bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acute motor axonal neuropathy: A frequent cause of acute flaccid paralysis in China
Authors: McKhann G. M. , Cornblath D. R. , Griffin J. W. , Ho T. W. , Li C. Y. , Jiang Z. , Wu H. S. , Zhaori G. , Liu Y. , Jou L. P. , Liu T. C. , Gao C. Y. , Mao J. Y. , Blaser M. J. , Mishu B. , Asbury A. K. 
Published: January 2005

AbstractIn northern China, annual epidemics of acute‐onset flaccid paralysis diagnosed clinically as Guillain‐Barré syndrome have been recognized for at least 20 years. On the basis of an historical analysis of more than 3,200 patients, distinctvie features include most cases occurring during the summer months among children and young adults, most of whom reside in rural areas. Of 90 patients with acute flaccid paralysis, 88 had a distinctive pattern that shares clinical and cerebrospinal fluid findings with demyelinating Guillain‐Barré syndrome, but that differs from Guillain‐Barré syndrome physiologically and pathologically. The clinical course is marked by rapidly progressive ascending tetraparesis, often with respiratory failure, but without fever, systemic illness, or sensory involvement. Cerebrospinal fluid is acellular, and elevations of protein content occur in the second or third week of illness. Electrodiagnostic studies show normal motor distal latencies and limb conduction velocities, but reduced compound muscle action potential amplitudes. Sensory nerve action potentials and, when elicitable, F waves are within the range of normal. Recovery is usually good. Autopsy studies have shown Wallerian‐like degeneration of motor fibers. These studies establish that this is a distinctive syndrome, distinguishable from poliomyelitis and demyelinating Guillain‐Barré syndrome.
Human immunodeficiency virus type 1 infection of the nervous system: Pathogenetic mechanisms
Authors: Epstein Leon G. , Gendelman Howard E. 
Published: January 2005

AbstractHuman immunodeficiency virus type 1 (HIV‐1) infection of the central nervous system is associated with characteristic virological, clinical, and neuropathological findings in adults and children. Productive infection in the brain and spinal cord occurs in blood‐derived macrophages, resident microglia, and multinucleated giant cells. Previous work implicated indirect mechanisms for neurotoxicity by HIV‐1 gene products or by factors secreted from HIV‐1‐infected macrophages. However, this cannot explain the paradox between the small numbers of infected cells and the widespread tissue pathology. Based on recent studies from our laboratories, we suggest that HIV‐1‐infected macrophages can initiate neurotoxicity, which is then amplified through cell‐to‐cell interactions with astrocytes. Macrophageastrocyte interactions produce cytokines tumor necrosis factor‐α and interleukin‐1b̃ and arachidonic metabolites that cause astroglial proliferation and neuronal injury. Inevitably, the astrogliosis serves to amplify these cellular processes while brain infection maintains itself in macrophage and microglia and possibly in astrocytes (by restricted infection). These findings, taken together, provide fresh insights into how low numbers of productively infected cells could elicit progressive and devastating neurological impairment during HIV‐1 disease, and suggest therapeutic strategies to interrupt the pathological process.
Expression of c‐fos and c‐jun family genes after focal cerebral ischemia
Authors: An Gang , Lin Teng‐Nan , Liu Jia‐Shou , Xue Juan‐Juan , He Yong‐Yue , Hsu Chung Y. 
Published: January 2005

AbstractThe expression of the protooncogenes, c‐fos, jun B, c‐jun, and jun D was investigated in a rat focal cerebral ischemia model by Northern analysis and in situ hybridization. Severe ischemia (reduction of regional blood flow by 88‐92%) in this model is confined to cerebral cortex irrigated by the right middle cerebral artery. Ischemia for 30 minutes, which caused only slight cortical damage (infarct size, <10 mm3), induced both jun B and c‐fos mRNAs exclusively in the right cerebral cortex. Ischemia for 90 minutes, which led to large cortical infarction (infarct size, >140 mm3), also induced the expression of these two genes in the right cerebral cortex as well as the ipsilateral hippocampus. The latter sustained very mild ischemia (reduction of regional blood flow by 10–20%). The coinduction of jun B and c‐fos expression occurred immediately after reperfusion and peaked at 60 minutes after reperfusion. The expression of c‐jun was enhanced in a similar pattern, but at a much lower magnitude. In contrast, no change in jun D expression was observed. Nuclear run‐on assays indicated that the increase in c‐fos, jun B, and c‐jun mRNA levels was due to the increase of transcription rate in these genes. Mobility shift assays showed a basal DNA binding activity of transcription factor AP‐1 in the right cerebral cortex. Ischemia for 30 or 90 minutes followed by reperfusion for 4 hours resulted in a four‐ to sixfold increase of AP‐1 binding activity. The enhanced DNA binding activity persisted for as long as 24 hours, These results suggest that the enhanced expression of AP‐1, a general transcription factor, may play a role in the alteration of gene regulation in the ischemic cortex.
Defecatory function in Parkinson's disease: Response to apomorphine
Authors: Edwards L. L. , Quigley E. M. M. , Harned R. K. , Hofman Ruth , Pfeiffer R. F. 
Published: January 2005

AbstractWe evaluated the effects of the dopaminergic agent apomorphine on defecation and anorectal function in patients with Parkinson's disease (PD). A gastrointestinal symptom survey, extrapyramidal assessment, defecating proctogram, and anorectal manometric study were performed in 8 subjects with PD. Basal studies showing abnormalities were repeated following apomorphine administration. Prior defecographic abnormalities were normalized following apomorphine injection in 1 of 3 subjects and significant improvements in manometric parameters were observed in all 5 subjects who underwent repeat anorectal manometry. We conclude that apomorphine can correct anorectal dysfunction in PD, and that these abnormalities may be a consequence of dopamine deficiency secondary to the PD process. These findings may also have therapeutic implications.
Experimental paraprotein neuropathy, demyelination by passive transfer of human IgM anti‐myelin‐associated glycoprotein
Authors: Tatum Arthur H. 
Published: January 2005

AbstractCirculating monoclonal IgM antibodies that react with myelin‐associated glycoprotein are strongly associated with a specific type of human peripheral nerve demyelination. There has been great interest in this syndrome because, if the paraprotein could be shown to cause the demyelination, then it would be the first proven example of antibody‐mediated demyelination in humans. Systemic transfusion of chickens with monoclonal IgM antibody isolated from one of these patients produced peripheral demyelination highly characteristic of the human syndrome. The experimental lesion consists of segmental demyelination and remyelination with minimal inflammation, specific antibody bound to myelin, and widening of the myelin lamellae. In the experimental model, antibody is concentrated in specialized myelin structures, the nodes of Ranvier, and Schmidt‐Lanterman incisures, suggesting that myelin‐associated glycoprotein may be the antigenic target in vivo. This demonstration that human myelin‐associated glycoprotein antibodies cause demyelination in vivo is the final information needed to prove that this type of human demyelination is antibody mediated. This strengthens the proposition that nerve antibodies, present in other human neurological syndromes, may also cause disease.
Use of [18F]fluorodeoxyglucose positron emission tomography in patients with primary malignant brain tumors
Authors: Janus Todd J. , Kim E. Edmund , Tilbury Roy , Bruner Janet M. , Yung W. K. Alfred 
Published: January 2005

AbstractIn patients with malignant gliomas, [18F]fluorodeoxyglucose positron emission tomography (FDG‐PET) may discriminate tumor progression from radionecrosis. We evaluated data from 50 patients undergoing FDG‐PET for suspicion of tumor progression. Forty‐nine were treated with surgery, 48 with radiotherapy, and 37 with chemotherapy. Twenty‐one had intensive radiotherapy with either three daily treatments in two 5‐day periods and intravenous carboplatin (17) or interstitial brachytherapy or stereotactic radiotherapy. Twenty underwent surgery after magnetic resonance imaging/FDG‐PET; 9 demonstrated increased uptake of FDG and evidence of tumor, whereas 6 had decreased uptake and no evidence of tumor. In 5 patients, there was no correlation (all had intensive radiotherapy). In 17 patients who received bromodeoxyuridine intravenously just before surgery, the bromodeoxyuridine labeling index corresponded to the histological appearance in all but 2 patients (both had received intensive radiotherapy). In 30 patients without surgery, decreased uptake of FDG suggested prolonged survival; increased uptake of FDG did not predict survival. Eight of 10 with intensive radiotherapy had decreased label uptake. We conclude FDG‐PET for evaluation of patients with possible recurrent tumors requires more study. In patients with intensive radiotherapy, FDG‐PET results cannot be correlated accurately with tumor progression.
Immune‐mediated demyelination
Authors: Hartung Hans‐Peter 
Published: January 2005

AbstractThe Guillain‐Barré syndrome (GBS) and multiple sclerosis (MS) are though to result from aberranr immune responses to myelin antigens. Recent evidence to implicate the cytokine tunmor necrosis factor‐α(TNF‐α) and the intercellular adhesion molecule‐1(ICAM‐1) in the pathogenesis of these disorders is reviewed. In GBS, elevared serum concentrations of TNF‐α are detectable in 20 to 50% of patients. TNF‐α released from autoreactive T cells, macrophages, or microglia may contribute to inflammatory demyelinative processes by upregulating the expression of recognition molecules on antigen‐presenting cells; by cytotoxic damage to endothlium; by stimulating the secretion of inflammatory mediators; by directly injuring the myelin sheath; or by interfering with impluse propagation. Its pathogenic potential in GBS is underscored by findings in experimental autoimmune neuritis. Soluble ICAM‐1, originating from T cells, macrophages, endothelium, or glial cells, circulates at increases concentrations in serum and cerebrospinal fluid of patients with active MS. ICAM‐1 may be crucially involved in the migration of autoreactive T lymphocytes from blood to brain. Whether ICAM‐1 can serve as a maker of acute inflammatory events in ms associated with clinical relapses warrants further investigation. TNF‐α and ICAM‐1 coud be targets for antigen nonspecific treatment approaches to the inflammatory demyelinating diseases GBS and MS.
Treatment of single brain metastasis: Radiotherapy alone or combined with neurosurgery
Authors: Vecht Charles J. , Haaxma‐Reiche Hanny , Noordijk Evert M. , Padberg George W. , Voormolen Joan H. C. , Hoekstra Foppe H. , Tans Joseph Th. J. , Lambooij Nanno , Metsaars Jan A. L. , Wattendorff A. Rolf , Brand Ronald , Hermans Jo 
Published: January 2005

AbstractMost patients treated for single or multiple brain metastases die from progression of extracranial tumor activity. This makes it uncertain whether the combination of neurosurgery and radiontherapy for treatment of single brain metastasis will lead to better results than less invasive treatment with radiotherapy alone. The effect of neurosurgical excision plus radiotherapy was compared with radiotherapy alone in a prospectively randomized trial with 63 evaluable patients with systemic cancer and a radiological diagnosis of single brain metastasis. Radiotherapy was given to the whole brain by a novel scheme of 2 faractions per day of each 2 Gy for a total of 40 Gy. Before randomization, patients were stratified by site (lung cancer vs nonlung cancer) and status of extracranial disease (progressive vs. stable). Survival as such and functionally independent survival (FIS; defined as world Health Organization performance status ≤ 1 and neurological funcition ≤ 1) were compared between both treatment arms. The combined treatment compared with radiotherapy alone led to a longer survival (p =0.04) and a longer FIS (p=0.06). This was most pronounced in patients with stable extracranial disease (median survival, 12vs 7 mo; median FIS 9 vs 4 Mo). Patients with progressive extracranial cancer had a median overall survival of 5 months and a FIS of 2.5 months irrespective of given treatment. Improvement in functional status occurred more rapidly and for longer periods of time after neurosurgial excision and radiotherapy than after radiotherpy alone. Patients older than 60 years had a hazard ratio of dying of 2.74(p=0.001) compared with younger patients, but in both age groups the combined treatment did better then radiotherapy alone. We coclude that patients with single brain metastasis and stable extracranial tumor activity should be treated with surgical excision and radiotherapy. For patients with progressive extracranial disease during the previous 3 months, radiotherapy alone appears to be sufficient. After treatment of single brain metastasis, parients remain functionally independent until a few months before death.
Evidence for mitochondrial dysfunction in patients with alternating hemiplegia of childhood
Authors: Arnold Douglas L. , Silver Kenneth , Andermann Frederick 
Published: January 2005

AbstractPhosphorus magnetic resonance spectra of resting muscle were obtained from 4 patients with alternating hemiplegia of childhood. All patients had abnormally high resonance intensities from inorganic phosphate and an abnormally law calculared cytosolic phosphorylation potential. Tow of the 4 patients had abnormally law resonance intensities from phosphocreatine and an abnormally high calculated cytosolic free adenosine diphosphare conecntration. These abnormalities are indicative of mitochondrial dysfunction. The combination of a central nervous system disorder and evidence of mitochondrial dysfunction in muscle suggests that alternating hemiplegia of childhood may represent a previously unrecognized phenotype of mitochondrial disease.
Human leukocyte antigens in Fisher's syundrome
Authors: Yuki Nobuhiro , Sato Shuzo , Tsuji Shoji , Ogawa Kimiaki , Miyatake Tadashi 
Published: January 2005

AbstractWe performed human leukocyte antigens(HLA)typing for class I antigens on 19 Japanese patients with Fisher's syndrome. We demonstrated a statistically significant association between the disease and the HLA‐B39 antigen.
Myoblast transfer in duchenne muscular dystrophy
Authors: Karpati George , Ajdukovic Djordje , Arnold Douglas , Gledhill Robert B. , Guttmann Ronald , Holland Paul , Koch Penelope A. , Shoubridge Eric , Spence Desmond , Vanasse Michel , Watters Gordon V. , Abrahamowicz Michael , Duff Catherine , Worton Ronald G. 
Published: January 2005

AbstractOne biceps muscle of 8 patients with Duchenne muscular dystrophy was injected at 55 sites with a total of 55 million viable, purified, and contamination‐free normal myoblasts (myoblast transfer). The other biceps of each patient was injected with a placebo to serve as a control. The procedure was blinded to the patients, parents, and investigators. Myoblasts derived from a biopsy specimen of the fathers were cultured and purified under strict conditions and carefully screened for microbial contamination. All patients received cyclophosphamide for immunosuppression for 6 or 12 months. No serious complications were observed after myoblast transfer, indicating that the procedure is safe. The overall therapeutic efficiency of myoblast transfer was poor as judged by the results in maximal voluntary force generation, dystrophin content of the muscle, magnetic resonance imaging of the muscle, and the lack of donor‐derived DNA and dystrophin messenger RNA in the injected muscle. An improved efficiency of the take of myoblasts might be achieved by using younger cells and injecting the myoblasts with a myonecrotic agent (to increase the prevalence of regeneration) and a basal laminal fenestrating agent.
Modulation of motor cortical outputs to the reading hand of braille readers
Authors: Pascual‐Leone Alvaro , Cammarota Angel , Wassermann Eric M. , Brasil‐Neto Joaquim P. , Cohen Leonardo G. , Hallett Mark 
Published: January 2005

AbstractWe used focal transcranial magnetic stimulation to map the motor cortical areas targeting the first dorsal interosseous and the abductor digiti minimi muscles bilaterally in 10 proficient braille readers and 10 blind controls who were matched for age (mean, 50.6 yr) and age at time of blindness (mean, 7.5 yr). The proficient braille readers had learned braille at age 8 to 14 years and used it daily for 5 to 10 hours. Controls had not learned braille until age 17 to 21 years and used it daily for <1 hour. In the controls, motor representations of the right and left first dorsal interosseous and abductor digiti minimi muscles were not significantly different. However, in the proficient braille readers, the representation of the first dorsal interosseous muscle in the reading hand was significantly larger than that in the nonreading hand or in either hand of the controls. Conversely, the representation of the abductor digiti minimi muscle in the reading hand was significantly smaller than that in the nonreading hand or in either hand of the controls. These differences were not due to differences in motor thresholds. Our results suggest that the cortical representation of the reading finger in proficient braille readers is enlarged at the expense of the representation of other fingers.
Heterogeneity of autoantibodies in stiff‐man syndrome
Authors: Grimaldi Luigi M. E. , Martino Gianvito , Braghi Simona , Quattrini Angelo , Furlan Roberto , Bosi Emanuele , Comi Giancarlo 
Published: January 2005

AbstractStiff‐man syndrome is a rare neurological disorder characterized by skeletal muscle rigidity and spasms in which detection of circulating anti–glutamic acid decarboxylase antibodies has suggested an autoimmune pathogenesis. To further define the role of autoimmunity in the pathogenesis, we studied anti–glutamic acid decarboxylase antibodies, as well as organ‐ and non–organ‐specific autoantibodies in 13 patients with stiff‐man syndrome and 127 patients with other neurological disorders. Thyrogastric antibodies were more frequent in patients with stiff‐man syndrome (46%) than in those with other neurological disorders (12%) (p < 0.05). Non–organ‐specific antibodies were found at a similar frequency in the patients with stiff‐man syndrome (61%) and those with other neurological disorders (65%). Islet‐cell autoantibodies and anti–glutamic acid decarboxylase antibodies were more common in stiff‐man syndrome patients (38% and 31%) compared to the patients with other neurological disorders (6% and 3%, respectively; p < 0.001). With the exception of 1 patient in the other neurological disorders group, anti–glutamic acid decarboxylase antibodies were always associated with islet‐cell autoantibodies. Four patients with stiff‐man syndrome had an associated solid tumor: 3 of them had antibodies recognizing a 125/130‐kd protein and not glutamic acid decarboxylase. Our study indicates that with immunological markers, patients with stiff‐man syndrome can be subdivided into three groups: (1) patients with circulating islet‐cell autoantibodies, anti–glutamic acid decarboxylase as well as other organ‐specific autoantibodies, and the frequent association with an autoimmune disease (autoimmune variant); (2) patients with associated neoplasms and circulating non‐organ‐specific autoantibodies but neither islet‐cell nor anti–glutamic acid decarboxylase autoantibodies (paraneoplastic variant); and (3) patients with no evidence of any known autoantibody or association with other clinically evident diseases (idiopathic variant). We conclude that the presence of anti–glutamic acid decarboxylase antibodies is restricted to a subgroup of stiff‐man syndrome patients with associated autoimmune diseases. The absence of these antibodies does not exclude a diagnosis of stiff‐man syndrome, which is still based on clinical and neurophysiological evidence.
Positron emission tomography measures of benzodiazepine receptors in Huntington's disease
Authors: Holthoff Vjera A. , Koeppe Robert A. , Frey Kirk A. , Penney John B. , Markel Dorene S. , Kuhl David E. , Young Anne B. 
Published: January 2005

AbstractWe performed positron emission tomographic (PET) measurements of the regional distribution volume of benzodiazepine receptors and regional glucose metabolism in 6 drug‐free patients with early Huntington's disease following injection of [11C] flumazenil, a nonsubtype selective central benzodiazepine receptor antagonist, and 18F‐2‐fluoro‐2‐deoxy‐D‐glucose, respectively. Flumazenil data were analyzed with a recently developed two‐compartment, two‐parameter tracer kinetic model. Benzodiazepine receptor density is related to distribution volume for flumazenil. In comparison with a group of healthy volunteers, benzodiazepine receptor density was significantly decreased in the caudate nucleus. Glucose metabolism was significantly reduced not only in the caudate nucleus but also in the putamen and thalamus. The changes in benzodiazepine receptor density observed in the caudate nucleus are commensurate with data obtained in postmortem autoradiographic studies of receptor density. Based on such postmortem studies we also anticipated changes in putamen and thalamic benzodiazepine receptor density. However, relatively little is known on receptor changes in early Huntington's disease, because the autoradiographic data available were obtained mostly in patients with advanced disease. The decreased glucose metabolism in the caudate and putamen agrees well with previously published results of PET studies, whereas metabolic impairment of the thalamus has not yet been described in Huntington's disease. The present study suggests that regional metabolism and γ‐aminobutyric acid (GABA)‐benzodiazepine receptor changes in subcortical structures of patients with early Huntington's disease do not occur with the same time course: Caudate benzodiazepine receptor density is already severely impaired when other subcortical structures reveal only minor abnormalities. Impairment of neuronal metabolism seems to predate GABA/benzodiazepine receptor changes since the putamen and thalamus demonstrate metabolic impairment without detectable loss of benzodiazepine receptor density.
Devic's neuromyelitis optica: A clinicopathological study of 8 patients
Authors: Mandler Raül N. , Davis Larry E. , Jeffery Douglas R. , Kornfeld Mario 
Published: January 2005

AbstractWe report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or the cerebellum affected, even after several years of disease. Various immunosuppressive treatments failed to benefit the patients, 5 of whom died. Autopsies of these 5 patients demonstrated a severe necrotizing myelopathy with thickening of blood vessel walls and no lymphocyte infiltrates. In the appropriate clinical setting, the lack of white matter abnormalities demonstrated by magnetic resonance imaging of the head facilitates the recognition of Devic's syndrome during life. Inasmuch as Devic's myelopathy is necrotizing, rather thatn demyelinating, the prognosis of this syndrome is poor.