bluuebunny/crossref_metadata_2025_split huggingface dataset:
Vasoactive peptide release in the extracerebral circulation of humans during migraine headache
Authors: Goadsby P. J. , Edvinsson L. , Ekman R. 
Published: January 2005

AbstractThe innervation of the cranial vessels by the trigeminal nerve, the trigeminovascular system, has recently been the subject of study in view of its possible role in the mediation of some aspects of migraine. Since stimulation of the trigeminal ganglion in humans leads to facial pain and flushing and associated release of powerful neuropeptide vasodilator substances, their local release into the extracerebral circulation of humans was determined in patients who had either common or classic migraine. Venous blood was sampled from both the external jugular and the cubital fossa ipsilateral to the side of headache. Plasma levels of neuropeptide Y, vasoactive intestinal polypeptide, substance P, and calcitonin gene‐related peptide were determined using sensitive radioimmunoassays for each peptide, and values for the cubital fossa and external jugular and a control population were compared. A substantial elevation of the calcitonin gene‐related peptide level in the external jugular but not the cubital fossa blood was seen in both classic and common migraine. The increase seen in classic migraine was greater than that seen with common migraine. The other peptides measured were unaltered. This finding may have importance in the pathophysiology of migraine.
Relapse of infant botulism
Authors: Glauser Tracy A. , Maguire Henry C. , Sladky John T. 
Published: January 2005

AbstractWe report on 3 infants who had relapse of infant botulism after apparent resolution of clinical symptoms. This group represented 5% of the infants with confirmed infant botulism who were treated at our institution since 1976. The exact cause for these relapses was unclear, but three potential mechanisms are examined. There were no historical, clinical, or electrophysiological predictors of relapse. Although at the time of writing recovery from relapse appeared complete, close follow‐up of patients recovering from a bout of infant botulism is necessary.
Myasthenic thymus and thymoma are selectively enriched in acetylcholine receptor–reactive T cells
Authors: Sommer N. , Willcox N. , Harcourt G. C. , Newsom‐Davis J. 
Published: January 2005

AbstractWe compared T‐cell proliferative responses to acetylcholine receptor (AChR) and to purified protein derivative (PPD) (of tuberculin) of hyperplastic thymus, thymoma, and blood cells from patients with myasthenia gravis (MG). Hyperplastic MG thymus cells gave significantly higher and more consistent responses to AChR than parallel cultures of autologous blood cells, whereas responses to PPD showed an opposite trend. Thus there was a preferential localization of AChR‐‐reactive T cells in the hyperplastic MG thymus. Furthermore, there was a strong correlation between blood and thymus cell responses to PPD (but not to AChR), arguing that the hyperplastic MG thymus contains a sample of sesitized peripheral T cells. By contrast, both AChR–and PPD–responsive T cells were almost undetectable in thymus from nonmyasthenic patients, which is evidently much less receptive to circulating T cells. Cells from MG thymomas showed the highest stimulations by AChR but did not consistently react to PPD. However, the uninvolved thymus adjacent to these thymomas behaved almost identically to the hyperplastic samples described above. Our interpretation is that AChR‐specific T cells are initially sensitized in the MG thymoma but are selectively trapped in the hyperplastic thymus after being primed elsewhere.
Substantia nigra: A site of action of muscle relaxant drugs
Authors: Turski Lechoslaw , Klockgether Thomas , Schwarz Michael , Turski Waldemar A. , Sontag Karl‐Heinz 
Published: January 2005

AbstractSites of action of centrally active muscle relaxant drugs are not well defined. Clinical experience with such drugs suggests that the spinal cord may be one of the important regions from which pathologically increased muscle tone may be relieved. Supraspinal centers that may also be involved in the expression of muscle relaxant action have not yet been defined. We report here that microinjections of therapeutically relevant muscle relaxants into the midbrain tegmentum of genetically spastic rats decrease muscle tone. The substantia nigra is the region from which midazolam, baclofen, and tizanidine (drugs used clinically in the treatment of spasticity), or gamma‐vinyl‐GABA, (−)‐2‐amino‐7‐phosphonoheptanoate, and [D‐pro2‐D‐phe7‐D‐trp9]‐substance P (experimental drugs active in animal models of spasticity), reduce muscle tone in genetically spastic rats and Hoffmann reflexes in normal rats. The effects of muscle relaxant drugs are topographically restricted to the substantia nigra pars reticulata and are receptor specific. These observations disclose a previously unknown function of the substantia nigra in mediating muscle relaxation.
Spinal cord compression with paraplegia in xanthomatosis due to normocholesterolemic sitosterolemia
Authors: Haranaka I. , Yasuda H. , Hidaka H. , Harada N. , Kobayashi M. , Okabe H. , Matsumoto K. , Hukuda S. , Shigeta Y. 
Published: January 2005

AbstractA 48‐year‐old woman with a 30‐year history of tendinous xanthomatosis developed paraplegia. The magnetic resonance image revealed extramedullary tumors. The analysis of her sera and tumors revealed increased amount of plant sterols, especially sitosterol, and the diagnosis of sitosterolemia was made. This is the first reported case of a patient with sitosterolemia who had the neurological complication of spinal cord compression due to extramedullary sitosterolemic xanthomas.
α1‐Antichymotrypsin as a possible biochemical marker for Alzheimer‐type dementia
Authors: Matsubara Etsuro , Hirai Shunsaku , Amari Masakuni , Shoji Mikio , Yamaguchi Haruyasu , Okamoto Koichi , Ishiguro Koji , Harigaya Yasuo , Wakabayashi Katsumi 
Published: January 2005

AbstractWe measured serum α1‐antichymotrypsin levels in 38 patients with Alzheimer‐type dementia, 89 control subjects, 2 subjects with Down's syndrome, 20 with vascular dementia, 18 with Parkinson's disease, 14 with spinocerebellar degeneration, 15 with cerebrovascular disease without dementia, and 14 with Duchenne muscular dystrophy. Cerebrospinal fluid (CSF) levels of α1‐antichymotrypsin were also measured in 15 patients with Alzheimer‐type dementia, 26 control subjects, 6 with vascular dementia, 7 with cerebrovascular disorder, and 11 with degenerative disorders. In control subjects, there were no age‐related changes or sex differences. Serum and CSF levels were significantly and specifically higher in patients with Alzheimer‐type dementia than in other subjects (serum, p <0.001; CSF, p <0.05). Serum levels of α1‐antichymotrypsin were significantly elevanted in the early stage of Alzheimer‐type dementia, whereas there was no definite correlation between serum levels and the degree of dementia. CSF levels of α1‐antichymotrypsin tended to parallel the severity of dementia. Serum levels were not correlated with CSF levels. These data indicate that serum and CSF levels of α1‐antichymotrypsin might be independently upregulated in Alzheimer‐type dementia. We concluded that the measurement of serum levels of α1‐antichymotrypsin could be useful as a screening marker for Alzheimer‐type dementia. In addition, CSF levels also could be a useful marker for Alzheimer‐type dementia, because they might reflect the state of dementia.
Normal dystrophin in McLeod myopathy
Authors: Danek Adrian , Witt Thomas N. , Stockmann H. B. A. C. , Weiss Barbara J. , Schotland Donald L. , Fishbeck Kenneth H. 
Published: January 2005

AbstractDystrophin and its gene were studied in a patient with McLeod syndrome. This X‐linked recessive myopathy has been localized to Xp21, as the Duchenne muscular dystrophy gene locus, which codes for dystrophin. Histopathological study of the patient's muscle showed mild subclinical myopathy. Immunological studies of dystrophin in two separate biopsy specimens and analysis of dystrophin gene DNA from a blood sample did not detect and abnormality. This suggests that the Duchenne muscular dystrophy gene, albeit close to the McLeod locus, is not involved in McLeod myopathy.
The functional anatomy of motor recovery after stroke in humans: A study with positron emission tomography
Authors: Chollet F. , Di Piero V. , Wise R. J. S. , Brooks D. J. , Dolan R. J. , Frackowiak R. S. J. 
Published: January 2005

AbstractWe have studied regional cerebral blood flow changes in 6 patients after their recovery from a first hemiplegic stroke. All had a single well‐defined hemispheric lesion and at least a brachial monoparesis that subsequently recovered. Each patient had 6 measurements of cerebral blood flow by positron tomography with 2 scans at rest, 2 during movement of fingers of the recovered hand, and 2 during movement of fingers of the normal hand. When the normal fingers were moved, regional cerebral blood flow increased significantly in contralateral primary sensorimotor cortex and in the ipsilateral cerebellar hemisphere. When the fingers of the recovered hand were moved, significant regional cerebral blood flow increases were observed in both contralateral and ipsilateral primary sensorimotor cortex and in both cerebellar hemispheres. Other regions, namely, insula, inferior parietal, and premotor cortex, were also bilaterally activated with movement of the recovered hand. We have also demonstrated, by using a new technique of image analysis, different functional connections between the thalamic nuclei and specific cortical and cerebellar regions during these movements. Our results suggest that ipsilateral motor pathways may play a role in the recovery of motor function after ischemic stroke.
Acceleration of scrapie in trisomy 16↔diploid aggregation chimeras
Authors: Epstein Charles J. , Foster Dallas B. , DeArmond Stephen J. , Prusiner Stanley B. 
Published: January 2005

AbstractWe studied the susceptibility to prion infection of the trisomy 16↔diploid chimeric mouse, a putative model of Down syndrome. When weanling chimeras were inoculated intracerebrally with scrapie prions, the time until appearance of the first symptoms of scrapie was reduced by 17 days (from a mean control time of 153 days) and the time to death was reduced by 30 days (from control time of 170 days). Our results with trisomy 16 chimeras argue that the susceptibility to central nervous system degeneration caused by prions can be modulated by chromosome imbalance.
Hippocampal sclerosis in temporal lobe epilepsy demonstrated by magnetic resonance imaging
Authors: Berkovic Samuel F. , Andermann Frederick , Olivier André , Ethier Roméo , Melanson Denis , Robitaille Yvon , Kuzniecky Ruben , Peters Terence , Feindel William 
Published: January 2005

AbstractThe value of magnetic resonance imaging in the detection of hippocampal sclerosis has been controversial. We studied 10 patients aged 22.5 ± 6.0 years with intractable temporal lobe epilepsy selected because of a history of a prolonged childhood convulsion, which is characteristic of a group of patients in whom hippocampal sclerosis is a constant finding. All 10 patients showed reduction in size of one hippocampus associated with increased signal intensity on T2‐weighted magnetic resonance images. These changes were reliably detected on coronal spin‐echo images, perpendicular to the long axis of the hippocampus. Appreciation of the normal imaging anatomy of the hippocampus allowed correct interpretation of the relative changes in signal intensities of the hippocampus and adjacent temporal horn on sequential echo images. The side of the abnormal hippocampus on magnetic resonance imaging accorded with the electroencephalographic localization in all 10 patients, and with the lateralization of the early convulsions in all 6 patients where this was known. Temporal lobectomy was performed in all 10 patients. Hippocampal sclerosis was confirmed in the 3 patients in whom hippocampal tissue was available for histological examination. The value of this technique was reinforced by the excellent postoperative results, with 80% being seizure free at a mean follow‐up time of 33 ± 4 months.
Loss of high‐affinity agonist binding to M1 muscarinic receptors in Alzheimer's disease: Implications for the failure of cholinergic replacement therapies
Authors: Flynn Donna D. , Weinstein David A. , Mash Deborah C. 
Published: January 2005

AbstractCholinergic replacement therapies have yielded little or no clinical improvement in Alzheimer's disease (AD). Since the number of postsynaptic muscarinic receptors remains unchanged in the cerebral cortex, the involvement of other neurotransmitter systems may account for this limited efficacy. Alternatively, there may be a defective coupling of the muscarinic receptor with its nucleotide‐binding protein in AD, which would severely limit the ability of cholinergic agonists to activate intracortical second messengers. To address this possibility, we assessed the ability of the putative M1 muscarinic receptor to form high‐affinity agonist‐receptor complexes with guanine nucleotide regulatory proteins in postmortem frontal cortex. Agonist affinity states of the M1 muscarinic receptor were measured by carbachol/[3H]‐pirenzepine competition. M1 muscarinic receptors exhibited both high (KH) and low (KL) affinities for the agonist carbachol. High‐affinity agonist binding to M1 receptors in postmortem frontal cortex samples from subjects with AD was reduced, demonstrated by an increase in the KH value. Low‐affinity agonist binding (KL value) was unchanged in AD and was not significantly different from the KL value for the uncoupled receptor determined in the presence of guanine nucleotides. The increase in the KH value resulted in a 70% decrease in the average KL/KH ratio for AD as compared to control samples. Choline acetyltransferase activities correlated significantly with the KL/KH ratios (r = 0.73,p < 0.001). These data suggest that the KL/KH ratio for muscarinic agonists may serve as a neurochemical marker of disease severity. The reduced ability of the M1 receptor subtype to form a high‐affinity agonist state in AD may account for the failure of cholinergic replacement therapies to improve specific features of memory and cognition.
Abnormal cerebral glucose metabolism in long‐term survivors of childhood acute lymphocytic leukemia
Authors: Phillips Peter C. , Moeller James R. , Sidtis John J. , Dhawan Vijay , Steinherz Peter G. , Strother Steven C. , Ginos James Z. , Rottenberg David A. 
Published: January 2005

AbstractChemotherapy and radiation treatment of the central nervous system may cause delayed neurotoxicity in children with acute lymphocytic leukemia. We evaluated 12 long‐term survivors of childhood leukemia using [18F]fluorodeoxyglucose positron emission tomography, computed tomography scans, clinical neurological examinations, and neuropsychological tests. Regional cerebral metabolic rate for glucose (rCMRGIc) values for white matter were lower in the older long‐term survivors (> 18 years old) treated with cranial radiation and intrathecal chemotherapy than in normal control subjects or survivors who had been treated with intrathecal chemotherapy alone. The ratio of white matter: cortex rCMRGlc values was lower than control values in the long‐term survivors treated with cranial radiation and intrathecal chemotherapy, regardless of age, but not in those treated with intrathecal chemotherapy alone. By contrast, thalamic rCMRGlc values were lower than control values in older survivors regardless of treatment, and the ratio for thalamus: cortex rCMRGIc values was lower in all the treatment groups than in control subjects. The highest rCMRGIc values were found in the youngest children, indicating an important effect of age on cerebral glucose metabolism. No neuropsychological deficits were identified in patients treated only with intrathecal chemotherapy; however, lower IQ scores were found in the long‐term survivors who had been treated with cranial radiation and intrathecal chemotherapy. Treatment of the central nervous system with cranial radiation and intrathecal chemotherapy may cause prolonged alterations in white‐matter and thalamic rCMRGIc, which may permit the identification and assessment of neurotoxicity in long‐term survivors of acute lymphocytic leukemia by [18F]fluorodeoxyglucose positron emission tomography.
A genetic study of idiopathic focal dystonias
Authors: Waddy H. M. , Fletcher N. A. , Harding A. E. , Marsden C. D. 
Published: January 2005

AbstractA genetic study of idiopathic focal dystonias was undertaken by examining 153 first‐degree relatives of 40 index patients with torticollis (14 patients), other focal cranial dystonias (16 patients), and writer's cramp (10 patients). Nine relatives with dystonia were identified in 6 families; 8 of these had symptoms such as clumsiness or tremor, but none were aware of any dystonia. A further 4 relatives, now deceased, were affected by history. Overall, 25% of index patients had relatives with dystonia. The results of segregation analysis suggested the presence of an autosomal dominant gene or genes with reduced penetrance as a common cause for focal dystonia. Segregation ratios were not significantly different from those ratios observed in generalized or segmental dystonia in the United Kingdom, and it is possible that a single autosomal dominant gene mutation is responsible for inherited dystonia in the majority of patients irrespective of distribution or severity.
Mitochondrial DNA deletions in inherited recurrent myoglobinuria
Authors: Ohno Kimji , Tanaka Masashi , Sahashi Ko , Ibi Tohru , Sato Wataru , Yamamoto Tomoko , Takahashi Akira , Ozawa Takayuki 
Published: January 2005

AbstractWe describe two brothers with inherited recurrent exertional myoglobinuria and alcohol intolerance associated with distinct morphological abnormalities of muscle mitochondria and multiple deletions of muscle mitochondrial DNA. Patient 1 (26 years old) and patient 2 (21 years old) had recurrent episodes of myoglobinuria provoked by strenuous exercise or alcohol intake, from the age of 18 years. Although their serum lactate and pyruvate levels were normal at rest, they were significantly elevated by aerobic exercise. Histochemistry of their biopsied limb muscles showed ragged‐red fibers and cytochrome c oxidase–negative fibers as well as degenerating and regenerating fibers. Electron microscopy showed pronounced accumulation of abnormal mitochondria containing paracrystalline inclusions and moderate increases of glycogen particles. The enzyme activities of the electron‐transfer complexes in the isolated muscle mitochondria of patient 2 were within normal ranges. Southern blot analysis revealed multiple deletions of mitochondrial DNA, some of which were common between the patients. Polymerase chain reaction of their muscle mitochondrial DNA detected multiple abnormal fragments indicating mitochondrial DNA deletions. We propose that a defect of the mitochondrial energy‐transducing system due to multiple mitochondrial DNA deletions is a novel genetic cause of inherited recurrent myoglobinuria.
Late onset dementia with argyrophilic grains and subcortical tangles or atypical progressive supranuclear palsy?
Authors: Masliah Eliezer , Hansen Lawrence A. , Quijada Sylvia , Deteresa Richard , Alford Michael , Kauss Joachim , Terry Robert 
Published: January 2005

AbstractMost clinically demented elderly patients are found at autopsy to have Alzheimer's disease, multi‐infarct dementia, Parkinson's disease, Pick's disease, or Creutzfeldt‐Jakob disease. We studied 5 patients clinically characterized by late onset dementia whose brains showed no pathological evidence of Alzheimer's disease, or any other specific neuropathological diagnosis. We found argyrophilic grains, coiled bodies, abundant Alz‐50‐positive and thioflavine S‐negative neurofibrillary tangles, and neuropil threads in the hippocampus, entorhinal cortex, locus ceruleus, substantia nigra, subthalamic nucleus, and inferior olives, Ultrastructurally, the grains, threads, and tangles were composed of straight tubulofilamentous structures, 25 nm in diameter, similar to those found in patients with progressive supranuclear palsy but different from the paired helical filaments of patients with Alzheimer's disease. These findings suggest that the late onset dementia with argyrophilic grains syndrome is also characterized by the presence of tangles and threads with the topographical distribution of progressive supranuclear palsy.
Percutaneous electrical stimulation of corticospinal pathways at the level of the pyramidal decussation in humans
Authors: Ugawa Y. , Rothwell J. C. , Day B. L. , Thompson P. D. , Marsden C. D. 
Published: January 2005

AbstractStimulation over the base of the skull can activate descending motor pathways to produce electromyograpnic (EMG) responses in muscles of the arm and leg. The evoked EMG responses were larger when the muscles were preactivated by a small voluntary contraction compared to when they were completely relaxed. The latency of these responses in preactivated muscles was approximately midway between that produced by electrical stimulation over the motor cortex, and by electrical stimulation over the cervical enlargements. With horizontally spaced electrodes, the latency difference between cortical and brainstem stimulation was 1.8 milliseconds in all muscles tested. The latency difference between cervical and brainstem stimulation was 3.9 milliseconds for the first dorsal interosseous and 2.6 milliseconds for tibialis anterior muscles. These values suggest that brainstem stimulation occurs at the level of the cervicomedullary junction. With vertically spaced electrodes in the midline, stimulation often occurs at a higher level. The EMG responses from brainstem stimulation differed from those following cortical stimulation in two ways: (1) They were simpler in form, and (2) their onset latency was the same in active as it was in relaxed muscles. This suggests that brainstem stimulation evoked a large descending motor volley in comparison with the multiple volleys that cortical stimulation can produce. Collision experiments between cortical and brainstem volleys indicated that the major part of the responses evoked by brainstem stimulation were conducted via the large‐diameter component of the corticospinal tract.