bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurological intensive care
Authors: Ropper Allan H. 
Published: January 2005

AbstractNeurological intensive care has evolved from the principles of respiratory care established during the poliomyelitis epidemics into a broad field encompassing all of the acute and serious aspects of neurological disease. The economic and political complexities of modern intensive care play a major role in organizing a unit and building a program. Central themes of practice in modern neurological intensive care units include the clinical physiology of intracranial pressure, verebral blood flow, and brain electrical activity; the systemic abnormalities and medical complications of nervous system, disease; postoperative care; and management of neuromuscular respiratory failure. Treatment of severe stroke andcerebral hemorrhage, brain death, ethical dilemmas of severe neurological illnesses, and neurological features of critically ill medical patients are also becoming neurological intensive care pursuits. The “neuro intensivist” is trained to defragment medical care by combining knowledge of neurological diseases with the techniques of intensive care. Future directions include the clinical implementation of brain resuscitation and brain‐sparing therapies, sophisticated monitoring of electrophysiological and intracranial physiological indices, and further understanding of the dysfunction of other organs that follows brain and nerve failure.
Ryanodine receptor autoantibodies in myasthenia gravis patients with a thymoma
Authors: Mygland Åse , Tysnes Ole‐Bjørn , Aarli Johan A. , Gilhus Nils Erik , Matre Roald , Volpe Pompeo 
Published: January 2005

AbstractSera from patients with myasthenia gravis were examined by Western blot for the presence of antibodies to proteins of the sarcoplasmic reticulum from rabbit skeletal muscle. Fourteen of 30 patients with myasthenia gravis and a thymoma had IgG autoantibodies to the calcium release channel of the sarcoplasmic reticulum (the ryanodine receptor), which plays a crucial role in the mechanism of excitation‐contraction coupling in striated muscle. Ryanodine receptor autoantibodies were not detected in any of the 45 sera from patients with myasthenia gravis without a thymoma. Ryanodine receptor autoantibodies may have pathogenetic relevance in thymoma‐associated myasthenia gravis.
α‐tocopherol levels in brain are not altered in Parkinson's disease
Authors: Dexter D. T. , Jenner P. , Ward R. J. , Peter T. J. , Wells F. R. , Daniel S. E. , Lees A. J. , Marsden C. D. 
Published: January 2005

Abstractα‐Tocopherol (vitamin E) levels in normal brain were lower in the cerebellum than in the cerebral cortex or basal ganglia. There was no difference in α‐tocopherol levels in the cerebellum, basal ganglia, or cerebral cortex between control subjects and patients with Parkinson's disease.
The identification of presymptomatic parkinsonism: Clinical and {18F}dopa positron emission tomography studies in an irish kindred
Authors: Sawle G. V. , Wroe S. J. , Lees A. J. , Brooks D. J. , Frackowiak R. S. J. 
Published: January 2005

AbstractAn Irish kindred is described in which 5 of 10 siblings in the fourth or fifth decade of life developed an akinetic‐rigid syndrome clinically indistinguishable from idiopathic Lewy body Parkinson's disease. Four of these patients were scanned by positron emission tomography (PET) with {18F}dopa after clinical diagnosis and in all, a profound impairment of tracer uptake into the striatum was recorded. The fifth patient was initially scanned at a time when he was asymptomatic and normal by clinical examination. His scan showed impaired tracer uptake, indicating a subclinical defect in the presynaptic nigrostriatal system. Within months of his scan, he too developed subtle symptoms and signs of parkinsonism although there was little further clinical piogression or change in his PET scan over the following year. A clinically normal sibling was also scanned and found to have subclinical impairment of [18F}dopa uptake in the putamen. The 19‐year‐old daughter of an affected sibling had a mild postural tremor but no other symptoms or signs. The {18F}dopa uptake in her putamen fell at the borderline between normal and parkinsonian values. This study confirms that PET can identify preclinical parkinsonism in at‐risk subjects. The finding of abnormalities in several clinically unaffected family members suggests that family studies based on clinical assessment alone may miss a significant number of subclinically affected individuals, leading to an underestimate of any genetic component to Parkinson's disease.
Neurological rehabilitation
Authors: Selzer Michael E. 
Published: January 2005

AbstractIncreasingly, neurologists are participating in the rehabilitation of disorders of the nervous system. Compelling reasons why neurologists, especially those with academic interests, should become involved in rehabilitation include the opportunity to observe patients in the recovery phases of their illnesses and to study issues relating to functional recovery, exposure to patients (such as those with spinal cord injury) who are often not seen by neurologists in the acute phase, and the opportunity to help establish a scientific base for rehabilitation medicine. Many areas of investigation can contribute to the scientific basis of neurological rehabilitation. Among the most promising are regeneration in the central (CNS) and peripheral nervous systems, adaptive mechanisms following CNS injury, computational neuroscience, neuromuscular physiology of chronic denervating conditions (e.g., the post‐polio syndrome), and outcome studies of both physical and pharmacological therapeutic modalities. While there may be financial advantages to involvement in neurological rehabilitation, these are probably overestimated and interest in this field would continue to grow without them. There is a need for enhanced training in this subspecialty, including the development of dual certification programs in neurology and rehabilitation medicine.
Increased levels of neuronal thread protein in cerebrospinal fluid of patients with Alzheimer's disease
Authors: de la Monte Suzanne M. , Volicer Ladislav , Hauser Stephen L , Wands Jack R. 
Published: January 2005

AbstractNeuronal thread protein is a recently characterized, ∼20‐kd protein that accumulates in brains with Alzheimer's disease (AD) lesions. This study examined whether concentrations of neuronal thread protein (NTP) were also increased in the cerebrospinal fluid (CSF) of individuals with probable (clinically diagnosed) and definite (histopathologically proved) AD. Using a highly sensitive three‐site monoclonal antibody–based immunoradiometric assay, we measured NTP concentrations in CSF from 84 patients with probable AD and mild dementia (duration, 4.05 ± 0.36 years), 45 with Parkinson's disease and minimal or no dementia (duration, 4.73 ± 0.78 years), 73 with multiple sclerosis, and 73 nondemented control subjects. NTP concentrations were also measured in postmortem ventricular fluid and temporal lobe neocortex extracts from 31 subjects with histopathologically proved AD and 14 age‐matched control subjects. The mean concentration of NTP in the CSF was higher in AD (4.15 ± 0.25 ng/ml; 95% confidence interval [CI] limits, 3.65–4.65) than in Parkinson's disease (1.96 ± 0.16 ng/ml; 95% CI, 1.65–2.27), multiple sclerosis (1.6 0.14 ng/ml; 95% CI limits, 1.33–1.88), or control subjects (1.27 ± 0.06 ng/ml; 95% CI limits, 1.15–1.40) (p < 0.001). In addition, 70% of the patients with probable AD had concentrations of NTP in CSF that were higher than 2.5 ng/ml (> upper 99% CI limit in the control group), compared with 23%of Parkinson's disease patients, 11% of multiple sclerosis patients, and 40% of control subjects. The mean concentrations of NTP in the ventricular fluid and brain tissue from individuals with documented AD and end‐stage dementia were threefold higher than the levels detected in the CSF from the remaining patients with probable AD and mild dementia. Moreover, of 9 patients with AD, postmortem brain and CSF manifested 5‐ to 50‐fold higher levels of NTP compared with the CSF samples obtained an average of 6 years earlier. These findings demonstrate that NTP levels are elevated in the CSF of individuals with AD and that NTP levels in the CSF increase strikingly with progression of dementia and neuronal degeneration.
Continuous lisuride effects on central dopaminergic mechanisms in Parkinson's disease
Authors: Baronti Fabio , Mouradian M. Maral , Davis Thomas L , Giuffra Marianne , Brughitta Giorgio , Conant Kathy E. , Chase Thomas N. 
Published: January 2005

AbstractEffects of the long term, continuous administration of a dopamine agonist on motor response complications attending levodopa therapy were studied in 7 patients with advanced Parkinson's disease under controlled conditions. After a 3‐month round‐the‐clock infusion of lisuride, the duration of antiparkinsonian action of levodopa increased by approximately 90%, and the therapeutic window for the acutely administered dopamine precursor widened by >300%. These benefits were more than three times greater than those produced by 9 days of continuous levodopa administration. In contrast to the effects on levodopa pharmacodynamics, the continuous infusion of lisuride did not prolong its action, suggesting a lisuride effect on presynaptic as well as postsynaptic dopaminergic mechanisms. These results lend further support to the view that continuous dopamine replacement ameliorates motor fluctuations and peak‐dose dyskinesias that complicate standard levodopa regimens. Our findings further suggest that alterations at both presynaptic and postsynaptic levels contributing to these motor complications tend to normalize with the more physiological stimulation afforded by continuous replacement strategies, especially when given chronically.
Laser microprobe analysis of brain aluminum in Alzheimer' disease
Authors: Lovell Mark A. , Ehmann William D. , Markesbery William R. 
Published: January 2005

AbstractAluminum (Al) levels were measured in the cytoplasm and nucleus of 241 neurofibrillary tangle (NFT)‐bearing neurons, in 217 NFT‐free neurons and adjacent neuropil from 7 autopsy‐confirmed Alzheimer's disease (AD) patients, and in 316 normal neurons from 5 control subjects, by laser micriprobe mass spectrometry. Grand mean Al levels (dry weight basis) in AD samples were 2.93 ± 1.24 μg/gm for NFT‐bearing neuron cytoplasm, 3.54 ± 1.39 μg/gm for NFT‐bearing neuron nuclei, 2.31 ± 1.09 μg/gm for NFT‐free neuron cytoplasm, and 3.23 ± 1.09μg/gm for NFT‐free neuron nuclei. Control values were 1.85 ± 0.78 μg/gm for cytoplasm and 2.01 ± 0.93 μg/gm for nuclei. The differences between corresponding regions of AD NFT‐bearing, AD NFT‐free, and control neurons were not significant (p > 0.05, analysis of variance). Al levels in neuropil were identical for AD and control samples at 2.16 ± 0.93 μg/gm. In contrast to some literature reports, we found very few (<2.5%) extremely high Al values (>20μg/gm, dry weight) on a cellular basis in AD samples. AD neurons did exhibit a higher number of Al values (9.6−14.3%) that were >sgrave; above the corresponding control means, than did control neurons (1.3–1.6%), indicating that small elevations of Al may exist in patients with AD. Our data suggest that any Al accumulation in patients with AD is small and generalized in both NFT‐free and NFT‐bearing neurons and that analyses of large bulk brain samples are likely to have AD/control differences masked by the large amount of unaffected neuropil sampled.
Alternating hemiplegia of childhood: Studies of regional cerebral blood flow using 99mTc‐hexamethylpropylene amine oxime single‐photon emission computed tomography
Authors: Aminian Abdolkarim , Strashun Arnold , Rose Arthur 
Published: January 2005

AbstractAlternating hemiplegia of childhood is a rare disorder of unknown cause associated with progressive neurological deterioration. We report the results of regional cerebral blood flow studies using 99mTc‐hexamethylpropylene amine oxime single‐photon emission computed tomography in 3 patients. These studies were performed during the hemiplegic attacks (n = 6) and during the symptom‐free periods (n = 2). Six single‐photon emission computed tomographic studies performed during hemiplegic attacks consistently showed relative hyperperfusion of the contralateral cerebral hemisphere. Two single‐photon emission computed tomographic studies performed during the asymptomatic phase showed normal and symmetrical cerebral perfusion. This is the first definite demonstration of unilateral increase of cerebral blood flow in alternating hemiplegia. These findings support the possibility of a relationship between the cause of alternating hemiplegia and migraine.
Okadaic acid induces hyperphosphorylated forms of tau protein in human brain slices
Authors: Harris K. A. , Oyler G. A. , Doolittle G. M. , Vincent I. , Lehman R. A. W. , Kincaid R. L. , Billingsley M. L. 
Published: January 2005

AbstractHyperphosphorylated forms of the microtubule‐associated protein tau are components of the paired helical filaments (PHFs) seen in patients with Alzheimer's disease. Slices of human lateral temporal cortex were obtained from tissues removed incidental to resections for intractable hippocampal epilepsy. Tau phosphorylation in temporal lobe slices was determined using mobility shifts after sodium dodecyl sulfate‐polyacrylamide gel electrophoresis and immunodetection with the monoclonal antibodies Alz‐50, 5E2, and Tau‐1. The results indicate that tau phosphorylation was altered in a dose‐dependent manner by the phosphatase inhibitor okadaic acid, but not by N‐methyl‐D‐aspartate, quisqualate, or kainate. The slowest mobility forms of tau, termed “PHF‐like tau,” produced by okadaic acid treatment were dephosphorylated by purified protein phosphatase 2B (calcineurin). Formation of PHF‐like tau peptides was blocked by KN‐62, 1{N,O‐bis(1,5‐isoquinolinesulfonyl)‐N‐methyl‐L‐tyrosyl}‐4‐phenylpiperazine, an inhibitor of Ca2+/calmodulin‐dependent protein kinase II. The protein kinase inhibitor staurosporine also prevented formation of PHF‐like tau. These data suggest that phosphorylation of tau is regulated by Ca2+‐dependent protein kinases and okadaic acid–sensitive protein phosphatases, alterations of which may be implicated in the pathogenesis of Alzheimer' disease.
Molecular dissection of the myelinated axon
Authors: Waxman Stephen G. , Ritchie J. Murdoch 
Published: January 2005

AbstractThe membrane of the myelinated axon expresses a rich repertoire of physiologically active molecules: (1) Voltagesensitive NA+ channels are clustered at high density (∼1,000/μm2) in the nodal axon membrane and are present at lower density(<25/μm2) in the internodal axon membrane under the myelin. Na+ channels are also present within Schwann cell processes (in peripheral nerve) and perinodal astrocyte processes (in the central nervous system) which contact the Na+ channel–rich axon membrane at the node. In some demyelinated fibers, the bared (formerly internodal) axon membrane reorganizes and expresses a higher‐than‐normal Na+ channel density, providing a basis for restoration of conduction. The presence of glial cell processes, adjacent to foci of Na+ channels in immature and demyelinated axons, suggests that glial cells participate in the clustering of Na+ channels in the axon membrane. (2) “Fast” K+ channels, sensitive to 4‐aminopyridine, are present in the paranodal of internodal axon membrane under the myelin; these channels may function to prevent reexcitation following action potentials, or participate in the generation of an internodal resting potential. (3) “Slow” K+ channels, sensitive to tetraethylammonium, are present in the nodal axon membrane and, in lower densities, in the internodal axon membrane; their activation produces a hyperpolarizing afterpotential which modulates repetitive firing. (4) The “inward rectifier” is activated by hyperpolarization. This channel is permeable to both Na+ and K+ ions and may modulate axonal excitability or participate in ionic reuptake following activity. (5) Na+/K+‐ATPase and (6) Ca2+‐ATPase are also present in the axon membrane and function to maintain transmembrane gradients of Na+, K+, and Ca2+. (7) A specialized antiporter molecule, the Na+/Ca2+ exchanger, is present in myelinated axons within central nervous system white matter. Following anoxia, the Na+/Ca2+ exchanger mediates an influx of Ca2+ which damages the axon. The molecular organization of the myelinated axon has important pathophysiological implications. Blockade of fast K+ channels and Na+/K+‐ATPase improves action potential conduction in some demyelinated axons, and block of the Na+/Ca2+ exchanger protects white matter axons from anoxic injury. Modification of ion channels, pumps, and exchangers in myelinated fibers may thus provide an important therapeutic approach for a number of neurological disorders.
Pathological findings at the site of conduction block in multifocal motor neuropathy
Authors: Kaji Ryuji , Oka Nobuyuki , Tsuji Teruyuki , Mezaki Takahiro , Nishio Takeshi , Akiguchi Ichiro , Kimura Jun 
Published: January 2005

AbstractWe report on 2 patients with multifocal motor neuropathy with focal conduction block involving motor but not sensory fibers at the site of nerve swelling. A nerve biopsy specimen from adjacent to the enlargement in 1 patient showed a perivascular area containing scattered demyelinated axons surrounded by small onion bulbs. The observed pathological findings are consistent with the conduction block considered characteristic of this neuropathy, although the underlying immunological mechanisms for selectivity and persistence remain undetermined.
Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis
Authors: Baram Tallie A. 
Published: January 2005

AbstractMassive infantile spasms are an age‐specific seizure syndrome of infancy. Uniquely, the spasms respond to hormonal manipulation using adrenocorticotropic hormone(ACTH) or glucocorticoids. A hypothesis explaining the efficacy of hormonal therapy, age ‐specificity, multiple causative factors, and spontaneous resolution of infantile spasms is presented. Corticotropin‐releasing hormone(CRH), an excitant neuropeptide supressed by ACTH/Steroids, is implicated. Evidence for the age‐specific vonvulsant properties of CRH is presented, and a putative secenario in which a stressinduced enhancement of endogenous CRH‐mediated seizures is discussed. Clinical resting of the CRH‐Excess theory and its therapeutic implication are suggested.
Dopamine agonist treatment of antegrade amnesia from a mediobasal forebrain injury
Authors: Dobkin Bruce H. , Hanlon Robert 
Published: January 2005

AbstractWe studied the persistent antegrade memory impairment in a woman whose brain had been surgically impaled, leaving a 1‐cm‐wide mediobasal tract of encephalomalacia that entended just enterior to the septal, nuclei and medial to the nucleus accumbens. In a blinded, controlled, alternating repeated‐measures protocol, bromocriptine signigicantly improved her verbal learning, functional memory, and dailyrecall, perhaps by acting on neurons that had been disconnected fron the ventral tegmental tract's dopaminergic inputs.
Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease)
Authors: Bronson Roderick T. , Lake Brian D. , Cook Susan , Taylor Susanne , Davisson Muriel T. 
Published: January 2005

AbstractPathological studies of mice homozygous for the motor neuron degeneration (Mnd) mutation show abnormalities similar to those of the human neuronal ceroid lipofuscinoses: sudanophilic, autofluorescent intraneuronal inclusions that are immunoreactive with antibodies to subunit c of mitochondrial ATP synthase. Ultrastructurally, the inclusions have the pentalaminar structure characteristic of some forms of human neuronal ceroid lipofuscinosis and of canine and ovine models of neuronal ceroid lipofuscinosis. Similar inclusions are observed in many somatic organs and in the retina, which develops photoreceptor degeneration. This mutation, previously considered a model of amyotrophic lateral sclerosis, may be a useful model for molecular and genetic studies of human neuronal ceroid lipofuscinosis because mice have been well characterized genetically. Since they are inexpensive to breed and maintain, they can also be used to test therapeutic interventions.
Successful outcome of progressive multifocal leukoencephalopathy with cytarabine and interferon
Authors: Steiger M. J. , Tarnesby G. , Gabe S. , McLaughlin J. , Schapira A. H. V. 
Published: January 2005

AbstractThe prognosis of patients with progressive multifocal leukoencephalopathy is poor, with few patients showing remission or surviving. We describe a 37‐year‐old man who developed progressive multifocal leukoencephalopathy in association with sarcoidosis. Despite treatment with cytarabine and acyclovir, he continued to deteriorate. Shortly following the addition of interferon alfa, he made a dramatic improvement, regaining full functional independence. The use of interferon alfa in addition to cytarabine in such patients offers a new therapeutic approach worthy of further trial.
Oscillopsia and horizontal nystagmus with accelerating slow phases following lumbar puncture in the Arnold‐chiari malformation
Authors: Barton Jason J. S. , Sharpe James A. 
Published: January 2005

AbstractOscillopsia and nystagmus began in a woman 2 weeks after an inadvertent lumbar puncture during anesthesia for childbirth. Examination showed horizontal‐torsional jerk nystagmus in all positions of gaze. Magnetic‐search‐coil oculography revealed accelerating slow phases, with an increase in nystagmus amplitude in darkness. Magnetic resonance images showed type 1 Arnold‐Chiari malformation. Three months after occipital decompressive surgery, nystagmus had almost disappeared. Accelerating slow phases should not be considered diagnostic of congenital nystagmus, especially with an onset of oscillopsia in adult life; imaging should be considered to exclude treatable hindbrain anomalies. Lumbar puncture in patients with the Arnold‐Chiari malformation may accentuate craniospinal pressure dissociation and precipitate neurological signs.
Neuroleptic medications inhibit complex I of the electron transport chain
Authors: Burkhardt Carolyn , Kelly James P. , Lim Young‐Hwa , Filley Christopher M. , Parker W. Davis 
Published: January 2005

AbstractNeuroleptic medications are prescribed to millions of patients, but their use is limited by potentially irreversible extrapyramidal side effects. Haloperidol shows striking structural similarities to the neurotoxin 1‐methyl‐4‐phenyl‐1,2,3,6‐tetra‐hydropyridine, which produces parkinsonism apparently through inhibition of NADH:ubiquinone oxidoreductase (complex I) of the mitochondrial electron transport chain. We now report that haloperidol chlorpromazine, and thiothixine inhibit complex I in vitro in rat brain mitochondria. Clozapine, an atypical antipsychotic reported to have little or no extrapyramidal toxicity, also inhibits complex I, but at a significantly higher concentration. Neuroleptic treated patients have significant depression of platelet complex I activity similar to that seen in idiopathic Parkinson's disease. Complex I inhibition may be associated with the extrapyramidal side effects of these drugs.