bluuebunny/crossref_metadata_2025_split huggingface dataset:
Amyotrophic lateral sclerosis: Amino acid levels in plasma and cerebrospinal fluid
Authors: Perry Thomas L. , Krieger Charles , Hansen Shirley , Eisen Andrew 
Published: January 2005

AbstractConcentrations of glutamic acid have been reported to be elevated in fasting plasma and cerebrospinal fluid (CSF) of patiends with amyotrophic lateral sclerosis (ALS); glycine concentrations have also been reported to be increased in the CSF of such patients. Autopsy studies have shown glutamate contents to be significantly decreased in brain and spinal cord in ALS. These observations suggested that a systemic abnormality of glutamate metabolism might underlie the pathogenesis of ALS. We report here the findings of our studies of amino acid concentrations in patients with the sporadic form of ALS. Glutamate concentrations were normal in the fasting plasma of a great majority of the patients with ALS. Concentrations of glutamate, aspartate, and glycine were normal in the CSF of all 17 patients examined. b̃‐N‐Methylamino‐L‐alanine, a plant neurotoxin possibly responsible for causing the Guamanian form of ALS, was not detectable in the plasma or CSF of any of our patients. Our findings do not lend support to the hypothesis that the sporadic form of ALS results from overexcitation of motor neurons by excitatory amino acids.
Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis
Authors: Rothstein Jeffrey D. , Tsai Guochuan , Kuncl Ralph W. , Clawson Lora , Cornblath David R. , Drachman Daniel B. , Pestronk Alan , Stauch Barbara L. , Coyle Joseph T. 
Published: January 2005

AbstractRecently, the excitatory amino acid neurotransmitter glutamate was implicated in the pathogenesis of a variety of chronic degenerative neurological diseases in humans and animals. This report describes abnormalities in excitatory amino acids in the central nervous system of 18 patients with amyotrophic lateral sclerosis (ALS). The concentration of the excitatory amino acids glutamate and aspartate in the cerebrospinal fluid were increased significantly (p > 0.01) by 100 to 200% in patients with ALS. Similarly, the concentrations of the excitatory neuropeptide N‐acetyl‐aspartyl glutamate and its metabolite, N‐acetyl‐aspartate, were elevated twofold to threefold in the cerebrospinal fluid from the patients. There was no relationship between amino acid concentrations and duration of disease, clinical impairment, or patient age. In the ventral horns of the cervical region of the spinal cord, the level of N‐acetyl‐‐aspartyl glutamate and N‐acetyl‐aspartate was decreased by 60% (p > 0.05) and 40% (p > 0.05), respectively, in 8 patients with ALS. Choline acetyltransferase activity was also diminished by 35% in the ventral horn consistent with motor neuron loss. We conclude that excitatory amino acid metabolism is altered in patients with ALS. Based on neurodegenerative disease models, these changes may play a role in motor neuron loss in ALS.
The role of plasmapheresis in childhood guillain‐barré syndrome
Authors: Epstein Mark A. , Sladky John T. 
Published: January 2005

AbstractPlasmapheresis has been advocated in the treatment of childhood Guillain‐Barré syndrome under the assumption that the results of adult series can be extrapolated to children. To test this assumption, we retrospectively evaluated the medical charts of all children who were admitted to The Children's Hospital of Philadelphia between January 1984 and March 1989, with the diagnosis of Guillain‐Barré syndrome. Of the 30 patients identified, 7 were excluded because they had mild disease. Of the remaining 23, 9 underwent plasmapheresis and 14 served as historic control subjects. The two groups were similar with respect to age, presenting symptoms, finding on initial physical examination, and antecedent illnesses at the time of diagnosis. The mean time to recover to Grade 2 (independent ambulation) was significantly shorter in the plasmapheresis‐treated group, 24.0 ± 25.4 days, compared to 60.2 ± 43.6 days in control subjects (mean ± 1 SD). Our results indicate that plasmapheresis diminishes morbidity in childhood Guillain‐Barré syndrome by shortening the interval until recovery of independent ambulation.
Middle cerebral artery strokes causing homonymous hemianopia: Positron emission tomography
Authors: Kiyosawa Motohiro , Bosley Thomas M. , Kushner Michael , Jamieson Dara , Alavi Abass , Reivich Martin 
Published: January 2005

AbstractEight patients were evaluated with 18F‐fluorodeoxyglucose positron emission tomography between 3 and 30 days after isolated stroke involving the middle cerebral artery territory that caused homonymous hemianopia. Diffuse hypometabolism was present throughout the damaged cerebral hemisphere, even in cortical areas not obviously ischemic by clinical examination or neuroimaging. Glucose metabolism in primary and association visual cortex of the damaged hemisphere was decreased by more than 47% (p < 0.01). Metabolism in the undamaged hemisphere was less profoundly affected, but significant decrements were found in calcarine (40%;p < 0.01) and lateral occipital cortex (35%;p < 0.05).
Motor neuron disease in a patient with a monoclonal IgMk directed against GM1, GD1b, and high‐molecular‐weight neural‐specific glycoproteins
Authors: Nobile‐Orazio E. , Legname G. , Daverio R. , Carpo M. , Giuliani A. , Sonnino S. , Scarlato G. 
Published: January 2005

AbstractIn a patient with motor neuron disease and benign IgMk monoclonal gammopathy, the M rprotein reacted with the glycolipids GM1, GD1b, and asialo GM1 and, by immunoblot, with some high‐molecular‐weight neural‐specific glycoproteins. The main reactive bands had an approximate molecular weight of 250 and 400 kd, were most concentrated in the spinal cord, and were also bound by the lectin peanut agglutinin. The presence of the Gal(b̃1‐3) GalNAc epitope on these neural‐specific glycoproteins may help to explain the selective neurological impairment of the patient.
Auras and subclinical seizures: Characteristics and prognostic significance
Authors: Sperling Michael R. , O'Connor Michael J. 
Published: January 2005

AbstractThe characteristics and prognostic significance of subclinical seizures and independent auras were studied in 40 patients with partial epilepsy who had long‐term electroencephalographic (EEG) monitoring with intracranial electrodes. Focal, restricted subclincal seizures were noted in 23 patients, and 11 patients experienced auras that were accompanied by ictal EEG discharges. Auras and subclinical seizures usually were identical in EEG appearance, but were distributed differently among patients. The subclinical seizures and auras usually had the same origin as complex partial seizures, but did not always reliably indicate complex partial seizure origin. Subclinical seizures and auras were of favorable prognostic significance for patients undergoing temporal lobectomy. A majority (>80%) of individuals with subclinical seizures and auras were free of complex partial seizures after surgery, whereas a minority (29%) of patients without subclinical seizures and auras became free of complex parital seizures.
Increased MEPP frequency as an early sign of experimental immune‐mediated motoneuron disease
Authors: García Jesús , Engelhardt Jozsef I. , Appel Stanley H. , Stefani Enrico 
Published: January 2005

AbstractIntracellular recordings of miniature end‐plate potentials were performed in extensor digitorum longus muscles from guinea pigs with experimental immune‐mediated motoneuron destruction. In the early stages of the disease, the miniature end‐plate potential frequency was elevated compared to that in control and normal animals. The amplitude and time course of the miniature end‐plate potentials as well as the resting potential of the muscle fibers were not altered, which implies integrity of the postjunctional membrane. The increase in frequency of miniature end‐plate potential reflects an increase of basal acetylcholine release and documents dysfunction of the presynaptic terminal of the neuromuscular junction. The increased frequency was associated with high levels of antimotoneuronal IgG in the blood and the presence of IgG at motor end‐plates. These data suggest that the presynaptic terminal of the neuromuscular junction may be involved in the immune attack in animal models of motoneuron degeneration.
Major histocompatibility complex antigen expression in the affected tissues in amyotrophic lateral sclerosis
Authors: Lampson Lois A. , Kushner Pinky D. , Sobel Raymond A. 
Published: January 2005

AbstractMonoclonal antibody immunocytochemsitry was used to examine spinal cord and muscle in amyotrophic lateral sclerosis for changes that would indicate ongoing or potential immune activity. Increased expression of class I and II major histocompatibility complex (MHC) antigens was seen in the affected areas of spinal cord. New MHC expression was concentrated in phagocytes, particularly in degenerating white matter in which they were dispersed in the tissue and also packed around blood vessels. MHC antigen was not revealed in motor neurons or skeletal muscle fibers. An anti‐pan–T‐cell monoclonal revealed small numbers of T cells in degenerating white matter. Similar changes have been seen in other neurodegenerative disorders. They suggest a potential for (secondary) cell‐mediated activity in the affected areas rather than an ongoing MHC‐restricted T‐Cell response. Vessel‐associated phagocytes may be a source of antigen to peripheral lymphoid tissue, stimulating production of the autoantibodies that have been described.
Brain microemboli during cardiac surgery or aortography
Authors: Moody D M. , Bell M. A. , Challa V. R. , Johnston W. E. , Prough D. S. 
Published: January 2005

AbstractWe have observed many focal dilatations or very small aneurysms in terminal arterioles and capillaries of 4 of 5 patients and 6 dogs who had recently undergone cardiopulmonary bypass. A smaller number of sausagelike dilatations distended medium‐sized arterioles. Two other patients had a small number of the same microvascular changes following proximal aortography. Thirty‐four patients and 6 dogs not undergoing cardiopulmonary bypass had none. (A 35th patient who had not undergone cardiopulmonary bypass or aortography showed a small number of dilatations; mediastinal air was a suggested source.) Some of the dilatations exhibited various forms of birefringence. Because most of the dilatations appear empty, we speculate that they are the sites of gas bubbles or fat emboli that have been removed by the solvents used in processing. These microvascular events, occurring only in conjunction with major arterial interventions, may be the anatomical correlate of the neurological deficits or moderate to severe intellectual dysfunction seen in at least 24% of patients after cardiac surgical procedures assisted by cardiopulmonary bypass.
Differing patterns of striatal 18F‐dopa uptake in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy
Authors: Brooks D. J. , Ibanez V. , Sawle G. V. , Quinn N. , Lees A. J. , Mathias C. J. , Bannister R. , Marsden C. D. , Frackowiak R. S. J. 
Published: January 2005

AbstractUsing positron emission tomography (PET), we studied regional striatal 18F‐dopa uptake in 16 patients with L‐dopa‐responsive Parkinson's disease (PD), 18 patients with multiple system atrophy, and 10 patients with progressive supranuclear palsy. Results were compared with those of 30 age‐matched normal volunteers. The patients with PD showed significantly reduced mean uptake of 18F‐dopa in the caudate and putamen compared to controls, but while function in the posterior part of the putamen was severely impaired (45% of normal), function in the anterior part of the putamen and in the caudate was relatively spared (62% and 84% of normal). Mean 18F‐dopa uptake in the posterior putamen was depressed to similar levels in all patients. Unlike patients with PD, the patients with progressive supranuclear palsy showed equally severe impairment of mean 18F‐dopa uptake in the anterior and posteior putamen. Caudate 18F‐dopa uptake was also significantly lower in patients with progressive supranuclear palsy than in patients with PD, being depressed to the same level as that in the putamen. Mean 18F‐dopa uptake values in the anterior putamen and caudate in patients with multiple system atrophy lay between PD and progressive supranuclear palsy levels. Locomotor disability of individual patients with PD or multiple system atrophy correlated with decline in striatal 18F‐dopa uptake, but this was not the case for the patients with progressive supranuclear palsy. We conclude that patients with PD have selective nigral pathological features with relative preservation of the dopaminergic function in the anterior putamen and caudate, whereas there is progressively more extensive nigral involvement in multiple system atrophy and progressive supranuclear palsy. If an akinetic‐rigid patient has equally depressed 18F‐dopa uptake in the caudate and putamen, that person is likely to have neuropathological changes other than those of idiopathic PD.
Human immuno deficiency virus (HIV)–associated myopathy: Immunocytochemical identification of an HIV antigen (gp 41) in muscle macrophages
Authors: Chad David A. , Smith Thomas W. , Blumenfeld Andrew , Fairchild Patrick G. , Degirolami Umberto 
Published: January 2005

AbstractIn a patient with acquired immunodeficiency disease syndrome (AIDS) and muscle biopsy specimen disclosed degeneration of muscle fibers, regeneration, and focal endomysial mononuclear inflammation. A conspicuous feature was the presence of perivascular macrophages within the endomysium that showed positive immunostaining for human immunodeficiency virus (HIV) (gp 41) antigen. HIV was not detected within myofibers. Our findings suggest an important role for the HIV‐infected macrophage in the pathogenesis of this myopathy.
Large‐scale neurocognitive networks and distributed processing for attention, language, and memory
Authors: Mesulam M‐Marsel 
Published: January 2005

AbstractCognition and comportment are subserved by interconnected neural networks that allow high‐level computational architectures including parallel distributed processing. Cognitive problems are not resolved by a sequential and hierarchical progression toward predetermined goals but instead by a simultaneous and interactive consideration of multiple possibilities and constraints until a satisfactory fit is achieved. The resultant texture of mental activity is characterized by almost infinite richness and flexibility. According to this model, complex behavior is mapped at the level of multifocal neural systems rather than specific anatomical sites, giving rise to brain‐behavior relationships that are both localized and distributed. Each network contains anatomically addressed channels for transferring information content and chemically addressed pathways for modulating behavioral tone. This approach provides a blueprint for reexploring the neurological foundations of attention, language, memory, and frontal lobe function.
Brain pH in head injury: An image‐guided 31P magnetic resonance spectroscopy study
Authors: Rango Mario , Lenkinski Robert E. , Alves Wayne M. , Gennarelli Thomas A. 
Published: January 2005

AbstractIt has been suggested that brain acidosis may follow head trauma, and therapies aimed at correcting acidosis have been proposed. Direct measurements of intracellular pH, however, have thus far not been possible in clinical situations. We have studied the intracellular brain pH in 22 patients after head injury (mean Glasgow Coma Score 6.1). Patients were investigated by a combined approach of phosphorus 31 magnetic resonance spectroscopy and magnetic resonance imaging (overall examination time 50–75 min) at a mean time of 11 days after injury (36 hours to 24 days). 31P spectra were obtained in 11 patients on assisted ventilation and in 11 patients on spontaneous ventilation. These spectra were analyzed to yield the pH in the regions studied in all the patients. All pH values were in the normal or alkalotic range when compared with 6 age‐matched normal controls. No differences were found between patients on assisted ventilation and patients on spontaneous ventilation. When analyzed as a group, the brain pH in the focal lesions appeared to increase in the first days, to reach a peak in the alkalotic range in the second week, and to return toward normal within 3 weeks from acute injury. Our results suggest that there is no evidence of posttraumatic intracellular brain acidosis in recent human head injury, and therefore, therapies aimed at alkalinizing brain cells need to be reconsidered.
Respiratory failure and unilateral caudal brainstem infarction
Authors: Bogousslavsky J. , Khurana R. , Deruaz J. P. , Hornung J. P. , Regli F. , Janzer R. , Perret C. 
Published: January 2005

AbstractWe report clinicotopographic correlations in 2 patients with central hypoventilation and unilateral infarct in the caudal brainstem. One patient had nearly complete loss of ventilation involving both automatic and voluntary components. He showed no ventilator response during a CO2 retention test (Paco2 62 mm Hg, Pao2 82 mm Hg), while consciousness was preserved until death. The infarct involved the reticular formation, nucleus tractus solitarius, nucleus ambiguus, and nucleus retroambiguus on the right but spared the dorsal motor nucleus of the tenth cranial nerve, and sensory and corticospinal tracts. The second patient showed hypoventilation more selectively involving automatic responses (Ondine's curse). The infarct involved the medullary reticular formation and nucleus ambiguus but spared the nucleus tractus solitarius. We suggest that unilateral involvement of pontomedullary reticular formation and nucleus ambiguus is sufficient for generating loss of automatic respiration, while associated lesion of the nucleus tractus solitarius may lead to more severe respiratory failure involving both automatic and voluntary responses.
Stiff‐man syndrome: A GABAergic autoimmune disorder with autoantigenic heterogeneity
Authors: Gorin Dr F. , Baldwin B. , Tait R. , Pathak R. , Seyal M. , Mugnaini E. 
Published: January 2005

AbstractAutoantibodies that reacted with cell bodies and axon terminals of gamma‐aminobutyric acid (GABA)ergic neurons were present in the serum and cerebrospinal fluid in a patient with stiff‐man syndrome with type I diabetes. Immunoblot experiments using this patient's serum and cerebrospinal fluid did not corroborate an earlier observation that these autoantibodies are directed against the GABAergic cytosolic enzyme, L‐glutamic acid decarboxylase. While L‐glutamic acid decarboxylase autoantibodies may be associated with this syndrome, they do not appear to be easily demonstrated.
A case‐control study of Alzheimer's disease
Authors: Graves Amy Borenstein , White Emily , Koepsell Thomas D. , Reifler Burton V. , Van Belle Gerald , Larson Eric B. , Raskind Murray 
Published: January 2005

AbstractA Case‐control study of Alzheimer's disease (AD) was conducted to evaluate the roles of family history of dementing disease and AD, and medical conditions, particularly those related to the viral immune hypotheses in AD. One hundred and eighty‐three clinically diagnosed patients were identified between 1980 and 1985 through the Geriatric and Family Services clinic at the University of Washington hospital, and 18 patients were identified during the same time period through the Veteran's Administration Medical Center in Seattle, Washington. Telephone interviews were conducted with spouses (87.7%) and children (12.3%) of the patients. Each Patient was matched to a friend or non‐blood relative of the patient by age, sex, and the relationship between the patient and his or her respondent, for a total of 130 matched pairs. The odds ratio for AD was elevated if a first‐degree relative was reported to have a history of dementia (odds ratio, 2.21; 95% confidence interval, 1.17 to 4.18). This risk was especially elevated if a sibling was affected (odds ratio, 4.04; 95% confidence interval, 1.37 to 11.90); the highest risk to the proband was observed if a sibling had presumed AD (odds ratio, 5.92; 95% confidence interval, 1.59 to 21.96). The risk increased with increasing numbers of first‐degree family members affected for both a history of dementia and presumed AD. We found little difference between patients and control subjects with respect to the age of the mother or father at the time of the subject's birth. No statistically significant differences were observed between the two groups with regard to conditions that might support the immune and viral hypothesis in AD, smoking, or alcohol problems.
Timing and topography of cerebral blood flow, aura, and headache during migraine attacks
Authors: Olesen Jes , Friberg Lars , Olsen Tom Skyhøj , Iversen Helle K. , Lassen Niels A. , Andersen Allan R. , Karle Agnete 
Published: January 2005

AbstractTen years of study has resulted in considerable but fragmented knowledge about regional cerebral blood flow in migraine with aura (classic migraine). In the present study, the number of repeatedly studied patients (n = 63) was large enough to determine statistically significant sequences of events and statistically significant spatial relations. The first observable event was a decrease of regional cerebral blood flow posteriorly in one cerebral hemisphere. Further development of this pathological process was accompanied by the aura symptoms. Thereafter headache occurred while regional cerebral blood flow remained decreased. During the headache phase, regional cerebral blood flow gradually changed from abnormally low to abnormally high without apparent change in headache. In some patients headache disappeared while regional cerebral blood flow remained increased. Although regional cerebral blood flow reduction and aura symptoms in the great majority of patients were unilateral, one‐third had bilateral headache. Unilateral headache usually localized to the side on which regional cerebral blood flow was reduced and from which the aura symptoms originated (i.e., aura symptoms were perceived to occur contralaterally but presumably originated in the hypoperfused hemisphere). Our results suggest a simple model for migraine attacks: A pathological disturbance in one cerebral hemisphere causes the aura symptoms and after a time delay, it also causes the headache by stimulating local vascular nociceptors. Bilateral headache caused by a unilateral cerebral disturbance may be explained by recent neuroanatomical and neurophysiological findings.
Ataxia and peripheral neuropathy: A benign variant of peroxisome dysgenesis
Authors: Maccollin Mia , De Vivo Darryl C. , Moser Ann B. , Beard Margaret 
Published: January 2005

AbstractA 5‐year‐old boy with panperoxisomal dysfunction is described. Clinical features included hypotonia, areflexia, and ataxia. Cognition, vision, hearing, and hepatic function were normal. A panel of peroxisomal markers, including very‐long‐chain fatty acids, phytanic acid, pipecolic acid, and catalase compartmentalization, were abnormal. This is a uniquely benign syndrome of disordered peroxisome biogenesis.
Apparent response of subacute sclerosing panencephalitis to intrathecal interferon alpha
Authors: Miyazaki Masahito , Hashimoto Toshiaki , Fujino Kayo , Goda Tomoko , Tayama Masanobu , Kuroda Yasuhiro 
Published: January 2005

AbstractAn 8‐years‐old boy with subacute sclerosing panencephalitis (SSPE) was treated with 1.0 to 3.0 × 106 IU human interferon alpha (IFN) by the intrathecal route weekly or fortnightly. Pronounced improvement of clinical and electroencephalographic findings were observed in a dose‐dependent manner. Our patient raises hope that IFN can induce sustained remission in patients with SSPE.