bluuebunny/crossref_metadata_2025_split huggingface dataset:
A follow‐up study of intractable seizures in childhood
Authors: Huttenlocher Peter R. , Hapke Ronald J. 
Published: January 2005

AbstractOne hundred forty‐five children with seizures that were refractory to medical therapy for at least 2 years were followed 5 to 20 years after onset. The majority of children with uncontrollable seizures (61%) were mentally retarded, and most of these (73%) had onset of seizures at younger than 2 years of age. Age of onset was significantly later (mean 5.0 ± 0.5 yr [SEM]) in the group of children with borderline to normal intelligence. Follow‐up data showed remission of seizures in a significant proportion of children with borderline or normal intelligence, with a linear decrease of the percentage with persistent seizures at a rate of about 4% per year. Remission of seizures was much less frequent (1.5%/yr) in the group with mental retardation. Seizure type had some effects on outcome. Children with focal atrophic brain lesions did no worse than those without definable pathology on brain‐imaging studies.
Extended levodopa release from a subcutaneously implanted polymer matrix in rats
Authors: Sabel B. A. , Dominiak P. , Häuser W. , During M. J. , Freese A. 
Published: January 2005

AbstractIt is well recognized that plasma fluctuations resulting from oral levodopa therapy may cause an unstable clinical response in parkinsonian patients. We have therefore developed a slow‐release polymer matrix system that can deliver levodopa continuously for extended periods of time (at least 225 days) after subcutaneous implantation in rats. Advantages of this approach include (1) the elimination of levodopa plasma fluctuations and (2) the possibility of reducing the required dose due to constant plasma levels and because the gastrointestinal tract is circumvented. The peripheral implantation of polymer systems containing levodopa, dopamine receptor agonists, or other anti‐Parkinson agents may constitute a novel technology of drug delivery to improve the care of patients with Parkinson's disease.
Developmental dyslexia in women: Neuropathological findings in three patients
Authors: Humphreys Peter , Kaufmann Walter E. , Galaburda Albert M. 
Published: January 2005

AbstractBrains from male cases with dyslexia show symmetry of the planum temporale and predominantly left‐sided cerebrocortical microdysgenesis. We now report on three women with dyslexia. In all brains, the planum temporale was again symmetrical. Also, in two of the brains, multiple foci of cerebrocortical glial scarring were present. In both women, many of the scars were myelinated, suggesting origination during late intrauterine or early postnatal life. In one, scars were mainly left perisylvian and involved portions of the vascular border zone of the temporal cortex. In the other, scars were more numerous and occurred in the border zone of the anterior, middle, and posterior cerebral arteries symmetrically. All three cases showed to a variable extent brain warts, molecular layer ectopias, and focal architectonic dysplasia identical to those seen in the male cases. Two women had primary brain neoplasms, an oligodendroglioma and a low‐grade astrocytoma, respectively, and two women showed small angiomas. Reexamination of previously reported male cases disclosed one with myelinated glial scars. Two control brains with asymmetrical plana temporale showed myelinated glial scars as well. The significance of the anatomical findings is discussed, and possible etiological factors are considered with known effects of autoimmune diseases on the nervous system.
Cerebral glucose hypermetabolism in Friedreich's ataxia detected with positron emission tomography
Authors: Gilman Sid , Junck Larry , Markel Dorene S. , Koeppe Robert A. , Kluin Karen J. 
Published: January 2005

AbstractLocal cerebral metabolic rate for glucose was studied with 18F‐2‐fluoro‐2‐deoxy‐D‐glucose and positron emission tomography (PET) in 22 patients with Friedreich's ataxia and 23 age‐matched normal control subjects. The diagnosis of Friedreich's ataxia was established by the history and physical findings and by excluding other diseases through laboratory investigations. PET studies revealed a statistically significant widespread increase of local cerebral metabolic rate for glucose in the brains of patients with Friedreich's ataxia who were still ambulatory, in comparison with normal control subjects. Nonambulatory patients with Friedrich's ataxia, in comparison with normal control subjects, had significantly increased local cerebral metabolic rates for glucose in the caudate and lenticular nuclei, but not in the other structures studied. The rate was significantly greater in ambulatory patients with Friedreich's ataxia than in nonambulatory patients in all structures studied except the caudate and lenticular nuclei. The data suggest that early in the course of Friedreich's ataxia, the local cerebral metabolic rate for glucose is increased extensively in the central nervous system, and as the disease progresses, it decreases in a regionally specific manner.
General assay for phosphoproteins in cerebrospinal fluid: A candidate market for paraneoplastic cerebellar degeneration
Authors: Gandy Samuel E. , Grebb Jack A. , Rosen Neal , Albert Katherine A. , Devinsky Orrin , Blumberg Hilary , Anderson Neil , Cedarbaum Jesse M. , Porter Roger J. , Sedvall Göran , Posner Jerome B. , Greengard Paul 
Published: January 2005

AbstractThe components of protein phosphorylation systems (protein kinases, protein phosphatases, and their phosphoprotein substrates) are highly enriched in neuronal cells compared with other cell types. We exploited this relative neuronal enrichment of protein phosphorylation system components to develop a general assay technique for putative protein kinase substrates (phosphoproteins) in human cerebrospinal fluid. Using this cerebrospinal fluid phosphoprotein assay, we have detected a putative protein kinase C substrate protein of apparent Mr 60 kd in 6 of 14 patients with paraneoplastic cerebellar degeneration but not in any of 55 patients with a variety of other neurological diseases. Phosphoproteins in cerebrospinal fluid may provide novel and unique markers for the diagnosis or staging of neuronal diseases as well as offer potential insights into the biochemical characterization of affected neuronal populations.
Nerve growth factor prevents toxic neuropathy in mice
Authors: Apfel Stuart C. , Lipton Richard B. , Arezzo Joseph C. , Kessler John A. 
Published: January 2005

AbstractTaxol is a promising new antitumor drug with therapeutic use that is limited by a toxic sensory neuropathy. Taxol is also cytotoxic to dorsal root ganglion neurons in vitro, but this effect is prevented by cotreatment with the trophic protein, nerve growth factor. We sought to develop an animal model and then to determine whether nerve growth factor can prevent taxol neuropathy in vivo. Administration of taxol to mice resulted in a profound sensory neuropathy characterized by decreases in dorsal root ganglion content of the peptide neurotransmitter, substance P, elevated threshold to thermally induced pain, and diminished amplitude of the compound action potential in the caudal nerve. Coadministration of nerve growth factor prevented all of these sings of neurotoxicity. These findings suggest that administration of nerve growth factor may prevent certain toxic sensory neuropathies.
Capillary prolactin measurement for diagnosis of seizures
Authors: Fisher Robert S. , Chan Daniel W. , Bare Mary , Lesser Ronald P. 
Published: January 2005

AbstractMeasurement of serum prolactin levels can be useful in the diagnosis of epilepsy, since prolactin levels often rise after seizures, but not after most imitators of epilepsy. Utility of the test is limited by the need to obtain blood 10 to 20 minutes after the episode. The present study documents the validity of prolactin measurements using capillary blood, which was obtained by the finger‐stick method after a possible seizure and then applied to filter paper. Venous and capillary prolactin levels were determined 10 to 20 minutes after seizure‐like episodes in 20 patients who were studied in an epilepsy monitoring unit. Venous and capillary prolactin values correlated, with a Pearson coefficient of 0.90. Using a criteria of any elevation above the laboratory upper limit of normal, capillary prolactin values correctly identified seizure versus pseudoseizure in 9 (100%) of 9 patients with generalized tonic‐clonic seizures, in 5 (71%) of 7 patients with complex partial seizures, and 4 (100%) of 4 patients with pseudoseizures. Prolactin values were unaffected by leaving filter paper samples at room temperature for up to 1 week. This study suggests the utility of diagnostic capillary blood collection kits to assist in the diagnosis of epilepsy in outpatients.
JC virus detection by in situ hybridization in brain tissue from elderly patients
Authors: Mori Mayumi , Kurata Hirokazu , Tajima Masako , Shimada Hiroyuki 
Published: January 2005

AbstractBrains from 10 patients aged 68 to 96 years at time of death were studied for JC viral DNA and common papovaviral capsid protein. In situ hybridization to JC viral DNA was performed by affinity cytochemistry using a biotinylated fragment of JC viral DNA. Immunohistochemistry was performed on brain tissue by the avidin DH‐biotinylated horseradish peroxidase technique using polyclonal antibody raised against the papovaviral capsid protein. Viral protein and DNA were detected in 4 of 10 patients. JC virus may be present in the brains of aged patients more frequently than previously suspected.
The clinical course of perimesencephalic nonaneurysmal subarachnoid hemorrhage
Authors: Rinkel Gabriel J. E. , Wijdicks Eelco F. M. , Vermeulen Marinus , Hasan Djo , Brouwers Paul J. A. M. , van Gijn Jan 
Published: January 2005

AbstractWe studied the early clinical course of 65 patients with perimesencephalic (nonaneurysmal) subarachnoid hemorrhage. None of the patients rebled; none had delayed cerebral ischemia; and only 3 patients (5%) developed clinical signs of acute hydrocephalus, 2 requiring ventricular shunting. Hyponatremia and electrocardiographic changes were found in the same proportions as in patients with aneurysmal rupture. All patients had a good outcome after 3 months. To control for the influence of the relatively small amount of cisternal blood in perimesencephalic hemorrhage on the absence of delayed cerebral ischemia, we compared these 65 patients with 49 patients who had aneurysmal subarachnoid hemorrhage. This control group with aneurysms was selected according to a similar amount of cisternal blood, a comparable level of consciousness on admission, and absence of other factors known to increase the risk for delayed cerebral ischemia. We found that 4 of the 49 patients with aneurysmal hemorrhage developed delayed cerebral ischemia (Fisher's exact test, p = 0.03); therefore the relatively small amount of blood does not account for the absence of delayed cerebral ischemia in perimesencephalic hemorrhage. Patients with a perimesencephalic pattern of hemorrhage and a normal angiogram should be considered to have a distinct subset of subarachnoid hemorrhage and should be excluded from future treatment trials of patients with subarachnoid hemorrhage.
Cold‐induced brain edema and infarction are reduced in transgenic mice overexpressing CuZn‐Superoxide dismutase
Authors: Chan P. H. , Yang G. Y. , Chen S. F. , Carlson E. , Epstein C. J. 
Published: January 2005

AbstractIt has been proposed that oxygen‐derived radicals, superoxide in particular, are involved in the alteration of blood‐brain barrier permeability and the pathogenesis of brain edema following trauma, ischemia, and reperfusion injury. Using transgenic mice that overexpress the human gene for copper‐zinc‐superoxide dismutase, we studied the role of superoxide radicals in the blood‐brain permeability changes, edema development, and delayed infarction resulting from cold‐trauma brain injury. At 2 hours after a 30‐second cold injury, cerebral water and Evans blue contents were reduced, respectively, from 80 ± 0.2% and 132.7 ± 12.9 m̈g/gm of dry weight for nontransgenic mice to 78.5 ± 0.3% and 87.1 ± 9.9% m̈g/gm of dry weight for transgenic mice. Infarction, as measured by 2,3,5‐triphenyltetrazolium chloride staining, was reduced by 52% in transgenic brains. These data indicate that an increased level of superoxide dismutase activity in the brain reduces the development of vasogenic brain edema and infarction. Superoxide radicals play an important role in the pathogenesis of these lesions in cold‐traumatized brain.
Altered excitatory and inhibitory amino acid receptor binding in hippocampus of patients with temporal lobe epilepsy
Authors: McDonald John W. , Garofalo Elizabeth A. , Hood Terry , Sackellares J. Chris , Gilman Sid , McKeever Paul E. , Troncoso Juan C. , Johnston Michael V. 
Published: January 2005

AbstractWe examined binding to excitatory amino acid and inhibitory amino acid receptors in frozen hippocampal sections prepared from surgical specimens resected from 8 individuals with medically refractory temporal lobe epilepsy. The excitatory receptors studied included N‐methyl‐D‐aspartate (NMDA), strychnine‐insensitive glycine, phencyclidine, and quisqualate. The inhibitory receptors studied were gamma‐aminobutyric acid type A (GABAA) and benzodiazepine. Excitatory and inhibitory amino acid receptor binding were differentially altered in the patients with temporal lobe epilepsy in comparison to 8 age‐comparable autopsy control subjects, and changes in receptor binding were regionally selective in four areas. Binding to phencyclidine receptors associated with the NMDA channel was reduced by 35 to 70% in all regions in the hippocampi of the patients. In contrast, binding to the NMDA recognition site and its associated glycine modulatory site was elevated by 20 to 110% in the cornu ammonis (CA) 1 area and dentate gyrus of the hippocampus of the patients. Binding to these sites was unaffected in area CA4. Binding to the quisqualate‐type excitatory amino acid receptor was unchanged in all regions except the stratum lacunosum moleculare CA1, where it was increased by 63%. GABAA and benzodiazepine receptor binding was reduced by 20 to 60% in CA1 and CA4, but unchanged in dentate gyrus. The data indicate that excitatory and inhibitory amino acid receptors are altered in the hippocampus of patients with temporal lobe epilepsy.
Medullary catecholaminergic neurons in the normal human brain and in Parkinson's disease
Authors: Saper Clifford B. , Sorrentino Denise M. , German Dwight C. , Lacalle Sonsoles De 
Published: January 2005

AbstractParkinson's disease is thought ot cause degeneration of melanin‐pigmented catecholaminergic neurons throughout the brainstem, but little quantitative information is available on the fate of catecholaminergic neurons associated with the dorsal vagal complex or medullary reticular formation. We therefore examined these neurons in the normal human medulla and in the brains of patients with Parkinson's disease, using both a melanin stain and immunohistochemical methods with an antiserum against tyrosine hydroxylase. The greatest numbers of catecholaminergic neurons in the ventrolateral reticular formation (A1/C1 group) were located in the far rostral medulla, whereas the largest populations of catecholaminergic cells in the dorsal vagal complex (A2/C2 group) were found at the level of the area postrema. No loss of cells was observed in the A1/C1 group in the parkinsonian brains. In contrast, the A2/C2 group showed moderate loss of neurons, most marked at the level of the area postrema. This difference was entirely due to the loss of neurons in the medial component of the A2 group, a population that normally is only lightly pigmented, while the heavily pigmented neurons in the ventral and intermediate components of the A2 complex were unaffected. Parkinson's disease causes degeneration only of selected populations of medullary catecholaminergic neurons, without apparent relationship to the extent of melanin pigmentation.
The European stroke prevention study (ESPS): Results by arterial distribution
Authors: Sivenius Juhani , Riekkinen Paavo J. , Smets Philippe , Laakso Markku , Lowenthal Armand 
Published: January 2005

AbstractThe European Stroke Prevention Study was a multicenter study comparing the effect of the combination of dipyridamole, 75 mg, and acetylsalicylic acid, 330 mg, three times a day, to that of placebo in 2,500 patients in the secondary prevention of stroke or death after one or more transient ischemic attacks, reversible ischemic neurological deficits, or strokes of antherothrombotic origin. The patients with vertebrobasilar events at entry comprised one‐third of the whole patient population. The overall total incidene of stroke or death (the end points) during the 2‐year follow‐up in the placebo group was lower in the vertebrobasilar group compared to the carotid group (14% versus 24%, respectively). The combination therapy of dipyridamole and acetylsalicylic acid caused a marked reduction in the incidence of stroke or death in patients with vertebrobasilar (51%) and carotid (30%) events. When only stroke was considered as the end point, dipyridamole and acetylsalicylic acid seemed to be more effective in reducing the risk of transient ischemic attacks than stroke, and more effective in men than in women.
Strongly succinate dehydrogenase–reactive blood vessels in muscles from patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes
Authors: Hasegawa Hitomi , Matsuoka Taro , Goto Yu‐ichi , Nonaka Ikuya 
Published: January 2005

AbstractIntramuscular blood vessels were examined with succinate dehydrogenase stain in skeletal muscle biopsy specimens from 6 patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes (MELAS). Almost all arteries had large granular deposits with high succinate dehydrogenase activity in their walls. Electron microscopic examination of serial frozen sections of these biopsies showed that the smooth muscle cells of the strongly succinate dehydrogenase–reactive blood vessels contained markedly proliferated mitochondria, characteristic of patients with MELAS. The presence of strongly succinate dehydrogenase–reactive blood vessels in muscle biopsy specimens provides an important clue toward understanding the underlying pathogenetic mechanism in patients with MELAS as well as another approach to the diagnosis of this disorder.
Focal capsular vascular lesions can selectively deafferent the prerolandic or the parietal cortex: Somatosensory evoked potentials evidence
Authors: Mauguière François , Desmedt John E. 
Published: January 2005

AbstractFour patients with a unilateral focal vascular accident involving the internal capsule (but not the cortex) were studied electrophysiologically. Averaged somatosensory evoked potentials (SEPs) to electrical stimulation of the median nerve on the left or the right side were analyzed. In the 3 patients with hemiparesis and normal somatic sensation, the precentral P22 and N30 SEP components were lost, whereas the parietal components were preserved. In another patient with clinical somatosensory loss unaccompanied by any central motor impairment, the precentral SEP components were preserved, whereas the parietal SEP components were lost. Thus, a small capsular lesion can eliminate distinct cortical SEP components by selectively involving either the axons of the thalamic VPLc nucleus going to parietal receiving cortex or the axons of thalamic VPLo going to motor area 4. These findings extend to subcortical lesions the diagnostic value of SEPS in patients with dissociated clinical motor and sensory signs.
The treatable dementia of sjögren's syndrome
Authors: Caselli Richard J. , Scheithauer Bernd W. , Bowles Carolyn A. , Trenerry Max R. , Meyer Fredric B. , Smigielski Jeffrey S. , Rodriguez Moses 
Published: January 2005

AbstractProgressive dementia developed during a 15‐month period in a 56‐year‐old woman with serologically and clinically documented primary Sjögren's syndrome. Findings from magnetic resonance imaging and angiography were normal, but a brain biopsy disclosed perivascular lymphocytic inflammation in leptomeningeal and parenchymal vessels. Treatment with high‐dose corticosteroids produced rapid and nearly complete resolution of the dementia.
Platelet‐activating factor in stroke and brain injury
Authors: Lindsberg Perttu J. , Hallenbeck John M. , Feuerstein Giora 
Published: January 2005

AbstractPlatelet‐activating factor, an endogenous phospholipid of proinflammatory, hemostatic, and vasoactive properties, is synthesized by neurons and in injured brain. Platelet‐activating factor is released together with eicosanoids such as thromboxane A2, prostacyclin, and leukotrienes. Its effects in neurons are mediated through a specific receptor coupled to phospholipase C and phosphoinositol metabolism. The cerebrovascular effects of platelet‐activating factor include disruption of the blood‐brain barrier, edema formation, and vasospasm. It has also been described to possess direct toxicity to neuronal cells in culture. Discovery and development of several highly potent and selective antagonists to platelet‐activating factor receptors facilitated experimental studies underscoring the role of this factor as an endogenous mediator in cerebral disorders, particularly cerebral ischemia and trauma. Significant biochemical, microvascular, functional, and behavioral recovery has been demonstrated using these antagonists in an array of experimental models of focal and global ischemia in the central nervous system (CNS). Clearly, studies of platelet‐activating factor in experimental models of CNS inchemia and reperfusion injury open a new perspective on phospholipid metabolism in stroke and offer an exceptionally promising therapeutic prospect. Data supporting this factor as a mediator of specific pathological sequelae in stroke and neuroinjury are surveyed in this review. We discuss the mechanisms and significance of platelet‐activating factor‐mediated effects and propose directions for future studies.
Control of cerebral circulation in the high‐risk Neonate
Authors: Pryds O. 
Published: January 2005

AbstractA knowledge of neonatal cerebrovascular physiology is essential to the understanding of diseases that frequently affect the subsequent development of the newborn brain. Recent observations indicate that the cerebral vessels of the healthy newborn infant, even the very preterm, respond to physiological stimuli in the same manner as in the mature organism. Thus, cerebral blood flow changes with changes in arterial carbon dioxide tension (Paco2), oxygen concentration (Cao2), or glucose concentration, whereas cerebral blood flow remains constant at minor fluctuations in arterial blood pressure. In pathological states, pressure autoregulation may become impaired, and in severe cases the vessels do not react to chemical or metabolic stimuli. These infants are at high risk for developing cerebral lesions, and they may be candidates for new „brain‐protecting regimens”︁.