bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intermittent obstructive hydrocephalus in the Arnold‐Chiari malformation
Authors: Vrabec Tamara R. , Sergott Robert C. , Savino Peter J. , Bosley Thomas M. 
Published: January 2005

AbstractA 26‐year‐old woman with a Chiari‐I malformation and intermittent symptoms of intracranial pressure elevation is described. Clinically, papilledema was accompanied by midperipheral retinal hemorrhages. Repeat computed tomographic scans and lumbar puncture showed normal results. Posterior fossa‐directed magnetic resonance image scanning revealed the Chiari‐I malformation. Intraventricular monitoring was necessary to demonstrate the marked but unsustained elevation of intracranial pressure.
Physostigmine treatment of progressive supranuclear palsy
Authors: Litvan Irene , Med Claudia Gomez , Atack John R. , Gillespie Marjorie , Kask Anne M. , Mouradian M. Maral , Chase Thomas N. 
Published: January 2005

AbstractCognitive and extrapyramidal effects of cholinomimetic therapy were evaluated in 8 patients with progressive supranuclear palsy. Each was randomized to a 10‐day double‐blind crossover trial of physostigimine and placebo. Physostigmine treatment was associated with marginal and inconsistent changes in long‐term memory, suggesting that cholinergic therapy alone is insufficient to restore cognitive function. Motor scores remained unchanged.
Selection bias in clinical trails of anaplastic glioma
Authors: Winger Michael J. , Macdonald David R. , Schold S. Clifford , Cairncross J. Gregory 
Published: January 2005

AbstractWe report a retrospective analysis of survival, and attrition to a study, of patients with anaplastic glioma seen at a regional cancer center during its participation in a prospective randomized clinical trial. Median length of survival for all patients was 35 weeks. Median length of survival for study patients was 60 weeks. Study patients were not representative of all patients; they were younger and less disabled than the nonstudy patients. Survival predictions for patients with anaplastic glioma based on the results of clinical trials may be overly optimistic. Many trials study a nonrepresentative subset of patients with anaplastic glioma, excluding those who do poorly at the outset and those who have poor function.
Anti–myelin‐associated glycoprotein IgM antibody titers in neuropathy associated with macroglobulinemia
Authors: Nobile‐Orazio E. , Francomano E. , Daverio R. , Barbieri S. , Marmiroli P. , Manfredini E. , Carpo M. , Moggio M. , Legname G. , Baldini L. , Scarlato G. 
Published: January 2005

AbstractTwenty‐seven patients with neuropathy and IgM monoclonal gammopathy were tested for antigen specificity of the M‐protein and for anti–myelin‐associated glycoprotein (MAG) IgM levels by immunoblot. In 16 patients (59.2%) the M‐protein reacted with MAG and with cross‐reactive glycoconjugates. Anti‐MAG IgM titers in these patients ranged between 1 : 12,800 and 1 : 100,000. A fainter IgM reactivity with MAG and related glycoconjugates was detected in 3 additional patients with neuropathy, but also in 8 of 24 patients with IgM M‐protein without neuropathy (33.3%). This reactivity was not due to the M‐protein and corresponded to antibody titers of 1:400 or less in all but 1 patient with a titer of 1 : 3,200. Low titers of anti‐MAG IgM (1 : 200 or less) were also detected in 17 of 101 control patients without IgM M‐proteins (16.8%), while 1 patient with neuropathy of unknown cause had anti‐MAG IgMk titers of 1 : 25,600. In 1 patient with neuropathy and IgM M‐protein that was not anti‐MAG, the M‐protein bound to other antigens in nerve, while in 6, other possible causes or mechanisms for the neuropathy were found. In this study, high titers of anti‐MAG IgM antibodies were always associated with neuropathy. The presence of low levels of anti‐MAG IgM in a significant proportion of controls suggests that monoclonal expansion of naturally occurring B‐cell clones secreting anti‐MAG IgM may be responsible for the high incidence of this antigen specificity of the M‐protein.
Striatal dopamine deficiency in parkinson's disease: Role of aging
Authors: Scherman Daniel , Desnos Claire , Darchen François , Pollak Pierre , Javoy‐Agid France , Agid Yves 
Published: January 2005

AbstractThe striatal dopaminergic innervation was investigated postmortem in 49 control and 57 parkinsonian brains by assessing the binding of tritiated α‐dihydrotetrabenazine ([3H]TBZOH), a specific ligand of the vesicular monoamine transporter. The density of [3H]TBZOH binding sites in the caudate nucleus of control subjects decreased significantly with age, suggesting an age‐dependent reduction in striatal dopamine innervation. In contrast, an increase with the age at time of death was observed in patients with Parkinson's disease, although the density of [3H]TBZOH binding sites was subnormal. Mean values represented 26.5% and 12.7% of control values in the caudate nucleus and in the putamen, respectively. The binding of [3H]TBZOH in the caudate nucleus decreased exponentially with the duration of Parkinson's disease. The rate of [3H]TBZOH binding decrease, an index of the rate of striatal dopaminergic denervation, was about twice as high in parkinsonian patients as in controls and was not related to the age at onset of the disease. The data suggest that (1) parkinsonian symptoms appear above a threshold degeneration state corresponding to 50% of the normal innervation at the age of 60, and (2) aging does not play a major role in the process of nigrostriatal neuron degeneration in Parkinson's disease.
Benzodiazepine receptors and diazepam‐binding inhibitor in human cerebral tumors
Authors: Ferrarese C. , Appollonio I. , Frigo M. , Gaini S. M. , Piolti R. , Frattola L. 
Published: January 2005

AbstractBenzodiazepines can regulate neoplastic growth and immune response through specific peripheral benzodiazepine receptors. We investigated the presence of peripheral and classic central benzodiazepine receptors as well as diazepam binding inhibitor, an endogenous ligand of both types of receptors, in different human cerebral tumors. Peripheral benzodiazepine receptors were present in all the tumor types studied, whereas central benzodiazepine receptors and diazepam‐binding inhibitor were detectable in astrocytomas and glioblastomas and undetectable in meningiomas, neurinomas, and metastases. The role of diazepam‐binding inhibitor and of the different benzodiazepine receptors in neoplastic cells is still to be defined.
Delayed onset of distal axonal neuropathy in primates after prolonged low‐level administration of a neurotoxin
Authors: Schaumburg H. H. , Arezzo J. C. , Spencer P. S. 
Published: January 2005

AbstractShort‐latency somatosensory evoked potentials were recorded from surface electrodes overlying peripheral nerve, spinal cord, and cortex in 4 monkeys during prolonged intoxication with low levels of acrylamide. A fifth animal served as a longitudinal control subject. Slowing of the response across the spinal‐medullary junction was a reliable sign, manifest only after prolonged exposure. Associated morphological changes were preterminal accumulation of axonal neurofilaments without synaptic disruption in the gracile nucleus. The induced alterations in the latency of shortlatency somatosensory evoked potentials and in axon morphology were reversible after 7 months of recovery. The extreme delay in onset of subtle neurological dysfunction (940 days) following administration of a presumed safe level of acrylamide suggests that permissible levels of human exposure to toxins of this type should be reassessed.
Peripheral nerve disorders in instrumentalists
Authors: Lederman Richard J. 
Published: January 2005

AbstractI evaluated 226 instrumentalists for playing‐related symptoms. There were 103 players (46%) of string instruments, 73 keyboard instrumentalists (32%), 44 players (19%) of wind instruments, and 6 percussionists (3%). The mean age was 32 years; 58% were women. Sixty‐five patients (29%) had a peripheral nerve disorder; 27 had symptoms of thoracic outlet syndrome; 12 had median neuropathies (carpal tunnel syndrome in 9); 9 had ulnar neuropathies; 6 had cervical radiculopathy; 5 had digital neuropathies; 3 had cranial neuropathies; and 3 had other peripheral nerve disorders. Treatment consisted of modification of playing schedules and technique, plus physical and occupational and therapy in 51 and surgical procedures in 9 patients; 5 were not treated. Follow‐up at 1 to 8 years showed 74% had a satisfactory result, 14percnt; had slight or minimal improvement, and 12percnt; had no improvement. At the time of writing, all but 4 patients remained in the music profession. It is concluded that playing‐related symptoms among instrumentalists are frequently neurological in origin and that the large majority can be helped by conservative or, on occasion, surgical means.
Positron emission tomography in manganese intoxication
Authors: Wolters E. Ch , Huang C.‐C. , Clark C. , Peppard R. F. , Okada J. , Chu N.‐S. , Adam M. J. , Ruth T. J. , Li D. , Calne D. B. 
Published: January 2005

AbstractWe employed 6‐fluorodopa to study the integrity of the nigrostriatal dopaminergic projection by positron emission tomography in 4 subjects with clinical features of mild parkinsonism caused by exposure to manganese. The 6‐fluorodopa scans were normal. This finding suggests that in early manganism sufficient to cause parkinsonian deficits, damage may occur in pathways postsynaptic to the nigrostriatal system, probably involving striatal or pallidal neurons. Fluorodeoxyglucose scans showed decreased cortical glucose metabolism, the significance of which is discussed.
Microtubular reorganization and dendritic growth response in alzheimer's disease
Authors: McKee Ann C. , Kowall Neil W. , Kosik Kenneth S. 
Published: January 2005

AbstractCytoskeletal disruption is a key pathological feature of Alzheimer's disease (AD). We used refined immunocytochemical techniques to define the range of abnormalities affecting the microtubule system in AD hippocampus. Minimal tau and tubulin immunoreactivity was granular and accumulated in otherwise normal neuronal perikarya. As tau‐reactive neurofibrillary tangles formed, granular tau and tubulin staining diminished, and ubiquitin reactivity developed. In regions of high neurofibrillary tangle density, microtubule‐associated protein 2 (MAP2) histochemical features of remaining nontangled neurons included apical dendritic degeneration with proliferation of basal dendrites. In addition to perisomatic dendritic proliferation, there was massive sprouting of tau‐immunoreactive distal dystrophic neurites. Sprouting proximal dendrites and dystrophic neurites often demonstrated growth‐cone‐like lamellipodia and filopodia. Degeneration of the perisomatic proliferating dendrites was characterized by the accumulation of fibrillar tau immunoreactivity. The colocalization of MAP2 and tau in growth structures recapitulated their codistribution in developing neurites. The data suggest that extensive plasticity and growth response occur in tandem with neuronal degeneration in AD, and that reorganization of the cytoskeletal microtubule system may underlie these proliferative changes.
Mitochondrial myopathies: Clinical and biochemical features of 30 patients with major deletions of muscle mitochondrial DNA
Authors: Holt I. J. , Harding A. E. , Cooper J. M. , Schapira A. H. V. , Toscano A. , Clark J. B. , Morgan‐Hughes J. A. 
Published: January 2005

AbstractAnalysis of mitochondrial DNA (mtDNA) in muscle and blood from 72 patients with mitochondrial myopathy showed that 30 had major deletions of a variable proportion of muscle mtDNA. All of these 30 patients presented with progressive external ophthalmoplegia and limb weakness, and 8 had the additional features of the Kearns‐Sayre syndrome. Of the 42 patients without detectable muscle mtDNA deletions, 10 had progressive external ophthalmoplegia and limb weakness, 2 had the Kearns‐Sayre syndrome, 11 had limb weakness without extraocular involvement and 19 had multisystem disorders predominantly affecting the central nervous system. Only 2 patients with mtDNA deletions had clinically affected relatives, compared with 10 of those without deletions. In the 4 patients with polarographic defects exclusively involving complex I (NADH coenzyme Q reductase), the deleted protein‐coding genes were confined to those for complex I subunits. Thirteen other patients with apparently identical deletions had variable clinical and biochemical features. Immunoblots of complex I polypeptides from patients with deletions were either indistinguishable from controls or showed only a mild generalized decrease in all identifiable subunits.
Pyruvate dehydrogenase complex deficiency: Biochemical and immunoblot analysis of cultured skin fibroblasts
Authors: Old Susan E. , de Vivo Darryl C. 
Published: January 2005

AbstractCultured skin fibroblasts were obtained from 11 children with lactic acidemia and neurological disturbances. The residual activities of pyruvate dehydrogenase complex were 9 to 45% of control values in all specimens. Immunoblot analysis of mitochondrial proteins using polyclonal antibodies against the alpha and beta subunits of the first component (E1) of the pyruvate dehydrogenase complex revealed markedly decreased amounts of cross‐reacting material in 4 boys who died in infancy. Two of the boys were half brothers related through a common mother. A fifth boy had an alteration of the electrophoretic mobility of the E1α subunit and normal E1β subunit abundance. The remaining 6 patients (2 boys and 4 girls) had normal findings on Western blot assay, and all 11 patients had normal E2 and E3 patterns. These findings suggest that the E1α subunit gene represents a genetically vulnerable site on the X chromosome. Decreased abundance of E1 components appears to be associated with death in infancy. A normal Western blot analysis is compatible with long‐term survival despite decreased catalytic activity of the pyruvate dehydrogenase complex.
Partial seizures with visual disturbance treated by radiotherapy of cavernous hemangioma
Authors: Lance James W. , Smee Robert I. 
Published: January 2005

AbstractA man aged 26 years had been subject to frequent transient episodes of left homonymous hemianopia, initially accompanied by visual hallucinations and left miosis, since the age of 8 years. These partial seizures recurred 3 to 30 times each month in spite of treatment with anticonvulsant medication. The causative lesion, which had escaped detection by cerebral angiography and repeated computed tomographic scanning, was shown by magnetic resonance imaging to be a small cavernous hemangioma in the right occipital lobe. This was treated by a course of fine‐beam radiotherapy directed to the lesion. After treatment the partial attacks ceased, although one nocturnal clonic seizure occurred 12 months after radiotherapy and 6 weeks after stopping all anticonvulsant medication.
Gm haplotypes in inflammatory demyelinating polyneuropathies
Authors: Feeney D. J. , Pollard J. D. , McLeod J. G. , Stewart G. J. , De Lange G. G. 
Published: January 2005

AbstractThe possible association of Gm haplotypes with inflammatory neuropathies has been studied in 59 patients with Guillain‐Barré syndrome and 55 patients with chronic inflammatory demyelinating polyradiculo‐neuropathy. The frequency of Gm haplotype 1,2,17;21 (or z,a,x;g) was significantly raised in the patients with Guillain‐Barré syndrome. These findings provide further evidence for an association of chromosome 14 genes with inflammatory neuropathies.
Neurophysiology of fastest voluntary muscle contraction in hereditary neuropathy
Authors: Logigian Eric L. , Hefter Harald H. , Reiners Karlheinz , Freund Hans‐Joachim 
Published: January 2005

AbstractIn patients with hereditary motor and sensory neuropathy types I (demyelinative) and II (neuronal) and in normal subjects isomatic force and electromyographic activity of the first dorsal interosseous muscle were recorded during fastest voluntary contractions and during twitches evoked by nerve stimulation. The maximum voluntary force of the first dorsal interosseous muscle was also measured. In patients, fastest voluntary contraction time (i.e., time from ooset of contraction to peak force) was prolonged and inversely proportional to maximum voluntary force. Maximum rate of rise of tension (i.e., slope of rise in force) was reduced and directly proportional to maximum voluntary force. In patients with hereditary motor and sensory neuropathy type I, contraction time was longer and the maximum rate of rise of tension was lower than in those with hereditary motor and sensory neuropathy type II. In patients and normal subjects, voluntary contraction time was closely correlated with the duration of electromyographic bursts. In patients, the twitch contraction time was prolonged and inversely proportional to maximum voluntary force. Twitch contraction amplitude was diminished and directly proportional to maximum voluntary force. Neither twitch contraction time nor amplitude were dependent on the type of hereditary motor and sensory neuropathy. Twitch contraction time evoked by proximal nerve stimulation was minimally longer than that evoked by distal stimulation. In fastest voluntary muscle contraction performed by patients with hereditary motor and sensory neuropathy, it is argued that: (1) Decrease in maximum rate of rise of tension is due to a reduced number of motor units, an increase in motor unit contraction time, and possibly lower motor unit firing rates in type I compared to type II neuropathy patients. (2) The central nervous system appears to adapt to a decreased rate of rise of tension by prolonging duration of the agonist electromyographic burst, thus lengthening contraction time. This may result in the higher peak forces than if burst duration were normal.
Immunoglobulins promote remyelination in the central nervous system
Authors: Rodriguez Moses , Lennon Vanda A 
Published: January 2005

AbstractRemyelination of central nervous system axons was promoted by systemic injection of serum immunoglobulin G (IgG) from donor mice hyperimmunized with homogenized spinal cord. In SJL/J mice infected with Theiler's virus, primary demyelination and inflammation in the spinal cord were extensive. Following treatment with IgG to spinal cord homogenate, new myelin synthesis as measured by quantitative morphometry increased sixfold. Cells in areas of remyelination that incorporated [3H]thymidine did not express differentiation markers of macrophages, oligodendrocytes, or astrocytes but were identified by electron microscopic autoradiography as progenitor glial cells. These findings raise the possibility that IgG secreted in demyelination lesions might have the protential to promote myelin repair.
Effects of seizures on learning, memory, and behavior in the genetically epilespy‐prone rat
Authors: Holmes Gregory L. , Thompson James L. , Marchi Teresa A. , Gabriel Pamela S. , Hogan Martha A. , Carl Frank G. , Feldman Daniel S. 
Published: January 2005

AbstractTo determine whether frequent seizures can cause deficits in learning and behavior, immature genetically epilepsyprone rats (GEPRs) were subjected to 66 audiogenic stimulations (Group 1). GEPR littermates were handled and placed in the sound chamber but were not stimulated (Group 2). Group 3 comprised genetically epilepsy‐resistant rats (GERRs) who received audiogenic stimulations but had no seizures. After 3 weeks of stimulations the rats were tested for learning, memory, and behavior using the T‐maze, water maze, open field activity test, home cage intruder test, and handling test. When compared with the control GEPRs and GERRs, Group 1 rats reached criteria less frequently in the T‐maze, required longer times to find the platform in the water maze, and were active in the open field activity test, less aggressive in the home cage intruder test, and more irritable and aggressive in the handling test. This study demonstrates that frequent, brief seizures in immature animals result in significant detrimental changes in learning, memory activity level, and behavior.
Activation of the epileptic focus by transcranial magnetic stimulation of the human brain
Authors: Hufnagel A. , Elger C. E. , Durwen H. F. , Böker D. K. , Entzian W. 
Published: January 2005

AbstractTo establish whether transcranial magnetic stimulation is able to activate the primary epileptic focus preferntially, 13 patients who had medically intractable complex partial seizures were examined prior to surgical therapy. Single or a series of magnetic stmuli were applied to various regions of the skull. The effects of transcranial magnetic stimulation were monitored via subdurally implanted electrodes. In the process of presurgical evaluation, the dosage of anticonvulsant medication had been reduced in all patient but one. Transcranial magnetic stimulation was able to activate the epileptic focus (or foci) in 12 of the 13 patients. Distinct patterns of focal activation were observed in 3 patients who had several foci. No epileptiform potentials were induced outside epileptic foci, which had been identified by corticographic recordings. In one patient a complex partial seizure that was induced was identical to her habitual seizures. In another patient, a complete transition from a nonactive theta focus to a self‐sustained epileptic focus occurred. A facilitation of epileptiform afterdischarge was seen with sequential stimulation. No adverse effects were either reported by the patients or observed by the investigators. In summary transcranial magnetic stimulation is able to activate the epileptic focus (or foci) and consequently may be an additional tool for the localization of epileptic foci in presurgical evaluation.
Opioid and nonopioid mechanisms may contribute to dynorphin's pathophysiological actions in spinal cord injury
Authors: Faden Alan I. 
Published: January 2005

AbstractIt has been suggested that the opioid dynorphin, an endogenous agonist for k‐opiate receptors, contributes to secondary tissue damage after spinal cord injury. To evaluate this hypothesis further, effects of intrathecally administered dynorphin (Dyn) A‐(1‐17), dynorphin antiserum, or the k‐selective opiate antagonist nor‐binaltorphimine (nor‐BNI) were studied in rats subjected to standardized impact trauma to the thoracic spinal cord. Effects of intrathecal Dyn A‐(1–17) were also compared to those of Dyn A‐(2–17), which is inactive at opiate receptors, in uninjured and injured animals. Both Dyn A‐(1–17) and Dyn A‐(2‐17) produced motor dysfunction in uninjured rats, but Dyn A‐(1–17) was approximately 2.5 times more potent. At lower doses of Dyn A‐(1–17), paraparesis was markedly attenuated by nor BNI; nor‐BNI was less effective at higher doses of Dyn A‐(1–17) and did not modify the motor dysfunction produced by Dyn A‐(2–17). Treatment with dynorphin antiserum significantly improved outcome after trauma as compared to control treatment with normal rabbit serum or leucine‐enkephalin antiserum. Dyn A‐(1–17), but not Dyn A‐(2–17) at similar doses, exacerbated neurological dysfunction after spinal cord injury. Pretreatment with nor‐BNI attenuated neurological dysfunction after traumatic spinal cord injury to a similar degree in rats administered saline or Dyn A‐(1–17). These observations support the hypothesis that dynorphin contributes to certain pathophysiological changes after traumatic spinal cord injury through both opiate‐receptor (k‐receptor)–mediated and nonopioid mechanisms.