bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effects of gangliosides on the expression of autoimmune demyelination in the peripheral nervous system
Authors: Ponzin Diego , Menegus Anna Maria , Kirschner Gunther , Nunzi M. Grazia , Fiori Mario G. , Raine Cedric S. 
Published: January 2005

AbstractTo test whether gangliosides (GA) might exert neuritogenic effects in vivo, experimental allergic neuritis (EAN) was studied clinically, neuropathologically, and immunologically in Lewis rats immunized with bovine peripheral nerve, P2 myelin protein, P2 myelin protein plus two different doses of GA, P2 with galactocerebroside (GC), and GA alone, each emulsified in adjuvant. All except the GA‐treated group developed signs of EAN between days 11 and 14 after the injection. Rats immunized with P2 alone were the most severely affected. Rats given P2 plus GA and those given P2 plus GC displayed a significantly lower clinical score. Histological analysis revealed a comparable degree of inflammation of the peripheral nervous system and demyelination in the spinal nerve roots of bovine peripheral nerve‐and P2‐immunized rats. The P2 plus GA and P2 plus GC groups revealed similar degrees of pathology in the spinal nerve roots but the latter group stood apart from the rest in that it showed widespread peripheral nervous system changes extending distally into the sciatic nerve. Serological analysis demonstrated that P2 and GC, but not GA, elicited antibody (IgG) responses, but there was no correlation between antibody titer and clinical or histological involvement. The present data fail to support an enhancing role for gangliosides in the expression of EAN and, by extrapolation, in the Guillain‐Barré syndrome, for which EAN serves as the laboratory model, and in which suggestions have been made that antibodies to GA may have pathogenetic significance.
Mitochondrial DNA sequence analysis in congenital myotonic dystrophy
Authors: Thyagarajan D. , Byrne E. , Noer S. , Lertrit P. , Utthanophol P. , Kapsa R. , Marzuki S. 
Published: January 2005

AbstractMitochondrial genetic modifying factors have been suspected in several autosomally inherited diseases. The congenital variant of myotonic dystrophy, in which there is a striking maternal inheritance pattern, is a likely candidate disease. To investigate this possibility, we sequenced completely the mitochondrial genome in 2 patients with congential myotonic dystrophy. Comparison of the two sequences with control data failed to reveal a specific nucleotide variant or length variant in this disease. We conclude that a mitochondrial genetic modifying factor is not present in congenital myotonic dystrophy.
Excitatory amino acid binding sites in the caudate nucleus and frontal cortex of huntington's disease
Authors: Dure Leon S. , Young Anne B. , Penney John B. 
Published: January 2005

AbstractHuntington's disease is a dominantly inherited, progressive neurodegenerative disorder causing marked pathology in the basal ganglia. The pathophysiology of the selective neuronal death in as yet unknown, but evidence suggests that the neurotoxicity may result from endogenous substances acting at excitatory amino acid receptors. Previous data have shown a selective decrease in binding to one class of glutamate receptors, the N‐methyl‐D‐aspartate (NMDA) receptor in the putamen of Huntington's disease. The present study was undertaken to determine the relative density of binding to all of the currently defined subpopulations of excitatory amino acid receptors in the caudate nuclei and frontal cortex of patients with Huntington's disease and of control subjects, using quantitative in vitro autoradiography. NMDA, MK‐801, glycine, kainate, and α‐amino‐3‐hydroxy‐5‐methylisoxazole propionic acid (AMPA) receptor binding were all decreased to a similar extent (50–60°). Binding to the metabotropic quisqualate receptor and to the non‐NMDA, nonkainate, nonquisqualate (NNKQ) site was decreased nonsignificantly by 31° and 26°, respectively. Autoradiograms of NMDA, MK‐801, AMPA, kainate, metabotropic, and NNKQ receptors in caudates revealed an inhomogeneous pattern of binding that is different from the binding pattern seen in control caudates. Binding to all receptor subtypes was the same in the frontal cortex from Huntington's disease patients and control subjects. The data suggest that no single excitatory amino acid receptor is selectively decreased in the caudate of Huntington's disease.
Changes of muscarinic cholinergic binding by lymphocytes in parkinson's disease with and without dementia
Authors: Rabey José Martin , Grynberg Elisheva , Graff Eran 
Published: January 2005

AbstractWe compared the muscarinic cholinergic binding in lymphocytes of 44 patients with idiopathic Parkinson's disease with 23 age‐matched normal volunteers, using [3H]quinuclidinyl benzilate. In 24 patients with Parkinson's disease without dementia, binding was normal in 12, below control values in 6, whereas the remaining 6 (all on anticholinergic medication) showed very high binding. In all 20 patients with Parkinson's disease and with dementia, the binding was below control levels, indicating that in these patients, as in patients with Alzheimer's dementia, the cholinergic muscarinic binding by lymphocytes is reduced.
Relapsing and remitting human immunodeficiency virus—associated leukoencephalomyelopathy
Authors: Berger Joseph R. , Tornatore Carlo , Major Eugene O. , Bruce Jocelyn , Shapshak Paul , Yoshioka Masaru , Houff Sydney , Sheremata William , Horton Gerald F. , Landy Howard 
Published: January 2005

AbstractWe describe a 33‐year‐old homosexual man with a steroid‐responsive, remitting and relapsing leukoencephalopathy associated with recent human immunodeficiency virus type 1 (HIV‐1) seroconversion. Biopsy of a parieto‐occipital lesion revealed demyelination and astrogliosis with focal necrosis. Detailed investigations demonstrated no pathogens in the brain other than HIV‐1. This patient illustrates that a neurological disorder clinically indistinguishable from multiple sclerosis may be the presenting manifestation of HIV‐1 infection and may occur in the absence of clinically significant immunosuppression.
Neurofilament and neural cell adhesion molecule immunocytochemistry of Huntington's disease striatum
Authors: Nihei Kuninobu , Kowall Neil W. 
Published: January 2005

AbstractWe examined normal and Huntington's disease (HD) human striatum with specific monoclonal antibodies to nonphosphorylated (SMI 32) and phosphorylated (SMI 31) neurofilament and neural cell adhesion molecule (NCAM). SMI 32 identifies medium‐sized neuronal perikarya and dendrites in normal striatum. Axons are not immunoreactive. In high‐grade HD striatum (grades 3 and 4) SMI 32 neurons are morphologically abnormal and significantly depleted. Dendritic arbors are intensely immunoreactive, tortuous, and fragmented, especially in the subependymal zone. Proliferative SMI 32–positive sprout‐like structures and axon‐like processes are seen. SMI 31 normally stains a fine meshwork of axon‐like processes that become intensely immunoreactive, condensed, convoluted, and fragmented in HD. NCAM staining is minimal in normal striatum, but, in HD striatum, many dot‐ and thread‐like structures are found, especially in the subependymal region. The abnormalities revealed by SMI 31, SMI 32, and NCAM suggest that neurofilament phosphorylation is altered and growth related proteins are reexpressed in HD. Dephosphorylation and destabilization of the cytoskeleton may contribute to neuronal injury and death in HD.
Breathing disorders during sleep in myasthenia gravis
Authors: Quera‐Salva M. A. , Guilleminault C. , Chevret S. , Troche G. , Fromageot C. , McCann C. Crowe , Stoos R. , de Lattre J. , Raphael J. C. , Gajdos Ph. 
Published: January 2005

AbstractTwenty consecutive patients (16 women and 4 men), with a mean age of 40 years, who were diagnosed and treated for myasthenia gravis were enrolled in a prospective investigation aimed at determining the amount of respiratory disturbance occurring during sleep while they received treatment. Patients were clinically evaluated to determine body mass index, presence of upper airway anatomical abnormalities, level of functional capacity and activity scored from 1 to 5, and presence of sleep‐related complaints. They underwent daytime pulmonary function tests, determination of maximal static inspiratory pressure, measurement of transdiaphragmatic pressure, and measurement of arterial blood gas levels. Polygraphic monitoring during sleep, evaluating respiration and oxygen saturation, was also performed. Results indicated that in the studied population, all subjects had evidence of daytime diaphragmatic weakness as demonstrated by transdiaphragmatic pressure measurements, independent of the degree of autonomy and functional capacity and activity level reached. Older patients with moderately increased body mass index, abnormal total lung capacity, and abnormal daytime blood gas concentrations were the primary candidates for development of diaphragmatic sleep apneas and hypopneas, and oxygen desaturation of less than 90% during sleep. However, these clear indicators were not found in all subjects with sleep‐related disordered breathing. Rapid‐eye‐movement sleep was the time of highest breathing vulnerability during sleep. Sleep‐related complaints may also help identify subjects at risk for abnormal breathing during sleep, even when daytime functional activity is judged normal.
The transcranial doppler appearance of acute carotid artery occlusion
Authors: Giller Cole A. , Mathews Dana , Purdy Phillip , Kopitnik Thomas A. , Batjer H. Hunt , Samson Duke S. 
Published: January 2005

AbstractTranscranial Doppler ultrasound is a noninvasive technique of cerebral blood velocity measurement that is being increasingly used to detect untoward hemodynamic changes. In this report, we describe striking transcranial Doppler ultrasound changes occurring in 2 patients after cervical carotid artery occlusion that led to cerebral infarction. These changes consist of the sudden appearance of a Doppler signal of extremely low pulsatility and moderately high velocity in the middle cerebral artery distribution. In 1 patient, this signal was the first sign of carotid occlusion. We suggest that these transcranial Doppler ultrasound changes can provide an early warning of carotid artery compromise.
Familial periodic cerebellar ataxia: A problem of cerebellar intracellular pH homeostasis
Authors: Bain P. G. , O'Brien M. D. , Keevil S. F. , Porter D. A. 
Published: January 2005

AbstractSix affected members of two families with familial periodic cerebellar ataxia were relieved of their symptoms with oral acetazolamide. Phosphorus 31 (31P) nuclear magnetic resonance spectroscopy showed abnormal intracellular pH levels in the cerebellum of all subjects when they were not treated. These levels returned to normal with treatment. Cerebral pH values were also measured in one family and were normal before and after treatment. An additional 3 patients with similar attacks, but without a family history, had normal untreated pH values in the cerebellum and cerebrum.
Infantile spasms: II. Lenticular nuceli and brain stem activation on positron emission tomography
Authors: Chugani Harry T. , Shewmon D. Alan , Sankar Raman , Chen Benjamin C. , Phelps Michael E. 
Published: January 2005

AbstractInfantile spasms are generalized sezures specific to early infancy, and are believed to result from complex cortical‐subcortical interactions during a critical period of development. We used positorn emission tomography (PET) to determine local cerebral metabolic rates for glucose (lCMRGlc) in 44 infants with spasms, in an attempt to define the neuroanatomical substrates that mediate these seizures. All infants were studied in the awake state during continuous electroencephalographic monitoring. The most consistent abnormality on PET, seen in 32 infants, was the symmetrical increase in lCMRGlc in the lenticular nuclei, compared to age‐matched normal infants (p < 0.05). In 21 infants, even though the brain stem appeared to be visually more prominent compared to normal infants, statistically significant differences could not be demostrated. Relative hypermetabolism of the lenticular nuceli (1) occured irrespective of whether the spasms were cryptogenic or symptomatic, (2) was associated with focal cortical hypometabolism in 22 and focal cortical hypermetabolism in 5 of the 44 infants, and (3) was not characterized by any specific electroencephalographic abnormality during PET. These findings suggest that the lenticular nuclei may contribute to the pathophysiological state that predisposes to infantile spasms, and is consistent with the observation that spasms are clinically symmetrical even when focal cortical lesions are present. A scheme describing the neuronal circuitry likely to be involved in the generation of infantile spasms is proposed.
The chiari type I malformation in two monozygotic twins and first‐degree relatives
Authors: Stovner Lars Jacob , Cappelen Johan , Nilsen Gunnar , Sjaastad Ottar 
Published: January 2005

AbstractThe presence of the Chiari type I malformation in 2 adult monozygotic female twins, their mother, and possibly in 2 of their 4 daughters is reported. The diagnosis was made by magnetic resonance imaging and confirmed at the time of surgery in 1 twin. Monozygosity of the twins was proved by DNA typing. The disorder in the present family should probably be classified together with the autosomal dominant craniocervical malformations. Nongenetic factors also seem to be important because the twins were discordant for the extent of herniation of the cerebellar tonsils and the presence of syringmyelia.
The spectrum of neurological disease in patients with systemic cancer
Authors: Clouston Paul D. , DeAngelis Lisa M. , Posner Jerome B. 
Published: January 2005

AbstractTo ascertain the range of neurological problems in patients with systemic cancer, we prospectively evaluated neurological sysmptoms, neurological diagnoses, and primary tumors in all patients with a history of systemic cancer examined by the Department of Neurology at the Memorial Sloan–Kettering Cancer Center, from Jul 1, 1990, to Dec 31, 1990. Of the 851 patients seen for neurological symptoms, less than half (45.2%) had metastatic involvement of the nervous system. The three most common symptoms were back pain (18.2%), altered mental status (17.1%), and headache (15.4%). The most common neurological diagnosis was brain metastasis (15.9%), followed by metabolic encephalopathy (10.2%), pain associated with bone metastases only (9.9%), and epidural extension or metastasis of tumor (8.4%). Of 133 patients with undiagnosed back or neck pain, 44 (33%) had epidural extension or metastases from tumor and 40 (30%) had pain associated with vertebral metastases only. In 15 (11%) the cause for the back pain was unrelated to metastatisc disease. Of 132 patients seen on initial consultation for altered mental status, metabolic encephalopathy was the major neurological diagnosis (80; 61%); 20 (15%) had intracranial metastases. Of 97 patients with undiagnosed headache, 59 (61%) had a nonstructural cause. Fifty‐three of these patients had either migraine, tension headache, or headache related to systemic illness (e.g., fever, sepsis). These results indicate that even in patients with systemic cancer, a group particularly prone to developing neurological disease that can be diagnosed radiologically, the role of clinicians remains important in helping distinguish noncancer‐related and nonmetastatisc neurological problems.
“Resetting” of postural tremors at the wrist with mechanical stretches in Parkinson's disease, essential tremor, and normal subjects mimicking tremor
Authors: Britton T. C. , Thompson P. D. , Day B. L. , Rothwell J. C. , Findley L. J. , Marsden C. D. 
Published: January 2005

AbstractThe response of postural wrist tremors to brief mechanical displacements was compared in two groups of patients, one with classical hereditary essential tremor (n = 18) and another with typical Parkinson's disease (n = 13). These groups were compared with an additional group of normal subjects mimicking wrist tremor (n = 9). The degree to which brief mechanical displacements of the wrist produced by torque pulses of three different sizes could modulate the timing of rhythmic electromyographic bursts in the forearm flexor muscles was quantified by deriving a resetting index, which could range between 0 (no phase resetting) and 1 (complete phase resetting). In all three groups of subjects studied, the resetting index varied significantly with the size of the mechanical perturbation and, in an inverse fashion, with the ongoing tremor amplitude. When due allowance for these factors was made, the difference in mean resetting indexes between the three groups of patients and subjects was reduced to the extent that no definitive statement could be made as to whether brief mechanical perturbations had more effect on essential tremor than parkinsonian tremor. The method is therefore unlikely to be useful in differentiating the common causes of postural wrist tremors.
Hypervolemic therapy prevents volume contraction but not hyponatremia following subarachnoid hemorrhage
Authors: Diringer Michael N. , Wu Katherine C. , Verbalis Joseph G. , Hanley Daniel F. 
Published: January 2005

AbstractHyponatremia is common following subarachnoid hemorrhage and has alternatively been attributed to either the inappropriate secretion of antidiuretic hormone or natriuresis causing intravascular volume contraction. We prospectively studied body sodium and intravascular volume regulation in 19 patients, beginning within 3 days after acute aneurysmal subarachnoid hemorrhage occurred, in order to determine the impact of hypervolemic therapy on both hyponatremia and volume contraction and to ascertain whether humoral factors account for hyponatremia. Serial measurements of plasma arginine vasopressin, atrial natriuretic factor, renin activity, aldosterone, and catecholamines were correlated with body sodium and fluid balance, change in blood volume, serum sodium concentration, and osmolality. Six patients (32%) developed hyponatremia, but only 2 had a negative sodium balance. In most patients, levels of atrial natriuretic factor were elevated, while plasma renin activity and aldosterone concentrations were generally suppressed. Plasma arginine vasopressin levels were not suppressed during hypo‐osmolality and did not correlate with serum osmolality in hyponatremic patients. Only 1 patient had a decrease in blood volume, which was associated with marked rises in aldosterone and plasma renin activity, but normal serum sodium and plasma atrial natriuretic factor levels. We conclude that following subarachnoid hemorrhage: (1) Hypervolemic therapy prevents volume contraction but not hyponatremia, (2) humoral factors may favor both sodium loss and water retention, and (3) arginine vasopressin regulation is disturbed and may contribute to hyponatremia.
Selective D2 receptor stimulation induces dyskinesia in parkinsonian monkeys
Authors: Luquin M. Rosario , Laguna Janeth , Obeso Jose A. 
Published: January 2005

AbstractStimulation of D1 striatal receptors has been proposed as the main mechanism mediating levodopa‐induced dyskinesia in Parkinson's disease. We used (+)‐PHNO, a selective D2 agonist, as the only treatment in 6 cynomolgus monkeys made parkinsonian by repeated 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine administration. All animals developed choreic dyskinesia after a mean treatment period of 12.8 days (range, 1–29). Administration of the D1 antagonist SCH‐23390 1 hour before administration of (+)‐PHNO did not change the dyskinesia. These results indicate that drug‐induced dyskinesia in a primate model of Parkinson's disease is not solely induced by D1 receptor activation.
Ascorbat neurotoxicity in cortical cell culture
Authors: Hisanaga Kinya , Sagar Stephen M. , Sharp Frank R. 
Published: January 2005

AbstractAscorbate (vitamin C)is believed to act as a neuromodulator that facilitates the release of neurotransmitters and inhibits neurotransmitter binding to receptors, including dopamine and N‐methyl‐D‐aspartate receptors. Extracellular levels of ascorbate are known to reach the low millimolar range after ischemic brain injury. This study shows that treatment of cultured cortical neurons with micromolar to low millimolar ascorbate first inhibits total protein synthesis and then results in late neuronal death. Astrocytes are much less vulnerable to ascorbate than neurons. Ascorbate may exacerbate neuronal and glial damage after brain ischemia, and it may play a pathological role in other neurological diseases.
Cholinesterases in the amyloid angiopathy of Alzheimer's disease
Authors: Mesulam Marsel , Carson Keith , Price Bruce , Geula Changiz 
Published: January 2005

AbstractVessels affected by amyloid angiopathy in patients with Alzheimer's disease also displayed intense acetylcholinestearase and butyrylcholinesterase activity when examined by light and electron microscopy. The enzymatic properties of the vessel‐bound cholinesterases were identical to those of the cholinesterases associated with senile plaques and neurofibrillary tangles. This cholinesterase activity is of unknown origin but represents one of the very few features common to all the major pathological markers of Alzheimer's disease.
Duplication of part of chromosome 17 is commonly associated with hereditary motor and sensory neuropathy type I (Charcot‐Marie‐Tooth disease type 1)
Authors: Hallam P. J. , Harding A. E. , Berciano J. , Barker D. F. , Malcolm S. 
Published: January 2005

AbstractHereditary motor and sensory neuropathy type I (HMSNI), also known as Charcot‐Marie‐Tooth disease type 1 (CMT1), has been shown to be genetically heterogeneous. A major gene maps to chromosome 17 (CMT1A). A set of loci, D17S122, D17S125, and D17S124, show tight linkage to the CMT1A locus, and a duplication of D17S122 has been detected in some families. We show that the locus D17S122 is duplicated in affected individuals from 7 informative families with HMSNI. The duplication was demonstrated either by differences in hybridization densities between two bands of a restriction fragment length polymorphism or by the presence of all three alleles. No normal individual had the duplication. A single recombinant exists between the MspI polymorphism of D17S122 and the duplicated band, suggesting that the duplication is of considerable size. Patients with HMSN type II do not show the duplication. These findings will have considerable impact on the diagnosis of chronic demyelinating neuropathies, in patients with or without similarly affected relatives.