bluuebunny/crossref_metadata_2025_split huggingface dataset:
Reduced phospholipase D activity in brain tissue samples from Alzheimer's disease patients
Authors: Kanfer Julian N. , Hattori Hiroshi , Orihel Danka 
Published: January 2005

AbstractBiochemical examinations of brain tissue samples obtained from patients with Alzheimer's disease have revealed a decreased quantity of the neurotransmitter acetylcholine and reduced activity of choline acetyl‐transferase (ChAT), the enzyme responsible for acetylcholine formation. It has been suggested that the choline moiety of lecithin, a substitute ubiquitously present in membranes of mammalian cells, could be mobilized for acetylcholine formation. The activity of phospholipase D, an enzyme which releases choline directly from lecithin, was measured in homogenates of Alzheimer brain tissue and found to be reduced by 63%. ChAT activity was reduced by 58% and 5′‐nucleotidase activity was reduced by 27% in these homogenates.
PET scan investigations of Huntington's disease: Cerebral metabolic correlates of neurological features and functional decline
Authors: Young Anne B. , Penney John B. , Starosta‐Rubinstein Simon , Markel Dorene S. , Berent Stanley , Giordani Bruno , Ehrenkaufer Richard , Jewett Douglas , Hichwa Richard 
Published: January 2005

AbstractFifteen drug‐free patients with early to midstage Huntington's disease were evaluated with quantitative neurological examinations, scales for functional capacity, computed tomographic (CT) scans, and positron emission tomographic (PET) scans of 18F‐2‐fluoro‐2‐deoxyglucose (18F‐FDG) uptake. All patients had abnormal indices of caudate metabolism on PET scanning, whereas in patients with early disease indices of putamen metabolism and CT measures of caudate atrophy were normal. Indices of caudate metabolism correlated highly with the patients' overall functional capacity (r = 0.906; p < 0.001) and bradykinesia/rigidity (r = −0.692; p < 0.01). Indices of putamen metabolism correlated highly with motor functions: Chorea (r = −0.841; p < 0.01), oculomotor abnormalities (r = −0.849; p < 0.01), and fine motor coordination (r = −0.866; p < 0.01). Indices of thalamic metabolism correlated positively with dystonia (r = 0.559; p < 0.05). The data suggest that PET scanning with 18F‐FDG is a sensitive measure of brain dysfunction in Huntington's disease and that basal ganglia metabolism is highly correlated with the overall functional capacity of individual patients and with the degree of their motor abnormalities.
Saccade responses to dopamine in human MPTP‐induced parkinsonism
Authors: Hotson J. R. , Langston E. B. , Langston J. W. 
Published: January 2005

AbstractDepletion of dopamine content in the substantia nigra resulting from 1‐methyl‐4‐phenyl‐1,2,5,6‐tetrahydropyridine (MPTP) toxicity produces parkinsonism. Management of 3 patients with MPTP‐induced parkinsonism required drug holidays during which there was a state of dopamine depletion followed by dopamine replacement. We used this opportunity to study the effect of the selective loss of pars compacta dopaminergic cells on vertical and horizontal saccade (fast) eye movements. During the drug holidays, visually guided saccades were hypometric and had long latencies but retained a normal saccade velocity–amplitude relationship. Dopamine agonists or precursors improved the accuracy and reaction times of saccades in all directions, but not their velocity. Two of the three patients also had intermittent blepharospasm during dopamine depletion. During the episodes of blepharospasm, saccade responses became slow eye movements. MPTP causes a dopaminergic‐responsive disorder of saccade initiation that is similar to idiopathic parkinsonism. The inhibition of voluntary eyelid opening during MPTP‐induced blepharospasm further increases this impairment of fast eye movements and altered saccade velocity, presumably via the pars reticulata of the substantia nigra.
Cholinergic muscarinic binding by human lymphocytes: Changes with aging, antagonist treatment, and senile dementia of the Alzheimer type
Authors: Rabey Jose M. , Shenkman Louis , Gilad Gad M. 
Published: January 2005

AbstractIn peripheral blood lymphocytes (mixed lymphocytes isolated on a Ficoll‐Hypaque density gradient) derived from normal human subject, cholinergic muscarinic binding capacity was found to increase with age. In contrast, lymphocytes derived from patients with “probable” senile dementia of the Alzheimer type (SDAT) exhibited a marked reduction in binding capacity. Treatment of these patients with antimuscarinic drugs was associated with increased muscarinic binding by lymphocytes. These results indicate that cholinergic muscarinic binding by peripheral blood lymphocytes may be useful in the study of alterations associated with aging and SDAT, as well as in evaluating changes induced by certain cholinergic drug treatments.
Spastic pure motor monoparesis
Authors: Ashizawa Tetsuo , Rolak Loren A. , Hines Marc 
Published: January 2005

AbstractPure motor hemiplegia (PMH) is, in most cases, caused by a lacunar infarction. However, pure motor monoparesis (PMM), i.e., isolated motor involvement with spasticity in one limb, has drawn little attention. We studied prospectively 5 patients with PMM and found that it was always due to a mass lesion in the contralateral superficial cerebral hemisphere. Our observation suggests that PMM should not be regarded as simply a variant of PMH.
Botulism in a patient with jejunoileal bypass
Authors: Freedman M. , Armstrong R. M. , Killian J. M. , Boland D. 
Published: January 2005

AbstractA 45‐year‐old woman was diagnosed as having the unclassified form of botulism. Her intestines may have been predisposed to colonization with Clostridium botulinum because of a jejunoileal bypass procedure that had been done several years earlier. One other similar case has been reported.
Acute herpetic and postherpetic neuralgia: Clinical review and current management
Authors: Portenoy Russell K. , Duma Christopher , Foley Kathleen M. 
Published: January 2005

AbstractThe pain of acute herpes zoster (HZ) may be severe, but it is usually transitory. A minority of patients, with the elderly at particular risk, go on to develop persistent, severe, often disabling pain called postherpetic neuralgia. Though the clinical features of these conditions are well known, the pathology of PHN is poorly described and the pathogenesis of the pain in both remains conjectural. During the past 60 years, an extraordinary number of pharmacological, anesthetic, and surgical therapies have been applied in an attempt to ameliorate the symptoms of acute herpes zoster, enhance its healing, prevent its transition to postherpetic neuralgia, and treat the pain of those with this complication. Relatively few treatments have been studied in a controlled manner, and fully reliable, safe, and effective therapeutic approaches for preventing and treating postherpetic neuralgia have not yet been found. This review summarizes current information on the epidemiology, clinical features, and pathology of herpes zoster and postherpetic neuralgia, and critically examines the accumulated experience with the various treatments. Guidelines for management are suggested.
Vasopressin in Alzheimer's disease: A study of postmortem brain concentrations
Authors: Mazurek Michael F. , Bed M. Flint , Bird Edward D. , Martin Joseph B. 
Published: January 2005

AbstractVasopressin (AVP) and its analogues are reported to improve learning‐ and memory‐related performance in experimental animals, and perhaps also in humans. Memory impairment is a clinical hallmark of the dementing disorder, Alzheimer's disease. We have examined AVP concentrations in postmortem brain tissue from 12 patients with histologically confirmed Alzheimer's disease and 13 control subjects. AVP was measured by a highly specific and sensitive radioimmunoassay, validated by parallel inhibition curves and high‐performance liquid chromatography. Alzheimer brains had either normal or slightly increased AVP levels in the neocortex, which does not have AVP cell bodies. Significant reductions in AVP content were found in the hippocampus, nucleus accumbens, and globus pallidus interna Levels were normal in all other regions studied. Abnormalities of the brain vasopressin system may contribute to the memory deficit associated with Alzheimer's disease.
Hemispheric brain damage in unilateral status epilepticus
Authors: Soffer Dov , Melamed Eldad , Assaf Yaakov , Cotev Shamay 
Published: January 2005

AbstractA 20‐year‐old woman with psychomotor seizures developed prolonged unilateral status epilepticus followed by coma, right hemiparesis, fever, liver failure, and eventually death. Neuropathological findings included severe neuronal ischemic cell change and massive brain edema, essentially restricted to the left cerebral hemisphere. Massive destruction of the hemisphere involved in epileptic activity with sparing of the contralateral hemisphere provides supportive evidence that in humans prolonged seizure activity can cause permanent brain damage.
Magnetic resonance imaging of the midbrain in Parkinson's disease
Authors: Duguid J. R. , De La Paz Robert , DeGroot Jack 
Published: January 2005

AbstractWe have analyzed magnetic resonance images of the midbrain in patients with Parkinson's disease, and have found that there is a narrowing of the signal from the pars compacta of the substantia nigra relative to controls. The nature of the histological changes that may be responsible for this effect is discussed. Magnetic resonance imaging has the potential of becoming a useful diagnostic tool in the management of parkinsonism.
Wernicke's encephalopathy in patients on peritoneal dialysis or hemodialysis
Authors: Jagadha Venkita , Deck John H. N. , Halliday William C. , Smyth H. S. 
Published: January 2005

AbstractAlthough the occurrence of Wernicke's encephalopathy (WE) in patients on dialysis is frequently alluded to, review of the literature reveals only 3 described cases. We describe 5 patients on dialysis who developed WE in the absence of alcoholism or other predisposing factors. The clinical diagnoses included uremic encephalopathy (2 patients), dysequilibrium syndrome (1), dialysis dementia (1), and brainstem hemorrhage (1). At postmortem examination, classic findings of WE were evident. The rarity of WE in patients on dialysis may in part be explained by studies indicating a genetic defect in transketolase activity. Patients on dialysis are also potentially at risk for thiamine deficiency because of anorexia, vomiting, and intravenous alimentation. Other factors altering thiamine requirements, such as glucose load or infections, may also contribute. Preventable and potentially curable, WE should be suspected in all patients on dialysis who have an unexplained neurological picture.
Beyond receptors: Multiple second‐messenger systems in brain
Authors: Worley Paul F. , Baraban Jay M. , Snyder Solomon H. 
Published: January 2005

AbstractSecond‐messenger systems play a major role in mediating neurotransmitter actions. In recent years our understanding of the organization and function of two prominent second‐messenger systems has progressed rapidly—the adenylate cyclase and phosphoinositide systems. Guanosine triphosphate–binding proteins, which are especially abundant in brain, couple transmitter receptors to the key second‐messenger generating enzymes in both of these systems. Whereas activation of adenylate cyclase produces a single intracellular messenger, cyclic AMP, stimulation of the phosphoinositide system generates at least two, inositol trisphosphate and diacylglycerol. Inositol trisphosphate mobilizes calcium from intracellular stores, and diacylglycerol, like cyclic adenosine monophosphate, activates a phosphorylating enzyme, protein kinase C. These second‐messenger systems are particularly enriched in the brain where they modulate many aspects of synaptic transmission.
Reinnervation of the hippocampal perforant pathway zone in Alzheimer's disease
Authors: Hyman B. T. , Kromer L. J. , van Hoesen G. W. 
Published: January 2005

AbstractThe perforant pathway originates from the entorhinal cortex of the anterior parahippocampal gyrus and terminates on the outer dendritic branches of the granule cells of the dentate gyrus and pyramidal cells of the subiculum and hippocampus. It carries the principal cortical input to the hippocampal formation. Destruction of the perforant pathway in experimental animals leads to a partial deafferentation of its target neurons, followed by a robust sprouting of acetylcholinesterase (AChE) terminals in the deafferented perforant pathway zone. In Alzheimer's disease, the cells of origin of the perforant pathway are laden with neurofibrillary tangles. AChE staining in the terminal zone of the perforant pathway in Alzheimer's disease shows several distinct patterns that are not found in control brains. These changes are consistent with the results of experimental studies demonstrating reinnervation in laboratory mammals, including nonhuman primates. The results suggest that in Alzheimer's disease sprouting of AChE‐containing systems occurs in the hippocampal formation in response to disease‐related cellular damage in the entorhinal cortex.
Class II antigen expression and inflammatory cells in the guillain‐barré syndrome
Authors: Pollard J. D. , Baverstock Julia , McLeod J. G. 
Published: January 2005

AbstractMonoclonal antibodies to T‐lymphocyte subsets and Class II molecules (Ia) have been used to characterize the inflammatory infiltrate in nerve tissue biopsied from 2 patients in the acute phase of Guillain‐Barré syndrome; the findings were compared with those in control nerve specimens. Normal control nerve was treated in the same way. In normal nerves, Class II molecules are expressed on endothelial cells and on occasional mononuclear and perineurial cells. In Guillain‐Barré nerves the inflammatory infiltrate consisted mainly of Class II–positive cells of the monocyte– macrophage lineage and of lesser numbers of T4 and T8 lymphoid cells. T4 cells predominated in perivascular collections. In the more severely affected patient, there was a marked increase of Class II molecules expressed on endothelial cells, perineurial cells, and most Schwann cells. Schwann cells associated with unmyelinated fibers and the Schwann cell processes of denervated Büngner bands all expressed Class II molecules. These histological changes were less marked in the more mildly affected patient. It is suggested that the expression of Class II molecules on the myelin forming cell, the Schwann cell, has important implications for the pathogenesis of the demyelination that occurs in Guillain‐Barré syndrome.
Hemophilus influenzae meningitis in the rat: Behavioral, electrophysiological, and biochemical consequences
Authors: Konkol Richard J. , Chapman Louisa , Breese George R. , Collier Albert M. , Kilts Clinton , Finley Charles , Vogel Richard R. , Mailman Richard B. , Bendeich Elizabeth G. 
Published: January 2005

AbstractHemophilus influenzae is the most common cause of bacterial meningitis in children, and a high percentage of survivors are at risk for long‐term sequelae. To explore the mechanisms responsible for these sequelae, a neonatal rat model was used to define the behavioral, electrophysiological, and biochemical changes following meningitis. Three days after inoculation of 6‐day‐old rats with a minimum of 1 × 107 colony‐forming units of a virulent Hemophilus influenzae, type b, cerebrospinal fluid and blood were cultured to confirm the presence of meningitis and bacteremia, respectively. At this time, forebrain norepinephrine and dopamine levels were significantly elevated in meningitic rats when standardized on a wet‐weight basis. No changes in brain serotonin or heart norepinephrine levels could be found in the 9‐day‐old rats. No residual changes were found in steady‐state concentrations of norepinephrine or dopamine in surviving adult rats. However, survivors that had had meningitis as neonates showed significant impairment in active and passive avoidance learning tasks and demonstrated a significantly higher level of activity during a habituation period in circular photocell activity cages. No change in the flinch‐jump threshold was detected. Brainstem auditory evoked potentials showed delays of various waves in 3 of 10 Hemophilus influenzae type b–treated adult rats tested. These rats also exhibited markedly augmented locomotory responses to d‐amphetamine (1 mg/kg), suggesting a long‐lasting perturbation of central monoamine neuronal transmission.
Abduction nystagmus in internuclear ophthalmoplegia
Authors: Zee David S. , Hain Timothy C. , Carl James R. 
Published: January 2005

AbstractWe tested the hypothesis that abnormalities of the abducting eye in internuclear ophthalmoplegia reflect an adaptive process that helps overcome the adduction weakness of the opposite eye. This response operates under the constraints of Hering's law of equal innervation: any attempt to increase the innervation to a weak muscle in one eye must be accompanied by a commensurate increase in innervation to the yoke muscle in the other eye. In 4 patients with internuclear ophthalmoplegia, we patched one eye for 1 to 5 days to allow time for the central nervous system to optimize innervation for the habitually viewing eye. We predicted that there would be a conjugate adjustment of innvervation that would diminish the abduction overshoot and backward postsaccardic drift made by the habitually viewing eye. This was the case in 3 of our 4 patients. Our findings show that the abduction nystagmus is a manifestation of a normal adaptive response in some patients with INO.
Disorders of the autonomic nervous system: Part 1. Pathophysiology and clinical features
Authors: McLeod J. G. , Tuck R. R. 
Published: January 2005

AbstractAutonomic dysfunction may result from diseases that affect primarily either the central nervous system or the peripheral autonomic nervous system. The most common pathogenesis of disturbed autonomic function in central nervous system diseases is degeneration of the intermediolateral cell columns (progressive autonomic failure) or disease or damage to descending pathways that synapse on the intermediolateral column cells (spinal cord lesions, cerebrovascular disease, brainstem tumors, multiple sclerosis). The peripheral autonomic nervous system may be damaged in isolation in the acute and subacute autonomic neuropathies or in association with a generalized peripheral neuropathy. The peripheral neuropathies most likely to cause severe autonomic disturbance are those in which small myelinated and unmyelinated fibers are damaged in the baroreflex afferents, the vagal efferents to the heart, and the sympathetic efferent pathways to the mesenteric vascular bed. Acute demyelination of the sympathetic and parasympathetic nerves in the Guillain‐Barré syndrome may also cause acute autonomic dysfunction. Although autonomic disturbances may occur in other types of peripheral neuropathy, they are rarely clinically important.
Recovery of vision after ischemic lesions: Positron emission tomography
Authors: Bosley Thomas M. , Dann Robert , Silver Frank L. , Alavi Abass , Kushner Michael , Chawluk John B. , Savino Peter J. , Sergott Robert C. , Schatz Norman J. , Reivich Martin 
Published: January 2005

AbstractWe used [18F]fluoro‐2‐deoxyglucose positron emission tomography (PET) to study serial changes in the local cerebral metabolic rate of glucose in 5 patients with ischemic lesions of the posterior afferent visual system causing homonymous visual field defects. All 5 patients had striking impairment of glucose metabolism in the striate cortex shortly after ictus. In 2 patients, visual field defects abated, and repeat PET scans showed reduced size of the metabolic lesion and improvement of striate metabolism. Three patients did not recover vision, and repeat PET scans did not show improvement. Patients who recovered vision had ischemic lesions outside the occipital lobe, while those who did not experience improvement had primary damage to the occipital lobe itself.
Altered metabolic properties of cultured skin fibroblasts in Alzheimer's disease
Authors: Sims Neil R. , Finegan Janet M. , Blass John P. 
Published: January 2005

AbstractAlzheimer's disease is associated with selective neuronal loss, the cause of which is undetermined. Evidence indicating a predisposing genetic factor associated with this disease suggests that important alterations may be expressed in tissues other than the brain. Because abnormal glucose and energy‐related metabolism have been identified in both in vivo and in vitro studies of brain, we conducted a study to examine related measures in cultured skin fibroblasts from six patients with Alzheimer's disease and seven age‐matched controls. After 60 minutes' incubation, the production of 14CO2 from [U‐14C]glucose and lactate production were significantly higher in the cells from the group of patients with Alzheimer's disease. The increase of 14CO2 production, but not the production of lactate, was most evident after a more rapid period of metabolic activity in the first 10 minutes of incubation. By contrast, 14CO2 production from [U‐14C]glutamine, which is probably the major substrate of oxidative metabolism in these cells, was significantly reduced in the Alzheimer's disease cells following longer (120‐minute) incubations. Oxygen uptake by cell suspensions was also significantly reduced in the group with Alzheimer's disease. These results indicate that complex metabolic differences are expressed in nonneural tissues from some patients with Alzheimer's disease and may provide important clues to the pathogenesis of this disorder.
Inactivation of GM1‐ganglioside β‐galactosidase by a specific inhibitor: A model for ganglioside storage disease
Authors: Singer Harvey S. , Tiemeyer Michael , Slesinger Paul A. , Sinnott Michael L. 
Published: January 2005

AbstractThis study was designed to establish an in vitro model with biochemical and morphological similarities to the human neurodegenerative disease GM1 gangliosidosis. Utilizing a specific inactivator of the lysosomal enzyme GM1‐ganglioside β‐galactosidase (β‐D‐galactopyranosylmethyl‐p‐nitrophenyltriazene [β‐GalMNT]) and neuroblastoma X glioma hybrid cells (NG108–15), we suppressed β‐galactosidase activity for up to 72 hours. Coincidental with suppression of this enzyme to levels less than 1% of control, we found up to a nine‐fold accumulation of its substrate, the GM1‐ganglioside, and the ultrastructural appearance of membranous cytoplasmic bodies. β‐GalMNT treatment suppressed growth but had little effect on the specific activity of choline acetyltransferase, lactate dehydrogenase, or other lysosomal enzymes including galactosylceramidase. This model should permit studies of the neurophysiological effects of increased ganglioside accumulation and their reversibility.
Immunoblotting of Creutzfeldt‐Jakob disease prion proteins: Host species—specific epitopes
Authors: Bockman Jeffrey M. , Prusiner Stanley B. , Tateishi Jun , Kingsbury David T. 
Published: January 2005

AbstractCreutzfeldt‐Jakob disease (CJD) is a rare dementia that is generally found in older people and is caused by unusual infectious pathogens or prions. Using rabbit antisera raised against hamster scrapie prion proteins (HaPrPSc), we identified by immunoblotting human CJD prion proteins (HuPrPCJD) in the brains of 14 patients dying of CJD. Extracts from 6 of the patients were transmitted to mice after prolonged incubation. The rabbit antisera raised against HaPrPSc also reacted with the mouse CJD prion proteins (MoPrPCJD) found in the brains of these experimentally infected mice. When mice were immunized with HuPrPCJD, they produced antibodies that reacted with HuPrPCJD but not with MoPrPCJD. Mice immunized with MoPrPCJD produced antibodies to neither murine nor human prion proteins. Our results provide evidence for host species‐‐specific epitopes on prion proteins. The existence of such epitopes is consistent with the apparent lack of an immune response during prion infections and the finding that prion protein molecules are encoded by host genes.