bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mendelian etiologies of stroke
Authors: Natowicz Marchvin , Kelley Richard I. 
Published: January 2005

AbstractThere are many genetic disorders associated with an increased risk for stroke that may easily be overlooked in the valuation of both adult and pediatric acute stroke victims. The recognition of a genetic disorder as the cause of a stroke has important implications not only for the immediate care of the stroke victim, but often also for others in the patient's family who may be at risk for the same disease and for whom preventive measures sometimes can be taken. We present here a comprehensive review of genetic disorders associated with stroke in the nongeriatric age groups for which a causative role in the evolution of stroke has been recognized or is likely. For each disorder, the major clinical and biochemical characteristics as well as the probable pathogenetic mechanisms of stroke are discussed, together with the appropriate testing required to screen for and confirm the diagnosis. The great variety of genetic disorders and mechanisms causing stroke underscores the increasing importance of understanding genetic disease for appropriate diagnosis and treatment of a common clinical problem affecting both children and adults.
Cholinesterases within neurofibrillary tangles related to age and Alzheimer's disease
Authors: Mesulam M‐Marsel , Asuncion Morán M. 
Published: January 2005

AbstractEnzyme histochemistry showed that a substantial number of cortical neurofibrillary tangles contain acetylcholinesterase and butyrylcholinesterase activities in patients with Alzheimer's disease and in nondemented aged individuals. In some areas of the brain, tangles containing each of these two enzymes were differentially distributed. In other areas, there was a very close overlap between the two types of cholinesterase.
Brain choline acetyltransferase reduction in dominantly inherited olivopontocerebellar atrophy
Authors: Kish Stephen J. , Currier Robert D. , Schut Lawrence , Perry Thomas L. , Morito Caryl L. 
Published: January 2005

AbstractWe measured the activity of choline acetyltransferase, the cholinergic marker enzyme, in the brains of 17 patients from five established pedigrees with dominantly inherited olivopontocerebellar atrophy (OPCA). OPCA is a group of cerebellar ataxia disorders in which serious intellectual impairment is not typically considered to be an accompanying feature. Patients from all five pedigrees demonstrated markedly reduced choline acetyltransferase activity in the cerebral cortex, with less severe changes in the hippocampus. Although the magnitude of the cortical choline acetyltransferase deficit is comparable to that seen in the brains of patients with Alzheimer's disease, none of our OPCA patients appeared, on last examination, to have severe global dementia of the Alzheimer type. Determination of the clinical significance of our biochemical data must await the results of studies in which the cognitive status of OPCA individuals has been accurately assessed.
Transplantation in Parkinson's disease: Two cases of adrenal medullary grafts to the putamen
Authors: Lindvall Olle , Backlund Erik‐Olof , Farde Lars , Sedvall Göran , Freedman Robert , Hoffer Barry , Nobin Anders , Seiger ÅKe , Olson Lars 
Published: January 2005

AbstractAutologous adrenal medullary tissue was transplanted unilaterally to the putamen in two patients with severe Parkinson's disease. The first patient exhibited a transient, two‐day improvement of motor performance in the limbs contralateral to the implantation site. He also had significantly longer episodes of normal function for about two months. The second patient reported a minor improvement of balance and gait, again lasting for two months. Electrophysiological studies of the motor readiness and auditory evoked potentials were consistent with increased catecholaminergic activity in the basal ganglia after transplantation in both patients. Positron emission tomography showed no postoperative alteration of receptor density in the putamen. No significant adverse effects of the transplantation were observed in the patients' performance on neurological and psychological tests or in their quantitative electroencephalogram and cerebral blood flow recordings. Immediately after the grafting, one patient showed transient signs of sympathetic hyperactivity, probably caused by release of catecholamines from the implanted tissue into the peripheral circulation. We conclude that catecholamine‐rich cellular implants in the basal ganglia have transient beneficial effects in patients with severe Parkinson's disease.
Motor fluctuations in Parkinson's disease: Pathogenetic and therapeutic studies
Authors: Mouradian M. M. , Juncos J. L. , Fabbrini G. , Chase T. N. 
Published: January 2005

AbstractThe response to continuous intravenous infusion of levodopa was evaluated in 23 patients with Parkinson's disease complicated by motor fluctuations. Conversion from oral to intravenous levodopa treatment resulted in an immediate and sustained stabilization of plasma levodopa levels in both the wearing‐off and on‐off groups. Motor variability also diminished within the first 24 hours of infusion, although to a much greater extent in patients with the wearing‐off phenomenon. Over the next 5 to 11 days of intravenous treatment, further reductions in motor fluctuations occurred in both groups, at a substantially faster rate in the wearing‐off group than in the on‐off group. The degree of immediate stabilization of parkinsonian motor response correlated best with disease duration, whereas the rate of subsequent improvement correlated most closely with levodopa dose. The results support the view that the wearing‐off phenomenon may reflect the degeneration of dopamine terminals as a consequence of natural disease progression with a resultant inability to buffer variations in levodopa availability; the on‐off phenomenon may reflect additional postsynaptic changes, possibly occurring in response to the nonphysiological fluctuations in synaptic dopamine, which gradually remit during continuous levodopa administration.
Magnetic resonance imaging in multiple sclerosis: Decreased signal in thalamus and putamen
Authors: Drayer Burton P. , Burger Peter , Hurwitz Barrie , Dawson Deborah , Cain John , Leong Jackie , Herfkens Robert , Allan Johnson G. 
Published: January 2005

AbstractHigh‐field strength (1.5 Tesla) magnetic resonance imaging in 15 patients with multiple and extensive white‐matter lesions and clinically definite multiple sclerosis delineated a previously underscribed finding of abnormally Decemberreased signal intensity on T2‐weighted images in the thalamus and putamen. The Decemberreased signal intensity (preferential Decemberreased T2 relaxation time) is most likely to be related to abnormally increased iron accumulation causing local magnetic field heterogeneities.
Abnormal glutamate metabolism in amyotrophic lateral sclerosis
Authors: Plaitakis Andreas , Caroscio James T. 
Published: January 2005

AbstractGlutamate levels were determined in the fasting plasma of 22 patients with early‐stage primary amyotrophic lateral sclerosis (ALS) and compared to those of healthy and diseased controls. There was a significant increase (by approximately 100%, p < 0.0005) in the plasma glutamate of the ALS patients as compared with the controls. Oral glutamate loading (60 mg of monosodium glutamate per kilogram of body weight, taken orally after overnight fasting) resulted in significantly greater elevations in the plasma glutamate and aspartate levels in the ALS patients than in the controls. Glutamate, a potentially neuroexcitotoxic compound, is thought to be the transmitter of the corticospinal tracts and certain spinal cord interneurons. A systemic defect in the metabolism of this amino acid may underlie primary ALS.
The presence of immunoreactive myelin basic protein peptide in urine of persons with multiple sclerosis
Authors: Whitaker John N. 
Published: January 2005

AbstractA polyclonal antiserum has been produced that can detect nanogram amounts of myelin basic protein (MBP)–like material in unconcentrated human urine. The urinary immunoreactive material is cross‐reactive with human MBP peptides 45–89 and 69–89, dialyzable, heat resistant, and is not an artifact of either degradation of radioligand or salt effect. An octapeptide, MBP peptide 82–89, was demonstrated to be the smallest peptide containing the main epitope against which this antiserum was directed. This epitope differed from the major epitope recognized by antisera detecting MBP‐like material in cerebrospinal fluid, implying that the MBP‐like material is altered, presumably degraded, in the kidney. Results of gel filtration and high‐performance liquid chromatography suggested a size of 1,000 daltons or less and a charge similar to that of human MBP peptide 80–89. In a group of 39 persons with multiple sclerosis, 48 with other neurological diseases, and 26 normal control subjects, the concentration of urinary MBP‐like material, related to the concentration of urinary creatinine, was significantly higher in the multiple sclerosis group (22.0 ng MBP‐like material/mg creatinine) than in the other neurological diseases or control groups, in which the values were 7.0 and 3.9 ng MBP‐like material/mg creatinine, respectively. Variations in the level of MBP‐like material appearing in the urine may provide a clinically feasible test for myelin damage. The precise identification of the chemical nature of the urinary MBP‐like material may also furnish a means for further analyzing the in vivo catabolism of the potentially autoantigenic MBP.
Magnetic brain stimulation: Central motor conduction studies in multiple sclerosis
Authors: Hess Christian W. , Mills Kerry R. , Murray Nicholas M. F. , Schriefer Thomas N. 
Published: January 2005

AbstractCentral motor conduction (CMC) was evaluated in 32 normal subjects and 83 patients with multiple sclerosis, and the findings were correlated with clinical signs and evoked potential data. CMC time was obtained from the latency difference in responses from the abductor muscle of the little finger to magnetic stimulation of the motor cortex and electrical stimulation at the C‐7/T‐1 interspace. Mean CMC time in normal subjects was 6.2 msec (SD 0.86 msec), and amplitudes of responses to cortical stimuli were at least 18% of those obtained with stimuli at the wrist. CMC was abnormal in 60 patients with multiple sclerosis (72%); this correlated well with brisk finger flexor jerks (p < 0.005). CMC was abnormal in 79% of patients with weakness of the abductor muscle of the little finger and in 54% with a normal muscle. Neurological examination was normal in 7 arms with abnormal CMC. Visual evoked potentials were abnormal in 67%, somatosensory evoked potentials in 59%, and brainstem auditory evoked potentials in 39% of those tested. For each procedure more subjects had abnormal CMC and normal evoked potentials than the reverse. The technique is of value for demonstrating and documenting central motor pathway lesions in multiple sclerosis, especially when physical signs are equivocal.
Dystonia in parkinson's disease: Clinical and pharmacological features
Authors: Poewe W. H. , Lees A. J. , Stern G. M. 
Published: January 2005

AbstractWe studied the features of dystonia in 9 patients with untreated idiopathic Parkinson's disease and in 56 patients on sustained treatment with L‐dopa. Dystonia was seen as an initial symptom in patients with both early‐ and late‐onset Parkinson's disease and included action dystonia of the limbs and cranial dystonia. Although the coexistence of parkinsonism and dystonia suggests a common pathophysiology, antiparkinsonian drugs did not consistently influence dystonic spasms. L‐dopa‐induced dystonia was seen as an off‐period, biphasic, or peak‐dose phenomenon. Each type showed a distinctive pattern of localization of dystonic spasms, possibly reflecting neurochemical aspects of basal ganglia somatotopy. Neuropharmacological studies performed in 12 patients suggest that off‐period dystonia is genuinely induced by L‐dopa and best relieved by antiparkinsonian agents.
Clinical, pathological, and neurochemical changes in dementia: A subgroup with preserved mental status and numerous neocortical plaques
Authors: Katzman Robert , Terry Robert , DeTeresa Richard , Brown Theodore , Davies Peter , Fuld Paula , Renbing Xiong , Peck Arthur 
Published: January 2005

AbstractPostmortem examination was performed on 137 residents (average age 85.5 years) of a skilled nursing facility whose mental status, memory, and functional status had been evaluated during life. Seventy‐eight percent were demented using conservative criteria; 55% had characteristic Alzheimer's disease. Choline acetyltransferase and somatostatin were significantly reduced in the brains of patients with Alzheimer's disease as compared with age‐matched nursing home control subjects, although the degree of the reduction was less severe than found in subjects less than 80 years of age. Ten subjects whose functional and cognitive performance was in the upper quintile of the nursing home residents, as good as or better than the performance of the upper quintile of residents without brain pathology (control subjects), showed the pathological features of mild Alzheimer's disease, with many neocortical plques. Plaque counts were 80% of those of demented patients with Alzheimer's disease. Choline acetyltransferase and somatostatin levels were intermediate between controls and demented patients with Alzheimer's disease. The unexpected findings in these subjects were higher brain weights and greater number of neurons (> 90μm2 in a cross‐sectional area in cerebral cortex) as compared to age‐matched nursing home control subjects. These people may have had incipient Alzheimer's disease but escaped loss of large neurons, or alternatively, started with larger brains and more large neurons and thus might be said to have had a greater reserve.
Motor unit synchronization in physiologic, enhanced physiologic, and voluntary tremor in man
Authors: Logigian Eric L. , Wierzbicka Margaret M. , Bruyninckx Francis , Wiegner Allen W. , Shahahi Bhagwan T. , Young Robert R. 
Published: January 2005

AbstractSynchronization between pairs of single motor units simultaneously recorded from wrist extensor muscles was quantitated in 3 normal subjects during physiologic tremor (PT), beta‐adrenergically enhanced physiologic tremor (EPT), and fast voluntary wrist flexion‐extension movements mimicking tremor (VT). Cross‐correlation histograms generated from the two spike trains of each motor unit pair demonstrated central or paracentral peaks in 13/19 recording during PT, 22/36 during EPT, and 6/7 during VT. Relative peak area was used as a quantitative index of synchronization between the two motor units of each pair. It was lowest in PT, progressively increased in EPT as tremor amplitude increased, and highest in VT. In PT and lower amplitude EPT, the synchronization indexes were higher between motor units that discharged at the same or nearly the same frequency. In contrast, in higher amplitude EPT and VT, motor units with different firing frequencies were sometimes strongly synchronized as a consequence of double discharges in faster‐firing motor units that had burst repetition rates in the range of slower‐firing motor units discharging as singlets. Greater motor unit synchronization with increasing tremor amplitude in EPT may be secondary to a simultaneous increase in muscle spindle afferent activity from the tremulous muscle. Greatest synchronization in VT presumably reflects near maximal supraspinal and segmental common synaptic input onto motoneurons that generate VT. These results support a longstanding hypothesis that synchronization of motor units is the physiological basis for higher amplitude tremor.
A new model of embolic stroke produced by photochemical injury to the carotid artery in the rat
Authors: Futrell Nancy , Watson Brant D. , Dietrich W. Dalton , Prado Ricardo , Millikan Clark , Ginsberg Myron D. 
Published: January 2005

AbstractWe report a new model of embolic stroke in the rat, based on endothelial disruption and platelet aggregation in the carotid artery, which leads to distal embolization and focal brain infarction. The common carotid artery was irradiated for 6.5 minutes with the focused beam of an argon laser operated at a wavelength of 514.5 nm with a peak power of 2 W and an average power of 400 mw. Ipsilateral cerebral infarcts, ranging in size from 0.1 to 1.7 mm, were produced by platelet emboli in 12 of 13 rats. A total of 44 infarcts were observed in the 12 rats: 29 in the cortex, 6 in the hippocampus, 5 in the thalamus, and 4 in the basal ganglia. Scanning electron microscopy identified platelet aggregates in the carotid artery and in a deep cortical arteriole 50 minutes after the photochemical lesion. Twenty‐four hours after the experiment, scanning electron microscopy of the carotid artery revealed damaged endothelium but few remaining adherent platelets. More intense laser irradiation in 8 rats, leading to carotid occlusion, produced an infarct in only 1. This new model can be used to study the acute and chronic pathological changes in the brain associated with platelet embolism.
Cerebrospinal fluid antibodies to myelin basic protein in acute idiopathic optic neuritis
Authors: Warren Kenneth G. , Catz Ingrid , Bauer Christopher 
Published: January 2005

AbstractFree and bound levels of anti‐myelin basic protein (anti‐MBP) antibodies were measured by radioimmunoassay in the cerebrospinal fluid of 20 patients with acute idiopathic optic neuritis, 133 patients with multiple sclerosis (MS) divided into three clinical subgroups, and 76 normal control subjects. Patients with idiopathic optic neuritis had elevated levels of anti‐MBP predominantly in free form, resulting in an elevated (above unity) free/bound anti‐MBP ratio similar to that of MS patients with acute relapses. These data suggest that acute idiopathic optic neuritis, like active MS, is associated with anti‐MBP.
The role of macrophages in experimental autoimmune neuritis induced by a P2‐Specific T‐Cell line
Authors: Heininger Kurt , Schäfer Bärbel , Hartung Hans‐Peter , Fierz Walter , Linington Christopher , Toyka Klaus V. 
Published: January 2005

AbstractA P2‐specific T‐cell line with a helper/inducer phenotype (W3/25+) mediates experimental autoimmune neuritis in the Lewis rat after adoptive transfer to naive recipients. Moderately severe disease was induced in these experiments by the injection of 1 × 107 T cells. Motor and mixed afferent nerve conduction, F responses, H reflexes, and lumbar somatosensory evoked potentials were monitored, and morphological alterations were scored semiquantitatively at the end of the experiments. The role of macrophages and macrophage‐derived inflammatory mediators in the effector phase of the disease was investigated by administering different inhibitors of macrophage metabolism, including silica, dexamethasone, and a variety of cyclooxygenase and lipoxygenase blockers. Silica and dexamethasone suppressed the clinical, electrophysiological, and morphological manifestations of the disease almost completely, indicating that macrophages are essential for the generation of inflammatory lesions. The inhibitors of arachidonic acid conversion failed to mitigate the severity of the disease. This is in contrast to observations in actively induced experimental autoimmune neuritis in which eicosanoid biosynthesis seems to play a decisive role in the pathogenesis of the disease.
Central pontine myelinolysis after rapid correction of hyponatremia: A magnetic resonance imaging study
Authors: Brunner John E. , Redmond Janice M. , Haggar Allan M. , Elias Stanton B. 
Published: January 2005

AbstractWe describe a patient with severe hyponatremia (serum sodium 94 mmol/L) who developed encephalopathy and decorticate posturing after a 29 mmol/L rise in serum sodium concentration during the first 24 hours of correction. High‐resolution computed tomography of the pons was normal during the first, second, and twelfth weeks of the illness. Subsequent magnetic resonance imaging revealed a pontine lesion consistent with central pontine myelinolysis.
Inflammatory myelinoclastic diffuse sclerosis
Authors: Mehler Mark F. , Rabinowich Lydia 
Published: January 2005

AbstractWe report on a 12‐year‐old girl with a severe subacute to chronic bifrontal leukoencephalopathy. By clinical, biochemical, radiological, and neuropathological criteria, a diagnosis of inflammatory myelinoclastic diffuse sclerosis was reached. This is the third fully documented case.
Magnetic resonance imaging in amyotrophic lateral sclerosis
Authors: Goodin Douglas S. , Rowley Howard A. , Olney Richard K. 
Published: January 2005

AbstractMagnetic resonance imaging (MRI) was used in 5 patients with clinically definite amyotrophic lateral sclerosis to determine the frequency of central white matter abnormalities. Two patients had symmetrical areas of increased signal intensity seen on MRI extending from the cortex, through the corona radiata, posterior limb of the internal capsule, and cerebral peduncles into the pons. These MRI abnormalities presumably relate to the pathological changes observed by others in the central white matter of patients with amyotrophic lateral sclerosis.
Diagnostic criteria for rett syndrome
Authors:   The Rett Syndrome Diagnostic Criteria Work Group
Published: January 2005

AbstractDiagnostic criteria for Rett syndrome (RS) were developed by representatives of the International Rett Syndrome Association and the Centers for Disease Control for use in future clinical and epidemiological studies. Necessary criteria are: normal prenatal and perinatal period; normal psychomotor development through the first 6 months of life; normal head circumference at birth, with subsequent deceleration of head growth; loss of purposeful hand skills; severely impaired expressive and receptive language; apparent severe mental retardation; and gait apraxia and truncal apraxia/ataxia. Supportive criteria include breathing dysfunction, seizures, spasticity, scoliosis, and growth retardation. The diagnosis of RS in considered tentative until 2 to 5 years of age. The differential diagnosis includes other disorders associated with mental retardation, cerebral palsy, and seizure disorders. These diagnostic criteria for RS should foster reliable communication among investigators and enhance the epidemiological and clinical research of this important disorder.
Duodenal and gastric delivery of levodopa in parkinsonism
Authors: Kurlan Roger , Nutt John G. , Woodward William R. , Rothfield Kenneth , Lichter David , Miller Charlyne , Carter Julie H. , Shoulson Ira 
Published: January 2005

AbstractTo clarify the influence of gastric emptying on levodopa‐related motor fluctuations in Parkinson's disease, we assessed mobility and plasma levodopa concentrations in 10 patients during five modes of levodopa administration: (1) standard intermittent oral (SIO), (2) intermittent duodenal (ID), (3) continuous duodenal infusion (CDI), (4) continuous gastric infusion (CGI), and (5) controlled‐release Sinemet (CR‐4). The rank order from greatest to least for both percentage of time “on” and average mobility score was CDI, CGI, ID, CR‐4, and SIO. The rank order for variance of means, a measure of fluctuation, from least to greatest for mobility was CDI, CGI, CR‐4, ID, SIO, and for plasma levodopa concentrations was CDI, CGI, ID, SIO, and CR‐4. The results demonstrate that it is possible to produce very steady plasma concentrations of levodopa with a corresponding reduction in motor fluctuations by continuous intraduodenal administration of the drug. This mode of delivery is an ideal model for the development of optimal continuous‐release preparations of levodopa. Other enteral routes have produced a more variable plasma levodopa concentration and clinical response.