groups had low levels of homovanillic acid (HVA) in their cerebrospinal fluid before treatment, relative to their controls. However, improvement with AH was not related to HVA levels.
Modulation of Locomotor Patterns and Spasticity with Clonidine in Spinal Cord Injured Patients
Authors: Stewart J.E. , Barbeau H. , Gauthier S. 
Published: July 2016

ABSTRACT:This double blind cross-over study, involving 9 chronic spinal cord injured (SCI) patients (6 paraplegic and 3 paretic), was a first attempt to investigate the effects of the noradrenergic agonist, clonidine, on the modulation of the locomotor pattern and spasticity in patients with spinal cord lesions. Electromyographic (EMG), footswitch and video recordings were made as the patients walked on a treadmill with the support of an overhead harness if needed. Overground locomotion was also assessed in the paretic patients. All 3 spastic paretic patients had kinematic deviations and abnormal EMG recruitment profiles during the premedication or placebo sessions. With clonidine therapy one patient demonstrated a marked improvement in locomotor function. This patient progressed from non-ambulation to limited independent ambulation as the extent of coactivation in antogonist muscles decreased. The other 2 paretics who presented limited spasticity showed minimal changes while on clonidine. In the paraplegic patients, clonidine did not elicit locomotor activity, although there were marked reductions in stretch reactions and clonus during assisted locomotion. They remained incapable of locomotion, either during the control period or during the clonidine therapy. These results indicate that clonidine may be a potentially useful medication for both locomotion and certain manifestations of spasticity in SCI patients but further investigation is warranted.
H-Reflex Modulation During Walking in Spastic Paretic Subjects
Authors: Yang J.F. , Fung J. , Edamura M. , Blunt R. , Stein R.B. , Barbeau H. 
Published: July 2016

ABSTRACT:Hoffmann (H) reflexes were elicited from the soleus muscle during treadmill walking in 21 spastic paretic patients. The soleus and tibialis anterior muscles were reciprocally activated during walking in most patients, much like that observed in healthy individuals. The pattern of H-reflex modulation varied considerably between patients, from being relatively normal in some patients to a complete absence of modulation in others. The most common pattern observed was a lack of H-reflex modulation through the stance phase and slight depression of the reflex in the swing phase, considerably less modulation than that of normal subjects under comparable walking conditions. The high reflex amplitudes during periods of the step cycle such as early stance seems to be related to the stretch-induced large electromyogram bursts in the soleus in some subjects. The abnormally active reflexes appear to contribute to the clonus encountered during walking in these patients. In three patients who were able to walk for extended periods, the effect of stimulus intensity was examined. Two of these patients showed a greater degree of reflex modulation at lower stimulus intensities, suggesting that the lack of modulation observed at higher stimulus intensities is a result of saturation of the reflex loop. In six other patients, however, no reflex modulation could be demonstrated even at very low stimulus intensities.
Auricular Myoclonus
Authors: Kirk Andrew , Heilman Kenneth M. 
Published: July 2016

ABSTRACT:We describe a young man with a two and a half year history of idiopathic irregular contractions of an antitragicus muscle in the absence of a more generalized movement disorder. These contractions persisted in sleep and could not be replicated voluntarily. Because proximal nerve block temporarily eliminated the movements and complex hand movements reduced their amplitude and frequency, we suspect a central generator. However, these movements were not associated with any known pathologic condition.
New Biochemical Insights to Unravel the Pathogenesis of Alzheimer's Lesions
Authors: Roher Alex E. , Palmer Kenneth C. , Capodilupo John , Wakade Arun R. , Ball Melvyn J. 
Published: July 2016

ABSTRACT:Purification of amyloid plaque core proteins (APCP) from Alzheimer's disease brains to complete homogeneity and in high yield permitted its chemical fractionation and characterization of its components. APCP is mainly made of β-amyloid (βA) and an assortment of glycoproteins (accounting for 20%) rich in carbohydrates compatible with N-and O-linked saccharides. When added to tissue culture of sympathetic and sensory neurons APCP and βA inhibited neuritic sprouting, a reversible phenomenon at low doses. Higher concentrations of both substances kill the neurons in culture. APCP is significantly more toxic than βA, suggesting the minor components may play an important role in increasing the toxicity of βA. If the observed toxic effects of APCP in situ are occurring in vivo during the course of AD, then the accumulation of these extracellular proteins could be largely responsible for some of the neuronal death observed in this neuropathology.
Relationships Between Interictal Spiking and Seizures: Human and Experimental Evidence
Authors: Gotman Jean 
Published: July 2016

ABSTRACT:Epileptic seizures of focal origin often occur unpredictably as do interictal spikes. It is often assumed that spikes increase prior to seizures of focal origin and that antiepileptic medication affects spikes and seizures in a parallel fashion. We review evidence that this assumption is invalid and that there is a clear dissociation between spikes and seizures: increases in spiking before seizures have not been clearly documented; decreases in antiepileptic medication do not result directly in increased spiking; seizures are often followed by long-lasting increases in spiking; finally, seizures are no more likely when spikes are frequent than when spikes are rare. It therefore appears that spikes and seizures are two quite distinct phenomena, both originating in the epileptic focus but varying over time differently from what is most often believed.